Laubry-Pezzi Syndrome: A Case Report of a Rare Entity

Abstract

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.

Keywords: aortic regurgitation; cardiothoracic and vascular surgery research; laubry-pezzi syndrome; surgical case reports; ventricular septal defect.

Figure 1. Perimembranous ventricular septal defect, dilatation of the aortic root and descending aorta, pseudocoarctation of the aorta, and severe LV dilatation (left ventricular ejection fraction: 51%).

Figure 2. Sternum of regular quality, trileaflet aortic valve, non-coapt dysplastic valves, right valve with calcium plaque extending to commissure with left valve, 12-mm perimembranous ventricular septal defect, and closed pleura.