1
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Sicim H, Noly PE, Naik S, Sood V, Ohye RG, Haft JW, Aaronson KD, Pagani FD, Si MS, Tang PC. Determinants of survival following heart transplantation in adults with congenital heart disease. J Cardiothorac Surg 2024; 19:83. [PMID: 38336724 PMCID: PMC10858543 DOI: 10.1186/s13019-024-02509-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2023] [Accepted: 01/23/2024] [Indexed: 02/12/2024] Open
Abstract
BACKGROUND Adult patients surviving with congenital heart disease (ACHD) is growing. We examine the factors associated with heart transplant outcomes in this challenging population with complex anatomy requiring redo-surgeries. METHODS We reviewed the United Network for Organ Sharing-Standard Transplant Analysis and Research database and analyzed 35,952 heart transplants from January 1st, 2000, to September 30th, 2018. We compared transplant characteristics for ischemic cardiomyopathy (ICM) (n = 14,236), nonischemic cardiomyopathy (NICM) (n = 20,676), and ACHD (n = 1040). Mean follow-up was 6.20 ± 4.84 years. Kaplan-Meier survival curves and Cox-proportional hazards analysis were used to analyze survival data. RESULTS Multivariable analysis confirmed that ACHD was associated greater in-hospital death compared to ICM (HR = 0.54, P < 0.001) and NICM (HR = 0.46, P < 0.001). Notable factors associated with increased mortality were history of cerebrovascular disease (HR = 1.11, P = 0.026), prior history of malignancy (HR = 1.12, P = 0.006), pre-transplant biventricular support (HR = 1.12, P = 0.069), postoperative stroke (HR = 1.47, P < 0.001) and postoperative dialysis (HR = 1.71, P < 0.001). ACHD transplants had a longer donor heart ischemic time (P < 0.001) and trend towards more deaths from primary graft dysfunction (P = 0.07). In-hospital deaths were more likely with ACHD and use of mechanical support such as use of right ventricular assist device (HR = 2.20, P = 0.049), biventricular support (HR = 1.62, P < 0.001) and extracorporeal membrane oxygenation (HR = 2.36, P < 0.001). Conditional survival after censoring hospital deaths was significantly higher in ACHD (P < 0.001). CONCLUSION Heart transplant in ACHD is associated with a higher post-operative mortality given anatomical complexity but a better long-term conditional survival. Normothermic donor heart perfusion may improve outcomes in the ACHD population by reducing the impact of longer ischemic times.
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Affiliation(s)
- Hüseyin Sicim
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
- Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA
| | | | - Suyash Naik
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
| | - Vikram Sood
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
| | - Richard G Ohye
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
| | - Jonathan W Haft
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
| | - Keith D Aaronson
- Department of Internal Medicine, Division of Cardiovascular Medicine, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
| | - Francis D Pagani
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA
| | - Ming-Sing Si
- Department of Surgery, Division of Cardiac Surgery, University of California Los Angeles, Los Angeles, CA, USA
| | - Paul C Tang
- Department of Cardiac Surgery, University of Michigan Frankel Cardiovascular Center, Ann Arbor, MI, USA.
- Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
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2
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Mahmoud M, El Kortbi K, Wang H, Wang J. Incidental Finding of Heterotaxy Syndrome in a Patient With Pulmonary Embolism: A Case Report and Concise Review. Cureus 2022; 14:e24326. [PMID: 35607583 PMCID: PMC9122844 DOI: 10.7759/cureus.24326] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/20/2022] [Indexed: 12/03/2022] Open
Abstract
Heterotaxy syndrome, also called atrial isomerism, is a rare congenital condition in which the internal organs are abnormally arranged across the left-right axis of the body. It is classified into polysplenia syndrome or left atrial isomerism and asplenia syndrome or right atrial isomerism. It is associated with high morbidity and mortality due to the severity of cardiac anomalies. It is important to be aware of the syndrome findings as they can be incidentally found on imaging in adults. Here, we report a case of a 33-year-old female who presented with worsening shortness of breath, found to have a pulmonary embolism, and heterotaxy was incidentally identified on her imaging. A concise review follows.
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3
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Yuki K. Anesthetic Management for Heart Transplantation in Adults with Congenital Heart Disease. ACTA ACUST UNITED AC 2020; 7:248-252. [PMID: 32377555 PMCID: PMC7202566 DOI: 10.31480/2330-4871/120] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Abstract
As the outcome of patients with congenital heart disease (CHD) has improved, the number of adults with congenital heart disease (ACHD) outnumbered pediatric population with CHD. Heart failure is responsible for 40% of mortality among ACHD, and the number of heart transplantation for ACHD is gradually increasing. However, the early mortality rate of heart transplantation is significantly higher in ACHD than in non-ACHD. Understanding the unique characteristics of heart transplantation in ACHD is critical. In contrast to their early outcome their long-term survival is better in ACHD than in non-CHD patients, and they are likely to present to anesthesia care after heart transplantation for various reasons. Understanding specific issues in post-transplant anesthesia care is another important aspect.
