Document Detail

Catecholaminergic polymorphic ventricular tachycardia: successful emergency treatment with intravenous propranolol.
MedLine Citation:
PMID:  15094576     Owner:  NLM     Status:  MEDLINE    
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.
Gabriella De Rosa; Angelica B Delogu; Marco Piastra; Antonio Chiaretti; Raffaella Bloise; Silvia G Priori
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric emergency care     Volume:  20     ISSN:  1535-1815     ISO Abbreviation:  Pediatr Emerg Care     Publication Date:  2004 Mar 
Date Detail:
Created Date:  2004-04-19     Completed Date:  2004-06-25     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  8507560     Medline TA:  Pediatr Emerg Care     Country:  United States    
Other Details:
Languages:  eng     Pagination:  175-7     Citation Subset:  IM    
Pediatric Cardiology, Catholic University Medical School, Rome, Italy.
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MeSH Terms
Adrenergic beta-Antagonists / administration & dosage,  therapeutic use*
Brain Edema / etiology
Catecholamines / metabolism
Decerebrate State
Infusions, Intravenous
Intensive Care Units, Pediatric
Nadolol / therapeutic use
Propranolol / administration & dosage,  therapeutic use*
Ryanodine Receptor Calcium Release Channel / genetics
Syncope / etiology
Tachycardia, Ventricular / diagnosis,  drug therapy*,  genetics,  ultrasonography
Reg. No./Substance:
0/Adrenergic beta-Antagonists; 0/Catecholamines; 0/Ryanodine Receptor Calcium Release Channel; 42200-33-9/Nadolol; 525-66-6/Propranolol

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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