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Ahmad R, Frishman WH, Aronow WS. Navigating Pregnancy in Congenital Heart Disease: A Comprehensive Review of Maternal Outcomes. Cardiol Rev 2025:00045415-990000000-00431. [PMID: 39998159 DOI: 10.1097/crd.0000000000000884] [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] [Indexed: 02/26/2025]
Abstract
Congenital heart disease (CHD) affects approximately 0.5-1% of the population, with advancements in cardiovascular care enabling 97% of these individuals to survive to adulthood. Pregnancy in women with CHD presents with unique challenges due to increased hemodynamic demands and associated risks. This review provides a comprehensive analysis of maternal outcomes in women with CHD, focusing on the physiological changes during pregnancy, classification of CHD types, and their specific consequences. The review highlights significant complications within this population, such as arrhythmias, heart failure, thromboembolic events, and aortic dissection, emphasizing the need for multidisciplinary management and individualized care. Despite considerable advancements, gaps in research persist, particularly in neonatal risk prediction and long-term maternal outcomes. Future directions prioritize the refinement of risk stratification models and leveraging emerging technologies to enhance care for this complex population.
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Affiliation(s)
- Rimsha Ahmad
- From the Department of Medicine, Westchester Medical Center and New York Medical College, Valhalla, NY
| | - William H Frishman
- From the Department of Medicine, Westchester Medical Center and New York Medical College, Valhalla, NY
| | - Wilbert S Aronow
- Departments of Cardiology and Medicine, Westchester Medical Center, and New York Medical College, Valhalla, NY
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2
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Maternal cardiovascular morbidity and mortality associated with pregnancy in individuals with Turner syndrome: a committee opinion. Fertil Steril 2024; 122:612-621. [PMID: 38980250 DOI: 10.1016/j.fertnstert.2024.06.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2024] [Accepted: 06/04/2024] [Indexed: 07/10/2024]
Abstract
In individuals with Turner syndrome, the risk of death from aortic dissection or rupture during pregnancy may be as high as 1%, and it is unclear whether this risk persists during the postpartum period owing to pregnancy-related aortic changes. Turner syndrome is a relative contraindication for pregnancy; however, it is an absolute contraindication for pregnancy in a patient with an aortic size index of >2.5 cm/m2 or an aortic size index of ≥2.0 cm/m2 with a documented cardiac anomaly or other risk factors. This document replaces the 2012 document of the same name.
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3
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Meng X, Song M, Zhang K, Lu W, Li Y, Zhang C, Zhang Y. Congenital heart disease: types, pathophysiology, diagnosis, and treatment options. MedComm (Beijing) 2024; 5:e631. [PMID: 38974713 PMCID: PMC11224996 DOI: 10.1002/mco2.631] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2023] [Revised: 05/23/2024] [Accepted: 05/27/2024] [Indexed: 07/09/2024] Open
Abstract
Congenital heart disease (CHD) is a structural abnormality of the heart and/or great vessels and patients with CHD are at an increased risks of various morbidities throughout their lives and reduced long-term survival. Eventually, CHD may result in various complications including heart failure, arrhythmias, stroke, pneumonia, and sudden death. Unfortunately, the exact etiology and pathophysiology of some CHD remain unclear. Although the quality of life and prognosis of patients with CHD have significantly improved following technological advancement, the influence of CHD is lifelong, especially in patients with complicated CHD. Thus, the management of CHD remains a challenge due to its high prevalence. Finally, there are some disagreements on CHD among international guidelines. In this review, we provide an update of the pathophysiology, diagnosis, and treatment in most common type of CHD, including patent foramen ovale, atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, congenitally corrected transposition of the great arteries, coronary anomalies, left and right ventricular outflow tract obstruction, tetralogy of Fallot and Ebstein anomaly. In particular, we focus on what is known and what is unknown in these areas, aiming to improve the current understanding of various types of CHD.
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Affiliation(s)
- Xiao Meng
- Department of CardiologyState Key Laboratory for Innovation and Transformation of Luobing TheoryQilu Hospital of Shandong UniversityJinanChina
- Key Laboratory of Cardiovascular Remodeling and Function ResearchChinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, Qilu Hospital of Shandong UniversityJinanChina
| | - Ming Song
- Department of CardiologyState Key Laboratory for Innovation and Transformation of Luobing TheoryQilu Hospital of Shandong UniversityJinanChina
- Key Laboratory of Cardiovascular Remodeling and Function ResearchChinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, Qilu Hospital of Shandong UniversityJinanChina
| | - Kai Zhang
- Department of CardiologyState Key Laboratory for Innovation and Transformation of Luobing TheoryQilu Hospital of Shandong UniversityJinanChina
- Key Laboratory of Cardiovascular Remodeling and Function ResearchChinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, Qilu Hospital of Shandong UniversityJinanChina
| | - Weida Lu
- Shandong Key Laboratory of Cardiovascular Proteomics and Department of Geriatric MedicineQilu Hospital of Shandong UniversityJinanChina
| | - Yunyi Li
- Department of CardiologyState Key Laboratory for Innovation and Transformation of Luobing TheoryQilu Hospital of Shandong UniversityJinanChina
- Key Laboratory of Cardiovascular Remodeling and Function ResearchChinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, Qilu Hospital of Shandong UniversityJinanChina
| | - Cheng Zhang
- Department of CardiologyState Key Laboratory for Innovation and Transformation of Luobing TheoryQilu Hospital of Shandong UniversityJinanChina
- Key Laboratory of Cardiovascular Remodeling and Function ResearchChinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, Qilu Hospital of Shandong UniversityJinanChina
| | - Yun Zhang
- Department of CardiologyState Key Laboratory for Innovation and Transformation of Luobing TheoryQilu Hospital of Shandong UniversityJinanChina
- Key Laboratory of Cardiovascular Remodeling and Function ResearchChinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, Qilu Hospital of Shandong UniversityJinanChina
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Sorice D, Barracano R, Sarubbi B. Against all odds: successful pregnancy in an adult patient with unrepaired coarctation of the aorta. Cardiol Young 2024; 34:1390-1392. [PMID: 38738395 DOI: 10.1017/s1047951124025113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 05/14/2024]
Abstract
According to the modified World Health Organization classification, pregnant women with unrepaired aortic coarctation are at very high risk for both maternal and fetal complications and should, therefore, be counselled against pregnancy. The most frequent maternal complications include systemic hypertension, renal failure, preeclampsia, and aortic dissection. Herein, we describe a successful pregnancy in an adult patient with unrepaired aortic coarctation.
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Affiliation(s)
- Davide Sorice
- University of Campania Luigi Vanvitelli, Naples, Italy
- Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital, Naples, Italy
| | - Rosaria Barracano
- Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital, Naples, Italy
| | - Berardo Sarubbi
- Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital, Naples, Italy
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5
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Salciccioli KB, Zachariah JP. Coarctation of the Aorta: Modern Paradigms Across the Lifespan. Hypertension 2023; 80:1970-1979. [PMID: 37476999 PMCID: PMC10530495 DOI: 10.1161/hypertensionaha.123.19454] [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] [Indexed: 07/22/2023]
Abstract
While coarctation of the aorta varies greatly in both severity and age at presentation, all patients are at increased risk of hypertension both before and after repair. Despite advances in knowledge about genetic etiologies, pathophysiologic mechanisms, and optimal repair strategies, patients with repaired coarctation of the aorta remain at increased risk of acquired cardiovascular disease. The aims of this review are to describe the management of coarctation of the aorta at all ages before and after repair, highlight pathophysiologic mechanisms of hypertension, and review long-term follow-up considerations.
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Affiliation(s)
- Katherine B Salciccioli
- Section of Pediatric Cardiology, Departments of Internal Medicine and Pediatrics, Baylor College of Medicine, Texas Children’s Hospital, Houston TX USA
| | - Justin P Zachariah
- Section of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital, Houston TX USA
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Mahabir S, Okumus N, Samra G, Mohammed A, Gupta M, Naser A, Puttur A, Bihler E, DuMont T, Nauer K, Khalif A, Ivanova V. Common Cardiovascular Diseases in Women. Crit Care Nurs Q 2023; 46:362-376. [PMID: 37684732 DOI: 10.1097/cnq.0000000000000473] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/10/2023]
Abstract
Cardiovascular disease is a leading cause of death for women in the United States. This article encompasses the epidemiology/etiology, clinical presentation, diagnostic assessment, management, and prognosis of some common cardiovascular disorders seen in women with a special focus on pregnancy.
