A 20-year-old male with Friedreich ataxia (FA) presented with dyspnea and palpitations over the prior 2 days. Atrial fibrillation with rapid ventricular response and inverted T waves in leads III, aVF, V5, V6 were found on the electrocardiogram. The transthoracic echocardiogram, with the patient being in atrial fibrillation, showed concentric hypertrophy and left ventricular ejection fraction of approximately 50%. Surprisingly, an incomplete membrane into the right atrium was noted. Cardiac magnetic resonance imaging was performed and documented the presence of large Eustachian valve in the presence of cardiomyopathy.

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