DOUBLE-INLET LEFT VENTRICLE

Double-inlet left ventricle (DILV) is a rare congenital heart defect where both the right and left atria connect to a single, enlarged left ventricle. This means that the right ventricle is either small or absent. DILV is typically diagnosed during infancy or early childhood.

In DILV, the left ventricle receives blood from both the right and left atria. The oxygen-rich blood from the lungs mixes with the oxygen-poor blood returning from the body. The single ventricle then pumps a combination of oxygenated and deoxygenated blood to the body.

DILV is often accompanied by other heart defects, such as a ventricular septal defect (VSD) or atrial septal defect (ASD). These defects allow blood to flow between the left and right sides of the heart, causing further mixing of oxygenated and deoxygenated blood.

Infants with DILV may exhibit symptoms such as rapid breathing, poor feeding, cyanosis (bluish skin color), and failure to thrive. Older children may experience fatigue, exercise intolerance, and recurrent respiratory infections.

TYPES

There are two main types of Double-Inlet Left Ventricle (DILV), known as DILV with transposition of the great arteries (TGA) and DILV with normally related great arteries. Here’s an overview of each type:

1. DILV with Transposition of the Great Arteries (TGA)

In this type, the blood vessels that carry blood away from the heart, namely the aorta and pulmonary artery, are switched. The aorta is connected to the right ventricle, which pumps deoxygenated blood to the body, while the pulmonary artery is connected to the left ventricle, which pumps oxygenated blood to the lungs. This leads to a mixed circulation of oxygenated and deoxygenated blood.

2. DILV with Normally Related Great Arteries

In this type, the aorta and the pulmonary artery are in their usual positions, with the aorta connected to the left ventricle and the pulmonary artery connected to the right ventricle. However, both the right and left atria still connect to the left ventricle. The left ventricle then receives blood from both atria, resulting in a mixture of oxygenated and deoxygenated blood being pumped to the body.

It’s important to note that DILV can also be associated with other heart defects, such as ventricular septal defects (VSDs) or atrial septal defects (ASDs), which further complicate the circulation of blood within the heart.

The specific type of DILV, along with associated abnormalities, anatomical variations, and the overall condition of the individual, will influence the treatment approach and long-term management. Consulting with a pediatric cardiologist is crucial to obtain an accurate diagnosis, determine the appropriate treatment plan, and provide ongoing care for individuals with DILV.

SYMPTOMS

The symptoms of Double-Inlet Left Ventricle (DILV) can vary depending on the severity of the defect and associated abnormalities. Here are some common symptoms that may be observed in individuals with DILV:

  • Cyanosis: Infants with DILV may have a bluish tint to their skin, lips, or nails due to insufficient oxygenation of the blood.
  • Rapid breathing: Infants may exhibit rapid and labored breathing, which can be a sign of the heart’s inability to pump oxygenated blood effectively.
  • Poor feeding: Infants may have difficulty with feeding, such as tiring easily during feeds or having trouble gaining weight.
  • Failure to thrive: Infants may not grow at the expected rate and may have difficulty reaching developmental milestones.
  • Fatigue and exercise intolerance: As children with DILV grow older, they may experience fatigue and have difficulty keeping up with physical activities due to inadequate oxygen supply to the body.
  • Recurrent respiratory infections: Individuals with DILV may be more prone to respiratory infections, such as pneumonia or bronchitis, due to compromised lung function.

It’s important to note that these symptoms can also be present in other heart conditions, and a proper diagnosis by a healthcare professional, such as a pediatric cardiologist, is necessary to determine the underlying cause and develop an appropriate treatment plan.

If you or someone you know is experiencing any symptoms or concerns related to DILV, it is advisable to seek medical attention promptly for a thorough evaluation and guidance.

DIAGNOSIS

The diagnosis of Double-Inlet Left Ventricle (DILV) is typically made through a combination of medical history evaluation, physical examinations, imaging tests, and diagnostic procedures. Here are some common methods used for diagnosing DILV:

  1. Physical examination: A healthcare professional, such as a pediatric cardiologist, will perform a thorough physical examination to assess any signs or symptoms of DILV. This may include listening to the heart sounds, checking for abnormal heart rhythms, and assessing oxygen saturation levels.
  2. Echocardiography: This is the primary imaging test used to diagnose DILV. It uses ultrasound waves to create detailed images of the heart’s structure and function. Echocardiography can help visualize abnormalities in the heart chambers, valves, and blood flow patterns.
  3. Fetal echocardiography: If DILV is suspected during pregnancy, a specialized ultrasound called fetal echocardiography can be performed to evaluate the baby’s heart structure and function.
  4. Cardiac catheterization: In some cases, a cardiac catheterization may be performed to further evaluate the anatomy and function of the heart. During this procedure, a thin tube is inserted into a blood vessel and guided to the heart, allowing for measurements of pressures and blood flow.
  5. Additional tests: Depending on the specific case, additional tests such as magnetic resonance imaging (MRI), computed tomography (CT), or genetic testing may be ordered to gather more information about the condition and associated abnormalities.

It’s important to consult with a healthcare professional experienced in congenital heart defects, such as a pediatric cardiologist, for an accurate diagnosis. They will guide you through the necessary tests and evaluations to determine the presence of DILV and develop an appropriate treatment plan.

TREATMENT

The treatment for Double-Inlet Left Ventricle (DILV) depends on several factors, including the severity of the condition, associated abnormalities, and the overall health of the individual. Treatment options may include:

1. Medications: Medications may be prescribed to help manage symptoms and improve heart function. These may include medications to regulate heart rhythm, lower blood pressure, or improve the efficiency of the heart’s pumping action.

2. Surgical interventions: Surgery is often necessary to correct the anatomical abnormalities associated with DILV. The specific surgical procedures will depend on the individual case, but they may include:

  • Fontan procedure: This surgery redirects blood flow from the lower body directly to the lungs, bypassing the heart chambers.
  • Pulmonary artery banding: A band is placed around the pulmonary artery to restrict blood flow and reduce the workload on the heart.
  • Glenn procedure: This surgery connects the superior vena cava directly to the pulmonary artery, allowing oxygen-poor blood from the upper body to bypass the heart.

3. Heart transplantation: In severe cases where the heart’s function is severely compromised or other treatments are not effective, a heart transplant may be considered. A donor heart is surgically implanted to replace the diseased heart.

4. Follow-up care: Individuals with DILV will require lifelong follow-up care with a team of specialists, including pediatric cardiologists, cardiac surgeons, and other healthcare professionals. Regular check-ups, diagnostic tests, and monitoring of heart function will be necessary to ensure the best possible outcomes.

It’s important to note that the treatment approach for DILV will be individualized to each person’s specific needs. Consulting with a healthcare professional experienced in congenital heart defects, such as a pediatric cardiologist, is crucial to determine the most appropriate treatment plan for an individual with DILV.

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