PulmonaryHypertensionPulmonaryModuleStevenW.HarrisMHS,PA-C.PulmonaryHypertensionPulmonar1PulmonaryHypertensionDefinedaspressurewithinthepulmonaryarterialsystemelevatedabovethenormalrange.greaterthan25mmHgatrestor30mmHgwithexercisePulmonaryarterialpressure(PAP)usually12-15mmHgLeftatrialpressure6-10mmHg10/7/2023

2.PulmonaryHypertensionDefined2Pulmonary

hypertensiondevelopswhenfloworresistancetoflowacrossthepulmonaryvascularbedincreases

FourCategoriesofPulmonaryHypertensionPulmonaryhypertensionfromdisordersoftherespiratorysystemorhypoxemia.Pulmonaryvenoushypertension.Pulmonaryhypertensionfromchronicthromboembolicdisease.Pulmonaryarterialhypertension.(PAH)10/7/20233.Pulmonaryhypertensiondevelop3Chronicpulmonaryhypertension

isanimportantcauseofrightventricularfailureintheUnitedStates.Manyoftheindividualswhodieeachyearofchronicobstructivepulmonarydisease(COPD)succumbsecondarilytorightventricular

failureresultingfrom

pulmonaryhypertension.

10/7/20234.Chronicpulmonaryhypertension4Inaddition,greaterthan200,000deathsoccuryearlyfromacutepulmonaryembolism,themostcommoncauseofsudden-onsetpulmonary

hypertensionandacuterightventricularfailure.10/7/20235.8/5/20235.510/7/20236.8/5/20236.610/7/20237.8/5/20237.710/7/20238.8/5/20238.8Inpersonsmorethan50yearsofage,Corpulmonaletheconsequenceofuntreatedpulmonaryhypertension,isthethirdmostcommoncardiacdisorder(aftercoronaryandhypertensiveheartdisease).10/7/20239.Inpersonsmorethan50years9EntitiesthatcauseincreasedpulmonarybloodflowCongenitalHeartDiseaseLefttorightshunt“Hyperkinetic"pulmonary

