Double Inlet Left Ventricle

Overview

What is double inlet left ventricle?

Double inlet left ventricle is a congenital heart defect, meaning that it is present at birth. In this condition, the upper collecting chambers in the heart — the left and right atria (plural for atrium) — are connected to the same lower pumping chamber or ventricle. In some cases, one of the ventricles of the heart may be extremely small.

The human heart has four chambers: the left atrium and right atrium (plural: atria) at the top, and the left and right ventricles on the bottom. Blood circulates through the atria and the ventricles to and from the rest of the body via arteries (which carry blood away from the heart) and veins (which carry blood to the heart).

In a normal heart, blood that needs oxygen is received in the right atrium from large veins called vena cava, then enters the right ventricle which pumps the blood to the lungs via the pulmonary artery to pick up oxygen. Blood that has picked up oxygen from the lungs returns to the heart at the left atrium where it is collected and passed into the left ventricle that then pumps this oxygenated blood through the a large artery called the aorta to the rest of the body.

Babies with double inlet left ventricle usually have a normal left ventricle (the pumping chamber of the heart that pumps blood to the body) and a small right ventricle (the pumping chamber that pumps blood to the lungs). Both atria supply blood to the left ventricle. As a result, oxygen-rich blood and oxygen-poor blood are combined. This mixture of blood flows to the baby’s lungs and body. Double inlet left ventricle is also called common ventricle or single ventricle.

Symptoms and Causes

What causes double inlet left ventricle?

The cause of double inlet left ventricle is unknown, but the defect is likely to occur early in a pregnancy while the baby’s heart is developing.

What are the symptoms of double inlet left ventricle?

Symptoms of double inlet left ventricle usually appear in the early weeks of a baby’s life and include:

  • Blue discoloration of the skin (cyanosis).
  • Failure to gain weight normally.
  • Trouble breathing.
  • Poor feeding.
  • Sweating.
  • Fast heartbeat.
  • Heart murmur (an unusual heart sound).
  • Heart failure.

Many babies with double inlet left ventricle have other abnormalities in the heart or the main arteries. These abnormalities can block the flow of blood to the lungs.

When should I call the doctor?

Call your doctor if your child:

  • Is having trouble breathing.
  • Isn’t eating well.
  • Appears to become easily tired.
  • Has skin or lips that are bluish in color.
  • Isn’t growing or gaining weight.

Diagnosis and Tests

How is double inlet left ventricle diagnosed?

Advancements in the care of pregnant women and accurate assessment of the heart in the developing fetus, many of these patients are now diagnosed before they are born. This allows for proper counseling for the family and preparations being made for when and where the baby is delivered as well as multiple visits to assess the health of the baby prior to delivery. After birth, the doctor will perform a physical examination and listen to the baby’s heartbeat with a stethoscope and the baby’s vital signs will be assessed. The doctor may also order various tests, including:

  • An electrocardiogram to measure the heart’s electrical activity.
  • An echocardiogram, or ultrasound imaging of the heart using sound waves.
  • A cardiac catheterization may be performed in some cases. This is a procedure performed under anesthesia in which slender tubes are inserted into blood vessels in the groin and moved toward the heart to measure the function of the heart and blood flow in the heart and lung.
  • Sometimes additional imaging such as CT scans or magnetic resonance imaging (MRI) may be needed to assess anatomy and function.

Management and Treatment

How is double inlet left ventricle treated?

Double inlet left ventricle is treated with surgery to repair the defect. Several surgeries may be needed in order to make the heart work effectively. In most cases, the heart can be repaired to the point where the child can lead a relatively normal life.

With surgery, blood circulation through the body and into the lungs can be improved. Your baby may have a number of surgeries in order to have a surgery called the Fontan procedure.

If blood flow to the pulmonary artery is restricted, a small tube called a shunt can be surgically placed from an artery attached to the aorta to the pulmonary artery. If there is too much blood flow to the lungs, the surgeon may perform a pulmonary artery banding, which protects the pulmonary arteries from high blood pressure.

The first surgery may have to be performed within the first few days of your baby’s life. Your baby usually goes home after the first surgery. A pediatric cardiologist will decide when your child should undergo the second surgery. The follow-up surgery (or initial surgery if your baby was not operated on in the first few days of life) is called a Glenn shunt procedure. This operation usually is performed when your baby is 4 to 6 months old.

The final procedure is the Fontan procedure and usually is done when your child is 3 to 6 years old. After the final stage of surgery, the baby’s oxygen levels will be similar to a child with a normal heart. Although the Fontan procedure doesn’t result in normal blood circulation in the body, it does improve the circulation and allows your child to grow.

Your child may need additional types of surgeries for related defects or to extend survival while waiting for the Fontan procedure. The Fontan procedure has risks and complications, which your child’s doctor will discuss with you.

Your child’s doctor may prescribe medication before and after surgery. These medications may include:

  • Digoxin to help with heart contractions.
  • ACE inhibitors to lower blood pressure.
  • Anticoagulants to prevent blood clots.
  • Diuretics to remove extra fluid from the body.

In addition, children with double inlet left ventricle have to take antibiotics before dental treatment in order to prevent endocarditis (infection of the heart lining).

In the most severe cases of double inlet left ventricle, a surgeon may recommend a heart transplant for your child.

Prevention

How can double inlet left ventricle be prevented?

Since the cause of double inlet left ventricle is not known, there is no absolute way to prevent this from happening. However, in general, you can begin your healthy pregnancy even before you are pregnant by doing things to take care of yourself. For instance:

  • Visit your healthcare provider before you get pregnant to find out how healthy you are and to share your family’s health history.
  • Eat healthy foods and exercise regularly to get to or keep a healthy weight.
  • Take a 400 mcg folic acid supplement to prevent spine and brain defects.
  • If you smoke, stop. The same goes for drinking and/or using prescription or street drugs. These things can hurt your baby if you become pregnant.

Outlook / Prognosis

What is the prognosis (outlook) for children who have double inlet left ventricle?

Factors that affect how well a baby with double inlet left ventricle does include:

  • How severe the defect is.
  • The overall condition of the baby when the diagnosis is made.
  • Whether other heart defects are present.
  • Genetic syndromes.

Many babies with double inlet left ventricle live to adulthood as a result of advances in surgical procedures. Many people with double inlet left ventricle need regular follow-ups. In addition, they can face various complications and their physical activity can be limited.

Last reviewed by a Cleveland Clinic medical professional on 10/28/2020.

References

  • Lan YT, Chang RK, Laks H. Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries. J Am Coll Cardiol. 2004;43(1):113-9.
  • Tiny Tickers. Accessed 11/5/2020.Double inlet left ventricle (DILV). (https://www.tinytickers.org/support-info/different-types-congenital-heart-defects/double-inlet-left-ventricle-dilv/)
  • March of Dimes. . Accessed 11/5/2020.Congenital heart defects and critical CHDs (https://www.marchofdimes.org/complications/congenital-heart-defects.aspx)

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