The role of thoracic epidural blockade in predicting responsiveness to left sympathetic denervation in patients with catecholaminergic polymorphic ventricular tachycardia.

摘要

POLYMORPHIC VENTRICULAR TACHYCARDIA (VT) is a rare event in children. Although the exact number of small children who have episodes of VT is unknown, VT is thought to be relatively uncommon. Authors have observed, in an unselected pediatric population, that the incidence of VT is 1.1 episodes/100,000 childhood years. PVT can be caused by congenital or acquired structural cardiac anomalies or by genetic mutations at the level of the cardiac calcium channels or the calcium-binding protein. Once cardiac structural anomalies such as hamartomas and long QT syndrome have been ruled out, the most common diagnosis is catecholaminergic polymorphic ventricular tachycardia (CPVT). Inherited mutations in cardiac ryan-odine receptor (RyR2) and calsequestrin genes (CASQ2) have been noted to account for autosomal dominant and recessive forms of CPVT, respectively.