Double inlet left ventricle (DILV) is a congenital heart defect that occurs in 5-10 of every 100,000 pregnancies. Of the nearly 4 million U.S. births each year, approximately 200-400 will be born with this condition. DILV is a "single ventricle" defect, which are among the most rare and complex congenital heart problems. Babies born with this condition have only one working pumping chamber (ventricle) in their heart. People often refer to the condition as having "half a heart", which is a simplistic yet essentially accurate way of describing the condition to family and friends.
In addition to DILV, Brent's heart has several other defects, many of which often occur along with DILV. His specific diagnosis is:
- Double inlet left ventricle (ventricles are reversed)
- Transposition of the great arteries (TGA)
- Ventricular septal defect (VSD)
- Atrial septal defect (ASD)
- Triscupid valve straddling
- Possible: coarctation of the aorta
The first open heart surgery may be needed within the first few days of life. The next open heart surgery is called the bidirectional Glenn shunt or Hemifontan procedure. This surgery is usually done when the child is 4 - 6 months old. The final step is called the Fontan procedure. This surgery is usually performed between 18 months - 3 years old. Additional surgeries may be needed for related defects or to extend survival while waiting for the Fontan procedure. For the most severe cases of DILV, a heart transplant may be recommended.
The following YouTube video does a good job of briefly explaining DILV further.
Additional Resources: You may also find the following resources beneficial regarding DILV if you wish to learn more.
MetroHealth
Heart News Links
Cove Point Foundation
National Institutes of Health
Cleveland Children's Clinic
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