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Affiliation(s)
- Sunita Mahabir
- Division of Cardiology, Allegheny General Hospital, Pittsburgh, Pennsylvania (Ms Mahabir and Drs Okumus, Samra, Mohammed, and Gupta); Division of Medicine (Drs Naser and Puttur), Division of Pulmonary and Critical Care Medicine (Drs Bihler and DuMont and Mr Nauer), Division of Cardiovascular Disease and Critical Care (Dr Khalif), and Division of Cardiovascular Medicine (Dr Ivanova), Allegheny Health Network Medicine Institute, Pittsburgh, Pennsylvania; and Drexel University College of Medicine, Philadelphia, Pennsylvania (Dr Ivanova)
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7
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Gronningsaeter L, Langesaeter E, Sørbye IK, Quattrone A, Almaas VM, Skulstad H, Estensen ME. High prevalence of pre-eclampsia in women with coarctation of the aorta. EUROPEAN HEART JOURNAL OPEN 2023; 3:oead072. [PMID: 37559925 PMCID: PMC10407978 DOI: 10.1093/ehjopen/oead072] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/05/2023] [Revised: 07/04/2023] [Accepted: 07/17/2023] [Indexed: 08/11/2023]
Abstract
Aims The aim was to study pregnancy outcomes in women with coarctation of the aorta (CoA) and associations to hypertensive disorders of pregnancy. Maternal morbidity and mortality are higher in women with heart disease and pre-eclampsia. Chronic hypertension, frequently encountered in CoA, is a risk factor for pre-eclampsia. Methods and results Clinical data from the National Unit for Pregnancy and Heart Disease database was reviewed for pregnant women with CoA from 2008 to 2021. The primary outcome was hypertensive pregnancy disorders. The secondary outcomes were other cardiovascular, obstetric, and foetal complications. Seventy-six patients were included, with a total of 87 pregnancies. Seventeen (20%) patients were treated for chronic hypertension before pregnancy. Fifteen (20%) patients developed pre-eclampsia, and 5 (7%) had pregnancy-induced hypertension. Major adverse cardiac events developed in four (5%) patients, with no maternal or foetal mortality. Maternal age at first pregnancy [odds ratio (OR) 1.37], body mass index before first pregnancy (OR 1.77), and using acetylsalicylic acid from the first trimester (OR 0.22) were statistically significantly associated with pre-eclampsia. At follow-up (median) 8 years after pregnancy, 29 (38%) patients had anti-hypertensive treatment, an increase of 16% compared to pre-pregnancy. Five (7%) patients had progression of aorta ascendens dilatation to >40 mm, seven (9%) had an upper to lower systolic blood pressure gradient >20 mmHg, and six (8%) had received CoA re-intervention. Conclusion Pre-eclampsia occurred in 20% of women with CoA in their first pregnancy. All pre-eclamptic patients received adequate anti-hypertensive treatment. All CoA patients were provided multi-disciplinary management, including cardiologic follow-up, to optimize maternal-foetal outcomes.
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Affiliation(s)
- Lasse Gronningsaeter
- Department of Anesthesiology, Division of Emergencies and Critical Care Medicine, Oslo University Hospital, Rikshospitalet,Postboks 4950 Nydalen, Oslo N-0424, Norway
- Faculty of Medicine, Oslo University Hospital, Oslo, Norway
| | - Eldrid Langesaeter
- Department of Anesthesiology, Division of Emergencies and Critical Care Medicine, Oslo University Hospital, Rikshospitalet,Postboks 4950 Nydalen, Oslo N-0424, Norway
| | - Ingvil Krarup Sørbye
- Department of Obstetrics, Division of Obstetrics and Gynecology, Oslo University Hospital, Rikshospitalet, Oslo N-0424, Norway
| | - Alessia Quattrone
- Department of Cardiology, Division of Heart, Lung, and Vessel diseases, Oslo University Hospital, Rikshospitalet, Oslo N-0424, Norway
| | - Vibeke Marie Almaas
- Department of Cardiology, Division of Heart, Lung, and Vessel diseases, Oslo University Hospital, Rikshospitalet, Oslo N-0424, Norway
| | - Helge Skulstad
- Department of Cardiology, Division of Heart, Lung, and Vessel diseases, Oslo University Hospital, Rikshospitalet, Oslo N-0424, Norway
- Institute of Clinical Medicine, University of Oslo, Oslo, Norway
| | - Mette-Elise Estensen
- Department of Cardiology, Division of Heart, Lung, and Vessel diseases, Oslo University Hospital, Rikshospitalet, Oslo N-0424, Norway
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Raza S, Aggarwal S, Jenkins P, Kharabish A, Anwer S, Cullington D, Jones J, Dua J, Papaioannou V, Ashrafi R, Moharem-Elgamal S. Coarctation of the Aorta: Diagnosis and Management. Diagnostics (Basel) 2023; 13:2189. [PMID: 37443581 DOI: 10.3390/diagnostics13132189] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2023] [Revised: 06/12/2023] [Accepted: 06/14/2023] [Indexed: 07/15/2023] Open
Abstract
Coarctation of the aorta (CoA) accounts for approximately 5-8% of all congenital heart defects. Depending on the severity of the CoA and the presence of associated cardiac lesions, the clinical presentation and age vary. Developments in diagnosis and management have improved outcomes in this patient population. Even after timely repair, it is important to regularly screen for hypertension. Patients with CoA require lifelong follow-up with a congenital heart disease specialist as these patients may develop recoarctation and complications at the repair site and remain at enhanced cardiovascular risk throughout their lifetime.
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Affiliation(s)
- Sadaf Raza
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Suneil Aggarwal
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Petra Jenkins
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Ahmed Kharabish
- Radiology Department, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
- Radiology Department, Al Kasr Al Aini, Old Cairo, Cairo 11562, Egypt
| | - Shehab Anwer
- Cardiology Department, University of Zurich, 8006 Zurich, Switzerland
| | - Damien Cullington
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Julia Jones
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Jaspal Dua
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Vasileios Papaioannou
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Reza Ashrafi
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
| | - Sarah Moharem-Elgamal
- Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK
- Cardiology Department, National Heart Institute, Giza 11111, Egypt
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9
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Halpern DG, Penfield CA, Feinberg JL, Small AJ. Reproductive Health in Congenital Heart Disease: Preconception, Pregnancy, and Postpartum. J Cardiovasc Dev Dis 2023; 10:jcdd10050186. [PMID: 37233153 DOI: 10.3390/jcdd10050186] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2023] [Revised: 04/20/2023] [Accepted: 04/21/2023] [Indexed: 05/27/2023] Open
Abstract
The prevalence of congenital heart disease (CHD) in pregnancy is rising due to the improved survival of patients with CHD into childbearing age. The profound physiological changes that occur during pregnancy may worsen or unmask CHD, affecting both mother and fetus. Successful management of CHD during pregnancy requires knowledge of both the physiological changes of pregnancy and the potential complications of congenital heart lesions. Care of the CHD patient should be based on a multidisciplinary team approach beginning with preconception counseling and continuing into conception, pregnancy, and postpartum periods. This review summarizes the published data, available guidelines and recommendations for the care of CHD during pregnancy.
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Affiliation(s)
- Dan G Halpern
- NYU Adult Congenital Heart Disease Program, Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine and NYU Langone Health, New York, NY 10016, USA
| | - Christina A Penfield
- Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, NYU Grossman School of Medicine and NYU Langone Health, New York, NY 10016, USA
| | - Jodi L Feinberg
- NYU Adult Congenital Heart Disease Program, Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine and NYU Langone Health, New York, NY 10016, USA
| | - Adam J Small
- NYU Adult Congenital Heart Disease Program, Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine and NYU Langone Health, New York, NY 10016, USA
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10
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Cherpak BV, Davydova YV, Kravchenko VI, Yaschuk NS, Siromakha SO, Lazoryshynets VV. Management of percutaneous treatment of aorta coarctation diagnosed during pregnancy. J Med Life 2022; 15:208-213. [PMID: 35419094 PMCID: PMC8999110 DOI: 10.25122/jml-2021-0363] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2021] [Accepted: 12/15/2021] [Indexed: 11/19/2022] Open
Abstract
Management of coarctation of the aorta (CoA) during pregnancy is complicated by increased procedural risks to the pregnant woman and her fetus. The aim of this research was to analyze 10-years of experience of CoA treatment diagnosed during pregnancy. During 2010–2020 we performed percutaneous stents implantations (SI) in 4 women during 15–23 weeks of pregnancy and in 6 women 48 hours – 5 years after delivery. In all presented cases, successful CoA repair was achieved. There was a significant decrease of peak-to-peak invasive systolic pressure gradient across the CoA (60.0±31.2 and 11.8±7.3 mmHg, p=0.001) and mean noninvasive systolic arterial pressure (163.0±46.2 and 120.5±9.2 mmHg, p=0.01) after SI. All percutaneously treated women during pregnancy (n=4) delivered healthy full-term babies. At follow-up (from 2 months to 10 years), all 10 women are alive without significant Doppler gradient across CoA with no signs of aortic aneurysm formation. To the best of our knowledge, we presented the largest published cohort of CoA percutaneous treatment during pregnancy. We categorized our experience in managing aortic coarctation diagnosed during pregnancy in one algorithm. Our experience demonstrates that excellent maternal and neonatal pregnancy outcomes can be obtained in women after CoA percutaneous repair, diagnosed during pregnancy. An aortic stent implantation is effective and safe for both mother and fetus.
