RT-94
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Long QT syndrome: clinical,
morphological and clinical features
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Leo A. Bockeria, Elena Z.
Goluchova, Irina P. Poliakova, Eugeni R. Pavlovitch.
Bakoulev Center for Cardiovascular Surgery, Academy of Medical Sciences, Moscow,
Russia
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Abstract
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The long QT syndrome (LQTS) is relatively rare but one
of the most malignant type of ventricular arrhythmias. Leading mechanisms have
theorethical potentials but still poorly defined in clinical practice. We evaluated 30 pts
with LQTS. Eighty-seven per cent of them experienced syncopes and 43% were resuscitated
from sudden cardiac death under B-blockers therapy. The aim of the study was the
elaboration of some approaches providing additional aid in the identification of pts with
LQTS and syncope of unknown origin and analysis of morphological features and possible
mechanisms of the disesase. According to our data body surface mapping was very helpful in
the diagnosis of concealed forms of the disease since its markers were quite specific. The
most expressive and significant feature in the pts with LQTS was the multipolar electrical
heart field distribution with additional minima (negative extreme) mainly on the right and
right anterior part of thorax. In 12 pts the biopsies from sinoatrial zone were studied.
In all those cases the destructive features of all tissue components (nerve fibres,
myocytes, connective tissue, blood vessels) were found. Most expressive and significant
damages were obtained precisely in nerve fibres of sinoatrial area. The multiplication of
bazal lamina, damage of nerve fibres and cells' contacts could be testified as the
existence of long-term continuously relapsing destructive and regenerative processes.
Thus, body surface mapping reflected the functional asymmetry of the ventricles and was
useful for the diagnosis of the concealed "beheaded" forms of the disease since
it had high specific and sensitive marker. It could be also used as screening tool in pts
with syncopes of unknown origin. The multiple arrhythmias and specific morphological
findings observed basically in the nerve fibres testified in favour of primary
cardioneuropathy.
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Key Words
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Long QT syndrome
malignant ventricular arrhythmia, body surface mapping, functional, morphological and
clinical features, primary cardioneuropathy, OA
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