Hypoplastic right heart syndrome

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Hypoplastic right heart syndrome
Synonyms	HRHS
Pronounce
Specialty	Cardiology, Pediatric cardiology
Symptoms	Cyanosis, dyspnea, fatigue, poor feeding
Complications	Heart failure, arrhythmias, pulmonary hypertension
Onset	Congenital
Duration	Lifelong
Types
Causes	Congenital heart defect
Risks	Genetic factors, maternal diabetes, maternal smoking
Diagnosis	Echocardiogram, fetal ultrasound, cardiac MRI
Differential diagnosis	Tetralogy of Fallot, tricuspid atresia, pulmonary atresia
Prevention
Treatment	Surgery, medication, heart transplant
Medication	Prostaglandin E1, diuretics, ACE inhibitors
Prognosis	Variable, depends on severity and treatment
Frequency	Rare
Deaths

Hypoplastic Right Heart Syndrome

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

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

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

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

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

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

• Congenital heart defect
• Heart failure
• Norwood procedure
• Glenn procedure
• Fontan procedure

Cardiovascular disease A-Z

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A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z

A

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B

• Brown atrophy of the heart

C

• Ebb Cade
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D

• Duke Treadmill Score
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E

• Eisenmenger's syndrome
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H

• Heart attack
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A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z

I

• Idiopathic giant-cell myocarditis
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K

• Keshan disease

L

• Loeffler endocarditis

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• Nonbacterial thrombotic endocarditis

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• OLR1
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R

• Right axis deviation
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• Saturated fat and cardiovascular disease
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T

• Traumatic cardiac arrest

V

• Ventricular aneurysm
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W

• Waon therapy

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