Uhl anomaly

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Uhl Anomaly

Uhl anomaly (pronounced: /uːl/), also known as Uhl's disease or Parchment heart disease, is a rare congenital heart disorder characterized by the partial or complete absence of the myocardium (heart muscle) in the right ventricle. The condition was first described by Henry Uhl, an American pathologist, in 1952.

Etymology

The term "Uhl anomaly" is derived from the name of the pathologist who first described the condition, Henry Uhl. The word "anomaly" comes from the Greek word 'anomalía' meaning 'irregularity' or 'deviation from the norm'.

Symptoms

Symptoms of Uhl anomaly can vary greatly depending on the severity of the condition. They may include shortness of breath, fatigue, arrhythmia, and heart failure. In severe cases, the condition can lead to sudden cardiac death.

Diagnosis

Diagnosis of Uhl anomaly is typically made through echocardiography, a type of ultrasound used to create images of the heart. Other diagnostic tests may include cardiac MRI and cardiac catheterization.

Treatment

Treatment for Uhl anomaly is typically focused on managing symptoms and preventing complications. This may include medications to manage heart failure and arrhythmias, as well as surgical interventions such as heart transplantation in severe cases.

Prognosis

The prognosis for individuals with Uhl anomaly can vary greatly depending on the severity of the condition and the individual's overall health. With appropriate treatment and management, many individuals with Uhl anomaly can lead relatively normal lives.

Related Terms

External links

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