Hypoplastic left heart syndrome

A congenital heart defect in which the left ventricle, mitral valve, aortic valve, and ascending aorta are severely underdeveloped, leaving the right ventricle to support both circulations.

Fetal circulation: The prenatal pattern of blood flow in which the placenta supplies oxygen and fetal shunts bypass the lungs. The fetal ductus arteriosus and foramen ovale help sustain circulation before birth.
Prostaglandin E1: A medication used in newborns to keep the ductus arteriosus open. It preserves ductal blood flow to the body while definitive treatment is arranged.
Left ventricle: The heart chamber that normally pumps oxygenated blood into the aorta and systemic circulation. Its severe underdevelopment prevents ordinary pumping to the body.
Single-ventricle physiology: A circulation in which one ventricle supports both the pulmonary and systemic circuits. Staged palliation leaves the right ventricle performing both pumping roles.
Patent ductus arteriosus: A persistent opening between the pulmonary artery and aorta that normally closes soon after birth. Keeping this vessel open allows blood from the right ventricle to reach the body.
Glenn procedure: A surgery connecting the superior vena cava directly to the pulmonary arteries. It is commonly the second stage of surgical palliation for this syndrome.
Aortic valve: The valve between the left ventricle and aorta that controls forward blood flow. It is often critically narrowed or closed in this syndrome.
Fontan circulation: A circulation in which systemic venous blood reaches the lungs without a pumping ventricle. It is the long-term circulation created by the final stage of palliation.
Atrial septostomy: A procedure that creates or enlarges an opening between the heart’s atria. An adequate atrial opening lets oxygenated pulmonary venous blood reach the right ventricle.
Fontan procedure: A surgery that directs systemic venous blood to the pulmonary arteries without passing through a ventricle. It completes the usual staged pathway after the Norwood and Glenn procedures.

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Tricuspid atresiaCompared with: It also requires staged single-ventricle palliation, but the underdeveloped side and flow obstruction differ.