Hypoplastic right heart syndrome
Hypoplastic Right Heart Syndrome (pronounced: hi-po-plas-tik rait hart sin-drohm) is a rare congenital heart defect in which the right side of the heart is underdeveloped. It affects the right atrium, right ventricle, pulmonary valve, and pulmonary artery.
Etymology
The term "Hypoplastic Right Heart Syndrome" is derived from the Greek word 'hypo' meaning 'under', 'plasticos' meaning 'formed', and the Latin word 'syndroma' meaning 'running together'.
Symptoms
Symptoms of Hypoplastic Right Heart Syndrome may include cyanosis (a bluish tint to the skin, lips, and fingernails), shortness of breath, and rapid breathing. Infants with this condition may also have difficulty feeding and fail to gain weight at the expected rate.
Causes
The exact cause of Hypoplastic Right Heart Syndrome is unknown, but it is believed to occur during the first 8 weeks of fetal development. Some researchers believe that a combination of genes and environmental factors may play a role in the development of this condition.
Diagnosis
Diagnosis of Hypoplastic Right Heart Syndrome typically occurs during pregnancy through a routine ultrasound or after birth if the infant shows symptoms. Further diagnostic tests may include an echocardiogram, cardiac catheterization, and MRI.
Treatment
Treatment for Hypoplastic Right Heart Syndrome typically involves multiple surgeries to redirect the flow of blood through the heart. The first surgery, known as the Norwood procedure, is usually performed within the first week of life. Subsequent surgeries include the Glenn procedure and the Fontan procedure.
Prognosis
The prognosis for individuals with Hypoplastic Right Heart Syndrome varies. With early diagnosis and treatment, many individuals can lead relatively normal lives. However, they will require lifelong medical care and may have limitations on their physical activity.
See also
External links
- Medical encyclopedia article on Hypoplastic right heart syndrome
- Wikipedia's article - Hypoplastic right heart syndrome
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