E Gandjbakhch1, E Varlet2, G Duthoit3, V Fressart4, P Charron5, C Himbert6, C Maupain3, C Bordet7, F Hidden-Lucet3, J Nizard8. 1. Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, ICAN, Institut de Cardiologie, F-75013 Paris, France; Centre de Référence Pour les Maladies Cardiaques Héréditaires, APHP, ICAN, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France; Sorbonne Universités, UPMC Univ Paris 06 Faculté de Médecine, Paris, France. Electronic address: estelle.gandjbakhch@aphp.fr. 2. Assistance Publique-Hôpitaux de Paris, Hôpital Bichat-Claude Bernard, F-75018, Département de Cardiologie, Paris, France. 3. Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, ICAN, Institut de Cardiologie, F-75013 Paris, France; Centre de Référence Pour les Maladies Cardiaques Héréditaires, APHP, ICAN, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France. 4. Centre de Référence Pour les Maladies Cardiaques Héréditaires, APHP, ICAN, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France; Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Département de Biochimie Métabolique, Cardiogénétique, F-75013 Paris, France. 5. Centre de Référence Pour les Maladies Cardiaques Héréditaires, APHP, ICAN, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France; Université Versailles Saint Quentin & AP-, HP, Service de Génétique, Hôpital Ambroise Paré, Boulogne-Billancourt, France. 6. Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, ICAN, Institut de Cardiologie, F-75013 Paris, France. 7. Centre de Référence Pour les Maladies Cardiaques Héréditaires, APHP, ICAN, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France. 8. Sorbonne Universités, UPMC Univ Paris 06 Faculté de Médecine, Paris, France; Service de Gynécologie Obstétrique, APHP, Hôpital de la Pitié Salpêtrière, F-75013 Paris, France.
Abstract
INTRODUCTION: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children. METHODS: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Cardiac and obstetrical events having occurred during pregnancy/delivery/post-partum periods and neonatal data/follow-up were collected. RESULTS: Sixty pregnancies in twenty-three patients were identified between 1968 and 2016. Only two major non-fatal cardiac events (one sustained non-documented tachycardia and one ventricular tachycardia) were recorded during pregnancy in two different mothers (3% of pregnancies, 9% of mothers). None occurred during delivery or in the postpartum period. No mother developed heart failure. Beta-blocker therapy during pregnancy (n=15) was associated with lower birthweight (2730 vs 3400g, p=0.004). Only two preterm deliveries occurred, unrelated to cardiac condition. Caesarean section was performed in 13% of cases. Premature sudden-death occurred in 10% (n=5) of children before 25years-old including two in the first year of life. CONCLUSION: ARVC/D is associated with a low rate of major cardiac events during pregnancy and vaginal delivery appears safe. The risk of sustained ventricular arrhythmia seems poorly predictable and supports the continuation of beta-blockers during pregnancy. Major cardiac events were frequent in childhood, justifying close cardiac monitoring.
INTRODUCTION: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children. METHODS: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Cardiac and obstetrical events having occurred during pregnancy/delivery/post-partum periods and neonatal data/follow-up were collected. RESULTS: Sixty pregnancies in twenty-three patients were identified between 1968 and 2016. Only two major non-fatal cardiac events (one sustained non-documented tachycardia and one ventricular tachycardia) were recorded during pregnancy in two different mothers (3% of pregnancies, 9% of mothers). None occurred during delivery or in the postpartum period. No mother developed heart failure. Beta-blocker therapy during pregnancy (n=15) was associated with lower birthweight (2730 vs 3400g, p=0.004). Only two preterm deliveries occurred, unrelated to cardiac condition. Caesarean section was performed in 13% of cases. Premature sudden-death occurred in 10% (n=5) of children before 25years-old including two in the first year of life. CONCLUSION: ARVC/D is associated with a low rate of major cardiac events during pregnancy and vaginal delivery appears safe. The risk of sustained ventricular arrhythmia seems poorly predictable and supports the continuation of beta-blockers during pregnancy. Major cardiac events were frequent in childhood, justifying close cardiac monitoring.
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