Hypoplastic left heart syndrome (HLHS) is a rare and serious congenital heart condition where the left ventricle is undersized and too weak to pump blood around the body. It can be diagnosed through routine ultrasound and confirmed through fetal echocardiography. Treatment options include compassionate care, Fontan surgery, or heart transplantation. Improved survival rates have changed the opinion of many cardiologists, although the value of compassionate care is still hotly contested. Fontan surgery is a set of staged surgical procedures performed over 3-4 years that can relieve the symptoms of HLHS, but cannot “fix” the heart. Heart transplantation means relatively few limitations and total heart health, but the availability of hearts for transplantation varies. The decision about how to deal with HLHS in a child is a highly personal one and should be well informed.
Hypoplastic left heart syndrome (HLHS) is a serious and rare congenital heart condition. In this syndrome, the fetal heart does not develop normally, so the left ventricle is undersized and too weak to pump blood around the body. Some babies survive a few weeks with this condition, but without surgery, heart failure is inevitable.
Often, hypoplastic left heart is diagnosed through routine ultrasound. The tiny size of the left ventricle is a sure indication of the condition for more experienced radiologists. Diagnosis is confirmed through fetal echocardiography, which is similar to a sonogram but looks more specifically at the fetal heart. Early diagnosis is valuable to parents, albeit stressful, because it gives them an opportunity to make decisions about the type of care to pursue.
Just a few decades ago, many parents were told that their children with hypoplastic left ventricles could not survive. However, more children are now surviving and living with the condition. Unless complications occur, a child with hypoplastic left heart syndrome can often enjoy many years of activity before further interventions are needed.
When parents are faced with a diagnosis of hypoplastic left heart syndrome, there are three options for treatment. The first is doing nothing, called compassionate care. Until recently, most cardiologists believed that compassionate care was indeed the best choice for children with HLHS. Instead of putting the baby through more surgeries, the baby was simply allowed to die naturally. Cardiologists and parents who chose this option felt that letting the children go without subjecting them to risky treatments dignified their deaths and was easier on all concerned, albeit excruciating.
However, the improved survival rates of surgeries have dramatically changed the opinion of many cardiologists, although the value of compassionate care is still hotly contested. The development of Fontan surgery, a set of staged surgical procedures performed over 3-4 years, provides the second option for parents who do not want compassionate care. Although it relieves the symptoms of HLHS, Fontan surgery cannot “fix” the heart. It simply buys time, up to 30 years, before transplanting.
Fontan’s surgery was initially performed as a single surgery, in which the inferior and superior vena cava were connected through a tunnel made through the heart. The superior cava was then connected to the pulmonary arteries, which established passive blood flow to the lungs. The heart then pumps blood only around the body through the right ventricle, making the hypoplastic left ventricle no longer needed.
Early survival of Fontan patients has not been favorable. To increase survival, surgeons began breaking up the stages of surgery. Over time, three passes became the preferred method. First, a shunt would be placed shortly after birth to help the heart. In about 3-4 months, the Glenn procedure would connect the superior vena cava to the pulmonary valve, and finally at 2-5 years, the Fontan procedure would connect the inferior and superior vena cava.
Many surgeons have begun to replace the intracardiac tunnel with an extracardiac shunt (ECC). Although some surgeons still prefer the traditional tunnel, ECC is performed on a warm beating heart and has far fewer complications. It is becoming the preferred method for the Fontan procedure, as studies show it to be as effective as the tunnel.
Many parents wish to avoid multiple surgeries and choose transplantation instead. A successful heart transplant means relatively few limitations and total heart health. However, the availability of hearts for transplantation varies. Also, life expectancy is far lower than those with Fontan surgery.
With Fontan surgery, there is still the future possibility of a transplant when the heart fails. The solution to a failed heart transplant is another transplant. If the initial heart was rejected, second transplants have an even lower success rate.
The decision about how to deal with hypoplastic left heart syndrome in a child is a highly personal one and should be well informed. If the fetal diagnosis is made, parents may have 20 weeks to find the best hospital, decide what the best surgical procedure is, and find support from other parents. Although hypoplastic left ventricle was once an impractical condition, improved survival rates give hope to all who may be faced with this diagnosis and the difficult decision it entails.
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