Re: hypertrophic cardiomyopathy

Posted By CCF Cardio MD-SGM on December 12, 1997 at 19:59:24:

In Reply to: hypertrophic cardiomyopathy posted by barbara on December 01, 1997 at 16:55:53:







: Hello, I posted back in October with no reply, so I am trying again. I am 35 and was diagnosed with IHSS in 1994 while I was pregnant with my 2nd child.  I went into CHF shortly after giving birth.  I had a heart biopsy which showed disarray of the myocites and mature scar tissue.  I was told that I had "burnt out hypertrophic cardiomyopathy."  Could you please give me a definition of this.  I understand that my heart is now dilating.  My septum measurement has gone from an abnormal measurement (I think it was 2.1) to a normal measurement of .9 within the past 2 years, however the left ventricle has enlarged.  Do I still have IHSS - even though my septum isn't thickened any more - could the septum have had a larger measurement because I was pregnant?   I haven't been able to find much information on burnt out hypertrophic cardiomyopathy - is this a rare condition?   What are the medications for this condition?  I am not in heart failure at this time.  Thanks so much for your help
Barbara



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Dear Barbara,
Sorry for the delay in answering your question.   Indeed, it appears that you have a familial form of hypertrophic cardiomyopathy(HCM) as evidenced by your mother's disease and also by your son's possible involvement.  The biopsy results and the initial echocardiographic features of septal hypertrophy would support this diagnosis, although it would be important to have the biopsy reviewed by an experienced pathologist to rule out other causes of increased ventricular wall thickness (i.e. infiltrative diseases of the heart).  I assume that this has been done, and that the diagnosis is indeed HCM.    
A subset of individuals with HCM develop progressive left ventricular dilation and pumping dysfunction.   This is sometimes referred to as "burnt out" HCM, although this label doesn't help explain the situation.   Regarding your current septal measurements, your initial diagnosis of HCM is still accurate, despite the fact that dilation of the ventricle has resulted in more normal measurements.  This is not an uncommon development in the long-term disease process.   However, given the fact that a dynamic obstruction to outflow from the ventricle doesn't seem to be the case at present, it would be wise to treat you with medications directed at slowing the progression of left ventricular dysfunction.  These would include beta-blockers(Coreg) and ACE inhibitors.    Regarding your question about pregnancy, it is true that pregnancy induces many significant changes in the cardiovascular system. However, significant hypertrophy(thickening) of the heart muscle is not part of the natural process.  Rarely, pregnant women experience a condition known as post-partum cardiomyopathy, where the pregnancy is associated with dysfunction and sometimes dilation of the left ventricle.  This process is distinct, and not typically associated with HCM.  The ventricular dilation and dysfunction that you describe are likely due to HCM and not post-partum cardiomyopathy.  
Regarding the issue of screening, it would be wise to periodically check both your children for this condition with echocardiograms, so that appropriate therapy can be prescribed, if necessary.  I recommend you discuss this with your pediatrician and cardiologist.   In your initial question, you raised the issue of PVC's and defibrillators.  Certainly, individuals with HCM are at risk of arrhythmias that can result in hemodynamic instability.  It is possible that this problem was at issue with your mother's premature death.  If you have evidence of ventricular tachycardia on Holter monitoring, or if you have experienced episodes of loss of consciousness, it may be wise to consider a defibrillator. Again, this is a complicated issue that needs thoughtful discussion with your cardiologist.  
You would do well to be seen by an expert in this field-- someone who manages HCM on a routine basis.   If you would like to be evaluated at the Cleveland Clinic, you can call 800 CCF-CARE for an appointment with Dr. Lever at desk F15.    I hope I have been of assistance.  
Information provided in the Cardiology Forum is for general purposes only.  Diagnosis and therapeutic decisions can only be made by your personal physician.