Uhl anomaly

Uhl anomaly is a rare congenital heart disease characterized by the partial or complete absence of the myocardium (heart muscle) of 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 Signs

The symptoms of Uhl anomaly can vary greatly depending on the severity of the condition. Some individuals may be asymptomatic, while others may experience symptoms such as fatigue, shortness of breath, palpitations, and edema. In severe cases, Uhl anomaly can lead to heart failure and sudden cardiac death.

Causes

The exact cause of Uhl anomaly is unknown. However, it is believed to be a result of a developmental defect during embryogenesis. Some studies suggest a possible genetic component, but more research is needed to confirm this.

Diagnosis

Diagnosis of Uhl anomaly is typically made through imaging studies such as echocardiography, cardiac MRI, or cardiac CT. These tests can reveal the characteristic thinning or absence of the right ventricular myocardium.

Treatment

There is currently no cure for Uhl anomaly. Treatment is focused on managing symptoms and preventing complications. This may include medications to manage heart failure symptoms, pacemaker implantation to regulate heart rhythm, or in severe cases, heart transplantation.

Prognosis

The prognosis for individuals with Uhl anomaly is variable and depends on the severity of the condition. Some individuals may live a normal lifespan with few symptoms, while others may experience severe heart failure and require heart transplantation.

See also

• Congenital heart disease
• Cardiomyopathy
• Heart transplantation

References

Uhl anomaly Resources
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