A 69-year-old man, with a history of moderate aortic regurgitation, was admitted to our hospital with symptoms of congestive heart failure. He had undergone implantation of a permanent DDD-pacemaker 2 years previously. On physical examination, his blood pressure was 150/50 mmHg and cardiac auscultation revealed a 3/6 diastolic murmur along the left sternal border. Transthoracic echocardiography showed a left ventricular hypertrophy with an enlarged cavity (left ventricular end-diastolic diameter, 70 mm), a normal left ventricular ejection fraction (55%), a pulmonary artery systolic pressure of 50 mmHg and a central severe aortic regurgitation. Sigmoid aortic valves were mildly thickened, without the usual Y-aspect closure, leading to suspicion of an abnormality of the aortic valve.
Transoesophageal echocardiography showed a quadricuspid aortic valve with three equal thin cusps and one minor, which presented as an ‘X-shaped’ commissural aspect in diastole. Central coaptation defect due to an incomplete juxtaposition of the four cusps resulted in a central aortic regurgitation ( Fig. 1 ).
