婴幼儿室间隔缺损并肺动脉高压的外科治疗

Surgical treatment for ventricular septal defect infants with pulmonary hypertension

目的评估婴幼儿VSD并肺动脉高压（PH）的纠治方法及效果。方法收集河北医科大学第一医院心外科VSD并PH婴幼儿215例。男109例，女106例；月龄1～36个月；体质量1．5～10．0kg。膜周型VSD156例，嵴内型18例，干下型41例。VSD直径0．8～2．1cm。并继发性ASD16例，卵圆孔未闭8例，PDA15例，并PDA、ASD14例，并PDA和主动脉瓣下隔膜3例，肺动脉瓣狭窄14例，右心室流出道狭窄33例，二尖瓣关闭不全22例，三尖瓣关闭不全42例，永存左上腔静脉12例。肺动脉压力4．66～11．31kPa。患儿均在全麻体外循环下行一期根治术。结果全组手术顺利。术后死亡3例。术后并发症：15例出现短暂低心排出量综合征，其中死亡2例；10例右束支传导阻滞；6例室上性心动过速；5例短暂Ⅲ度房室传导阻滞；5例术后发生PH危象，其中死亡l例；3例气胸；2例肺不张。随访期均行X线胸片、ECG、心脏彩超检查，术后心功能明显改善。结论婴幼儿VSD并PH病情发展快，易发生低心排出量综合征和低氧血症，早期手术效果良好，治疗的关键在于加强心肌保护，提高手术操作技术，缩短手术时间；术后加强监护，及时处理各种术后并发症。

Objective To evaluate the efficiency of urgent surgical correction for ventricular septaJ defect （VSD） infants with pulmonary hypertension（PH）. Methods Two hundred and fifteen infants （109 male, 106 female） with VSD and PH received surgical correction from Department of Cardiac Surgery, Center of Congenital Heart Disease, the First Hospital of Hebel Medical University. The age of patients ranged from 1 to 36 months on admission, the body mass ranged from 1.5 to 10.0 kg. Among all the cases, the perimembranous type was found in 156 children, the conal septal defect in 18 cases, and subpulmonary VSD in 41 cases. The diameter of defect was 0.8 -2.1 em. Sixteen patients were associated with atrial septal defect （ASD）, 8 cases with patent foramen ovale, 15 cases with ductus arteriosus （ PDA）, 14 cases with ASD and PDA, and 3 eases with PDA and coarctation of the aorta, 14 cases with pulmonary valve stenosis,33 cases with stricture of right ventricular outflow tract, 22 cases with incompetence of mitral valve directly ligation,42 eases with incompetence of tritral valve directly ligation, and the rest 12 cases with persistent left superior vena cava. The pressure of pulmonary artery was 4.66 - 11.31 kPa. All patients received cardiopulmonary bypass under hypothermic anaesthesia through extracorporeal blood circulation. Results Urgent operations were performed in all pa- tients, but 3 patients died after surgery. Postoperative complications included low cardiac output syndrome（ 15 cases） , a- mong whom 2 cases died ; right bundle-branch block （ 10 cases ） , supraventricular tachycardia （ 6 cases ） , transient atrio- ventricular block of grade Ⅲ （5 cases） ,inhalation of nitric oxide was given to 5 cases for the management of significant postoperative PH, among whom 1 case died during surgery, 3 cases were pneumothorax ,2 cases were atelectasis. Chest radiography, echocardiography, and ECG were performed during this period. As a result, most children had good cardiac functions. Conclusions Low cardiac output syndrome and hypoxemia present easily in infants and children with ven- tricular septal defect with pulmonary hypertension. The key step during operation is to protect the myocardium skillfully, and extracorporeal circulation lesion should be alleviated ; the duration of operation should be shortened; after operation, extensive supervision must be undertaken and various complications must be managed promptly.