Supravalvular aortic stenosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Supravalvular aortic stenosis is the most uncommon cause of left ventricular outflow tract obstruction (LVOT) accounting for 8% of congenital cases of LVOT obstruction.

Pathophysiology

1. Obstruction occurs just above the coronary ostium at the level of the sinotubular junction:
   • Hourglass type (the most common)
   • Hypoplastic type: uniform narrowing of the ascending aorta.
2. Associated lesion is peripheral pulmonary arterial stenosis
3. Because of high perfusion pressure of the coronary arteries there is premature coronary artery disease.
4. Coronary arteries may be obstructed by an adjacent stenotic ring.

Genetics

1. One third of cases are transmitted as an autosomal dominant trait.

Diagnosis

Physical Examination

1. 50% have a characteristically greater pulse and systolic blood pressure in the right carotid and brachial arteries than in the left.
2. The systolic murmur is maximal below the right clavicle and radiates primarily to the right carotid artery.
3. No ejection click is present, no diastolic murmur.

Echocardiography

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Treatment

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ACC / AHA Guidelines- Recommendations for Evaluation of the Unoperated Patient (DO NOT EDIT)

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Class I Class I 1. TTE and/or TEE with Doppler and either MRI or CT should be performed to assess the anatomy of the LVOT, the ascending aorta, coronary artery anatomy and flow, and main and branch pulmonary artery anatomy and flow. (Level of Evidence: C) 2. Assessment of anatomy and flow in the proximal renal arteries is recommended in ACHD patients with SupraAS. (Level of Evidence: C) 3. Assessment of systolic and diastolic ventricular function is recommended in ACHD patients with SupraAS. (Level of Evidence: C) 4. Assessment of aortic and mitral valve anatomy and function is recommended in ACHD patients with SupraAS. (Level of Evidence: C) 5. Adults with a history or presence of SupraAS should be screened periodically for myocardial ischemia. (Level of Evidence: C) Class IIa 1. Exercise testing, dobutamine stress testing, positron emission tomography, or stress sestamibi with adenosine studies can be useful to evaluate the adequacy of myocardial perfusion. (Level of Evidence: C)

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ACC / AHA Guidelines- Recommendations for Interventional and Surgical Therapy (DO NOT EDIT)

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Class I Class I 1. Operative intervention should be performed for patients with supravalvular LVOT obstruction (discrete or diffuse) with symptoms (ie, angina, dyspnea, or syncope) and/or mean gradient greater than 50 mm Hg or peak instantaneous gradient by Doppler echocardiography greater than 70 mm Hg. (Level of Evidence: B) 2. Surgical repair is recommended for adults with lesser degrees of supravalvular LVOT obstruction and the following indications: a. Symptoms (ie, angina, dyspnea, or syncope). (Level of Evidence: B) b. LV hypertrophy. (Level of Evidence: C) c. Desire for greater degrees of exercise or a planned pregnancy. (Level of Evidence: C) d. LV systolic dysfunction. (Level of Evidence: C) 3. Interventions for coronary artery obstruction in patients with SupraAS should be performed in ACHD centers with demonstrated expertise in the interventional management of such patients. (Level of Evidence: C)

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ACC / AHA Guidelines- Recommendations for Key Issues to Evaluate and Follow-Up (DO NOT EDIT)

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Class I Class I 1. Both operated and unoperated patients with SupraAS should be followed up annually at a regional ACHD center. (Level of Evidence: C) 2. Long-term psychosocial assessment and oversight, including the need for legal guardianship, are recommended for patients with Williams syndrome. (Level of Evidence: C)

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