Pregnancy in arrhythmogenic cardiomyopathy

Abstract

Arrhythmogenic cardiomyopathy (AC) is a rare heart muscle disease with a genetic background and autosomal dominant mode of transmission. The clinical manifestation is characterized by ventricular arrhythmias (VA), heart failure (HF) and the risk of sudden cardiac death (SCD). Pregnancy in young female patients with AC represents a challenging condition for the life and family planning of young affected women. In addition to genetic mechanisms that influence the complex pathophysiology of AC, experimental and clinical data have confirmed the pathogenetic role of strenuous exercise and competitive sports in the early onset and rapid progression of AC symptoms and complications. Pregnancy and exercise share a number of physiological aspects of adaptation. In AC, both result in ventricular volume overload and myocardial stretch. Therefore, pregnancy has been postulated as a potential risk factor for HF, VA, SCD, and pregnancy-related obstetric complications in patients with AC. However, the available evidence on pregnancy in AC does not confirm this hypothesis. In most women with AC, pregnancies are well tolerated, uneventful, and follow a benign course. Pregnancy-related symptoms (VA, syncope, HF) and mortality, as well as obstetric complications, are uncommon in AC patients and range in the order of background populations and cohorts with AC and no pregnancy. The number of completed pregnancies is not associated with an acceleration of AC pathology or an increased risk of VA or HF during pregnancy and follow-up. Accordingly, there is no medical indication to advise against pregnancy in patients with AC. Preconditions include stability of rhythm and hemodynamics at baseline, as well as clinical follow-ups and the availability of multidisciplinary expert consultation during pregnancy and postpartum. Genetic counseling is recommended prior to pregnancy for all couples and their families affected by AC.

Pregnancy in Women with Arrhythmogenic Left Ventricular Cardiomyopathy

“…Moreover, the comparison of ACM patients who experienced a pregnancy with nulliparous affected patients demonstrated no difference in clinical event rates and acceleration of VA and HF, either during pregnancy or after childbirth, both early and long after [9,24,25]. Thus, published data appears to prove that pregnancy does not constitute a driving force for disease progression in ACM [23]. Furthermore, the number of pregnancies appear to have no impact on the outcome and incidence of maternal complications [9].…”

“…Available data seem to indicate that in the majority of women with ACM the course of pregnancy and postpartum are uneventful with regard to pregnancy-related mortality and complications [23]. Depending on the number of patients enrolled in the study, individual risk profile, and duration of follow-up, the rates of maternal death from all causes varied from 0 to 4%.…”

Cardiopulmonary Arrest During Pregnancy: A Review Article

“…Due to autotransfusion, cardiac output jumps by 80% right away after delivery. It then rapidly declines within 10 min and returns to pre-labor levels within 24 h [ 13 ].…”

Considerations for specific cardiomyopathies during pregnancy

“…Patients with ARVC should undergo extensive preconception evaluation and workup including genetic counseling and testing [40]. As in other CMs, antepartum management is aimed at reducing the risk of decompensated HF and ventricular arrhythmias, and includes both medications and cardiac devices [40].…”

“…Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, inherited CM characterized by right ventricular dysfunction and arrhythmias due to replacement of normal myocardium by fibrofatty tissue [39]. Patients with ARVC should undergo extensive preconception evaluation and workup including genetic counseling and testing [40]. As in other CMs, antepartum management is aimed at reducing the risk of decompensated HF and ventricular arrhythmias, and includes both medications and cardiac devices [40].…”