Hypoplastic left heart syndrome: Difference between revisions
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{{ | {{SI}} | ||
{{Infobox medical condition | {{Infobox medical condition | ||
| name = Hypoplastic left heart syndrome | | name = Hypoplastic left heart syndrome | ||
| image = [[File:Hlhs-web.jpg|250px]] | |||
| image = Hlhs-web.jpg | | caption = Diagram of a heart with hypoplastic left heart syndrome | ||
| caption = | | synonyms = HLHS | ||
| pronounce = | | pronounce = | ||
| | | specialty = [[Cardiology]], [[Pediatric cardiology]] | ||
| symptoms = [[Cyanosis]], | | symptoms = [[Cyanosis]], [[dyspnea]], [[tachypnea]], [[poor feeding]] | ||
| complications = Heart failure, | | complications = [[Heart failure]], [[shock]], [[death]] | ||
| onset = | | onset = [[Neonatal]] | ||
| duration = Lifelong | | duration = Lifelong | ||
| causes = [[Congenital heart defect]] | |||
| causes = | | risks = [[Genetic factors]], [[environmental factors]] | ||
| risks = Genetic factors, | | diagnosis = [[Echocardiogram]], [[fetal ultrasound]] | ||
| diagnosis = | | differential = [[Other congenital heart defects]] | ||
| differential = Other | | prevention = None | ||
| prevention = | | treatment = [[Surgery]], [[heart transplant]] | ||
| treatment = | | medication = [[Prostaglandin E1]] | ||
| medication = Prostaglandin E1 | | prognosis = Variable, depends on treatment | ||
| prognosis = Variable | | frequency = 1 in 4,344 live births | ||
| frequency = | | deaths = | ||
| deaths = | |||
}} | }} | ||
'''Hypoplastic left heart syndrome''' (HLHS) is a rare [[congenital heart defect]] in which the left side of the heart is underdeveloped. This condition affects normal blood flow through the heart, as the left side of the heart is unable to effectively pump blood to the body. | '''Hypoplastic left heart syndrome''' (HLHS) is a rare [[congenital heart defect]] in which the left side of the heart is underdeveloped. This condition affects normal blood flow through the heart, as the left side of the heart is unable to effectively pump blood to the body. | ||
==Anatomy and Pathophysiology== | ==Anatomy and Pathophysiology== | ||
In HLHS, several structures on the left side of the heart are affected, including the [[left ventricle]], [[mitral valve]], [[aortic valve]], and [[aorta]]. The left ventricle is typically small and non-functional, the mitral and aortic valves may be stenotic or atretic, and the ascending aorta is often underdeveloped. | In HLHS, several structures on the left side of the heart are affected, including the [[left ventricle]], [[mitral valve]], [[aortic valve]], and [[aorta]]. The left ventricle is typically small and non-functional, the mitral and aortic valves may be stenotic or atretic, and the ascending aorta is often underdeveloped. | ||
The underdevelopment of these structures leads to a reliance on the [[right ventricle]] to pump blood to both the lungs and the rest of the body. Blood flow to the body is dependent on a patent [[ductus arteriosus]], a fetal blood vessel that connects the pulmonary artery to the aorta, which normally closes shortly after birth. | The underdevelopment of these structures leads to a reliance on the [[right ventricle]] to pump blood to both the lungs and the rest of the body. Blood flow to the body is dependent on a patent [[ductus arteriosus]], a fetal blood vessel that connects the pulmonary artery to the aorta, which normally closes shortly after birth. | ||
==Clinical Presentation== | ==Clinical Presentation== | ||
Newborns with HLHS may appear normal at birth but can rapidly develop symptoms as the ductus arteriosus begins to close. Symptoms include: | Newborns with HLHS may appear normal at birth but can rapidly develop symptoms as the ductus arteriosus begins to close. Symptoms include: | ||
| Line 37: | Line 33: | ||
* Lethargy | * Lethargy | ||
* Cold extremities | * Cold extremities | ||
Without intervention, HLHS is usually fatal within the first few days or weeks of life. | Without intervention, HLHS is usually fatal within the first few days or weeks of life. | ||
==Diagnosis== | ==Diagnosis== | ||
HLHS can be diagnosed prenatally using [[fetal echocardiography]]. Postnatal diagnosis is typically confirmed with an [[echocardiogram]], which can visualize the underdeveloped structures of the heart. Additional tests may include [[chest X-ray]] and [[electrocardiogram]] (ECG). | HLHS can be diagnosed prenatally using [[fetal echocardiography]]. Postnatal diagnosis is typically confirmed with an [[echocardiogram]], which can visualize the underdeveloped structures of the heart. Additional tests may include [[chest X-ray]] and [[electrocardiogram]] (ECG). | ||
==Treatment== | ==Treatment== | ||
Treatment for HLHS involves a series of surgeries or, in some cases, a [[heart transplant]]. The surgical approach, known as the Norwood procedure, is typically performed in three stages: | Treatment for HLHS involves a series of surgeries or, in some cases, a [[heart transplant]]. The surgical approach, known as the Norwood procedure, is typically performed in three stages: | ||
===Stage 1: Norwood Procedure=== | ===Stage 1: Norwood Procedure=== | ||
This surgery is performed shortly after birth. It involves reconstructing the aorta and connecting it to the right ventricle, allowing the right ventricle to pump blood to the body. | This surgery is performed shortly after birth. It involves reconstructing the aorta and connecting it to the right ventricle, allowing the right ventricle to pump blood to the body. | ||
