Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia in Pregnancy: Literature Review and Case Update

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal dominant condition that may predispose patients to life-threatening arrhythmia, posing a potentially significant cardiac risk in pregnancy. Management of this condition is performed on a case-to-case basis as research is limited.

A 28 year old woman was diagnosed with Arrhythmogenic right ventricular cardiomyopathy/dysplasia following genetic screening performed after a sudden sporting-induced collapse of her brother at age 21, at which point a prophylactic implantable cardioverter defibrillator (ICD) was placed. She became pregnant after the placement of the ICD and underwent a spontaneous miscarriage at 10 weeks gestation, which was successfully managed medically. ARVC/D is uncommonly considered alongside pregnancy; therefore, patients are managed individually, which requires a thorough understanding of current strategies.

A literature review was performed using PubMed to assess current knowledge of ARVC/D in pregnancy.

Although limited, published case reports and few retrospective cohort and systematic reviews conclusively describe safe pregnancy in mothers with Arrhythmogenic right ventricular cardiomyopathy/dysplasia. In the majority of cases, pregnancy is safe to progress to term and vaginal delivery is preferred, where Caesarean section is only indicated in cases of obstetric complications. Prophylactic ICD placement is the first-line management to prevent arrhythmia; flecainide or radio-ablation are the preferred second line treatments in the absence of an ICD. To reduce the risk of arrhythmia, beta-blockers should be continued throughout the pregnancy.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia poses a cardiac complication that requires unique management and counselling. Although the majority of cases will result in vaginal delivery, safe management of pregnancy includes frequent cardiac monitoring of the mother and health of the foetus as well as precautions regarding delivery and genetic counselling.

Author(s): Mariah Colussi, Jasmeet Kumari , Etop Akpan and Sasikala Selvamani

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