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Affiliation(s)
- Koichi Yuki
- Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, USA.,Department of Anaesthesia, Harvard Medical School, USA
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4
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Advanced Heart Failure Therapies for Adults With Congenital Heart Disease. J Am Coll Cardiol 2019; 74:2295-2312. [DOI: 10.1016/j.jacc.2019.09.004] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/12/2019] [Revised: 08/20/2019] [Accepted: 09/03/2019] [Indexed: 12/15/2022]
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5
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Bradford TT, Daily JA, Lang SM, Gossett JM, Tang X, Collins RT. Comparison of inhospital outcomes of pediatric heart transplantation between single ventricle congenital heart disease and cardiomyopathy. Pediatr Transplant 2019; 23:e13495. [PMID: 31169342 DOI: 10.1111/petr.13495] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/29/2018] [Revised: 03/25/2019] [Accepted: 04/23/2019] [Indexed: 11/29/2022]
Abstract
Data investigating the impact of household income and other factors on SV patient status-post-Fontan palliation after heart transplantation are lacking. We aim to evaluate factors affecting outcomes after OHT in this population. The PHIS database was interrogated for either SV or myocarditis/primary CM who were 4 years or older who underwent a single OHT. There were 1599 patients with a median age of 13.2 years (IQR: 9.3-16.1). Total hospital costs were significantly higher in the SV group ($408 000 vs $294 000, P < 0.0001), but as median household income increased, the risk of inhospital mortality, post-transplant LOS, and LOS-adjusted total hospital costs all decreased. The risk of inhospital mortality increased 6.5% per 1 year of age increase at the time of transplant. Patients in the SV group had significantly more diagnoses than those in the CM group (21 vs 15, P < 0.0001) and had longer total hospital LOSs as a result of longer post-transplant courses (25 days vs 15, P < 0.0001). Increased median household income and younger age are associated with decreased resource utilization and improved inhospital mortality in SV CHD patients who undergo OHT. In conclusion, earlier consideration of OHT in this population, coupled with improved selection criteria, may increase survival in this population.
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Affiliation(s)
- Tamara T Bradford
- University of Arkansas for Medical Sciences, Little Rock, Arkansas.,Arkansas Children's Research Institute, Little Rock, Arkansas
| | - Joshua A Daily
- University of Arkansas for Medical Sciences, Little Rock, Arkansas.,Arkansas Children's Research Institute, Little Rock, Arkansas
| | - Sean M Lang
- University of Cincinnati College of Medicine, Cincinnati, Ohio.,Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
| | - Jeffrey M Gossett
- University of Arkansas for Medical Sciences, Little Rock, Arkansas.,Arkansas Children's Research Institute, Little Rock, Arkansas
| | - Xinyu Tang
- University of Arkansas for Medical Sciences, Little Rock, Arkansas.,Arkansas Children's Research Institute, Little Rock, Arkansas
| | - R Thomas Collins
- Stanford University School of Medicine, Palo Alto, California.,Lucile Packard Children's Hospital Stanford, Palo Alto, California
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6
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7
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Transplantation and Mechanical Circulatory Support in Adult Congenital Heart Disease-Related Advanced Heart Failure. HEART FAILURE IN ADULT CONGENITAL HEART DISEASE 2018. [DOI: 10.1007/978-3-319-77803-7_13] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
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8
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Matsuda H, Ichikawa H, Ueno T, Sawa Y. Heart transplantation for adults with congenital heart disease: current status and future prospects. Gen Thorac Cardiovasc Surg 2017; 65:309-320. [PMID: 28439697 DOI: 10.1007/s11748-017-0777-x] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2017] [Accepted: 04/10/2017] [Indexed: 02/07/2023]
Abstract
Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved. Survivors of Fontan procedures are a vulnerable cohort, particularly when single ventricle physiology fails, mostly with protein-losing enteropathy and hepatic dysfunction. Therefore, we reviewed single-institution and larger database analyses of adults who underwent heart transplantation for CHD, to enable risk stratification by identifying the indications and outcomes. As the results, despite relatively high early mortality, long-term results were encouraging after heart transplantation. However, further investigations are needed to improve the indication criteria for complex CHD, especially for failed Fontan. In addition, the current system of status criteria and donor heart allocation system in heart transplantation should be arranged as suitable for adults with complex CHD. Furthermore, there is a strong need to develop ventricular assist devices as a bridge to transplantation or destination therapy, especially where right-sided circulatory support is needed.
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Affiliation(s)
- Hikaru Matsuda
- Professor Emeritus Osaka University, Cardiovascular Surgery, Higashi-Takarazuka Satoh Hospital, Nagao-cho 2-1, Takarazuka, Hyogo, Japan.
| | - Hajime Ichikawa
- Division of Pediatric Cardiac Sergey, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Takayoshi Ueno
- Department of Cardiovascular Surgery, Graduate School of Medicine, Osaka University, Suita, Osaka, Japan
| | - Yoshiki Sawa
- Department of Cardiovascular Surgery, Graduate School of Medicine, Osaka University, Suita, Osaka, Japan
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9
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Smith ML, McGuinness J, O'Reilly MK, Nolke L, Murray JG, Jones JFX. The role of 3D printing in preoperative planning for heart transplantation in complex congenital heart disease. Ir J Med Sci 2017; 186:753-756. [PMID: 28124282 DOI: 10.1007/s11845-017-1564-5] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2017] [Accepted: 01/16/2017] [Indexed: 02/06/2023]
Abstract
BACKGROUND The presence of a structural cardiac defect in the setting of dextrocardia is extremely rare. Graspable models allow enhanced appreciation of aberrant structures and vascular relations, particularly in rare and complex cases. This is the first case report of the use of a replica of a patients' anatomy to plan the surgical strategy in the setting of dextrocardia. AIMS We intend to demonstrate the benefit of three-dimensional printing to enhance preoperative planning in complex congenital heart disease undergoing heart transplantation. The anomalous structures encountered include situs inversus dextrocardia, transposition of the great vessels, a single atrium and a dilated double-outlet single right ventricle. METHODS Computed Tomography acquisition was performed with the use of ECG multiphase gating technology and contrast enhancement. The structures of interest were segmented and the generated 3D mesh was exported as a stereolithographic (STL) file. The model was printed on a Z-Corp 250 binder jetting printer. Post processing techniques were used to enhance model strength. RESULTS Pre-operative 3D visualisation of the patients' anatomy allowed for a more comprehensive surgical strategy to be planned, thus reducing the intra-operative duration and cross-clamp time which are recognised to correlate with reduced patient morbidity. CONCLUSION The ongoing advances in medical image procurement and 3D processing software and printing technology will continue to enhance preoperative planning and thereby improve patient care. We demonstrate the pivotal role played by such technologies in advancing spatial comprehension of complex aberrant anatomy.
