Hypoplastic left heart syndrome
Other Names for this Disease
Your QuestionMy best friend's baby has hypoplastic left heart syndrome and she been told that it's not common here in Australia. The doctors don't know a lot about it and her baby has only days to live. How common is it? What is the prognosis?
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Questions on this page
- What is hypoplastic left heart syndrome (HLHS)?
- What are the signs and symptoms of hypoplastic left heart syndrome (HLHS)?
- How common is hypoplastic left heart syndrome (HLHS)?
- How might hypoplastic left heart syndrome (HLHS) be treated?
- What is the prognosis of hypoplastic left heart syndrome (HLHS)?
At first, a newborn with HLHS may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include:
- Bluish (cyanosis) or poor skin color
- Cold hands and feet (extremities)
- Poor pulse
- Poor suckling and feeding
- Pounding heart
- Rapid breathing
- Shortness of breath
In healthy newborns, bluish color in the hands and feet is a response to cold (this reaction is called peripheral cyanosis). However, a bluish color in the chest or abdomen, lips, and tongue is abnormal (called central cyanosis). It is a sign that there is not enough oxygen in the blood. Central cyanosis often increases with crying.
- Using the pulmonary valve and artery
- Connecting the hypoplastic old aorta and coronary arteries to the new aorta
- Removing the wall between the atria (atrial septum)
- Making an artificial connection from either the right ventricle or a body-wide artery to the pulmonary artery to maintain blood flow to the lungs (called a shunt)
Afterwards, the baby usually goes home. The child will need to take daily medicines and be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be done. Stage II of the operation is called the Glenn shunt or hemi-Fontan procedure. This procedure connects the major vein carrying blue blood from the top half of the body (the superior vena cava) directly to blood vessels to the lungs (pulmonary arteries) to get oxygen. The surgery is usually done when the child is 4 to 6 months of age. During stages I and II, the child may still appear somewhat blue (cyanotic).Stage III, the final step, is called the Fontan procedure. The rest of the veins that carry blue blood from the body (the inferior vena cava) are connected directly to the blood vessels to the lungs. The right ventricle now serves only as the pumping chamber for the body (no longer the lungs and the body). This surgery is usually performed when the baby is 18 months - 3 years old. After this final step, the baby is no longer blue. Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure.
In some hospitals, heart transplantation is considered a better choice than the three-step surgery process. However, there are few donated hearts available for small infants.
- Schumacher KR. Hypoplastic left heart syndrome. MedlinePlus. December 2009; http://www.nlm.nih.gov/medlineplus/ency/article/001106.htm. Accessed 7/21/2011.
- Facts about Hypoplastic Left Heart Syndrome. Centers for Disease Control and Prevention. April 2011; http://www.cdc.gov/ncbddd/heartdefects/hlhs.html. Accessed 7/21/2011.
- Syamasundar Rao P. Pediatric Hypoplastic Left Heart Syndrome . eMedicine. June 2011; Facts about Hypoplastic Left Heart Syndrome. Accessed 7/21/2011.