Alvaro Garcia Ropero1, Shankar Baskar2, Jolien W Roos Hesselink3, Andrea Girnius4, Dominica Zentner5, Lorna Swan6, Magalie Ladouceur7, Nicole Brown2,3, Gruschen R Veldtman8,3. 1. Department of Cardiology, Instituto de Investigación Sanitaria-Fundación Jiménez Díaz, Madrid, Spain (A.G.R.). 2. Heart Institute, Cincinnati Children's Hospital Medical Center, OH (S.B. G.R.V., N.B.). 3. Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands (J.W.R.H., N.B., G.R.V.). 4. Department of Anesthesiology, University of Cincinnati, OH (A.G.). 5. Department of Cardiology, Royal Melbourne Hospital and Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Parkville, Australia (D.Z.). 6. Department of Adult Congenital Heart Disease, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom (L.S.). 7. Department of Adult Congenital Heart Disease, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris Descartes University, Sorbonne Paris Cité, Paris Centre de Recherche Cardiovasculaire, INSERM U970, France (M.L.). 8. Heart Institute, Cincinnati Children's Hospital Medical Center, OH (S.B. G.R.V., N.B.) Gruschen.veldtman@cchmc.org.
Abstract
BACKGROUND: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. This article seeks to review the published literature and identify lessons learnt from this collective experience. METHODS AND RESULTS: We conducted a systematic review to evaluate maternal and fetal outcomes of pregnancy in women with a Fontan circulation. Among 1150 studies that were screened, 6 studies had sufficient longitudinal data points to qualify for meaningful inclusion, yielding 255 pregnancies in 133 women after Fontan procedure resulting in 115 live births (45%; including reports from 1986 to 2015). There was a total of 137 pregnancy losses (69%), with 115 miscarriages (45%), 19 elective terminations of pregnancy (7%), 2 stillbirths (1%), and 1 ectopic pregnancy (1%).The most common cardiovascular adverse events were supraventricular arrhythmia affecting 8.4% (range, 3%-37%) and heart failure affecting 3.9% (range, 3%-11%) of pregnancies. These complications were successfully managed with conventional approaches. No maternal deaths were reported. Postpartum hemorrhage was the predominant obstetric complication affecting 14% of the patients. Most patients were on antiplatelet agents (27%) or anticoagulants (50%) whereas only a minority (11%) were on neither. Among the 115 live births, 68 were premature (59%), 17 were small for gestational age (20%), and neonatal death occurred in 6 patients (5%). CONCLUSIONS: The most commonly reported cardiovascular complications in patients with Fontan physiology-associated pregnancy were arrhythmia and heart failure. Miscarriages were highly prevalent as was prematurity and intrauterine growth restriction. Postpartum hemorrhage seems to be the most common obstetric complication. Large-scale data sets are needed to confirm these early observations and address the late sequelae of pregnancy in women with a Fontan circulation.
BACKGROUND: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. This article seeks to review the published literature and identify lessons learnt from this collective experience. METHODS AND RESULTS: We conducted a systematic review to evaluate maternal and fetal outcomes of pregnancy in women with a Fontan circulation. Among 1150 studies that were screened, 6 studies had sufficient longitudinal data points to qualify for meaningful inclusion, yielding 255 pregnancies in 133 women after Fontan procedure resulting in 115 live births (45%; including reports from 1986 to 2015). There was a total of 137 pregnancy losses (69%), with 115 miscarriages (45%), 19 elective terminations of pregnancy (7%), 2 stillbirths (1%), and 1 ectopic pregnancy (1%).The most common cardiovascular adverse events were supraventricular arrhythmia affecting 8.4% (range, 3%-37%) and heart failure affecting 3.9% (range, 3%-11%) of pregnancies. These complications were successfully managed with conventional approaches. No maternal deaths were reported. Postpartum hemorrhage was the predominant obstetric complication affecting 14% of the patients. Most patients were on antiplatelet agents (27%) or anticoagulants (50%) whereas only a minority (11%) were on neither. Among the 115 live births, 68 were premature (59%), 17 were small for gestational age (20%), and neonatal death occurred in 6 patients (5%). CONCLUSIONS: The most commonly reported cardiovascular complications in patients with Fontan physiology-associated pregnancy were arrhythmia and heart failure. Miscarriages were highly prevalent as was prematurity and intrauterine growth restriction. Postpartum hemorrhage seems to be the most common obstetric complication. Large-scale data sets are needed to confirm these early observations and address the late sequelae of pregnancy in women with a Fontan circulation.
Authors: Maria Victoria Ordoñez; Giovanni Biglino; Massimo Caputo; Brenda Kelly; Aarthi Mohan; Johanna Trinder; Stephanie L Curtis Journal: Open Heart Date: 2021-02