Hypoplastic Left Heart Syndrome (HLHS) is a complex and severe congenital heart defect that affects the left side of the heart, leading to significant underdevelopment or absence of critical structures. It is a life-threatening condition that is present at birth.
In a normal heart, the left side is responsible for pumping oxygen-rich blood throughout the body. However, in HLHS, the left side of the heart is underdeveloped, causing insufficient blood flow to the body. The structures affected may include the left ventricle (the main pumping chamber), mitral valve (which controls blood flow between the left atrium and the left ventricle), and aorta (the main artery which carries oxygenated blood to the body). As a result, the right side of the heart must work harder to compensate for the inadequate function of the left side.
Babies born with HLHS typically exhibit cyanosis (bluish skin coloration) due to decreased oxygen levels, rapid breathing, poor feeding, and lethargy. Without early intervention, HLHS is fatal within the first few days or weeks of life.
Treatment for HLHS usually involves a series of corrective surgeries, with the ultimate goal of rerouting blood flow bypassing the underdeveloped left side of the heart. This can be achieved through a surgical procedure known as the Norwood procedure, followed by subsequent surgeries such as the Glenn shunt and the Fontan procedure.
While advances in medical technology and surgical techniques have improved outcomes for those with HLHS, the condition remains a complex and challenging heart defect that requires specialized medical care and continuous monitoring throughout an affected individual's life.
