A major manifestation of Friedreich's ataxia (FRDA) is cardiomyopathy, caused by mitochondrial proliferation in myocytes. As lifespan for people with FRDA improves with better treatment modalities, more people are becoming pregnant, meaning that more medical providers must know how to care for this population. This paper provides a review of the literature on multi-disciplinary management of pregnant patients with FRDA and cardiomyopathy from pre-conception 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 (IUDs). EKG should be obtained at baseline and each trimester, looking for atrial arrhythmias and ST segment changes, as should transthoracic echocardiogram (TTE), with a focus on left ventricular ejection fraction (LVEF) - which is typically normal in FRDA cardiomyopathy - as well as relative wall thickness (RWT), and global longitudinal strain - which tend to decrease as cardiomyopathy progresses. Brain natriuretic peptide (BNP) 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 beta blockers or electrical cardioversion and anticoagulation as necessary. Most patients with FRDA can deliver vaginally, and neuraxial analgesia is recommended during labor due to the risks associated with general anesthesia. Breastfeeding is encouraged, even for those taking cardiac medications.

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