47
4.2.2. Congenital Mitral Stenosis, Including Shone Complex
COR LOE
Recommendations
1 B-NR
1. Adults with congenital mitral stenosis or a parachute mitral
valve should be evaluated for additional left-sided obstructive
lesions to guide management.
1 C-LD
2. Adults with multiple left-sided obstructive lesions (Shone
complex) and suspected pulmonary hypertension should
undergo invasive hemodynamic assessment to identify and
classify pulmonary hypertension.
Table 20. Congenital Mitral 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
36–60 12–24 6–12 3–6
Electrocardiogram 36–60 12–24
12 12
Transthoracic
echocardiogram
36–60 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.
4.2.3. Subaortic Stenosis
COR LOE
Recommendations
Diagnostic
2b C-EO
1. For patients with subaortic stenosis and equivocal symptoms,
exercise stress testing may be considered to assess the
physiological response to exercise and to determine whether
symptoms are attributable to subaortic stenosis.
erapeutic
1 C-EO
2. For patients with symptoms attributable to subaortic stenosis,
surgical repair is recommended to alleviate symptoms.
2b C-LD
3. For asymptomatic patients with LV dysfunction or LV
hypertrophy in the setting of subaortic stenosis, surgical
repair may be considered to mitigate worsening LV function,
hypertrophy, or both.
2b C-LD
4. For asymptomatic patients with subaortic stenosis and at least
mild aortic regurgitation, surgical repair may be considered to
prevent progressive aortic regurgitation.