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Affiliation(s)
- Bogdan Volodymyrovych Cherpak
- Pediatric Cardiology and Cardiosurgery Department, National Amosov Institute of Cardiovascular Surgery NAMS, Kyiv, Ukraine,*Corresponding Author: Bogdan Volodymyrovych Cherpak, Pediatric Cardiology and Cardiosurgery Department, National Amosov Institute of Cardiovascular Surgery NAMS, Kyiv, Ukraine. E-mail:
| | - Yulia Volodymyrivna Davydova
- Obstetrics Department for Extragenital Pathology in Pregnant Women, Institute of Pediatrics, Obstetrics and Gynecology NAMS, Kyiv, Ukraine
| | - Vitalii Ivanovich Kravchenko
- Department of Surgical Treatment of Aortic Pathology, National Amosov Institute of Cardiovascular Surgery NAMS, Kyiv, Ukraine
| | - Natalia Sergiivna Yaschuk
- Pediatric Cardiology Intervention Department, National Amosov Institute of Cardiovascular Surgery NAMS, Kyiv, Ukraine
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Increased Risk of Aortic Dissection Associated With Pregnancy in Women With Turner Syndrome: A Systematic Review. Obstet Gynecol Surv 2021; 75:566-575. [PMID: 32997149 DOI: 10.1097/ogx.0000000000000833] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
Importance Turner syndrome (TS) is one of the most common chromosomal abnormalities in women. The condition is characterized by gonadal dysgenesis and is associated with structural cardiac abnormalities. Assisted reproductive technology with oocyte donation may be successful but places women with TS at increased risk of aortic dissection and death. Objective To summarize all cases of aortic dissection associated with pregnancy in women with TS and provide guidance regarding the safety of pregnancy. Evidence Acquisition Systematic review of PubMed for reports of women with TS, aortic dissection, and pregnancy. Results There are 14 total reported cases of aortic dissection associated with pregnancy in women with TS. Ten of these cases occurred during pregnancy or in the first month postpartum. The majority of affected pregnancies resulted from oocyte donation, 2 of which were multiple gestations. Two women had a documented history of hypertension, and 3 pregnancies were complicated by preeclampsia. Bicuspid aortic valve and coarctation of the aorta were the most common associated cardiac anomalies. More than half of women had some degree of aortic dilatation. Two women had no identifiable risk factors. Conclusions and Relevance Women with TS who desire pregnancy must be thoroughly counseled regarding the increased risk of aortic dissection during pregnancy and postpartum. Preconception consultation with maternal-fetal medicine, reproductive endocrinology, and cardiology is necessary along with a comprehensive physical evaluation. If women with TS choose to pursue pregnancy, they require rigorous cardiac monitoring each trimester during pregnancy and postpartum.
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12
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Ramlakhan KP, Tobler D, Greutmann M, Schwerzmann M, Baris L, Yetman AT, Nihoyannopoulos P, Manga P, Boersma E, Maggioni AP, Johnson MR, Hall R, Roos-Hesselink JW. Pregnancy outcomes in women with aortic coarctation. Heart 2020; 107:heartjnl-2020-317513. [PMID: 33122301 PMCID: PMC7873427 DOI: 10.1136/heartjnl-2020-317513] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/08/2020] [Revised: 09/22/2020] [Accepted: 09/30/2020] [Indexed: 12/30/2022] Open
Abstract
OBJECTIVE Pregnancy in women with aortic coarctation (CoA) has an estimated moderately increased risk (mWHO II-III) of adverse cardiovascular, obstetric or fetal events, but prospective data to validate this risk classification are scarce. We examined pregnancy outcomes and identified associations with adverse outcomes. METHODS Pregnancies in women with CoA were selected from the worldwide prospective Registry of Pregnancy and Cardiac Disease (ROPAC, n=303 out of 5739), part of the European Society of Cardiology EURObservational Research Programme. The frequency of and associations with major adverse cardiac events (MACE) and hypertensive disorders (pregnancy-induced hypertension, (pre-)eclampsia or haemolysis, elevated liver enzymes and low platelets syndrome) were analysed. RESULTS Of 303 pregnancies (mean age 30 years, pregnancy duration 39 weeks), 9.6% involved unrepaired CoA and 27.1% were in women with pre-existing hypertension. No maternal deaths or aortic dissections occurred. MACE occurred in 13 pregnancies (4.3%), of which 10 cases were of heart failure (3.3%). Univariable associations with MACE included prepregnancy clinical signs of heart failure (OR 31.8, 95% CI 6.8 to 147.7), left ventricular ejection fraction <40% (OR 10.4, 95% CI 1.8 to 59.5), New York Heart Association class >1 (OR 11.4, 95% CI 3.6 to 36.3) and cardiac medication use (OR 4.9, 95% CI 1.3 to 18.3). Hypertensive disorders of pregnancy occurred in 16 (5.3%), cardiac medication use being their only predictor (OR 3.2, 95% CI 1.1 to 9.6). Premature births were 9.1%, caesarean section was performed in 49.7% of pregnancies. Of 4 neonatal deaths, 3 were after spontaneous extreme preterm birth. CONCLUSIONS The ROPAC data show low MACE and hypertensive disorder rates during pregnancy in women with CoA, suggesting pregnancy to be more safe and better tolerated than previously appreciated.
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Affiliation(s)
| | - Daniel Tobler
- Department of Cardiology, University Hospital Basel, University of Basel, Basel, Switzerland
| | - Matthias Greutmann
- Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland
| | - Markus Schwerzmann
- Center for Congenital Heart Disease, University Hospital Inselspital, University of Bern, Bern, Switzerland
| | - Lucia Baris
- Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands
| | - Anji T Yetman
- Division of Pediatric Cardiology, University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha, Nebraska, USA
| | - Petros Nihoyannopoulos
- Department of Cardiology, National Heart and Lung Institute, Hammersmith Hospital, London, UK
| | - Pravin Manga
- Division of Cardiology, Department of Internal Medicine, University of Witwatersrand, Johannesburg, South Africa
| | - Eric Boersma
- Department of Clinical Epidemiology, Erasmus MC, Rotterdam, The Netherlands
| | - Aldo P Maggioni
- EURObservational Research Programme, European Society of Cardiology, Sophia Antipolis, France
- Maria Cecilia Hospital, GVM Care & Research, Cotignola, Italy
| | - Mark R Johnson
- Department of Obstetric Medicine, Imperial College London, Chelsea and Westminster Hospital, London, UK
| | - Roger Hall
- Department of Cardiology, University of East Anglia, Norwich, UK
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13
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Browne LP, Barker AJ, Vargas D. Imaging Follow-up of Repaired Aortic Coarctation. Semin Roentgenol 2020; 55:301-311. [PMID: 32859346 DOI: 10.1053/j.ro.2020.06.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Lorna P Browne
- Department of Radiology, Section of Pediatric Radiology, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, CO.
| | - Alex J Barker
- Department of Radiology, Section of Pediatric Radiology, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, CO; Department of Bioengineering, University of Colorado Anschutz Medical Campus, Aurora, CO
| | - Daniel Vargas
- Department of Radiology, University of Colorado and University of Colorado School of Medicine, Aurora, CO
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14
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Accelerating massively parallel hemodynamic models of coarctation of the aorta using neural networks. Sci Rep 2020; 10:9508. [PMID: 32528104 PMCID: PMC7289812 DOI: 10.1038/s41598-020-66225-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2020] [Accepted: 05/18/2020] [Indexed: 11/09/2022] Open
Abstract
Comorbidities such as anemia or hypertension and physiological factors related to exertion can influence a patient’s hemodynamics and increase the severity of many cardiovascular diseases. Observing and quantifying associations between these factors and hemodynamics can be difficult due to the multitude of co-existing conditions and blood flow parameters in real patient data. Machine learning-driven, physics-based simulations provide a means to understand how potentially correlated conditions may affect a particular patient. Here, we use a combination of machine learning and massively parallel computing to predict the effects of physiological factors on hemodynamics in patients with coarctation of the aorta. We first validated blood flow simulations against in vitro measurements in 3D-printed phantoms representing the patient’s vasculature. We then investigated the effects of varying the degree of stenosis, blood flow rate, and viscosity on two diagnostic metrics – pressure gradient across the stenosis (ΔP) and wall shear stress (WSS) - by performing the largest simulation study to date of coarctation of the aorta (over 70 million compute hours). Using machine learning models trained on data from the simulations and validated on two independent datasets, we developed a framework to identify the minimal training set required to build a predictive model on a per-patient basis. We then used this model to accurately predict ΔP (mean absolute error within 1.18 mmHg) and WSS (mean absolute error within 0.99 Pa) for patients with this disease.
