Hypoplastic right heart syndrome

From WikiMD's Wellness Encyclopedia

Hypoplastic right heart syndrome (HRHS) is a rare congenital heart defect in which the right side of the heart is underdeveloped. It affects the right ventricle and the valves that control the flow of blood between the right atrium and the right ventricle (the tricuspid and pulmonary valves).

Causes[edit | edit source]

The exact cause of HRHS is unknown, but it is believed to occur during the first 8 weeks of fetal development. Some research suggests that a combination of genes and environmental factors may play a role. Certain conditions, such as diabetes in the mother, have been associated with a higher risk of having a child with HRHS.

Symptoms[edit | edit source]

Symptoms of HRHS can vary greatly depending on the severity of the condition. They may include rapid breathing, difficulty feeding, lethargy, and a bluish color to the skin, lips, and nails (known as cyanosis) due to low oxygen levels in the blood. In severe cases, HRHS can lead to heart failure.

Diagnosis[edit | edit source]

HRHS is typically diagnosed through an echocardiogram, which uses sound waves to create a detailed image of the heart's structure and function. Other tests, such as a cardiac catheterization, may be used to confirm the diagnosis and assess the severity of the condition.

Treatment[edit | edit source]

Treatment for HRHS typically involves multiple surgeries to re-route the blood flow around the underdeveloped right side of the heart. The first surgery, known as the Norwood procedure, is usually performed within the first week of life. Subsequent surgeries, including the Glenn procedure and the Fontan procedure, are performed over the next several years.

Prognosis[edit | edit source]

With early diagnosis and treatment, many children with HRHS can lead relatively normal lives. However, they will require lifelong medical care and may have limitations on their physical activity.

See also[edit | edit source]




Cardiovascular disease A-Z

Most common cardiac diseases

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Hypoplastic right heart syndrome Resources
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Contributors: Prab R. Tumpati, MD