[SCD-FORUM] 36E RE: A female with burned-out phase of HCM? Dr. Furlani

[SCD Symposium]

Dear Dr Alizadeh:
What an interesting case! A difficult one too.
It sounds like familial dilated cardiomyopathy versus final dilated  
phase of hypertrophic cardiomyopathy, but I would like to have more  
information. What exactly is her LV ejection fraction?  Were there  
any LV wall motion abnormalities in her Echo? Has she ever had  
typical chest pain? What about her cholesterol levels? (a familial  
dyslipemia could explain an early onset of ischaemic heart disease in  
this patient).
Does she have a history of hypertension? I mean, even though LV  
hypertrophy secondary to hypertension is usually concentric,  
sometimes severe hypertension could lead to asymetric LV hypertrophy.
Assuming there is no any significant left sided valvular disease, the  
presence of severe RV dysfunction and moderate to severe pulmonary  
hypertension (PAPS 65-70 mm Hg) implies long standing severe LV  
dysfunction in this patient and usually poor prognosis (and lack of  
response to CRT). Moreover, if this patient has severe mitral  
regurgitation secondary to severe LV dilatation CRT is unlikely to  
benefit her.
Regarding her medical treatment, I assume she is on low dose  
diuretics and ACEI but she is off beta blockers and anti-aldosterone  
drugs. After pacemaker implantation, an optimize medical treatment  
including appropriate dose of ACEI, beta-blockers and anti- 
aldosterone drugs will probably improve her LVsystolic function  
rendering unnecessary ICD/CRT therapy.
Finally, There is a clear indication for permanent pacemaker  
implantation (complete AV block with a wide QRS complex escape  
suggesting infra-hisian block). RV apical pacing will induce, LV  
dyssynchony that, in turn, will probably lead to worsening of both LV  
systolic function and CHF symptoms, but high septal RV pacing is less  
likely to cause severe LV dyssynchrony.
In summary, If her LVEF is over 30% I will go for a DDD pacemaker  
with an active fixation lead in the high RV septum plus optimization  
of medical treatment (ACEI, BB, anti-aldost). However, if her LVEF is  
clearly below 30% (by nuclear ventriculography ideally) I will  
implant either a CRT-ICD in the case of lack of severe mitral  
regurgitation or DDD-ICD (usually as a bridge to cardiac  
transplantation) when severe MR is present. The lack of response to  
CRT will make this young patient a good candidate to cardiac  
transplantation.
Thank you all.
Congratulations to the organizers.

Aldo Alberto Furlani MD
Cardiac Electrophysilogist
Consultant Cardiologist
Heart Institute of the Caribbean
afurlani at caribbeanheart.com
www.caribbeanheart.com

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee




El 12/10/2006, a las 23:12, SCD Symposium escribió:

> Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium
> ______________________________________________________________________
>
> Medtronic's free physician resource center:
> http://www.medtronicconnect.com
> ______________________________________________________________________
>
> Dear Dr Dubner
>
>   thank you for this excellent  educational site
>
>   A 38 year old femal referred to our center with new onset dyspnea  
> from a few month ago. She had family history of sudden cardia  
> death. She had CHB with wide ventricular escape rhythm.  
> TT.echocardiography revealed sever LV and RV systolic dysfunction,  
> asymetric septal hyperthrophy, pulmonary hypertention  
> (PAP=65-70mmhg) and without LVOT gradient.patient,s dyspnea  
> improved with low dose diuretics , ACE inhibitor and TPM implantation.
>   Do you recommend DDD pacemaker for this patient?
>   or
>   Do you recommend  ICD-DR or ICD-CRT?
>
>   what is your opinion about clinical course of her disease?
>
>   does she need further workup before device implantation?
>
>   sincerely yours
>
>   Hormoz Alizadeh MD
>   Department of Pacemaker and Electrophysiology
> Rajaie Cardiovascular Medical and Research Center
> Tehran, Iran
>
> --
> Dr. Sergio Dubner
> President of Scientific Committee
>
> Dr. Edgardo Schapachnik
> President of Steering Committee
>
>
>
>
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