Javier Delmo Eva Maria, Javier Mariano Francisco Del Maria, Hetzer Roland
Department of Cardiothoracic Surgery, Cardio Centrum Berlin, Berlin, Germany.
Eur J Cardiothorac Surg. 2020 Jan 1;57(1):54-62. doi: 10.1093/ejcts/ezz143.
This study focuses on the consecutive sequelae of children and adolescents with Marfan syndrome after primary cardiovascular surgical interventions and the long-term outcomes.
A retrospective review of children with Marfan syndrome who underwent primary surgical intervention in childhood (<18 years) and sequential cardiovascular operations between July 1986 and September 2014 was undertaken.
Thirty-six children (mean age 12.38 ± 4.4, range 0.62-17.14 years, at the first operation; 12 females) underwent a total of 97 sequential cardiovascular operations. Primary pathologies were annuloaortic ectasia, aortic root and ascending aorta aneurysm, aortic valve insufficiency, floppy mitral valve and midaortic syndrome. Initial operations were emergency in 2 and urgent and elective in 10 and 24 patients, respectively. Associated skeletal abnormalities were seen in 8. All underwent a second operation; 17 underwent a third operation; 6 had a fourth; and 2 had a fifth. The survival rate of children <13 years of age at the first operation compared to those who were older and male, was not statistically significant (P = 0.244). The cumulative survival rate based on age at first operation was 63.24 ± 0.16% at a mean follow-up period of 13.9 ± 6.8 (median 11.8, range 2-32.6) years.
Patients with Marfan syndrome undergoing surgery during childhood needed sequential reinterventions. However, even with multiple consecutive surgeries, long-term outcome is deemed satisfactory.
本研究聚焦于马凡综合征儿童和青少年在初次心血管外科手术后的连续后遗症及长期预后。
对1986年7月至2014年9月期间在儿童期（<18岁）接受初次手术干预及后续心血管手术的马凡综合征患儿进行回顾性研究。
36名儿童（首次手术时平均年龄12.38±4.4岁，范围0.62 - 17.14岁；12名女性）共接受了97次连续心血管手术。主要病变包括主动脉环扩张、主动脉根部及升主动脉瘤、主动脉瓣关闭不全、二尖瓣脱垂和主动脉中段综合征。初次手术中，2例为急诊手术，10例为限期手术，24例为择期手术。8例伴有骨骼异常。所有患儿均接受了第二次手术；17例接受了第三次手术；6例接受了第四次手术；2例接受了第五次手术。首次手术时年龄<13岁的儿童与年龄较大及男性儿童相比，生存率无统计学差异（P = 0.244）。在平均随访13.9±6.8（中位数11.8，范围2 - 32.6）年时，基于首次手术年龄的累积生存率为63.24±0.16%。
儿童期接受手术的马凡综合征患者需要进行连续的再次干预。然而，即使进行多次连续手术，长期预后仍被认为是令人满意的。