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15
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Agasthi P, Pujari SH, Tseng A, Graziano JN, Marcotte F, Majdalany D, Mookadam F, Hagler DJ, Arsanjani R. Management of adults with coarctation of aorta. World J Cardiol 2020; 12:167-191. [PMID: 32547712 PMCID: PMC7284000 DOI: 10.4330/wjc.v12.i5.167] [Citation(s) in RCA: 20] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/23/2019] [Revised: 03/21/2020] [Accepted: 03/26/2020] [Indexed: 02/06/2023] Open
Abstract
Coarctation of the aorta (CoA) is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose. The hallmark finding on physical examination is upper extremity hypertension, and for this reason, CoA should be considered in any young hypertensive patient, justifying measurement of lower extremity blood pressure at least once in these individuals. The presence of a significant pressure gradient between the arms and legs is highly suggestive of the diagnosis. Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications. Surgical repair has traditionally been the mainstay of therapy for correction, although advances in endovascular technology with covered stents or stent grafts permit nonsurgical approaches for the management of older children and adults with native CoA and complications. Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease, which, remains the greatest cause of long-term mortality. Thus, blood pressure control and periodic reassessment with transthoracic echocardiography and three-dimensional imaging (computed tomography or cardiac magnetic resonance) for should be performed regularly as cardiovascular complications may occur decades after the intervention.
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Affiliation(s)
- Pradyumna Agasthi
- Department of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, United States
| | - Sai Harika Pujari
- Department of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, United States
| | - Andrew Tseng
- Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States
| | - Joseph N Graziano
- Division of Cardiology, Phoenix Children's Hospital, Children's Heart Center, Phoenix, AZ 85016, United States
| | - Francois Marcotte
- Department of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, United States
| | - David Majdalany
- Department of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, United States
| | - Farouk Mookadam
- Department of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, United States
| | - Donald J Hagler
- Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States
| | - Reza Arsanjani
- Department of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, United States.
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16
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Bhatia K, Eccles J, Meessala DK. Anesthetic management of a parturient with Shone's syndrome -a case report with review of literature. Korean J Anesthesiol 2020; 74:342-349. [PMID: 32434290 PMCID: PMC8342832 DOI: 10.4097/kja.20181] [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: 04/18/2020] [Accepted: 05/18/2020] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Shone's syndrome is a rare complex congenital cardiac condition, characterized by a supra-valvular mitral ring, parachute deformity of the mitral valve, aortic stenosis, and coarctation of the aorta. CASE A 26-year-old parturient with partial Shone's syndrome presented to our delivery unit in pulmonary edema. She underwent a scheduled cesarean section performed under a combined spinal-epidural anesthetic at 33 weeks. She had multidisciplinary input from the cardiac, obstetric, and anesthetic teams, which led to a good outcome. A review of the five published case reports of Shone's syndrome in pregnancy is presented along with key findings. CONCLUSIONS Our case report and the review highlight the successful use of combined spinal-epidural anesthetic and provides guidance to the multidisciplinary team on the varied presentation and the optimum management of women with Shone's syndrome during the peripartum period.
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Affiliation(s)
- Kailash Bhatia
- Department of Anesthesia and Peri-operative Medicine, Manchester University Hospital NHS Foundation Trust, University of Manchester, Manchester, United Kingdom
| | - Jennifer Eccles
- Department of Anesthesia, Royal Oldham Hospital, Manchester, United Kingdom
| | - Dinesh K Meessala
- Department of Anesthesia, New Cross Hospital, Wolverhampton, West Midlands, United Kingdom
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17
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Nair GKK, Bhagra C, Sermer M, Silversides CK, Pfaller B. Pregnancy in a woman with a chronic descending thoracic aortic dissection. Obstet Med 2020; 14:102-104. [PMID: 34394719 DOI: 10.1177/1753495x19900410] [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: 08/26/2019] [Revised: 11/21/2019] [Accepted: 12/17/2019] [Indexed: 11/15/2022] Open
Abstract
Pregnancy increases aortic wall stress and, for a woman with a chronic dissection, this can lead to extension of the dissection, aortic rupture, and death. We report a pregnancy in a woman with a history of a chronic type B aortic dissection. As a child, she had repeat balloon dilation of aortic coarctation, and one of the procedures was complicated by an iatrogenic dissection at the dilation site. At the age of 27 years, she had a planned pregnancy.
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Affiliation(s)
- Govind Krishna Kumar Nair
- Division of Cardiology, University of Toronto, Pregnancy and Heart Disease Program, Sinai Health System, Toronto, Canada
| | - Catriona Bhagra
- Department of Cardiology, Cambridge University and Royal Papworth NHS Foundation Trusts, Cambridge, UK
| | - Mathew Sermer
- Department of Obstetrics & Gynecology, Division of Maternal Fetal Medicine, University of Toronto, Sinai Health System, Toronto, Canada
| | - Candice K Silversides
- Division of Cardiology, University of Toronto, Pregnancy and Heart Disease Program, Sinai Health System, Toronto, Canada
| | - Birgit Pfaller
- Division of Cardiology, University of Toronto, Pregnancy and Heart Disease Program, Sinai Health System, Toronto, Canada
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18
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Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2020; 139:e698-e800. [PMID: 30586767 DOI: 10.1161/cir.0000000000000603] [Citation(s) in RCA: 276] [Impact Index Per Article: 55.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
Affiliation(s)
- Karen K Stout
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Curt J Daniels
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Jamil A Aboulhosn
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Biykem Bozkurt
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Craig S Broberg
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Jack M Colman
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Stephen R Crumb
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Joseph A Dearani
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Stephanie Fuller
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Michelle Gurvitz
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Paul Khairy
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Michael J Landzberg
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Arwa Saidi
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - Anne Marie Valente
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
| | - George F Van Hare
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative
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19
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Ciresi CM, Patel PR, Asdell SM, Hopkins KA, Hoyer MH, Kay WA. Management of Severe Coarctation of the Aorta During Pregnancy. JACC Case Rep 2020; 2:116-119. [PMID: 34316977 PMCID: PMC8301703 DOI: 10.1016/j.jaccas.2019.11.060] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/28/2019] [Revised: 11/27/2019] [Accepted: 11/29/2019] [Indexed: 01/03/2023]
Abstract
An 18-year-old primigravida woman underwent emergent percutaneous balloon stent repair of a severe coarctation of the aorta, allowing her to undergo an uneventful remaining pregnancy and the delivery of a healthy baby. Her case also demonstrates the association between maternal coarctation and fetal Shone complex. (Level of Difficulty: Beginner.)
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Affiliation(s)
| | - Pooja R. Patel
- Indiana University School of Medicine, Indianapolis, Indiana
| | | | - Kali A. Hopkins
- Department of Pediatrics, Section of Pediatric Cardiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois
| | - Mark H. Hoyer
- Department of Pediatrics, Division of Pediatric Cardiology, Indiana University School of Medicine, Indianapolis, Indiana
- Address for correspondence: Dr. Mark H. Hoyer, Indiana University School of Medicine, Division of Pediatric Cardiology, 705 Riley Hospital Drive, Riley 1134, Indianapolis, Indiana 46202.
| | - W. Aaron Kay
- Department of Pediatrics, Division of Pediatric Cardiology, Indiana University School of Medicine, Indianapolis, Indiana
- Department of Medicine, Krannert Institute of Cardiology, Indiana University School of Medicine, Indianapolis, Indiana
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20
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Söderström-Anttila V, Pinborg A, Karnis MF, Reindollar RH, Paulson RJ. Should women with Turner syndrome be allowed to carry their own pregnancies? Fertil Steril 2019; 112:220-225. [DOI: 10.1016/j.fertnstert.2019.06.003] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/04/2019] [Accepted: 06/04/2019] [Indexed: 02/08/2023]
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21
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Williamson J. Echocardiogram reveals unexpected finding in the investigation of murmur in a hypertensive postpartum patient. SONOGRAPHY 2019. [DOI: 10.1002/sono.12174] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
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22
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Chung EYM, Tiku A, Seeho S, Mather A. Significant infrarenal aortic stenosis in pregnancy: a case report. J Med Case Rep 2019; 13:115. [PMID: 31039808 PMCID: PMC6492387 DOI: 10.1186/s13256-019-2057-0] [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: 02/27/2019] [Accepted: 03/15/2019] [Indexed: 11/19/2022] Open
Abstract
Background Hypertension is common in pregnant women presenting with aortic coarctation or Takayasu’s arteritis. Uncontrolled hypertension leads to increased adverse maternal and neonatal events. Case presentation A 36-year-old gravida 2, para 1 Caucasian woman presented at 9 weeks of gestation with headaches but normal blood pressure. She had a past medical history of an in vitro fertilization pregnancy complicated by preeclampsia at 27 weeks of gestation (birth weight 1900 g) and infrarenal aortic stenosis. In the current pregnancy, she received aspirin and calcium as preeclampsia prophylaxis, remained normotensive throughout pregnancy, and was delivered by elective cesarean section at 37 weeks without complications. Conclusions This case demonstrates a significant chronic aortopathy in pregnancy with normal fetal growth and uterine blood flow through collateral supply from the internal mammary and epigastric arteries. Electronic supplementary material The online version of this article (10.1186/s13256-019-2057-0) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Edmund Yin Man Chung
- Renal Department, Royal North Shore Hospital, St Leonards, Australia. .,Northern Clinical School, The University of Sydney, Camperdown, Australia.
