Schenone Aldo L, Cohen Aaron, Pettersson Gosta, Majdalany David
Internal Medicine Department, Medicine Institute at Cleveland Clinic, Cleveland, OH, USA
Internal Medicine Department, Medicine Institute at Cleveland Clinic, Cleveland, OH, USA.
World J Pediatr Congenit Heart Surg. 2016 May;7(3):389-93. doi: 10.1177/2150135115592034. Epub 2015 Dec 23.
Bicuspid aortic valve (BAV) is the most common form of congenital heart disease, with 20% of asymptomatic adults with BAV presenting with significant valve insufficiency. Yet, limited data exist regarding surgical indications and outcomes when BAV is accompanied by left ventricular dilation, systolic dysfunction, or left ventricle non-compaction (LVNC) syndrome. We present a case of dilated cardiomyopathy due to severe BAV regurgitation and partial LVNC syndrome and the decision to undergo aortic valve replacement. Our patient represents the most extreme documented case of regurgitant BAV with dilated, dysfunctional, and partially non-compacted left ventricle. Yet, surgical intervention provided improvement in systolic performance and ventricular dimensions.
二叶式主动脉瓣（BAV）是先天性心脏病最常见的形式，20%无症状的BAV成年患者存在明显的瓣膜关闭不全。然而，关于BAV伴有左心室扩张、收缩功能障碍或左心室心肌致密化不全（LVNC）综合征时的手术指征和结果的数据有限。我们报告一例因严重BAV反流和部分LVNC综合征导致的扩张型心肌病病例以及行主动脉瓣置换术的决策。我们的患者是有记录的反流性BAV伴左心室扩张、功能障碍和部分心肌致密化不全的最极端病例。然而，手术干预改善了收缩功能和心室大小。
