EnglishRoundsF/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysdyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:ModeraterightventriculardilatationFlattened,paradoxicalseptalmotionEstimatedpulmonaryarterysystolicpressure:60mmHgNormal:15-30mmHgPulmonaryHypertension?PulmonaryHypertension(PH)

DefinitionmPAP(mean

pulmonaryarterialpressure)

≥

25mmHgatrestechocardiography

or

rightheartcatheterization

ClassificationGroup

1-5Group1:PAH(pulmonaryarterialhypertension)

IdiopathicandheritablePAHDrugsandtoxinsConnectivetissuediseasesHIVPortalhypertensionCongenital

heart

diseaseSchistosomiasisPulmonaryveno-occlusivediseasepersistentpulmonaryhypertensionofthenewborn(PPHN)ClassificationGroup2:

PH(left

heart

disease)Systolic

dysfunctionDiastolic

dysfunctionValvular

heart

diseaseInflow/outflow

tract

obstructionCongenital

cardiomyopathyClassificationGroup3:

PH(lung

disease)COPD(Chronic

obstructive

pulmonary

disease)ILD(Interstitial

lung

disease)CPFE(Combined

pulmonary

fibrosis

and

emphysema)Sleep

related

breathing

disorderClassificationGroup4:

PH

(thromboembolic

disease)Group5:

PH(multifactorial)Hemolytic

anemiaMyeloproliferative

disordersSystemic

disorders(eg.sarcoidosis)epidemiologyPrevalence

of

Group

1:

0.5-1.5

cases/100,000Traditional

point:

women

of

childbearing

age.

Now:

all

age

groups,

all

racial

populations,

both

gendersPathogenesisGeorgOhmChangeinpressure

=

flow

x

resistancePpaPpvpathogenesisChangeinpressure

=

flow

x

resistancePpa-Ppv

=

Q

x

PVRPpa

=

(Q

x

PVR)

+

PpvPpa

=

(QxPVR)+PCWPPVR:pulmonary

vascular

resistancePCWP:

pulmonarycapillarywedgepressureLAP:

left

atrial

pressureLVEDP:

leftventricularend-diastolicpressurePCWPPpvLAPLVEDPpathogenesis??pulmonaryvascularresistanceVascular

problemsIdiopathic

PAHConnective

tissue

diseaseHIV

infectionDecreasetheareaofthepulmonaryvascularbedPulmonary

emboliInterstitial

lung

diseaseconditionsthatinducehypoxicvasoconstrictionHypoventilation

syndromesPathogenesisPathogenesis??flow:Congenital

heart

defects

with

left-right

shuntASDVSDPDA??pulmonary

venous

pressure(mainlyLHD)Mitral

valve

diseaseLeft

ventricular

systolic

or

diastolic

dysfunctionConstrictive

pericarditisRestrictive

cardiomyopathyPulmonary

venous

obstructionPathologySymptoms?output?exertionaldyspnealethargyfatigueRight

heart

failureExertionalchest

pain

(subendocardial

hypoperfusion)Peripheral

edema

(right

ventricular

failure)Anorexia

and

abdominal

pain

(liver

congestion)Alteredbioenergetics(ischaemia,mitochondrialremodelling)Neurohormonaland

immunologicalactivationPathophysiologyofRVdysfunctioninPAHVonk-NoordegraafA,etal.JAmCollCardiol2013;62:D22-33.PAHRVpressureoverload↑RVwallstressMyocardialremodellingHypertrophy;matrixremodellingIncreaseinRVcontractilityGeneticDeterminantsAdaptiveremodelling(minimallyalteredEes/Ea)Maladaptive

remodellingDilatationandfailureArrhythmiasIschaemiaEa:arterialelastance;Ees:ventricularelastance;RV:rightventricularRVanatomy:NormalversusPAHNormalPAHChinKM,etal.CoronArteryDis2005;16:13-8.LV120mmHgRVLVPRVThicknessStressCoronaryperfusionpressure+O2demand=Supply/DemandRVdistension&LVfilling=Cardiacoutput20mmHgLV:leftventricle/ventricular;RV:rightventricle/ventricularPhysical

examinationSplit

S2MurmurJugularveindistension:

prominent

A

waveEnlarged

liver

and

spleenDiagnosticevaluationChest

radiographElectrocardiographyEchocardiographyPulmonaryfunctiontestsV/QscanExercisetestingRightheartcatheterizationChest

radiography

Swan-GanzcatheterDiagnosticcriteria

(requiresrightheartcatheterization)PulmonaryhypertensionmPAP≥25mmHgPulmonaryarterialhypertensionMeanpulmonaryarterypressureis≥25mmHgatrestMeanpulmonarycapillarywedgepressure<15mmHg

