KEGG   DISEASE: H01154Help
Entry
H01154                      Disease                                

Name
Wolff-Parkinson-White (WPW) syndrome;
Preexcitation syndrome
Description
Wolff-Parkinson-White (WPW) syndrome is the most common cause of ventricular pre-excitation, a condition where all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that mutations in PRKAG2, that encodes a subunit of the AMP-activated protein kinase, is responsible for WPW syndrome.
Category
Cardiovascular disease
BRITE hierarchy
Pathway
Insulin signaling pathway
Adipocytokine signaling pathway
Hypertrophic cardiomyopathy (HCM)
Gene
PRKAG2 [HSA:51422] [KO:K07200]
Marker
(Electrocardiogram) Shortened PQ interval, widened QRS complex, and slowed delta wave.
Drug
Vagal maneuvers
Injection of an anti-arrhythmic medication [DR:D00045] [DR:D00638] [DR:D00640]
Radiofrequency catheter ablation
Comment
The use of Verapamil [DR:D00619] or Digoxin [DR:D00298] for long-term therapy appears to be contraindicated for many patients with WPW syndrome.
Other DBs
ICD-10: 
OMIM: 
Reference
  Authors
Sternick EB, Oliva A, Gerken LM, Magalhaes L, Scarpelli R, Correia FS, Rego S, Santana O, Brugada R, Wellens HJ
  Title
Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: the role of PRKAG2 mutation.
  Journal
Heart Rhythm 8:58-64 (2011)
Reference
  Authors
Light PE
  Title
Familial Wolff-Parkinson-White Syndrome: a disease of glycogen storage or ion channel dysfunction?
  Journal
J Cardiovasc Electrophysiol 17 Suppl 1:S158-S161 (2006)
Reference
  Authors
Redfearn DP, Krahn AD, Skanes AC, Yee R, Klein GJ
  Title
Use of medications in Wolff-Parkinson-White syndrome.
  Journal
Expert Opin Pharmacother 6:955-63 (2005)
Reference
PMID:17547624 (Drug)
  Authors
Fragakis N, Iliadis I, Papanastasiou S, Lambrou A, Katsaris G
  Title
Brugada type electrocardiographic changes induced by concomitant use of lithium and propafenone in patient with Wolff-Parkinson-White syndrome.
  Journal
Pacing Clin Electrophysiol 30:823-5 (2007)

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