重度肺动脉瓣狭窄的外科治疗

Surgical treatment for severe pulmonary valve stenosis

目的探讨先天性心脏病重度肺动脉瓣狭窄外科治疗的手术效果。方法选择2006年10月至2013年1月共纠治重度肺动脉瓣狭窄患儿74例。男51例，女23例；年龄2个月～13岁（中位年龄26个月）；体质量5～47kg（中位数16b）。合并其他心脏畸形，其中动脉导管未闭（PDA）20例，卵圆孔未闭（PFO）22例，房间隔缺损（ASD）32例，室间隔缺损（VSD）13例，右心室双腔心4例。患儿均在全身麻醉体外循环下行一期根治术，46例行右心室流出道至肺动脉跨瓣补片加宽，28例经肺动脉切口行肺动脉瓣膜交界切开术。结果体外循环时间40～100min（中位数72min），主动脉阻断时间13．～79min（中位数43min）。术后呼吸机辅助呼吸4～128h（中位数15h），重症监护病房（ICU）滞留4～18d（中位数5d）。全组手术顺利，无手术死亡病例。围术期并发症：1例术中发生室颤，经电除颤后转为正常，术后4例出现低心排综合征，8例出现顽固性低氧血症，2例气胸，2例胸腔积液，1例急性肾衰竭，1例肺不张。随访期患儿均行x线胸片、心电图和心脏彩超检查，术后心功能明显改善。结论重度肺动脉瓣狭窄病情发展快，易发生低心排综合征和低氧血症，早期诊断，早期手术，效果良好。手术的关键在于应用自体心包或带瓣心包补片合理加宽肺动脉，充分解除右心室流出道梗阻；同时提高术后监护水平，及时处理各种术后并发症。

Objective To evaluate the effectiveness of urgent surgical procedures for severe pulmonary valve stenosis in infants and children. Methods Seventy-four （51 male, 23 female ） children with severe pulmonary valve stenosis who received surgical correction were collected from Oct. 2006 to Jan. 2013. The age of patients ranged from 2 months to 13 years（ median age was 26 months） , and the body weight was 5 -47 kg （median weight was 16 kg）. Pa- tients had other cardiac deformities, and among them 20 patients were associated with the patent ductus arteriosus （PDA） ,22 cases with patent foramen ovale （PFO） ,32 cases with atrial septal defect （ASD）, 13 cases with ventricular septal defect （VSD） , and 4 cases with double-chambered right ventricle. Radical corrections were performed in all pa- tients under hypothermic general anaesthesia with cardiopulmonary bypass. There were 46 patients undergoing transan- nular patching with autologous pericardial flake and 28 patients undergoing transarterial pulmonary valvotomy. Results Urgent operations were performed in all patients and no death occurred during surgery. Median bypass time was 72 minutes（ranging from 40 to 100 minutes） and median aortic cross-clamp time was 43 minutes（ranging from 13 to 79 minutes）. The time for the use of respirator was 4 - 128 h （ median time was 15 h） ,and ICU stay lasted 4 - 18 days （ median time was 5 days）. Perioperative complications ： included 1 case of ventricular fibrillation during operation, ＇after electrical shock the patient returned to normal;postoperative complications included low cardiac output syndrome （4 ca- ses ） , refractory hypoxemia （ 8 cases ） , acute renal failure （ 1 case ） , pneumothorax （ 2 cases ） , hydrothorax （ 2 cases ） , ate- lectasis（ 1 case）. All patients received chest radiography, electrocardiogram and echocardiography during hospital stay. As a result, postoperative cardiac function was improved significantly. Conclusions Low cardiac output syndrome and hypoxemia aften exist in severe pulmonary stenosis. If severe pulmonary stenosis is diagnosed and operated early, the prognosis is good. The key to this kind of surgery is the application of autologous pericardium or disc pericardium patc- hing for widening pulmonary artery appropriately, which fully rescind the right ventricular outflow tract obstruction. At the same time, the level of postoperative monitoring should be improved, and postoperative complications must be han- dled timely.