The Hypoplastic right heart syndrome (HRHS) is rare congenital disorder. Hypoplasia means underdevelopment of an organ. Thus Hypoplastic right heart syndrome is underdevelopment or incomplete development of the right side of the heart. The structures of the right side of the heart, which includes the right atrium, right ventricles, pulmonary valve, and pulmonary artery, are poorly developed. As a result, the functions of the right sides of the heart are hampered leading to a series of symptoms described as a syndrome. Since the ventricles fails to grow, the right ventricular muscles are also not developed and cannot pump the blood efficiently into the lungs. Thus it results in diminished oxygenation and the baby becomes cyanosed or blue.
HRHS needs to be treated immediately as the heart fails immediately after birth. The baby shows difficulty in breathing, and the skin, nails, lips and the rest of the body turns blue. Though highly fatal, corrective surgery can rescue the baby for an improved life expectancy. Open heart surgeries, Norwood operation and cardiac transplantation are the treatment options usually considered. After the Fontan (Norwood) procedure, patients have lived a normal life for up to 15 to 30 years, after which the heart functions may show deterioration. Today most parents choose heart transplant for longer and healthier life.
Children with HRHS should be restricted from heavy activities and sports that could easily exhaust them. Besides a regular cardiac assessment and check up is important.
