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Jacob D, Babu S, Gopakumar G, Gadhinglajkar SV. Multiple Levels of Obstruction at Atrial Level After Senning Procedure Conversion to Arterial Switch Operation: Role of Intraoperative Transesophageal Echocardiography. A A Pract 2025; 19:e01929. [PMID: 39969043 DOI: 10.1213/xaa.0000000000001929] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/20/2025]
Affiliation(s)
- Divya Jacob
- From the Division of Cardiothoracic and Vascular Anesthesia, Department of Anesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India
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Kormányos Á, Gyenes N, Ruzsa Z, Achim A, Ambrus N, Havasi K, Vámos M, Szili-Török T, Lengyel C, Nemes A. Three-dimensional speckle-tracking echocardiography-derived deformation analysis of the morphologic left ventricle in adults with corrected dextro-transposition of the great arteries-insights from the CSONGRAD Registry and MAGYAR-Path Study. Cardiovasc Diagn Ther 2024; 14:1038-1047. [PMID: 39790193 PMCID: PMC11707480 DOI: 10.21037/cdt-24-266] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2024] [Accepted: 11/15/2024] [Indexed: 01/12/2025]
Abstract
Background Dextro-transposition of the great arteries (dTGA) stands out as a prevalent cyanotic congenital heart defect (CHD), characterized by an intricate reversal in the arrangement of the major arteries. In the past, several surgical procedures have been used to treat dTGA, including the atrial switch. Although the method is no longer used, survivors of the procedure still living among us. Recent advancements in cardiovascular imaging have led to the emergence of several novel echocardiographic techniques, notably three-dimensional (3D) and/or speckle-tracking echocardiography (STE). The present study aimed to employ 3DSTE to determine morphologic left ventricle (mLV) strain parameters in adults with dTGA who underwent Senning or Mustard procedure at infancy. Furthermore, it was also aimed to assess whether the type of correction procedure had any impact on mLV deformation parameters. Methods Eleven dTGA patients, with a mean age of 28.8±8.5 years (6 males, 6 Senning- and 5 Mustard-operated patients) were enrolled. They were compared to 34 healthy controls matched for age and gender (age: 35.7±12.8 years, 21 males). All subjects underwent complete two-dimensional (2D) Doppler echocardiography with 3DSTE data acquisition as per recent guidelines. Results Comparing all dTGA patients to the control group no mean segmental or global mLV strains showed significant differences, however out of the regional strains, midventricular mLV longitudinal strain was significantly better (higher) in dTGA compared to the healthy group (-16.8%±7.4% vs. -13.3%±2.5%, P<0.05). The Mustard-operated patients showed significantly worse (lower) global mLV circumferential strain compared to that of controls (-22.1%±12.4% vs. -28.9%±4.7%, P=0.05). Out of the regional strains the Mustard procedure group had a significantly worse (lower) apical mLV circumferential strain (-24.8%±11.9% vs. -35.6%±9.0%, P=0.05), better (higher) midventricular mLV longitudinal strain (-20.1%±9.2% vs. -13.3%±2.5%, P=0.04) and a worse (lower) apical mLV area strain (-36.3%±17.0% vs. -48.8%±9.7%, P=0.03). Conclusions Significant mLV strain abnormalities are present in dTGA late after atrial switch procedures. These differences are more pronounced in the Mustard-operated group.
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Affiliation(s)
- Árpád Kormányos
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Nándor Gyenes
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Zoltán Ruzsa
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Alexandru Achim
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Nóra Ambrus
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Kálmán Havasi
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Máté Vámos
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Tamás Szili-Török
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Csaba Lengyel
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Attila Nemes
- Department of Medicine, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
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Cedars A, Jacobs ML, Gottlieb-Sen D, Jacobs JP, Alejo D, Habib RH, Parsons N, Tompkins BA, Mettler B. Reoperations in Adolescents and Adults After Prior Arterial Switch Operation: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis. Ann Thorac Surg 2024; 118:1080-1087. [PMID: 38878952 DOI: 10.1016/j.athoracsur.2024.05.038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2024] [Revised: 04/18/2024] [Accepted: 05/13/2024] [Indexed: 07/14/2024]
Abstract
BACKGROUND Arterial switch operation (ASO) has supplanted physiologic repairs for transposition of the great arteries and related anomalies. As survival rates have increased, so has the potential need for cardiac reoperations to address ASO-related complications arising later in life. METHODS The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2021) was reviewed to assess prevalence and types of cardiac reoperations for patients aged ≥10 years with prior ASO for transposition of the great arteries or double-outlet right ventricle/transposition of the great arteries type. A hierarchical stratification designating 13 procedure categories was established a priori by investigators. Each eligible surgical hospitalization was assigned to the single highest applicable hierarchical category. Outcomes were compared across procedure categories, excluding hospitalizations limited to pacemaker-only and mechanical circulatory support-only procedures. Variation during the study period in relative proportions of left heart vs non-left heart procedure category encounters was assessed. RESULTS There were 698 cardiac surgical hospitalizations for patients aged 10 to 35 years at 100 centers. The most common left heart procedure categories were aortic valve procedures (n = 146), aortic root procedures (n = 117), and coronary artery procedures (n = 40). Of 619 hospitalizations eligible for outcomes analysis, major complications occurred in 11% (67/619). Discharge mortality was 2.3% (14/619). Year-by-year analysis of surgical hospitalizations reveals substantial growth in numbers for the aggregate of all procedure categories. Growth in relative proportions of left heart vs non-left heart procedures was significant (P = .0029; Cochran-Armitage trend test). CONCLUSIONS This large multicenter study of post-ASO reoperations beyond early childhood documents year-over-year growth in total reoperations. Left-sided heart procedures recently had the highest rate of rise. These observations have implications for counseling, surveillance, and management.
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Affiliation(s)
- Ari Cedars
- Division of Cardiology, Department of Internal Medicine, Johns Hopkins University, Baltimore, Maryland.
| | - Marshall L Jacobs
- Division of Cardiothoracic Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Maryland
| | - Danielle Gottlieb-Sen
- Division of Cardiothoracic Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Maryland
| | - Jeffrey P Jacobs
- Division of Cardiothoracic Surgery, Department of Surgery, University of Florida, Gainesville, Florida
| | - Diane Alejo
- Division of Cardiothoracic Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Maryland
| | - Robert H Habib
- The Society of Thoracic Surgeons Research and Analytic Center, Chicago, Illinois
| | - Niharika Parsons
- The Society of Thoracic Surgeons Research and Analytic Center, Chicago, Illinois
| | - Bryon A Tompkins
- Division of Cardiothoracic Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Maryland
| | - Bret Mettler
- Division of Cardiothoracic Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Maryland
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Zubrzycki M, Schramm R, Costard-Jäckle A, Morshuis M, Gummert JF, Zubrzycka M. Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II. J Clin Med 2024; 13:4823. [PMID: 39200964 PMCID: PMC11355351 DOI: 10.3390/jcm13164823] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2024] [Revised: 08/04/2024] [Accepted: 08/13/2024] [Indexed: 09/02/2024] Open
Abstract
Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5-7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood.
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Affiliation(s)
- Marek Zubrzycki
- Department of Surgery for Congenital Heart Defects, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany;
| | - Rene Schramm
- Clinic for Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany; (R.S.); (A.C.-J.); (M.M.); (J.F.G.)
| | - Angelika Costard-Jäckle
- Clinic for Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany; (R.S.); (A.C.-J.); (M.M.); (J.F.G.)
| | - Michiel Morshuis
- Clinic for Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany; (R.S.); (A.C.-J.); (M.M.); (J.F.G.)
| | - Jan F. Gummert
- Clinic for Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany; (R.S.); (A.C.-J.); (M.M.); (J.F.G.)
| | - Maria Zubrzycka
- Department of Clinical Physiology, Faculty of Medicine, Medical University of Lodz, Mazowiecka 6/8, 92-215 Lodz, Poland
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Sasaki K, Nakajima I, Kasagawa A, Harada T, Akashi YJ. Helix-fixation leadless pacemaker as a potential alternative to conventional transvenous pacemaker in post-Mustard baffle stenosis. J Arrhythm 2024; 40:1041-1044. [PMID: 39139894 PMCID: PMC11317723 DOI: 10.1002/joa3.13108] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2024] [Accepted: 06/18/2024] [Indexed: 08/15/2024] Open
Abstract
Obstruction of a systemic venous pathway is relatively common after the Mustard operation. A helix-fixation leadless pacemaker was successfully implanted in the subpulmonic but morphologic LV in a d-TGA patient with post-Mustard baffle stenosis and failure of a previously implanted epicardial lead.
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Affiliation(s)
- Kenichi Sasaki
- Division of Cardiology, Department of Internal MedicineSt. Marianna University School of MedicineKawasakiJapan
| | - Ikutaro Nakajima
- Division of Cardiology, Department of Internal MedicineSt. Marianna University School of MedicineKawasakiJapan
| | - Akira Kasagawa
- Division of Cardiology, Department of Internal MedicineSt. Marianna University School of MedicineKawasakiJapan
| | - Tomoo Harada
- Division of Cardiology, Department of Internal MedicineSt. Marianna University School of MedicineKawasakiJapan
| | - Yoshihiro J. Akashi
- Division of Cardiology, Department of Internal MedicineSt. Marianna University School of MedicineKawasakiJapan
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Shaji S, Alsaied T, Saraf A, Hoskoppal A, Olivieri L, Christopher A. T1 mapping: a non-invasive tool to assess the systemic right ventricle. Int J Cardiovasc Imaging 2024; 40:1779-1785. [PMID: 38949675 PMCID: PMC11819913 DOI: 10.1007/s10554-024-03168-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2024] [Accepted: 06/22/2024] [Indexed: 07/02/2024]
Abstract
Ventricular remodeling leads to fibrotic changes in systemic right ventricles (RV). Native T1 mapping provides a quantitative measure in myocardial tissue characterization. The aim of our study was to correlate native T1 values of the systemic RV to function and volumetric data. Native T1 maps were generated with a single breath hold Modified Look-Locker Inversion-recovery pulse (MOLLI) sequence was acquired in the mid-ventricular short axis. Regions of interest (ROI) were drawn in both ventricular free walls, the interventricular septum (IVS), superior insertion point (SIP) and inferior insertion point (IIP) to obtain native T1 values. T1 values were compared to CMR ventricular volumes and function using Spearman correlation. The median age was 36 years (IQR 27-48 years). There were elevated mean native left ventricular (LV) T1 and IIP T1 values at 1122 ± 171 ms and 1117 ± 96 ms, respectively. RV dysfunction was associated with elevated IIP T1 (p = 0.007). Significant moderate negative correlations were seen between RV T1 and LV ejection fraction (LVEF) (r= -0.63, p = 0.01), between RV: IVS T1 ratio and LVEF (r= -0.68, p = 0.006), between LVEF and SIP: IVS T1 ratios (r= -0.54, p = 0.04), and RVEF and IIP T1 (r= -0.59, p = 0.02). Fibrosis measured by native T1 mapping in the systemic RV is most prominent in the LV wall and septal insertion point and correlates with decreased function. T1 values can be used in non-invasive imaging assessment of the RV, but further studies with larger cohorts are needed to assess ability to risk stratify and guide therapy.
