Wu J R, Huang T Y, Wang C T, Lin Y T
Department of Pediatrics, Kaohsiung Medical College, Taiwan, Republic of China.
Gaoxiong Yi Xue Ke Xue Za Zhi. 1992 Oct;8(10):562-8.
Congenital absence of the pulmonary valve is an uncommon combination of anomalies. Recently, we encountered two such cases, one associated with tetralogy of Fallot (case 1), the other with ventricular septal defect (case 2). Clinically, our cases had minimum symptoms. The congenital cardiac anomalies were diagnosed with physical examination, chest x-ray film, echocardiography, and confirmed with cineangiography. These procedures disclosed a rudimentary pulmonary valve, pulmonary regurgitation and aneurysmal dilatation of the main and right pulmonary artery. In case 2, the right lower pulmonary artery occlusion and left hydronephrosis were also demonstrated by magnetic resonance imaging. The two cases underwent surgical repair to correct the tetralogy of Fallot and the ventricular septal defect respectively. Our experience in diagnosing these cardiac anomalies is reported.
先天性肺动脉瓣缺如是一种少见的联合畸形。最近，我们遇到了两例这样的病例，一例合并法洛四联症（病例1），另一例合并室间隔缺损（病例2）。临床上，我们的病例症状轻微。通过体格检查、胸部X线片、超声心动图诊断先天性心脏畸形，并通过心血管造影术得以证实。这些检查显示肺动脉瓣发育不全、肺动脉反流以及主肺动脉和右肺动脉瘤样扩张。在病例2中，磁共振成像还显示右下肺动脉闭塞和左肾积水。两例分别接受了手术修复，以纠正法洛四联症和室间隔缺损。本文报告了我们诊断这些心脏畸形的经验。
