Uhl

Uhl's Anomaly is a rare congenital heart defect characterized by the partial or complete absence of the myocardium (heart muscle) in the right ventricle. This condition is also known as Uhl's Disease or Parchment Heart Disease. It was first described by Henry Uhl, an American pathologist, in 1952.

Symptoms and Diagnosis

The symptoms of Uhl's Anomaly vary depending on the severity of the condition. In severe cases, symptoms may appear in infancy and include heart failure, arrhythmia, and cyanosis. In milder cases, symptoms may not appear until adulthood and can include palpitations, dyspnea, and syncope.

Diagnosis of Uhl's Anomaly is typically made through echocardiography, which can reveal the thinning or absence of the right ventricular myocardium. Other diagnostic tests may include cardiac MRI and cardiac catheterization.

Treatment

There is currently no cure for Uhl's Anomaly. Treatment is focused on managing symptoms and preventing complications. This may include medications to manage heart failure and arrhythmias, and in severe cases, heart transplantation may be considered.

Prognosis

The prognosis for individuals with Uhl's Anomaly is generally poor, with many individuals not surviving past infancy or early childhood. However, with advances in treatment and management, some individuals may live into adulthood.

See Also

• Congenital heart defect
• Cardiomyopathy
• Heart transplantation

References

Uhl Resources
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