Aortic valve repair versus replacement in Laubry and Pezzi syndrome

Aimar, Farid; Lakehal, Redha; Bendjaballah, Soumaya; Bouzid, Abdelmalek

doi:10.1016/s1878-6480(17)30932-1

Cited by 1 publication

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“…The location of the VSD frequently found is juxtaarterial or perimembranous, as is the case in our three patients 4 – 7 , 16 . The prolapse of the noncoronary cusp and that of the right coronary cusp are most implicated in the onset of aortic insufficiency, as found in the literature 6 – 8 .…”

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confidence: 77%

“…Laubry–Pezzi syndrome is a rare congenital heart disease occurring insidiously 2 , 3 . The prevalence of aortic valve prolapse is 5–8% in perimembranous VSD, with a male predominance 4 , 9 . The first description of the clinical features of Laubry–Pezzi syndrome was made in 1921 by Charles Laubry and Cesare Pezzi, in a patient presenting with VSD associated with AR by prolapse of an aortic cusp 2 , 5 .…”

Section: Discussionmentioning

confidence: 99%

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Laubry–Pezzi syndrome: three case reports and review of the literature

Charfo

Ettagmouti

Mahoungou-Mackonia

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction and importance: 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). Case presentation: 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. Clinical discussion: 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. Conclusion: Management must be early by closing the VSD with or without aortic valve intervention to prevent the onset or worsening of AR.

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