===Stage 2: Glenn Procedure=== | ===Stage 2: Glenn Procedure=== | ||
Performed at 4-6 months of age, this procedure connects the superior vena cava to the pulmonary arteries, reducing the workload on the right ventricle. | Performed at 4-6 months of age, this procedure connects the superior vena cava to the pulmonary arteries, reducing the workload on the right ventricle. | ||
===Stage 3: Fontan Procedure=== | ===Stage 3: Fontan Procedure=== | ||
This final surgery, performed at 18 months to 3 years of age, connects the inferior vena cava to the pulmonary arteries, completing the separation of oxygenated and deoxygenated blood. | This final surgery, performed at 18 months to 3 years of age, connects the inferior vena cava to the pulmonary arteries, completing the separation of oxygenated and deoxygenated blood. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis for children with HLHS has improved significantly with advances in surgical techniques. However, these children require lifelong follow-up with a cardiologist and may face complications such as arrhythmias, heart failure, and the need for additional surgeries. | The prognosis for children with HLHS has improved significantly with advances in surgical techniques. However, these children require lifelong follow-up with a cardiologist and may face complications such as arrhythmias, heart failure, and the need for additional surgeries. | ||
==See also== | |||
== | |||
* [[Congenital heart defect]] | * [[Congenital heart defect]] | ||
* [[Echocardiography]] | * [[Echocardiography]] | ||
* [[Heart transplant]] | * [[Heart transplant]] | ||
{{Congenital malformations and deformations of circulatory system}} | {{Congenital malformations and deformations of circulatory system}} | ||
{{Channelopathy}} | {{Channelopathy}} | ||
Latest revision as of 00:38, 8 April 2025
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| Hypoplastic left heart syndrome | |
|---|---|
| |
| Synonyms | HLHS |
| Pronounce | |
| Specialty | Cardiology, Pediatric cardiology |
| Symptoms | Cyanosis, dyspnea, tachypnea, poor feeding |
| Complications | Heart failure, shock, death |
| Onset | Neonatal |
| Duration | Lifelong |
| Types | N/A |
| Causes | Congenital heart defect |
| Risks | Genetic factors, environmental factors |
| Diagnosis | Echocardiogram, fetal ultrasound |
| Differential diagnosis | Other congenital heart defects |
| Prevention | None |
| Treatment | Surgery, heart transplant |
| Medication | Prostaglandin E1 |
| Prognosis | Variable, depends on treatment |
| Frequency | 1 in 4,344 live births |
| Deaths | |
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is underdeveloped. This condition affects normal blood flow through the heart, as the left side of the heart is unable to effectively pump blood to the body.
Anatomy and Pathophysiology[edit]
In HLHS, several structures on the left side of the heart are affected, including the left ventricle, mitral valve, aortic valve, and aorta. The left ventricle is typically small and non-functional, the mitral and aortic valves may be stenotic or atretic, and the ascending aorta is often underdeveloped. The underdevelopment of these structures leads to a reliance on the right ventricle to pump blood to both the lungs and the rest of the body. Blood flow to the body is dependent on a patent ductus arteriosus, a fetal blood vessel that connects the pulmonary artery to the aorta, which normally closes shortly after birth.
Clinical Presentation[edit]
Newborns with HLHS may appear normal at birth but can rapidly develop symptoms as the ductus arteriosus begins to close. Symptoms include:
- Cyanosis (bluish skin color)
- Rapid breathing
- Poor feeding
- Lethargy
- Cold extremities
Without intervention, HLHS is usually fatal within the first few days or weeks of life.
Diagnosis[edit]
HLHS can be diagnosed prenatally using fetal echocardiography. Postnatal diagnosis is typically confirmed with an echocardiogram, which can visualize the underdeveloped structures of the heart. Additional tests may include chest X-ray and electrocardiogram (ECG).
Treatment[edit]
Treatment for HLHS involves a series of surgeries or, in some cases, a heart transplant. The surgical approach, known as the Norwood procedure, is typically performed in three stages:
Stage 1: Norwood Procedure[edit]
This surgery is performed shortly after birth. It involves reconstructing the aorta and connecting it to the right ventricle, allowing the right ventricle to pump blood to the body.
Stage 2: Glenn Procedure[edit]
Performed at 4-6 months of age, this procedure connects the superior vena cava to the pulmonary arteries, reducing the workload on the right ventricle.
Stage 3: Fontan Procedure[edit]
This final surgery, performed at 18 months to 3 years of age, connects the inferior vena cava to the pulmonary arteries, completing the separation of oxygenated and deoxygenated blood.
Prognosis[edit]
The prognosis for children with HLHS has improved significantly with advances in surgical techniques. However, these children require lifelong follow-up with a cardiologist and may face complications such as arrhythmias, heart failure, and the need for additional surgeries.
See also[edit]
| Congenital heart defects | ||||||
|---|---|---|---|---|---|---|
|
| Diseases of ion channels | ||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
See also: ion channels
|
