[SCD-FORUM] 48E RE: Young woman with HCM? Dr. Cano

[SCD Symposium]

Dear Organizers of the Symposium,

Thank you very much for this extraordinary Symposium!!!

I was very impressed with the HCM cases.

I work since 1998 with patients carriers of obstructive HCM, very  
symptomatic. We perform septal ablation by alcohol on them (already  
37 patients).

In the histories they describe, no patient had echo stress to  
determine if significant gradient appears with stress, if there is  
hypotension with strain. Often times it is difficult for them to  
articulate their symptoms, and when asked, we see that they are  
virtually invalid; they hardly leave their houses. They don't walk;  
well, they live in a secluded way. We only have one patient with ICD  
and it never shocked.

The symptoms only improved after the obstruction was removed, and  
pulmonary hypertension disappeared when we decompressed the atrium.

   Silvia Judith Fortunato de Cano

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee


> I write to you to introduce a clinical case. This patient is a 30  
> year old woman, with a family history of non obstructive HCM with  
> genetic study that found a mutation in the Troponin T gene (TNT)  
> located in chromosome 1. The mutation is Arg92Gln. Four cousins  
> under 20 have suffered sudden death and one 45 year old aunt has  
> also suffered sudden death. Her mother and 3 other cousins have a  
> CDI because of  the family history and evident HCM in Echo. Another  
> aunt and one cousin also have a CDI because syncope and evident HCM.
>
> Currently this  patient has not presented symptoms and the EKG  
> shows a negative T wave in V1-V3 ( not present years ago), and the  
> previously normal Echo has changed, and now shows a mild  
> enlargement of left atrium and mild  mid septum thickening of 12-  
> 13 mm, with no abnormal aspect and no obstruction. MRI is similar  
> to the Echo, and shows normal aspect of septum but thickness is  
> 13mm. No arrhythmias during 24 hrs Holter nor exercise testing.
>
> She is on atenolol but we would like to have your input in this  
> case on whether this justifies a CDI now.
>
> Besides, we would like to know your opinion about young members of  
> this family. There are children and young persons with apparently  
> normal Echo but they have one or two brothers with sudden death or  
> syncope. ¿Do you think they also need a CDI? Finally, what about  
> the adult and older members of this family? Probably most  have an  
> evident HCM in Echo. Would it be enough with atenolol or with  
> amiodarore alone, or do they also need a CDI?.
>
> We appreciate and thank you for your time and suggestions.
>
> Most Sincerely
>
> Rene Asenjo <reneasenjo at vtr.net>
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