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Jambon-Barbara C, Hlavaty A, Bernardeau C, Bouvaist H, Chaumais MC, Humbert M, Montani D, Cracowski JL, Khouri C. Development and validation of a code-based algorithm using in-hospital medical records to identify patients with pulmonary arterial hypertension in a French healthcare database. ERJ Open Res 2024; 10:00109-2024. [PMID: 39135662 PMCID: PMC11317892 DOI: 10.1183/23120541.00109-2024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2024] [Accepted: 04/11/2024] [Indexed: 08/15/2024] Open
Abstract
Introduction Pulmonary arterial hypertension (PAH) is a rare and severe disease for which most of the evidence about prognostic factors, evolution and treatment efficacy comes from cohorts, registries and clinical trials. We therefore aimed to develop and validate a new PAH identification algorithm that can be used in the French healthcare database "Système National des Données de Santé (SNDS)". Methods We developed and validated the algorithm using the Grenoble Alpes University Hospital medical charts. We first identified PAH patients following a previously validated algorithm, using in-hospital ICD-10 (10th revision of the International Statistical Classification of Diseases) codes, right heart catheterisation procedure and PAH-specific treatment dispensing. Then, we refined the latter with the exclusion of chronic thromboembolic pulmonary hypertension procedures and treatment, the main misclassification factor. Second, we validated this algorithm using a gold standard review of in-hospital medical charts and calculated sensitivity, specificity, positive and negative predictive value (PPV and NPV) and accuracy. Finally, we applied this algorithm in the French healthcare database and described the characteristics of the identified patients. Results In the Grenoble University Hospital, we identified 252 unique patients meeting all the algorithm's criteria between 1 January 2010 and 30 June 2022, and reviewed all medical records. The sensitivity, specificity, PPV, NPV and accuracy were 91.0%, 74.3%, 67.9%, 93.3% and 80.6%, respectively. Application of this algorithm to the SNDS yielded the identification of 9931 patients with consistent characteristics compared to PAH registries. Conclusion Overall, we propose a new PAH identification algorithm developed and adapted to the French specificities that can be used in future studies using the French healthcare database.
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Affiliation(s)
- Clément Jambon-Barbara
- Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France
- Univ. Grenoble Alpes, HP2 Laboratory, Inserm U1300, Grenoble, France
| | - Alex Hlavaty
- Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France
- Univ. Grenoble Alpes, HP2 Laboratory, Inserm U1300, Grenoble, France
| | - Claire Bernardeau
- Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France
| | - Hélène Bouvaist
- Cardiology Unit, Grenoble Alpes University Hospital, Grenoble, France
| | - Marie-Camille Chaumais
- INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
- Assistance Publique-Hôpitaux de Paris (AP-HP), Department of Pharmacy, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
- Faculty of Pharmacy, Université Paris-Saclay, Saclay, France
| | - Marc Humbert
- INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
- Faculty of Medicine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
- AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Centre, Hôpital Bicêtre, DMU 5 Thorinno, Le Kremlin-Bicêtre, France
| | - David Montani
- INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
- Faculty of Medicine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
- AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Centre, Hôpital Bicêtre, DMU 5 Thorinno, Le Kremlin-Bicêtre, France
| | - Jean-Luc Cracowski
- Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France
- Univ. Grenoble Alpes, HP2 Laboratory, Inserm U1300, Grenoble, France
| | - Charles Khouri
- Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France
- Univ. Grenoble Alpes, HP2 Laboratory, Inserm U1300, Grenoble, France
- Grenoble Alpes University Hospital, Clinical Pharmacology Department INSERM CIC1406, Grenoble, France
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Khan S, Randhawa S, Patail H, Spira Y, Frishman WH, Aronow WS, Lanier GM. Pharmacological Update and Emerging Treatments of Pulmonary Hypertension. Cardiol Rev 2024:00045415-990000000-00192. [PMID: 38294225 DOI: 10.1097/crd.0000000000000647] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2024]
Abstract
Pulmonary hypertension (PH) is defined as elevated pressures in the pulmonary artery and is associated with significant morbidity and mortality. The World Health Organization classifies PH into 5 distinct groups based on underlying etiology, pathology, and modality of treatment. Therapeutic approach may be challenging due to the extensive spectrum of causes and underlying mechanisms mediating PH. The 5 groups include pulmonary arterial hypertension (group 1), PH secondary to left heart disease (group 2), PH secondary to chronic lung disease (group 3), chronic thromboembolic pulmonary hypertension (group 4), and PH due to miscellaneous causes (group 5). Although significant progress has been made in the treatment of group 1 PH, there is a continued need to develop new therapies for all types of PH. Additionally, most treatments currently available improve functional capacity and symptoms but without a significant benefit in mortality. In this review, we aim to describe the various etiologies of PH and their established pharmacotherapies, as well as expand on emerging therapeutic options for each group.
