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Double inlet left ventricle (DILV) is a very rare congenital heart defect in which the child develops only one ventricle — the pumping chamber of the heart. individuals with DILV may have other heart problems as well, such as coarctation of the aorta, pulmonary atresia, or pulmonary valve stenosis. It accounts for 1% of all congenital cardiac anomalies and is seen in 4% of neonates with congenital cardiac disease.
In the normal heart, the right and left ventricles receive blood from the right and left atria. The atria are upper chambers of the heart. 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. This is the blood vessel that carries blood to the lungs to pick up oxygen.
Blood with fresh oxygen 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. The aorta is the major artery leading out of the heart.
In people with DILV, only the left ventricle is developed. Both atria empty blood into this ventricle. This means that oxygen-rich blood mixes with oxygen-poor blood. The mixture is then pumped to both the body and the lungs.
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 operations. These surgeries are similar to the ones used to treat hypoplastic left heart syndrome and tricuspid atresia.
The first surgery may be needed when the baby is only a few days old. In most cases, the baby can go home from the hospital afterward. The child will most often need to take medicines every day and be closely followed by a pediatric heart doctor (cardiologist). The child’s doctor will determine when the second stage of surgery should be done.
The next surgery (or first surgery, if the baby didn’t need a procedure as a newborn) is called the bidirectional Glenn shunt or Hemifontan procedure. This surgery is usually done when the child is 4 to 6 months old.
Even after the above operations, the child may still look blue (cyanotic). The final step is called the Fontan procedure. This surgery is most often done when the child is 18 months to 3 years old. After this final step, the baby is no longer blue.
The Fontan operation does not create normal circulation in the body. But, it does improve blood flow enough for the child to live and grow.
A child may need more surgeries for other defects or to extend survival while waiting for the Fontan procedure.