Aortic valve replacement by mechanical prosthesis in children: A positive long-term outcome including for patients with an infantile Marfan syndrome

Abstract

Aortic mechanical valve replacement (AVR) in children, is commonly associated with a high complication rate and a poor long-term prognosis, especially in children with infantile Marfan syndrome (IMS). We reviewed the clinical and surgical records of patients undergoing AVR at our institution between 2000 and 2017. Sixty-five children (49 boys and 16 girls) required 67 AVR at a median age of 12.5 years (range: 7 months to 17.9 years) and a median weight of 41.7 kg (range: 7.5 to 110 kg). Fourteen patients (21.5%) had a connective tissue disorder diagnosed in early childhood (IMS or Loeys–Dietz syndrome or osteogenesis imperfecta). Median follow-up for operative survivors was 4.6 years (IQR: 2.0–9.0 y, up to 16 y, 94% complete). Global cardiac-related mortality was low 6% ( n = 4) and occurred mainly in the first post-operative days caused by acute bleeding. One-year, five-year and ten-year survival was respectively 94%, 91% and 91%. Among survivors, the 10-year freedom from reoperation was 82%. Complications after AVR included heart block requiring pacemaker implantation (5%), bleeding (4%), stroke (11%) and endocarditis (4%). There was no prosthesis thrombosis. All the patients with an IMS ( n = 12) had associated a moderate to severe aortic valve regurgitation and a significant aortic root dilatation. They required an ascending aorta replacement by composite graft concomitantly the AVR (Bentall procedure). Eight of these (73%) had previous aortic root replacement with a valve sparing technique (Yacoub or Tirone–David procedures). All patients with IMS survived. They remained free from aortic reoperation except one who required a larger aortic prosthesis 9 years later. Long-term results after AVR are excellent. Complications associated with surgery and long-term anticoagulation are rare. This study gives confidence in aortic mechanical prosthesis valve replacement including for children with an infantile MS.