| | - Anushree Tiku
- Renal Department, St George Hospital, Kogarah, Australia
| | - Sean Seeho
- Faculty of Medicine and Health, The University of Sydney, Camperdown, Australia.,Clinical and Population Perinatal Health Research, Kolling Institute, St Leonards, Australia
| | - Amanda Mather
- Renal Department, Royal North Shore Hospital, St Leonards, Australia
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23
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24
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Sinning C, Zengin E, Kozlik-Feldmann R, Blankenberg S, Rickers C, von Kodolitsch Y, Girdauskas E. Bicuspid aortic valve and aortic coarctation in congenital heart disease-important aspects for treatment with focus on aortic vasculopathy. Cardiovasc Diagn Ther 2018; 8:780-788. [PMID: 30740325 DOI: 10.21037/cdt.2018.09.20] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
Prevalence of congenital heart disease (CHD) is constantly increasing during the last decades in line with the treatment options for patients ranging from the surgical as well to the interventional spectrum. This mini-review addresses two of the most common defects with bicuspid aortic valve (BAV) and coarctation of the aorta (CoA). Both diseases are connected to aortic vasculopathy which is one of the most common reasons for morbidity and mortality in young patients with CHD. The review will focus as well on other aspects like medication and treatment of pregnant patients with BAV and CoA. New treatment aspects will be as well reviewed as currently there are additional treatment options to treat aortic regurgitation or aortic aneurysm especially in patients with valvular involvement and a congenital BAV thus avoiding replacement of the aortic valve and potentially improving the future therapy course of the patients.
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Affiliation(s)
- Christoph Sinning
- Department of General and Interventional Cardiology, University Heart Center Hamburg, Hamburg, Germany.,German Center of Cardiovascular Research (DZHK), Partner Site Hamburg/Kiel/Lübeck, Germany
| | - Elvin Zengin
- Department of General and Interventional Cardiology, University Heart Center Hamburg, Hamburg, Germany
| | | | - Stefan Blankenberg
- Department of General and Interventional Cardiology, University Heart Center Hamburg, Hamburg, Germany.,German Center of Cardiovascular Research (DZHK), Partner Site Hamburg/Kiel/Lübeck, Germany
| | - Carsten Rickers
- Department of Pediatric Cardiology, University Heart Center Hamburg, Hamburg, Germany
| | - Yskert von Kodolitsch
- Department of General and Interventional Cardiology, University Heart Center Hamburg, Hamburg, Germany
| | - Evaldas Girdauskas
- Department of Cardiac and Cardiovascular Surgery, University Heart Center Hamburg, Hamburg, Germany
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25
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Foeller ME, Foeller TM, Druzin M. Maternal Congenital Heart Disease in Pregnancy. Obstet Gynecol Clin North Am 2018; 45:267-280. [PMID: 29747730 DOI: 10.1016/j.ogc.2018.01.011] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Congenital heart disease comprises most maternal cardiac diseases in pregnancy and is an important cause of maternal, fetal, and neonatal morbidity and mortality worldwide. Pregnancy is often considered a high-risk state for individuals with structural heart disease as a consequence of a limited ability to adapt to the major hemodynamic changes associated with pregnancy. Preconception counseling and evaluation are of utmost importance, as pregnancy is contraindicated in certain cardiac conditions. Pregnancy can be safely accomplished in most individuals with careful risk assessment before conception and multidisciplinary care throughout pregnancy and the postpartum period.
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Affiliation(s)
- Megan E Foeller
- Obstetrics and Gynecology, Stanford University, Stanford Hospital, 300 Pasteur Drive, Room G302, 5317, Stanford, CA 94305-5317, USA.
| | - Timothy M Foeller
- Internal Medicine, Stanford Health Care-ValleyCare, 5555 West Positas Boulevard, 1 West Hospitalist Room 1, Pleasanton, CA 94588, USA
| | - Maurice Druzin
- Obstetrics and Gynecology, Stanford University, Stanford Hospital, 300 Pasteur Drive, Room G302, 5317, Stanford, CA 94305-5317, USA
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Abstract
Objective Congenital heart defects represent the most common major congenital anomalies. The objective of this review was to define the most common forms of congenital heart disease (CHD) in pregnancy, outline preconception counseling, discuss the associated morbidity and mortality of each lesion, and review current recommendations for management of CHD in pregnancy. Evidence Acquisition A MEDLINE search of "congenital heart disease in pregnancy" and specific conditions in pregnancy including "ventricular septal defect," "atrial septal defect," "left outflow obstruction," "right outflow obstruction," "tetralogy of Fallot," and "transposition of the great vessels" was performed. Results The evidence included in the review contains 18 retrospective studies, 8 meta-analyses or systematic reviews or expert opinions, 5 case reports including surgical case reports, 2 prospective studies, and 2 clinical texts. Conclusions Given advances in surgical and medical management, women with a history of congenital cardiac defects are more frequently reaching childbearing age and requiring obstetric care. Many women with CHD can have successful pregnancies, although there are a few conditions that confer significant maternal risk, and pregnancy may even be contraindicated. Appropriate care for women with CHD requires a knowledge of cardiac physiology in pregnancy, the common lesions of CHD, and coordinated care from cardiology and maternal-fetal medicine specialists.
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27
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Phillips S, Pirics M. Congenital Heart Disease and Reproductive Risk: An Overview for Obstetricians, Cardiologists, and Primary Care Providers. Methodist Debakey Cardiovasc J 2018; 13:238-242. [PMID: 29744016 DOI: 10.14797/mdcj-13-4-238] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022] Open
Abstract
Patients with congenital heart disease have improved survival rates, and most patients are now expected to survive into adulthood. This improved survival has resulted in increasing numbers of women with congenital heart disease who are of childbearing age. This patient population requires specialized advice on contraception and pregnancy risk. Understanding the unique challenges this population presents is key to providing appropriate care.
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28
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Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2018; 73:e81-e192. [PMID: 30121239 DOI: 10.1016/j.jacc.2018.08.1029] [Citation(s) in RCA: 586] [Impact Index Per Article: 83.7] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
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29
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Niwa K. Adult Congenital Heart Disease with Pregnancy. Korean Circ J 2018; 48:251-276. [PMID: 29625509 PMCID: PMC5889976 DOI: 10.4070/kcj.2018.0070] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2018] [Accepted: 03/06/2018] [Indexed: 12/17/2022] Open
Abstract
The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk. Most frequent complications during pregnancy and delivery are heart failure, arrhythmias, bleeding or thrombosis, and rarely maternal death. Complications of fetus are prematurity, low birth weight, abortion, and stillbirth. Risk stratification of pregnancy and delivery relates to functional status of the patient and is lesion specific. Medication during pregnancy and post-delivery (breast feeding) is a big concern. Especially prescribing medication with teratogenicity should be avoidable. Adequate care during pregnancy, delivery, and the postpartum period requires a multidisciplinary team approach with cardiologists, obstetricians, anesthesiologists, neonatologists, nurses and other related disciplines. Caring for a baby is an important issue due to temporarily pregnancy-induced cardiac dysfunction, and therefore familial support is mandatory especially during peripartum and after delivery. Timely pre-pregnancy counseling should be offered to all women with CHD to prevent avoidable pregnancy-related risks. Successful pregnancy is feasible for most women with CHD at relatively low risk when appropriate counseling and optimal care are provided.
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Affiliation(s)
- Koichiro Niwa
- Department of Cardiology, Cardiovascular Center, St. Luke's International Hospital, Tokyo, Japan. ,
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Affiliation(s)
- Stephanie Venning
- Department of Cardiology, Norfolk and Norwich University Hospital NHS Trust, Norwich NR4 7UZ, UK
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Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, Lin AE, Mauras N, Quigley CA, Rubin K, Sandberg DE, Sas TCJ, Silberbach M, Söderström-Anttila V, Stochholm K, van Alfen-van derVelden JA, Woelfle J, Backeljauw PF. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol 2017; 177:G1-G70. [PMID: 28705803 DOI: 10.1530/eje-17-0430] [Citation(s) in RCA: 654] [Impact Index Per Article: 81.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2017] [Accepted: 06/07/2017] [Indexed: 12/14/2022]
Abstract
Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016. Prior to this meeting, five groups each addressed important areas in TS care: 1) diagnostic and genetic issues, 2) growth and development during childhood and adolescence, 3) congenital and acquired cardiovascular disease, 4) transition and adult care, and 5) other comorbidities and neurocognitive issues. These groups produced proposals for the present guidelines. Additionally, four pertinent questions were submitted for formal GRADE (Grading of Recommendations, Assessment, Development and Evaluation) evaluation with a separate systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society for Endocrinology and the Pediatric Endocrine Society, in collaboration with The European Society for Pediatric Endocrinology, The Endocrine Society, European Society of Human Reproduction and Embryology, The American Heart Association, The Society for Endocrinology, and the European Society of Cardiology. The guideline has been formally endorsed by the European Society for Endocrinology, the Pediatric Endocrine Society, the European Society for Pediatric Endocrinology, the European Society of Human Reproduction and Embryology and the Endocrine Society. Advocacy groups appointed representatives who participated in pre-meeting discussions and in the consensus meeting.
