Abstract
Background
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is a mitochondrial DNA disorder in which medications that impair mitochondrial function may worsen disease manifestations. Management of heart failure and arrhythmias in this population is therefore challenging.Case summary
A 54-year-old woman with MELAS presented with acute hypoxemic respiratory failure, atrial fibrillation with rapid ventricular response, and newly reduced left ventricular ejection fraction. Transesophageal echocardiography demonstrated a left atrial appendage thrombus, precluding immediate cardioversion. In collaboration with medical genetics, rate-control and guideline-directed heart failure therapies were cautiously initiated and titrated. Following anticoagulation, she underwent successful cardioversion and subsequently demonstrated recovery of left ventricular systolic function.Discussion
This case illustrates the individualized use of conventional cardiovascular therapies in a patient with MELAS and probable arrhythmia-mediated cardiomyopathy. Multidisciplinary assessment, cautious medication titration, and close monitoring allowed effective treatment while minimizing potential mitochondrial toxicity.Take-home messages
Conventional heart failure and atrial fibrillation therapies may be used in selected patients with MELAS after careful assessment of clinical benefit and potential mitochondrial risk. Multidisciplinary collaboration, cautious dose titration, and close monitoring are essential during treatment.