A rare pediatric cardiac anomaly: Quadricuspid aortic valve with aortic regurgitation
A 9-year-old male was referred to the cardiology clinic for evaluation of an asymptomatic diastolic heart murmur. On physical examination, a 2/4 early, medium-frequency, diastolic murmur was audible at the right mid-sternal border. The remainder of his physical examination was unremarkable and an electrocardiogram was normal. Transthoracic echocardiography showed a type B quadricuspid aortic valve (QAV) (three cusps of equal size and one smaller cusp) associated with mild aortic regurgitation by color Doppler imaging, likely from a coaptation defect [ and ].[] The left ventricular end-diastolic dimension measured by M-mode was normal (4.58 cm, z-score + 0.3) with a shortening fraction of 31%. The remainder of the cardiac anatomy was normal, including the origins of the coronary arteries.\nTwo years later, the patient remained asymptomatic and the echocardiography showed stable, mild aortic regurgitation [], now associated with mild left ventricular dilation []. By M-mode the left ventricular end-diastolic dimension measured 5.82 cm (z-score + 2.8), the end-systolic dimension was 3.7 cm (z-score + 1.9), and the shortening fraction was 35%.