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Double inlet left ventricle

Definition

Double inlet left ventricle (DILV) is a congenital heart defect that affects the valves and chambers of the heart. Congenital means it is present from birth. Babies born with this condition have only one working lower chamber (ventricle) in their heart.

Alternative Names

DILV; Single ventricle; Common ventricle

Causes, incidence, and risk factors

DILV is one of several heart defects known as single (or common) ventricle defects. People with this condition generally have a large left ventricle (the pumping chamber of the heart that supplies the body with blood), and a small right ventricle (the pumping chamber that supplies the lungs with blood).

In the normal heart, the right and left lower chambers (ventricles) receive blood from the right and left upper chambers (atria). Oxygen-poor blood returning from the body flows to the right atrium and right ventricle. The right ventricle then pumps blood to the pulmonary artery, which carries blood to the lungs to pick up oxygen.

The blood, now oxygen rich, returns to the left atrium and left ventricle. The aorta than carries oxygen-rich blood to the rest of the body from the left ventricle.

However, in those with DILV, only the left lower heart chamber is developed, and both upper chambers carry blood into this ventricle. This means that oxygen-rich blood mixes with oxygen-poor blood. The mixture is then carried into the body and the lungs.

DILV can occur with transposition of the great vessels (in which the aorta arises from the small right ventricle and the pulmonary artery arises from the left ventricle), or it can occur with the arteries arising from their normal positions. Blood flows from the left to right ventricle through a ventricular septal defect (VSD) -- a hole between the chambers.

Double inlet left ventricle occurs in about 5 - 10 of 100,000 live births. The problem most likely occurs early in the pregnancy, when the baby's heart develops. However, the exact cause of DILV is unknown.

Patients with DILV often also have other heart problems, such as:

Symptoms

Symptoms of DILV may include:

  • Becoming tired easily, especially during feeding
  • Bluish skin color (the lips may also be blue)
  • Failure to gain weight and grow
  • Pale skin (pallor)
  • Poor feeding
  • Sweating
  • Swollen legs or abdomen
  • Trouble breathing

Signs and tests

Signs of DILV may include:

  • Abnormal heart rhythm, as seen on an electrocardiogram
  • Build-up of fluid around the lungs
  • Heart failure
  • Heart murmur
  • Rapid heartbeat

Tests to diagnose DILV may include:

  • Measurement of the electrical activity in the heart (electrocardiogram, or ECG)
  • Passing a thin, flexible tube into the heart to examine the arteries (cardiac catheterization)
  • Ultrasound exam of the heart (echocardiogram)
  • Using magnets to create images of the heart (MRI)

Treatment

Surgery is needed to improve blood circulation through the body and into the lungs. The most common surgeries to treat DILV are the ones leading up to the Fontan operation, which may require several stages. (Each stage in the Fontan procedure is a separate surgery.)

These surgeries are similar to the ones used to treat hypoplastic left heart syndrome and tricuspid atresia.

The first surgery may be needed within the baby's first few days of life. Afterward, the baby will usually go home. The child will need to take one or more daily medications and be closely followed by a pediatric cardiologist, who will decide when the second stage of surgery should be done.

The next surgery (or first surgery, if the baby didn't need the procedure mentioned above) is called the bidirectional Glenn shunt or Hemifontan procedure. This surgery is usually done when the child is 4 - 6 months old.

After the child has had the above operations, he or she may still look blue (cyanotic). The final step is called the Fontan procedure. This surgery is usually performed when the child is 18 months - 3 years old. After this final step, the baby is no longer cyanotic.

The Fontan operation does not create normal circulation in the body, but it creates the type of circulation a child can live and grow with. However, even this surgery carries many risks and complications.

A child may need additional types of surgeries for related defects or to extend survival while waiting for the Fontan procedure.

The doctor may prescribe your child medication before and after surgery. Medications may include:

  • Anticoagulants to prevent blood clotting
  • ACE inhibitors to reduce blood pressure
  • Digoxin to help the heart contract

For the most severe cases of DILV, a heart transplant may be recommended.

Children with this congenital heart disease should take antibiotics before dental treatment. This prevents infections around the heart.

See also: Pediatric heart surgery

Expectations (prognosis)

DILV is usually a very complex, hard-to-treat heart defect. How well the baby does depends on several factors. They include:

  • Baby's overall condition at the time of diagnosis and treatment
  • Presence of other heart abnormalities
  • Severity of the defect

Advances in surgical techniques allow many infants with DILV to reach adulthood. However, many of these children and adults require regular follow-ups, face many complications, and may be limited in the type of physical activities they can pursue.

Complications

Complications of DILV include:

  • Clubbing (thickening of the nail beds) on toes and fingers (late sign)
  • Congestive heart failure
  • Death
  • Frequent pneumonia
  • Heart rhythm problems
  • High blood pressure in the lungs
  • Lung damage

Calling your health care provider

Call your health care provider if your child seems to tire easily, has trouble breathing, or has bluish skin or lips. You should also consult your health care provider if your baby is not growing or gaining weight.

Prevention

There is no known prevention.

References

Park MK. Park: Pediatric Cardiology for Practitioners, 5th ed. Philadelphia, PA: Mosby Elsevier; 2008:283-287:chap 14.

Kliegman RM, Behrman RE, Jenson HB, Stanton BF. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007.

Townsend Jr. CM, Beauchamp RD, Evers BM, et al, eds. Townsend: Sabiston Textbook of Surgery, 18th ed. Philadelphia, PA: Saunders Elsevier; 2008:1777-1783: chap 43.


Review Date: 4/30/2010
Reviewed By: Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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