2025
Exercise Pulmonary Hypertension and Beyond: Insights in Exercise Pathophysiology in Pulmonary Arterial Hypertension (PAH) from Invasive Cardiopulmonary Exercise Testing
Tarras E, Singh I, Kreiger J, Joseph P. Exercise Pulmonary Hypertension and Beyond: Insights in Exercise Pathophysiology in Pulmonary Arterial Hypertension (PAH) from Invasive Cardiopulmonary Exercise Testing. Journal Of Clinical Medicine 2025, 14: 804. PMID: 39941482, PMCID: PMC11818252, DOI: 10.3390/jcm14030804.Peer-Reviewed Original ResearchInvasive cardiopulmonary exercise testingPulmonary arterial hypertensionCardiopulmonary exercise testingArterial hypertensionAssociated with pulmonary vascular remodelingExercise testRight heart failureExercise pulmonary hypertensionPulmonary vascular remodelingPulmonary hypertensionProgressive diseasePulmonary vasculatureTherapeutic optionsExercise pathophysiologyHigh morbidityHeart failureEarly diagnosisVascular remodelingTherapeutic approachesPersonalized treatmentHypertensionDisease subtypesDiagnosisSkeletal muscleDisease
2024
Global, regional, and national burden of pulmonary arterial hypertension, 1990–2021: a systematic analysis for the Global Burden of Disease Study 2021
Collaborators G, Leary P, Lindstrom M, Johnson C, Emmons-Bell S, Rich S, Corris P, DuBrock H, Ventetuolo C, Abate Y, Abdelmasseh M, Aboagye R, Abualruz H, Abu-Gharbieh E, Aburuz S, Adamu L, Adão R, Addo I, Adedoyin R, Adetunji J, Adzigbli L, Ahinkorah B, Ahmad F, Ahmadzade A, Ahmed A, Ahmed H, Ahmed S, Akhlaghi S, Akkaif M, Al Awaidy S, Alalalmeh S, Albakri A, Aldawsari K, Almahmeed W, Alshahrani N, Altaf A, Aly H, Alzoubi K, Al-Zyoud W, Amani R, Amusa G, Andrei C, Anwar S, Anyasodor A, Aravkin A, Areda D, Asmerom H, Aujayeb A, Azzam A, Babu A, Bagherieh S, Baltatu O, Barqawi H, Bastan M, Batra K, Bayleyegn N, Behnoush A, Bhalla J, Bhaskar S, Bhat V, Bitaraf S, Bitra V, Boloor A, Braithwaite D, Brauer M, Bulto L, Bustanji Y, Chattu V, Chi G, Chichagi F, Chong B, Chowdhury R, Cindi Z, Cruz-Martins N, Dadana S, Dadras O, Dahiru T, Dai X, Dashtkoohi M, DeAngelo S, Debopadhaya S, Demessa B, Desai H, Dhulipala V, Diaz M, Diress M, Chi T, Phuong T, Doan K, dos Santos W, Doshi R, Dowou R, Dziedzic A, Elhadi M, Etaee F, Fabin N, Fagbamigbe A, Faris P, Feyisa B, Rodrigues C, Gandhi A, Ganiyani M, Gela Y, Getie M, Jolfayi A, Ghasemzadeh A, Goldust M, Golechha M, Guan S, Gudeta M, Gupta M, Gupta R, Hadei M, Hammoud A, Hasnain S, Tabatabaei M, Hay S, Hegazi O, Hemmati M, Hiraike Y, Hoan N, Hultström M, Huynh H, Ibitoye S, Ilesanmi O, Ismail N, Iwu C, Jaggi K, Jain A, Jakovljevic M, Jee S, Jeswani B, Jha A, Jokar M, Joseph N, Jozwiak J, Kabir H, Kahe F, Kamireddy A, Kanmanthareddy A, Karimi H, Behnagh A, Kazemian S, Keshavarz P, Khalaji A, Khan M, Khidri F, Kim M, Kondlahalli S, Kothari N, Krishan K, Kulimbet M, Kumar A, Latifinaibin K, Le T, Ledda C, Lee S, Li M, Lim S, Liu S, Mahmoudi E, Makram O, Malhotra K, Malik A, Malta D, Manla Y, Martorell M, Mehrabani-Zeinabad K, Merati M, Mestrovic T, Mirdamadi N, Misra A, Mokdad A, Moni M, Ghalibaf A, Moraga P, Morovatdar N, Motappa R, Mousavi-Aghdas S, Mustafa A, Naik G, Najafi M, Najdaghi S, Nanavaty D, Davani D, Natto Z, Nauman J, Nguyen D, Nguyen P, Niazi R, Oancea B, Olanipekun T, Oliveira G, Omar H, A M, Pan F, Pandi-Perumal S, Pantazopoulos I, Parikh R, Petcu I, Pham H, Pham