成人先天性主动脉闭锁的影像学检查

AORTIC ATRESIA IN ADULT

本文报告一例经较全面的影像学检查明确诊断的先天性主动脉闭锁病例,属Roberts分型中的IB型。讨论了X线平片、心血管造影、二维超声心动图和磁共振成像(MRI)等对本畸形的诊断价值和限度。

Congenital aortic atresia is a rare congenital malformation of the cardiovascular system with very high early mortality. The basic pathology includes absence of aortic valve and atretic aortic root with hypoplastic ascending aorta. 95% of the cases have intact ventricular septum and hypoplastic left heart--so called 'Hypoplastic Left Heart Syndrome', 5% of the cases have ventricular septal defect and basically normal left heart. An adult case, type IIB of Rorbert's classification, the diagnosis established by various procedures of imaging at the age of 20 was reported in this article with a pertinent discussion on the advantages and limitations of radiography, angiography, 2D-ultrasound and magnetic resonance imaging etc for the diagnosis of this disease entity.