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Affiliation(s)
- Shawn Shaji
- Department of Pediatrics, Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
| | - Tarek Alsaied
- Department of Pediatrics, Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Anita Saraf
- Department of Pediatrics, Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Arvind Hoskoppal
- Department of Pediatrics, Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Laura Olivieri
- Department of Pediatrics, Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Adam Christopher
- Department of Pediatrics, Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
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Mahdi NA, Guerma L, Desrosiers-Gagnon C, Dore A, Mongeon FP, Mondésert B, Ibrahim R, Poirier N, Guertin MC, Leduc L, Codsi E, Khairy P, Chaix MA. Sex-Related Differences and Influence of Pregnancy in Transposition of Great Arteries With Systemic Right Ventricle. JACC. ADVANCES 2024; 3:101015. [PMID: 39130012 PMCID: PMC11312776 DOI: 10.1016/j.jacadv.2024.101015] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/14/2022] [Revised: 03/19/2024] [Accepted: 03/29/2024] [Indexed: 08/13/2024]
Abstract
Background There is a paucity of data regarding sex-related differences on cardiac outcomes in the context of transposition of the great arteries (TGA) with a systemic right ventricle and biventricular physiology (sRV-biV). Moreover, the long-term impact of pregnancy on cardiac outcomes remains unknown. Objectives The purpose of this study was to identify sex-related differences and the influence of pregnancy on cardiac outcomes in TGA sRV-biV population. Methods A retrospective cohort study was conducted on 213 adults with TGA sRV-biV, 82 (38.4%) women, age 42.6 ± 12.8 years, with a median follow-up of 16 years. Cardiac events, interventions, last follow-up sRV-biV dysfunction, and heart failure (HF) medications were compared between men vs women, and women with vs without pregnancies resulting in live births. Results Women had a lower incidence of nonsustained ventricular tachycardia (HR: 1.80; 95% CI: 1.04-3.09, P = 0.035) and nonsignificantly fewer HF-related hospitalizations than men (HR: 2.10; 95% CI: 0.95-4.67, P = 0.069) in univariable analysis. At the last follow-up, women had a lower prevalence of moderate to severe sRV-biV dysfunction than men (P < 0.001) and were less frequently prescribed HF therapy. Women had fewer implantable cardioverter-defibrillators for primary prevention than men (P = 0.016), with no difference for secondary prevention. Women who had pregnancies resulting in live births (N = 47), had a high prevalence of cardiac events in the 15 (IQR: 9-28) years following pregnancy with no significant differences with those without (N = 32) pregnancies. Conclusions Women with a sRV-biV have fewer adverse cardiovascular events than men. Due to sRV-biV, pregnancy remains with high maternal risk but is not associated with worse long-term cardiac outcomes under rigorous multidisciplinary cardio-obstetrical care.
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Affiliation(s)
- Naïma-Ayane Mahdi
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Léa Guerma
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Charles Desrosiers-Gagnon
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Annie Dore
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - François-Pierre Mongeon
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Blandine Mondésert
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Réda Ibrahim
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Nancy Poirier
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
| | - Marie-Claude Guertin
- Montreal Health Innovation Coordinating Center (MHICC), Montreal, Quebec, Canada
| | - Line Leduc
- Centre de médecine fœto-maternelle, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
| | - Elisabeth Codsi
- Centre de médecine fœto-maternelle, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
| | - Paul Khairy
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
- Montreal Health Innovation Coordinating Center (MHICC), Montreal, Quebec, Canada
| | - Marie-A Chaix
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
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Ragheb DK, Healan S, Frischhertz B, Nicholson G, Janssen D, Bichell D, Doyle T, Weingarten A. Hybrid Stenting of Pulmonary Venous Baffle Stenosis: Subxiphoid Approach in Transposition of the Great Arteries. JACC Case Rep 2024; 29:102294. [PMID: 38576773 PMCID: PMC10992688 DOI: 10.1016/j.jaccas.2024.102294] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2023] [Revised: 02/14/2024] [Accepted: 02/20/2024] [Indexed: 04/06/2024]
Abstract
A 31-year-old woman with transposition of the great arteries status post-Senning operation presents with severe pulmonary venous baffle obstruction. Both standards of care (percutaneous stenting or open repair) were deemed suboptimal and/or high risk. A multidisciplinary, hybrid approach via subxiphoid incision, guided by 3-dimensional modeling, provided a lower risk and minimally invasive intervention.
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Affiliation(s)
- Daniel Kyrillos Ragheb
- Vanderbilt University School of Medicine, Nashville, Tennessee, USA
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
| | - Steven Healan
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee, USA
| | - Benjamin Frischhertz
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee, USA
| | - George Nicholson
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee, USA
| | - Dana Janssen
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee, USA
| | - David Bichell
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee, USA
| | - Thomas Doyle
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
| | - Angela Weingarten
- Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee, USA
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Chaix MA, Dore A, Mondésert B, Mongeon FP, Roy V, Desrosiers-Gagnon C, Guertin MC, White M, Ibrahim R, O’Meara E, Rouleau JL, Khairy P. Angiotensin receptor-neprilysin inhibitor vs. placebo in congenital systemic right ventricular heart failure: the PARACYS-RV trial. Eur Heart J 2024; 45:1481-1483. [PMID: 38445853 PMCID: PMC11032706 DOI: 10.1093/eurheartj/ehad890] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/25/2023] [Revised: 12/19/2023] [Accepted: 12/21/2023] [Indexed: 03/07/2024] Open
Affiliation(s)
- Marie-A Chaix
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - Annie Dore
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - Blandine Mondésert
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - François-Pierre Mongeon
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - Véronique Roy
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - Charles Desrosiers-Gagnon
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - Marie-Claude Guertin
- Montreal Health Innovations Coordinating Center (MHICC), Montreal Heart Institute, Université de Montréal, 5000 Belanger St. E., Montreal H1T 1C8, QC, Canada
| | - Michel White
- Heart failure clinic, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, QC, Canada
| | - Réda Ibrahim
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
| | - Eileen O’Meara
- Heart failure clinic, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, QC, Canada
| | - Jean-Lucien Rouleau
- Heart failure clinic, Montreal Heart Institute, Department of Medicine, Université de Montréal, Montreal, QC, Canada
| | - Paul Khairy
- Adult Congenital Heart Center, Montreal Heart Institute, Department of Medicine, Université de Montréal, 5000 Belanger St.E., Montreal H1T 1C8, QC, Canada
- Montreal Health Innovations Coordinating Center (MHICC), Montreal Heart Institute, Université de Montréal, 5000 Belanger St. E., Montreal H1T 1C8, QC, Canada
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Bevilacqua F, Pasqualin G, Ferrero P, Micheletti A, Negura DG, D'Aiello AF, Giamberti A, Chessa M. Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review. Diagnostics (Basel) 2023; 13:2205. [PMID: 37443599 DOI: 10.3390/diagnostics13132205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2023] [Revised: 06/10/2023] [Accepted: 06/24/2023] [Indexed: 07/15/2023] Open
Abstract
The population of patients with a systemic right ventricle (sRV) in biventricular circulation includes those who have undergone an atrial switch operation for destro-transposition of the great arteries (d-TGA) and those with congenitally corrected transposition of the great arteries (ccTGA). Despite the life expectancy of these patients is significantly increased, the long-term prognosis remains suboptimal due to late complications such as heart failure, arrhythmias, and premature death. These patients, therefore, need a close follow-up to early identify predictive factors of adverse outcomes and to implement all preventive therapeutic strategies. This review analyzes the late complications of adult patients with an sRV and TGA and clarifies which are risk factors for adverse prognosis and which are the therapeutic strategies that improve the long-term outcomes. For prognostic purposes, it is necessary to monitor sRV size and function, the tricuspid valve regurgitation, the functional class, the occurrence of syncope, the QRS duration, N-terminal pro B-type natriuretic peptide levels, and the development of arrhythmias. Furthermore, pregnancy should be discouraged in women with risk factors. Tricuspid valve replacement/repair, biventricular pacing, and implantable cardioverter defibrillator are the most important therapeutic strategies that have been shown, when used correctly, to improve long-term outcomes.
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Affiliation(s)
- Francesca Bevilacqua
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
| | - Giulia Pasqualin
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
| | - Paolo Ferrero
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
| | - Angelo Micheletti
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
| | - Diana Gabriela Negura
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
| | - Angelo Fabio D'Aiello
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
| | - Alessandro Giamberti
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
- Congenital Cardiac Surgery Unit, IRCCS-Policlinico San Donato, 20097 Milano, Italy
| | - Massimo Chessa
- Adult Congenital Heart Disease Unit, Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, 20132 Milano, Italy
- European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart: ERN GUARD-Heart, 00165 Rome, Italy
- Vita Salute San Raffaele University, 20132 Milano, Italy
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11
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TGA + IVS + LVOTO: patterns of practice and outcomes. Cardiol Young 2023; 33:342-348. [PMID: 36799034 DOI: 10.1017/s1047951122003924] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/18/2023]
Abstract
PURPOSE Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. RESULTS These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). CONCLUSION TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.
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12
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Chowdhury UK, George N, Sushamagayatri B, Manjusha S, Gupta S, Goja S, Sharma S, Kapoor PM. Atrial Correction (Modified Senning) of Transposition of the Great Arteries and Intact Atrial Septum with Regressed Left Ventricle and Pulmonary Hypertension: A Video Presentation. JOURNAL OF CARDIAC CRITICAL CARE TSS 2023. [DOI: 10.25259/mm_jccc_ujjwalsenning(video)] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023] Open
Abstract
A 3-year-old male child diagnosed as D-transposition of the great arteries and intact atrial septum with regressed left ventricle, Yacoub’s Type-B coronary arterial pattern, successfully underwent modified Senning operation under moderately hypothermic cardiopulmonary bypass and St. Thomas based cold blood cardioplegia. At 8 months of follow-up, there was no mitral or tricuspid regurgitation with good biventricular function in Ross clinical score of 2.
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Affiliation(s)
- Ujjwal K. Chowdhury
- Department of Cardiothoracic and Vascular Surgery, National Institute of Medical Sciences and Research, Jaipur, Rajasthan, India,
| | - Niwin George
- Cardiothoracic Sciences Centre, CNC, AIIMS, New Delhi, India,
| | | | - Sai Manjusha
- Cardiothoracic Sciences Centre, CNC, AIIMS, New Delhi, India,
| | - Sraddha Gupta
- Cardiothoracic Sciences Centre, CNC, AIIMS, New Delhi, India,
| | - Shikha Goja
- Cardiothoracic Sciences Centre, CNC, AIIMS, New Delhi, India,
| | - Srikant Sharma
- Cardiothoracic Sciences Centre, CNC, AIIMS, New Delhi, India,
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13
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Ferguson HN, Martinez HR, Pride PM, Swan EA, Hurwitz RA, Payne RM. Biomarker sST2 in Adults with Transposition of the Great Arteries Palliated by Mustard Procedure: A Five-Year Follow-up. Pediatr Cardiol 2023; 44:927-932. [PMID: 36705684 DOI: 10.1007/s00246-023-03105-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/14/2022] [Accepted: 01/13/2023] [Indexed: 01/28/2023]
Abstract
The Mustard procedure was an early cardiac surgery for transposition of the great arteries (TGA). Despite being successful, it has been associated with long-term arrhythmias and heart failure. A key factor complicating management in adults with congenital heart disease (CHD) is the deficiency of biomarkers predicting outcome. Soluble suppression of tumorogenicity-2 (sST2) is secreted by cardiomyocytes in response to mechanical strain and fibrosis. We hypothesized that adults with a Mustard procedure would have higher levels of sST2 than healthy individuals, and this would correlate with clinical outcome. We performed a single-center study in patients managed during childhood with a Mustard procedure versus age-matched controls. Clinical and demographic data were collected and biomarkers (sST2, cTnI, BNP, lipid panel, insulin, and glucose) were obtained. There were 18 patients (12 male) in the Mustard cohort and 18 patients (6 male) in the control group (22-49 years, mean of 35.8 vs. mean 32.6 years, respectively, p = ns). Nine Mustard subjects were NYHA class II, and 9 subjects were class III. The control group was asymptomatic. sST2 in the Mustard group was elevated in 56% vs. 17% in controls (p = 0.035). Of the Mustard subjects with elevated sST2, 60% had elevated cTnI and BNP, and 90% had low HDL. Over five years, the Mustard patients with elevated sST2 values had greater medication use, arrhythmias, hospitalizations, and ablation/pacer implantations than Mustard subjects with normal sST2. Mustard subjects with elevated sST2 had other biomarker abnormalities and clinically worse outcomes. Thus, sST2 may add a predictive value to cardiac-related morbidity and mortality.
