A case of bicuspid aortic valve with two raphes

A 60-year-old woman was referred to our hospital for exertional shortness of breath. She was made aware of a heart murmur as a high school student. She had exertional dyspnea and chest pain. Her symptoms were getting worse over the previous year. On admission, her blood pressure was 160/54 mmHg, heart rate was 72 bpm, and grade 3/6 regurgitant murmur over the precordium was present. She was diagnosed with severe aortic regurgitation by transthoracic echocardiography and the other clinical examination. Transthoracic echocardiography showed severe aortic regurgitation with normal left ventricular systolic function. Two raphes of the aortic valve were not diagnosed by transthoracic echocardiography because of poor quality echo images. Two-dimensional transesophageal echocardiograms showed a bicuspid aortic valve (BAV) with fused cusps between the two components of the right and noncoronary cusps (Fig. 1). Furthermore there were two raphes on the fused cusps. Real-time three-dimensional echocardiograms showed two commissures and two raphes on the fused area in systole and diastole (Fig. 2a, b). The perioperative findings were exactly the same as the information obtained by three-dimensional transesophageal echocardiography (Fig. 2c). Discussion