48
Specific Conditions
4.2.4. Congenital Diseases of the Aortic Valve
COR LOE
Recommendations
Diagnostic
1 B-NR
1. Adults with a BAV should be screened for the presence of
COA and aortic aneurysm to obtain early diagnosis and
determine a management plan.
1 B-NR
2. Adults with a BAV who have an aortic diameter >4 cm at
the level of the sinuses or the ascending aorta, or who have
risk factors for aortic dissection,* should undergo lifelong
surveillance for progression of thoracic aortic disease to
improve early detection of aortic growth.
†
2a B-NR
3. In first-degree relatives of adults with a BAV, 1-time
echocardiographic screening for the presence of a
congenitally abnormal aortic valve or aortic aneurysm is
reasonable to obtain early diagnosis.
erapeutic
1 C-LD
4. In adults with a congenitally abnormal aortic valve who meet
indication for valve replacement, surgical and transcatheter
options should be reviewed by heart valve and ACHD expert
teams in a shared decision-making process to determine a
management plan.
2b B-NR
5. In young adults with congenital aortic valve stenosis, a
noncalcified valve with no more than mild aortic regurgitation,
and an indication for intervention, balloon valvuloplasty may
be reasonable to delay the need for aortic valve surgery.
* Risk factors: family history of aortic dissection, aortic growth rate ≥0.3 cm/year, COA,
"root phenotype" aortopathy.
†
Modified from the "2022 ACC/AHA Guideline for the Diagnosis and Management of
Aortic Disease."
Table 21. Subaortic 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
36 24 6–12 3–6
Electrocardiogram 36 24
12 12
Transthoracic
echocardiogram
36 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 on the ACHD anatomic and physiological classification system.
ACHD indicates adult congenital heart disease.
