[SCD-FORUM] 37E Young woman with HCM? Dr. Asenjo

[SCD Symposium]

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>



--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee




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