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Fixed subaortic stenosis in atrioventricular canal defect: a Doppler echocardiographic study
G S Reeder1, G K Danielson, J B Seward
1Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota 55905.
Journal of the American College of Cardiology
|August 1, 1992
Summary
Subaortic stenosis can develop anew and worsen over time in patients with atrioventricular (AV) canal defects. Echocardiography is crucial for diagnosing and monitoring this progressive condition, especially after AV canal repair.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- The co-occurrence of fixed subaortic stenosis and atrioventricular (AV) canal defects is rare.
- Limited data exists on the combined presentation and progression of these conditions.
Purpose of the Study:
- To describe echocardiographic and Doppler features of fixed subaortic stenosis in patients with AV canal defects.
- To document the de novo development and progression of subaortic stenosis using sequential echocardiography.
Main Methods:
- Retrospective analysis of 21 patients with subaortic stenosis and AV canal defects (complete or partial).
- Review of patient charts, operative notes, and echocardiographic/Doppler studies over 13 years.
Main Results:
- Subaortic stenosis occurred de novo in 6 patients and progressed in 5 patients over time.
- Diagnosis often followed repair of the AV canal defect, with a mean interval of 6.8 years.
- Surgical resection was performed in 16 patients, with high diagnostic confirmation.
Conclusions:
- Subaortic stenosis can arise de novo and is progressive in patients with AV canal defects.
- Diagnosis and monitoring are often delayed until after AV canal repair.
- Doppler echocardiography is the preferred method for diagnosis and follow-up.