Surgical Intervention
Surgery is the primary treatment for supravalvar aortic stenosis (SVAS). Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 50 mm Hg or more should have surgical intervention. The choice of procedures in these patients is similar to that indicated for valvar aortic stenosis. [20]
Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 30-50 mm Hg may be considered for surgical intervention if they are symptomatic, with angina, syncope, or dyspnea on exertion (class I). Asymptomatic patients who have developed ST/T-wave changes over the left precordium on ECG at rest or with exercise should also be considered for surgical intervention (class I). Aortic valve involvement and lower supravalvar gradients may also warrant surgical intervention.
Surgical resection of the supravalvar obstruction and patch aortoplasty and multiple-sinus reconstructions (inverted bifurcated patch plasty and 3-sinus reconstruction) are the procedures of choice for the fibrous diaphragm and hourglass deformities. [21]
Associated coronary artery involvement is addressed with the following measures, which are performed at the same time as aortoplasty:
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Patch aortoplasty encompassing the left main ostium for circumferential narrowing of the left main ostium
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Excision of the fused leaflet from the aortic wall for ostial obstruction caused by a fusion of the aortic cusp to the supravalvar ridge [22]
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Bypass grafting for diffuse narrowing of the left main coronary artery
In patients who have SVAS with diffuse narrowing, the ascending aorta and the arch of the aorta can be reconstructed using an aortic allograft or a pulmonary autograft.
Surgical treatment of associated abnormalities of aortic valve and aortic arch vessels should be undertaken at the same time to optimize the overall surgical outcome. [23]
Standard postoperative care and precautions for pediatric cardiac patients are also required for patients with SVAS. Postoperative complications include aortic insufficiency (in 25% of patients).
Activity
Activity restrictions may be indicated, depending on the severity of the disorder. Exercise recommendations for children with SVAS and no coronary artery involvement are as follows:
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Mild stenosis (< 20 mm Hg), normal ECG findings, no symptoms - Full sports participation
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Moderate stenosis (21-49 mm Hg), mild left ventricular hypertrophy (LVH), no symptoms - Low static or moderately dynamic sports participation
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Severe stenosis (>50 mm Hg) or moderate degree of stenosis with symptoms - No competitive sports participation (at most recreational)
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Coronary artery stenosis or abnormal anatomy - No competitive sports participation (at most recreational and dependent on the level of obstruction)
Additional Management
Williams syndrome, which is found in many children with supravalvar aortic stenosis (SVAS), may be associated with infantile hypercalcemia with some risk of nephrocalcinosis, osteosclerosis with progressive joint limitation and abnormal gait, and neurodevelopmental delay. These children require multidisciplinary support. Use a coordinated management approach. They are also at risk of higher mortality than the normal population is, because of cardiac and noncardiac causes.
No published reports have documented the outcome of pregnancy in postoperative patients with SVAS. Address pregnancy on an individual basis, taking into account the type of lesion and surgical procedure performed, the presence of residual lesion, and the associated cardiac and noncardiac conditions and syndromes.
Long-Term Monitoring
Follow-up care is recommended for all patients with SVAS, whether or not their condition has been surgically corrected, and the frequency is based on the severity of the obstruction and whether there is coronary artery involvement. Rapid progression of SVAS may occur preoperatively.
Detailed cardiac examination and echocardiography should be performed at the follow-up visit. Changes in intensity of the murmur may indicate progressive stenosis; the development of ST-segment or T-wave changes may signal coronary involvement.
Consult a pediatric cardiologist and a pediatric cardiac surgeon, as needed.
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Two-dimensional suprasternal echocardiographic image of supravalvar aortic stenosis.
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Aortogram of a patient with supravalvar aortic stenosis and dilated sinus of Valsalva.
