2018 年第 5 期 第 13 卷

少年儿童马方综合征主动脉根部瘤合并二尖瓣病变的外科治疗

Surgical treatment of aortic root aneurysm complicated with mitral valve disease in children with Marfan′s syndrome

作者：里程楠葛翼鹏夏瑀杨祎郭世超肖付诚刘永民朱俊明孙立忠

英文作者：

单位：100029首都医科大学附属北京安贞医院心脏外科

英文单位：

关键词：马方综合征；主动脉根部瘤；二尖瓣关闭不全

英文关键词：

• 摘要：

• 目的    探讨少年儿童马方综合征主动脉根部瘤合并二尖瓣病变的外科治疗方法以及近中期结果。方法    2010年3月至2016年7月期间，共5例少年儿童主动脉根部瘤合并二尖瓣关闭不全者在首都医科大学附属北京安贞医院接受主动脉根部替换术（Bentall）＋二尖瓣置换术(MVR)，所有患儿均为马方综合征。全组平均年龄为（12.0±1.6）岁（10～14岁）；其中男2例，女3例。术前左心室射血分数为（60±4）%，左心室舒张末径为（56±9）mm。术后通过门诊随访、信件或者电话随访的方式进行随访。观察患儿近中期结果。结果    全组无院内死亡。体外循环时间为（154±23）min，阻断时间为（114±15）min。术中2例使用直径21 mm机械瓣带瓣管道，2例使用直径23 mm机械瓣带瓣管道，1例使用25 mm机械瓣带瓣管道；1例使用直径25 mm机械二尖瓣，3例使用直径27 mm机械二尖瓣，1例使用直径29 mm机械二尖瓣。重症监护室停留时间为21.0（20.0，107.0）h。呼吸机机械通气时间为18.0(16.5，42.5)h。无肾功能衰竭、二次开胸止血、纵膈感染等严重并发症。所有患儿均参与随访，随访时间为56.0(5.5，63.5)个月，1例患儿随访过程中出现恶性心律失常导致猝死，整体5年生存率为78.0%。结论    少年儿童马方综合征主动脉根部瘤合并二尖瓣病变行Bentall＋MVR具有良好的近中期结果，术中可选用与成人相仿的人造移植物型号。

• 【Abstract】Objective    To investigate the short-to-midterm outcomes of surgical treatment of aortic root aneurysm complicated with mitral valve disease in children with Marfan′s syndrome. Methods    Five aortic root aneurysm complicated with mitral valve insufficiency children with Marfan′s syndrome had aortic root replacement（Bentall）＋mitral valve replacement(MVR) from March 200 to July 2016 in Beijing Anzhen Hospital, Capital Medical University. The average age was （12.0±1.6）years old（10-14 years old）. There were 2 males and 3 females. Preoperative left ventricular ejection fraction was （60±4）%; preoperative left ventricular end-diastolic diameter was （56±9）mm. Postoperative outcomes were followed via phone calls, mails and outpatient visits. Results    There was no in-hospital death. Cardiopulmonary bypass time was （154±23）min; aortic clamp time was （114±15）min. Two cases used 21 mm diameter composite mechanical-valved aortic conduit; 2 cases used 23 mm diameter composite mechanical-valved aortic conduit; 1 case used 25 mm diameter composite mechanical-valved aortic conduit. One case used 25 mm diameter mechanical mitral valve; 3 cases used 27 mm diameter mechanical mitral valve; 1 case used 29 mm diameter mechanical mitral valve. Intensive care unit stay time was 21.0（20.0, 107.0）h; ventilator mechanical ventilation time was 18.0(16.5, 42.5)h. There was no renal failure, re-exploration for hemorrhage and mediastinal infection. All patients were followed up for 56.0(5.5, 63.5)months; 1 case suffered sudden death due to malignant arrhythmia; the 5-year survival rate was 78.0%. Conclusion    Bentall＋MVR shows good short-to-midterm results in treatment of aortic root aneurysm complicated with mitral valve disease in children with Marfan′s syndrome; the prosthetic valve and graft size in children are similar to those in adults.

  【Key words】Marfan′s syndrome;Aortic root aneurysm;Mitral insufficiency

  【Fund program】National Science and Technology Pillar Program of China（2015BAI12B03）; Research Project of Beijing Municipal Science and Technology Commission（Z171100001017083）