H, Philip A, Prates E, Puvvula J, Qian G, Rafferty Q, Rahim F, Rahimi M, Rahman M, Rahman M, Rahmanian M, Rahmanian N, Rahmati M, Rahmati R, Ramadan M, Ramphul K, Rana J, Rao I, Rashedi S, Ravikumar N, Rawaf S, Ray A, Reddy M, Redwan E, Rezaei N, Roy P, Saad A, Saddik B, Sadeghi M, Saeb M, Sharif-Askari F, Sharif-Askari N, Saleh M, Sani N, Saraswati U, Saravanan A, Saulam J, Schuermans A, Schumacher A, Semagn B, Sethi Y, Seylani A, Shafeghat M, Shahwan M, Shamim M, Shamsi A, Sharfaei S, Sharma K, Sharma N, Sherif A, Shiue I, Shorofi S, Siddig E, Singh H, Singh J, Singh P, Singh S, Sobia F, Solanki R, Solanki S, Spartalis M, Swain C, Szarpak L, Tabatabaei S, Tabche C, Tamuzi J, Tan K, Teramoto M, Tharwat S, Thienemann F, Truyen T, Tsegay G, Udoakang A, Van den Eynde J, Varthya S, Verma M, Vervoort D, Vinayak M, Viskadourou M, Wang F, Wickramasinghe N, Wilandika A, Xu S, Yu C, Zare I, Zeineddine M, Zhang Z, Zhu L, Zhumagaliuly A, Zielińska M, Zyoud S, Murray C, Roth G. Global, regional, and national burden of pulmonary arterial hypertension, 1990–2021: a systematic analysis for the Global Burden of Disease Study 2021. The Lancet Respiratory Medicine 2024, 13: 69-79. PMID: 39433052, PMCID: PMC11698691, DOI: 10.1016/s2213-2600(24)00295-9.Peer-Reviewed Original ResearchPulmonary arterial hypertensionBurden of diseaseArterial hypertensionCases of pulmonary arterial hypertensionGlobal burdenProgressive right heart failureRight heart failureAge-standardised mortality ratesChronic myeloid leukemiaGlobal Burden of Disease StudyBurden of Disease StudyGlobal Burden of DiseaseAge-standardised prevalenceRisk Factors StudyCause of deathPulmonary arteryMyeloid leukemiaHeart failureGroup 2Crohn's diseaseMorbid diseaseCirculatory diseasesNational burdenOlder adultsVascular diseasePalliative Pharmacotherapy for Cardiovascular Disease: A Scientific Statement From the American Heart Association
Di Palo K, Feder S, Baggenstos Y, Cornelio C, Forman D, Goyal P, Kwak M, McIlvennan C, Nursing O. Palliative Pharmacotherapy for Cardiovascular Disease: A Scientific Statement From the American Heart Association. Circulation Cardiovascular Quality And Outcomes 2024, 17: e000131. PMID: 38946532, DOI: 10.1161/hcq.0000000000000131.Peer-Reviewed Original ResearchQuality of lifeCardiovascular diseaseExcess health care useGoal-oriented careHealth care useShared decision-makingEnhance quality of lifePalliative pharmacotherapyMedical managementScientific statementEvidence-based medical therapyPulmonary arterial hypertensionSpectrum of cardiovascular diseasesAmerican Heart AssociationCoronary heart diseaseEnd-stage heart failurePalliative medicineCare usePalliative approachArterial hypertensionGuideline-directedMedical therapyDevice therapyHeart failureWorsening symptomsCellular Mechanisms Underlying the Pathogenetic Role of the Primary Cilium in Pulmonary Arterial Hypertension
Melnikov A, Solymosi P, Pennitz P, Schupp J, Nouailles G, Perret P, Gallo K, Hegemann N, Nambiar Veetil N, Klepetko W, Sinn K, Erfinanda L, Simmons S, Kucherenko M, Knosalla C, Preissner R, Kwapiszewska G, Witzenrath M, Kuebler W. Cellular Mechanisms Underlying the Pathogenetic Role of the Primary Cilium in Pulmonary Arterial Hypertension. 2024, a6790-a6790. DOI: 10.1164/ajrccm-conference.2024.209.1_meetingabstracts.a6790.Peer-Reviewed Original ResearchPulmonary arterial hypertensionArterial hypertensionPathogenetic roleCellular mechanismsPrimary ciliaHypertensionEndothelial HIFα-PDGF-B to smooth muscle Beclin1 signaling sustains pathological muscularization in pulmonary hypertension
