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Discrete subvalvular aortic stenosis in childhood. Study of 51 patients
Insights
Discrete subvalvular aortic stenosis in children presents with severe obstruction, often masked by other heart defects. Surgical intervention is common, but residual or recurrent gradients necessitate careful follow-up.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Congenital Heart Disease
Background:
- Discrete subvalvular aortic stenosis (SAS) is a significant cause of left ventricular outflow tract obstruction in children.
- The condition can be associated with other congenital cardiac anomalies, complicating diagnosis and management.
Purpose of the Study:
- To analyze the anatomic types, clinical presentation, and outcomes of surgical management for discrete SAS in a pediatric cohort.
- To establish criteria for operability and understand factors contributing to postoperative pressure gradients.
Main Methods:
- Retrospective review of 51 children with discrete SAS diagnosed and treated between 1951 and 1974.
- Analysis of cardiac catheterization data, including pressure gradients and angiographic findings.
- Evaluation of surgical outcomes and postoperative catheterization results.
Main Results:
- Three main anatomic types identified: thin membranous (43%), fibromuscular collar (5%), and tunnel (3%).
- Median left ventricular to aortic systolic pressure gradient was 90 mm Hg, indicating severe obstruction.
- Associated cardiac defects were present in 57% of patients, often delaying diagnosis.
- Forty patients underwent surgical resection; postoperative gradients were observed in some, potentially due to incomplete resection or restenosis.
Conclusions:
- Discrete SAS requires careful surgical consideration, especially when associated with other cardiac defects.
- Angiographic evidence of a discrete subvalvular diaphragm and a resting gradient ≥40 mm Hg are key criteria for operability.
- Postoperative gradients highlight the complexity of complete obstruction relief and the potential for recurrence.
Abstract:
Fifty-one children with discrete subvalvular aortic stenosis were studied between 1951 and 1974. The three anatomic types of obstruction found were the thin membranous type (43 cases), the fibromuscular collar type (5 cases) and the tunnel type (3 cases). The obstruction was usually severe, and the median left ventricular to aortic systolic pressure gradient was 90 mm Hg. Progressive obstruction with an increasing gradient was documented in 10 patients by serial cardiac catherizations. Significant associated cardiac defects, present in 57 percent of patients, often masked the typical clinical and cardiac catheterization features of subaortic stenosis. The stenosis was often not discovered until after surgery for the associated defect. Forty patients underwent surgical resection of the discrete subaortic obstruction. After surgery significant left ventricular to aortic pressure gradients can be found at postoperative cardiac catheterization. These gradients may reflect inadequate resection of the more complex discrete obstructions or represent proliferation and regrowth of the previously resected subvalvular fibrous tissue. The criteria for operability of discrete subaortic stenosis should be the angiographic demonstration of a discrete subvalvular diaphragm and the presence of a resting left ventricular to aortic systolic pressure gradient of 40 mm Hg or more.