[SCD-FORUM] 92E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Silvia Priori

[SCD Symposium]

Dear Sergio, dear Dr Cagnolatti,
I do not think that it is possible to conlcude that the index case has
Brugada Syndrome. Based on the description the 54 year old man has no
ECG pattern suggestive for Brugada Syndrome, he has no typical
arrhythmic event (monomorphic VT is no typical of the syndrome) .
Obviously the pharmacological challenge (at least with procainamide if
in the country of Dr Cagnolatti ajmaline or flecainide are not
available) should be performed. I presume that Dr Cagnolatti has
excluded short QT Syndrome, long QT, CPVT and the other inherited
arrhythmogenic syndromes. I would like to know the trigger for the first
cardiac arrest, if the patient had experienced syncopal events or
palpitations before the cardiac arrest: such information may help assess
the probability that the patient has Brugada Syndrome. I would also try
to get an ECG other family members (does the patient  have brothers and
sisters?) to evaluate if in the family there are other individuals with
an ECG suggestive for Brugada Sydnrome.
Obviously the management of the son is the fundamental clinical
challenge. If the boy is asymptomatic, even in the presence of a
spontaneous pattern, I would do nothing else than monitoring his
clinical course. I would consider the implant of a Reveal subcutaneous
events monitor just to be sure that there are no arrhythmic events. I
think that data from several labs concur nowadays to dicourage
programmed electrical stimulation for risk stratification, but of course
Dr Cagnolatti should decide whether he wants to propose this option to
his patient: I would not.
Silvia G Priori, MD;PhD


>
> Dear Sergio,
> About a case of reanimation of sudden death in a 54-year-old  
> patient, with no structural heart disease, with all the studies  
> made: coronary angiography, MNR, Holter, EPS.
> Everything appears to be normal. Monomorphic arrhythmic storm is  
> detected at the moment the patient is admitted. The patient is  
> provided life support and he recovers in 24 hs. ICD implantation is  
> decided. After three months he displays three shocks by arrhythmic  
> storm, incessant ventricular tachycardia. Mapping and focus  
> ablation is decided, in outflow tract with mismatch.
> To this moment, the patient has not displayed any event. It's been  
> three months, and in a Holter register in one of his sons, typical  
> Brugada pattern is observed. The patient is 17 years old,  
> asymptomatic. I cannot perform pharmacological test. What should I  
> do next?
>
> Dr. Cagnolatti  <drcagnolattia at hotmail.com>
>
> --
> Dr. Sergio Dubner
> President of Scientific Committee
>
> Dr. Edgardo Schapachnik
> President of Steering Committee
>
>
>
>
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--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee




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