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Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia

HAYASHI, Meiso ; DENJOY, Isabelle ; KAMBLOCK, Joël ; MESSALI, Anne ; GUICHENEY, Pascale ; LUNARDI, Joël ; LEENHARDT, Antoine ; EXTRAMIANA, Fabrice ; MALTRET, Alice ; ROUX BUISSON, Nathalie ; LUPOGLAZOFF, Jean-Marc ; KLUG, Didier ; HAYASHI, Miyuki ; TAKATSUKI, Seiji ; VILLAIN, Elisabeth

Circulation (New York, N.Y.), 2009-05, Vol.119 (18), p.2426-2434 [Periódico revisado por pares]

Hagerstown, MD: Lippincott Williams & Wilkins

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Título:
Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia
Autor: HAYASHI, Meiso ; DENJOY, Isabelle ; KAMBLOCK, Joël ; MESSALI, Anne ; GUICHENEY, Pascale ; LUNARDI, Joël ; LEENHARDT, Antoine ; EXTRAMIANA, Fabrice ; MALTRET, Alice ; ROUX BUISSON, Nathalie ; LUPOGLAZOFF, Jean-Marc ; KLUG, Didier ; HAYASHI, Miyuki ; TAKATSUKI, Seiji ; VILLAIN, Elisabeth
Assuntos: Adolescent ; Adrenergic beta-Antagonists ; Adrenergic beta-Antagonists - therapeutic use ; Adult ; Biological and medical sciences ; Blood and lymphatic vessels ; Calsequestrin ; Calsequestrin - genetics ; Cardiology and cardiovascular system ; Cardiology. Vascular system ; Child ; Child, Preschool ; Coronary heart disease ; Death, Sudden, Cardiac ; Death, Sudden, Cardiac - epidemiology ; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous ; Exercise Test ; Family Health ; Female ; Follow-Up Studies ; Heart ; Human health and pathology ; Humans ; Incidence ; Kaplan-Meier Estimate ; Kaplan-Meiers Estimate ; Life Sciences ; Male ; Medical sciences ; Middle Aged ; Polymorphism, Genetic ; Risk Factors ; Ryanodine Receptor Calcium Release Channel ; Ryanodine Receptor Calcium Release Channel - genetics ; Syncope ; Syncope - genetics ; Syncope - mortality ; Tachycardia, Ventricular ; Tachycardia, Ventricular - genetics ; Tachycardia, Ventricular - mortality ; Tachycardia, Ventricular - prevention & control ; Young Adult
É parte de: Circulation (New York, N.Y.), 2009-05, Vol.119 (18), p.2426-2434
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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Descrição: The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32% in the total population and 27% and 58% in the patients with and without beta-blockers, respectively. Absence of beta-blockers (hazard ratio [HR], 5.48; 95% CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95% CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13%. Absence of beta-blockers (HR, 5.54; 95% CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95% CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking beta-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.
Editor: Hagerstown, MD: Lippincott Williams & Wilkins
Idioma: Inglês

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Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia

HAYASHI, Meiso ; DENJOY, Isabelle ; KAMBLOCK, Joël ; MESSALI, Anne ; GUICHENEY, Pascale ; LUNARDI, Joël ; LEENHARDT, Antoine ; EXTRAMIANA, Fabrice ; MALTRET, Alice ; ROUX BUISSON, Nathalie ; LUPOGLAZOFF, Jean-Marc ; KLUG, Didier ; HAYASHI, Miyuki ; TAKATSUKI, Seiji ; VILLAIN, Elisabeth

Hagerstown, MD: Lippincott Williams & Wilkins

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