SciELO - Scientific Electronic Library Online

vol.31 número2Actualización sobre el manejo de los pacientes con el patrón electrocardiográfico del síndrome de BrugadaRevascularización coronaria incompleta y eventos cardíacos posteriores a intervencionismo coronario percutáneo índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados




  • No hay articulos citadosCitado por SciELO

Links relacionados

  • No hay articulos similaresSimilares en SciELO


Revista Cubana de Investigaciones Biomédicas

versión impresa ISSN 0864-0300


CRUZ CARDENTEY, Marleny. Catecholaminergic polymorphic ventricular tachycardia. Rev Cubana Invest Bioméd [online]. 2012, vol.31, n.2. ISSN 0864-0300.

Catecholaminergic polymorphic ventricular tachycardia is a channelopathy characterized by the induction of polymorphic ventricular arrhythmias in the presence of catecholamines. It should be suspected in any young patient, especially a child or adolescent, presenting with syncope associated with physical exercise or emotional stress, with no structural heart disease and an ECG showing a normal QT interval. It is a rare disease, its importance lying in the high risk of sudden death, which may sometimes be its debut. Ventricular arrhythmias may be polymorphic or bidirectional. They are highly inducible by physical exercise and Isuprel infusion, their threshold is predictable and their complexity progressive. A family history of sudden death is reported in 30 to 40 % of patients. Two genetic mutations have been identified as causes of the condition (ryanodine receptor 2 with autosomal dominant inheritance and calsequestrin 2, with autosomal recessive inheritance). However, a causal mutation has been found in only 50-55 % of patients. Mutations influence sarcoplasmic reticulum Ca 2+ leak, facilitating the appearance of late post-depolarisations, which will in turn induce ventricular ectopic activity. Beta-blockers are the treatment of choice. The automatic implantable defibrillator is indicated in patients recovered from a sudden death event and in those who remain symptomatic despite medical therapy. Left cardiac sympathetic denervation, verapamil, flecainide and propafenone are alternative options for patients who remain symptomatic despite the use of beta-blockers.

Palabras clave : catecholaminergic polymorphic ventricular tachycardia; sudden death; channelopathies.

        · resumen en Español     · texto en Español     · Español ( pdf )