Which statement describes Hypoplastic Left Heart Syndrome?

Which statement describes Hypoplastic Left Heart Syndrome?

• A. Underdevelopment of the left side of the heart with absent or nonfunctional left ventricle and hypoplasia of the ascending aorta
• B. Oversized left ventricle with a large ascending aorta
• C. Always acyanotic during the entire course
• D. Prostaglandins are never used in management

Answer: (A)

Hypoplastic left heart syndrome is a congenital defect where the structures on the left side of the heart are severely underdeveloped, leading to a tiny or absent left ventricle and a hypoplastic ascending aorta. Because the left ventricle can’t supply the systemic circulation, the right ventricle must pump blood to both the lungs and the body, and systemic flow relies on a patent ductus arteriosus to route blood from the pulmonary artery to the aorta. After birth, keeping the ductus arteriosus open is essential, so prostaglandin E1 is given to maintain patency until surgical palliation (such as the Norwood procedure) is performed. This description accurately reflects HLHS. The other ideas don’t fit HLHS: the condition is not due to an oversized left ventricle, and it is typically associated with cyanosis due to poor systemic perfusion and mixing of blood. Prostaglandins are used to keep the ductus arteriosus open, not never used.

Hypoplastic left heart syndrome is a congenital defect where the structures on the left side of the heart are severely underdeveloped, leading to a tiny or absent left ventricle and a hypoplastic ascending aorta. Because the left ventricle can’t supply the systemic circulation, the right ventricle must pump blood to both the lungs and the body, and systemic flow relies on a patent ductus arteriosus to route blood from the pulmonary artery to the aorta. After birth, keeping the ductus arteriosus open is essential, so prostaglandin E1 is given to maintain patency until surgical palliation (such as the Norwood procedure) is performed. This description accurately reflects HLHS.

The other ideas don’t fit HLHS: the condition is not due to an oversized left ventricle, and it is typically associated with cyanosis due to poor systemic perfusion and mixing of blood. Prostaglandins are used to keep the ductus arteriosus open, not never used.