Hypoplastic Heart Syndrome
Hypoplastic heart syndrome is a complex congenital heart condition where the one side of the heart is severely underdeveloped. If the left side of the heart is underdeveloped, it is called the hypoplastic left heart syndrome and in cases where the right side of the heart has not completed the developmental process, it is called hypoplastic right heart syndrome.
Most of the symptoms of the hypoplastic right heart syndrome and a hypoplastic left heart syndrome are the same since they result in inadequate functioning of the heart. In most cases of the hypoplastic right heart syndrome, surgery that lasts for many years and involves Glenn shunt, Fontan and the Blalock-Taussig Shun ensures that the right ventricle is completely bypassed.
Hypoplastic left heart syndrome causes are unknown. The condition is however known to be related to genetic and if one child in the family has a hypoplastic heart, the risk of another child with hypoplastic heart condition is high. In a normal heart, the right side of the heart sends blood to the lungs for oxygenation and the oxygenated blood returns to the left side of the heart from where it is pumped from the aorta and other arteries to the rest of the body. With an underdeveloped left side of the heart, the heart is unable to pump blood to the remaining parts of the body adequately. This is a condition that does not become known before birth because the oxygenated blood is provided by the mother. Even after a few days of birth the healthy right side of the heart is able to pump oxygenated blood to the entire body. It is only after a couple of days that the condition gets severe.
Since the specific causes of hypoplastic left heart syndrome are not known, there are no known risk factors that can be guarded against. The only aspect that one does know is that there seems to be a genetic connection and therefore a baby who has a sibling with the condition is at high risk.
Hypoplastic Left heart Syndrome is recognized by the grayish blue color of the skin, difficulty in breathing, cold hands and feet and drowsiness. If the condition is not diagnosed within a couple of days of birth, the baby may go into shock. Some signs that can help you identify shock include weak pulse, dilated pupils, staring eyes and almost complete inactivity.
While some medications and assistance is needed for left hypoplastic heart syndrome treatment, the actual cure requires surgery. In most cases, medicines are given to ensure that the blood vessels stay dilated. Breathing assistance through a tube in the nose or a face mask along with intravenous fluids for adequate nutrition is also given. Sometimes atrial septostomy is also undertaken if the foramen ovale closes.
Surgery involves three basic steps that include the Norwood operation, a Glenn stunt or hemi-Fondan procedure and a final Fondan procedure. These procedures are done over a period of three years and are tough on the patient’s family. Even after the surgical correction, lifelong follow ups with the cardiologist will be required if hypoplastic heart syndrome has been diagnosed and treated.