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Affiliation(s)
- Shazli Khan
- From the Department of Cardiology, Westchester Medical Center, New York Medical College, Valhalla, NY
| | - Sukhbir Randhawa
- Department of Internal Medicine, Samaritan Medical Center, Watertown, NY
| | - Haris Patail
- Department of Internal Medicine, University of Connecticut School of Medicine, Hartford, CT
| | - Yaakov Spira
- Department of Internal Medicine, Westchester Medical Center, New York Medical College, Valhalla, NY
| | - William H Frishman
- Department of Internal Medicine, Westchester Medical Center, New York Medical College, Valhalla, NY
| | - Wilbert S Aronow
- From the Department of Cardiology, Westchester Medical Center, New York Medical College, Valhalla, NY
| | - Gregg M Lanier
- From the Department of Cardiology, Westchester Medical Center, New York Medical College, Valhalla, NY
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3
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Ling WHI, Wong CF, Yan SW, Fan YYK, Wong KL. The hemodynamic characteristics of severe chronic lung disease referred for lung transplantation. Pulm Circ 2022; 12:e12082. [PMID: 35864910 PMCID: PMC9294294 DOI: 10.1002/pul2.12082] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/08/2022] [Revised: 03/07/2022] [Accepted: 04/11/2022] [Indexed: 11/09/2022] Open
Abstract
Severe pulmonary hypertension (PH) is not common even in patients with severe chronic lung disease (CLD) but data on hemodynamic characteristics among patients with severe CLD is scarce. All adult patients who had right heart catheterization for lung transplant assessment for severe CLD in the only lung transplant service and for PAH management in the only tertiary pulmonary hypertension service in Hong Kong from 2010 to 2020 were included and classified into CLD group and PAH group. Patient characteristics and hemodynamic parameters were analyzed. There were 153 patients included with 106 patients in the CLD group and 47 in the PAH group. There were only 19.8% of the patients in the CLD group had severe pulmonary hypertension. Patients in the CLD group had significantly lower systolic pulmonary arterial pressure (PAPs), lower mean pulmonary arterial pressure (PAPm), higher cardiac index, and lower PVR when compared with the PAH group (p < 0.001). The area under curve (AUC) of PAPs, PAPm, and PVR were excellent, 0.973, 0.970, and 0.938, respectively for discrimination between CLD and PAH on receiver operator characteristics curve analysis. Optimal cutoff values were 55.5 mmHg, 35.5 mmHg, and 6.1 Wood Units for PAPs, PAPm, and PVR with Youden Index 0.85, 0.80, and 0.82, respectively. There were distinct hemodynamic characteristics between the CLD group and the PAH group. Systolic pulmonary arterial pressure, mean pulmonary arterial pressure, and pulmonary vascular resistance are useful to discriminate between the phenotype of severe CLD and PAH.
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Affiliation(s)
| | - Chi F. Wong
- Tuberculosis and Chest Medical UnitGrantham HospitalAberdeenHong Kong SAR
| | - See W. Yan
- Tuberculosis and Chest Medical UnitGrantham HospitalAberdeenHong Kong SAR
| | - Yue Y. K. Fan
- Cardiac Medical UnitGrantham HospitalAberdeenHong Kong SAR
| | - Ka L. Wong
- Cardiac Medical UnitGrantham HospitalAberdeenHong Kong SAR
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4
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Luca E, Bodrug N. The frequency of pulmonary hypertension in chronic obstructive pulmonary disease of geriatric patients: a narrative literature review. THE EGYPTIAN JOURNAL OF INTERNAL MEDICINE 2022. [DOI: 10.1186/s43162-022-00135-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Pulmonary hypertension (PH) is a serious complication with complex pathogenesis in the natural history of chronic obstructive pulmonary disease COPD, with a progressively increasing frequency with a meanwhile decreasing in functional capacity.
Purpose
Assessment of the incidence, pathogenesis, peculiarities, and complications of PH in COPD in geriatric population worldwide.
