Arrhythmogenic RV Cardiomyopathy in Pregnancy

Study Questions:

What are the course and outcomes of arrhythmogenic right ventricular cardiomyopathy (ARVC) during pregnancy?


Study subjects and data were collected from the prospective Johns Hopkins and Interuniversity Cardiology Institute of the Netherlands ARVC registry. Patient data were collected for the registry from time of ARVC diagnosis. Patients with a diagnosis of ARVC during pregnancy were compared to female controls of child bearing age with ARVC not having a pregnancy during their time in the registry. Primary outcomes were development of ventricular arrhythmias (VAs) and onset of heart failure.


There were 26 ARVC patients experiencing 39 pregnancies during the registry period. These were compared to 117 controls. Sixteen of the 39 pregnancies were considered potentially high risk due to major RV abnormalities and/or history of VA. Five patients with VA prior to pregnancy did not have VA during pregnancy. Five of 30 patients, without prior VA, developed VA during pregnancy (17%); two of these occurred during interruption of beta-blocker. Complaints of palpitations occurred in 11 of 39 pregnancies, but only three of these had VA. Two patients developed heart failure; both had prior major RV structural abnormalities. Overall, 32 of 39 pregnancies (82%) were without major events. All pregnancies advancing beyond the first trimester were born healthy. First trimester losses occurred at the normal population rate.


The authors concluded that pregnancy was well tolerated in this population of ARVC patients, with VAs developing in 13%.


In this largest to date study of ARVC in pregnancy, patients who developed VAs had no history of prior VA, and those with a history of VA had uneventful pregnancies, suggesting that risk stratification based on prior events may not be accurate. Symptoms during pregnancy also did not correlate well with VA. Most patients did very well, although 18% with RV structural abnormalities developed heart failure during pregnancy. Pregnancy outcomes were all good.

Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias

Keywords: Adrenergic beta-Antagonists, Arrhythmias, Cardiac, Arrhythmogenic Right Ventricular Dysplasia, Cardiomyopathies, Pregnancy, Pregnancy Outcome

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