Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve

摘要: You are contacted by a concerned 34-year-old airline pilot with leaky bicuspid aortic valve recently diagnosed an echocardiogram that had been requested his new employer as part of routine medical assessment. He claims he is perfectly healthy but at risk losing job over condition. disease hereditary and children may also be risk. The patient, attorney, insurance company have statement from you to the cause, possible complications, treatment options associated congenital malformation valve. The (BAV) most common cardiac malformation, occurring in 1% 2% population. majority BAV patients develop complications requiring treatment. Physicians often challenged when asked provide evidence-based advice about because pathogenesis pathophysiology this not well understood. BAVs result abnormal cusp formation during valvulogenesis. Adjacent cusps fuse form single aberrant cusp, larger than its counterpart yet smaller 2 normal combined. BAVs likely complex developmental process, simply fusion cusps. In fact, malformations reflect phenotypic continuum unicuspid valves (severe form), (moderate tricuspid (normal), rare quadricuspid forms1 (Figure 1). Figure 1. A, Normal valve. (arrowheads) 3 commissures (arrows) clearly seen. Three sinuses Valsalva coapt normally give functionally B, Congenitally valve, one (asterisk) other. show thickening due fibrosis. Two One raphe (arrowhead) …