What prenatal findings are typical of hypoplastic left heart syndrome (HLHS)?

Prepare for the Fetal Echocardiography Test. Utilize flashcards and multiple-choice questions that come with hints and explanations. Ace your exam preparation!

Multiple Choice

What prenatal findings are typical of hypoplastic left heart syndrome (HLHS)?

Hypoplastic left heart syndrome is defined by underdevelopment of the left-sided heart structures so the right ventricle must drive the systemic circulation via the ductus arteriosus. Prenatally you’d expect a small left ventricle with mitral and/or aortic valve hypoplasia and a diminutive aortic arch. Because the systemic flow depends on the ductus, this circulation is ductus-dependent. After birth, closure of the ductus would critically compromise systemic perfusion, which is why management focuses on keeping the ductus open and planning staged palliation. This description best fits the HLHS pattern. The other scenarios do not match HLHS: a normal left heart, or a large left ventricle with valve hyperplasia, or a ductus-independent systemic circulation would imply different fetal heart physiology.

Hypoplastic left heart syndrome is defined by underdevelopment of the left-sided heart structures so the right ventricle must drive the systemic circulation via the ductus arteriosus. Prenatally you’d expect a small left ventricle with mitral and/or aortic valve hypoplasia and a diminutive aortic arch. Because the systemic flow depends on the ductus, this circulation is ductus-dependent. After birth, closure of the ductus would critically compromise systemic perfusion, which is why management focuses on keeping the ductus open and planning staged palliation. This description best fits the HLHS pattern. The other scenarios do not match HLHS: a normal left heart, or a large left ventricle with valve hyperplasia, or a ductus-independent systemic circulation would imply different fetal heart physiology.

More practice questions