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Affiliation(s)
- Haley N Ferguson
- Indiana University School of Medicine, Indianapolis, IN, 46202, USA
| | - Hugo R Martinez
- Division of Pediatric Cardiology, Le Bonheur Children's Hospital, University of Tennessee Health Science Center, Memphis, TN, 38105, USA
| | - P Melanie Pride
- Wells Center for Pediatric Research, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, 46202, USA
| | - Elizabeth A Swan
- Clinical Trials Management Organization, The Ohio State University, Columbus, OH, 43210, USA
| | - Roger A Hurwitz
- Division of Cardiology, Department of Pediatrics, Riley Hospital for Children, Indiana University School of Medicine, 1044 West Walnut St, Room R4-302b, Indianapolis, IN, 46202, USA
| | - R Mark Payne
- Wells Center for Pediatric Research, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, 46202, USA. .,Division of Cardiology, Department of Pediatrics, Riley Hospital for Children, Indiana University School of Medicine, 1044 West Walnut St, Room R4-302b, Indianapolis, IN, 46202, USA.
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14
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Sabbah BN, Arabi TZ, Shafqat A, Abdul Rab S, Razak A, Albert-Brotons DC. Heart failure in systemic right ventricle: Mechanisms and therapeutic options. Front Cardiovasc Med 2023; 9:1064196. [PMID: 36704462 PMCID: PMC9871570 DOI: 10.3389/fcvm.2022.1064196] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2022] [Accepted: 12/19/2022] [Indexed: 01/12/2023] Open
Abstract
d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.
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Affiliation(s)
| | | | - Areez Shafqat
- College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
| | | | - Adhil Razak
- College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
| | - Dimpna Calila Albert-Brotons
- Department of Pediatric Cardiology, Pediatric Heart Failure and Heart Transplant, Heart Center, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
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15
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Taguchi Y, Matsumoto K, Shoda M, Nitta M, Hosoda J, Ishikawa T. A case of successful catheter ablation for biatrial reentrant tachycardia after a Mustard operation for dextro-transposition of the great arteries. HeartRhythm Case Rep 2022; 9:140-143. [PMID: 36970393 PMCID: PMC10030292 DOI: 10.1016/j.hrcr.2022.11.014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
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16
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Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects. Cardiol Young 2022; 32:1235-1245. [PMID: 34658317 DOI: 10.1017/s1047951121003954] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]
Abstract
BACKGROUND Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. METHODS A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. RESULTS Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. CONCLUSION A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.
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17
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Antonová P, Rohn V, Chaloupecky V, Simkova I, Kaldararova M, Zeman J, Popelova J, Havova M, Janousek J. Predictors of mortality after atrial correction of transposition of the great arteries. Heart 2022; 108:1881-1886. [DOI: 10.1136/heartjnl-2021-320035] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2022] [Accepted: 07/05/2022] [Indexed: 11/04/2022] Open
Abstract
ObjectivesTo determine the long-term and transplantation-free survival of all patients after atrial correction of transposition of the great arteries (TGA) in the Czech and Slovak republics, including its preoperative and perioperative determinants.MethodsRetrospective analysis of all 454 consecutive patients after atrial correction of TGA was performed. Of these, 126 (27.8%) were female, median age at operation was 7.4 months (Q1 5.3; Q3 13.3) and 164 (36.1%) underwent the Mustard procedure. The relationships between age, weight, the complexity of TGA, operative technique, additional surgical procedures, immediate postoperative presence of tricuspid regurgitation and revision procedures during follow-up to major composite outcome, as such defined as long-term and transplantation-free survival, were tested.ResultsEarly 30-day mortality did not differ between the Mustard (9.76%) and Senning (8.97%) cohorts (p=0.866). The long-term and transplantation-free survival, which differed between the Mustard and Senning cohorts in favour of the Senning procedure (HR 0.43; 95% CI 0.21 to 0.87), was shorter in complex TGA (HR 2.4; 95% CI 1.59 to 3.78) and in complex surgical interventions (HR 3.51; 95% CI 2.31 to 5.56). The immediate presence of at least moderate tricuspid regurgitation after correction was associated with a shorter long-term and transplantation-free survival in the univariate but not in the multivariable model.ConclusionsThe lower long-term survival of patients after an atrial switch operation of TGA in the Czech and Slovak republics is associated with greater complexity of TGA, complex surgical interventions and application of the the Mustard operative procedure.
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18
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Marzullo R, Ladouceur M, Gaio G, Giordano M, Russo MG, Sarubbi B. Impact of pregnancy on natural history of systemic right ventricle in women with transposition of the great arteries. Int J Cardiol 2022; 366:20-24. [PMID: 35842002 DOI: 10.1016/j.ijcard.2022.07.021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/19/2022] [Revised: 05/26/2022] [Accepted: 07/12/2022] [Indexed: 11/05/2022]
Abstract
In the recent years, the pregnancy trend among women with Congenital Heart Disease (CHD) has increased; this has leaded to a growing demand for specialized care both in mother and in children. Although pregnancy is often well tolerated, maternal CHD may affect in some cases a maladaptive hemodynamic response carrying additional risks of cardiovascular events like arrhythmias, heart failure and, in rare cases, death. The impaired utero-placental perfusion due to maternal cardiac status may result in placental dysfunction, which may be associated with fetal growth restriction, preeclampsia, premature birth and perinatal morbidity. Systemic Right Ventricle (SRV) is one of the main conditions under which pregnancy is challenging. The sub-aortic position of morphological Right Ventricle (RV) is "physiologically" predisposed to fail at the adult age and may be potentially inadequate to support the hemodynamic stress of the pregnancy. Current literature about pregnancy in women with SRV consists of small retrospective series not providing conclusive evidence about the feasibility of a successful pregnancy outcomes. In addition, the long-term effects of pregnancy on SVR are not still adequately investigated and it remains unclear if maternal complications reported are due to pregnancy or to natural history of SVR. The aim of this paper is to offer a critical review of the knowledges at regard and to provide a practice update on the risk assessment and the pregnancy management in women with SRV in order to support the decision making and to optimize outcomes in these patients.
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Affiliation(s)
- Raffaella Marzullo
- Pediatric Cardiology, University of Campania 'Luigi Vanvitelli', Former Second University of Naples, Monaldi Hospital-AORN Ospedali dei Colli, Naples, Italy.
| | - Magalie Ladouceur
- Unité de cardiologie congénitale adulte, Hôpital Européen Georges-Pompidou, Centre de référence des malformations cardiaques congénitales complexes, M3C, Paris, France
| | - Gianpiero Gaio
- Pediatric Cardiology, University of Campania 'Luigi Vanvitelli', Former Second University of Naples, Monaldi Hospital-AORN Ospedali dei Colli, Naples, Italy
| | - Mario Giordano
- Pediatric Cardiology, University of Campania 'Luigi Vanvitelli', Former Second University of Naples, Monaldi Hospital-AORN Ospedali dei Colli, Naples, Italy
| | - Maria Giovanna Russo
- Pediatric Cardiology, University of Campania 'Luigi Vanvitelli', Former Second University of Naples, Monaldi Hospital-AORN Ospedali dei Colli, Naples, Italy
| | - Berardo Sarubbi
- Adult Congenital Heart Diseases Unit, AORN dei Colli, Monaldi Hospital, Naples, Italy
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19
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Rosseel T, Santens B, Droogne W, Voros G, Van Cleemput J, Van Aelst L, Budts W, Jacobs S, Van De Bruaene A, Meyns B. Ventricular assist device implantation in adults with a systemic right ventricle - A single center experience and review of the literature. INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE 2022; 8:100365. [PMID: 39712050 PMCID: PMC11657773 DOI: 10.1016/j.ijcchd.2022.100365] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2022] [Revised: 03/22/2022] [Accepted: 03/26/2022] [Indexed: 11/19/2022] Open
Abstract
Patients with a systemic right ventricle (sRV) have a high risk of developing heart failure (HF), with heart transplantation as the only long-term treatment option. Unfortunately, more than half of sRV patients have pulmonary hypertension (PH) which may impact the decision to opt for heart-only transplantation. Moreover, an increased scarcity of donor hearts prolongs time to transplantation, increasing the probability of dying on the waiting list. Hence, for selected sRV patients, a ventricular assist device (VAD) could provide a solution to bridge the time to transplantation. This case series aimed at evaluating all patients with a sRV who received a VAD at our institution, with a focus on technical aspects of VAD implantation and invasive hemodynamics prior to VAD implantation and after heart transplantation. In total 5 patients with a sRV who received a VAD at our institution were included. All five were male with a median age of 38 years (19-49) at the time of VAD implantation. All patients were subsequently successfully transplanted. Pulmonary artery (PA) pressures were significantly elevated prior to VAD implantation and decreased significantly after VAD implantation and subsequent heart transplantation. With this case series we highlight the ability of a VAD to support the failing sRV, as well as to reduce PA pressures, making patients eligible for heart-only transplantation.
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Affiliation(s)
- Thomas Rosseel
- Department of Cardiology, University Hospital Leuven, Leuven, Belgium
| | - Beatrice Santens
- Department of Cardiology, University Hospital Leuven, Leuven, Belgium
| | - Walter Droogne
- Department of Cardiology, University Hospital Leuven, Leuven, Belgium
| | - Gabor Voros
- Department of Cardiology, University Hospital Leuven, Leuven, Belgium
| | | | - Lucas Van Aelst
- Department of Cardiology, University Hospital Leuven, Leuven, Belgium
| | - Werner Budts
- Department of Cardiology, University Hospital Leuven, Leuven, Belgium
| | - Steven Jacobs
- Department of Cardiac Surgery, University Hospital Leuven, Leuven, Belgium
| | | | - Bart Meyns
- Department of Cardiac Surgery, University Hospital Leuven, Leuven, Belgium
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20
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Gonzalez-Fernandez O, De Rita F, Coats L, Crossland D, Nassar MS, Hermuzi A, Santos Lopes B, Woods A, Robinson-Smith N, Petit T, Seller N, O'Sullivan J, McDiarmid A, Schueler S, Hasan A, MacGowan G, Jansen K. Ventricular assist devices in transposition and failing systemic right ventricle: role of tricuspid valve replacement. Eur J Cardiothorac Surg 2022; 62:6542520. [PMID: 35244691 DOI: 10.1093/ejcts/ezac130] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2021] [Revised: 01/31/2022] [Accepted: 02/23/2022] [Indexed: 11/12/2022] Open
Abstract
OBJECTIVES Ventricular assist device (VAD) for systemic right ventricular (RV) failure patients post-atrial switch, for transposition of the great arteries (TGA), and those with congenitally corrected TGA has proven useful to reduce transpulmonary gradient and bridge-to-transplantation. The purpose of this study is to describe our experience of VAD in systemic RV failure and our move towards concomitant tricuspid valve replacement (TVR). METHODS This is a single-centre retrospective study of consecutive adult patients receiving HeartWare VAD for systemic RV failure between 2010 and 2019. From 2017, concomitant TVR was performed routinely. Demographic, clinical variables and echocardiographic and haemodynamic measurements pre- and post-VAD implantation were recorded. Complications on support, heart transplantation and survival rates were described. RESULTS Eighteen patients underwent VAD implantation. Moderate or severe systemic tricuspid regurgitation was present in 83.3% of patients, and subpulmonic left ventricular impairment in 88.9%. One-year survival was 72.2%. VAD implantation was technically feasible and successful in all but one. Post-VAD, transpulmonary gradient fell from 16 (15-22) to 10 (7-13) mmHg (P = 0.01). Patients with TVR (n = 6) also demonstrated a reduction in mean pulmonary and wedge pressures. Furthermore, subpulmonic left ventricular end-diastolic dimension (44.3 vs 39.6 mm; P = 0.03) and function improved in this group. After 1 year of support, 72.2% of patients were suitable for transplantation. CONCLUSIONS VAD is an effective strategy as bridge-to-candidacy and bridge-to-transplantation in patients with end-stage systemic RV failure. Concomitant TVR at the time of implant is associated with better early haemodynamic and echocardiographic results post-VAD.
