Inherited Wolff–Parkinson–White Syndrome

(整期优先)网络出版时间:2016-06-25
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Wolff-Parkinson-White(WPW)syndromeisacongenitaldisorderofcardiacconductionsystemcharacterizedbyelectrocardiographicpreexcitationandepisodesofparoxysmalsupraventriculartachycardia.Itiscausedbyacardiacdevelopmentaldefectintheelectricalinsulationbetweentheatriaandtheventriclesduetothepresenceofanaccessorypathway.WPWsyndromeisacommoncauseofsupraventriculartachycardiawithbenignprognosis.However,thisclinicalentityalsopredisposespatientstoanincreasedriskofsuddencardiacdeath,especiallyinthesettingofpreexcitedatrialfibrillation.WPWsyndromeisusuallysporadicandofunknownetiologyinmostcases.Duringthepast10years,asignificantheritablefactorisincreasinglyrecognized.IdentificationofthegeneticbasisamongpatientswithWPWsyndromehasimportantimplicationsforunderstandingthemolecularmechanismofventricularpreexcitationandthedevelopmentoftherapeuticstrategiesforriskstratificationandmanagement.Thegoalofthisreviewistoexaminethepreviousstudiesonhereditaryvariants,aswellastooutlinepotentialfutureavenuestowarddefiningtheheritabilityofWPWsyndrome.