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Hypoplastic left heart syndrome

Congenital heart defects

What is hypoplastic left heart syndrome?

Hypoplastic left heart syndrome (hypoplastic left heart syndrome - HLHS) is a very serious congenital heart disease in which the left heart is underdeveloped (hypoplastic). There are different degrees of HLHS. The syndrome is relatively rare, and the cause is usually not known. Girls with Turner syndrome have an increased incidence of HLHS.

What are the symptoms of the condition?
Pregnancy and childbirth extends generally normal. Symptoms usually occur within 1-2 days after birth. Because of heart failure can not child's heart to pump adequate amounts of blood throughout the body, and it is grÄblekt, limp and have trouble sucking milk. The organs do not get enough oxygen and quickly takes damage unless treatment is started early. The consequences can include being kidney failure, damage to the intestines, seizures, heart attacks and death.

How the diagnosis?
It is important that the diagnosis is made quickly. The suspicion of serious heart defects arise when newborn few days after birth suddenly becomes poor. The main technique for detecting HLHS is ultrasound examination of the heart ( echocardiography ). This survey is completely harmless and takes place at the ultrasound device is placed on the child's chest. The doctor will then submit a picture of your child's heart and see if it has serious flaws.

Ultrasound scanning in pregnancy can often prove HLHS. The advantage of detecting the condition during pregnancy is that the birth may be prepared and treatment is initiated quickly.

What treatments are available?
Children with hypoplastic left heart disease need immediate treatment to survive. In the first place it involves oxygen and various medications. It may be necessary to use the respirator for the baby to get enough oxygen. If conditions are right for it, the child as soon as possible transferred to a hospital with surgical expertise to operate such errors. This at the hospital. It is a very demanding operation called Norwood-Fontan method and for the result to be successful, it must be done in three steps. Second step is done when the child is 4-6 months old, and the last step is accomplished by 1.5-2 years of age. Sometimes the error is of such a nature that the operation is useless, and the child must be allowed to die as peacefully as possible.

How is long-term prospects?
The prognosis depends partly on the extent of heart failure is and how quickly treatment is started. For the group as a whole, ca. half of the children born with HLHS, survive all three steps of Norwood-Fontan method. For these are relatively good prognosis.

Children who have surgery for HLHS are at increased risk of getting inflammation of the choroid in the heart ( endocarditis ) in connection with subsequent surgical treatment. This also applies to engagement of the mouth, such as tooth extraction. It is therefore important to inform physicians and dentists so that you get prophylactic antibiotic treatment in such situations.

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