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[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
- 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[edit source]
- 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[edit source]
C[edit source]
- 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[edit source]
E[edit source]
H[edit source]
- 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[edit source]
- Idiopathic giant-cell myocarditis
- Interventricular dyssynchrony
- Intraventricular dyssynchrony
- Isolated atrial amyloidosis
K[edit source]
L[edit source]
M[edit source]
- Mydicar
- Myocardial bridge
- Myocardial disarray
- Myocardial rupture
- Myocardial scarring
- Myocardial stunning
- Myocarditis
N[edit source]
O[edit source]
P[edit source]
- Papillary fibroelastoma
- Pathophysiology of heart failure
- Postpericardiotomy syndrome
- Pulmonary vein stenosis
R[edit source]
S[edit source]
- 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[edit source]
V[edit source]
W[edit source]
Pediatric glossary of terms | Glossary of medical terms | Dictionary of pediatrics
| Hypoplastic right heart syndrome Resources | |
|---|---|
|
 |
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available.
Advertise on WikiMD
|
WikiMD's Wellness Encyclopedia |
| Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD
