{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,3,30]],"date-time":"2022-03-30T19:37:51Z","timestamp":1648669071458},"reference-count":20,"publisher":"Akademiai Kiado Zrt.","issue":"52","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2010,12,1]]},"abstract":"<jats:p>A szerz\u0151k \u00e1ttekintik az arrhythmogen jobb kamrai cardiomyopathia\/dysplasia t\u00e9mak\u00f6r\u00e9vel kapcsolatos legfontosabb ismereteket. Igazolt arrhythmogen jobb kamrai cardiomyopathia\/dysplasia eset\u00e9n \u2013 t\u00fcnetmentes egy\u00e9nekben \u2013 a terhel\u00e9ses vizsg\u00e1lat sor\u00e1n \u00e9szlelt kamrai tachycardia prognosztikus jelent\u0151s\u00e9g\u00e9r\u0151l nem rendelkez\u00fcnk adatokkal. A szerz\u0151k k\u00f6zlem\u00e9ny\u00fckben egy 25 \u00e9ves n\u0151beteg eset\u00e9t ismertetik, akin\u00e9l bokas\u00e9r\u00fcl\u00e9s ell\u00e1t\u00e1sa kapcs\u00e1n \u00e9szleltek k\u00f3ros EKG-t \u00e9s emiatt k\u00fcldt\u00e9k kardiol\u00f3giai vizsg\u00e1latra. A fizik\u00e1lis vizsg\u00e1lat k\u00f3ros elt\u00e9r\u00e9st nem mutatott, az EKG-felv\u00e9telen norm\u00e1lis sinusrhythmus \u00e9s bal tengely\u00e1ll\u00e1s mellett a II, III, aVF- \u00e9s V2-V6 elvezet\u00e9sekben negat\u00edv T-hull\u00e1m l\u00e1tszott. A mellkasr\u00f6ntgen \u00e9s a laborat\u00f3riumi vizsg\u00e1latok k\u00f3ros elt\u00e9r\u00e9st nem igazoltak. Az echokardiogr\u00e1fia norm\u00e1lis bal kamrai ejekci\u00f3s frakci\u00f3 mellett inferior akinesist \u00e9s t\u00e1gabb jobb kamr\u00e1t igazolt. A terhel\u00e9ses vizsg\u00e1lat sor\u00e1n a beteg 9 MET-et teljes\u00edtett, a terhel\u00e9s sor\u00e1n el\u0151bb sporadikus kamrai extrasystolia, majd 3 percig tart\u00f3 kamrai tachycardia jelentkezett, amely a terhel\u00e9s megszak\u00edt\u00e1sa \u00e9s haspr\u00e9s alkalmaz\u00e1s\u00e1ra megsz\u0171nt. A sz\u00edv-MR-vizsg\u00e1lat norm\u00e1lis balkamra-m\u00e9reteket, falmozg\u00e1st \u00e9s ejekci\u00f3s frakci\u00f3t igazolt. Az emelkedett jobb kamrai v\u00e9gszisztol\u00e9s \u00e9s v\u00e9gdiasztol\u00e9s volumen mellett a jobb kamrai ejekci\u00f3s frakci\u00f3 jelent\u0151sen cs\u00f6kkent (31,6%), \u00e9s a jobb kamr\u00e1nak megfelel\u0151en akinetikus ter\u00fcletek \u00e1br\u00e1zol\u00f3dtak. K\u00e9s\u0151i t\u00edpus\u00fa kontraszthalmoz\u00f3d\u00e1s a bal kamr\u00e1ban nem igazol\u00f3dott, a v\u00e9kony fal\u00fa jobb kamra fala e tekintetben pontosan nem volt meg\u00edt\u00e9lhet\u0151. Az EKG- \u00e9s a CMR-elt\u00e9r\u00e9sek alapj\u00e1n arrhythmogen jobb kamrai cardiomyopathia\/dysplasia diagn\u00f3zis\u00e1t \u00e1ll\u00edtott\u00e1k fel. Tekintettel arra, hogy a beteg panaszmentes volt \u00e9s a csal\u00e1dban hirtelen hal\u00e1l nem fordult el\u0151, prevent\u00edv c\u00e9lzattal gy\u00f3gyszeres kezel\u00e9s \u00e9s\/vagy ICD-implant\u00e1ci\u00f3 nem t\u00f6rt\u00e9nt. H\u00e1rom h\u00f3nappal k\u00e9s\u0151bb a beteg hirtelen meghalt. A sectio jelent\u0151sen t\u00e1g jobb kamr\u00e1t igazolt, a jobb kamra fala elv\u00e9konyodott, amelyben jellegzetes lokaliz\u00e1ci\u00f3ban (be\u00e1raml\u00e1si \u00e9s ki\u00e1raml\u00e1si traktus, valamint a cs\u00facs) az izomsz\u00f6vet helyett felszaporodott zs\u00edrsz\u00f6vet volt l\u00e1that\u00f3. Az \u00e9rintett jobb kamra fal\u00e1ban a Mallory-f\u00e9le foszfor-volfr\u00e1m savas hematoxilinfest\u00e9ssel fibrosist is igazolni lehetett. A szerz\u0151k azt a k\u00f6vetkeztet\u00e9st vonj\u00e1k le, hogy t\u00fcnetmentes betegben, igazolt arrhythmogen jobb kamrai cardiomyopathia\/dysplasia eset\u00e9n a terhel\u00e9ses EKG-vizsg\u00e1lat sor\u00e1n provok\u00e1lt kamrai tachycardia rossz prognosztikai jel, \u00e9s ilyen esetben ICD-implant\u00e1ci\u00f3 indokolt lehet. Orv. 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