HRHS stands for Hypoplastic Right Heart Syndrome. It occurs when the right side of the heart is underdeveloped. It disrupts the normal flow of blood in the heart and requires early medical intervention. 1
In a normal heart, the right side is responsible for pumping oxygen-poor blood from the heart to the lungs to receive oxygen. The left side is responsible for pumping the now oxygen-rich blood to the rest of the body. 2
In a HRHS heart, the right side does not work correctly. Structures on the right side of the heart such as chambers, valves and related blood vessels are malformed. This leaves the HRHS patient with oxygen-poor blood. The right side is not able to function properly and get blood to the lungs to be oxygenated. This in turn makes the rest of the body and organs suffer from low oxygen. 3
HRHS is more of a broad-term used to describe many malformations dealing with the right side of the heart. The most common defect associated with HRHS is tricuspid atresia. 4 Tricuspid atresia occurs when the tricuspid valve, which is responsible for allowing oxygen-poor blood to flow from the right atrium to the right ventricle, is not formed or is blocked off. This does not allow a way for blood to reach the right ventricle. In conjunction, the right ventricle is small and there is usually narrowing in the pulmonary valve and artery. 5 Overall this disrupts the normal flow of blood through the right side of the heart to the lungs.
Before life saving interventions and surgeries, HRHS meant death shortly after birth. Now, past research has given way to advancement in the medical field that is letting HRHS patients live to adulthood. Though these advancements have proven life saving, they are still only temporary fixes until the next needed intervention. These surgeries also have unknown long term outcomes and are thought to create other organ problems as time progresses. 6
Living to adulthood is not enough for us. We want to ensure HRHS and CHD patients as a whole live to an old age with a limitless life.We need a cure for HRHS. We need a cure for CHD. A change in the way we look at CHD treatment needs to change. We have set a goal to collaborate with researchers all over the world and grow a heart made from a patient’s own stem cells by 2040. Will you join us?