When are other options needed

My husband is 40 yrs old and has been treated for HOCM for 3
years.  He has been on atenolol and within the past 4-5 months
Tiazac.  The Tiazac has been increased once due to PVC's. He is on 180 mg daily.  The PVC,s are under fair control but he is
short of breath at times at rest and has fatigue.  At present
he is able to work but worried that this may soon interfer
with working.  His cardiologist is not real familiar with HOCM
but looking.  I would like to know when we need to look into
the other options such as septal ablation or septal myectomy.
Also we have a 10 yr old daughter who plays sports, how often
should she be monitored tobe sure she hasn,t inherited this.

[Cleveland Clinic, MD]

Dear Shirley,

I think you are right that your husband needs to see a specialist in the area of HOCM.  Dr. Harry Lever here is a world expert in this condition and I would highly recommend him if you wish to come to Cleveland.  You daughter should be screened with echocardiogram at least once before she is 18 to make sure she does not have HOCM.  Below is some general information about HOCM.

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.


Additional web sites with information about HOCM are listed below.

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

HOCM Support groups

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

http://www3.bravenet.com/forum/show.asp?userid=qf175386

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.