Process of Care and a Practical Toolkit for Evaluating and Managing Arrhythmic Risk in the Cardiogenetic Pregnant Patient

Abstract

Patients with inherited arrhythmia syndromes (IASs) and inherited cardiomyopathies (ICs) are periodically encountered in both general and specialist practices. These syndromes include long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, early repolarisation syndrome, and hypertrophic and arrhythmogenic cardiomyopathies. In general, the presence of an IAS or IC is not a contraindication to pregnancy, but does require additional expertise and patient engagement. In this review, we summarise the various pregnancy-related considerations in patients with IAS and IC, including the impact of physiologic/hemodynamic changes on heart failure progression or arrhythmia propensity, maternal and fetal pregnancy risk stratification, prenatal genetic testing, and the specialised care and monitoring required through pregnancy, labour, and delivery and into the postpartum period. Management of patients with IASs and IC during pregnancy and the postpartum period requires collaboration between patient and provider, with a shared understanding of the general safety and potential risks during the pregnancy and postpartum periods. Patients should be aware of the safety of various medications throughout pregnancy, and those with implantable cardioverter-defibrillators should be managed according to device guidelines. A peripartum care and delivery plan should be established, with multidisciplinary input from various specialists including obstetrics, cardiac obstetrics, and inherited arrhythmia specialists wherever appropriate.