Pregnancy course and outcomes in women with arrhythmogenic right ventricular cardiomyopathy

Abstract

ObjectivesTo characterise pregnancy course and outcomes in women with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).MethodsFrom a combined Johns Hopkins/Dutch ARVD/C registry, we identified 26 women affected with ARVD/C (by 2010 Task Force Criteria) during 39 singleton pregnancies >13 weeks (1–4 per woman). Cardiac symptoms, treatment and episodes of sustained ventricular arrhythmias (VAs) and heart failure (HF) ≥ Class C were characterised. Obstetric outcomes were ascertained. Incidence of VA and HF were compared with rates in the non-pregnant state. Long-term disease course was compared with 117 childbearing-aged female patients with ARVD/C who had not experienced pregnancy with ARVD/C.ResultsTreatment during pregnancy (n=39) included β blockers (n=16), antiarrhythmics (n=6), diuretics (n=3) and implantable cardioverter defibrillators (ICDs) (n=28). In five pregnancies (13%), a single VA occurred, including two ICD-terminated events. Arrhythmias occurred disproportionately in probands without VA history (p=0.045). HF, managed on an outpatient basis, developed in two pregnancies (5%) in women with pre-existing overt biventricular or isolated right ventricular disease. All infants were live-born without major obstetric complications. Caesarean sections (n=11, 28%) had obstetric indications, except one (HF). β Blocker therapy was associated with lower birth weight (3.1±0.48 kg vs 3.7±0.57 kg; p=0.002). During follow-up children remained healthy (median 3.4 years), and mothers were without cardiac mortality or transplant. Neither VA nor HF incidence was significantly increased during pregnancy. ARVD/C course (mean 6.5±5.6 years) did not differ based on pregnancy history.ConclusionsWhile most pregnancies in patients with ARVD/C were tolerated well, 13% were complicated by VA and 5% by HF.

Diagnostic and therapeutic strategies for arrhythmogenic right ventricular dysplasia/cardiomyopathy patient

“…Atrial fibrillation may reflect electrical instability and has been associated with life-threatening arrhythmic events 66 . Risk of ventricular arrhythmia does not seem to significantly increase during pregnancy 133 . Unwillingness to restrict exercise has also been associated with higher risk of ventricular arrhythmia, 17 , 62 The absence of risk factors confers a low risk of sudden death (<1% per year) and ICD is not indicated 1 …”

Number of pregnancies and subsequent phenotype in a cross-sectional cohort of women with arrhythmogenic cardiomyopathy

“…As the disease is frequently diagnosed in women of childbearing age, 10 data regarding the effect of pregnancy on disease progression is needed to improve patient advice 11 . However, such data are sparse with only two published studies on the effect of pregnancy in 6 and 26 women with AC, 12 , 13 respectively, in addition to case reports 14 , 15 . We aimed to investigate the relation between parity and cardiac structure, function, arrhythmias, and clinical course in female AC patients.…”

Unexpected sudden death in pregnancy – arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report

“…Though ARVC/D is a potentially life threatening disease, it may be managed conservatively even during pregnancy or with beta-blocker or sometime with implantable cardioverter-defibrillator who are at high risk, and one study revealed that approximately 13% and 5% were complicated by ventricular arrhythmias and heart failure, respectively [ 24 ].…”