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 pumping chamber (ventricle) in their heart.
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 then 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 pumped into both 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" ventricles. 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 of DILV may include:
Signs of DILV may include:
Tests to diagnose DILV may include:
Surgery is needed to improve blood circulation through the body and into the lungs. The most common surgeries to treat DILV are a series of two to three surgeries. 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 usually need to take 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.
A child may need more 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:
For the most severe cases of DILV, a heart transplant may be recommended.
DILV is usually a very complex, hard-to-treat heart defect. How well the baby does depends on several factors, including:
Advances in surgical techniques allow many infants with DILV to reach adulthood. However, 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 of DILV include:
DILV; Single ventricle; Common ventricle
Baldwin HS, Dees E. Embryology and physiology of the cardiovascular system. In: Gleason CA, Devaskar S, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 50.
Updated 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. Health Solutions, Ebix, Inc.
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