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Affiliation(s)
- Claus H Gravholt
- Departments of Endocrinology and Internal Medicine
- Departments of Molecular Medicine
| | - Niels H Andersen
- Departments of Cardiology, Aarhus University Hospital, Aarhus, Denmark
| | - Gerard S Conway
- Department of Women's Health, University College London, London, UK
| | - Olaf M Dekkers
- Department of Clinical Epidemiology, Leiden University Medical Centre, Leiden, The Netherlands
| | - Mitchell E Geffner
- The Saban Research Institute, Children's Hospital Los Angeles, Los Angeles, California, USA
| | - Karen O Klein
- Rady Children's Hospital, University of California, San Diego, California, USA
| | - Angela E Lin
- Department of Pediatrics, Medical Genetics Unit, Mass General Hospital for Children, Boston, Massachusetts, USA
| | - Nelly Mauras
- Division of Endocrinology, Nemours Children's Health System, Jacksonville, Florida, USA
| | | | - Karen Rubin
- Connecticut Children's Medical Center, Hartford, Connecticut, USA
| | - David E Sandberg
- Division of Psychology, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA
| | - Theo C J Sas
- Department of Pediatric Endocrinology, Sophia Children's Hospital, Rotterdam, The Netherlands
- Department of Pediatrics, Dordrecht, The Netherlands
| | - Michael Silberbach
- Department of Pediatrics, Doernbecher Children's Hospital, Portland, Oregon, USA
| | | | - Kirstine Stochholm
- Departments of Endocrinology and Internal Medicine
- Center for Rare Diseases, Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark
| | | | - Joachim Woelfle
- Department of Pediatric Endocrinology, Children's Hospital, University of Bonn, Bonn, Germany
| | - Philippe F Backeljauw
- Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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Abstract
OPINION STATEMENT Advances in cardiac surgical interventions in infancy and childhood have led to an increased number of women with congenital heart disease of childbearing age. For these women, individualized preconception counseling and pregnancy planning should be a vital component of their medical management, and presentation for obstetric care may even be an opportunity to re-establish cardiovascular care for patients who have been lost to follow-up. These patients have unique cardiovascular anatomy and physiology, which is dependent upon the surgical intervention they may have undergone during childhood or adolescence. These factors are associated with a variety of long-term complications, and the normal hemodynamic changes of pregnancy may unmask cardiac dysfunction and pose significant risk. Among three published risk assessment algorithms, the World Health Organization classification is the most sensitive in predicting maternal cardiovascular events in this population. Women with simple congenital heart defects generally tolerate pregnancy well and can be cared for in the community with careful monitoring. Conversely, women with complex congenital defects, with or without surgical repair and/or residual defects, should be managed in tertiary care centers under a multidisciplinary team of physicians experienced in adult congenital heart disease and high-risk obstetrics, who collaboratively participate in pregnancy planning, management, and care through childbirth and postpartum. Women who are cyanotic with oxygen saturation less than 85%, have significant pulmonary arterial hypertension of any cause, or have systemic ventricular dysfunction should be counseled to avoid pregnancy due to a very high risk of maternal and fetal mortality.
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Affiliation(s)
- Evin Yucel
- Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 02114, USA
| | - Doreen DeFaria Yeh
- Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 02114, USA. .,Adult Congenital Heart Disease Program, Massachusetts General Hospital, Division of Cardiology, Harvard Medical School, Boston, MA, USA.
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Sato H, Kamiya CA, Sawada M, Horiuchi C, Tsuritani M, Iwanaga N, Ohuchi H, Shiraishi I, Ichikawa H, Yoshimatsu J. Changes in echocardiographic parameters and hypertensive disorders in pregnancies of women with aortic coarctation. Pregnancy Hypertens 2017; 10:46-50. [PMID: 29153689 DOI: 10.1016/j.preghy.2017.05.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2016] [Revised: 05/14/2017] [Accepted: 05/27/2017] [Indexed: 12/30/2022]
Abstract
OBJECTIVES Pregnancy can be well tolerated after the repair of aortic coarctation. However, a higher incidence of hypertensive disorders during these pregnancies was reported. We analyzed the perinatal changes in echocardiographic parameters in women with aortic coarctation and investigated the risk factors of gestational hypertension (GH). METHODS We retrospectively identified 15 pregnancies in nine Japanese women with aortic coarctation between 1982 and 2015. We categorized the patients according to the presence/absence of GH as the group with GH(n=3) and that without GH(n=12). The echocardiographic parameters were compared between groups. RESULTS Our analysis revealed that a pre-pregnancy Doppler-measured pressure gradient≥20mmHg and a left ventricular mass index≥95g/m2 were significant risk factors for GH. The left ventricular end-diastolic diameters at the first and the third trimesters, the left ventricular end-systolic diameters at the first trimester, and the left ventricular ejection fraction at the third trimester were also significantly higher in the pregnancies with GH. All of these findings had been obtained before the patients' GH occurred. CONCLUSIONS Hypertrophy of the left ventricle with a lower ejection fraction and a high pressure gradient across the coarctation were risk factors for GH in the patients with aortic coarctation. Thus, serial measurements using echocardiography are important for predicting GH in women with aortic coarctation. However, further research investigating this finding with a larger sample size is needed.
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Affiliation(s)
- Hiroshi Sato
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan.
| | - Chizuko A Kamiya
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Masami Sawada
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Chinami Horiuchi
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Mitsuhiro Tsuritani
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Naoko Iwanaga
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Hideo Ohuchi
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Isao Shiraishi
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Hajime Ichikawa
- Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
| | - Jun Yoshimatsu
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
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Bhargava RA, Sabnis GR, Phatarpekar A, Lanjewar CP, Shah HC, Kerkar PG. A rare and treatable cause of hypertension in pregnancy. J Clin Hypertens (Greenwich) 2017; 19:801-802. [PMID: 28548295 DOI: 10.1111/jch.13029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Affiliation(s)
- Rishi A Bhargava
- Department of Cardiology, Seth G.S.M.C & K.E.M. Hospital, Parel, Mumbai, India
| | - Girish R Sabnis
- Department of Cardiology, Seth G.S.M.C & K.E.M. Hospital, Parel, Mumbai, India
| | - Ankur Phatarpekar
- Department of Cardiology, Seth G.S.M.C & K.E.M. Hospital, Parel, Mumbai, India
| | - Charan P Lanjewar
- Department of Cardiology, Seth G.S.M.C & K.E.M. Hospital, Parel, Mumbai, India
| | - Hetan C Shah
- Department of Cardiology, Seth G.S.M.C & K.E.M. Hospital, Parel, Mumbai, India
| | - Prafulla G Kerkar
- Department of Cardiology, Seth G.S.M.C & K.E.M. Hospital, Parel, Mumbai, India
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Bianca I, Geraci G, Gulizia MM, Egidy Assenza G, Barone C, Campisi M, Alaimo A, Adorisio R, Comoglio F, Favilli S, Agnoletti G, Carmina MG, Chessa M, Sarubbi B, Mongiovì M, Russo MG, Bianca S, Canzone G, Bonvicini M, Viora E, Poli M. Consensus Document of the Italian Association of Hospital Cardiologists (ANMCO), Italian Society of Pediatric Cardiology (SICP), and Italian Society of Gynaecologists and Obstetrics (SIGO): pregnancy and congenital heart diseases. Eur Heart J Suppl 2017; 19:D256-D292. [PMID: 28751846 PMCID: PMC5526477 DOI: 10.1093/eurheartj/sux032] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the USA alone the number is estimated to exceed 1 million. In this population, many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counselling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge. This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant.
