婴儿主动脉缩窄合并心内畸形的一期矫治

目的总结婴儿主动脉缩窄（CoA）合并心内畸形一期矫治的手术方法和临床经验。方法2001年1月至2006年1月，对28例CoA合并心内畸形患者行一期手术矫治。CoA为导管前型18例，邻近或正对导管处10例；伴主动脉弓发育不良6例，动脉导管未闭22例。合并的心内畸形包括：室间隔缺损16例、室间隔缺损＋房间隔缺损5例、完全性房室间隔缺损3例，完全型大动脉错位伴室间隔缺损（D—TGA／VSD）2例，右心室双出口2例。采用左胸后外侧联合胸骨正中切口（双切口）径路手术12例，胸骨正中切口径路（单一切口）手术16例。采用缩窄段切除端端吻合术20例，Gore—Tex补片扩大成形术4例，左锁骨下动脉翻转扩大成形术4例。结果全组无手术死亡，术后呼吸机辅助呼吸时间7h～13d，住ICU时间3～18d。因术后心脏扩大延迟48～72h关胸4例，术后发生急性肾功能衰竭行腹膜透析3例，吸入一氧化氮（NO）治疗重度肺动脉高压3例。术后下肢收缩压高于上肢10～20mmHg18例（64．3％），收缩压上、下肢相差不大8例（28．6％），上肢分别高于下肢15mmHg和20mmHg2例（7．1％）。所有患者均得到随访，随访时间3～50个月，1例D—TGA／VSD患者因重度三尖瓣反流、肺部严重感染和呼吸功能衰竭于术后3个月死亡；其余27例患者恢复顺利。心脏超声心动图提示：1例采用端端吻合法、1例采用Gore～Tex补片扩大成形术的患者在CoA纠治处仍存在压差，分别为25mmHg、28mmHg，均未再次手术。结论一期手术矫治CoA合并心内畸形可以缩短疗程，有利于患者术后心、肺功能的恢复，避免二次手术的痛苦，降低治疗费用。可采用单一胸骨正中切口或胸骨正中联合左胸后外侧切口完成手术。

One-stage Repair of Coarctation of Aorta and Associated Intracardiac Malformation in Infants

Objective To summarize one-stage repair of coarctation of the aorta(CoA) with intracardiac malformation in infants. Methods From January 2001 to January 2006, 28 patients with CoA and associated intracardiac malformation underwent one-stage repair. There were 18 patients with preductal and another 10 patients with juxtaductal CoA. Six patients were accompanied by diffuse hypoplasia of aortic arch and 22 patients were associated with patent ductus arteriosus (PDA). Associated cardiac malformation included ventricular septal defect in 16 patients, ventricular septal defect and atrial septal defect in 5 patients, atrioventricular canal defect in 3 patients, transposition of great artery and ventricular septal defect in 2 patients and double outlet right ventricle in 2 patients. Left posterolateral thoracotomy combined with median sternotomy were used in 12 patients and single median sternotomy was used in 16 patients. End to end anastomosis of aorta with removal of the coarctated segment were performed in 20 patients, Gore-Tex patch graft aortoplasty were performed in 4 patients and aortoplasty with left subclavain flap were performed in 4 patients. Results There was no death.The lasting time of intubation was 7h to 13d, and intensive care unit (ICU) staying time was 3 to 18d. Four patients received delayed sternal closure in 48 to 72h after surgery because of cardiac dilatation. Three patients received peritoneal dialysis for acute renal failure following cardiac surgery. Three patients received inhalation of nitric oxide for treatment of postoperative pulmonary hypertension. The systolic pressure difference between lower limb and upper limb measured after operation was 10 to 20mmHg in 18 patients.There were no obvious systolic pressure difference between arm and leg in 8 patients.The systolic pressure of upper limb was higher than that of lower limb in 2 patients, and the pressure difference was 15mmHg and 20mmHg. All patients were followed up from 3 to 50 months. They were all survival except one died of severe tricuspid regurgitation, pulmonary infection and respiratory failure 3 months after surgery which diagnosis was transposition of great artery and ventricular septal defect. The results of other 27 patients were satisfactory. Re-coarctation occurred in 1 patient with end to end anastomosis and 1 patient with Gore-Tex patch graft aortoplasty. The pressure difference through the measurement of echocardiography was 25mmHg and 28mmHg. No reoperation were undergone. Conclusion CoA with intracardiac malformation can be repaired by single median sternotomy or left posterolateral thoracotomy combined with median sternotomy in one-stage. This operative approach is beneficial, not only with shorten period of therapy and less operative cost, but also advantageous for the recovery of cardiac and pulmonary function.