Idiopathic Hypertrophic Subaortic Stenosis: A Case Study


  • Jawed H. Siddiqui


Idiopathic Hypertrophic Subaortic Stenosis,


Though the nomenclature and criteria for diagnosis of hypertrophic cardiom yopathy is not entirely satisfactory, the basic pathology is disproportionate hypertrophy of the interventricular septum. An abnormal systolic anterior motion of the mitral valve and dynamic obstruction of the left ventricular outflow tract over the upper portion of the body of the left ventricle. Though the disease could exist in various subjects which may slightly deviate from the typical picture like presence of asymmetrical septal hypertrophy without abnormal systolic anterior motion of the mitral valve and a concentric hypertrophy of the left ventricular pattern with or without obstruction. The left ventricular cavity is usually small, but at least it is not dilated. Because of the abnormality of relaxation of the left ventricle, the left ventricular end diastolic pressure increases which may lead to enlargement of the left atrium. This abnormality of the left ventricular relaxation and obstruction of the left ventricular out-flow may explain various symptoms, but the severity of the symptoms do not directly correlate with the cardiac catheterization and pathology findings.1 6 The left ventricular out-flow obstruction is dynamic in nature which changes from time to time in various maneuvers which effect the left ventricular volume, preload, and after load. Medical therapy is very rewarding in this disease. Surgical treatment is rarely needed, but is available for selected patients who do not respond to medical therapy. Calcium channel blocker drugs may play an important role in the medical management of this disease.