Aortic valve cusp prolapse with severe aortic regurgitation secondary to perimembranous ventricular septal defect - the Laubry-Pezzi syndrome

Arunothayaraj, Sandeep; Adams, H.; Arunothayaraj, P.; MacIsaac, A.

doi:10.1016/j.hlc.2015.06.729

Cited by 3 publications

(3 citation statements)

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“…4 VSDs can also lead to AV-prolapse (AVP) and associated regurgitation, known as Laubry-Pezzi syndrome, with the mechanism being chronic venturi effect from VSD flow coupled with reduced support from RCC of the AV. 5,6 VSDs have known to be associated with infective endocarditis (IE). In their case of a peri-membranous VSD complicated by an aorto-RV fistula, Miles et al 7 also noted that flow across moderate-to-large VSDs can entrain the RCC of the AV causing AVP and regurgitation.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Color Flow Jet in Left Ventricular Outflow Tract Mimicking a Ventricular Septal Defect: Establishing Differentials and Diagnosis and Role of Transesophageal Echocardiography

Elliott,

Abraham,

Arain

2024

A&A Practice

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Section: Discussionmentioning

confidence: 99%

Abnormal Color Flow Jet in Left Ventricular Outflow Tract Mimicking a Ventricular Septal Defect: Establishing Differentials and Diagnosis and Role of Transesophageal Echocardiography

Elliott,

Abraham,

Arain

2024

A&A Practice

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“…Patients are either asymptomatic or describe progressive exertional dyspnea, whether or not associated with palpitations. Auscultation may find a pan-systolic murmur in the lower left sternum related to the VSD and possibly a diastolic murmur of AR 10 .…”

Section: Discussionmentioning

confidence: 99%

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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“…Surgery is the only valuable management option. Early diagnosis permit to treat of the VSD before the AR installment [3]. The trans-aortic approach allowed us to do a single step procedure allowing the repair of the septal and the aortic disorders at the same time.…”

Section: Discussionmentioning

confidence: 99%

Surgical repair of perimembranous ventricular septal defect and aortic regurgitation in an adult patient with Laubry-Pezzi syndrome.

2020

JMR

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Laubry-Pezzi syndrome is a congenital heart disease that consist in a prolapse of aortic valve cusping into a subjacent ventricular septal defect due to Venturi effect. It results in progressive aortic valve insufficiency. The perimembranous type is the most common due to the proximity of the aortic annulus to such defects. The aim of this report is to highlight the specificity of the diagnosis and the surgical management of this syndrome in adult patients.

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Aortic valve cusp prolapse with severe aortic regurgitation secondary to perimembranous ventricular septal defect - the Laubry-Pezzi syndrome

Abstract: S427http://dx.Aims: Aortic dissection at a young age is often the first manifestation of a genetic aortopathy, including the lesser

Cited by 3 publications

References 0 publications

Abnormal Color Flow Jet in Left Ventricular Outflow Tract Mimicking a Ventricular Septal Defect: Establishing Differentials and Diagnosis and Role of Transesophageal Echocardiography

Abnormal Color Flow Jet in Left Ventricular Outflow Tract Mimicking a Ventricular Septal Defect: Establishing Differentials and Diagnosis and Role of Transesophageal Echocardiography

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

Surgical repair of perimembranous ventricular septal defect and aortic regurgitation in an adult patient with Laubry-Pezzi syndrome.

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