Hypertrophic Subaortic stenosis - Erler Zimmer

Hypertrophic Subaortic stenosis - Erler Zimmer

Clinical History

A slender 42-year-old American tourist was discovered deceased in his hotel bedroom, leading to a coroner's autopsy.

Pathology

This longitudinal section through the heart reveals the left and right ventricles and interventricular septum. The notable abnormality is a significantly thickened interventricular septum and left ventricular hypertrophy. The visible aortic cusps and mitral valve appear unremarkable, but the ventricular septum is so enlarged that it encroaches on the lumen of the left ventricle.

Diagnosis

Idiopathic hypertrophic subaortic stenosis, also known as hypertrophic cardiomyopathy.

Further Information

Subaortic stenosis is considered acquired rather than congenital, believed to stem from an underlying defect in the architecture of the left ventricular outflow tract (LVOT). This defect can result in turbulent blood flow, leading to progressive thickening and fibrosis of the LVOT and the aortic valve. Disease progression often leads to hypertrophic cardiomyopathy due to the increased aortic pressure the left ventricle must overcome. Mild or moderate stenosis may be asymptomatic, but as the condition becomes severe, symptoms such as exertional dyspnoea and syncope may manifest. Physical examination may reveal an ejection systolic murmur, prompting investigation and subsequent diagnosis using echocardiography.
Modern treatment of subaortic stenosis typically involves surgical correction of the obstruction.