左心发育不良综合征38例

Clinical analysis of 38 infants with hypoplastic left heart syndrome

目的探讨左心发育不良综合征（HLHS）的临床特点及治疗。方法回顾性分析2004年1月至2014年12月在广东省人民医院小儿心脏专科中心救治的HLHS患儿资料，并对其进行随访。结果HLHS患儿共38例，占同期住院先天性心脏病患儿总数（25698例）的0．15％。其中男23例，女15例；发病年龄1h～5个月；入院年龄1d-11个月；入院体质量1．55～14．00kg（平均3．90kg）；住院时间1～88d（平均46d）。24例入院即存在合并症，其中20例并肺炎，2例并脓毒症，3例出现多器官功能障碍，1例并巨细胞病毒感染，4例并缺氧性脑损伤，1例并先天性呼吸道狭窄，1例并肾衰竭。共18例接受手术治疗，治愈8例，16例放弃治疗出院，3例术前放弃治疗死亡，10例术中或术后死亡，1例未手术治疗存活至4岁。18例手术患儿中，I期手术年龄2d-4个月，10例术前需呼吸机辅助通气，8例行急诊手术，10例行改良Blalock—Taussig分流（MBTS）术式，8例行sanoshunt术式；治愈8例中，4例Ⅰ期为MBTS，4例Ⅰ期为sanoshunt；Ⅰ期术后死亡者5例为MBTS术式，3例为sanoshunt术式。术后7例出现重度低心排综合征，1例出现心包填塞，3例出现肺部感染，1例术后应用体外膜肺，1例出现抽搐。结论HLHS属罕见心脏畸形，目前分期姑息手术是HLHS治疗的最主要方法。

Objective To discuss the clinical features and treatment of hypoplastic left heart syndrome （HLHS）. Methods A single - center retrospective study of infants with HLHS who were admitted to the Department of Pediatric Cardiology of Guangdong General Hospital from January 2004 to December 2014 was performed, and the infants were followed - up. Results There were 38 cases of HLHS,including 23 boys and 15 girls, accounting for 0.15 % of the 25 698 in - patients with congenital heart disease during the corresponding period. The ages of onset ranged from 1 hour to 5 months, while the ages at admission ranged from 1 day to 11 months. Weight at admission ranged from 1.55 to 14.00 kg （the mean weight was 3.9 kg） ,and hospital day ranged from 1 to 88 days （the mean hospital day was 46 days）. Twenty- four of the patients had comorbidities at admission,including 20 patients with pneumonia,2 with septicemia,3 developed multi- organ dysfumction, 1 with cytomegalovirus infection ,4 with brain injury caused by hypoxia, 1 with congenital airway constriction, and 1 with renal failure. Eighteen patients received operation with 8 survivals, 16 patients gave up and were discharged,3 died before operation because of give -up, 10 patients died during or after operation. One of the children who did not receive the operation lived until 4 years old. Patients underwent operation at the ages between 2 days and 4 months, and 10 of them needed ventilation before operation ,8 needed emergency operation. Modified Blalock - Taussig shunt（MBTS） was applied in 10 patients while sano - shunt in 8 patients. In the 8 survivals, MBTS and sano - shunt was performed equally in 4 patients in Norwood stage Ⅰ , while 5 received MBTS and 3 sano - shunt who died after stage Ⅰ. After the operation,7 patients developed severe low cardiac output, and 1 had pericardial tamponade,3 suffered from pulmonary infection, 1 had to use extracorporeal membrane oxygenation, and 1 underwent seizure. Conclusions HLHS is a rare malformation of heart. Norwood Procedure Staged palliative surgery is the predominant management option for HLHS.