Hypoplastic Right Heart Syndrome (HRHS)

Information taken from fellow heart charity Little Hearts Matter

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.

For more information, read Little Hearts Matter’s booklet on Single ventricle heart conditions that affect the flow of blood to the lungs: https://www.lhm.org.uk/3d-flip-book/single-ventricle-heart-conditions-that-affect-the-flow-of-blood-to-the-lungs/

Contact

Please note: Children’s Heart Surgery Fund is not a medical resource and cannot provide medical advice. Heart conditions are very individual and what can be right for one person could potentially be dangerous for another. If you have any questions about yours or your child’s condition, please get in touch with Leeds Congenital Heart Unit directly, or the specialist nurses.

If you have any questions about your or your child’s heart condition, please contact the Cardiac Nurse Specialists

Children’s Cardiac Nurse Specialists

Phone number: 0113 3925467

Email: ccns.lgi@nhs.net

Adult Cardiac Nurse Specialists

Phone number: 0113 3928154
Email: leedsth-tr.achdnurse@nhs.net