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

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.

hypoplastic right heart syndrome

Causes

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.

Symptoms

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.

 Read more about Hypoplastic Right Heart Syndrome life expectancy

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Hypoplastic Right Heart Syndrome (HRHS)

From the article it is clear that HRHS is most commonly found in new born. So does it means that it is a genetical disorder ? 

Hypoplastic Right Heart

Increases life expectancy by 15-20 yrs at least? Meaning..... from birth? Like, they'll die at 15-20 yrs old??

Hypoplastic Right Heart

Not quite. Think of it as the expectancy of the heart itself. So, with follow-up, 15-20 years later it will be determined if the person should be listed for a heart transplant.

I'm alive!!

Hi i'm a 24 year old Truck driver with Hypoplastic right ventricle, i've been travling been skydiving ect... no plans of dieing soon!!

Medical Advice

I am 16 years old girl and already a few days my heart palpitations. Do not get stressed, do not drink any alcohol, not smoke. This may indicate a heart pounding heart disease? In the four years I was sent to theÿ echocardiography without cardiac problems only preventive pediatrician that due to my hydrocephalus exclude hypoplastic heart syndrome. This syndrome was excluded by echocardiography. If these my current heart problems (palpitations at rest) meant something more serious, followed by heart surgery? So what would it be? Heart disease? If it was this, what is the treatment?

Medical Advice

First of all palpitations can be normal. A serious heart defect was ruled out by echocardiography. I would advise to have blood checked, especially potassium level and thyroid function. Additionally you should have an holter ecg done, to verify if there really is any abnormality in heart beat (which can even arise in normal healthy people).

Please have a look at our articles section ( e.g holter ecg  etc).
If you have the results, please feel free to contact us again.

 

Medical Team, Heart Consult

hrhs

My daughter Jehnesys has hypoplastic right ventricle and actually the life span is 15-30 says my daughter's Surgeon. I have faith and I belong to an internet group and one child actually grew her right ventricle and veins and they actually had to go in and reverse the procedures so anyone can say anything but faith moves mountains!!!

HRHS

Thank you so much for your post,,,,was having a bad day and your post gave me hope and reminded me to pray.... Thanks,

fatih

yes we do have to have faith, My daughter is 10 and has HRHS. So far so good, she has had no serious complications, and God willing, will live as long as I will!!!