Saddouk F, Kuzemczak A, Saito J, Greif D. Endothelial HIFα-PDGF-B to smooth muscle Beclin1 signaling sustains pathological muscularization in pulmonary hypertension. JCI Insight 2024, 9: e162449. PMID: 38652543, PMCID: PMC11141934, DOI: 10.1172/jci.insight.162449.Peer-Reviewed Original ResearchSmooth muscle cellsArteriole smooth muscle cellsPulmonary hypertensionPlatelet-derived growth factor-BDistal muscularizationSugen 5416Endothelial cellsHuman idiopathic pulmonary arterial hypertensionHypoxia-induced pulmonary vascular remodelingPulmonary artery smooth muscle cellsIdiopathic pulmonary arterial hypertensionHypoxia-inducible factor (HIF)-1aArtery smooth muscle cellsDistal pulmonary arteriolesPulmonary arterial hypertensionPulmonary vascular remodelingDeletion of Hif1aLung endothelial cellsGrowth factor BEC-specific deletionPulmonary arteriolesArterial hypertensionLung lysatesMuscle cellsVascular remodeling
2023
Cognitive impairment in pulmonary arterial hypertension
Heller S, See C, Singh I, Fredericks C. Cognitive impairment in pulmonary arterial hypertension. Annals Of Clinical And Translational Neurology 2023, 10: 1899-1903. PMID: 37550957, PMCID: PMC10578884, DOI: 10.1002/acn3.51867.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionCognitive impairmentArterial hypertensionPulmonary artery pressureRight heart dysfunctionPulmonary vascular remodelingCognitive impairment syndromeVascular cognitive impairmentArtery pressureCardiopulmonary exerciseHeart dysfunctionNoninvasive markerCognitive dysfunctionVascular remodelingAdult subjectsImpairmentHypertensionUnderlying mechanismAbnormal increaseDysfunctionSyndromeCognitive evaluationSubjectsEffects of sotatercept on lung diffusion capacity and blood gases in patients with pulmonary arterial hypertension
Olsson K, Fuge J, Park D, Kamp J, Brod T, Harrigfeld B, Schupp J, Hoeper M. Effects of sotatercept on lung diffusion capacity and blood gases in patients with pulmonary arterial hypertension. European Respiratory Journal 2023, 62: 2301070. PMID: 37474157, DOI: 10.1183/13993003.01070-2023.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionLung diffusion capacityArterial hypertensionDiffusion capacityRight heart functionRight ventricular afterloadSmall pulmonary vesselsVentricular afterloadBlood gasesPulmonary vesselsHeart functionRare diseaseHypertensionPatientsSotaterceptProgressive increaseSymptomsVariable alterationsDisease symptomsGas exchangeAfterloadDiseaseSeverityIs low-risk status a surrogate outcome in pulmonary arterial hypertension? An analysis of three randomised trials
Blette B, Moutchia J, Al-Naamani N, Ventetuolo C, Cheng C, Appleby D, Urbanowicz R, Fritz J, Mazurek J, Li F, Kawut S, Harhay M. Is low-risk status a surrogate outcome in pulmonary arterial hypertension? An analysis of three randomised trials. The Lancet Respiratory Medicine 2023, 11: 873-882. PMID: 37230098, PMCID: PMC10592525, DOI: 10.1016/s2213-2600(23)00155-8.