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
For the most severe cases of DILV, a heart transplant may be recommended.
All children with congenital heart disease should take antibiotics before dental treatment. This prevents infections around the heart, a common problem with heart disease.
Prognosis (Expectations)
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.
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Review Date : 4/30/2008
Reviewed By : Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children’s Hospital of Philadelphia, Division of Cardiology, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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