Double inlet left ventricle: Difference between revisions

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'''Double Inlet Left Ventricle''' ('''DILV''') is a rare congenital heart defect characterized by both atria (the two upper chambers of the heart) connecting to the same ventricle. Typically, the heart has four chambers: two atria and two ventricles. In DILV, the right ventricle is underdeveloped, and the left ventricle receives blood from both atria, leading to a single functional pumping chamber. This condition is part of a group of defects known as single ventricle defects.
{{SI}}
 
{{Infobox medical condition
==Etiology==
| name                    = Double inlet left ventricle
The exact cause of DILV is unknown, but it is believed to involve genetic and environmental factors. It occurs early in fetal development when the heart is forming. Some genetic syndromes and maternal conditions have been associated with an increased risk of congenital heart defects, including DILV.
| image                  = [[File:HLHS.jpg|250px]]
 
| caption                = Heart with double inlet left ventricle
==Pathophysiology==
| synonyms                = DILV
In a normal heart, the right atrium receives deoxygenated blood from the body and pumps it into the right ventricle, which then sends it to the lungs to be oxygenated. The left atrium receives oxygenated blood from the lungs and pumps it into the left ventricle, which then distributes it to the rest of the body. In DILV, the left ventricle performs the function of pumping both the oxygenated and deoxygenated blood, leading to mixing of blood and inefficient circulation.
| pronunciation          =
 
| specialty              = [[Cardiology]], [[Pediatric cardiology]]
==Clinical Presentation==
| symptoms                = [[Cyanosis]], [[shortness of breath]], [[fatigue]], [[poor feeding]]
Symptoms of DILV may vary depending on the presence and severity of associated defects, such as [[Pulmonary Stenosis|pulmonary stenosis]] or [[Transposition of the Great Arteries|transposition of the great arteries]]. Common symptoms include cyanosis (a bluish tint to the skin, lips, and nails), difficulty breathing, poor feeding, and failure to thrive.
| complications          = [[Heart failure]], [[arrhythmias]], [[pulmonary hypertension]]
 
| onset                  = [[Congenital]]
==Diagnosis==
| duration                = Lifelong
Diagnosis of DILV is typically made through echocardiography, which provides detailed images of the heart's structure and function. Other diagnostic tests may include electrocardiogram (ECG), chest X-ray, and cardiac MRI.
| types                  =
 
| causes                  = [[Congenital heart defect]]
==Treatment==
| risks                  = [[Genetic factors]], [[maternal diabetes]], [[maternal alcohol use]]
Treatment for DILV often involves multiple surgeries performed in stages. The goal is to improve the heart's function and blood flow, which may involve creating a pathway for the blood to flow more effectively between the heart and lungs. The most common surgical approach is the staged [[Fontan Procedure|Fontan procedure]], which is completed in three stages: the Norwood procedure, the Glenn procedure, and the Fontan completion.
| diagnosis              = [[Echocardiogram]], [[cardiac MRI]], [[cardiac catheterization]]
 
| differential            = [[Tricuspid atresia]], [[single ventricle defects]]
==Prognosis==
| prevention              =
The prognosis for individuals with DILV varies. Advances in surgical techniques and postoperative care have significantly improved outcomes, allowing many patients to lead active lives. However, long-term complications can include heart rhythm problems, heart failure, and the need for heart transplantation.
| treatment              = [[Surgical intervention]], [[medication]]
 
| medication              = [[Diuretics]], [[ACE inhibitors]], [[beta blockers]]
==See Also==
| prognosis              = Variable, depends on severity and treatment
* [[Congenital Heart Defect]]
| frequency              = Rare
* [[Hypoplastic Left Heart Syndrome]]
| deaths                  =
* [[Fontan Procedure]]
}}
 
{{Short description|Overview of Double Inlet Left Ventricle (DILV)}}
[[Category:Cardiology]]
'''Double Inlet Left Ventricle''' (DILV) is a rare congenital heart defect characterized by both atria connecting to a single ventricle, typically the left ventricle. This condition is a type of [[single ventricle defect]], which affects the normal flow of blood through the heart.
[[Category:Congenital heart defects]]
=== Anatomy and Physiology ===
[[Category:Pediatric cardiology]]
In a normal heart, the [[right atrium]] and [[left atrium]] are connected to the right and left ventricles, respectively. However, in DILV, both atria are connected to the left ventricle. The right ventricle is often underdeveloped or hypoplastic. This abnormality leads to a mixing of oxygenated and deoxygenated blood, which can result in [[cyanosis]] and other complications.
 