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionPulmonary arterial hypertension trialsWorsening pulmonary arterial hypertensionFood and Drug AdministrationLow-risk statusClinical worseningLong-term outcomesRisk scoreArterial hypertensionPAH associated with connective tissue diseaseIdiopathic pulmonary arterial hypertensionPulmonary arterial hypertension treatmentSurrogate outcomesObservational study of outcomesLong-term follow-upDiscontinuation of study treatmentWHO functional classUS Food and Drug AdministrationMeta-analysisMeta-analysis of RCTsAll-cause deathConnective tissue diseaseEffects of therapyPredictive of outcomeTreatment effectsA Case of Fulminant Right Heart Failure Owing to Tumoral Pulmonary Hypertension
Wang K, Verma A, Fish K, Hu J, Miller P, Morrow J, Singh I, Young L. A Case of Fulminant Right Heart Failure Owing to Tumoral Pulmonary Hypertension. JACC Case Reports 2023, 10: 101715. PMID: 36974052, PMCID: PMC10039392, DOI: 10.1016/j.jaccas.2022.101715.Peer-Reviewed Case Reports and Technical Notes
2022
Multiple Intravenous Bolus Dosing and Invasive Hemodynamic Assessment in a Hypoxia-Induced Mouse Pulmonary Artery Hypertension Model.
Qin L, Jiang B, Zsebo K, Duckers H, Simons M, Chen P. Multiple Intravenous Bolus Dosing and Invasive Hemodynamic Assessment in a Hypoxia-Induced Mouse Pulmonary Artery Hypertension Model. Journal Of Visualized Experiments 2022 PMID: 36440832, DOI: 10.3791/63839.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionInvasive hemodynamic assessmentHemodynamic assessmentPAH modelProgressive life-threatening diseaseGroup 3 diseaseRight ventricle catheterizationSmall pulmonary arteriolesIntravenous bolus dosingNew experimental therapiesLife-threatening diseaseAdministration of compoundsMouse jugular veinHuman clinical manifestationsArterial hypertensionHypertension modelPulmonary arteriolesClinical manifestationsBolus dosingExperimental therapiesIntravenous administrationJugular veinPAH researchMultiple injectionsTime courseAbstract 15751: Trends and Disparities in Pulmonary Arterial Hypertension Over the Past Decade: A National Inpatient Sample Database Study
Sana M, ASEMOTA I, Park D, Hammo H, Yilmaz M, Murthi M, Sharma B, Shaka H. Abstract 15751: Trends and Disparities in Pulmonary Arterial Hypertension Over the Past Decade: A National Inpatient Sample Database Study. Circulation 2022, 146: a15751-a15751. DOI: 10.1161/circ.146.suppl_1.15751.Peer-Reviewed Original ResearchDiagnosis of pulmonary arterial hypertensionPulmonary arterial hypertensionMedian household incomePrincipal diagnosisLength of hospital stayArterial hypertensionCrude admission ratesNo significant differenceInpatient mortality rateRate of pulmonary arterial hypertensionRisk of pulmonary arterial hypertensionAssociated with increased riskOutcome analysisNational Inpatient Sample databasePrimary outcome analysisSecondary outcome analysisAsian/Pacific IslanderSignificant differenceInpatient Sample databaseAdult hospitalsInpatient mortalityICD-9Household incomeCalendar yearAfrican AmericansBezold–Jarisch reflex mediated syncope in pulmonary arterial hypertension: An illustrative case series
Agboola K, Kowlgi G, Csecs I, DuBrock H, Cajigas H, Allison T. Bezold–Jarisch reflex mediated syncope in pulmonary arterial hypertension: An illustrative case series. Pulmonary Circulation 2022, 12: e12147. PMID: 36325510, PMCID: PMC9618286, DOI: 10.1002/pul2.12147.Peer-Reviewed Original ResearchEndothelial mechanosensing: A forgotten target to treat vascular remodeling in hypertension?
