Kim W S, Yeon K M, Kim I, Han M C, Chi J G
Department of Radiology, Seoul National University, Children's Hospital, Korea.
Pediatr Radiol. 1993;23(1):6-11. doi: 10.1007/BF02020210.
Congenital obstruction of the pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veins and pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which were accompanied by other cardiac anomalies. Right pulmonary veins were involved in all seven cases; one case was bilateral. Pulmonary veins were occluded totally in five and partially in three lungs. Diagnostic pulmonary catheterization and angiography were performed. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural changes, which were quite specific, whereas pulmonary venous dilatation was the dominant finding in partial obstruction cases. Pulmonary perfusion scan (n = 3) showed total perfusion defects in the cases with total occlusion of pulmonary veins. Magnetic resonance (MR) imaging (n = 2) demonstrated totally occluded pulmonary veins at the venoatrial junction in two lungs and membranous focal obstruction in one lung. Two children underwent pneumonectomy and had the diagnosis histologically confirmed. Although catheterization and angiography are essential for the diagnosis of pulmonary vein obstruction, MR imaging is a useful adjunct.
无异常引流的先天性肺静脉梗阻可导致长期的肺淤血和肺动脉高压，其可能包括单个肺静脉狭窄和肺静脉闭锁。我们回顾了7例肺静脉梗阻病例，其中5例伴有其他心脏异常。所有7例均累及右肺静脉；1例为双侧受累。5例肺静脉完全闭塞，3例部分闭塞。均进行了诊断性心导管检查和血管造影。完全闭塞病例的胸部X线片显示肺容积减小、肺水肿特征、间质病变和胸膜改变，这些表现非常具有特异性，而部分梗阻病例的主要表现为肺静脉扩张。3例肺灌注扫描显示肺静脉完全闭塞的病例存在完全灌注缺损。2例磁共振成像显示2个肺的肺静脉在静脉心房交界处完全闭塞，1个肺存在膜性局灶性梗阻。2例患儿接受了肺切除术，组织学诊断得以证实。尽管心导管检查和血管造影对肺静脉梗阻的诊断至关重要，但磁共振成像也是一种有用的辅助检查方法。