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Affiliation(s)
- M L Smith
- Discipline of Anatomy, School of Medicine, University College Dublin, Dublin 4, Ireland.
| | - J McGuinness
- Department of Cardiothoracic Surgery, Mater Misericordiae University Hospital, Eccles Street, Dublin, Ireland
| | - M K O'Reilly
- Department of Radiology, Mater Misericordiae University Hospital, Eccles Street, Dublin, Ireland
| | - L Nolke
- Department of Cardiothoracic Surgery, Mater Misericordiae University Hospital, Eccles Street, Dublin, Ireland
| | - J G Murray
- Department of Radiology, Mater Misericordiae University Hospital, Eccles Street, Dublin, Ireland
| | - J F X Jones
- Discipline of Anatomy, School of Medicine, University College Dublin, Dublin 4, Ireland
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10
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Pundi KN, Pundi K, Driscoll DJ, Dearani JA, Li Z, Dahl SH, Mora BN, O'Leary PW, Daly RC, Cetta F, Johnson JN. Heart transplantation after Fontan: Results from a surgical Fontan cohort. Pediatr Transplant 2016; 20:1087-1092. [PMID: 27397767 DOI: 10.1111/petr.12753] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/07/2016] [Indexed: 01/12/2023]
Abstract
We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart-lung, one heart-liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post-bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non-transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post-transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate-term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.
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Affiliation(s)
- Kavitha N Pundi
- Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
| | - Krishna Pundi
- Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN, USA
| | - David J Driscoll
- Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
| | - Joseph A Dearani
- Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA
| | - Zhuo Li
- Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA
| | - Sonja H Dahl
- Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
| | - Bassem N Mora
- Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA
| | - Patrick W O'Leary
- Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.,Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA
| | - Richard C Daly
- Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA
| | - Frank Cetta
- Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.,Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA
| | - Jonathan N Johnson
- Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.,Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA
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11
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Shah DK, Deo SV, Althouse AD, Teuteberg JJ, Park SJ, Kormos RL, Burkhart HM, Morell VO. Perioperative mortality is the Achilles heel for cardiac transplantation in adults with congenital heart disease: Evidence from analysis of the UNOS registry. J Card Surg 2016; 31:755-764. [DOI: 10.1111/jocs.12857] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Affiliation(s)
- Dipesh K. Shah
- Cardiothoracic Surgery; Heart and Vascular Institute, UPMC; Pittsburgh Pennsylvania
| | - Salil V. Deo
- Cardiothoracic Surgery; University Hospitals; Cleveland Ohio
| | - Andrew D. Althouse
- Biostatistician, Heart and Vascular Institute; UPMC; Pittsburgh Pennsylvania
| | - Jeffery J. Teuteberg
- Cardiovascular Diseases; Heart and Vascular Institute, UPMC; Pittsburgh Pennsylvania
| | - Soon J. Park
- Cardiothoracic Surgery; University Hospitals; Cleveland Ohio
| | - Robert L. Kormos
- Cardiothoracic Surgery; Heart and Vascular Institute, UPMC; Pittsburgh Pennsylvania
| | - Harold M. Burkhart
- Pediatric Cardiothoracic Surgery; University of Oklahoma Health Sciences Center; Oklahoma City Oklahoma
| | - Victor O. Morell
- Pediatric Cardiothoracic Surgery; Children's Hospital of Pittsburgh; Pittsburgh Pennsylvania
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12
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Krishnamurthy Y, Cooper LB, Lu D, Schroder JN, Daneshmand MA, Rogers JG, Milano CA, Hernandez AF, Patel CB. Trends and outcomes of patients with adult congenital heart disease and pulmonary hypertension listed for orthotopic heart transplantation in the United States. J Heart Lung Transplant 2016; 35:619-24. [PMID: 26856668 PMCID: PMC9793424 DOI: 10.1016/j.healun.2015.12.017] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2015] [Revised: 11/16/2015] [Accepted: 12/21/2015] [Indexed: 12/30/2022] Open
Abstract
BACKGROUND Heart transplantation is increasing in patients with adult congenital heart disease (ACHD). In this population, the association of pulmonary hypertension (PH) with post-transplant outcomes is not well-defined. METHODS Using data from the United Network for Organ Sharing database (1987 to 2014), we identified ACHD patients listed for heart transplantation, and examined survival between those with and without PH (pre-transplant PH defined as transpulmonary pressure gradient >12 mm Hg). RESULTS Among 983 ACHD patients, 216 (22%) had PH. At time of listing, PH patients had a transpulmonary pressure gradient of 17.0 mm Hg vs 6.0 mm Hg (p < 0.01) in the no-PH group. Although left ventricular assist device (LVAD) use was infrequent, 3.1% of PH patients were treated with an LVAD versus 6.8% of the no-PH patients. Days from listing to transplant, days from listing to death on the waitlist and length of post-transplant hospitalization were not significantly different between the PH and no-PH groups. However, PH was associated with higher waitlist mortality (HR 1.73, CI 1.25 to 2.41). Pre-transplant PH was not associated with post-transplant mortality at 30 days (HR 0.51, CI 0.23 to 1.13), 1 year (HR 0.68, 95% CI 0.40 to 1.18) or 5 years (HR 0.84, 95% CI 0.55 to 1.29). CONCLUSIONS PH is common among ACHD patients listed for transplant and is associated with increased waitlist mortality. Conversely, PH was not associated with worse survival after transplant. Bridge-to-transplant LVAD therapy was uncommon in this ACHD population.
