50
Specific Conditions
Table 23. Supravalvar Aortic Stenosis: Routine Follow-Up and
Testing Intervals
Type of Follow-Up
or Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
Outpatient ACHD
cardiologist
24 24 6–12 3–6
Electrocardiogram 24 24
12 12
Transthoracic
echocardiogram
†
24 24 12 12
Modified with permission from Stout et al. Copyright © 2018 American Heart Association, Inc.
and American College of Cardiolog y Foundation.
* See Section 2.2 for details for ACHD anatomic and physiological classification system.
†
Routine transthoracic echocardiography may be unnecessary if CMR imaging is performed
to assess aortic anatomy.
ACHD indicates adult congenital heart disease; and CMR, cardiovascular magnetic resonance.
(cont'd)
4.2.5. Supravalvar Aortic Stenosis
COR LOE
Recommendations
Diagnostic
1 C-LD
1. In adults with suspected supravalvar aortic stenosis or with
Williams syndrome, baseline aortic imaging is recommended
to define the anatomy and severity of supravalvar aortic
stenosis.
1 C-LD
2. In adults with Williams syndrome and supravalvar aortic
stenosis, baseline coronary imaging is recommended to
evaluate for abnormalities such as coronary ostial stenosis.
erapeutic
1 B-NR
3. In adults with supravalvar aortic stenosis who are
symptomatic and/or have diminished LV systolic function
due to stenosis, surgical repair is recommended to alleviate
symptoms and preserve ventricular function.
1 C-LD
4. Adults with supravalvar aortic stenosis and coronary ostial
stenosis with symptoms of ischemia should undergo coronary
artery revascularization to alleviate symptoms and preserve
ventricular function.
2b C-EO
5. In adults with asymptomatic severe supravalvar aortic
stenosis, normal ventricular size and function, and a normal
exercise stress test, surgical repair may be considered to avoid
worsening of cardiac function and symptoms.