ChroniclungdiseasesandothercausesofhypoxemiaaremildorabsentVenousthromboembolicdiseaseisabsentCertainmiscellaneousdisordersareabsentsystemicdisorders(eg,sarcoidosis)hematologicdisorders(eg,myeloproliferativediseasesmetabolicdisorders(eg,glycogenstoragedisease)26PAH的治療流程：循證醫(yī)學(xué)證據(jù)GalièN,etal.JAmCollCardiol2013;62:D60-72.

指導(dǎo)下運(yùn)動(dòng)(I-A)

心理-社會(huì)支持(I-C)避免過度體力勞動(dòng)(I-C)

避孕(I-C)流感和肺炎球菌疫苗接種(I-C)一般性治療和支持性治療口服抗凝:特發(fā)性PAH,遺傳性PAH及食欲抑制劑相關(guān)PAH(IIa-C)繼發(fā)性PAH(IIb-C)利尿劑(I-C)氧療(I-C)地高辛(IIb-C)專病轉(zhuǎn)診(I-C)急性血管擴(kuò)張?jiān)囼?yàn)

(特發(fā)性PAH：I-C)(繼發(fā)性PAH：IIb-C)開始PAH靶向藥物治療陰性FCI-IIICCB

(I-C)持續(xù)有效(FCI-II)繼續(xù)CCBNo陽(yáng)性YesCase1F/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgDidthepatienthaspulmonaryhypertension?Whatsymptomsandsignsmayindicatesuchcondition?Chestpain?Dyspnea?JugularveindistentionProminentP2Diagnostic:RHC,butnotstronglyindicatedbeforefurtherexaminationF/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgWhatarethepossibleunderlyingdiseasesofPHinthiscase?ComplicationsofNS?InfectionThrombosisARFAtherosclerosisElectrolytedisturbanceF/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgWhatarethepossibleunderlyingdiseaseofPHinthiscase?ComplicationsofNS?Thrombosis-pulmonaryembolism?Infection-pneumonia?Atherosclerosis-ACS?AthirdconditioncausingNSandPH?Autoimmune-SLE?ANCA-associatedvasculitis?F/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgHowtoproveorruleoutthesehypothesis?ComplicationsofNS?Thrombosis-pulmonaryembolism?Infection-pneumonia?Atherosclerosis-ACS?AthirdconditioncausingNSandPH?Autoimmune-SLE?ANCA-associatedvasculitis?F/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgHowtoproveorruleoutthesedisorders?Detailedmedicalhistory:ChestpainCough&HemoptysisDyspneaPedaledemaSymptomsassociatedwithautoimmunediseaseF/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgHowtoproveorruleoutthesedisorders?PulmonaryembolismABG,D-DimerCTPA,V/Q,BUSoflowerextremityPneumoniaCBC,PCT,sputumcultureChestX-ray,CTACSECG,Mb,CK-MB,cTnIAutoimmuneANA,dsDNA,ANCASupplementofhistoryChestPain:bilateral,exacerbatedbycoughinganddeepinspirationCough&Hemoptysis:whitishsputum,smallamountofbloodDyspneaUponexercisePedaledemapitting,sincetheonsetofNSSymptomsassociatedwithautoimmunediseasenegativeECG“SIQIIITIII”CBC16.212.310.3497ABGhypoxia,respiratoryalkalosisBloodbiochemistrySignificantdecreaseofalbuminMildelevationofNTpro-BNPCoagulationSignificantelevationofD-DimerAutoimmune:negativeCTPAIntraluminalthrombimainpulmonaryarteryrightpulmonaryarteryleftpulmonaryarterysegmentalpulmonaryarteriesbilaterallySmallbilateralpleuraleffusionsCTPA–reconstructionBilateral,peripheral,wedge-shapedopacitiesF/17yoPMHx:nephroticsyndrome4monthHPI:Chestpainfor2weeks.Cough,hemoptysisfor6daysDyspneafor1dayPE:T38.3℃,HR148/min,RR34/min,BP138/72mmHg,SpO292%@RAJugularveindistention,1+edemaofbothlegsProminentP2,systolicmurmur(grade2/6)overtheleftlowersternalborderUCG:pulmonaryarterysystolicpressureof60mmHgDiagnosis?PulmonaryembolisminthesettingofthenephroticsyndromeWhichgroupofPHdoesitbelongto?GroupIIIthromboembolicdiseaseCase2F/46yoTransferredtothishospitalbecauseofseverePHHPI:2monthsearlier:Proximal-muscleweakness,myalgia

