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Increased mitral-aortic separation in discrete subaortic stenosis
Abstract:
We recently speculated that mitral-aortic separation (MAS) might be increased in discrete subaortic stenosis (DSS). We have examined this hypothesis in 22 heart specimens in which the subaortic obstruction originated on the muscular ventricular septum below the right aortic sinus, either as a discrete band, an accumulation of several bands or a diffuse ridge, and extended posteriorly into the MAS or anterior leaflet of the mitral valve or both, with a variable relationship to the aortic cusps and sinuses. No specimen had ventricular septal defect, supravalvular aortic stenosis or other features of Shone syndrome. The mean MAS was nearly twice that of 80 normal specimens (4.9 vs 2.5 mm), the range of MAS was increased from normal (0.11 vs 0.7 mm) and the mean diameter of the aortic annulus was decreased compared with the normal specimens, data that will be of interest to echo- and angiocardiographers in the clinical description of DSS, and to the surgeon who must resect these lesions.
Insights
Discrete subaortic stenosis (DSS) is associated with increased mitral-aortic separation (MAS). This finding may aid in the clinical description and surgical resection of DSS.
Area of Science:
- Cardiovascular Pathology
- Congenital Heart Disease
Background:
- Discrete subaortic stenosis (DSS) is a congenital heart defect characterized by obstruction below the aortic valve.
- The relationship between DSS and mitral-aortic separation (MAS) has not been extensively studied.
Purpose of the Study:
- To investigate the hypothesis that mitral-aortic separation (MAS) is increased in patients with discrete subaortic stenosis (DSS).
Main Methods:
- Examination of 22 heart specimens with DSS, excluding other congenital anomalies.
- Comparison of MAS measurements with 80 normal heart specimens.
- Analysis of the anatomical relationship between the subaortic obstruction and the mitral-aortic junction.
Main Results:
- The mean MAS in specimens with DSS was nearly double that of normal specimens (4.9 mm vs 2.5 mm).
- The range of MAS was significantly increased in DSS specimens compared to normal controls.
- A decreased mean aortic annulus diameter was observed in DSS specimens.
Conclusions:
- Discrete subaortic stenosis is associated with significantly increased mitral-aortic separation.
- These findings provide valuable data for echocardiographers, angiocardiographers, and surgeons involved in the diagnosis and treatment of DSS.