Madisons Foundation - Moms And Dads In Search Of Needed Support

What

Some babies can be born with the hypoplastic left heart syndrome (HLHS), a form of heart problem where the left side of the heart, which pumps blood to the body, is small and abnormally formed. As a result of poorly developed structures, the heart is unable to pump sufficient amounts of blood to the rest of the body which eventually leads to heart failure.

Who

Heart failure in the first days of life is rare, but when it does occur, HLHS is among the most common causes. It has an occurrence of about 0.3 per 1000 live births and comprises close to 9% of all heart disorders diagnosed in the first year of life. Boys and children with other congenital syndromes such as Turner’s syndrome or Noonan’s syndrome are more likely to develop HLHS. All racial groups are affected equally.

Signs and Symptoms

Children with HLHS may exhibit the following signs and symptoms within the first few hours to days of life: -rapid, labored breathing -difficulty feeding -poor energy, weakness -pale color in the extremities and blue color around the lips -a heart murmur may be heard on physical examination -weak pulses reflecting poor circulation -enlarged liver may be present as a result of heart failure.

Possible Causes

The exact cause of HLHS is not known. However, one thought is that there may be inadequate blood flow through the left side of the heart during fetal development, leaving that side of the heart small.

Diagnosis

HLHS is diagnosed through imaging tests, blood work, x-rays and thorough examination by the child’s doctor. Once a child is suspected to have HLHS, he or she is admitted to a hospital with an intensive care unit. The child will receive extra oxygen while blood work in the form of a complete blood count, electrolytes, liver tests, and blood gas analysis are obtained. An ultrasound of the heart (echocardiogram) is obtained as soon as possible to make the diagnosis and evaluate the structures of the infant’s heart. X-rays of the chest and an electrocardiogram (EKG) may also be needed to evaluate the lungs and electrical rhythm of the heart respectively. A procedure called cardiac catheterization is usually performed to gather more detailed anatomical information in preparation for corrective surgery. In this procedure, a long tube with a probe is inserted into a big vein and threaded up to the heart where special pictures can be taken more precisely of the heart, its position and internal structures. Consultation with a pediatric cardiologist and cardiac surgeon is usually sought for this problem.

Treatment

HLHS is treated with a combination of medical and surgical therapy. After an infant is stabilized with oxygen and other supportive measures, a medicine known as prostaglandin or PGE is usually started. PGE is a natural protein that dilates a blood vessel known as the ductus arteriosus. The ductus arteriosus is a blood vessel that the fetus uses before birth to circulate blood to the body. After birth, the ductus arteriosus normally closes but in HLHS, it needs to stay open to allow blood flow to the rest of the body. Other non-surgical measures include creating a small hole in the wall separating the upper or atrial chambers of the heart (balloon atrial septostomy). This can be done through the cardiac catheterization procedure and will temporarily help the body receive more blood flow. The ultimate treatment of HLHS, however, is through a series of heart surgeries in the first years of life or a heart transplant. Determining whether transplant or surgical repair is the best option remains controversial. Surgical repair is begun shortly after diagnosis in the neonatal period (Norwood procedure), continued at 6months (bidirectional Glenn operation), and completed at 18 to 24 months of age (modified Fontan operation). The time course for a heart transplant varies depending on the availability of a suitable donor.

Prognosis

Without surgery, children with HLHS usually do not survive past the first 4 weeks of life. Close to 70% of children with HLHS who are deemed a low operative risk and undergo heart transplant or staged surgical repair have been known to survive 5 years or longer. The most difficult period for children born with HLHS is after the first stage of surgery, with only about 45% surviving to the next surgical procedure.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Left Heart Matters
Parent support group specific to HLHS.

HLHS information page
Well put together support group website with information as well as links to other websites.

Congenital Heart Information Network
Online discussion group and support website. Little Hearts Organization http://www.littlehearts.net/ A website with information and support for families of children with congenital heart disease. Children s Heart Institute http://www.childrenheartinstitute.org/educate/defects/hypo1.htm Nice

Google Search for Hypoplastic Left Heart Syndrome (HLHS)

References and Sources

Park, M. (2002) Pediatric Cardiology for Practitioners. 4th ed., Mosby, St Louis, p 383-386. Turner, D. emedicine.com article: www.emedicine.com/ped/topic1131.htm