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Affiliation(s)
- Yamini Krishnamurthy
- Duke University School of Medicine, Durham, North Carolina; Department of Medicine, Duke University Medical Center, Durham, North Carolina
| | - Lauren B Cooper
- Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina
| | - Di Lu
- Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina
| | - Jacob N Schroder
- Department of Surgery, Duke University Medical Center, Durham, North Carolina
| | - Mani A Daneshmand
- Department of Surgery, Duke University Medical Center, Durham, North Carolina
| | - Joseph G Rogers
- Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina
| | - Carmelo A Milano
- Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina; Department of Surgery, Duke University Medical Center, Durham, North Carolina
| | - Adrian F Hernandez
- Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina
| | - Chetan B Patel
- Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina.
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13
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Kanter KR. Heart Transplantation in Children after a Fontan Procedure: Better than People Think. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2016; 19:44-49. [PMID: 27060042 DOI: 10.1053/j.pcsu.2015.11.004] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2015] [Accepted: 11/10/2015] [Indexed: 06/05/2023]
Abstract
Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 273 primary pediatric heart transplants. Since 1988, 33 (12.1%) of 273 children <18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 240 (87.9%) non-Fontan primary transplants, the Fontan patients were older (8.8 ± 5.2 vs 6.6 ± 5.9 years; P = .023), but were similar in presensitization and pre-transplant clinical status. More Fontan patients had prior operations (100% vs 51.7%; P < .0001) and needed pulmonary artery reconstruction (100% vs 21.7%; P < .0001). Thirteen (39%) had protein-losing enteropathy. Donor ischemic times (213 ± 73 vs 177 ± 57 minutes; P = .0013) and cardiopulmonary bypass times (199 ± 86 vs 125 ± 53 minutes; P < .0001) were greater in the Fontan group, as were durations of ventilator support (4.4 ± 6.0 vs 2.5 ± 4.3 days; P = .035) and hospital stay (18.6 ± 16.1 vs 14.7 ± 13.1 days; P = NS). The Fontan group had one 30-day mortality. One-year actuarial survival (84.8% vs 86.9%, Fontan vs non-Fontan) and 5-year actuarial survival (70.8% vs 70.3%, Fontan vs non-Fontan) were similar, as was rejection incidence at 1 year (2.0 ± 2.0 vs 1.7 ± 1.9 episodes/patient; P = .3972). Five Fontan patients (18.5%) required retransplantation 4.9 ± 3.6 years post-transplant, compared with 22 non-Fontan patients (9.2%) retransplanted 5.2 ± 3.4 years post-transplant. Contrary to prior reports, we did not identify any early or mid-term disadvantage for children undergoing heart transplantation after a previous Fontan procedure, despite more complex transplant operations. We contend that carefully selected children with a failing Fontan circulation can do as well as other children with heart transplantation.
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Affiliation(s)
- Kirk R Kanter
- Division of Cardio-Thoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, GA.
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14
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Fontan Liver Disease: Review of an Emerging Epidemic and Management Options. CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE 2015; 17:51. [PMID: 26407544 DOI: 10.1007/s11936-015-0412-z] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
OPINION STATEMENT Adults with complex congenital heart disease that resulted in a Fontan procedure frequently experience late cardiac failure. Increasingly, liver disease is recognized as an important complication of single-ventricle anatomy and Fontan physiology; however, there is no consensus regarding liver evaluation in this population. Here, we review what is known about liver disease in this unique group and propose screening and prevention measures. We also review controversial treatment areas including assist devices and transplantation, with a review of outcomes in isolated heart and combined heart-liver transplant.
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15
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Bhatt AB, Foster E, Kuehl K, Alpert J, Brabeck S, Crumb S, Davidson WR, Earing MG, Ghoshhajra BB, Karamlou T, Mital S, Ting J, Tseng ZH. Congenital Heart Disease in the Older Adult. Circulation 2015; 131:1884-931. [DOI: 10.1161/cir.0000000000000204] [Citation(s) in RCA: 164] [Impact Index Per Article: 16.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
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16
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Mauchley DC, Mitchell MB. Transplantation in the Fontan patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2015; 18:7-16. [PMID: 25939837 DOI: 10.1053/j.pcsu.2015.01.001] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2014] [Revised: 12/30/2014] [Accepted: 01/08/2015] [Indexed: 06/04/2023]
Abstract
The failing Fontan circulation presents difficult treatment challenges. When Fontan revision and or intervention for treatable arrhythmias is not feasible, heart transplantation is the only therapeutic option. Particular challenges presented by these patients include limited ability to assess hemodynamics, complex anatomy, multiple prior procedures, and unique underlying pathologic states. These issues complicate the decision-making process for further surgical intervention verses transplantation. The pre-transplant evaluation, transplant operation, and post-operative management are more problematic for these patients compared with most patients undergoing transplantation. Consequently, failing Fontan patients constitute one of the highest risk heart transplant subsets.
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Affiliation(s)
- David C Mauchley
- Instructor of Surgery, Department of Surgery, Division of Cardiothoracic Surgery, University of Colorado at Denver Health Sciences Center, Aurora, CO
| | - Max B Mitchell
- Professor of Surgery, Department of Surgery, Division of Cardiothoracic Surgery, University of Colorado at Denver Health Sciences Center and Children's Hospital Colorado Heart Institute, Aurora, CO.