hypertensioncanbeseeninpatientswithcongenitalheartdiseasewhohaveextensiveleft-to-rightcardiacshuntsthatproducealargepulmonarybloodflowIncreasedcardiacoutputstatessevereanemias10/7/202310.Entitiesthatcauseincreased10EntitiesthatcauseanincreasedresistancetoflowPulmonaryembolismInsitupulmonaryembolismPulmonaryfibrosisSarcoidosis,scleroderma,orextensivepulmonaryresectionSevereCOPDThoracicdeformitiesLargetumororinfiltrate10/7/202311.Entitiesthatcauseanincreas11EntitiesthatcausechangesinarteriolesHypoxia(altitude)COPDHypoventilation(sleepapnea)AcidosisDrugsPulmonaryarterialhypertension(PAH)10/7/202312.Entitiesthatcausechangesin12Entitiesthatcausevenouspressurechanges&vascularresistanceLeftatrialhypertension=MitralStenosisLeftventricularfailurePulmonaryvenousthrombosisMediastinitis10/7/202313.Entitiesthatcausevenouspre13PulmonaryHTNElevatedpulmonaryvenouspressureinthesettingofleftventricularfailureormitralstenosisisassociatedwithanimmediateincreaseinpulmonaryarterialpressurewhichmaintainsforwardbloodflowthroughthelungsdespitetheincreaseinpulmonaryvenouspressure.10/7/202314.PulmonaryHTNElevatedpulmonar1410/7/202315.8/5/202315.15EntitiesthatincreasebloodviscosityPolycythemiaveraLeukemiaswithhighWBC’s10/7/202316.Entitiesthatincreasebloodv16Entitiesthatincreaseintra-thoracicpressureCOPDMechanicalVentilation:especiallywithPEEP10/7/202317.Entitiesthatincreaseintra-t17SymptomsMildtomoderatepulmonaryhypertensionareoftenasymptomaticMoreseverepulmonaryhypertensionusuallycomplainofdyspneaonexertionsecondarytoexercise-induceddecreasesincardiacoutputandincreasesinpulmonaryarterialpressure.10/7/202318.SymptomsMildtomoderatepulmo18SymptomsEasyfatigability,lethargyExertionalchestdiscomfortSyncopewithexertionCoughHemoptysisHoarsenesssecondarytocompressionoftheleftrecurrentlaryngealnervebyadilatedpulmonaryartery(rare)10/7/202319.SymptomsEasyfatigability,let19PhysicalExamIncreasedintensityofthepulmoniccomponentofthesecondheartsound(P2)Systolicejectionmurmurfromtricuspidregurg…AdvancedDiseaseDiastolicmurmurofpulmonicregurgitationinpatientswithseverepulmonaryhypertensionEvidenceofrightventriculardilatation(leftparasternalliftorheave)Apulseprominent‘a(chǎn)’waveinjugularvenoussystem10/7/202320.PhysicalExamIncreasedintensi20PE(cont’d)Signsofrightventricularfailure: jugularvenousdistensionRightventricularS4(increasedloudnessoftheS4oninspiration)HepatomegalyAscites,and/orperipheraledema10/7/202321.PE(cont’d)Signsofrightvent21CaveatsPatientswithsevereemphysemaandincreasedthoracicanteroposteriordiametermaynotdisplaythefindingsusuallyassociatedwithadvancedpulmonaryhypertensionbecausechestexpansionmakepalpationandauscultationmoredifficult.TheJVDmayactuallybeabovethejaw-line!10/7/202322.CaveatsPatientswithsevereem22IncidenceofS&SLoudP2(>80%)Rightventricularlift(>80%)Dyspnea(>75%)Murmuroftricuspidinsufficiency(50-80%)Increasedjugularvenouspressure(50-80%)RightventricularS4(50-80%)Chestpain(>50%)Fatigue(>50%)10/7/202323.IncidenceofS&SLoudP2(>823S&Scont’dPalpitations(<50%)Syncope;dizziness(<50%)Cough(<50%)Raynaud'sphenomenon(<10%)Hepatomegaly(<50%)Pulmonicejectionclick(<50%)10/7/202324.S&Scont’dPalpitations(<5024S&Scont’dRightventricularS3(<50%)Murmurofpulmonicinsufficiency(<50%)Lowerextremityedema(<50%)Superficialthrombophlebitis(5%)10/7/202325.S&Scont’dRightventricular25PulmonaryArterialHypertension(PAH)Incurablediseasecharacterizedbymedialhypertrophy,intimalfibrosisandinsituthrombiVeryrare1-2casespermillionAge34-36&again50-59Females>males(1.7:1)10/7/202326.PulmonaryArterialHypertensio26CausesofPAHUnknown(IPAH)HIVDrugsAppetitesuppressants(fenfluramineanddexfenfluramine)AmphetaminesCocaine10/7/202327.CausesofPAHUnknown(IPAH)8/52710/7/202328.8/5/202328.28DiagnosticTestsChestx-ray,pulmonaryfunctiontestsALLshouldhaveaV/Qscanr/oPEEKG2-DechocardiographywithDopplerflowstudydiseaseSleepstudy6minutewalkCardiaccatheterization-rightheartcatheterizationisnecessarytomeasurepulmonaryarterypressuresandhemodynamics;ruleoutunderlyingcardiac10/7/202329.DiagnosticTestsChestx-ray,p29EKGFindingsRVH(latefinding)RADRBBBRVstrain(acuteiePulmembolus)SwaveinleadIQwave/invertedTinLeadIII10/7/202330.EKGFindingsRVH(latefinding)30..31..3210/7/202333.8/5/202333.3310/7/202334.8/5/202334.34DiagnosticTestsPulmonaryangiography-shouldbedoneifsegmentalorlargerdefectonV/Qscan.Cautioninpulmonaryhypertensionascanleadtohemodynamiccollapse;uselowosmolaragents,subselectiveangiograms.Lungbiopsynotrecommended10/7/202335.DiagnosticTestsPulmonaryangi3510/7/202336.8/5/202336.36WorldHealthOrganizationClassificationofFunctionalStatusofPatientswithPHClassI-patientswithPHwhoexperiencenolimitationofusualphysicalactivity;ordinaryphysicalactivitydoesnotcauseincreaseddyspnea,fatigue,chestpainorpresyncopeClassII-patientswithPHwhohavemildlimitationofphysicalactivity.Thereisnodiscomfortatrest,butnormalphysicalactivitycausesincreaseddyspnea,fatigue,chestpainorpresyncopeClassIII-patientswithPHwhohavemarkedlimitationofphysicalactivity.Thereisnodiscomfortatrest,butlessthanordinaryactivitycausesincreaseddyspnea,fatigue,chestpain,orpresyncopeClassIV-patientswithPHwhoareunabletoperformanyphysicalactivityatrestandwhomayhavesignsofrightventricularfailure.Dyspneaand/orfatiguemaybepresentatrest,andsymptomsareincreasedbyalmostanyphysicalactivity10/7/202337.WorldHealthOrganizationClas37TxofPulmonaryHTNTreatthecause!Phosphodiesteraseinhibitors(sildenafil)RevatioProsanoids(prostacyclinanalogues)Flolan,IloprostEndothelinreceptorantagonistsBosentanVasodilatorsL-arginine?Coumadin10/7/202338.TxofPulmonaryHTNTreatthec38TxofPulmonaryHTNPhosphodiesteraseinhibitors(sildenafil)RevatioNitricoxidestimulationofendotheliumincreasescGMPresultinginvasorelaxationPhosphodiesterasebreaksdowncGMP10/7/202339.TxofPulmonaryHTNPhosphodies39TxofPulmonaryHTNProsanoids(prostacyclinanalogues)Flolan,IloprostProstacyclinisapotentvasodilatorproducedinthevascularendothelium.Alsoinhibitsgrowthofsmoothmusclecells10/7/202340.TxofPulmonaryHTNProsanoids40TxofPulmonar