Congenital bicuspid aortic valve (BAV) is one of the most common types of congenital heart disease in adults, with a prevalence of approximately 1% in the general population.
Pathological Anatomy
A bicuspid aortic valve is characterized by two cusps and two sinuses instead of the normal three. With aging, BAV can lead to aortic valve stenosis and/or aortic valve regurgitation.
Pathophysiology
When BAV causes aortic valve stenosis or regurgitation, it may result in corresponding hemodynamic changes.
Clinical Manifestations
When valve function is normal, patients may remain asymptomatic without any signs or symptoms. When valve dysfunction occurs, leading to stenosis or regurgitation, symptoms and signs will appear.
Auxiliary Examinations
Echocardiography is the most direct and reliable method for diagnosing BAV. Aortic valve stenosis may result in secondary left ventricular hypertrophy. Aortic valve regurgitation may lead to secondary left ventricular dilation. ECG and chest x-ray may show corresponding findings.
Diagnosis and Differential Diagnosis
Diagnosis is straightforward based on echocardiographic findings. The main differential diagnoses include rheumatic valvular disease and hypertrophic obstructive cardiomyopathy.
Treatment
Treatment options include interventional therapy and surgical therapy.
For patients with aortic valve stenosis and corresponding symptoms, surgical valve repair or replacement is recommended when the transvalvular pressure gradient is ≥50 mmHg.
For patients with aortic valve regurgitation and progressive left ventricular enlargement, valve replacement surgery should be considered.
Prognosis
The prognosis of isolated bicuspid aortic valve malformation depends on the severity of the associated functional impairment. Additionally, patients with BAV are prone to infective endocarditis, which can lead to rapid clinical deterioration.