Myocardial ischemia due to congenital hypoplastic left coronary cusp in adult

摘要

Chest radiography and resting electrocardiography were normal in a 49-year-old healthy woman with a diastolic murmur noted on a routine check-up. Transthoracic echocardiography showed satisfactory left ventricular systolic function but severe aortic regur-gitation. Transesophageal echocardiography revealed a tricuspid aortic valve with a hypoplastic left coronary cusp (Figure 1). An aortic root angiogram confirmed a hypoplastic left coronary cusp with dynamic obstruction of the left main coronary ostium (Figure 2). In fact, the left coronary ostium could not be cannulated.Selective right coronary angiography indicated a normal right coronary artery with retrograde filling of the left anterior descending coronary artery and circumflex artery up to the proximal left main coronary artery (Figure 3). A thallium scan demonstrated reversible perfusion abnormalities in an extensive area of the left anterior descending coronary artery and circumflex artery territories. Aortic valve replacement was offered as definite treatment for both severe aortic regurgita-tion and myocardial ischemia, but the patient refused as she was asymptomatic.