Symptoms of Hypoplastic Left Heart Syndrome: Causes and Long Term Effects

Hypoplastic left heart syndrome is a congenital disorder of the heart. It is characterized by structural underdevelopment of different parts present in left side of heart. This includes the left ventricle, aorta, aortic valve, and mitral valve. The left ventricle is a lower left chamber of heart. Its main function is to pump oxygenated blood to entire body through aorta, the great blood vessel. In hypoplastic left heart, the ventricle is extremely small sized or may be absent.

Hence the body may not receive enough oxygenated blood. If the mitral valve or aortic valve is narrow, the flow of blood is obliterated. In some case the aorta can be narrow itself.

Since the left ventricle has diminished function because of the anomaly, the right ventricle takes over its function. However, in doing so impure blood as well as pure oxygenated blood gets mixed. The body now receives this mixed blood which can be detrimental to health.

Normally after the birth, for few days there remains a connection between the right ventricle and left ventricle of the heart. It is called ductus arteriosus. It closes within one or two days after birth naturally. When hypoplastic left heart syndrome is detected, doctors try to retain this passage with medication until the neonate is fit for surgical repair.

Causes of Hypoplastic Left Heart Syndrome:

Hypoplastic left heart syndrome is a congenital malformation of left side of heart. It means the malformation is present since birth. Actually any structure in the left side of heart such as left ventricle, aorta, or the aortic and mitral valves is underdeveloped or may be absent. The structural defect develops during the development of the heart. The heart starts developing in first month of pregnancy. The exact cause is not known, but there is increased risk if there is anyone in the family suffering from congenital heart disease. The defect is present in at least 7 to 9 percent of all infants suffering from congenital heart disease. The term hypoplastic left heart syndrome was coined in 1958 by Noonan and Nadas.

Hypoplastic Left Heart Syndrome Symptoms:

The symptoms of hypoplastic left heart syndrome develop soon after the birth of the neonate. In some cases it may develop in one or two days. The baby usually appears severely ill. Following are the symptoms that may occur soon after the baby is born in hypoplastic left heart syndrome:

  • Bluish color of skin, lip, tongue. In medical terminology it is called cyanosis.
  • Increased heart beats.
  • Breathlessness
  • The infant is not able to feed properly.
  • Limbs are cold
  • Inactive
  • Increased drowsiness.
  • Pale skin

Diagnosis and Treatment:

Hypoplastic left heart syndrome is diagnosed when the fetus is in the womb. During routine sonogram examination conducted in pregnancy period, this congenital heart defect is detected. After birth, the anomaly can be diagnosed from the symptoms, clinical examination and confirmed with the help of tests such as echocardiogram, doppler ultrasound, chest X-ray, and ECG.

Once the symptoms are detected and the diagnosis is confirmed, the child is immediately admitted in intensive care unit. Here different medical instruments and machines are used to stabilize the vital functions such as breathing and heart rate. The infant may also need feeding tube since the child experiences difficulty in feeding. Intravenous fluids are administered as and when required. In the beginning it is very important to keep the ductus arteriosus open as this may get closed within one or two days. Medicines are administered to keep it dilated or patent. As the child is stabilized surgical repair is necessary. Different types of surgical procedures include the Norwood procedure, Fontan surgery, Glenn operation. The various procedures are carried out at different time period. Usually the child requires 3 to 4 surgery over a period of few years for correcting the defect.