Hypoplastic left heart syndrome (HLHS) affects the development of the left side of a baby’s heart. As a congenital condition, this defect is present from birth. It can impact the aorta, the heart’s largest blood vessel, as well as the left ventricle and mitral and aortic valves.
Under normal conditions, the left and right sides of the heart work in conjunction to facilitate proper blood flow. The left side of the heart transports oxygenated blood to the body, while the right side sends blood to the lungs to become oxygenated. When the left side cannot efficiently pump blood, the right side must take over.
HLHS is rare and doctors are not entirely sure why it occurs. Certain genetic mutations and disorders (such as Turner syndrome) can increase the risk.
HLHS symptoms can occur just after birth or within a few hours or days. Symptoms include:
Without prompt treatment, HLHS can be fatal. Improper function of the left side of the heart causes blood to flow through the ductus arteriosus, a temporary blood vessel that connects the aorta and pulmonary arteries. This blood vessel eventually closes, which impedes the flow of blood.
Initial treatment involves the administration of a medicine called prostaglandin. As a vasodilator, prostaglandin ensures the ductus arteriosus remains open to help with blood flow. Additional heart medications and oxygen therapy may also be necessary.
The primary HLHS treatment is surgery. Surgeons perform three separate procedures to correct this heart defect:
In some cases, heart transplantation may be recommended. The outlook for your child depends on the seriousness of the heart defect and their general health. While there are numerous risks, surgery is the only way to correct HLHS and prevent life-threatening complications from occurring.