PulmonaryHypertension:HowtheRadiologistcanhelp杜倩妮2019.08目录CONTENTS01.02.DefinitionClassification03.PathophysiologyandClinicalCourse04.DiagnosisandAssessmentofPulmonaryHypertensionDefinitionPART01肺高压(Pulmonaryhypertension,PH):是一组由异源性疾病和不同发病机制引起的以肺血管阻力持续增高为特征的临床病理生理综合征。右心导管术是金标准（有创），海平面状态下、静息时，肺动脉平均压≥25mmHg。ClassificationPART02第4届世界PH研讨会对PH的分类①动脉性肺动脉高压特发性/遗传性/药物和毒物诱导/与疾病相关性（结缔组织疾病、HIV感染、门脉高压、先天性心脏病、血吸虫病和慢性溶血性贫血）、新生儿持续性肺动脉高压、肺静脉闭塞病、肺毛细血管瘤样增生症②与左心疾病相关的肺高压③与肺疾病和/或低氧相关的肺高压④慢性血栓栓塞性肺高压(CTEPH)⑤多种机制和/或不明机制引起的肺高压血液系统疾病（骨髓增生性疾病，脾脏切除术）、系统性疾病（结节病，肺朗格汉斯细胞组织细胞增生症，淋巴管肌瘤病，多发性神经纤维瘤，血管炎）、代谢性疾病（糖原贮积病，高雪氏病，甲状腺疾病）、其他（肿瘤阻塞，纤维性纵隔炎，透析治疗的慢性肾衰竭）Pulmonaryarterialhypertensionisrestrictedtothosewithahemodynamicprofileinwhichhighpulmonarypressureisaresultofelevatedprecapillarypulmonaryresistanceandnormalpulmonaryvenouspressureandismeasuredasapulmonarywedgepressureof15mmHgorless,ahemodynamicprofilethatissharedbygroups3,4,and5intheDanaPointclassificationsystem.Pulmonaryhypertensionresultingfromheartdisease(group2)impliesanincreaseinpulmonaryarterialpressureduetobackwardtransmissionofpressureelevation(postcapillarypulmonaryhypertension)andisdefinedasameanpulmonaryarterialpressureof20mmHgormoreandapulmonarywedgepressuregreaterthan15mmHg.PathophysiologyandClinicalCoursePART03TheclinicalcourseofPHisdividedintothreephases:AsymptomaticcompensatedSymptomaticdecompensatingAdvanceddecompensatedDiagnosisandAssessmentofPulmonaryHypertensionPART04InpatientswithsuspectedPH,thediagnosticapproachincludesfourstages:SuspicionDetectionClassificationFunctionalevaluationDiagramshowstheimagingwork-upofpatientswithPHDiagramshowstheimagingalgorithmusedforpatientswithsuspectedPHChestradiographyisusuallytheinitialimagingstudyperformed.Theclassicradiographicfindingsofpulmonaryhypertensionareevidentonlylateinthediseaseprocess.Suchlatefindingsinclude:1.centralpulmonaryarterialdilatation;2.pruningoftheperipheralarteries;3.increaseddiameter(15mminwomenand16mminmen)oftherightinterlobarartery;4.reducedretrosternalairspaceonlateralviews,aresultofrightventriculardilatation.ChestRadiographyMultidetectorCTPA（vascular,cardiac,andparenchymal）•VascularSigns1.Themainpulmonaryarterywithadiameterof29mmormore(positivepredictivevalueof97%,sensitivityof87%,andspecificityof89%)2.Asegmentalartery–to-bronchusdiameterratioof1:1ormoreinthreeorfourlobes(specificityof100%)3.Themainpulmonaryarterialdiameterlargerthanthatoftheascendingaorta(positivepredictivevalueof96%andspecificityof92%,especiallyinpatientsyoungerthan50yearsold)•CardiacSignsMultidetectorCTPA（vascular,cardiac,andparenchymal）1.Rightventricularhypertrophy(wallthicknessofmorethan4mm)2.Straighteningorleftwardbowingoftheinterventricularseptum3.Rightventriculardilatation(rightventricle–to–leftventriclediameterratioofmorethan1:1atthemidventricularlevelonaxialimages)4.Decreasedrightventricularejectionfraction5.Dilatationoftheinferiorvenacavaandhepaticveins6.PericardialeffusionMultidetectorCTPA（vascular,cardiac,andparenchymal）•Pare...
