Multicentre experience with valve-sparing aortic root replacement by means of combined remodelling and external aortic ring annuloplasty in patients with Marfan syndrome

Abstract

Objectives: The most recent valve-sparing root replacement technique combines the advantages of the reimplantation (David) and remodelling (Yacoub) techniques. The aortic root is reconstructed according to the remodelling technique, the aortic valve is repaired according to the principle of effective height, and an external ring provides annular support. The purpose of this study was to evaluate operative and mid-term outcomes using this technique in patients with Marfan syndrome.

Methods: Adult patients with Marfan syndrome who had an indication for aortic root surgery according to European Society of Cardiology guidelines and were operated on using this new root replacement technique were retrospectively evaluated. Follow-up was obtained from standard outpatient visits and included echocardiography.

Results: The study group comprised 22 patients (mean age 36 years, 68% males). Mean follow-up was 7.5 years. There were no mortalities. Two patients required aortic valve replacement because of aortic regurgitation. In both patients, the aortic root was severely dilated (≥65 mm) preoperatively, with grade III aortic valve regurgitation and aortic valve cusps that were very fragile. Aortic regurgitation was grade ≤I on follow-up in 18 of the remaining 20 patients.

Conclusions: Valve-sparing root replacement using remodelling combined with aortic-ring annuloplasty is safe in patients with Marfan syndrome. The mid-term outcome is promising in patients undergoing elective valve-sparing root replacement at recommended root diameters. However, in patients with extremely dilated aortic roots and already severe aortic regurgitation, the technique should be used cautiously as aortic cusps are fragile and might not be suitable for durable repair.

Clinical registration number: UMCG Research registry #11208.

Keywords: Aortic root replacement; Aortic valve repair; Marfan syndromes.

Figure 1:

External and internal view of the aortic root and aortic valve after valve-sparing root replacement.

Figure 2:

Grade of aortic regurgitation preoperative, postoperative and during follow-up. Two patients eventually underwent aortic valve replacement (*), respectively, 1 week and 2 years after valve-sparing root replacement due to the recurrence of grade III aortic valve regurgitation. Both had initially presented with decompensated heart failure due to grade III aortic regurgitation with extremely dilated aortic roots, 88 and 65 mm, respectively.