先天性心脏畸形合并肺静脉狭窄的外科治疗

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

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）mmHg。人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～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）ramHg。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。

Objective Pulmonary vein stenosis （PVS） is a rare congenital disease. It leads to progressive pulmonary hypertension and heart failure with a high mortality. PVS may be isolated or associated with other cardiac malformations. There were few literatares regarding surgical treatment 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： xtrocardia（n = 1）, patent ductus arteriosus（n = 2）, ventricular septal defect（n =4）, atrial septal defect（n= 2）, donblechambered right ventricle（n = 1 ）, pulmonary arterial stenesis （n = 1 ）, tricuspid valve insufficiency（n = 2 ）, partial anomalous pulmonary venous connection（n= 1 ）, persistent left superior vena cava（n = 1 ）. The mean age was（8.5 ±6.4） years. The mean body weight was（ 15.2±6.3） kg. The mean gradient pressure through the stenotic pulmonary veins was（ 22.0 ±6.2 ） mmHg. Nine stenotic pulmonary veins were found, including 6 cristal stenosises located at venoatrial junctions and 3 tubular stenesises outside of the lung. The surgical procedures included cristal stenesis ring resection （n = 6） and two of them repaired additionally by ＂longitudinally open and transversely suture of the endomembrane＂ plasty method. Pulmonary veins repair used auto-pericardium （n = 1 ） and unitization of neighboufing pulmonary veins（n = 1）, etc. Results Cardiopulmonary bypass and aortic cross-clamp time were（ 129.2±74.6） rain and （74.2 ±39.1 ） rain, respectively. All the patients had a satisfying homedynamie and no death happened. The mean length of hospital stay after operation was （ 10 ± 3） days. Fonow-up was completed in a duration of 6 month - 3 years. There was a trace residual shunt of VSD and PDA and 1/ degree anriculo-ventricular block happened in one patient. Residual stenosis was found by color ultrasonograph in a cristal stenesis case, whose cfista was resected simply without ＂longitudinally open and transversely suture＂ technique. The mean blood flow rate and gradient pressure through the stenotic pttlinonary 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 stenosis was found. All the cases were doing well after discharged. Conclusion PVS is often complicated by other cardiac abnormalities. Surgery shotdd be done as early as possible. Plasty by ＂longitudinally open and transversely suture＂ of the stenesis part and ＂unitization of neighboring pulmonary veins＂ are the key techniques. For some patients, combined with different methods for PVS is effective. The early outcome of surgery was satisfactory.