24例镰刀综合征心血管造影征象分析

笔者分析了２４例镰刀综合征心肺血管病变的造影征象及其病理生理意义，并与手术（１４例）和尸解（４例）资料对照。２４例心血管造影示全部或部分右肺静脉异常引流，分别为１７例和７例。异常引流静脉近端局限性狭窄９例，其中２例异常和正常引流静脉间有侧支吻合，右下肺异常体动脉侧支供血１８例。并存各种心脏病变者１８例，马蹄肺６例，右下肺肺隔离症３例，下腔静脉闭锁和狭窄分别为２和１例。右下肺异常体动脉侧支和心脏病变是患者严重症状和肺动脉高压的主要因素。除肺隔离症外，经导管栓塞异常体动脉侧支可使用者明显受益。异常引流静脉狭窄具有限制血液分流的作用。

Angiocardiographic Findings in 24 Patients with Scimitar Syndrome

The angiocardiographic manifestations of pulmonary and cardiovascular lesions in 24 patients with Scimitar syndrome were reviewed and their pathologic-physiologic features explored.14 cases were operated and autopsy done in 4 of the operated cases. Correlation of angiocard iographic and surgical-pathologic findings was performed as well. Total and partial anomalous venous drainage of right iung was noted in 17 and 7 respectively. A localized stenosis at proximal anomalous vein was shown in 9,and an anastomosis between anomalous and normal draining veins in 2 of them. The lower lobe of the right lung was supplied in part by a systemic artery arising from the abdominal aorta in 18.A wide variety of associated cardiac malformations occurred in 18,horseshoe lung anomaly in 6,and pul-monary sequestration in right lung in 3.Atresia and severe stenosis of inferior vena cava were present in 2 and 1 respectively. Anomalous syStemic arterial supply and associated cardiac anomalies play a signifi-cant role for the severe symptoniatology and pulmonary hypertension. The embolization of systemic arte-rial supply was beneficial, except in patients with bronchial sequestration. The localized stenosis of anomalous vein may limit the size of left-to-right shunting from anomalous pulmonary venous con-nection.