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.

Laubry-Pezzi syndrome is a rare congenital cardiac condition characterized by a ventricular septal defect (VSD) and aortic regurgitation (AR) due to aortic cusp prolapse. Although the management of this syndrome is not well-established, early closure of the VSD is recommended to prevent or minimize the progression of AR. This report presents a case of a 23-year-old female diagnosed with Laubry-Pezzi syndrome. Our findings emphasize the importance of early diagnosis and prompt intervention to reduce the risk of complications, such as infective endocarditis and progressive aortic valve dysfunction. This report underscores the need for tailored management strategies, with the possibility of aortic valve repair or replacement depending on the severity of AR and VSD.

Aortic valvuloplasty frequency has significantly increased over the past 15 years. Surgical repair varies in complexity depending on valvular lesions. Our aim is to report results on the whole spectrum of aortic valvuloplasty techniques. All children who consecutively underwent aortic valvuloplasty for aortic stenosis and/or aortic insufficiency between January 2006 and December 2020 at Necker Sick Children's Hospital (Paris, France) were included in a retrospective cohort study. Aortic valvuloplasty techniques were classified into 3 difficulty levels: (1) simple repair, corresponding to commissurotomy and/or shaving in aortic stenosis (AS) in neonates (group 1) and children >1 month (group 2); (2) intermediate-complexity repair, corresponding to commissuroplasty, leaflet resuspension, and fenestration closure in aortic insufficiency (leaflet prolapse in connective tissue disease, isolated leaflet prolapse and Laubry-Pezzi groups); and (3) complex repair requiring a pericardial patch to restore a functional aortic valve in mixed aortic valve disease (bicuspidization with neocommissure and cusp extension groups). During the study period, 324 children underwent aortic valvuloplasty. Survival and freedom from aortic valve reintervention at 10 years were, respectively, 86.1% and 50.9% in neonates with AS, 95.2% and 71.7% in children >1 month with AS, 93.8% and 79.5% in leaflet prolapse in connective tissue disease, 97.7% and 91.9% in isolated leaflet prolapse, 100% and 88% in those with Laubry-Pezzi syndrome, 97.4% and 84.8% in bicuspidization with neocommissure, and 100% and 54.2% in the cusp extension. Durability of aortic valvuloplasty techniques is satisfactory and offers the possibility to delay the Ross procedure, regardless of the lesion's complexity.

Laubry-Pezzi syndrome is a rare congenital heart disease characterized by the association of ventricular defect septal to aortic cusp prolapse responsible for aortic regurgitation (AR). We reported three cases of Laubry-Pezzi syndrome diagnosed in our department of cardiology on a cohort of more than 3000 cases of congenital heart disease. A 13-year-old patient presented a Laubry-Pezzi syndrome with severe AR and significant volumetric left ventricle overload and was operated on in time to allow a good evolution of his condition. A 43-year-old patient, followed for congenital cardiac pathology, presented with severe dyspnea. The echocardiogram found global dysfunction of the left ventricle with an ejection fraction of 35%, a perimembranous ventricular septal defect (VSD) almost completely closed by the prolapse of the noncoronary cusp, and severe eccentric aortic insufficiency due to the prolapse of the noncoronary cusp. Aortic valve replacement and VSD closure were indicated. The third patient is a 21-year-old patient with Down syndrome, in whom a grade 2/6 systolic murmur was detected. Transthoracic echocardiography revealed a perimembranous VSD measuring 4 mm without any hemodynamic repercussions and a moderate aortic insufficiency due to the prolapse of the noncoronary cusp. Clinical and echocardiographic monitoring with Osler prevention was indicated as a modality of management. The pathophysiology is explained by the Venturi effect, the restrictive shunt of the VSD creates an area of low pressure that sucks the adjacent cusp resulting in aortic prolapse and regurgitation. The diagnosis is essentially based on transthoracic echocardiography; it must be conducted before the emergence of AR. The management of this rare syndrome still remains nonconsensual, whether it is through timing or operative techniques. Management must be early by closing the VSD with or without aortic valve intervention to prevent the onset or worsening of AR.

Persistent left superior vena cava (LSVC) that drains into the left atrium (LA) via the left superior pulmonary vein (LSPV) is a rare systemic venous drainage anomaly. It can cause cyanosis and unexplained recurrent strokes. Undiagnosed, it can seriously disrupt the conduct of the cardiopulmonary bypass (CPB), causing sudden air lock and/or flooding of the operative field with venous blood. Its connection with the LSPV outside the pericardium makes its intraoperative diagnosis difficult. We report here the case of a 48-year-old man operated for mitral and aortic valve endocarditis, complicating a Laubry-Pezzi syndrome. The opening of the LA was followed immediately by the entrance of high volume of air bubbles into the superior vena cava cannula which resulted in sudden air lock of the venous outflow line. After multiple lowerings and cessations of pump flow, partial clamping of this cannula resulted in flooding of the LA with venous blood coming from the LSPV. The heart luxation did not allow us to find the LSVC in its usual intrapericardial location, between the LSPV and the left appendage. We had to widely open the left pleura to expose its completely extrapericardial path and its communication with the LSPV. The LSVC was temporally clamped during the remainder of the surgical procedure, then ligated at both ends. The patient underwent mitral valve repair, closure of the infundibular septal defect, aortic valve replacement and tricuspid annuloplasty. He was discharged 10 days later.