Raynaud’sphenomenonMaculopapularrashoverneckandanteriorchestDyspnea1weeksearlier:CoughandnonpleuriticchestpainontherightPE:FinecracklesatbothlungbasesLoudP2,splitS2,rightS2Liver1cmbelowcostalmarginPhysicalExamination:Confluentareasoferythemaontheneck,chest,andupperarmsVsignPhysicalExamination:Reddish-purpleeruptionontheuppereyelidandswellingoftheeyelidHeliotropesignF/46yoTransferredtothishospitalbecauseofseverePHHPI:2monthsearlier:Proximal-muscleweakness,myalgiaRaynaud’sphenomenonMaculopapularrashoverneckandanteriorchestDyspnea1weeksearlier:CoughandnonpleuriticchestpainontherightPE:FinecracklesatbothlungbasesLoudP2,splitS2,rightS2Liver1cmbelowcostalmarginPhysicalExamination:Gottron’ssignMultipleerythematousontheextensorsurfaceofthejointsF/46yoTransferredtothishospitalbecauseofseverePHHPI:2monthsearlier:Proximal-muscleweakness,myalgia

Raynaud’sphenomenonMaculopapularrashoverneckandanteriorchestDyspnea1weeksearlier:CoughandnonpleuriticchestpainontherightPE:FinecracklesatbothlungbasesLoudP2,splitS2,rightS2Liver1cmbelowcostalmarginWhatsymptomsindicatetheseverePH?Non-pleuriticchestpainontherightsideLoudP2,splitS2,rightventricularS2Liver1cmbelowcostalmarginProgressivedyspnea?F/46yoTransferredtothishospitalbecauseofseverePHHPI:2monthsearlier:Proximal-muscleweakness,myalgia

Raynaud’sphenomenonMaculopapularrashoverneckandanteriorchestDyspnea1weeksearlier:CoughandnonpleuriticchestpainontherightPE:FinecracklesatbothlungbasesLoudP2,splitS2,rightS2Liver1cmbelowcostalmarginVsign,heliotropesign,Gottron’ssignWhatisthemostlikelydiseasethatcausePHinthispatient?Dermatomyositis(DM)IdiopathicinflammatorymyopathySymmetricproximalmuscleweakness,20%amyopathicCharacteristicskinmanifestations5-30%interstitiallungdiseaseF/46yoTransferredtothishospitalbecauseofseverePHHPI:2monthsearlier:Proximal-muscleweakness,myalgia

Raynaud’sphenomenonMaculopapularrashoverneckandanteriorchestDyspnea1weeksearlier:CoughandnonpleuriticchestpainontherightPE:FinecracklesatbothlungbasesLoudP2,splitS2,rightS2Liver1cmbelowcostalmarginVsign,heliotropesign,Gottron’ssignWhatisthemostlikelydiseasethatcausethePHinthispatient?InterstitiallungdiseaseinDMMaybemorecommoninpatientswithnormalCKAssociatedwithanti-Jo-1Antisynthetasesyndrome:myositis,ILD,polyarthritis,mechanic’shand,feverandRaynaudphenomenonF/46yoTransferredtothishospitalbecauseofseverePHHPI:2monthsearlier:Proximal-muscleweakness,myalgia

Raynaud’sphenomenonMaculopapularrashoverneckandanteriorchestDyspnea1weeksearlier:CoughandnonpleuriticchestpainontherightPE:FinecracklesatbothlungbasesLoudP2,splitS2,rightS2Liver1cmbelowcostalmarginVsign,heliotropesign,Gottron’ssignHowtofurtherprovethediagnosis?Dermatomyositis:SerumcreatinekinaseElectromyographyMusclebiopsyESR,CRP,ANA,RFMRIInterstitiallungdisease: