马方综合征少年儿童主动脉根部瘤的外科治疗

Surgical treatment of aortic root aneurysm in adolescent patients with Marfan syndrome

目的探讨马方综合征少年儿童主动脉根部瘤患者行主动脉根部置换术的近中期效果。方法 2009年2月至2016年7月期间,共10例儿童主动脉根部瘤患者在北京安贞医院接受主动脉根部置换术(Bentall)。所有患者均诊断为马方综合征。全组年龄9~16(12.30±2.31)岁,男6例、女4例,平均身高(172.50±12.55)mm,平均体重(48.60±17.08)kg。Bentall手术同期行二尖瓣置换术5例,三尖瓣成形2例。结果全组无院内死亡发生。呼吸机机械通气时间中位数为15.50(9.00~18.75)h。其中1例患者呼吸功能衰竭,其余患者呼吸机机械通气时间均<24 h。重症监护室停留时间中位数为20.00(13.50~21.25)h。无肾功能衰竭、二次开胸止血、纵隔感染等严重并发症。出院前复查超声心动图主动脉瓣平均流速(209.17±48.86)cm/s,平均压差(18.00±7.95)mm Hg。所有患者均参与随访,随访时间中位数为62个月,6年生存率为89.47%。1例患者随访过程中出现恶性心律失常导致猝死,其余9例截至最后一次随访时均生存,无抗凝相关并发症,无主动脉事件发生,心功能分级(NYHA)均为Ⅰ级。结论马方综合征少年儿童主动脉根部瘤患者Bentall术后严重抗凝并发症发生率不高,生长发育也未受限,具有良好的近中期结果。

Objective To study the mid-term and long-term postoperative results of Bentall procedure in patients with Marfan syndrome. Methods From February 2009 to July 2016, ten adolescent patients（mean age of12.30±2.31 years ranged 9-16 years, mean height of 172.50±12.55 mm, mean weight of 48.60±17.08 kg） underwent Bentall procedure in our hospital. All these teenage patients were with Marfan syndrome, 6 boys and 4 girls. Five of them underwent mitral valve replacement procedure at the same time while 2 of them with tricuspid valvuloplasty. Results No mortality was found in hospitalization. One patient suffered respiratory failure after surgery. No renal failure, mediastinal infection or re-operation caused by bleeding were observed. All were well followed up for 62 months. One died of malignant arrhythmia, the other were in good condition with the latest interview. Conclusion Teenage patients with Marfan syndrome who underwent Bentall procedure have favorable outcomes in mid-term and long-term follow-up, they probably will not suffer anti-coagulation complications and restriction of growth.