Bata Abdel Kémal Bori, Gaël Biaou, Ibrahim Ahmad, Nékoua Désiré, Sonou Arnaud, Demondion Pierre
University Cardiology Clinic (CNHU-HKM), Faculty of Health Sciences, University of Abomey-Calavi, Cotonou, Benin; University Visceral Surgery Clinic (CNHU-HKM), Faculty of Health Sciences, University of Abomey-Calavi, Cotonou, Benin.
University Cardiology Clinic (CNHU-HKM), Faculty of Health Sciences, University of Abomey-Calavi, Cotonou, Benin.
Int J Surg Case Rep. 2025 Sep 22;136:111963. doi: 10.1016/j.ijscr.2025.111963.
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.
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.
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.
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.
劳布里 - 佩齐综合征是一种罕见的先天性心脏病，其特征是室间隔缺损（VSD）与因瓣膜脱垂导致的主动脉反流（AR）并存。这种疾病的自然病程可能导致严重的瓣膜损害和主动脉根部扩张，尤其是在诊断延迟的情况下。
我们报告了在贝宁接受手术治疗的两例成年劳布里 - 佩齐综合征病例。首例患者为一名49岁男性，表现为严重的主动脉反流、巨大的主动脉下室间隔缺损和升主动脉瘤。他接受了室间隔缺损修补术和机械性Bentall手术。术后，他出现了完全性房室传导阻滞，需要植入起搏器，在24个月内未出现进一步并发症。第二例患者为一名19岁男性，有严重的主动脉反流、16毫米的室间隔缺损和扩张的主动脉瓣窦。他接受了室间隔缺损修补术、机械性主动脉瓣置换术和三尖瓣环成形术。恢复过程顺利，在四个月的随访中结果良好，并计划进行持续监测。
这两例病例展示了成人未经治疗的劳布里 - 佩齐综合征的临床进展，突出了与诊断延迟相关的挑战。尽管解剖学发现复杂，但在资源有限的情况下成功进行了包括Bentall手术和瓣膜置换在内的手术修复。在病理学和结果方面，这些发现与全球文献一致，支持了撒哈拉以南非洲地区进行高级心脏手术的可行性。
早期发现和干预对于预防劳布里 - 佩齐综合征中的严重瓣膜和主动脉并发症至关重要。本病例报告表明，即使在资源匮乏的情况下，通过采用适当的技术和跨学科合作也可实现成功的手术结果。
