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The risk of having additional obstructive lesions in neonatal coarctation of the aorta
J C Levine1, S P Sanders, S D Colan
1Department of Cardiology, Children's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Insights
Infants with aortic coarctation often develop additional left heart obstructions, increasing risks. Echocardiography can identify infants at higher risk for these serious, late-appearing stenotic lesions.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Echocardiography
Background:
- Coarctation of the aorta (CoA) can be associated with other left heart obstructions.
- These additional stenoses may not be evident on initial echocardiograms.
- Risk stratification for these late-appearing lesions is poorly defined.
Purpose of the Study:
- To determine the frequency of additional, late-appearing stenotic lesions in infants with CoA.
- To identify predictive morphologic features on initial echocardiograms for these lesions.
Main Methods:
- Retrospective study of 101 infants diagnosed with CoA by 3 months of age.
- Exclusion of complex cardiac disease or existing additional stenoses.
- Follow-up echocardiography to identify new stenotic lesions.
Main Results:
- 31 stenotic lesions were diagnosed in 23 patients during follow-up.
- Mitral stenosis (11), aortic stenosis (10), and subaortic stenosis (8) were most common.
- Smaller mitral valve Z-scores (< -1) predicted higher risk for any obstructive lesion.
Conclusions:
- Associated left heart stenoses are common in infants with coarctation of the aorta.
- Initial echocardiographic features can identify high-risk infants.
- Early identification aids in managing potential complications.
Abstract:
Infants with coarctation of the aorta may have obstructions at other sites within the left heart which are not always apparent on the initial echocardiogram. The magnitude of the risk of having the additional obstructions is not well described, with few reliable quantitative criterions for identifying patients at the highest risk. We determined the frequency of additional, late appearing, stenotic lesions within the left heart, and the predictive morphologic features on the initial cross-sectional echocardiogram. We identified all patients with coarctation of the aorta diagnosed by 3 months of age, excluding those with complex cardiac disease or definite additional stenotic lesions at presentation, leaving 101 patients for study. At follow-up, 31 stenotic lesions were diagnosed in 23 patients, 15 of whom had at least 1 intervention. Mitral stenosis was diagnosed in 11 patients, aortic stenosis in 10, subaortic stenosis in 8, and supravalvar aortic stenosis in 2. The probability for freedom from obstructive lesions was 81% at 1 year, 74% at 3 years, and 70% at 5 years. Echocardiographic predictors of mitral stenosis included smaller mitral valvar annuluses, presence of a mean transmitral gradient between 2.5 and 5.0 mmHg, and elongation of the area of intervalvar fibrous continuity. Predictors of aortic stenosis were smaller mitral valvar annuluses, an initial aortic valvar gradient between 15 and 20 mmHg, and obliteration of the commissure between the right and non-coronary leaflets of the aortic valve. Predictors of subaortic stenosis were smaller mitral valvar annuluses and elongation of the area of intervalvar fibrous continuity. Patients with Z-scores for the diameter of the mitral valve of less than -1 were at the highest risk for manifesting obstructive lesions at any level. Associated stenoses in the left heart are common in the setting of aortic coarctation. When Doppler data is equivocal, features of the cross-sectional echocardiogram can identify the sub-group of infants at increased risk.