Hypoplastic left heart syndrome
Hypoplastic left heart syndrome (pronunciation: hye-puh-PLAS-tik left hahrt sin-drohm) is a rare congenital heart defect that affects the normal blood flow through the heart. The term "hypoplastic" originates from the Greek words "hypo" meaning "under" and "plasticos" meaning "formed", indicating the underdevelopment of the left side of the heart.
Definition
Hypoplastic left heart syndrome (HLHS) is a condition in which the left side of the heart is critically underdeveloped. The condition is present at birth (congenital). The left side of the heart is responsible for pumping oxygenated blood to the rest of the body. In HLHS, the left side of the heart can't effectively pump blood to the body, so the right side of the heart must pump blood both to the lungs and to the rest of the body.
Symptoms
Infants with HLHS might seem normal at birth, but within a few days, as the ductus arteriosus (an extra vessel found in the fetus) closes, the infant rapidly becomes critically ill. Symptoms include:
- Difficulty breathing
- Pounding heart or rapid breathing
- Bluish color (cyanosis) in skin, lips, and nail beds
- Poor feeding or abnormal sleepiness
Causes
The exact cause of HLHS is unknown. However, it is thought to occur as a result of multiple genetic and environmental factors. Some genetic disorders, such as Turner syndrome, are associated with HLHS.
Treatment
Treatment for HLHS usually involves several surgical procedures or a heart transplant. The goal of treatment is to reconstruct the heart so that it can pump blood to both the lungs and the rest of the body.
Prognosis
With early diagnosis and appropriate surgical treatment, many children with HLHS now survive, but the outlook (prognosis) varies depending on the presence of other heart defects and the development of complications.
See also
External links
- Medical encyclopedia article on Hypoplastic left heart syndrome
- Wikipedia's article - Hypoplastic left heart syndrome
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