Double inlet left ventricle
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Obesity, Sleep & Internal medicine
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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
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
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.
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Contributors: Prab R. Tumpati, MD