Histiocytoid cardiomyopathy

What it means

A type of cardiomyopathy characterized pathologically by hamartomatous lesions of cardiac Purkinje cells.

This HPO term intends to refer to the histological changes that characterize histiocytoid cardiomyopthy. The disease entity Histiocytoid cardiomyopathy is a rare arrhythmogenic disorder characterized by incessant ventricular tachycardia, cardiomegaly, and often sudden death by age 2 years.

Rare diseases that can present with this1

The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.

Part of the broader category

Also called: Arachnocytosis of the myocardium · Foamy myocardial transformation · Focal lipid cardiomyopathy · Infantile cardiomyopathy with histiocytoid changes · Infantile xanthomatous cardiomyopathy · Isolated cardiac lipidosis · Myocardial or conduction system hamartoma · Oncocytic cardiomyopathy