Moreau de Bellaing Anne, Pontailler Margaux, Bajolle Fanny, Gaudin Régis, Murtuza Bari, Haydar Ayman, Vouhé Pascal, Bonnet Damien, Raisky Olivier
Department of Pediatric Cardiology, M3C-Necker, Hôpital Universitaire Necker-Enfants Malades, AP-HP, Paris, France.
Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
Eur J Cardiothorac Surg. 2020 Feb 1;57(2):373-379. doi: 10.1093/ejcts/ezz210.
Aortic root and ascending aorta replacements (AARs) are rarely required in the paediatric population. We report here a series of AAR performed in young children using different surgical techniques.
Between 1995 and 2017, 32 children under the age of 10 years (median age 5.4 years) underwent AAR procedures at our institution. Twenty-two (69%) had a connective tissue disease (infantile Marfan syndrome or Loeys-Dietz syndrome). We performed 11 AAR using a composite graft with a mechanical prosthesis and 21 valve-sparing procedures (10 Yacoub operations and 11 David operations). Median follow-up for operative survivors was 7.7 years (interquartile range 4.2-12.8 years).
The cardiac-related early mortality rate was 6%. Patient survival was 91% at both 1 and 10 years. Eleven survivors (38%), all with a status of post-valve-sparing procedure, required an aortic root reintervention with an aortic valve replacement after a median interval of 4.2 years. Interestingly, only patients with infantile Marfan syndrome tended to be associated with risk of reoperation.
Aortic root and AARs are safe in young children whatever the surgical procedure. Aortic valve-sparing procedures show good long-term results except in children with infantile Marfan syndrome whose ineluctable aortic annulus dilatation or aortic valve regurgitation requires reintervention after a short period.
小儿患者很少需要进行主动脉根部和升主动脉置换术（AAR）。我们在此报告一系列针对幼儿采用不同手术技术进行的AAR。
1995年至2017年间，32名10岁以下儿童（中位年龄5.4岁）在我们机构接受了AAR手术。22名（69%）患有结缔组织病（婴儿型马方综合征或洛伊斯-迪茨综合征）。我们使用带机械瓣膜的复合移植物进行了11例AAR，以及21例保留瓣膜的手术（10例亚库布手术和11例大卫手术）。手术存活者的中位随访时间为7.7年（四分位间距4.2 - 12.8年）。
心脏相关早期死亡率为6%。1年和10年时患者生存率均为91%。11名存活者（38%），均为保留瓣膜手术后状态，在中位间隔4.2年后需要进行主动脉根部再次干预并置换主动脉瓣。有趣的是，只有婴儿型马方综合征患者往往与再次手术风险相关。
无论采用何种手术方式，主动脉根部和AAR对幼儿都是安全的。保留主动脉瓣手术显示出良好的长期效果，但婴儿型马方综合征患儿除外，其不可避免的主动脉瓣环扩张或主动脉瓣反流需要在短时间后进行再次干预。
