Cardiac Outcomes in Adults With Supravalvar Aortic Stenosis

Study Questions:

What are the clinical outcomes for adult patients with supravalvar aortic stenosis?

Methods:

In a multicenter retrospective study, cardiac outcomes were assessed for adult patients (≥18 years) with supravalvar aortic stenosis. Adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and the need for cardiac surgery were examined.

Results:

A total of 113 adults (median age at first visit, 19 years; 55% with Williams–Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams–Beuren syndrome had more severe supravalvar aortic stenosis and more often had associated left ventricular outflow tract obstructions (p < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams–Beuren syndrome. Of 133 adult patients, 96 (85%) had serial follow-up information (median follow-up, 6.0 years). On average, the gradients changed little during the follow-up (median gradient at the first clinic visit in adulthood, 28 mm Hg [IQR, 21–47] vs. median gradient at the last follow-up, 24 mm Hg [IQR, 16–40]). Of 96 patients with follow-up, 12 (13%) had an adverse cardiac event and 12 (13%) had a cardiac operation (seven valve repair or replacements, four supravalvar aortic stenosis repairs, one other). Cardiac surgery was more common in adults without Williams–Beuren syndrome (p = 0.007).

Conclusions:

Although progression of supravalvar aortic stenosis in adulthood is rare, adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperation. Valve surgery is the most common indication for cardiac surgery in adulthood.

Perspective:

Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. This multicenter study showed a low rate of progression of left ventricular outflow obstruction, but a significant risk for cardiac complications and requirement for reoperation.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Interventions and Structural Heart Disease, Mitral Regurgitation

Keywords: Stroke, Myocardial Infarction, Follow-Up Studies, Mitral Valve Insufficiency, Heart Diseases, Reoperation, Endocarditis, Ventricular Outflow Obstruction, Heart Failure, Cardiac Surgical Procedures, Williams Syndrome


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