?url_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Adc&rft.title=Genetic+basis+and+pathogenesis+of+Familial+WPW+Syndrome+%0A&rft.creator=Sidhu%2C+Jasvinder&rft.creator=Roberts%2C+Robert&rft.subject=Indian+Pacing+and+Electrophysiology+Journal&rft.description=The+Wolff-Parkinson-White+(WPW)+syndrome+has+been+a+known+clinical+entity+for+over+fifty+years.+In+1967+Durrer+et+al+postulated+WPW+syndrome+was+due+to+an+accessory+pathway%2C+bypassing+the+AV+node%2C+from+the+atria+to+the+ventricles1.++This+was+later+confirmed+by+epicardial+mapping.++WPW+is+the+second+most+common+cause+of+paroxysmal+supraventricular+tachycardias+in+the+western+world+and+the+most+common+cause+in+China2.+WPW+syndrome+has+a+prevalence+of+1.5+to+3.1+per+1000+persons+in+western+countries3%2C4%2C5+.++Patients+with+the+WPW+syndrome+may+present+with+palpitations%2C+presyncope%2C+syncope%2C+or+sudden+cardiac+death+(SCD).++In+some+patients+the+first+and+only+manifestation+of+the+disease+is+SCD.++This+is+more+likely+to+occur+in+the+setting+of+atrial+fibrillation+with+a+rapid+ventricular+response.++%0A%0A++++++++++++Electrocardiographic+findings+of+WPW+syndrome+consist+of+preexcitation+manifested+by+a+shortened+PR+interval+(%3C120+msec)%2C+a+widened+QRS+(%3E100+msec)+a+delta+wave+(abnormal+initial+QRS+vector)+and+supraventricular+tachycardia.++The+EKG+finding+of+preexcitation+is+a+result+of+early+ventricular+depolarization+through+the+accessory+pathway.++SVT+can+occur+with+retrograde+or+antigrade+conduction+through+the+accessory+pathway+forming+the+basis+of+the+so-called+%E2%80%9Cmacro-reentrant%E2%80%9D+arrhythmia+model+6.++Rapid+conduction+through+the+accessory+pathway+in+the+setting+of+atrial+fibrillation+markedly+increases+the+risk+of+SCD7.+%0A%0A++++++++++++We+recently+identified+the+gene+responsible+for+familial+Wolff-Parkinson-White+8.++The+gene+(PRKAG2)+which+encodes+for+a+protein+AMPK+(AMP-+activated+protein+kinase)+was+identified+as+the+causal+gene.++Missense+(single+nucleotide+change)+mutations+in+this+gene+were+identified+in+families+with+WPW.+++Six+such+mutations+have+been+identified.++Along+with+preexcitation+the+affected+families+had+conduction+abnormalities+(AV+block)+and+cardiac+hypertrophy.++%0A&rft.publisher=Indian+Pacing+and+Electrophysiology+Group&rft.contributor=Singh%2C+Balbir&rft.contributor=Lokhandwala%2C+Yash&rft.contributor=Francis%2C+Johnson&rft.contributor=Gupta%2C+Anup&rft.date=2003-10&rft.type=Journal+(Paginated)&rft.type=PeerReviewed&rft.format=text%2Fhtml&rft.identifier=http%3A%2F%2Fcogprints.org%2F4243%2F1%2Froberts.htm&rft.identifier=++Sidhu%2C+Jasvinder+and+Roberts%2C+Robert++(2003)+Genetic+basis+and+pathogenesis+of+Familial+WPW+Syndrome.++%5BJournal+(Paginated)%5D+++++&rft.relation=http%3A%2F%2Fcogprints.org%2F4243%2F