[SCD-FORUM] 93C RE: Comments to clinical case Cuilan Li et al. Clinical patient with catecholamine sensitive polymorphic VT. Dr. Cuilan Li to Dr. Makarov

[SCD Symposium]

Dear Dr. Makarov:
About the essentiality to perform diagnostic studies in family  
members of a victim of SD by noncoronary origin and had no background  
of cardiological disease, from my point of view, they really need  
it.     We saw a 28-y-o young man last year who asked for help to  
make sure if he has the risk to die from heart disease.  The reason  
is that  his mother had SD when she was riding bicycle on the going  
to work road and his younger sister had SD when she was doing  
physical exercise in Junior High School.  The young man and his  
father were scared by these events.  Fortunately, after doing  
examinations such as 12 lead ECG, Holter, Exercise test,  
Echocardiogram, no abnormality was found for him and his father,  
suggesting that the carriers of disease-causing gene (whatever they  
suffer from) might all gone and the left people may not be a  
carrier.  The first three useful diagnostic studies should be ECG  
(sometimes including Holter recording), Exercise test,  
Echocardiogram.  Based on the indication from these data, further  
study such as EPS may be considered.

Cuilan Li
Cuilan Li, Ph. D
Department of Cardiology,
Peking University People’s Hosp.,
Beijing 100044, China
Tel: 86-10-68314422 Ext. 5940
licuilan at gmail.com

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee


>
> Dear collegues! I want to add my comments to clinical case thad  
> presented Dr. Cuilan Li et al. “Clinical presentation and  
> management of a patient with catecholamine sensitive polymorphic  
> ventricular tachycardia (CPVT)”. By my mention it is typical  
> clinical and ECG pattern for children and young patients with some  
> of kind of catecholaminergic VT (CVT) that we described before  
> (Makarov L. et al. Short PR interval, high circadian index and  
> bradycardia – pattern with high risk of syncope and sudden death in  
> children with catecholaminergic ventricular tachycardia. Europ  
> Heart J 2004; 25: 222, Suppl. Abstract) and during this symposium:  
> bidirectional VT, bradycardia, short PR interval, and double  
> tachycardia (VT and SVT).  Some of this patients also demonstrated  
> of the shortening of the QT interval (possibly as result of calcium  
> overload of cardiomyocites).  I am sure in arrythmogenic origin of  
> syncope in present patient and useful of the active anthyarrhythmic  
> therapy for it.   From drugs more effective Beta-blockers (1-2 mg/ 
> kg) or/and calcium antagonists (verapamil), but authors had  
> detected proarrhythmia from verapamil, that not typical for our  
> experience.  In the some patients the propafenone can be effective.  
> Most of our patients with CVT showed improvement in their clinical  
> symptoms: syncope events and ventricular tachycardia did not recur.  
> But in a numerous patients that tolerant to therapy with recurrent  
> syncope implantation of the ICD could be useful. I am not sure in  
> useful of the EPI study for this patient, repetitive of Holter,  
> stress test and/or IV infusion of isoproterenol more frequently  
> detected typical bidirectional VT. Concern authors question   # 3  
> (The catecholamine concentration in blood was normal for this  
> patient. What do we expect of the catecholamine concentrations in  
> CPVT patients in general?)- Fisher J.et al. (JACC1999; 34 (7):  
> 2015-22) showed normal or lower level of plasma catecholapimes in  
> rest in this patients, but parid increasing of it during exercise,  
> to compare with normal range.
>
> Dr. Leonid Makarov M.D., Ph.D. professor (Pediatry)
>
> Moscow Institute pediatry and children surgery.
>
> 125412 Taldomskaya str.2 , Moscow, Russia.
>
> leo at oss.ru
>
>
> --
> Dr. Sergio Dubner
> President of Scientific Committee
>
> Dr. Edgardo Schapachnik
> President of Steering Committee
>
>
>
>
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