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UHL Anomaly

UHL Anomaly Causes

The disease is heterogenous and has been reported as an autosomal dominant, autosomal recessive, and x-linked recessive trait.

UHL Anomaly Definition

First described in 1952, UHL anomaly is a rare congenital heart disease characterized by a partial or total loss of the myocard muscle in the right ventricle. This results in decreased output of the right side of the heart.

UHL Anomaly Diagnosis

An echocardiography or more sophisticated methods of imaging suggest the diagnosis of UHL.

UHL Anomaly Symptoms and Signs

UHL symptoms include dyspnoea, fatigue, chest pain, syncope on exertion, and non-specific systolic murmur. The latter can be a cause of cardiac failure and sudden death in neonates and young adults.

UHL Anomaly Treatment

Treatment relies on palliative care but the only hope of extended survival is surgery, which includes cardiac transplantation.

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