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FromMedscapeEducationCardiologyDeliveringCareforPatientswithPulmonaryArterialHypertension:WhatAretheChallenges?CMENickH.Kim,MD;DavidLangleben,MD

Pulmonaryarterialhypertension(PAH)isarare,chronic,andprogressivediseaseofthepulmonaryvasculaturewherebyanincreaseinpulmonaryvascularresistance(PVR)resultsinrightventricular(RV)overloadandhypertrophy,eventuallyleadingtoRVfailureanddeath.RegistryevidenceindicatesthattheannualincidenceofPAHrangesfrom.0to.4casespermillionindividuals,whiletheprevalenceis0to5casespermillionindividuals.SeveraldiseasesandconditionsincreasetheriskforthedevelopmentofPH,includingconnectivetissuediseasesuchassystemicsclerosis(SSc),congenitalheartdisease,HIVinfection,andchroniclungdiseasesuchaschronicobstructivepulmonarydisease.

Despiterecentresearch-ledgainsintheunderstandingofthepathophysiologyoftheconditionandtheassociateddevelopmentofnovelPAH-specifictherapies,severalchallengesinthediagnosisandmanagementofpatientswithPAHpresentanobstacletoimprovinglong-termout







































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