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Affiliation(s)
- Oscar Gonzalez-Fernandez
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Universidad Autonoma de Madrid, Madrid, Spain
| | - Fabrizio De Rita
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK
| | - Louise Coats
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK
| | - David Crossland
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Mohamed S Nassar
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Antony Hermuzi
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK
| | - Bruno Santos Lopes
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Andrew Woods
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Nicola Robinson-Smith
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Thibault Petit
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,University Hospitals Leuven, Leuven, Belgium
| | - Neil Seller
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - John O'Sullivan
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Biosciences Institute, Newcastle University, Newcastle upon Tyne, UK
| | - Adam McDiarmid
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Stephan Schueler
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Asif Hasan
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
| | - Guy MacGowan
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Biosciences Institute, Newcastle University, Newcastle upon Tyne, UK
| | - Katrijn Jansen
- Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.,Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK
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21
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Moya A, De Meester P, Troost E, Roggen L, Weidemann F, Moons P, Eyskens B, Claus P, Budts W, Van De Bruaene A. 15-Year follow-up of regional right and left ventricular function after the Senning operation: a Colour-Doppler myocardial imaging study. Acta Cardiol 2021; 76:689-696. [PMID: 32539571 DOI: 10.1080/00015385.2020.1770459] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
INTRODUCTION Despite right ventricular (RV) dysfunction being a major concern in Senning patients, long-term follow-up data is lacking. This study aimed (1) at evaluating regional (base-mid-apex) RV and left ventricular (LV) function using Colour-Doppler myocardial imaging over a 15-year follow-up period and (2) at comparing results with matched controls. METHODS For the longitudinal analysis (2004-2019), we compared systolic and diastolic function in 10 Senning patients. For the cross-sectional analysis, we compared the subaortic RV (sRV) of Senning patients with the RV and LV of matched controls and the subpulmonary LV (spLV) of Senning patients with the LV of matched controls. RESULTS The longitudinal analysis of sRV function showed a significant decrease in apical peak systolic strain (-17 ± 7% vs -12 ± 4%; p = 0.025) and apical peak systolic strain rate (-1.1 ± 0.3s-1 vs -0.8 ± 0.4s-1; p = 0.012). spLV function showed a significant decrease in peak systolic velocity (mid; p = 0.013 and apex; p = 0.011) and peak systolic strain rate (mid; p = 0.048). The cross-sectional analysis revealed significant lower values for basal, mid and apical peak systolic velocity, peak systolic strain rate, peak systolic strain of the sRV of Senning patients when compared to both LV and RV of matched controls (all p < 0.05). CONCLUSION Our study showed that systolic and diastolic sRV function did not change over a 15-year follow-up period, except in the apical region. There was a decline in spLV systolic function, which may be of clinical value. On the other hand, when compared to age- and gender-matched controls, the sRV of Senning patients exhibits significantly decreased measurements of longitudinal systolic function.
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Affiliation(s)
- Ana Moya
- Faculty of Biomedical Sciences, KU Leuven, Leuven, Belgium
| | - Pieter De Meester
- Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium
- Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium
| | - Els Troost
- Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium
| | - Leen Roggen
- Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium
| | | | - Philip Moons
- Department of Public Health and Primary Care, KU Leuven, Leuven, Belgium
| | - Benedicte Eyskens
- Division of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium
| | - Piet Claus
- Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium
| | - Werner Budts
- Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium
- Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium
| | - Alexander Van De Bruaene
- Division of Structural and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium
- Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium
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22
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Is arterial switch operation possible with neonatology-focused intensive care unit modality. TURK GOGUS KALP DAMAR CERRAHISI DERGISI-TURKISH JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 2021; 29:136-142. [PMID: 34104506 PMCID: PMC8167476 DOI: 10.5606/tgkdc.dergisi.2021.20309] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/15/2020] [Accepted: 01/06/2021] [Indexed: 11/29/2022]
Abstract
Background
In this study, we aimed to examine the feasibility of arterial switch operation and its perioperative management with neonatology-focused intensive care modality in a region of Turkey where the birth rate and the number of asylum seekers who had to leave their country due to regional conflicts are high.
Methods
Between December 2017 and June 2020, a total of 57 patients (48 males, 9 females; median age: 12.2 days; range, 2 to 50 days) who were diagnosed with transposition of the great arteries in our clinic and underwent arterial switch operation were retrospectively analyzed. All patients were followed by the neonatologist in the neonatal intensive care unit during the preoperative and postoperative period.
Results
Thirty-eight (66.7%) patients had intact ventricular septum, 16 (28.1%) had ventricular septal defect, two (3.5%) had coarctation of the aorta, and one (1.7%) had Taussig-Bing anomaly. Coronary artery anomaly was present in 14 (24.5%) patients. The most common complications in the intensive care unit were renal failure requiring peritoneal dialysis in seven (12.3%) patients, supraventricular tachyarrhythmia in six (10.5%) patients, and eight (14%) patients left their chests open. The median length of stay in intensive care unit was 13.8 (range, 9 to 25) days and the median length of hospital stay was 24.5 (range, 16 to 47) days. The overall mortality rate for all patients was 12.3% (n=7). The median follow-up was 8.2 months. A pulmonary valve peak Doppler gradient of ≥36 mmHg was detected in five patients (8.7%) who were followed, and these patients were monitored by providing medical treatment. None of the patients needed reoperation or reintervention.
Conclusion
We believe that arterial switch operation, one of the complex neonatal cardiac surgery, can be performed with an acceptable mortality and morbidity rate with the use of neonatology-focused intensive care modality, which is supported by pediatric cardiology and pediatric cardiac surgery.
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23
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Woudstra OI, Zandstra TE, Vogel RF, van Dijk APJ, Vliegen HW, Kiès P, Jongbloed MRM, Egorova AD, Doevendans PAFM, Konings TC, Mulder BJM, Tanck MWT, Meijboom FJ, Bouma BJ. Clinical Course Long After Atrial Switch: A Novel Risk Score for Major Clinical Events. J Am Heart Assoc 2021; 10:e018565. [PMID: 33615824 PMCID: PMC8174274 DOI: 10.1161/jaha.120.018565] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Background Patients with transposition of the great arteries corrected by an atrial switch operation experience major clinical events during adulthood, mainly heart failure (HF) and arrhythmias, but data on the emerging risks remain scarce. We assessed the risk for events during the clinical course in adulthood, and provided a novel risk score for event‐free survival. Methods and Results This multicenter study observed 167 patients with transposition of the great arteries corrected by an atrial switch operation (61% Mustard procedure; age, 28 [interquartile range, 24–36] years) for 13 (interquartile range, 9–16) years, during which 16 (10%) patients died, 33 (20%) had HF events, defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF‐related death, and 15 (9%) had symptomatic ventricular arrhythmias. Five‐year risk of mortality, first HF event, and first ventricular arrhythmia increased from 1% each at age 25 years, to 6% (95% CI, 4%–9%), 23% (95% CI, 17%–28%), and 5% (95% CI, 2%–8%), respectively, at age 50 years. Predictors for event‐free survival were examined to construct a prediction model using bootstrapping techniques. A prediction model combining age >30 years, prior ventricular arrhythmia, age >1 year at repair, moderate or greater right ventricular dysfunction, severe tricuspid regurgitation, and mild or greater left ventricular dysfunction discriminated well between patients at low (<5%), intermediate (5%–20%), and high (>20%) 5‐year risk (optimism‐corrected C‐statistic, 0.86 [95% CI, 0.82–0.90]). Observed 5‐ and 10‐year event‐free survival rates in low‐risk patients were 100% and 97%, respectively, compared with only 31% and 8%, respectively, in high‐risk patients. Conclusions The clinical course of patients undergoing atrial switch increasingly consists of major clinical events, especially HF. A novel risk score stratifying patients as low, intermediate, and high risk for event‐free survival provides information on absolute individual risks, which may support decisions for pharmacological and interventional management.
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Affiliation(s)
- Odilia I Woudstra
- Department of Clinical and Experimental Cardiology Heart Center Amsterdam Cardiovascular SciencesAmsterdam University Medical CenterUniversity of Amsterdam the Netherlands.,Department of Cardiology University Medical Center Utrecht Utrecht the Netherlands
| | - Tjitske E Zandstra
- Department of Cardiology Leiden University Medical Center Leiden the Netherlands
| | - Rosanne F Vogel
- Department of Clinical and Experimental Cardiology Heart Center Amsterdam Cardiovascular SciencesAmsterdam University Medical CenterUniversity of Amsterdam the Netherlands
| | - Arie P J van Dijk
- Department of Cardiology Radboud University Medical Center Nijmegen the Netherlands
| | - Hubert W Vliegen
- Department of Cardiology Leiden University Medical Center Leiden the Netherlands
| | - Philippine Kiès
- Department of Cardiology Leiden University Medical Center Leiden the Netherlands
| | - Monique R M Jongbloed
- Department of Cardiology Leiden University Medical Center Leiden the Netherlands.,Department of Anatomy and Embryology Leiden University Medical Center Leiden the Netherlands
| | - Anastasia D Egorova
- Department of Cardiology Leiden University Medical Center Leiden the Netherlands
| | | | - Thelma C Konings
- Department of Clinical and Experimental Cardiology Heart Center Amsterdam Cardiovascular SciencesAmsterdam University Medical CenterVrije Universiteit Amsterdam Amsterdam the Netherlands
| | - Barbara J M Mulder
- Department of Clinical and Experimental Cardiology Heart Center Amsterdam Cardiovascular SciencesAmsterdam University Medical CenterUniversity of Amsterdam the Netherlands
| | - Michael W T Tanck
- Department of Clinical Epidemiology, Biostatistics and Bioinformatics Amsterdam University Medical CenterUniversity of Amsterdam the Netherlands
| | - Folkert J Meijboom
- Department of Cardiology University Medical Center Utrecht Utrecht the Netherlands
| | - Berto J Bouma
- Department of Clinical and Experimental Cardiology Heart Center Amsterdam Cardiovascular SciencesAmsterdam University Medical CenterUniversity of Amsterdam the Netherlands
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24
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Subramani S, Sharma A, Arora L, Hanada S, Krishnan S, Ramakrishna H. Perioperative Right Ventricular Dysfunction: Analysis of Outcomes. J Cardiothorac Vasc Anesth 2021; 36:309-320. [PMID: 33593648 DOI: 10.1053/j.jvca.2021.01.032] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/14/2021] [Accepted: 01/15/2021] [Indexed: 11/11/2022]
Abstract
Right ventricular dysfunction (RVD) is a well-known prognostic factor for adverse outcomes in cardiovascular medicine. The right ventricle (RV) in medically managed heart failure patients and in surgical patients perioperatively generally is overshadowed by left ventricular disease. However, with advancement of various diagnostic tools and better understanding of its functional anatomy, the role of the RV is emerging in many clinical conditions. The failure of one ventricle has significant effect on the function of the other ventricle and it is predominantly due to ventricular interdependence.1 The etiology of RVD is multifactorial and irrespective of etiology. RVD has been associated with significant increases in morbidity and mortality in various clinical scenarios.2,3 The primary objective of this comprehensive review is to analyze various etiology-related outcomes of RVD in the perioperative population.
