Management of Friedreich Ataxia-Associated Cardiomyopathy in Pregnancy: A Review of the Literature.

ABSTRACT

A major manifestation of Friedreich ataxia (FRDA) is cardiomyopathy, caused by mitochondrial proliferation in myocytes. Because the lifespan for patients with FRDA improves with better treatment modalities, more patients are becoming pregnant, meaning that more medical providers must know how to care for this population. This report provides a review of the literature on multidisciplinary management of pregnant patients with FRDA and cardiomyopathy from preconception through lactation. A cardio-obstetrics team, including cardiology, anesthesiology, and obstetrics, should be involved for this entire period. All patients should be counseled on pregnancy risk using elements of existing stratification systems, and contraception should be discussed, highlighting the safety of intrauterine devices. Electrocardiogram should be obtained at baseline and each trimester, looking for atrial arrhythmias and ST-segment changes, as should transthoracic echocardiogram, with a focus on left ventricular ejection fraction-which is typically normal in FRDA cardiomyopathy-and relative wall thickness and global longitudinal strain-which tend to decrease as cardiomyopathy progresses. Brain natriuretic peptide is also a helpful marker to detect adverse events. If heart failure develops, it should be treated like any other etiology of heart failure during pregnancy. Atrial arrhythmias should be treated with ß blockers or electrical cardioversion and anticoagulation, as necessary. Most patients with FRDA can deliver vaginally, and neuraxial analgesia is recommended during labor because of the risks associated with general anesthesia. Breastfeeding is encouraged, even for those taking cardiac medications.