=== Pathophysiology ===
{{Medicine-stub}}
The mixing of blood in the left ventricle causes the body to receive less oxygenated blood, leading to symptoms such as [[cyanosis]], [[fatigue]], and [[shortness of breath]]. Over time, this can lead to complications such as [[heart failure]] and [[pulmonary hypertension]].
=== Diagnosis ===
DILV is typically diagnosed using [[echocardiography]], which allows visualization of the heart's structure and function. Other diagnostic tools may include [[MRI]] and [[cardiac catheterization]].
=== Treatment ===
Treatment for DILV often involves surgical intervention. The [[Fontan procedure]] is a common surgical approach used to redirect blood flow and improve oxygenation. Other procedures may include the [[Glenn procedure]] or [[Norwood procedure]], depending on the specific anatomy and needs of the patient.
=== Prognosis ===
The prognosis for individuals with DILV varies depending on the severity of the defect and the success of surgical interventions. With advances in surgical techniques, many individuals can lead relatively normal lives, although they may require ongoing medical care and monitoring.
== See also ==
* [[Congenital heart defect]]
* [[Hypoplastic left heart syndrome]]
* [[Single ventricle defect]]
* [[Fontan procedure]]
[[Category:Congenital heart disease]]

Latest revision as of 19:19, 5 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
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Double inlet left ventricle
Synonyms DILV
Pronounce N/A
Specialty Cardiology, Pediatric cardiology
Symptoms Cyanosis, shortness of breath, fatigue, poor feeding
Complications Heart failure, arrhythmias, pulmonary hypertension
Onset Congenital
Duration Lifelong
Types
Causes Congenital heart defect
Risks Genetic factors, maternal diabetes, maternal alcohol use
Diagnosis Echocardiogram, cardiac MRI, cardiac catheterization
Differential diagnosis Tricuspid atresia, single ventricle defects
Prevention
Treatment Surgical intervention, medication
Medication Diuretics, ACE inhibitors, beta blockers
Prognosis Variable, depends on severity and treatment
Frequency Rare
Deaths


Overview of Double Inlet Left Ventricle (DILV)


Double Inlet Left Ventricle (DILV) is a rare congenital heart defect characterized by both atria connecting to a single ventricle, typically the left ventricle. This condition is a type of single ventricle defect, which affects the normal flow of blood through the heart.

Anatomy and Physiology[edit]

In a normal heart, the right atrium and left atrium are connected to the right and left ventricles, respectively. However, in DILV, both atria are connected to the left ventricle. The right ventricle is often underdeveloped or hypoplastic. This abnormality leads to a mixing of oxygenated and deoxygenated blood, which can result in cyanosis and other complications.

Pathophysiology[edit]

The mixing of blood in the left ventricle causes the body to receive less oxygenated blood, leading to symptoms such as cyanosis, fatigue, and shortness of breath. Over time, this can lead to complications such as heart failure and pulmonary hypertension.

Diagnosis[edit]

DILV is typically diagnosed using echocardiography, which allows visualization of the heart's structure and function. Other diagnostic tools may include MRI and cardiac catheterization.

Treatment[edit]

Treatment for DILV often involves surgical intervention. The Fontan procedure is a common surgical approach used to redirect blood flow and improve oxygenation. Other procedures may include the Glenn procedure or Norwood procedure, depending on the specific anatomy and needs of the patient.

Prognosis[edit]

The prognosis for individuals with DILV varies depending on the severity of the defect and the success of surgical interventions. With advances in surgical techniques, many individuals can lead relatively normal lives, although they may require ongoing medical care and monitoring.

See also[edit]

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