What is a hypoplastic left heart syndrome?

The 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 chamber is undersized and too weak to draw blood into the body. Some children will survive for several weeks with this condition, but without surgical intervention, heart failure is inevitable.

The hypoplastic left heart is often diagnosed with routine sonograms. The small size of the left ventricle is a certain sign for the most experienced radiologists. The diagnosis is confirmed through a fetal echocardiogram that is similar to a sonogram, but looks more specifically on the fetal heart. Early diagnosis is valuable for parents, albeit stressful, because it gives them the opportunity to decide what type of care is to watch.

Just a few decades ago, many parents were told that their children with hypoplastic left ventricles could not survive. However, a larger number of children are now surviving and life with this condition. If there are no complications,A child with a hypoplastic left heart syndrome can often enjoy many years of activity than necessary interventions.

When parents face a diagnosis of left heart hypoplastic syndrome, there are three treatment options. The first is to do nothing, called compassionate care . Until recently, most cardiologists felt that compassionate care was really the best choice for children with HLHS. Instead of going through several operations, the child was simply allowed to die naturally. Cardiologists and parents who chose this option felt that letting the children go without being subject to risky treatment, their death was dignity and was the easiest to all involved, though heartbreaking.

However, the improved rate of surgery of surgery has radically changed the illustration of many cardiologists, although the value of compassionate care is still warmly questioned. Development of surgery Fontan, set of staged surgeryThe procedures performed for 3-4 years, provide the second option to parents who do not want compassionate care. Although it alleviates the symptoms of HLHS, fontan surgery cannot "repair" the heart. It only buys time for up to 30 years before transplantation.

Fontan surgery was originally performed as the only surgery in which the lower and superior vena cavae was connected by a tunnel built by the heart. Excellent Cava was then associated with pulmonary arteries that introduced passive blood flow to the lungs. The heart would then pump blood only to the body by the right ventricle, so the hypoplastic left ventricle is no longer necessary.

The premature survival of patients with fontan was not favorable. To increase survival, surgeons began to break the steps of surgery. In time, three steps have become a preferred method. It would first be included shortly after birth to help the heart. In about 3-4 months, Glenn's procedure would join the superior vena Cava to the lung valve and finally combined after 2-5 years the fontan procedure and superiorAzena vena cavae.

Many surgeons began to replace the intra-kardial tunnel with an extra cardiac short circuit (ECC). Although some surgeons still prefer a traditional tunnel, ECC is done on a warm whipping heart and has much less complications. It becomes the preferred method of fontana procedure because the studies show that it is as effective as the tunnel.

Many parents want to avoid more operations and instead choose a transplant. Successful heart transplantation means relatively few restrictions and overall heart health. However, the availability of hearts for transplantation is variable. Furthermore, life expectancy is much lower than those who have fontan surgery.

Sontane surgery, there is still a future transplantation option when the heart fails. The solution to the failing transplanted heart is further transplantation. If the initial heart was rejected, the second transplantation has an even lower success rate.

Decision on how to deal with a hypoplastic left heart syndromeFor a child, it is very personal and should be well informed. If the fetal diagnosis is made, parents may have 20 weeks in which they find the best hospital, decide what the best surgery is, and find support from other parents. Although the hypoplastic left ventricle was once an unparally condition, improvement of survival levels gives hope to all who can face this diagnosis and the difficult decision it causes.