Klinik für Innere Medizin / Kardiologie, Niels-Stensen-Kliniken, Marienhospital Osnabrück, Herzzentrum Osnabrück/Bad Rothenfelde, Bischofsstr. 1, 49074, Osnabrück, Germany.
Herzschrittmacherther Elektrophysiol. 2021 Jun;32(2):186-198. doi: 10.1007/s00399-021-00770-7. Epub 2021 May 25.
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.
致心律失常性右室心肌病（AC）是一种罕见的遗传性心肌疾病，具有常染色体显性遗传方式。临床表现为室性心律失常（VA）、心力衰竭（HF）和心源性猝死（SCD）风险。患有 AC 的年轻女性怀孕对年轻受影响女性的生活和计划生育构成挑战。除了影响 AC 复杂病理生理的遗传机制外，实验和临床数据还证实了剧烈运动和竞技运动在 AC 症状和并发症的早期发生和快速进展中的致病作用。怀孕和运动具有许多适应的生理方面。在 AC 中，两者都导致心室容量超负荷和心肌拉伸。因此，怀孕被认为是 AC 患者 HF、VA、SCD 和与妊娠相关产科并发症的潜在危险因素。然而，AC 中关于怀孕的现有证据并不能证实这一假设。在大多数患有 AC 的女性中，怀孕都能耐受良好，无并发症，且病情良好。与怀孕相关的症状（VA、晕厥、HF）和死亡率，以及产科并发症在 AC 患者中并不常见，其范围与 AC 患者和无怀孕的背景人群和队列相当。已完成的妊娠次数与 AC 病理的加速或怀孕期间和随访期间 VA 或 HF 的风险增加无关。因此，AC 患者怀孕没有医学禁忌。前提条件包括基线时心律和血液动力学稳定，以及在怀孕期间和产后提供多学科专家咨询和临床随访。建议所有受 AC 影响的夫妇及其家庭在怀孕前进行遗传咨询。