Tiezzi M, Deng H, Baeyens N. Endothelial mechanosensing: A forgotten target to treat vascular remodeling in hypertension? Biochemical Pharmacology 2022, 206: 115290. PMID: 36241094, DOI: 10.1016/j.bcp.2022.115290.Peer-Reviewed Original ResearchConceptsFuture drug development strategiesMechanistic cuesVascular remodelingNew potential therapeutic approachReceptor complexIon channelsPulmonary arterial hypertensionMechanosensitive organMesenchymal transitionPotential therapeutic approachDrug development strategiesCommon mechanismPleiotropic actionsArterial hypertensionEssential hypertensionEndothelial inflammationTherapeutic approachesBlood flowTissue perfusionVascular integrityHypertensionDistinct diseasesCrucial roleRecent studiesRemodelingBMPR1A promotes ID2–ZEB1 interaction to suppress excessive endothelial to mesenchymal transition
Lee H, Adachi T, Pak B, Park S, Hu X, Choi W, Kowalski PS, Chang CH, Clapham KR, Lee A, Papangeli I, Kim J, Han O, Park J, Anderson DG, Simons M, Jin S, Chun HJ. BMPR1A promotes ID2–ZEB1 interaction to suppress excessive endothelial to mesenchymal transition. Cardiovascular Research 2022, 119: 813-825. PMID: 36166408, PMCID: PMC10409893, DOI: 10.1093/cvr/cvac159.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsBone Morphogenetic Protein Receptors, Type IEndothelial CellsEndotheliumEpithelial-Mesenchymal TransitionHypertension, PulmonaryInhibitor of Differentiation Protein 2LungMicePulmonary Arterial HypertensionReceptor, Transforming Growth Factor-beta Type IIZinc Finger E-box-Binding Homeobox 1ConceptsPathogenesis of PAHPulmonary arterial hypertensionEndothelial cellsOnset of PAHAmeliorate pulmonary arterial hypertensionPotential novel therapeutic targetType 1 receptorType 2 receptorEndothelial-mesenchymal transitionNovel therapeutic targetGrowth factor-beta stimulationSmooth muscle cellsBone morphogenetic proteinPAH patientsArterial hypertensionVascular disordersBMP type 1 receptorsResponse of ECsAdult miceEndoMTTherapeutic targetBeta stimulationPathogenesisMesenchymal transitionMuscle cellsA Morphomolecular Approach to Alveolar Capillary Dysplasia
Kamp J, Neubert L, Ackermann M, Stark H, Plucinski E, Shah H, Janciauskiene S, Bergmann A, Schmidt G, Welte T, Haverich A, Werlein C, Braubach P, Laenger F, Schwerk N, Olsson K, Fuge J, Park D, Schupp J, Hoeper M, Kuehnel M, Jonigk D. A Morphomolecular Approach to Alveolar Capillary Dysplasia. American Journal Of Pathology 2022, 192: 1110-1121. PMID: 35649494, DOI: 10.1016/j.ajpath.2022.05.004.Peer-Reviewed Original ResearchConceptsAlveolar capillary dysplasiaHigh prevalencePersistent pulmonary arterial hypertensionPulmonary arterial hypertension groupC motif chemokine receptor 4CXCR4 ligand CXCL12Pulmonary arterial hypertensionEndothelial tyrosine kinase receptorHypoxia-inducible factor-1αChemokine receptor 4Anti-angiogenic agentsIntussusceptive angiogenesisArterial hypertensionHypertension groupTyrosine kinase receptorsFatal outcomeHealthy controlsReceptor 4Ligand CXCL12Vascular remodelingMicrovascular corrosion castsACD groupMicrovascular morphologyFactor-1αExome sequencingPressure‐based estimation of right ventricular ejection fraction
Heerdt PM, Singh I, Elassal A, Kheyfets V, Richter MJ, Tello K. Pressure‐based estimation of right ventricular ejection fraction. ESC Heart Failure 2022, 9: 1436-1443. PMID: 35150211, PMCID: PMC8934966, DOI: 10.1002/ehf2.13839.Peer-Reviewed Original ResearchConceptsRight ventricular ejection fractionCardiac magnetic resonance imagingRight heart catheterizationPulmonary arterial hypertensionMagnetic resonance imagingRV pressure waveformsVentricular ejection fractionRV responseEjection fractionMale PAH patientsCardiac MRI measurementsReceiver operator characteristic curvePressure waveformROC curve analysisClinical reference standardOperator characteristic curveSignificant reductionMeasurements of RVEFPAH patientsArterial hypertensionHeart catheterizationSecondary outcomesStudy cohortLimits of agreementBland-Altman analysis9 Systemic and pulmonary arterial hypertension
