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

Most common cardiac diseases

• Cardiac arrhythmia‎
• Cardiogenetic disorders‎
• Cardiomegaly‎
• Cardiomyopathy‎
• Cardiopulmonary resuscitation‎
• Chronic rheumatic heart diseases‎
• Congenital heart defects‎
• Heart neoplasia‎
• Ischemic heart diseases‎
• Pericardial disorders
• Syndromes affecting the heart‎
• Valvular heart disease‎

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

• Accelerated idioventricular rhythm
• Acute decompensated heart failure
• Arteriosclerotic heart disease
• Athletic heart syndrome
• Atrial flutter
• Atrioventricular fistula
• Cardiovascular disease in Australia
• Autoimmune heart disease

B

• Brown atrophy of the heart

C

• Ebb Cade
• Cardiac allograft vasculopathy
• Cardiac amyloidosis
• Cardiac asthma
• Cardiac tamponade
• Cardiogenic shock
• Cardiogeriatrics
• Cardiorenal syndrome
• Cardiotoxicity
• Carditis
• Coronary artery aneurysm
• Coronary artery anomaly
• Coronary artery disease
• Spontaneous coronary artery dissection
• Coronary artery ectasia
• Coronary occlusion
• Coronary steal
• Coronary thrombosis
• Coronary vasospasm
• Cœur en sabot
• Coxsackievirus-induced cardiomyopathy

D

• Duke Treadmill Score
• Duroziez's disease

E

• Eisenmenger's syndrome
• Embryocardia
• Endocardial fibroelastosis
• Eosinophilic myocarditis

H

• Heart attack
• Heart failure
• Heart failure with preserved ejection fraction
• Heart to Heart (1949 film)
• High-output heart failure
• Hyperdynamic precordium

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
• Interventricular dyssynchrony
• Intraventricular dyssynchrony
• Isolated atrial amyloidosis

K

• Keshan disease

L

• Loeffler endocarditis

M

• Mydicar
• Myocardial bridge
• Myocardial disarray
• Myocardial rupture
• Myocardial scarring
• Myocardial stunning
• Myocarditis

N

• Nonbacterial thrombotic endocarditis

O

• OLR1
• Ostial disease

P

• Papillary fibroelastoma
• Pathophysiology of heart failure
• Postpericardiotomy syndrome
• Pulmonary vein stenosis

R

• Right axis deviation
• Roemheld syndrome

S

• Saturated fat and cardiovascular disease
• SCAR-Fc
• Shone's syndrome
• Strain pattern
• Subacute bacterial endocarditis
• Sudden cardiac death of athletes

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

T

• Traumatic cardiac arrest

V

• Ventricular aneurysm
• Vibroacoustic disease
• Viral cardiomyopathy

W

• Waon therapy

Pediatric glossary of terms | Glossary of medical terms | Dictionary of pediatrics

Hypoplastic right heart syndrome Resources
Need Help Finding a Doctor? Need help finding a doctor or specialist anywhere in the world? Explore our world directory. WikiMD's DocFinder can assist you in locating millions of physicians.	Medical Knowledge Access the latest research - Most recent articles Ongoing trials.

Translate to: East Asian 中文, 日本, 한국어, South Asian हिन्दी, Urdu, বাংলা, తెలుగు, தமிழ், ಕನ್ನಡ,
Southeast Asian Indonesian, Vietnamese, Thai, မြန်မာဘာသာ, European español, Deutsch, français, русский, português do Brasil, Italian, polski