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17
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Heart Failure in Adults who had the Fontan Procedure: Natural History, Evaluation, and Management. CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE 2013; 15:587-601. [DOI: 10.1007/s11936-013-0257-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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18
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Bhama JK, Shulman J, Bermudez CA, Bansal A, Ramani R, Teuteberg JJ, Shullo M, McNamara DM, Kormos RL, Toyoda Y. Heart transplantation for adults with congenital heart disease: Results in the modern era. J Heart Lung Transplant 2013; 32:499-504. [DOI: 10.1016/j.healun.2013.01.1047] [Citation(s) in RCA: 62] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2012] [Revised: 01/07/2013] [Accepted: 01/25/2013] [Indexed: 11/28/2022] Open
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Greutmann M, Tobler D. Changing epidemiology and mortality in adult congenital heart disease: looking into the future. Future Cardiol 2012; 8:171-7. [PMID: 22413977 DOI: 10.2217/fca.12.6] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023] Open
Abstract
Advances in surgical and medical treatment of children born with congenital heart disease have led to a growing number of adult survivors, particularly to a growing number of adults with complex congenital heart disease. Childhood mortality has continuously decreased over the last few decades and mortality has shifted almost entirely to adulthood. However, most patients are not cured and many remain at risk of premature death. The extent of excess mortality among individual congenital disease entities is not well defined. In this article we outline the current demographics of adults with selected congenital heart lesions. Based on these contemporary patient cohorts, we delineate future changes in patient demographics. A better understanding of these trends may help in the optimal planning of future resource allocation for medical care and optimal planning of multicenter research, for this novel and growing population of young, chronically ill adults.
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Affiliation(s)
- Matthias Greutmann
- Department of Cardiology, University Hospital Basel, Petersgraben 4, CH-4031 Basel, Switzerland.
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20
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Transplantation for complex congenital heart disease in adults: a subanalysis of the Spanish Heart Transplant Registry. Clin Transplant 2012; 26:755-63. [DOI: 10.1111/j.1399-0012.2012.01611.x] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/05/2012] [Indexed: 11/26/2022]
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21
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Olowu WA. Epidemiology, pathophysiology, clinical characteristics and management of childhood cardiorenal syndrome. World J Nephrol 2012; 1:16-24. [PMID: 24175238 PMCID: PMC3782210 DOI: 10.5527/wjn.v1.i1.16] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/09/2011] [Revised: 10/16/2011] [Accepted: 12/27/2011] [Indexed: 02/06/2023] Open
Abstract
Cardiorenal syndrome (CRS) is a new term recently introduced to describe the acute or chronic comorbid state of the heart and kidney that has been long known and frequently managed in very sick individuals. The tight and delicate coordination of physiological functions among organ systems in the human body makes dysfunction in one to lead to malfunction of one or more other organ systems. CRS is a universal very common morbidity in the critically ill, with a high mortality rate that has received very little research attention in children. Simultaneous management of heart and renal failures in CRS is quite challenging; the therapeutic choice made for one organ must not jeopardize the other. This paper reviews the epidemiology, pathophysiology, clinical characteristics and management of acute and chronic CRS in children.
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Affiliation(s)
- Wasiu A Olowu
- Wasiu A Olowu, Paediatric Nephrology and Hypertension Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State 234, Nigeria
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22
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Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment. Cardiol Young 2011; 21:363-77. [PMID: 21349233 DOI: 10.1017/s1047951111000102] [Citation(s) in RCA: 50] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.
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23
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Davies RR, Russo MJ, Yang J, Quaegebeur JM, Mosca RS, Chen JM. Listing and Transplanting Adults With Congenital Heart Disease. Circulation 2011; 123:759-67. [DOI: 10.1161/circulationaha.110.960260] [Citation(s) in RCA: 146] [Impact Index Per Article: 10.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Background—
An increasing number of patients with congenital heart disease (CHD) are reaching adulthood and may require heart transplantation. The survival of these patients after listing and transplantation has not been evaluated.
Methods and Results—
A total of 41 849 patients (aged >18 years) were listed for primary transplantation during 1995–2009. Patients with a history of CHD (n=1035; 2.5%) were compared with those with other causes (non-CHD group) (n=40 814; 97.5%); 26 055 (62.3%) reached transplantation and were subdivided into those with (reoperation group; n=10 484; 40.2%) and without (nonreoperation group; n=15 571; 59.8%) a previous sternotomy. Survival on the waiting list was similar between groups, but mechanical ventricular assistance was not associated with superior survival to transplantation among CHD patients. CHD patients were more likely to have body mass index <18.5 at transplantation (
P
<0.0001), were younger, and had fewer comorbidities. Early mortality among patients with CHD was high (reoperation, 18.9% versus 9.6%;
P
<0.0001; nonreoperation, 16.6% versus 6.3%;
P
<0.0001), but by 10 years, overall survival was equivalent (53.8% versus 53.6%). Analysis was limited by the lack of specific information regarding the CHD diagnosis in most patients.
Conclusions—
Adults with CHD have high 30-day mortality but better late survival after heart transplantation. Mechanical circulatory assistance does not improve waiting list survival in these patients. This may be due to a combination of highly complex reoperative surgery and often poor preoperative systemic health.