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Affiliation(s)
- Innocenzo Bianca
- Pediatric Cardiology Unit, Maternity and Neonatal Department, ARNAS Garibaldi, Catania, Italy
| | - Giovanna Geraci
- Cardiology Department, PO Cervello, Az. Osp. Riuniti Villa Sofia-Cervello, Via Trabucco, 180, 90146 Palermo, Italy
| | - Michele Massimo Gulizia
- Cardiology Department, Ospedale Garibaldi-Nesima, Azienda di Rilievo Nazionale e Alta Specializzazione ‘Garibaldi’, Catania, Italy
| | - Gabriele Egidy Assenza
- Pediatric Cardiology and Adult Congenital Heart Program, Azienda Ospedaliera-Universitaria Sant’Orsola-Malpighi, Bologna, Itlay
| | - Chiara Barone
- Genetics Unit, Maternity and Neonatal Department, ARNAS Garibaldi, Catania, Italy
| | - Marcello Campisi
- Pediatric Cardiology Unit, Maternity and Neonatal Department, ARNAS Garibaldi, Catania, Italy
| | - Annalisa Alaimo
- Pediatric Cardiology Department, PO Di Cristina, ARNAS Civico, Palermo, Italy
| | - Rachele Adorisio
- Pediatric Cardiology Department, Ospedale Pediatrico Bambino Gesù, Roma, Italy
| | - Francesca Comoglio
- SCDU 2, Dipartimento di Scienze Chirurgiche (Surgical Sciences Department), Università di Torino, Italy
| | - Silvia Favilli
- Pediatric Cardiology Department, Azienda-Ospedalliero-Universitaria Meyer, Firenze, Italy
| | - Gabriella Agnoletti
- Pediatric Cardiology Department, Ospedale Regina Margherita, Città della Salute e della Scienza, Torino, Italy
| | - Maria Gabriella Carmina
- Cardiology Department, PO Cervello, Az. Osp. Riuniti Villa Sofia-Cervello, Via Trabucco, 180, 90146 Palermo, Italy
| | - Massimo Chessa
- Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato Milanese San Donato Milanese (MI), Italy
| | - Berardo Sarubbi
- Pediatric Cardiology and Cardiology SUN, Seconda Università di Napoli, AORN dei Colli, Ospedale Monaldi, Napoli, Italy
| | - Maurizio Mongiovì
- Pediatric Cardiology Department, PO Di Cristina, ARNAS Civico, Palermo, Italy
| | - Maria Giovanna Russo
- Pediatric Cardiology and Cardiology SUN, Seconda Università di Napoli, AORN dei Colli, Ospedale Monaldi, Napoli, Italy
| | - Sebastiano Bianca
- Genetics Unit, Maternity and Neonatal Department, ARNAS Garibaldi, Catania, Italy
| | - Giuseppe Canzone
- Women and Children Health Department, Ospedale S. Cimino, Termini Imerese (PA), Italy
| | - Marco Bonvicini
- Pediatric Cardiology and Adult Congenital Heart Program, Azienda Ospedaliera-Universitaria Sant’Orsola-Malpighi, Bologna, Itlay
| | - Elsa Viora
- Echography and Prenatal Diagnosis Centre, Obstetrics and Gynaecology Department, Città della Salute e della Scienza di Torino, Italy
| | - Marco Poli
- Intensive Cardiac Therapy Department, Ospedale Sandro Pertini, Roma, Italy
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Chetty S, Norton ME. Obstetric care in women with genetic disorders. Best Pract Res Clin Obstet Gynaecol 2017; 42:86-99. [PMID: 28392223 DOI: 10.1016/j.bpobgyn.2017.03.006] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/02/2016] [Revised: 03/07/2017] [Accepted: 03/10/2017] [Indexed: 01/04/2023]
Abstract
The management of pregnant women who are themselves affected with genetic diseases is an increasingly relevant and important issue. Improvements in early diagnosis and management of genetic disease, as well as advances in assisted reproductive technology have impacted pregnancy rates in a cohort of women who may not have otherwise been able to conceive. A multidisciplinary approach is key to the management of pregnant women with complex health conditions, including genetic diseases. Pertinent issues should be addressed in the preconception, antepartum, intrapartum and postpartum periods to optimize maternal and fetal health. Additionally, counseling regarding risk of inheritance in offspring and options for prenatal diagnosis should be reviewed if available. This reviews aims to help provide background and insight into the management strategies for various commonly encountered and complex genetic conditions in the setting of pregnancy.
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Affiliation(s)
- Shilpa Chetty
- Department of Obstetrics, Gynecology and Reproductive Sciences, 550 16th St, 7th Floor, University of California, San Francisco, CA 94143, USA.
| | - Mary E Norton
- Department of Obstetrics, Gynecology and Reproductive Sciences, 550 16th St, 7th Floor, University of California, San Francisco, CA 94143, USA
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Abstract
Coarctation of the aorta is a congenital cardiac defect that results in a variable degree of obstruction to the left side of the heart. It can present with either acute shock in the neonatal period as a critical lesion or it can appear more insidiously with hypertension or other findings later in life. This article summarises aspects of outpatient care of patients with this lesion, including clinical history and physical examination, ancillary testing, and issues associated with long-term follow-up and management. Increasing knowledge and experience with this group of patients have allowed for a higher level of evidence-based care over the long term.
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Nienaber CA, Clough RE. Management of Acute Aortic Syndromes. Interv Cardiol 2016. [DOI: 10.1002/9781118983652.ch71] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
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Barcellos BM, Loureiro FM, Sampaio LF, de Resende MAC. Double Invasive Blood Pressure Monitoring for Cesarean Delivery in a Pregnant Woman With Aortic Coarctation. ACTA ACUST UNITED AC 2016; 7:67-70. [PMID: 27310898 DOI: 10.1213/xaa.0000000000000344] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Aortic coarctation is a discrete narrowing of the proximal thoracic aorta. It is poorly tolerated during pregnancy because of its association with hypertension, cerebrovascular accident, and aortic rupture. We report a case of severe uncorrected congenital aortic coarctation in a 31-year-old symptomatic pregnant woman at 29 weeks of gestation who underwent successful cesarean delivery with an epidural anesthetic technique. Transthoracic echocardiography showed a gradient of 75 mm Hg. To avoid undiagnosed arterial hypotension and inadequate uteroplacental flow distal to the coarctation, double (radial and femoral) invasive arterial blood pressure measurement was used to monitor both pre- and postcoarctation arterial blood pressure.
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Affiliation(s)
- Bruno Mendonça Barcellos
- From the *Department of Anesthesiology, and †Department of Obstetrics and Gynecology, Hospital Universitário Antônio Pedro, Rio de Janeiro, Brazil
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Xu H, van Deel ED, Johnson MR, Opić P, Herbert BR, Moltzer E, Sooranna SR, van Beusekom H, Zang WF, Duncker DJ, Roos-Hesselink JW. Pregnancy mitigates cardiac pathology in a mouse model of left ventricular pressure overload. Am J Physiol Heart Circ Physiol 2016; 311:H807-14. [PMID: 27371681 DOI: 10.1152/ajpheart.00056.2016] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2016] [Accepted: 06/27/2016] [Indexed: 02/05/2023]
Abstract
In Western countries heart disease is the leading cause of maternal death during pregnancy. The effect of pregnancy on the heart is difficult to study in patients with preexisting heart disease. Since experimental studies are scarce, we investigated the effect of pressure overload, produced by transverse aortic constriction (TAC) in mice, on the ability to conceive, pregnancy outcome, and maternal cardiac structure and function. Four weeks of TAC produced left ventricular (LV) hypertrophy and dysfunction with marked interstitial fibrosis, decreased capillary density, and induced pathological cardiac gene expression. Pregnancy increased relative LV and right ventricular weight without affecting the deterioration of LV function following TAC. Surprisingly, the TAC-induced increase in relative heart and lung weight was mitigated by pregnancy, which was accompanied by a trend towards normalization of capillary density and natriuretic peptide type A expression. Additionally, the combination of pregnancy and TAC increased the cardiac phosphorylation of c-Jun, and STAT1, but reduced phosphoinositide 3-kinase phosphorylation. Finally, TAC did not significantly affect conception rate, pregnancy duration, uterus size, litter size, and pup weight. In conclusion, we found that, rather than exacerbating the changes associated with cardiac pressure overload, pregnancy actually attenuated pathological LV remodeling and mitigated pulmonary congestion, and pathological gene expression produced by TAC, suggesting a positive effect of pregnancy on the pressure-overloaded heart.
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Affiliation(s)
- Hong Xu
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands; Department of Cardiac Surgery, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, Peoples Republic of China
| | - Elza D van Deel
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Mark R Johnson
- Academic Department of Obstetrics and Gynaecology, Imperial College London, Chelsea and Westminster Hospital, United Kingdom; and
| | - Petra Opić
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Bronwen R Herbert
- Academic Department of Obstetrics and Gynaecology, Imperial College London, Chelsea and Westminster Hospital, United Kingdom; and
| | - Els Moltzer
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands; Division of Pharmacology and Vascular Medicine, Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Suren R Sooranna
- Academic Department of Obstetrics and Gynaecology, Imperial College London, Chelsea and Westminster Hospital, United Kingdom; and
| | - Heleen van Beusekom
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Wang-Fu Zang
- Department of Cardiac Surgery, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, Peoples Republic of China
| | - Dirk J Duncker
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Jolien W Roos-Hesselink
- Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, The Netherlands;
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Lansman SL, Goldberg JB, Kai M, Tang GHL, Malekan R, Spielvogel D. Aortic surgery in pregnancy. J Thorac Cardiovasc Surg 2016; 153:S44-S48. [PMID: 27431443 DOI: 10.1016/j.jtcvs.2016.06.015] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/15/2016] [Revised: 05/05/2016] [Accepted: 06/11/2016] [Indexed: 02/03/2023]
Abstract
Pregnancy engenders changes in hemodynamics and the aortic wall that make a woman more susceptible to aortic dilatation and dissection. This is particularly true of women with aortic dilatation and an aortopathy, including the inherited fibrillinopathies, bicuspid aortic valve, and Turner syndrome. Women in these risk groups may be served best by undergoing elective aortic surgery before becoming pregnant. However, some women present during pregnancy with significant aortic dilatation, rapid expansion, or aortic dissection, and strategies to deal with these situations, while optimizing maternal and fetal outcomes, change as gestation progresses. This review summarizes the approaches to the management of aortic diseases and the conduct of aortic surgery in pregnancy.
