UHL Anomaly

Below you will find more information about UHL Anomaly from Medigest. If you believe that you are suffering from any of the symptoms of UHL Anomaly it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with UHL Anomaly and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to UHL Anomaly comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.


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.


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


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.


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

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