Surgical repair of Laubry–Pezzi syndrome with aortic root dilatation in two adult patients: Case reports from Benin

Introduction

Laubry–Pezzi syndrome is a rare congenital heart disease characterized by the association of a ventricular septal defect (VSD) and aortic regurgitation (AR) due to valvular prolapse. The natural course of this condition may lead to severe valvular damage and aortic root dilatation, particularly in cases of delayed diagnosis.

Presentation of case

We report two adult cases of Laubry–Pezzi syndrome managed surgically in Benin. The first patient, a 49-year-old man, presented with severe AR, a large subaortic VSD, and an ascending aortic aneurysm. He underwent VSD closure and a mechanical Bentall procedure. Postoperatively, he developed complete atrioventricular block requiring pacemaker implantation, with no further complications over 24 months. The second patient, a 19-year-old man, had severe AR, a 16 mm VSD, and dilated sinus of Valsalva. He underwent VSD closure, mechanical aortic valve replacement, and tricuspid annuloplasty. Recovery was uneventful, with favorable outcomes at the four-month follow-up, and ongoing surveillance is planned.

Discussion

These two cases demonstrate the clinical progression of untreated Laubry–Pezzi syndrome in adults, highlighting challenges related to delayed diagnosis. Despite complex anatomical findings, surgical repair including Bentall and valve replacement was successfully performed in a resource-limited setting. Findings align with global literature in terms of pathology and outcomes, supporting the feasibility of advanced cardiac surgery in sub-Saharan Africa.

Conclusion

Early detection and intervention are critical to prevent severe valve and aortic complications in Laubry–Pezzi syndrome. This case report illustrates that, even in low-resource contexts, successful surgical outcomes are achievable through adapted techniques and interdisciplinary collaboration.