Hypoplastic Right Heart

Last updated December 2021

Some doctors give their patients the diagnosis of Hypoplastic Right Heart. This is a collection of problems on the right side of the heart. There is usually a malformation of the valve (tricuspid valve) between the right-sided collecting chamber (atrium) and the right pumping chamber (ventricle). The right ventricle is very small (hypoplastic).

There is a malformation of the lung artery valve (pulmonary valve) and a small (hypoplastic) lung blood vessel (pulmonary artery). These malformations affect the circulation of returning blue (deoxygenated) blood to the lungs where it normally picks up oxygen to take around the body. Babies with this condition will usually require surgery within the first week of life.

The babies may be blue (cyanosed) at birth because of a lack of red oxygen-filled blood flowing around the body. To give the baby a chance to recover from the birth and for the doctors and parents to discuss and plan treatment it is important to maintain the fetal blood flow described above. Doctors start an infusion of Prostaglandin, a drug that mimics the mother’s hormones, straight after birth. This drug slows the closure of the fetal ducts and holes, giving the team time to plan treatment.