Polymorphic ventricular tachyarrhythmias in the absence of organic heart disease: classification, differential diagnosis, and implications for therapy.

Different polymorphic ventricular tachyarrhythmias may cause syncope or cardiac arrest in patients with no heart disease: (1) Catecholamine-sensitive polymorphic ventricular tachycardia (VT) presents during childhood: the hallmark is the reproducible provocation of atrial and polymorphic ventricular arrhythmias during exercise, despite a normal QT. Beta-blockers are the treatment of choice. (2) In the long QT syndromes (LQTS), malfunction of ion channels leads to prolonged ventricular repolarization, early afterdepolarizations, and triggered ventricular arrhythmias. Therapeutic options include: beta-blockers, genotype-specific therapy, cardiac sympathetic denervation, and implantation of pacemakers or defibrillators. (3) The "short-coupled variant of torsade de pointes" is a malignant disease that shares several characteristics with idiopathic ventricular fibrillation. Although verapamil is frequently recommended, mortality rates remain high. (4) Idiopathic ventricular fibrillation (VF) with normal electrocardiogram (ECG) strikes young adults of both genders. In contrast to other polymorphic tachyarrhythmias, idiopathic VF is not generally related to stress. Also, familial involvement is rare. Therapeutic options include implantation of defibrillators and therapy with class 1A drugs. (5) The "Brugada syndrome" and the "syndrome of nocturnal sudden death" strike males almost exclusively. Right bundle branch block (RBBB) with ST elevation in the right precordial leads-the "Brugada sign"--is seen in the ECG of both patient populations. Implantation of defibrillators is recommended.