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Affiliation(s)
- Ryan R. Davies
- From the Departments of Surgery, Columbia University Medical Center (M.J.R., J.Y., J.M.Q.), New York University Langone Medical Center (R.S.M.), and Weill Medical College of Cornell University (J.M.C.), New York, NY; and Department of Cardiothoracic Surgery, Lucille-Packard Children's Hospital/Stanford University, Palo Alto, CA (R.R.D.)
| | - Mark J. Russo
- From the Departments of Surgery, Columbia University Medical Center (M.J.R., J.Y., J.M.Q.), New York University Langone Medical Center (R.S.M.), and Weill Medical College of Cornell University (J.M.C.), New York, NY; and Department of Cardiothoracic Surgery, Lucille-Packard Children's Hospital/Stanford University, Palo Alto, CA (R.R.D.)
| | - Jonathan Yang
- From the Departments of Surgery, Columbia University Medical Center (M.J.R., J.Y., J.M.Q.), New York University Langone Medical Center (R.S.M.), and Weill Medical College of Cornell University (J.M.C.), New York, NY; and Department of Cardiothoracic Surgery, Lucille-Packard Children's Hospital/Stanford University, Palo Alto, CA (R.R.D.)
| | - Jan M. Quaegebeur
- From the Departments of Surgery, Columbia University Medical Center (M.J.R., J.Y., J.M.Q.), New York University Langone Medical Center (R.S.M.), and Weill Medical College of Cornell University (J.M.C.), New York, NY; and Department of Cardiothoracic Surgery, Lucille-Packard Children's Hospital/Stanford University, Palo Alto, CA (R.R.D.)
| | - Ralph S. Mosca
- From the Departments of Surgery, Columbia University Medical Center (M.J.R., J.Y., J.M.Q.), New York University Langone Medical Center (R.S.M.), and Weill Medical College of Cornell University (J.M.C.), New York, NY; and Department of Cardiothoracic Surgery, Lucille-Packard Children's Hospital/Stanford University, Palo Alto, CA (R.R.D.)
| | - Jonathan M. Chen
- From the Departments of Surgery, Columbia University Medical Center (M.J.R., J.Y., J.M.Q.), New York University Langone Medical Center (R.S.M.), and Weill Medical College of Cornell University (J.M.C.), New York, NY; and Department of Cardiothoracic Surgery, Lucille-Packard Children's Hospital/Stanford University, Palo Alto, CA (R.R.D.)
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24
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Francis GS, Greenberg BH, Hsu DT, Jaski BE, Jessup M, LeWinter MM, Pagani FD, Piña IL, Semigran MJ, Walsh MN, Wiener DH, Yancy CW. ACCF/AHA/ACP/HFSA/ISHLT 2010 clinical competence statement on management of patients with advanced heart failure and cardiac transplant: a report of the ACCF/AHA/ACP Task Force on Clinical Competence and Training. Circulation 2010; 122:644-72. [PMID: 20644017 DOI: 10.1161/cir.0b013e3181ecbd97] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Affiliation(s)
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- American College of Cardiology Foundation, USA
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25
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Francis GS, Greenberg BH, Hsu DT, Jaski BE, Jessup M, LeWinter MM, Pagani FD, Piña IL, Semigran MJ, Walsh MN, Wiener DH, Yancy CW. ACCF/AHA/ACP/HFSA/ISHLT 2010 Clinical Competence Statement on Management of Patients With Advanced Heart Failure and Cardiac Transplant. J Am Coll Cardiol 2010; 56:424-53. [DOI: 10.1016/j.jacc.2010.04.014] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
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26
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Silversides CK, Salehian O, Oechslin E, Schwerzmann M, Vonder Muhll I, Khairy P, Horlick E, Landzberg M, Meijboom F, Warnes C, Therrien J. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: complex congenital cardiac lesions. Can J Cardiol 2010; 26:e98-117. [PMID: 20352139 DOI: 10.1016/s0828-282x(10)70356-1] [Citation(s) in RCA: 66] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part III of the guidelines includes recommendations for the care of patients with complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Fontan operations and single ventricles, Eisenmenger's syndrome, and cyanotic heart disease. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.
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27
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Affiliation(s)
- Daphne T Hsu
- Department of Pediatrics, Children's Hospital at Montefiore, Albert Einstein College of Medicine, New York, NY, USA
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28
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Patel ND, Weiss ES, Allen JG, Russell SD, Shah AS, Vricella LA, Conte JV. Heart Transplantation for Adults With Congenital Heart Disease: Analysis of the United Network for Organ Sharing Database. Ann Thorac Surg 2009; 88:814-21; discussion 821-2. [DOI: 10.1016/j.athoracsur.2009.04.071] [Citation(s) in RCA: 101] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2008] [Revised: 04/15/2009] [Accepted: 04/16/2009] [Indexed: 10/20/2022]
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29
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Lamour JM, Kanter KR, Naftel DC, Chrisant MR, Morrow WR, Clemson BS, Kirklin JK. The Effect of Age, Diagnosis, and Previous Surgery in Children and Adults Undergoing Heart Transplantation for Congenital Heart Disease. J Am Coll Cardiol 2009; 54:160-5. [PMID: 19573734 DOI: 10.1016/j.jacc.2009.04.020] [Citation(s) in RCA: 199] [Impact Index Per Article: 12.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/05/2007] [Revised: 03/25/2009] [Accepted: 04/02/2009] [Indexed: 10/20/2022]
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30
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Pediatric Cardiac Care Consortium: an instrument for evidence-based clinical decision support. J Cardiovasc Transl Res 2009; 2:219-24. [PMID: 20559990 DOI: 10.1007/s12265-009-9091-z] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/15/2009] [Accepted: 02/10/2009] [Indexed: 10/21/2022]
Abstract
Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease.
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31
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Orthotopic heart transplantation in a patient with situs invs, transposition of the great arteries and Mustard operation. Eur J Cardiothorac Surg 2008; 34:219-21. [PMID: 18485722 DOI: 10.1016/j.ejcts.2008.04.014] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/19/2008] [Revised: 04/15/2008] [Accepted: 04/16/2008] [Indexed: 11/16/2022] Open
Abstract
Orthotopic heart transplantation has become standard treatment for end-stage cardiomyopathy, but experience with this technique for complex congenital heart diseases is limited. We report a patient with visceroatrial situs invs, transposition of the great arteries and previous Mustard correction, who successfully underwent orthotopic heart transplantation.
