Abstract
An 18-year-old boy presented with difficulty in breathing since birth. He had ejection systolic murmur heard loudest in the right second intercostal space radiating to the right carotids. On blood examination, he had severe pancytopenia. Electrocardiogram revealed left ventricular hypertrophy with a prominent Q-wave. On ultrasonography, it was revealed that the patient had splenomegaly with multiple collaterals. Echocardiography showed a tricuspid aortic valve with three cusps with a subaortic membrane. Concomitant splenectomy and subaortic membrane excision on cardiopulmonary bypass under general anesthesia was done. His platelet counts recovered soon after splenectomy. The following core competencies are addressed in this article: Patient care and procedural skills, Medical knowledge, and Systems-based practice.
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