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Hypoplastic Right Heart Syndrome

Hypoplastic right heart syndrome is a congenital heart defect (rarer than hypoplastic left heart syndrome), in which the parts of the right side of the heart (the valves, right ventricle, and pulmonary artery) do not develop fully during pregnancy.

It causes a lack of blood flow to the body and with it a lack of oxygen. This condition often occurs along with atrial septal defect, a hole between the left and right upper chambers (atria) of the heart.

How Is Hypoplastic Right Heart Syndrome Diagnosed?

Hypoplastic right heart syndrome may be diagnosed during pregnancy or soon after the baby is born. It may be diagnosed during pregnancy with an ultrasound. If heart problems are suspected, the health care provider may request a fetal echocardiogram to confirm the diagnosis.

How Is Hypoplastic Right Heart Syndrome Treated?

After birth, the baby will be treated with medicines to assist heart function, special nutrition to ensure weight gain, and multiple surgical procedures to rebuild the structures of the heart.

Heart Center Family Resource Guide

To help prepare families for their care with Lurie Children's Heart Center, we have compiled a list of resources about treatment and recovery. Learn how to get ready for an inpatient stay or outpatient visit, and read about our support services for patients and families.

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