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What Is Hypoplastic Right Heart Syndrome?

Hypoplastic right heart syndrome is a type of congenital heart defect that is characterized by the inability of the heart’s right ventricle to grow and develop normally. The underdeveloped right ventricle is not able to hold the right amount of blood that is pumped from the right atrium. The muscle of the affected ventricle is also very weak, which further compounds the problems as the heart tries to pump blood to the pulmonary valve in order to transport it to the lungs.

Hypoplastic right heart syndrome is a heart defect that needs immediate treatment because the heart starts to fail from the moment the baby is delivered. The poor blood supply to the lungs results in the return of inadequately oxygenated blood to the body, which results in the muscles in the ventricle to “fatigue” more easily.

Babies who are born suffering from hypoplastic right heart syndrome are usually cyanotic, having a bluish hue, from the moment immediately after birth. If the defect was not diagnosed in utero, the obstetrician or pediatrician who will check the baby will hear a discernible murmur with a stethoscope. The diagnosis can be further confirmed with an echocardiogram. When the baby is still in utero, radiologists usually already notice a marked difference in size in the ventricles when prenatal ultrasounds are performed. Unfortunately, not all are able to spot this and fetal echocardiograms are not a routine prenatal procedure.

When fetuses who are found to have hypoplastic right heart syndrome the parents are asked to go to a pediatric cardiology and pediatric cardiothoracic surgical team and they are usually on hand when the baby is delivered.

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