Uhl anomaly

Uhl anomaly is a rare heart condition in which the muscle layer (myocardium) of the right ventricle is almost completely missing. This results in a thin-walled, nonfunctional right ventricle. The lack of muscle tissue in the right ventricle can lead to serious heart rhythm problems and right ventricular failure. In some people, a partial absence of the myocardium may cause few or mild symptoms until adulthood, making the condition less apparent early in life.

People with Uhl anomaly may experience heart rhythm abnormalities (cardiac arrhythmias) and signs of right ventricular failure. In cases with partial myocardium absence, symptoms can be mild or even not noticeable until later in life.

Information about causes is currently limited for this condition.

For people with complete Uhl anomaly, treatment options may include evaluation for cardiac transplantation due to significant heart dysfunction. Management strategies are tailored based on the severity of the condition.

The outlook for people with complete Uhl anomaly can be serious because of the risk of cardiac arrhythmias and right ventricular failure. In cases with partial absence, the condition may be less severe, but careful monitoring remains important.

Information about research is currently limited for this condition.