[SCD-FORUM] Seven year old boy with suspected LQT3 Dr. Makarov reply for Dr. Schwartz

[SCD Symposium]

Dear Dr. Schwartz, thank you very match for your comments. Of course in my
comment to Dr. Costa's pts I  mean first of all of replay of the QT interval
under beta blockers, previously in pts with LQTS patients without genotype
confirmation or LQT1-LQT2 which under follow up in our clinic a long period
(Makarov L et al. Holter monitoring in the long QT syndrome of  children and
adolescents. COR VASA 32(6): 474-483 1990 - S.N. Chuprova, et al. The
particularities of clinical features in families with different genetics
variants of long QT syndrome. Eur Heart J 2006, 27. Abstract Suppl,
724-725). From unrelated Russian families with LQTS LQT3 type was found in
6,5% pts. Against a background of regular intake of beta-blockers 15,7% pts
with LQTS had recurrence of syncope and 12,9% pts suddenly died in most
cases - pts with LQT2 and LQT3 (S.N. Chuprova  et al. The analysis of
structure of sudden cardiac death in families with various molecular-
genetic variants of inherited long QT syndrome Eur Heart J 2006, 27,
Abstract Suppl, 725). In some pts we noted shorthening of the QT interval
under therapy (beta blockers, K and spironolacton in probably and confirm
LQT2, mexelitine in probably LQT3 but without complete normalization of the
QT interval). Our experience in using of mexelitine  in LQT3 not intensive
becouse we not have regulary it drug in Russia (flecainid not use in
Russia). As I understood dr. Costa not have confirmation of the LQT3 variant
in her patient that need by my opinion or dying of it test or using standard
for LQTS nongenospecific therapy.   Thank you very match for your comments
and mention another time. No doubt that your biggest in the world experience
in LQTS pts diagnostic, treatment and follow up is mostly important for us. 
It is a pity that this terrific Symposium had finished so soon. Thak you 
very match for all organaizers and experts.

Sincerely yours Dr. Leonid Makarov