Hypoplastic left heart syndrome
Key points
- Hypoplastic left heart describes the underdevelopment of the left side of the heart (left ventricle, aortic valve, and aorta).
- Hypoplastic left heart is a rare type of congenital heart disease.
- Initially, a newborn with hypoplastic left heart may appear normal.
- A physical examination usually reveals a blue, critically ill patient who may show signs of congestive heart failure, liver enlargement, rapid breathing, and lethargy.
Alternative names
Hypoplastic left heart; HLHS
Definition
Hypoplastic left heart describes the underdevelopment of the left side of the heart (left ventricle, aortic valve, and aorta). The condition is congenital (present at birth).
Causes, incidence, and risk factors
Hypoplastic left heart is a rare type of congenital heart disease. The problem develops before birth when there is inadequate growth of the left ventricle and associated structures (aortic and mitral valves that guard the exit and entrance of the ventricle and the aorta which is the blood vessel that carries oxygenated blood from the left ventricle to the entire body).
In patients with this condition, the left side of the heart is unable to maintain blood circulation for the body. As a result, the right side of the heart must maintain the circulation for both the lungs and the body. This extra workload eventually causes the heart to fail.
The only possibility of survival is a connection between the right and the left side of the heart through which blood may pass. This is called a shunt. Babies are normally born with two of these connections (the foramen ovale and the ductus arteriosus), which spontaneously close a few days after birth.
If these structures are allowed to close in a baby with hypoplastic left heart syndrome, however, the patient will quickly die because no blood will be pumped to the body.
There is no known cause of hypoplastic left heart syndrome. Up to 40% of patients with this condition have other birth defects.
Symptoms
Initially, a newborn with hypoplastic left heart may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include:
Written and revised by
Andrey Nailor, M.D.
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