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32
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Izquierdo MT, Almenar L, Martínez-Dolz L, Moro J, Agüero J, Sanchez-Lázaro I, Cano O, Ortiz V, Sánchez R, Salvador A. Mortality After Heart Transplantation in Adults With Congenital Heart Disease: A Single-Center Experience. Transplant Proc 2007; 39:2357-9. [PMID: 17889188 DOI: 10.1016/j.transproceed.2007.06.045] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
UNLABELLED The number of congenital heart disease (CHD) patients transplanted to date is small. The results are comparable to those undergoing heart transplantation (HT) for other etiologies. However, advances in pediatric surgery over recent years (eg, the Fontan procedure) has increased the demand for HT by a growing number of children who reach adulthood and who also have a different profile. We analyzed the clinical profile and survival of our CHD patients compared with other etiologies. MATERIALS AND METHODS From July 17, 1991 to December 31, 2006, eight HT were performed in our center for CHD. A descriptive study determined the baseline characteristics and survival of these patients, compared with those of the overall transplant group and other subgroups (dilated cardiomyopathy, ischemic heart disease). RESULTS Mean age was 26 years. Four (50%) CHD patients were diagnosed with single-ventricle anatomy, associated or not with other lesions; none had been operated with the Fontan procedure. Two patients died prematurely. Early, 1-, and 10-year survival was 75% at each time point. Early, 1-, and 10-year survival in the group with other diagnoses was 90%, 78%, and 60%, respectively, and in the dilated cardiomyopathy group it was 94%, 86%, and 72%, respectively. CONCLUSION The current number of CHD transplant patients was small and young. The most common etiology was single-ventricle anatomy without a prior Fontan operation. Overall survival was comparable to HT for dilated cardiomyopathy.
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Affiliation(s)
- M T Izquierdo
- Heart Failure and Transplant Unit, Department of Cardiology, La Fe University Hospital, Valencia, Spain.
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Abstract
The number of grown-up patients with congenital heart disease (GUCH) is constantly increasing and will equal the number of children requiring surgery for congenital heart disease (CHD). Specialized centers dealing with the medical and paramedical problems of these patients are required. GUCH patients can be divided into the following groups: (1) patients with minor cardiac malformations presenting at adult age for first treatment; (2) patients presenting for correction as adults because they are either naturally balanced or were surgically palliated; (3) patients presenting for expected reoperations after correction in childhood; (4) patients requiring repair of residual defects after correction; (5) patients developing heart failure after correction or palliation of CHD requiring thoracic transplantation; and (6) patients developing acquired heart disease in addition to CHD. Special aspects of malformations frequently occurring in GUCH patients are discussed in detail. Acquired heart disease in this patient population is expected to increase in the coming decades as survivors of CHD grow old.
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Affiliation(s)
- Sabine H Daebritz
- Department of Cardiac Surgery, University Hospital Grosshadern, Marchioninistrasse 15, 81377 Munich, Germany.
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34
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Coskun O, Coskun T, El-Arousy M, Parsa MA, Reiss N, Blanz U, Von Knyphausen E, Sandica E, Schulz U, Knobl H, Tenderich G, Bairaktaris A, Kececioglu D, Körfer R. Heart Transplantation in Adults With Congenital Heart Disease. ASAIO J 2007; 53:103-6. [PMID: 17237656 DOI: 10.1097/01.mat.0000250956.08459.8e] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
End-stage congenital heart disease (CHD) is an important indication for pediatric heart transplantation (HTx) as well as transplantation in adult populations. The purpose of this retrospective analysis was to compare the survival rate of adults who underwent HTx for end-stage CHD with those who underwent HTx for other causes. To find out whether HTx is a viable therapeutic option for adult patients with preoperated CHD, data from 15 adult patients with different forms of CHD, who had previously undergone different corrective and palliative procedures, were retrospectively analyzed and compared with the HTx data of 1400 adult patients (>15 years old) whose indications for HTx were other than end-stage CHD. From 1989 to 2005, 15 adult patients (eight men/seven women) were given transplantation for end-stage CHD. Ten patients had been preoperated once, five patients twice. Their mean age was 34.06 +/- 3.9 years. In five cases, patients had development of acute renal failure. One female patient died 40 days after surgery, after having a cerebral infarction; one male patient died 4 years after HTx for OKT 3 monoclonal antibody-resistant rejection; and two patients died as the result of multiple organ failure at 4 days and 30 days after HTx, respectively; 11 patients are still alive. The cumulative survival rate at 1 year is 80% versus 80% in patients given transplantation for noncongenital indications. Heart transplantation in adults with end-stage CHD can be performed with a good long-term prognosis. Previous palliative operations do not affect outcome after HTx.
