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hypoplastic left heart syndrome
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left heart is severely underdeveloped. The ''International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease'' defined HLHS as
==Signs and symptoms==
In typical anatomy, the left side of the heart receives oxygen-rich blood from the lungs and pumps it to the rest of the body. In patients with HLHS, the aorta and left ventricle are underdeveloped (beginning ''in utero''), and the aortic and mitral valves are either too small to allow sufficient blood flow or are atretic (closed) altogether.〔(Hypoplastic Left Heart Syndrome | Congenital Heart Disease - Cove Point Foundation | Johns Hopkins Children's Hospital )〕 As blood returns from the lungs to the left atrium, it must pass through an atrial septal defect to the right side of the heart so it can be pumped to the rest of the body. This blood must flow through a patent (open) ductus arteriosus. Since the ductus arteriosus usually closes within eleven days after birth, blood flow may be severely restricted and eventually blocked, leading to dangerously low circulation and, eventually, shock. Babies are often cyanotic at birth to varying degrees, depending on the severity of their defects.
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