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.
Diagnosis:
An echocardiography or more sophisticated methods of imaging suggest the diagnosis of UHL.
Treatment:
Treatment relies on palliative care but the only hope of extended survival is surgery, which includes cardiac transplantation.
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.
Causes:
The disease is heterogenous and has been reported as an autosomal dominant, autosomal recessive, and x-linked recessive trait.
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