Outcomes and management of arrhythmogenic right ventricular cardiomyopathy in pregnancy: a case report

Abstract

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease with an estimated prevalence of up to 1:5000 in the general population. Few cases of ARVC during pregnancy are described in literature.

Case summary: A 32-year-old primigravida was referred to our clinic during the 32nd gestational week. Arrhythmogenic right ventricular cardiomyopathy diagnosis with biventricular involvement was made according to Task Force criteria. Beta-blocker therapy was started and an elective caesarean section was planned, during the 37th gestational week; no complications occurred. Thirteen months after delivery, the patient was readmitted in our hospital due to an episode of pre-syncope and after team discussion, an implantable cardioverter-defibrillator (ICD) was implanted.

Discussion: This case suggests that the absence of signs and symptoms of heart failure (HF) at a first evaluation plays a major role to predict maternal and foetal outcome in ARVC. Our experience is consistent with the evidence that indicates a favourable outcome in asymptomatic patients treated with optimal medical therapy during pregnancy. In our case, despite no major HF or arrhythmic complications during pregnancy, delivery, and puerperium, we observed an arrhythmic disease progression more likely independent from pregnancy, leading to ICD implantation.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Case report; Heart failure; Implantable cardioverter-defibrillator; Pregnancy; Sudden cardiac death.

Figure 1

Basal 12-lead electrocardiogram: sinus rhythm, incomplete right bundle branch block, epsilon wave in V2 (see red arrow), negative T waves in V1–V4, and premature ventricular contractions with negative concordance.

Figure 2

Holter electrocardiogram strip: frequent premature ventricular contractions, couplets, and triplets.

Figure 3

Cardiac magnetic resonance static cine images (short-axis and four-chamber views) showing right ventricular dilatation.

Figure 4

Cardiac magnetic resonance late gadolinium enhancement phase sensitive inversion recovery sequences (four-chamber views) marking areas of diffuse fibrofatty infiltration in the basal sub-tricuspid segment and mid right ventricular walls and in the lateral wall of the left ventricular (see red arrows).

Figure 5

Cardiac magnetic resonance late gadolinium enhancement phase sensitive inversion recovery sequences (short-axis views) marking areas of fibrofatty infiltration in the right ventricular (diffusely) and in the antero-lateral wall of the left ventricular (see red arrows).