IHSS

[BWD]

I am a 41-year-old white male diagnosed with IHSS in April 2001. Subsequently I had a Guidant AICD implanted in May 2001 and an ablation in June 2001 after several discharges from the ICD. I was very fit and active in athletics and now the quality of my life has been dramatically reduced. I am extremely fatigued, experiencing shortness of breath, dizziness and several blackouts.

I have taken 120 Mg. of Verapamil, 50 Mg. of Atenol with no significant improvement and subsequently both meds was discontinued. I was medication free for a while with the same symptoms. I am now on 100 Mg of Toprol Ex with no significant improvement. The left ventricular outflow tract gradient was 40 mmHg back in March. On Dec. 19th I had another angiogram to see if these symptoms I'm having were due to "clogged arteries" which proved to be negative. During this procedure "the catheter was unable to be placed at the left venticle apex due to extreme ventricular ectopy and irritability" and "left ventricular angiography reveals hyperdynamic left ventricle".  It should also be noted that my brother passed away at age 31 (enlarged heart), my aunt in her early 40's of a heart attack and my father in his early 50's.

The fact of the matter is that my "quality of life" has been severely diminished and my current medical management seems to be at a standstill. I want my life back and I want to "feel good" again without being fatigued or having shortness of breath, chest pains, dizziness or blacking out.
I am hopeful that you might give me some direction?

[CCF-M.D.-CRC]

Dear Brian,
40 mmHg is only a moderate gradient and would not explain all your symptoms.  I suspect you are having a much higher gradient with exercise.  Did they do any maneuvers to try to increase your gradient such as exercise or give you something to smell under your nose?  If not I would suggest you seek the opinion of someone who specializes in IHSS such as Dr. Harry Lever here at the Cleveland Clinic.  If your gradient is increasing with exercise then you may need either surgery or a catheter procedure to help decrease the gradient.  You should be able to have an active life with the proper treatment.  Here is some general information about IHSS.

IHSS (Idiopathic Hypertrophic Subaortic Stenosis), now commonly referred to as HOCM (Hypertrophic Obstructive CardioMyopathy), is a congenital (meaning that it runs in families) condition that results in thickening of the heart muscle (hypertrophy), a increased pressure gradient (obstruction) across the outflow tract and a cardiomyopathy (abnormal function of the heart muscle).  

Symptoms of HOCM include shortness of breath, lightheadedness, fainting and chest pain. Some patients experience cardiac rhythm disturbances which in some cases may lead to sudden death.   Due to this possibility patients with this condition are advised to avoid competitive sports (normal activity levels are OK). The obstruction to blood flow from the left ventricle increases the work the ventricle must do, and a heart murmur may be heard.

The treatment of HOCM may be several fold.  Medications, such as beta blockers and calcium channel blockers, are often given to attempt to decrease the workload of the heart.  Surgery may be recommended in patients with very high pressure gradients in the heart.  In a septal myectomy he surgeon goes into the heart and removes part of the muscle that is obstructing the blood outflow.   A alternative procedure that is being developed here and at several other centers that avoids the need for surgery.  A catheter (tiny tube) is threaded through the leg to the heart and the blood vessels that supply the thickened heart muscle are identified and then closed off with a solution of alcohol.  This results in a thinning of that particular area of the heart.  Not all patients are eligible for this procedure and there are benefits and risks to both procedures.

The issue of sudden death is not addressed by surgical means but rather by the implantation of a device called a defibrillator.  This device is similar to a pacemaker and keeps track of the heart rhythm.  If it detects a life-threatening rhythm it delivers a shock of electricity to the heart to get it back into regular rhythm.  Once again, not everyone is eligible for this device and there are benefits and risks involved.

Additional web sites with information about HOCM are listed below.

http://www.americanheart.org/Heart_and_Stroke_A_Z_Guide/cmyopa.html

HOCM Support group

http://www.kanter.com/hcm/

National Organization for rare diseases

http://www.rarediseases.org/

HOCM of Canada

http://www.cadvision.com/hcmac/hcmac.html

Mayo Clinic HOCM Clinic

http://www.mayo.edu/cv/wwwpg_cv/hocm_cln/hocm.htm

Article on septal ablation

http://www-east.elsevier.com/jac/3102/jac5431fla.htm

More info

http://www.arrhythmia.com/patient/other/HCM.html

I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Only your physician can provide specific diagnoses and therapies.  Please feel free to write back with additional questions.

If you would like to make an appointment at the Cleveland Clinic Heart Center, please call 1-800-CCF-CARE or inquire online by using the Heart Center website at www.ccf.org/heartcenter.  The Heart Center website contains a directory of the cardiology staff that can be used to select the physician best suited to address your cardiac problem.

[Ruthie334]

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[Experanixx]

Hi everyone..My name is Ryan and I am a 20 year old male in Columbus, Ohio.  I was diagnosed with IHSS at birth, and it has gotten progressively worse.  I was wondering if anyone had any knowledge of a support group in which to talk about this, as I am having a lot of emotions and thoughts.  Any advice would be appreciated.  Thank you.

Ryan Holy
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[Neale]

I totally understand your position. I am a 38 yr. old married female with 3 children.  I was also diagnosed with severe hypertrophic cardiomyopathy in 2000.  I  received a guidant ICD in April 2000. I have many of the same symptoms that you described. But, I have not gotten my first jolt yet!

I would strongly urge you to visit Dr. Harry Lever at the Cleveland Clinic, if you have not already been there.  He is a world expert on IHSS.  Because the condition is fairly rare, I visited Dr.Lever, who sees this condition on a daily basis.  I was confident of the testing and the answers that I was given during my visit.  I left with a positive emotional and physical well being.  Perhaps this could help you too.

Best of Health