Selzer A, Fontes M, Heerdt P. 9 Systemic and pulmonary arterial hypertension. 2022, 187-202. DOI: 10.1016/b978-0-323-71860-8.00018-5.Peer-Reviewed Original ResearchPulmonary artery pressurePulmonary hypertensionSystemic hypertensionArterial pressurePrevalence of pulmonary arterial hypertensionDiagnosis of pulmonary hypertensionSixth World SymposiumRight heart catheterizationPulmonary arterial hypertensionAmerican College of Cardiology/American Heart AssociationPharmacological treatment modalitiesPrimary systemic hypertensionSystolic blood pressureHeart catheterizationPerioperative morbidityThoracic surgeryArterial hypertensionSecondary hypertensionAdult patientsTreatment modalitiesWorld SymposiumMm HgPerioperative implicationsHeart AssociationPostoperative phaseCardiopulmonary Exercise Testing in Pulmonary Vascular Disease
Joseph P, Systrom D. Cardiopulmonary Exercise Testing in Pulmonary Vascular Disease. 2022, 582-596. DOI: 10.1016/b978-0-12-801238-3.11478-3.ChaptersInvasive cardiopulmonary exercise testingCardiopulmonary exercise testingPulmonary vascular diseaseArtery catheterExercise testingCardiac outputPulmonary circulationVascular diseaseNoninvasive cardiopulmonary exercise testingNormal diagnostic workupPulmonary parenchymal diseaseRight heart catheterizationPulmonary arterial hypertensionPulmonary artery catheterArterial blood gasesRadial artery catheterModern medical therapyUseful screening testFick cardiac outputMeasurements of rightArterial hypertensionHeart catheterizationPulmonary hypertensionVenous hypertensionExercise impairment
2021
Right predominant electrical remodeling in a pure model of pulmonary hypertension promotes reentrant arrhythmias
Strauss B, Bisserier M, Obus E, Katz MG, Fargnoli A, Cacheux M, Akar JG, Hummel JP, Hadri L, Sassi Y, Akar FG. Right predominant electrical remodeling in a pure model of pulmonary hypertension promotes reentrant arrhythmias. Heart Rhythm 2021, 19: 113-124. PMID: 34563688, PMCID: PMC8742785, DOI: 10.1016/j.hrthm.2021.09.021.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionVT/VFExtrapulmonary toxicityPN ratsVentricular tachycardia/fibrillationCardiac magnetic resonance imagingRight ventricular hypertrophySprague-Dawley ratsMultiple reentrant circuitsConnexin 43 expressionMagnetic resonance imagingConnexin 43 phosphorylationRV activationArterial hypertensionMonocrotaline modelVentricular hypertrophyLeft pneumonectomyElectrical remodelingMyocardial fibrosisConduction slowingSevere formAP durationArrhythmic vulnerabilityReentrant circuitAP alternansSex Differences and the Role of Sex Hormones in Pulmonary Hypertension
Oakland HT, Joseph P. Sex Differences and the Role of Sex Hormones in Pulmonary Hypertension. Clinics In Chest Medicine 2021, 42: 457-465. PMID: 34353451, DOI: 10.1016/j.ccm.2021.04.006.ChaptersConceptsPulmonary hypertensionProgressive pulmonary vascular remodelingRight heart failurePulmonary arterial hypertensionPulmonary vascular remodelingSex differencesArterial hypertensionHeart failureFemale sexSex hormonesVascular remodelingHypertensionFuture treatmentBeneficial effectsEventual developmentMultiple mechanismsVasoconstrictionEarlier descriptionsInflammationPathophysiologyEstrogenPrevalenceDiseaseHormone
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