Hypoplastic Right Heart Syndrome (HRHS): Causes,Symptoms and Treatment

Submitted by webmaster on Wed, 10/17/2018 - 14:34

The Hypoplastic right heart syndrome (HRHS) is a heart condition when the right hand side of the heart is less developed than the left one. In fact, the right hand side of the heart is not strong enough to perform all the heart functions smoothly.

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The most common cause that results in this syndrome is due to the development of a small pulmonary valve and as a result, there is not only a small pulmonary artery but also a small ventricle, an atrium and a small tricuspid valve on the right side of the heart. If there is not sufficient flow of blood through all these parts, their growth is stalled or hampered. The concern arises out of the fact that the pulmonary valve which opens and closes and is responsible for the blood to flow to the pulmonary artery ,is absent altogether. As a result, the blood flow into the coronary arteries is not normal and hence leads to damage of the heart muscles.
It is clear that the development of these structures around the pulmonary valve is what results in Hypoplastic right heart syndrome. Yet the severity of the ailment depends on the time at which it started and the amount of excess development around the pulmonary valve.


In babies with hypoplastic right heart syndrome, the symptoms start to appear after they are born because when in the womb, there is Foramen Ovale and a Patent Ductus Arteriosus;the two connections in the heart which get closed when the baby is born. That is when the first signs of Hypoplastic right heart syndrome start to appear as breathing becomes difficult. Consequently, a bluish hue starts to appear on the skin, the lips ,and  other parts of the body.

If these excess structures develop in an infant’s heart, then the condition gets severe with passing time because  blood flow to the right side of the heart will not be sufficient to assist in the growth of the heart, as the blood is distributed amongst the excess structures. Hence the right side of the heart stops growing or does not grow as much as the left hand side.  As the ventricle fails to grow and the development of the muscle structure is poor, while one side of the heart grows strong ,the right hand side of the heart does not attain the optimum growth or strength. When the heart tries to pump blood to the lungs from the pulmonary valve, it causes problems with the flow and one starts to have breathing problems.  The condition usually worsens with time.
It is not difficult to diagnose this heart condition as there is a “murmur” that can be heard because of the uneven beating of the heart, and an echocardiogram is all that is required to confirm it.

Life Expectancy

However, once the condition is diagnosed, there are surgical procedures which once carried out on the heart increases life expectancy by fifteen to twenty years at least.