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Hypoplastic left heart syndrome (also known as HLHS), is a rare congenital heart defect in which the left side of the heart is severely underdeveloped.
While many authorities believe the cause of HLHS is unknown,[1] [2][3] recent research indicates that HLHS may be due to genetic factors. [4][5]
There is evidence associating it with Gap junction protein, alpha 1.[6]
In babies with HLHS, the aorta and left ventricle are very small, and the aortic and mitral valves are either too small to allow sufficient blood flow or are atretic (closed) altogether. 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.
In a healthy human, the left side of the heart receives oxygen-rich blood from the lungs and pumps it out to the rest of the body; with these structures underdeveloped, they cannot circulate blood to other organs, and the right ventricle must pump blood to both the lungs, as it would normally, and to the rest of the body, a situation which cannot be sustained for long.
In cases of HLHS, the right side of the heart often must pump blood to the body through a patent ductus arteriosus. As the ductus arteriosus usually closes within eleven days after birth, blood flow is severely restricted and eventually cut off, leading to dangerously low circulation and eventually to shock.
Without treatment, HLHS is fatal, but with intervention, an infant may survive. A pediatric cardiothoracic surgeon may perform a series of operations or a full heart transplant. In the meantime, the ductus may be kept open to allow bloodflow using medication containing prostaglandin. Because these operations are complex and need to be individualized for each patient, a cardiologist must assess all medical and surgical options on a case-by-case basis. After assessment the child’s parents are given a percentage chance of survival and the choice of surgery or to take the child home to pass away.
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