Ascending aorta and aortic root replacement (with or without valve sparing) in early childhood: surgical strategies and long-term outcomes

Abstract

OBJECTIVESAortic 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.METHODSBetween 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).RESULTSThe 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.CONCLUSIONSAortic 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.

Systematic review and meta-analysis of aortic valve-sparing surgery versus replacement surgery in ascending aortic aneurysms and dissection in patients with Marfan syndrome and other genetic connective tissue disorders

“…The valvular and annular preservation and stabilization provided by reimplantation of the aortic valve within a graft with a pseudosinus can be a longerlasting alternative to a composite prosthetic replacement in children. Other case series of annuloplasty have shown that some patients developed dilated cardiomyopathy, moderate aortic insufficiency and cardiac dysfunction (22).…”

Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child

“…Nevertheless, in some previous series, aortic aneurysm surgical correction with native valve preservation in early childhood seems to be the technique of choice with good results in the late follow-up [ 7 , 8 ] .…”

Ascending aorta and aortic root replacement in an infant: a case report

“…В группе с синдромом Лоуйса -Дитца выполнили операции Дэвида (n = 5) и Бенталла (n = 2). Остальные пациенты не имели генетических аномалий соединительной ткани и прооперированы по поводу врожденных пороков сердца [26].…”

“…Различные центры используют либо критерии взрослой популяции [28], либо при принятии решения о сроке операции ориентируются на динамику увеличения диа метра корня аорты и степень аортальной регургитации [29]. Выбор оперативной техники делает хирург, в то время как при операции Якуба в большинстве случаев описаны повторные вмешательства по поводу прогрессирующей регургитации на клапане аорты, что связано с расширением неукрепленного клапанного кольца в отдаленном периоде [26].…”