功能性单心室的外科治疗及近期疗效

Surgical treatment of the patients with functional single ventricle

目的总结功能性单心室的外科治疗经验。方法回顾性分析我科2003年12月至2010年7月59例功能性单心室患儿，对其手术时机，手术方式及手术效果进行分析，并用Logistic回归分析Glenn手术的高危因素。结果死亡8例（病死率13．56％），其中4例为Glenn术后死于低心排和多器官功能衰竭，2例为Banding术后死于低心排，1例为TCPC术后死于多器官功能衰竭，1例为B-T分流术后死于低心排。Glenn术后的主要并发症为上腔静脉梗阻综合症和低心排，Fontan术后的主要并发症是房性心律失常和胸腔积液。Logistic回归分析得出房室瓣反流是Glenn术后患儿死亡的高危因素。结论功能性单心室心内畸形复杂，需根据肺血流的情况及早进行姑息手术，选择合适的术式。改良Fontan是进行生理矫正的理想术式，保留肺动脉前向血流的Glenn手术是保证患儿顺利进行Fontan术的有效方法，但需根据肺动脉狭窄的程度进行处理，避免上腔静脉综合症的发生。

Objective To retrospectively analyze the outcome of surgical treatment of patients with functional single ventricle. Methods Between December 2003 and July 2010, 59 patients with functional single ventricle underwent corrective surgeries, and were followed up at this center. Their median age was 3. 0 months old at pulmonary artery band surgery （PAB）, 13. 5 months at bidirectional Glenn procedure （BDG）, and 11 years old at Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, age at surgery, and type of the surgical procedure were retrospectively analyzed. Logistic regression analysis was also performed to determine the risk factors associated with Glenn. Results Duration of mechanical ventilation was 30. 4 ± 19. 7 hours. Length of stay in ICU was 4. 3 ± 1.6 days. Length of hospital stay was 34. 8 ± 15. 9 d. SpO2 increased from 75 ±6. 97% to 88 ± 6. 28% after operation. Exercise tolerance was also improved remarkably. Eight deaths were noted after surgery （13. 56 %）. The leading complications after Glenn surgery were superior vena cava syndrome and low cardiac output syndrome. Complications after Fontan surgery were atrial tachycardia and pleural effusions. Logistic regression analysis suggested atrioventricular valve regurgitation was an important risk factor for the death of the patients had Glenn surgery. Conclusions The clinical outcome of surgical treatment of patients with functional single ventricle is satisfied. The surgical strategy should be made according to anatomic abnormality of the functional single ventricle and pulmonary circulation. Modified Fontan operations can become an alternative therapy for patients who are not suitable for double ventricle correction. Glenn with antegrade pulmonary blood flow may be a temporary palliation prior to Fontan surgery, but the pulmonary blood flow should be controlled to avoid the occurrence of superior vena cava syndrome.