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Coarctation of the aorta or subaortic stenosis with atrioventricular septal defect
Insights
Atrioventricular septal defects combined with aortic coarctation or subaortic stenosis are complex congenital heart conditions. Subaortic stenosis in these patients is often due to discrete anatomical issues and can progress over time.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Atrioventricular (AV) septal defects frequently coexist with other congenital heart anomalies.
- Coarctation of the aorta (C of A) and subaortic stenosis (SAS) are significant associated conditions that can complicate management.
- Understanding the interplay between AV septal defects and these aortic abnormalities is crucial for effective treatment.
Purpose of the Study:
- To analyze the clinical presentation and anatomical characteristics of patients with atrioventricular septal defects and coarctation of the aorta or subaortic stenosis.
- To investigate the mechanisms and progression of subaortic stenosis in the context of AV septal defects.
- To evaluate the hemodynamic significance of these combined defects.
Main Methods:
- Retrospective review of 30 patients with atrioventricular septal defects and either C of A or SAS or both.
- Assessment of left ventricular status via angiography or necropsy.
- Classification into groups based on the presence and type of associated aortic abnormalities.
- Hemodynamic evaluation and angiographic diagnosis of subaortic stenosis.
Main Results:
- Nineteen patients had AV septal defects with C of A (with or without SAS), and 11 had AV septal defects with SAS.
- Preductal C of A was common (16/19).
- Subaortic stenosis was often caused by discrete anatomical lesions, including fibrous diaphragms and fibromuscular obstruction, with some cases showing progression.
Conclusions:
- Atrioventricular septal defects associated with coarctation of the aorta and/or subaortic stenosis represent a distinct clinical entity.
- Subaortic stenosis in this population is typically related to specific anatomical abnormalities.
- The potential for hemodynamic progression of subaortic stenosis necessitates careful monitoring before and after surgical intervention.
Abstract:
Thirty patients are reported with atrioventricular (AV) septal defect and either coarctation of the aorta (C of A) or subaortic stenosis (SAS) or both. All patients had normal left ventricles as assessed by angiography (21 of 30 patients) or necropsy (9 of 30). Three groups were recognized. Groups I and II included 19 patients with AV septal defect (12 complete, 7 partial) and C of A with or without SAS, 11 patients with AV septal defect (5 complete, 6 partial) and SAS. In Group I, preductal C of A was diagnosed in 16 of 19 patients. Concomitant angiographic evidence of SAS was present in 2 cases, the mechanism being exaggerated anterior displacement of the left AV valve. In Group III, at the time of diagnosis left ventricular-aortic peak systolic pressure gradients of greater than 20 mm Hg were present in 9 patients, 2 of whom had gradients greater than 50 mm Hg. Angiographic diagnoses were: discrete fibrous diaphragm in 4, fibromuscular obstruction in 5, dynamic tunnel in 1, and chordae from left AV valve to LV outflow tract in 1. Thus, SAS in AV septal defect is most often due to a discrete anatomic lesion. Hemodynamic data show that SAS can be progressive, both before and after the surgical management of the AV septal defect.