From the Guidelines
Bicuspid aortic valve (BAV) is commonly associated with both flail mitral leaflet and coarctation of the aorta as part of a spectrum of left-sided congenital heart defects, with patients having approximately a 20-30% chance of having associated aortic coarctation, and management requires comprehensive cardiac evaluation and treatment based on the severity of each component. This association occurs because these structures share developmental origins in the embryonic cardiac neural crest. The presence of a flail mitral valve leaflet with coarctation of the aorta in patients with BAV is a complex condition that requires careful evaluation and management to prevent morbidity and mortality.
Key Considerations
- Patients with BAV have a higher risk of aortic coarctation, with approximately 20-30% chance of having this associated condition 1.
- The association with mitral valve abnormalities like flail leaflet is less common but still significant, and requires comprehensive evaluation and management 1.
- Management requires comprehensive cardiac evaluation including echocardiography, cardiac MRI, and sometimes cardiac catheterization to assess all components of this constellation.
- Treatment depends on the severity of each component: surgical repair for significant coarctation, mitral valve repair or replacement for symptomatic flail leaflet causing regurgitation, and monitoring or intervention for the bicuspid aortic valve based on stenosis, regurgitation, or associated aortopathy.
Recommendations
- Operative intervention to repair or replace the aortic root (sinuses) or replace the ascending aorta is indicated in asymptomatic patients with BAV if the diameter of the aortic root or ascending aorta is 5.5 cm or greater 1.
- All patients with this combination require lifelong cardiac follow-up, endocarditis prophylaxis according to current guidelines, and screening of first-degree relatives due to the genetic component of these conditions.
- The prognosis varies based on the severity of each component and the timing of intervention, but early detection and appropriate management significantly improve outcomes 1.
From the Research
Association between Bicuspid Aortic Valve and Coarctation of the Aorta
- Bicuspid aortic valve (BAV) is a common congenital cardiac pathology associated with dilatation of the aorta, known as bicuspid valve-associated aortopathy or bicuspid aortopathy 2.
- BAV disease is associated with several anomalies of the aorta, including coarctation, aneurysm formation, and dissection 3.
- Coarctation of the aorta (CoA) is a congenital heart defect that is associated with a BAV, ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic disease 4.
Presence of a Flail Mitral Valve Leaflet
- There is no direct evidence in the provided studies to suggest an association between a flail mitral valve leaflet and BAV with coarctation of the aorta.
- The studies primarily focus on the association between BAV and coarctation of the aorta, as well as the management and treatment of BAV disease 3, 4, 5, 6.
Management and Treatment
- Several surgical options exist for patients with BAV disease, depending on the age of presentation and the size and appearance of the aorta 3.
- Treatment for CoA with associated BAV and aneurysm formation of the collateral network may involve balloon dilatation, placement of a covered stent, and percutaneous coronary intervention (PCI) 4.
- Management techniques for aortic valve repair versus replacement, indications for aortic surgery, and the emergence of the Ross procedure as a viable management option are discussed in the context of BAV disease 5.