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
