children s health issues
Hypoplastic Left Heart Syndrome
Treatment of Hypoplastic Left Heart Syndrome
A medication (a prostaglandin) to keep the ductus arteriosus open until surgery Surgical repair Sometimes heart transplantation Because most affected children with hypoplastic left heart syndrome are identified before birth, doctors begin giving a medication to keep the ductus arteriosus open immediately after birth, even before any symptoms develop. Affected infants are treated in a neonatal intensive care unit or pediatric cardiac intensive care unit. A prostaglandin is given by vein to prevent closure of the ductus arteriosus or to reopen a closed ductus. Newborns, particularly those that are critically ill, usually require assistance with breathing (mechanical ventilation). Severely ill newborns may require medications to improve their heart function. Survival ultimately requires a series of surgical procedures that enable the right ventricle to take over the work of the underdeveloped left ventricle. Surgery is done in several stages over the course of 2 to 3 years. Stage 1: Done during the first weeks of life. This involves a complex repair called a Norwood procedure, which allows the right side of the heart to supply blood to the body. Stage 2: Done when the infant is 3 to 6 months of age. This operation is known as a Glenn procedure. It directs the blood coming back to the heart from the upper half of the body directly into the pulmonary artery, bypassing the heart. Stage 3: Done when the child is 18 to 36 months of age. If infants have had good results from the first 2 stages of repair, then they undergo the third stage, which is called a Fontan procedure. This operation directs the blood returning from the lower half of the body to the pulmonary artery, resulting in all of the venous blood bypassing the heart on its way to the lungs. In some medical centers, Heart Transplantation is considered the procedure of choice for hypoplastic left heart syndrome. Infants must be given an infusion of a prostaglandin to keep the ductus arteriosus open until a donor heart is available. Because availability of donor hearts is very limited, about 20% of infants die while awaiting transplant. The 5-year survival rates after transplantation and after multistage surgery are similar. However, because of the limited donor issue and improved surgical outcomes, most congenital heart disease treatment centers recommend the multistage surgical approach. After heart transplantation, medications to suppress the activity of the immune system (immunosuppressants) are required for the rest of the child's life. These medications make people more susceptible to infections and increase the risk of developing certain tumors. Immunosuppressants may also damage the coronary arteries of the transplanted heart. The only known treatment for coronary artery damage is retransplantation. Some children, depending on the result of their surgical procedures, need to take antibiotics before visits to the dentist and before certain surgeries (such as on the respiratory tract). These antibiotics are used to prevent serious a heart infection called Infective Endocarditis . Most children need to takeaspirinor a blood thinner such aswarfarinorenoxaparinto help prevent clots from forming in the heart. Many affected children need to take one or more other medications to help their hearts continue to function optimally. 