title: Catecholaminergic polymorphic ventricular tachycardia: successful emergency treatment with intravenous propranolol.
abstract: Catecholaminergic polymorphic ventricular tachycardia (VT) is a rare arrhythmogenic disorder, which may cause sudden death and whose relationships with mutations in cardiac ryanodine receptor gene have been recently established. The present article reports a catecholaminergic polymorphic VT case of a 9-year-old girl, without any previous history of syncope, who has been found unconscious while playing and referred comatose to pediatric intensive care unit. The electrocardiogram pattern showed runs of bidirectional and polymorphic VT degenerating into ventricular fibrillation, without QT interval abnormalities. Various attempts of cardioversion, lidocaine, and magnesium sulfate intravenous infusions were only partially effective. Owing to catecholaminergic polymorphic VT highly suggesting electrocardiogram pattern, intravenous propranolol was administered, achieving immediate VT interruption. Long-term nadolol therapy effectively prevented further arrhythmias, with no relapses up to 10 months later; a good neurologic recovery was also obtained. Genetic evaluation revealed in this patient-but not in relatives-a mutation in ryanodine receptor gene on chromosome 1.
authors.affiliation: *Pediatric Cardiology, Catholic University Medical School, Rome, Italy; †Pediatric Intensive Care Unit, Catholic University Medical School, Rome, Italy; ‡Molecular Cardiology, IRCCS Fondazione "Salvatore Maugeri," University of Pavia, Pavia, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: De Rosa
authors.forename: Angelica B
authors.initials: AB
authors.lastname: Delogu
authors.forename: Marco
authors.initials: M
authors.lastname: Piastra
authors.forename: Antonio
authors.initials: A
authors.lastname: Chiaretti
authors.forename: Raffaella
authors.initials: R
authors.lastname: Bloise
authors.forename: Silvia G
authors.initials: SG
authors.lastname: Priori
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000008:administration & dosage / Q000627:therapeutic use*; D001929:Brain Edema / Q000209:etiology; D002395:Catecholamines / Q000378:metabolism; D002648:Child; D003655:Decerebrate State; D004562:Electrocardiography; D004630:Emergencies; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D015278:Intensive Care Units, Pediatric; D009248:Nadolol / Q000627:therapeutic use; D011433:Propranolol / Q000008:administration & dosage / Q000627:therapeutic use*; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D013575:Syncope / Q000209:etiology; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D014463:Ultrasonography
chemical_list: D000319:Adrenergic beta-Antagonists; D002395:Catecholamines; D019837:Ryanodine Receptor Calcium Release Channel; D009248:Nadolol; D011433:Propranolol