Treatment for hypoplastic left heart syndrome involves either a three-step surgical procedure or a heart transplant. If the diagnosis has been made before the baby is born, doctors usually recommend delivery at a hospital with a cardiac surgery center.
Your baby's doctor may recommend several options to help manage the condition before surgery or transplant. They include:
- Medication. The medication alprostadil (Prostin VR Pediatric) helps dilate the blood vessels and keeps the ductus arteriosus open.
- Breathing assistance. If your baby has trouble breathing, he or she may need help from a breathing machine (ventilator), which ensures adequate oxygen delivery.
- Intravenous fluids. Your baby will receive fluids through a tube inserted into a vein.
- Atrial septostomy. This procedure is used to create an opening between the heart's upper chambers (atria) if the foramen ovale closes. If your baby already has an atrial septal defect, an atrial septostomy may not be necessary.
Surgical options include a three-step surgical procedure designed to create normal blood flow in and out of the heart, allowing the body to receive the oxygen-rich blood it needs.
- Step 1: The Norwood operation. This surgery is usually performed within the first two weeks of life. It involves reconstructing the aorta and connecting it directly to the heart's lower right chamber (right ventricle). This allows the right ventricle to effectively pump blood to both the lungs and the body. After this operation, your baby's skin will still have a blue cast because oxygen-rich and oxygen-poor blood continue to mix within the heart. Once your baby successfully passes through this stage of treatment, the odds of survival increase.
- Step 2: A bi-directional Glenn or hemi-Fontan procedure. This procedure is typically performed between 4 and 12 months of age. It reduces the work of the right ventricle by allowing it to pump blood mainly to the aorta and allowing most of the blood returning from the body to flow directly into the lungs. After this operation, all the blood returning from the upper body is sent to the lungs, so blood with more oxygen is pumped to the aorta to supply organs and tissues throughout the body.
- Step 3: The Fontan procedure. This procedure is done between 18 months and 3 years of age. It allows the rest of the blood coming back from the body to go to the lungs. After this procedure, there's no mixing of oxygen-rich and oxygen-poor blood in the heart, and your child's skin will no longer look blue.
Another surgical option is a heart transplant, especially when the defects are complex. Babies with hypoplastic left heart syndrome can be considered for a transplant. While awaiting a heart transplant, your baby may require medications until a donor heart becomes available.
After surgery or a transplant, your baby will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease, to monitor his or her heart health. Some medication may be necessary to regulate heart function. If your baby receives a heart transplant, anti-rejection medication will be necessary for the rest of his or her life to combat rejection of the new heart.
Your child's cardiologist may recommend taking antibiotics before certain dental or other procedures to prevent infections, especially if the heart hasn't been fully repaired yet or if there are small defects that remain after repair. In some cases, he or she may also recommend that your child limit physical activity.
Aug. 24, 2012
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- Facts about hypoplastic left heart syndrome. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/HLHS.html. Accessed July 24, 2012.
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- Grossfield P, et al. Hypoplastic left heart syndrome: New genetic insights. Journal of the American College of Cardiology. 2009;53:1072.
- Murtuza B, et al. Changing attitudes to the management of hypoplastic left heart syndrome: A European perspective. Cardiology in the Young. 2011;21:148.
- Said SM, et al. Longer-term issues for young adults with hypoplastic left heart syndrome: Contraception, pregnancy, transition, transfer, counselling, and re-operation. Cardiology in the Young. 2011;21: 93.
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