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Affiliation(s)
- Oguz Coskun
- Department of Thoracic and Cardiovascular Surgery, Heart and Diabetes Center North Rhine-Westphalia, Ruhr University of Bochum, Bad Oeynhausen, Germany
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35
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Weinstein S, Chan D. Extracardiac Fontan Conversion, Cryoablation, and Pacemaker Placement for Patients with a Failed Fontan. Semin Thorac Cardiovasc Surg 2005; 17:170-8. [PMID: 16087088 DOI: 10.1053/j.semtcvs.2005.02.007] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
OBJECTIVE We review our experience with Fontan conversion and cryoablation in patients with an atriopulmonary Fontan in low cardiac output from arrhythmia or venous obstruction, including two patients with protein losing enteropathy. METHODS Fifteen patients (mean age 25.0 +/- 8.4 years) underwent extracardiac Fontan conversion, cryoablation, and pacemaker placement between November 1999 and December 2004. Twelve patients were in NYHA class III and three were in NYHA class IV. Twelve had clinically important intraatrial reentry tachycardia refractory to medical therapy. RESULTS Follow-up was between 2 and 62 months (mean 38.4 +/- 17.7). One death occurred at seven days after surgery due to sepsis and multisystem organ failure. The second death occurred at five days from myocardial depression following surgery. One patient with PLE preoperatively died to malnutrition and sepsis on POD number 52. The second patient with protein losing enteropathy had improved NYHA classification, cessation of albumin transfusions, and a normal stool alpha antitrypsin level (down from 4.1 mg/g preoperatively). All surviving patients improved NYHA classification to class I or II. Sustained arrhythmias could not be induced in any patient. One patient had recurrence of intraatrial reentrant tachycardia eleven months postoperatively that required electrical cardioversion and is currently well controlled on one medication. The other patients are not on any antiarrhythmic medical therapy. CONCLUSION Extracardiac Fontan, cryoablation, and pacemaker placement reduced atrial arrhythmias and improved NYHA classification. In selected patients, this operation offers improvement in clinical outcome and is an alternative to transplantation. Protein losing enteropathy may not be a contraindication to performing Fontan conversion with cryoablation.
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Affiliation(s)
- Samuel Weinstein
- Division of Pediatric Cardiothoracic Surgery, Montefiore Medical Center, Bronx, New York 10467, USA.
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36
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Mitchell MB, Campbell DN, Boucek MM. Heart transplantation for the failing Fontan circulation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 7:56-64. [PMID: 15283353 DOI: 10.1053/j.pcsu.2004.02.013] [Citation(s) in RCA: 38] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/30/2023]
Abstract
The failing Fontan circulation presents difficult treatment challenges. When Fontan revision and or intervention for treatable arrhythmias are not feasible, heart transplantation is the only therapeutic option. Particular challenges presented by these patients include limited ability to assess hemodynamics, complex anatomy, multiple prior procedures, and unique underlying pathologic states. These issues complicate the decision-making process for further surgical intervention verses transplantation. The pretransplant evaluation, transplant operation, and postoperative management are more problematic for these patients compared with most patients undergoing transplantation. Consequently, failing Fontan patients constitute one of the highest risk heart transplant subsets.
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Affiliation(s)
- Max B Mitchell
- Department of Surgery, Division of Cardiothoracic Surgery, University of Colorado Health Sciences Center and the Children's Hospital, Denver, CO, USA
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37
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Khan AN, Boatman J, Anderson AS. Management of new-onset congestive heart failure in a patient with complex congenital heart disease. CONGESTIVE HEART FAILURE (GREENWICH, CONN.) 2002; 8:54-6. [PMID: 11821629 DOI: 10.1111/j.1527-5299.2002.00814.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
The authors describe a 37-year-old African American female patient with congenital tricuspid stenosis, a secundum atrial septal defect, a ventricular septal defect, and subpulmonic stenosis whose only surgical intervention was a Glenn shunt procedure at the age of 11. She had been in reasonably good health for 26 years, but developed congestive heart failure. Her deteriorating clinical course was difficult to manage with only medical treatment, and cardiopulmonary transplantation is now being considered for the management of her complicated clinical condition.
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Affiliation(s)
- A Nasser Khan
- Department of Medical Education, Weiss Memorial Hospital/University of Chicago Hospitals, Chicago, IL 60640, USA.
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38
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Pigula FA, Gandhi SK, Ristich J, Stukus D, McCurry K, Webber SA, Keenan R, Griffith BP, Kormos R. Cardiopulmonary transplantation for congenital heart disease in the adult. J Heart Lung Transplant 2001; 20:297-303. [PMID: 11257555 DOI: 10.1016/s1053-2498(00)00229-1] [Citation(s) in RCA: 38] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
BACKGROUND Patients surviving into adulthood with congenital heart disease (CHD) often succumb to progressive cardiopulmonary dysfunction. For these patients transplantation is often considered. METHODS We performed a retrospective review of 69 adults (age >18 years) with CHD transplanted between 1984 and 1999. RESULTS We evaluated 31 heart-lung (HLTxp), 30 lung (LTxp), and 8 heart (HTxp) transplants performed in 22 men and 47 women with CHD. Mean age was 37 +/- 10 years with a mean follow-up of 3.1 +/- 3.5 years. A concomitant cardiovascular procedure was performed in 1 HLTxp, 23 LTxp, and 2 HTxp. Early mortality (>30 days) was 26% (8/31) for HLTxp, mostly due to bleeding. Early LTxp mortality was 23% (7/30), largely due to graft failure. One and 3-year survival was similar in adults transplanted for CHD and adults transplanted for other disease. Early mortality among HTxp recipients was 50% (4/8) from rejection or technical complications. Survival for patients undergoing HLTxp versus LTxp with cardiac repair was similar. When examined by era, the survival of patients transplanted for CHD between 1992 and 1999 was greater than that of patients transplanted between 1984 and 1991. CONCLUSIONS Adults undergoing HLTxp and LTxp for CHD can expect survival comparable to that of non-CHD adults. In the presence of a reparable cardiac lesion, LTxp with cardiovascular repair for CHD is an attractive option, optimizing organ allocation. Specific technical concerns are discussed. Survival of adults undergoing cardiopulmonary transplantation for CHD has improved over time.
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Affiliation(s)
- F A Pigula
- Department of Cardiothoracic Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
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