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Affiliation(s)
- Sudhakar Subramani
- Department of Anesthesia, University of Iowa Carver College of Medicine, Iowa City, IA
| | - Archit Sharma
- Department of Anesthesia, University of Iowa Carver College of Medicine, Iowa City, IA
| | - Lovkesh Arora
- Department of Anesthesia, University of Iowa Carver College of Medicine, Iowa City, IA
| | - Satoshi Hanada
- Department of Anesthesia, University of Iowa Carver College of Medicine, Iowa City, IA
| | - Sundar Krishnan
- Department of Anesthesia, Duke University School of Medicine, Durham, NC
| | - Harish Ramakrishna
- Division of Cardiovascular and Thoracic Anesthesiology, Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, MN.
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25
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Malankar DP, Patil S, Mali S, Dhake S, Mhatre A, Bind D, Soni B, Kandavel D, Garekar S. Primary Arterial Switch Operation for TGA/IVS and Regressed Left Ventricle: How and When to Use Left Ventricular Assist Device. World J Pediatr Congenit Heart Surg 2020; 11:97-100. [PMID: 31835987 DOI: 10.1177/2150135119879876] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
PURPOSE Numerous attempts have been made to extend the boundaries of arterial switch operation (ASO) in children presenting late with transposition of great arteries with intact ventricular septum (TGA/IVS) and regressed left ventricle (rLV). Many children tolerate the delayed ASO uneventfully, whereas others need mechanical circulatory support (MCS) to sustain the systemic circulation while the left ventricle undergoes retraining. DESCRIPTION In this article, we describe six consecutive children with TGA/IVS and rLV who underwent primary ASO. RESULTS Three were managed medically, while three required MCS in the form of Centrimag left ventricular assist device (LVAD). All patients survived the operation and were discharged home in a stable condition. CONCLUSIONS Primary ASO can be safely performed in children with TGA/IVS and rLV, provided the center has MCS options. Supporting the rLV with LVAD is feasible and can be achieved safely.
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Affiliation(s)
- Dhananjay P Malankar
- Department of Paediatric Cardiac Surgery, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Sachin Patil
- Department of Paediatric Cardiac Anaesthesia and Critical Care, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Shivaji Mali
- Department of Paediatric Cardiac Anaesthesia and Critical Care, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Shyam Dhake
- Department of Paediatric Cardiac Anaesthesia and Critical Care, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Amit Mhatre
- Department of Paediatric Cardiac Anaesthesia and Critical Care, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Dilip Bind
- Department of Paediatric Cardiac Anaesthesia and Critical Care, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Bharat Soni
- Department of Paediatric Cardiac Surgery, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Dinesh Kandavel
- Department of Paediatric Cardiac Surgery, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
| | - Swati Garekar
- Department of Paediatric Cardiology, Fortis Paediatric and Congenital Heart Centre, Mulund, Mumbai, India
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26
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Taha FA, Elshedoudy S, Adel M. Quantitative assessment of contractile reserve of systemic right ventricle in post-Senning children: Incorporating speckle-tracking strain and dobutamine stress echocardiography. Echocardiography 2020; 37:2091-2101. [PMID: 33200504 DOI: 10.1111/echo.14924] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/26/2020] [Revised: 10/05/2020] [Accepted: 10/24/2020] [Indexed: 10/23/2022] Open
Abstract
INTRODUCTION The systemic load on the right ventricle (RV) after Senning atrial switch leads to ventricular dysfunction. Quantitative assessment of RV contractile reserve is mandatory to anticipate the need for anti-fibrotic treatment. We aimed to quantitatively assess RV contractile reserve in Senning children by estimating speckle-based global longitudinal strain (GLS) during dobutamine stress echocardiography (DSE). METHODS This prospective study compared thirty-one post-Senning children (group I) and thirty controls (group II). In post-Senning children, echocardiographic RV systolic function using one-plane ejection fraction (RVEF), RV fractional area change (RVFAC), tricuspid annulus plane systolic excursion (TAPSE), its Z-score, and RVGLS were recorded at rest and peak DSE. Contractile reserve was defined as improvement >5% in RVEF, >2% in GLS, and/or to near normal TAPSE. RESULTS RVEF, RVFAC, TAPSE, and TAPSE Z-score were significantly lower in patients than controls [RVEF:40.13 ± 2.93% vs 53.17 ± 3.17% (P < .001*), RVFAC: 21.17 ± 2.37% vs 37.23 ± 2.13% (P < .001*), TAPSE:13.81 ± 1.26 vs 17.45 ± 2.93 mm (P < .001*), TAPSE Z-score: -3.47 ± 0.46 vs -2.09 ± 0.48 (P < .001*)]. Also, RVGLS was significantly impaired in Senning children than controls[ (-11.89 ± 2.31% vs -22.35 ± 6.73% (P < .001*)]. At peak DSE, contractile reserve was not evident as measured by RVEF which increased none significantly to 42.47 ± 2.80% (P = .063). However, RVGLS improved significantly to -15.78 ± 0.93% (P < .001*) and discovered the masked contractile reserve in Senning children. The 19(61.29%) children who showed masked contractile reserve (improvement in RVGLS > 2%) underwent continuation of anti-fibrotic medications. CONCLUSIONS Despite systemic RV function in post-Senning children was impaired at rest and during DSE, RVGLS was useful in quantitative assessment of masked contractile thus promoted continuing anti-fibrotic treatment.
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Affiliation(s)
- Fatma Aboalsoud Taha
- Congenital Heart Disease Unit, Cardiology Department, Tanta University, Tanta, Egypt
| | - Sahar Elshedoudy
- Congenital Heart Disease Unit, Cardiology Department, Tanta University, Tanta, Egypt
| | - Mona Adel
- Congenital Heart Disease Unit, Cardiology Department, Tanta University, Tanta, Egypt
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27
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Morfaw F, Leenus A, Mbuagbaw L, Anderson LN, Dillenburg R, Thabane L. Outcomes after corrective surgery for congenital dextro-transposition of the arteries using the arterial switch technique: a scoping systematic review. Syst Rev 2020; 9:231. [PMID: 33028389 PMCID: PMC7542944 DOI: 10.1186/s13643-020-01487-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/04/2019] [Accepted: 09/17/2020] [Indexed: 11/24/2022] Open
Abstract
BACKGROUND Dextro-transposition of the great arteries (D-TGA) is the most frequent cyanotic congenital heart pathology in neonates. Surgical correction of this condition is possible using the arterial switch operation (ASO) which was first performed by Jatene in 1975. OBJECTIVES The aim of this study was to summarise the evidence on short- (less than 1 year), medium- (1-20 years), and long-term (more than 20 years) outcomes of children with D-TGA treated with the ASO. The primary outcome was survival. Secondary outcomes were freedom from cardiac reoperations, occurrence of aortic insufficiency, pulmonary stenosis, coronary artery anomalies, neuropsychological development problems and quality of life. METHODS We searched MEDLINE, EMBASE, CINAHL, LILACS, and reference lists of included articles for studies reporting outcomes after ASO for D-TGA. Screening, data extraction and risk of bias assessment were done independently by two reviewers. We pooled data using a random-effects meta-analysis of proportions and, where not possible, outcomes were synthesized narratively. We used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system to assess the certainty of the evidence for each outcome. MAIN RESULTS Following ASO for TGA, short-term survival was 92.0% (95% CI 91.0-93.0%; I2 = 85.8%, 151 studies, 30,186 participants; moderate certainty evidence). Medium-term survival was 90.0% (95% CI 89.0-91.0%; I2 = 84.3%, 133 studies; 23,686 participants, moderate certainty evidence), while long-term survival was 87.0% (95% CI 80.0-92.0 %; I2 = 84.5%, 4 studies, 933 participants, very low certainty evidence). Evaluation of the different secondary outcomes also showed satisfactory results in the short, medium and long term. Subgroup analysis suggests slightly higher survival following ASO for TGA in the second surgical era (1998 to 2018) than in the first surgical era (1975 to 1997) in the short and medium term [93.0% (95% CI 92.0-94.0) vs 90.0% (95% CI 89.0-92.0) and 93.0% (95% CI 91.0-94.0) vs 88.0% (87.0-90.0%) respectively] but not in the long term [81.0% (95% CI 76.0-86.0%) vs 89.0% (80.0-95.0%)]. CONCLUSIONS Pooled data from many sources suggests that the ASO for D-TGA leads to high rates of survival in the short, medium, and long term.
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Affiliation(s)
- Frederick Morfaw
- Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, ON Canada
- Department of Obstetrics and Gynecology, Faculty of Medicines and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Cameroon
- Faculty of Health Sciences, University of Bamenda, Bamenda, Cameroon
| | - Alvin Leenus
- Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, ON Canada
| | - Lawrence Mbuagbaw
- Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, ON Canada
- Biostatistics Unit/FSORC, St Joseph Healthcare—Hamilton, 50 Charlton Avenue East, 3rd Floor Martha Wing, Room H321, Hamilton, ON L8N 4A6 Canada
- Centre for Development of Best Practices in Health, Yaoundé Central Hospital, Yaoundé, Cameroon
| | - Laura N. Anderson
- Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, ON Canada
| | - Rejane Dillenburg
- Departments of Pediatrics and Anesthesia, McMaster University, Hamilton, Ontario Canada
| | - Lehana Thabane
- Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, ON Canada
- Biostatistics Unit/FSORC, St Joseph Healthcare—Hamilton, 50 Charlton Avenue East, 3rd Floor Martha Wing, Room H321, Hamilton, ON L8N 4A6 Canada
- Departments of Pediatrics and Anesthesia, McMaster University, Hamilton, Ontario Canada
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28
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Cheung YF, Lam WWM, So EKF, Chow PC. Differential myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle after atrial switch operation for complete transposition of the great arteries. IJC HEART & VASCULATURE 2020; 30:100612. [PMID: 32817881 PMCID: PMC7424203 DOI: 10.1016/j.ijcha.2020.100612] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2020] [Revised: 07/27/2020] [Accepted: 07/29/2020] [Indexed: 11/03/2022]
Abstract
Background This study aimed to assess diffuse myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle in patients after Senning or Mustard operation for complete transposition of the great artery (TGA) using cardiac magnetic resonance (CMR) T1 mapping. Methods Thirty-one adult TGA patients after Senning (n = 24) or Mustard (n = 7) operation were studied at the age of 33.3 ± 4.0 years. Systemic right ventricular (RV) and subpulmonary left ventricular (LV) volumes, ejection fraction, and myocardial T1 values and extracellular volume fraction (ECV) were determined using CMR. Results The RV and LV ejection fractions were 47.0 ± 10.9% and 61.3 ± 7.4%, respectively. Compared to published normative values, patients had significantly greater RV and LV native T1 and ECV values (all p < 0.001). For each of the basal, mid, and apical segments, the LV native T1 and ECV values were significantly greater in the left than the right ventricle (all p < 0.05). There is a significant trend on progressive increase in ECV value from the basal towards the apical segments in both the right (p = 0.002) and the left (p < 0.001) ventricle. Modestly strong correlations were found between RV and LV native T1 (r = 0.60, p < 0.001) and ECV (r = 0.49, p = 0.005) values but not with ejection fractions of the respective ventricles. Conclusions Differential myocardial fibrosis, with greater involvement of the subpulmonary left ventricle than the systemic right ventricle, is present in patients with TGA after atrial switch operation. Associations between the magnitude of RV and LV fibrosis suggests adverse ventricular-ventricular interaction at the cardiac extracellular matrix level.
