先天性心脏畸形合并肺静脉狭窄的外科治疗

目的 总结先天性心脏畸形合并肺静脉狭窄(CPVS)的外科治疗经验.方法 CPVS病儿5例,平均年龄(8.5±6.4)岁;平均体重(15.2±6.3)kg.术前狭窄段肺静脉前向血流速度(2.3±1.2)m/s,压差(22.0±6.2)mm Hg.入左房处嵴样狭窄肺静脉6支,均行嵴样狭窄环切除术,其中2支同时用肺静脉开口处内膜"纵切横缝"法成形;肺外管状狭窄肺静脉3支,其中1支为单支狭窄,采用新鲜心包补片扩大法,另2支为同一病例的同侧2支肺静脉,采用相关静脉的单元化术式.结果 平均体外循环(129.2±74.6)min、主动脉阻断(74.2±39.1)min,所有病儿术后血流动力学稳定,肺静脉前向血流速度(0.7±0.4)m/s,压差(2.0±0.6)mm Hg.平均术后住院(10±3)d.随访6个月～3年,结果满意.结论 CPVS往往合并其他先心病,病情重,宜早期诊治.内膜"纵切横缝"法及相邻狭窄肺静脉的"单元化"术式是新的手术方法,技术上是可行的.治疗时可综合应用多种方法,力求彻底解决狭窄并保持其生长潜能;术后早、中期疗效满意.

Surgical treatment for congeaital pulmonary vein stenosis combined with other cardiac malformations

Objective Pulmonnary vein stenosis (PVS) is a rare congenital disease. It leads to progressive pulmonary hyperten-sion and heart failure with a high mortality. PVS may be isolated or asaseiated with other cardiac malformtions. There were few litera- tores regarding surgical treatmenta and the timing for intervention. The aim of this article is to summarize the surgical treatment for PVS combined with other cardiac malformations. Methods Five patients were diagnosed as PVS. The accompanied cardiac malformations were: xtrocordia(n = 1), patent duetus arteriosuss(n = 2), ventricular septal defect(n = 4), atrial septal defect(n = 2), double- chambered right ventricle(n = 1), pulmonary arterial stenosis (n = 1), tricuspid valve insufficiency(n= 2), partial anomalous pulmo- nary venous connection(n = 1), persistent left superior vena cava(n = 1). The mean age was(8.5 4± 6.4) years. The mean body weight was(15.2 ± 6.3) kg. The mean gradient pressure through the stenotie pulmonary veins was(22.0 ± 6.2) mmHg. Nine stenotic pulmonary veins wore found, including 6 cristal stenosises located at venoatrial junetiom and 3 tubular stauosises outside of the lung. The surgical procedures included eristal stenosis ring resection (n=6) and two of them repaired additionally by "longitudinally open and transeversoly suture of the endomembrane" plasty method. Pulmonary veins repair used auto-pericardium (n= 1) and unitization of neighbonring pulmonary veins(n = 1), etc. Remits Cardiopulmonary bypass and aortic cross-clamp time were(129.2 ± 74.6) and (74.24±39.1) rain, respectively. All the petients had a satisfying honmdynmnic aud no death happened. The mean length of hos- pital stay after operation was (10±3) days. Follow-up waa completed in a duration of 6 month - 3 years. There was a trace residual shunt of VSD and PDA and Ⅱ degree auriculo-ventricular block happened in one patient. Residual stenosis was found by color ulltra- sonograph in a cristal stenosis case, whose crista was resectcd simply without "longitudinally open and transversely suture" technique. The mean blood flow rate and gradient pressure through the stenotic pulmonary veins after operation was (0.7±0. 4) m/s and (2.0±0.6) mmHg, respectively. There was no more aggravated residual or new stensis waa found. All the eases were doing well after dis- charged. Conclusion PVS is often complicated by other cardiac abnormalities. Surgery should be done as early as possible. Plasty by "longitudinally open and transversely suture" of the stenosis part and "unitization of neighboring pulmonary veins" are the key tech- niques. For some patients, combined with different methods for PVS is effective. The early outcome of surgery was satisfactory. udinally