Family History of IHSS

[Rmhley]

My father passed away at the age of 31 from Idiopathic Hypertrophic Subaortic Stenosis.  I am a 25 yr old female who is pregnant.  I had an echocardiogram when I was 24 and there was no sign of IHSS.  I was wondering what the chances are of me developing this condition.  

[Rmhley]

My father did have it, but it wasn't dianosed until he was in his early-mid twenties.  He also did have rheumatic fever as a child, which has some implications with the disease as well.  Everyone in my family has been tested for it, but no one has been diagnosed with it.

[gtojohnny]

You would only know it is genetic if one of your parents had it. If you have developed the first case in your family, then it started with you, but could be genetic from that point on.

[Rmhley]

I did indeed have a Stress Echo and everything with that was just fine.  I was on an exercise progrm prior to getting pregnant and had had no problems with pain, lightheadedness, or anything. No loss of conciousness ever, no shortness of breath.  

Since it use to be IHSS doesn't idiopathic mean that they don't know where it came from?  How would you know if it were genetic or congenital?

[Cleveland Clinic]

Provided this echocardiogram was performed specifically looking for HOCM ( IHSS is an older term not used very much concurrently), and read by a specialist who is used to looking at other associated abnormalities including papillary muscle and mitral valve abnormalities, and at the LVOT gradients both with amyl nitrite or valsalva maneuver, then I think the findings on the echocardiogram are reassuring that you do not have HOCM. Perhaps a better way to look for this is with a stress echocardiogram, where a functional capacity can be measured and LVOT gradient with exercise. Have you had any symptoms of syncope, or lightheadedness? Exertional chest pain or angina? What about dyspnea. These are all important associated symptoms that have to be taken nto account in assessing the risk for sudden cardiac death.

[gtojohnny]

IHSS usually presents shortly after puberty(can present earlier) in some sort of detectable form through echo, murmur or symptoms. Although, there is still risk and you should continue to be checked periodically. My father passed away at 41 from IHSS and I was officially diagnosed at 30, but I had a murmur in my teens. My GP always told me not to worry about it. Boy, was he wrong. At 30 I developed an accelerated heart rate and went to a Cardiologist. Right away he diagnosed me from the sound of the murmur. Without a genetic test, it can't be ruled out but I would say your'e beating the odds so far. Good Luck!