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Affiliation(s)
- Yiu-Fai Cheung
- Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China
| | - Wendy W M Lam
- Department of Radiology, Queen Mary, Hospital, Hong Kong, China
| | - Edwina K F So
- Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China
| | - Pak-Cheong Chow
- Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China
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29
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Merrill M, Krebsbach A, Przybylowicz R, Broberg CS, Burch G, Henrikson CA. Extract-stent-replace for treatment of upper baffle stenosis with pacing leads after atrial switch procedures for transposition of the great arteries: An approach to avoid "jailing" the lead. J Cardiovasc Electrophysiol 2020; 31:2744-2750. [PMID: 32776621 DOI: 10.1111/jce.14710] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/15/2020] [Accepted: 08/01/2020] [Indexed: 11/26/2022]
Abstract
INTRODUCTION Venous stenosis is a late complication of the atrial switch (Mustard/Senning) procedure seen in patients with transposition of the great arteries ( d-TGA). Many atrial switch patients require cardiac implantable electronic devices (CIEDs) which further increases the incidence of venous stenosis. Stenosis of the superior limb of the systemic venous pathway (SLSVP) in the presence of CIED leads presents a management challenge. We propose a method for navigating SLSVP stenosis in atrial switch patients with CIEDs. METHODS The pulse generator and leads were removed using standard extraction techniques. Axillary access was retained via existing leads or new access was obtained. The interventional cardiology team, via groin access, performed stent-angioplasty of the stenotic SLSVP. After stent deployment, the axillary access wire was snared from below, guided through the stent, and pulled into a long groin sheath. A sheath was then advanced over the axillary wire and into the groin sheath creating a path for passage of leads through the stent. New leads were advanced through the axillary sheath into the heart. Leads were secured using standard techniques. RESULTS All patients had a history of d-TGA and prior atrial switch procedures. In each case, there was stenosis of the SLSVP in the setting of a CIED lead. There were no immediate complications and there was no restenosis on follow-up. CONCLUSION Post-atrial switch patients with CIEDs can develop stenosis of the SLSVP. A collaboration between electrophysiology and interventional cardiology can allow for device extraction, stent-angioplasty, and lead reimplantation to avoid "jailing" the leads.
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Affiliation(s)
- Miranda Merrill
- Division of Cardiovascular Medicine, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA
| | - Angela Krebsbach
- Division of Cardiovascular Medicine, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA
| | - Ryle Przybylowicz
- Division of Cardiovascular Medicine, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA
| | - Craig S Broberg
- Division of Cardiovascular Medicine, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA
| | - Grant Burch
- Division of Cardiovascular Medicine, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA
| | - Charles A Henrikson
- Division of Cardiovascular Medicine, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA
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30
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Sung SC, Kim H, Choi KH. Modification of the Senning procedure in the double-switch operation: The triangular double-door technique. J Card Surg 2020; 35:2347-2349. [PMID: 32579767 DOI: 10.1111/jocs.14763] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
We developed a modified Senning procedure in the double-switch operation for the patients with congenitally corrected transposition of the great arteries (ccTGA). In our technique, the right atrial (RA) free wall is not used as a baffle for draining systemic venous blood to the left atrium. Instead, a patch material is used for the baffling. A wide communication between the pulmonary venous chamber and RA is created by making the triangular double door with the RA-free wall, and the window is closed with in situ pericardial flap. We have successfully adopted this technique in our recent two consecutive ccTGA patients.
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Affiliation(s)
- Si Chan Sung
- Department of Thoracic and Cardiovascular Surgery, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan-si, Republic of Korea
| | - Hyungtae Kim
- Department of Thoracic and Cardiovascular Surgery, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan-si, Republic of Korea
| | - Kwang Ho Choi
- Department of Thoracic and Cardiovascular Surgery, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan-si, Republic of Korea
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31
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Angelini A, di Gioia C, Doran H, Fedrigo M, Henriques de Gouveia R, Ho SY, Leone O, Sheppard MN, Thiene G, Dimopoulos K, Mulder B, Padalino M, van der Wal AC. Autopsy in adults with congenital heart disease (ACHD). Virchows Arch 2020; 476:797-820. [PMID: 32266476 PMCID: PMC7272495 DOI: 10.1007/s00428-020-02779-8] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2019] [Revised: 12/31/2019] [Accepted: 01/08/2020] [Indexed: 02/07/2023]
Abstract
The adult congenital heart diseases (ACHD) population is exceeding the pediatric congenital heart diseases (CHD) population and is progressively expanding each year, representing more than 90% of patients with CHD. Of these, about 75% have undergone surgical and/or percutaneous intervention for palliation or correction. Autopsy can be a very challenging procedure in ACHD patients. The approach and protocol to be used may vary depending on whether the pathologists are facing native disease without surgical or percutaneous interventions, but with various degrees of cardiac remodeling, or previously palliated or corrected CHD. Moreover, interventions for the same condition have evolved over the last decades, as has perioperative myocardial preservations and postoperative care, with different long-term sequelae depending on the era in which patients were operated on. Careful clinicopathological correlation is, thus, required to assist the pathologist in performing the autopsy and reaching a diagnosis regarding the cause of death. Due to the heterogeneity of the structural abnormalities, and the wide variety of surgical and interventional procedures, there are no standard methods for dissecting the heart at autopsy. In this paper, we describe the most common types of CHDs that a pathologist could encounter at autopsy, including the various types of surgical and percutaneous procedures and major pathological manifestations. We also propose a practical systematic approach to the autopsy of ACHD patients.
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Affiliation(s)
- Annalisa Angelini
- Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
| | - Cira di Gioia
- Department of Radiological, Oncological and Pathological Sciences, Sapienza, University of Rome, Rome, Italy
| | - Helen Doran
- Department of Pathology, Manchester Foundation Trust Wythenshawe Hospital, Manchester, UK
| | - Marny Fedrigo
- Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Rosa Henriques de Gouveia
- Department of Pathology, Hospital de Santa Cruz (CHLO), Lisbon & Forensic Pathology, INMLCF & FMUC, Coimbra, Portugal
| | - Siew Yen Ho
- Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, London, UK
| | - Ornella Leone
- Department of Pathology, Sant'Orsola-Malpighi University Hospital, Bologna, Italy
| | - Mary N Sheppard
- Department of Cardiovascular Pathology, St Georges Medical School, London, UK
| | - Gaetano Thiene
- Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Konstantinos Dimopoulos
- Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, London, UK
| | - Barbara Mulder
- Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands
| | - Massimo Padalino
- Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Allard C van der Wal
- Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands.
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Iriart X, Le Quellenec S, Pillois X, Jaussaud J, Jalal Z, Roubertie F, Douard H, Cochet H, Thambo JB. Heart rate response during exercise predicts exercise tolerance in adults with transposition of the great arteries and atrial switch operation. Int J Cardiol 2020; 299:116-122. [PMID: 31405586 DOI: 10.1016/j.ijcard.2019.07.054] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2019] [Revised: 06/11/2019] [Accepted: 07/15/2019] [Indexed: 11/17/2022]
Abstract
BACKGROUND To assess the relationship between heart rate response and exercise tolerance in adults with systemic right ventricle (sRV) after atrial switch repair for Transposition of the Great Artery (TGA) in addition to other physiological parameters. METHODS All patients with a sRV after atrial switch repair for TGA followed in our institution between June 2015 and April 2018 who underwent cardiopulmonary exercise testing (CPET) were analyzed. Cardiac imaging performed within a six-month time period of the CPET were also collected. Chronotropic incompetence was defined as the inability to achieve 80% of age-predicted maximal heart rate reserve (HRR) and <62% on a beta-blocker regimen. Patient characteristics were assessed according to tertiles of the percentage of predicted pVO2 (%ppVO2). RESULTS We studied 70 patients (mean of age 32.4 ± 7.6 years old, 51 males). Mean peak oxygen uptake was 21.5 ± 5.8 mL/kg/min, corresponding to a %ppVO2 of 57 ± 14.1% while mean VE/VCO2 slope was 37.1 ± 8.2. There was a trend toward more exaggerated hyperventilation in patient with lower pVO2. Mean age-adjusted HRR was 68.5 ± 19%. Chronotropic incompetence was observed in 65.7% and was correlated with %ppVO2 (r = 0.482; p < 0.001) as physical training evaluated with Ventilatory Anaerobic threshold (r = 0.571; p < 0.001), while no difference was found based on respiratory parameters. No echocardiographic or Magnetic Resonance Imaging parameters assessing sRV systolic function at rest were correlated with %ppVO2. CONCLUSIONS Exercise limitation is related to the inability to increase cardiac output during exercise and is notably due to the degree of chronotropic incompetence.
