Mitral Regurgitation in Obstructive Hypertrophic Cardiomyopathy: Systolic Anterior Motion or a More Unusual Cause?

Abstract

A 52-YEAR-OLD, 100-kg, 191-cm transitioned woman, receiving hormonal therapy, with a history of hypertension, hyperlipidemia, tobacco abuse, and known coronary artery disease, presented to a community hospital with recurrent chest pain and dyspnea. An electrocardiogram demonstrated new ST-segment elevation consistent with an anterior wall myocardial infarction. The patient was taken to the cardiac catheterization laboratory where drug-eluting stents were implanted in the left anterior descending and diagonal coronary arteries. Her recovery was complicated by cardiogenic shock, refractory pulmonary edema, and acute respiratory failure that was unresponsive to vasoactive medications and mechanical ventilation. She was transferred to the authors’ institution for definitive care. On admission, the physical examination was notable for sinus tachycardia (110 beats/min) and a grade III of VI holosystolic murmur heard best at the left sternal border. Leukocytosis was present (19.4 103/µL), but blood cultures were negative. Transesophageal echocardiography (TEE) was performed as part of the diagnostic evaluation and revealed the following images (Fig. 1, 2, and 3; Videos 1, 2, and 3). What is the diagnosis? Fig 2Midesophageal five-chamber transesophageal echocardiography view, focus on mitral valve. Single white arrow highlights contact between the anterior mitral leaflet and hypertrophied septum. Double white arrows highlight the perforation in the anterior mitral leaflet. View Large Image Figure Viewer Download Hi-res image Fig 3Midesophageal five-chamber transesophageal echocardiography color Doppler view, focus on mitral valve. Single white arrow highlights regurgitant jet from malcoaptation between mitral leaflets. Double white arrows highlight regurgitant jet from perforation. View Large Image Figure Viewer Download Hi-res image