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Affiliation(s)
- Steven L Lansman
- Westchester Medical Center, Valhalla, NY; Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Valhalla, NY.
| | - Joshua B Goldberg
- Westchester Medical Center, Valhalla, NY; Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Valhalla, NY
| | - Masashi Kai
- Westchester Medical Center, Valhalla, NY; Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Valhalla, NY
| | - Gilbert H L Tang
- Westchester Medical Center, Valhalla, NY; Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Valhalla, NY
| | - Ramin Malekan
- Westchester Medical Center, Valhalla, NY; Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Valhalla, NY
| | - David Spielvogel
- Westchester Medical Center, Valhalla, NY; Department of Surgery, Section of Cardiothoracic Surgery, New York Medical College, Valhalla, NY
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Pillutla P, Nguyen T, Markovic D, Canobbio M, Koos BJ, Aboulhosn JA. Cardiovascular and Neonatal Outcomes in Pregnant Women With High-Risk Congenital Heart Disease. Am J Cardiol 2016; 117:1672-1677. [PMID: 27055756 DOI: 10.1016/j.amjcard.2016.02.045] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/29/2015] [Revised: 02/23/2016] [Accepted: 02/23/2016] [Indexed: 12/01/2022]
Abstract
Congenital heart disease (CHD) increases the risk of adverse maternal and neonatal outcomes. However, previous studies have included mainly women with low-risk features. A single-center, retrospective analysis of pregnant women with CHD was performed. Inclusion criteria were the following high-risk congenital lesions and co-morbidities: maternal cyanosis; New York Heart Association (NHYA) functional class >II; severe ventricular dysfunction; maternal arrhythmia, single ventricle (SV) physiology, severe left-sided heart obstruction and severe pulmonary arterial hypertension. Multivariate analyses for predictors of adverse maternal cardiovascular and neonatal outcomes were performed. Forty-three women reported 61 pregnancies. There were no maternal or neonatal deaths. Maternal cardiac (31%) and neonatal (54%) complications were frequent. The most frequent cardiac events were pulmonary edema, arrhythmia, and reduced NYHA class. Previous arrhythmia conferred a 12-fold increase in the odds of experiencing at least one major cardiac complication. Maternal SV physiology was an independent risk factor for low birth weight, risk of neonatal intensive care unit admission and lower gestational age. Maternal cyanosis and severe pulmonary arterial hypertension also predicted adverse neonatal outcomes. In conclusion, mothers without antepartum arrhythmia or functional incapacity are unlikely to experience arrhythmias or a decrease in NYHA class during pregnancy. In addition, SV physiology is a robust predictor of neonatal complications. Antepartum counseling and assessment of maternal fitness are crucial for the woman with CHD.
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Affiliation(s)
- Priya Pillutla
- Division of Cardiology, Department of Medicine, Harbor-University of California, Los Angeles Medical Center, Torrance, California.
| | - Tina Nguyen
- Department of Obstetrics and Gynecology, Ronald Reagan University of California, Los Angeles Medical Center, Los Angeles, California
| | - Daniela Markovic
- Department of Biostatics, University of California Los Angeles, Los Angeles, California
| | - Mary Canobbio
- Ahmanson/UCLA ACHD Center Lecturer, University of California Los Angeles, School of Nursing, Los Angeles, California
| | - Brian J Koos
- Department of Obstetrics and Gynecology, Ronald Reagan University of California, Los Angeles Medical Center, Los Angeles, California
| | - Jamil A Aboulhosn
- Division of Cardiology, Department of Medicine, Ahmanson/University of California, Los Angeles Adult Congenital Heart Disease Center, Los Angeles, California
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Hebson C, Saraf A, Book WM. Risk Assessment and Management of the Mother with Cardiovascular Disease. Clin Perinatol 2016; 43:1-22. [PMID: 26876118 DOI: 10.1016/j.clp.2015.11.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Chronic medical conditions account for most nonobstetrical pregnancy-related maternal complications. Preconception counseling of women with cardiovascular disease can be aided by an understanding of cardiovascular physiology in pregnancy and risk scores to guide management.
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Affiliation(s)
- Camden Hebson
- Department of Medicine, The Sibley Heart Center, Emory University School of Medicine, 1365 Clifton Road Northeast, Clinic A, 2nd Floor, Cardiology, Atlanta, GA 30322, USA; Department of Pediatrics, The Sibley Heart Center, Emory University School of Medicine, 1365 Clifton Road Northeast, Clinic A, 2nd Floor, Cardiology, Atlanta, GA 30322, USA
| | - Anita Saraf
- Division of Cardiology, Department of Medicine, Emory University School of Medicine, 1365 Clifton Road Northeast, Atlanta, GA 30322, USA
| | - Wendy M Book
- Division of Cardiology, Department of Medicine, Emory Adult Congenital Heart Center, Emory University School of Medicine, 1365 Clifton Road Northeast, Clinic A, 2nd Floor, Cardiology, Atlanta, GA 30322, USA.
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Akil MA, Bilik MZ, Yildiz A, Acet H, Ertas F, Simsek H, Polat N, Zengin H, Akilli R, Agacayak E, Kayan F, Ozdemir M, Alan S. Peripartum cardiomyopathy in Turkey: Experience of three tertiary centres. J OBSTET GYNAECOL 2016; 36:574-80. [DOI: 10.3109/01443615.2015.1107531] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Affiliation(s)
- Mehmet Ata Akil
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Mehmet Zihni Bilik
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Abdulkadir Yildiz
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Halit Acet
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Faruk Ertas
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Hakki Simsek
- Department of Cardiology, Faculty of Medicine, Yuzuncu Yıl University, Van, Turkey,
| | - Nihat Polat
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Halit Zengin
- Department of Cardiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey,
| | - Rabia Akilli
- Department of Cardiology, Faculty of Medicine, Cukurova University, Adana, Turkey, and
| | - Elif Agacayak
- Department of Obstetrics and Gynecology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey
| | - Fethullah Kayan
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
| | - Mahmut Ozdemir
- Department of Cardiology, Faculty of Medicine, Yuzuncu Yıl University, Van, Turkey,
| | - Sait Alan
- Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey,
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Jalal Z, Iriart X, Thambo JB. Aortic Coarctation Diagnosed During Pregnancy in a Woman With Repaired Tetralogy of Fallot. Ann Thorac Surg 2015; 100:e45-7. [PMID: 26354665 DOI: 10.1016/j.athoracsur.2015.01.072] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2014] [Revised: 12/05/2014] [Accepted: 01/16/2015] [Indexed: 11/26/2022]
Abstract
Aortic coarctation is thought to be a rare condition in patients with tetralogy of Fallot. We report the case of a 26 year old woman presenting with systemic hypertension at 17 weeks of pregnancy after repair of tetralogy of Fallot in childhood. Echocardiography and magnetic resonance imaging revealed right aortic arch with severe isthmic coarctation. Her blood pressure was controlled medically during the rest of her pregnancy, and delivery was uneventful. Successful transcatheter placement of a covered stent at the level of the coarctation was performed after delivery. To our knowledge, this is the first reported case of aortic coarctation diagnosed in an adult patient late after repair of tetralogy of Fallot.
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Affiliation(s)
- Zakaria Jalal
- Department of Paediatric Cardiology, University Hospital of Bordeaux, Bordeaux, France.
| | - Xavier Iriart
- Department of Paediatric Cardiology, University Hospital of Bordeaux, Bordeaux, France
| | - Jean-Benoit Thambo
- Department of Paediatric Cardiology, University Hospital of Bordeaux, Bordeaux, France
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Pregnancy and Thoracic Aortic Disease: Managing the Risks. Can J Cardiol 2015; 32:78-85. [PMID: 26604124 DOI: 10.1016/j.cjca.2015.09.003] [Citation(s) in RCA: 52] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2015] [Revised: 09/11/2015] [Accepted: 09/11/2015] [Indexed: 12/24/2022] Open
Abstract
The most common aortopathies in women of childbearing age are bicuspid aortic valve, coarctation of the aorta, Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, SMAD3 aortopathy, Turner syndrome, and familial thoracic aneurysm and dissection. The hemodynamic and hormonal changes of pregnancy increase the risk of progressive dilatation or dissection of the aorta in these women. The presence of hypertension increases the risk further. Therefore, appropriate preconception counselling is advised. For women who become pregnant, serial follow-up by a specialized multidisciplinary team throughout pregnancy and postpartum period is required. In this review we discuss risk assessment and management strategies for women with aortopathies.
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Abstract
Most women with known congenital heart disease can have successful pregnancy, labor, and delivery. Preconception assessment is essential in understanding anatomy, repairs, and current physiology, all of which can influence risk in pregnancy. With that foundation, a multidisciplinary cardio-obstetric team can predict and prepare for complications that may occur with superimposed hemodynamic changes of pregnancy. Individuals with Eisenmenger syndrome, pulmonary hypertension, cyanosis, significant left heart obstruction, ventricular dysfunction, or prior major cardiac event are among the highest risk for complications.
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