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Affiliation(s)
- Xavier Iriart
- Department of Pediatric and Adult Congenital Cardiology, Bordeaux University Hospital, 33604 Pessac, France.
| | - Soazig Le Quellenec
- Department of Pediatric and Adult Congenital Cardiology, Bordeaux University Hospital, 33604 Pessac, France
| | - Xavier Pillois
- LIRYC, L'institut de rythmologie et de modélisation cardiaque, Bordeaux University, France
| | | | - Zakaria Jalal
- Department of Pediatric and Adult Congenital Cardiology, Bordeaux University Hospital, 33604 Pessac, France; LIRYC, L'institut de rythmologie et de modélisation cardiaque, Bordeaux University, France
| | - François Roubertie
- Department of Pediatric and Adult Congenital Cardiology, Bordeaux University Hospital, 33604 Pessac, France; LIRYC, L'institut de rythmologie et de modélisation cardiaque, Bordeaux University, France
| | - Hervé Douard
- Cardiac Rehabilitation Department, Bordeaux University Hospital, 33604 Pessac, France
| | - Hubert Cochet
- Department of Cardiovascular imaging, Bordeaux University Hospital, 33604 Pessac, France; LIRYC, L'institut de rythmologie et de modélisation cardiaque, Bordeaux University, France
| | - Jean-Benoît Thambo
- Department of Pediatric and Adult Congenital Cardiology, Bordeaux University Hospital, 33604 Pessac, France; Clinique du sport, Mérignac, France
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Affiliation(s)
- Marie-A Chaix
- Montreal Heart Institute Adult Congenital Center, Université de Montréal, Montreal, Canada
| | - Paul Khairy
- Montreal Heart Institute Adult Congenital Center, Université de Montréal, Montreal, Canada
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Venkatesh P, Evans AT, Maw AM, Pashun RA, Patel A, Kim L, Feldman D, Minutello R, Wong SC, Stribling JC, LaPar D, Holzer R, Ginns J, Bacha E, Singh HS. Predictors of Late Mortality in D-Transposition of the Great Arteries After Atrial Switch Repair: Systematic Review and Meta-Analysis. J Am Heart Assoc 2019; 8:e012932. [PMID: 31642369 PMCID: PMC6898856 DOI: 10.1161/jaha.119.012932] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Background Existing data on predictors of late mortality and prevention of sudden cardiac death after atrial switch repair surgery for D‐transposition of the great arteries (D‐TGA) are heterogeneous and limited by statistical power. Methods and Results We conducted a systematic review and meta‐analysis of 29 observational studies, comprising 5035 patients, that reported mortality after atrial switch repair with a minimum follow‐up of 10 years. We also examined 4 additional studies comprising 105 patients who reported rates of implantable cardioverter‐defibrillator therapy in this population. Average survival dropped to 65% at 40 years after atrial switch repair, with sudden cardiac death accounting for 45% of all reported deaths. Mortality was significantly lower in cohorts that were more recent and operated on younger patients. Patient‐level risk factors for late mortality were history of supraventricular tachycardia (odds ratio [OR] 3.8, 95% CI 1.4–10.7), Mustard procedure compared with Senning (OR 2.9, 95% CI 1.9–4.5) and complex D‐TGA compared with simple D‐TGA (OR 4.4, 95% CI 2.2–8.8). Significant risk factors for sudden cardiac death were history of supraventricular tachycardia (OR 4.7, 95% CI 2.2–9.8), Mustard procedure (OR 2.2, 95% CI 1.1–4.1), and complex D‐TGA (OR 5.7, 95% CI 1.8–18.0). Out of a total 124 implantable cardioverter‐defibrillator discharges over 330 patient‐years in patients with implantable cardioverter‐defibrillators for primary prevention, only 8% were appropriate. Conclusions Patient‐level risk of both mortality and sudden cardiac death after atrial switch repair are significantly increased by history of supraventricular tachycardia, Mustard procedure, and complex D‐TGA. This knowledge may help refine current selection practices for primary prevention implantable cardioverter‐defibrillator implantation, given disproportionately high rates of inappropriate discharges.
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Affiliation(s)
- Prashanth Venkatesh
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Arthur T Evans
- Division of Hospital Medicine Weill Cornell Medicine New York Presbyterian Hospital New York NY
| | - Anna M Maw
- Division of Hospital Medicine Weill Cornell Medicine New York Presbyterian Hospital New York NY
| | - Raymond A Pashun
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Agam Patel
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Luke Kim
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Dmitriy Feldman
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Robert Minutello
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - S Chiu Wong
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Judy C Stribling
- Weill Cornell Medicine Samuel J. Wood Library Myra Mahon Patient Resource Center New York NY
| | - Damian LaPar
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Ralf Holzer
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Jonathan Ginns
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Emile Bacha
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
| | - Harsimran S Singh
- Division of Cardiology Departments of Medicine and Pediatrics Weill Cornell Medicine New York Presbyterian Hospital Cornell Center for Adult Congenital Heart Disease New York NY
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Abstract
Cardiac defects are the most common congenital defects, accounting for approximately 9 per 1000 births. Patients with structural heart disease related to congenital diseases are prone to develop intrinsic rhythm abnormalities as a result of altered physiology. In addition, they are at an increased risk of developing acquired arrhythmias secondary to the nature of surgical interventions done to improve physiologic function in the setting of these defects. Arrhythmia management and risk stratification pose particularly complex challenges to clinicians managing this population.
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Affiliation(s)
- Jessica Kline
- Summa Health Heart & Vascular Institute, Summa Health System, 95 Arch Street, Suite 300, Akron, OH 44304, USA
| | - Otto Costantini
- Cardiovascular Disease Fellowship, Summa Health Heart & Vascular Institute, Summa Health System, 95 Arch Street, Suite 350, Akron, OH 44304, USA.
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Devlin PJ, Jegatheeswaran A, Williams WG, Blackstone EH, DeCampli WM, Lambert LM, Mussatto KA, Prospero CJ, Bondarenko I, McCrindle BW. Late Survival and Patient-Perceived Health Status of the Congenital Heart Surgeons' Society dextro-Transposition of the Great Arteries Cohort. Ann Thorac Surg 2019; 108:1447-1455. [PMID: 31348901 DOI: 10.1016/j.athoracsur.2019.05.081] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/05/2019] [Revised: 05/17/2019] [Accepted: 05/21/2019] [Indexed: 11/28/2022]
Abstract
BACKGROUND Improved survival for patients with dextro-transposition of the great arteries (d-TGA) has led to an increased focus on functional health status (FHS). We assessed late survival and patient-perceived FHS for repaired TGA patients. METHODS From 1985-1990, 830 neonates admitted to 24 Congenital Heart Surgeons' Society (CHSS) institutions with d-TGA underwent repair, including 516 arterial switch, 110 Mustard, 175 Senning, and 29 Rastelli operations. Median follow-up was 24.0 years (range, 0-32.7 years). We performed multiphase parametric hazard analysis for death after repair. Patients completed Pediatric Quality of Life Inventory (PedsQL) Core Scales and Cardiac Module Adult Forms. Patient and operative factors and CHSS General Questionnaire responses were analyzed for association with FHS using multiple linear regression. RESULTS Survival at 30 years after repair was arterial switch, 80% ± 2%; Mustard, 81% ± 5%; Senning, 70% ± 4%; and Rastelli, 86% ± 8%. The arterial switch had the lowest hazard for late death. TGA patients reported FHS similar to a healthy population in all domains except physical health (lower scores). Symptoms, including chest pain and fainting, and having a pacemaker were associated with lower, and being employed with higher, self-reported physical health. Arterial switch patients reported higher FHS than the atrial switch patients in all domains. CONCLUSIONS Arterial switch patients have a lower risk of premature death and better FHS than those with an atrial switch. Increased surveillance in atrial switch patients is warranted because of their increased risk of late death. Presence of symptoms, pacemaker, and lack of employment are associated with reduced FHS.
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Affiliation(s)
- Paul J Devlin
- Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
| | - Anusha Jegatheeswaran
- Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada
| | - William G Williams
- Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Eugene H Blackstone
- Division of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio
| | - William M DeCampli
- Division of Pediatric Cardiac Surgery, Arnold Palmer Hospital for Children, Orlando, Florida
| | - Linda M Lambert
- Department of Pediatrics, Primary Children's Hospital, Salt Lake City, Utah
| | - Kathleen A Mussatto
- Herma Heart Institute, Children's Hospital of Wisconsin, Milwaukee, Wisconsin
| | - Carol J Prospero
- Nemours Cardiac Center, Alfred I. DuPont Hospital for Children, Wilmington, Delaware
| | - Igor Bondarenko
- Department of Cardiovascular Surgery, Children's Hospital of Michigan, Detroit, Michigan
| | - Brian W McCrindle
- Division of Pediatric Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada
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Roberts WC, Jameson LC, Bahmani A, Roberts CS, Rafael AE, Hall SA. Morphological and Functional Characteristics of the Right Ventricle Functioning as a Systemic Ventricle for Decades After an Atrial Switch Procedure for Complete Transposition of the Great Arteries. Am J Cardiol 2019; 123:1863-1867. [PMID: 30955865 DOI: 10.1016/j.amjcard.2019.02.052] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2019] [Revised: 02/27/2019] [Accepted: 02/28/2019] [Indexed: 10/27/2022]
Abstract
Described herein are certain clinical and cardiac morphologic findings in 4 adults with complete transposition of the great arteries who underwent the Mustard procedure in the newborn period or in childhood and each lived >30 years thereafter before either having orthotopic heart transplantation (3 patients) or dying while awaiting orthotopic heart transplantation. Compared with the wall of the left ventricle, the wall of the right ventricle (the systemic one) was much thicker, the myofibers much larger, and either grossly-visible or microscopic-sized scars were present in its wall. Additionally, some intramural coronary arteries in the right ventricular wall were numerous, large, had thick walls, and often narrowed lumens. That the Mustard operation provided the necessary time for the right ventricle (the systemic one) to develop to its fullest is a tribute to this procedure.
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Van De Bruaene A, Toh N, Hickey EJ, Benson L, Horlick E, Granton JT, Williams WG, Roche SL. Pulmonary hypertension in patients with a subaortic right ventricle: prevalence, impact and management. Heart 2019; 105:1471-1478. [DOI: 10.1136/heartjnl-2019-314756] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/15/2019] [Revised: 04/04/2019] [Accepted: 04/10/2019] [Indexed: 02/06/2023] Open
Abstract
ObjectiveThis study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV).MethodsWe analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy.ResultsA total of 141 patients (median age 39 (IQR 33–45) years, 68% men) underwent 191 cardiac catheterisations. At baseline, 55% had PH (isolated postcapillary 24%, combined precapillary and postcapillary 26% and precapillary 5%). BNP (area under the curve 0.80; 95% CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3–3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time.ConclusionPH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.
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Garside H, Leyva F, Hudsmith L, Marshall H, de Bono J. Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population. PACING AND CLINICAL ELECTROPHYSIOLOGY: PACE 2018; 42:65-70. [DOI: 10.1111/pace.13537] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/20/2018] [Revised: 09/03/2018] [Accepted: 10/03/2018] [Indexed: 11/29/2022]
Affiliation(s)
- Hannah Garside
- Department of Cardiology; Queen Elizabeth Hospital; Birmingham United Kingdom
| | - Francisco Leyva
- Department of Cardiology; Queen Elizabeth Hospital; Birmingham United Kingdom
- Aston Medical Research Institute, Aston Medical School; Aston University; Birmingham United Kingdom
| | - Lucy Hudsmith
- Department of Cardiology; Queen Elizabeth Hospital; Birmingham United Kingdom
| | - Howard Marshall
- Department of Cardiology; Queen Elizabeth Hospital; Birmingham United Kingdom
| | - Joseph de Bono
- Department of Cardiology; Queen Elizabeth Hospital; Birmingham United Kingdom
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Bishnoi AK, Patel K, Agrawal P, Ananthanarayanan C, Garg P, Pandya H. Damus-Kaye-Stansel: Valuable Option for Retraining of Left Ventricle in Late Arterial Switch for Transposition of the Great Arteries. Ann Thorac Surg 2018; 107:e389-e391. [PMID: 30481515 DOI: 10.1016/j.athoracsur.2018.10.044] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2018] [Revised: 10/11/2018] [Accepted: 10/13/2018] [Indexed: 10/27/2022]
Abstract
Management of dextro-transposition of the great arteries with intact interventricular septum along with regression of the left ventricle remains a challenge. Atrial switch always raises concerns about the fate of the right ventricle as a systemic ventricle in the long run. A two-stage repair with pulmonary artery banding and a modified Blalock-Taussig shunt or a single-stage arterial switch with mechanical support has a higher mortality and morbidity. This report describes a case of a 3-year-old girl with dextro-transposition of the great arteries with intact interventricular septum that was managed by a Damus-Kaye-Stansel procedure with a right bidirectional cavopulmonary anastomosis in the first stage to train the left ventricle, followed by a definitive operation (arterial switch) in the second stage.
