Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) in pregnancy: a case series of nine patients and review of literature

Abstract

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy characterized by myocardial necrosis and fibrofatty substitution of the myocardium, predominantly of the right ventricle. The evaluation of risk associated with gestation and delivery in patients with ARVC is difficult due to the small number of already reported cases. We present our experience of patients with ARVC who completed a pregnancy and delivery. Methods A case series of nine women in Calgary, Canada, from 2013 to 2018, who were diagnosed with ARVC before or during pregnancy. Patients were identified using our Cardiac-Obstetrics database, and information was collected through electronic charts and patient recollection. Results All pregnancies reported were singleton with an average maternal age of 31 years. Six patients had a related genetic mutation. Beta blockers were being used by eight, and five had an implantable cardioverter-defibrillator (ICD) prior to the pregnancy. None of the patients developed heart failure during pregnancy, but one had a complicated antepartum and postpartum course. All pregnancies delivered at term with eight receiving neuroaxial analgesia. Five patients delivered vaginally. Those without an ICD had continuous cardiac monitoring intrapartum. The incidence of small for gestational age (33%) was higher than the general population. All of the patients breastfed the newborns. Conclusions Pregnancies in these patients with ARVC were generally well tolerated. Given the rarity of the disease and absence of any clinical guidelines, multidisciplinary care is essential in the management of these patients.