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Affiliation(s)
- Arvind Kumar Bishnoi
- Department of Cardiovascular and Thoracic Surgery, Kasturba Medical College, Manipal, India
| | - Kartik Patel
- Department of Cardiovascular and Thoracic Surgery, U.N. Mehta Institute of Cardiology and Research Centre, B.J. Medical College, Ahmedabad, India.
| | - Prashant Agrawal
- Department of Pediatric Cardiology, U.N. Mehta Institute of Cardiology and Research Centre, B.J. Medical College, Ahmedabad, India
| | - Chandrasekaran Ananthanarayanan
- Department of Cardiovascular and Thoracic Surgery, U.N. Mehta Institute of Cardiology and Research Centre, B.J. Medical College, Ahmedabad, India
| | - Pankaj Garg
- Department of Cardiovascular and Thoracic Surgery, U.N. Mehta Institute of Cardiology and Research Centre, B.J. Medical College, Ahmedabad, India
| | - Himani Pandya
- Department of Research, U.N. Mehta Institute of Cardiology and Research Centre, B.J. Medical College, Ahmedabad, India
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Couperus LE, Vliegen HW, Zandstra TE, Kiès P, Jongbloed MRM, Holman ER, Zeppenfeld K, Hazekamp MG, Schalij MJ, Scherptong RWC. Long-term outcome after atrial correction for transposition of the great arteries. Heart 2018; 105:790-796. [DOI: 10.1136/heartjnl-2018-313647] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/21/2018] [Revised: 10/18/2018] [Accepted: 10/25/2018] [Indexed: 11/04/2022] Open
Abstract
ObjectiveThis study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to Mustard or Senning for transposition of the great arteries (TGA).MethodsIn 76 adult patients with TGA (59% male) after atrial correction, long-term survival and morbidity were investigated in three periods: early (<15 years postoperatively), midterm (15–30 years postoperatively) and late (>30 years postoperatively).ResultsThe Mustard technique was performed in 41 (54%) patients, and the Senning technique was performed in 35 (46%) patients aged 3.1 (IQR: 2.1–3.8) and 1.0 (IQR: 0.6–3.1; p<0.01) years, respectively. Adult survival was 82% at 39.7 (IQR: 35.9–42.4) years postoperatively and exceeded 50 years in four patients. Supraventricular tachycardia (SVT) occurred in 51% of patients. The incidences of ventricular arrhythmia (0%, 8% and 13%; p<0.01), heart failure (0%, 5% and 19%; p<0.01) and surgical reinterventions (0%, 5% and 11%; p=0.01) increased from early to late follow-up. At last follow-up, RV function was depressed in 31 (46%) patients, and New York Heart Association functional class was ≥2 in 34 (48%) patients. Bradyarrhythmia, SVT and ventricular arrhythmia were associated with depressed RV function (OR: 4.47, 95% CI 1.50 to 13.28, p<0.01; OR: 3.74, 95% CI 1.26 to 11.14, p=0.02; OR: 14.40, 95% CI 2.80 to 74.07, p<0.01, respectively) and worse functional capacity (OR: 2.10, 95% CI 0.75 to 5.82, p=0.16; OR: 2.87, 95% CI 1.06 to 7.81, p=0.04; OR: 8.47, 95% CI 1.70 to 42.10, p<0.01, respectively).ConclusionsIn adult patients with TGA, survival was 82% at 39.7 (IQR: 35.9–42.4) years after atrial correction. Morbidity was high and included SVT as most frequent adverse event. Ventricular arrhythmias, heart failure and surgical reinterventions were common during late follow-up. Adverse events were associated with depressed right ventricle function and reduced functional class.
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Michel E, Orozco Hernandez E, Enter D, Monge M, Nakano J, Rich J, Anderson A, Backer C, McCarthy P, Pham D. Bridge to Transplantation With Long-Term Mechanical Assist Devices in Adults With Transposition of the Great Arteries. Artif Organs 2018; 43:90-96. [PMID: 30129258 DOI: 10.1111/aor.13347] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2018] [Revised: 07/07/2018] [Accepted: 08/15/2018] [Indexed: 11/30/2022]
Abstract
Prior to the widespread adoption of the arterial switch operation, patients with transposition of the great arteries (TGA) commonly underwent atrial switch operation (Mustard or Senning). It is not uncommon for these patients to progress to end stage heart failure and increasingly ventricular assist devices (VADs) are used to support these patients as a bridge to transplantation, though there is limited experience with this worldwide. A retrospective review of our institution's VAD database was undertaken and revealed seven adult patients with a history of TGA and subsequent systemic ventricular failure were implanted with a VAD: four of whom received the VAD as a bridge to transplantation (BTT) at the time of implantation, two who were initially designated as destination therapy secondary to severe pulmonary hypertension, and one who was designated as destination therapy secondary to a high risk of life-threatening non-compliance. Seven patient cases who received a VAD for severe systemic ventricular failure were included in this study. The mean age of the patients was 40 years and the majority of patients were male (6/7, 85%). Five of the patients (71.4%) had previously undergone an atrial switch operation and all of these were Mustard procedures. Two of the seven patients (28.5%) had congenitally corrected transposition of the great arteries (CC-TGA). Two of the seven patients (28.5%) had supra-systemic pulmonary pressures before VAD implantation and were designated as destination therapy (DT). One of these patients was later designated as BTT as an improvement in his pulmonary vascular resistance was observed, and subsequently underwent heart transplantation. Because of anatomic considerations, four of the patients (57%) underwent redo-sternotomy with outflow cannula anastomosis to the ascending aorta, one patient underwent VAD implantation via a left subcostal incision with anastomosis of the outflow graft to the descending thoracic aorta, and two patients (28.5%) underwent VAD implantation via a left thoracotomy and anastomosis of the outflow cannula to the descending thoracic aorta. Six of the seven patients had a HeartWare HVAD VAD implanted; one received a Thoratec Heartmate II VAD. Two patients underwent VAD explant and orthotopic heart transplant, 222 days and 444 days after VAD implant, respectively. One patient died on postoperative day 17 after complications from recurrent VAD thrombosis despite multiple pump exchanges. Four patients remain on VAD support, three of these patients are awaiting transplantation at last follow-up (mean days on support, 513 days). Bridge to transplantation with a durable VAD is technically feasible and relatively safe in patients with TGA. Multiple redo-sternotomies can be avoided with a left posterior thoracotomy approach and outflow graft anastomosis to the descending thoracic aorta after careful anatomic considerations.
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Affiliation(s)
- Eriberto Michel
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Erik Orozco Hernandez
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Daniel Enter
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Michael Monge
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Ann & Robert H. Lurie Children's Hospital of Chicago, Cardiovascular-Thoracic Surgery, Chicago, IL, USA
| | - Jota Nakano
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Bluhm Cardiovascular Institute, Northwestern Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Jonathan Rich
- Bluhm Cardiovascular Institute, Northwestern Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Division of Cardiology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Allen Anderson
- Bluhm Cardiovascular Institute, Northwestern Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Division of Cardiology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Carl Backer
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Ann & Robert H. Lurie Children's Hospital of Chicago, Cardiovascular-Thoracic Surgery, Chicago, IL, USA
| | - Patrick McCarthy
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Bluhm Cardiovascular Institute, Northwestern Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Duc Pham
- Division of Cardiac Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.,Bluhm Cardiovascular Institute, Northwestern Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
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Management of Heart Failure in Adult Congenital Heart Disease. Prog Cardiovasc Dis 2018; 61:308-313. [DOI: 10.1016/j.pcad.2018.08.003] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/09/2018] [Accepted: 08/09/2018] [Indexed: 12/14/2022]
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Dennis M, Kotchetkova I, Cordina R, Celermajer DS. Long-Term Follow-up of Adults Following the Atrial Switch Operation for Transposition of the Great Arteries – A Contemporary Cohort. Heart Lung Circ 2018. [DOI: 10.1016/j.hlc.2017.10.008] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Ghelani SJ, Colan SD, Azcue N, Keenan EM, Harrild DM, Powell AJ, Geva T, Rathod RH. Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients. Circ Cardiovasc Imaging 2018; 11:e006738. [DOI: 10.1161/circimaging.117.006738] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/30/2017] [Accepted: 05/10/2018] [Indexed: 11/16/2022]
Affiliation(s)
- Sunil J. Ghelani
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - Steven D. Colan
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - Nina Azcue
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - Ellen M. Keenan
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - David M. Harrild
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - Andrew J. Powell
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - Tal Geva
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
| | - Rahul H. Rathod
- Department of Cardiology, Boston Children’s Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA
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Abstract
OBJECTIVE Cardiovascular disease is the major cause of pregnancy-related maternal mortality in the United States, and congenital heart disease (CHD) is the most common form of structural heart disease affecting women of childbearing age. Most females born with CHD will reach childbearing age and consider pregnancy. Adult CHD and maternal-fetal medicine (MFM) specialists managing women with CHD should provide preconception counseling, cardiovascular risk assessment prior to pregnancy that estimates maternal and fetal risk, management during pregnancy, and in the peripartum period and also know the potential complications and special circumstances that may occur in the post-partum period. This chapter will review the population at risk, patient risk prior to pregnancy, management during pregnancy, management in the peripartum and post-partum periods, and outline specific cardiovascular complications. The chapter will also briefly review some common or high-risk congenital cardiovascular lesions commonly encountered. CONCLUSION Management of patients with most forms of CHD encountered during pregnancy requires a multidisciplinary approach and careful team-based care to facilitate safe and appropriate management and pregnancy success.
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Affiliation(s)
- Heidi M Connolly
- Department of Cardiovascular Medicine, Mayo Clinic, 200 First St, SW, Rochester, MN 55905.
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Jouan J, Grinda JM, Bricourt MO, Iserin L, Fabiani JN. Non-Pulsatile Axial flow Ventricular Assist Device for Right Systemic Ventricle failure Late after Senning Procedure. Int J Artif Organs 2018; 32:243-5. [DOI: 10.1177/039139880903200409] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Cardiac transplantation is an ever more frequent requirement for patients presenting with end-stage right systemic ventricular failure late after atrial switch operations. But as the time on the donor waiting list lengthens, the clinical conditions of these patients can easily and abruptly deteriorate, sometimes requiring systemic ventricular assistance. We document the first case of right systemic ventricular assistance with a De Bakey VAD axial pump in a patient presenting with systemic ventricular failure 23 years after procedure. He rapidly recovered total autonomy and was thus able to participate in a rehabilitation program to optimize his condition for heart transplantation.
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Affiliation(s)
- Jérôme Jouan
- Department of Cardiovascular Surgery, Georges Pompidou European Hospital, Paris Descartes University, Paris
| | - Jean-Michel Grinda
- Department of Cardiovascular Surgery, Georges Pompidou European Hospital, Paris Descartes University, Paris
| | - Marie-Odile Bricourt
- Department of Cardiovascular Surgery, Georges Pompidou European Hospital, Paris Descartes University, Paris
| | - Laurence Iserin
- Department of Cardiology, Georges Pompidou European Hospital, Paris Descartes University, Paris - France
| | - Jean-Noël Fabiani
- Department of Cardiovascular Surgery, Georges Pompidou European Hospital, Paris Descartes University, Paris
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Van De Bruaene A, Meier L, Droogne W, De Meester P, Troost E, Gewillig M, Budts W. Management of acute heart failure in adult patients with congenital heart disease. Heart Fail Rev 2017; 23:1-14. [DOI: 10.1007/s10741-017-9664-x] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
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