Methods
We performed an analysis of randomized, retrospective, and prospective clinical, case-control and observational studies, published at the international level, according to the subject studied and target population. Four hundred ninety-seven full articles were identified after the search through engine Google Search and databases PubMed, Hinari, SpringerLink, and Scopus (Elsevier) according to the keywords and subsequent filters.
Results
Depending on various factors, like the population examined, the definition used for mPAP (mPAP> 20 mm Hg or ≥25 mm Hg), the severity of the lung disease, and the method of measuring PAP, a varied incidence of COPD patients with PH complication was discovered, namely 10–91%. PH prevalence increases with the COPD severity. The presence of PH is associated with acute exacerbations of COPD, reduced survival, and increasing expenses for healthcare programs. Mild to moderate levels of PH (mPAP 25–34 mm Hg) are relatively common in COPD and usually are associated with severe airflow obstruction or parenchymal destruction. Only a minority of patients (1–5%) have severe PH (mPAP ≥35 mm Hg).
Conclusions
Diagnosis of PH in COPD is difficult, especially in a mild form, and requires a clinical approach associated with a comprehensive set of investigations for confirming the etiology, evaluation of the functional and hemodynamical impairment severity, and important factors in the appropriate treatment election.
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5
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Abstract
Pulmonary hypertension (PH) because of chronic lung disease is categorized as Group 3 PH in the most recent classification system. Prevalence of these diseases is increasing over time, creating a growing need for effective therapeutic options. Recent approval of the first pulmonary arterial hypertension therapy for the treatment of Group 3 PH related to interstitial lung disease represents an encouraging advancement. This review focuses on molecular mechanisms contributing to pulmonary vasculopathy in chronic hypoxia, the pathology and epidemiology of Group 3 PH, the right ventricular dysfunction observed in this population and clinical trial data that inform the use of pulmonary vasodilators in Group 3 PH.
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Affiliation(s)
- Navneet Singh
- Division of Pulmonary, Critical Care and Sleep Medicine (N.S., C.E.V.), Brown University, Providence, RI
| | - Peter Dorfmüller
- Department of Pathology, Universities of Giessen and Marburg Lung Center (UGMLC), Justus-Liebig University, Germany (P.D.).,German Center for Lung Research (DZL), Giessen, Germany (P.D.)
| | - Oksana A Shlobin
- Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA (O.A.S.)
| | - Corey E Ventetuolo
- Division of Pulmonary, Critical Care and Sleep Medicine (N.S., C.E.V.), Brown University, Providence, RI.,Department of Health Services, Policy and Practice (C.E.V.), Brown University, Providence, RI
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6
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Alkhanfar D, Shahin Y, Alandejani F, Dwivedi K, Alabed S, Johns C, Lawrie A, Thompson AAR, Rothman AMK, Tschirren J, Uthoff JM, Hoffman E, Condliffe R, Wild JM, Kiely DG, Swift AJ. Severe pulmonary hypertension associated with lung disease is characterised by a loss of small pulmonary vessels on quantitative computed tomography. ERJ Open Res 2022; 8:00503-2021. [PMID: 35586449 PMCID: PMC9108962 DOI: 10.1183/23120541.00503-2021] [Citation(s) in RCA: 19] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2021] [Accepted: 02/10/2022] [Indexed: 11/28/2022] Open
Abstract
Background Pulmonary hypertension (PH) in patients with chronic lung disease (CLD) predicts reduced functional status, clinical worsening and increased mortality, with patients with severe PH-CLD (≥35 mmHg) having a significantly worse prognosis than mild to moderate PH-CLD (21-34 mmHg). The aim of this cross-sectional study was to assess the association between computed tomography (CT)-derived quantitative pulmonary vessel volume, PH severity and disease aetiology in CLD. Methods Treatment-naïve patients with CLD who underwent CT pulmonary angiography, lung function testing and right heart catheterisation were identified from the ASPIRE registry between October 2012 and July 2018. Quantitative assessments of total pulmonary vessel and small pulmonary vessel volume were performed. Results 90 patients had PH-CLD including 44 associated with COPD/emphysema and 46 with interstitial lung disease (ILD). Patients with severe PH-CLD (n=40) had lower small pulmonary vessel volume compared to patients with mild to moderate PH-CLD (n=50). Patients with PH-ILD had significantly reduced small pulmonary blood vessel volume, compared to PH-COPD/emphysema. Higher mortality was identified in patients with lower small pulmonary vessel volume. Conclusion Patients with severe PH-CLD, regardless of aetiology, have lower small pulmonary vessel volume compared to patients with mild-moderate PH-CLD, and this is associated with a higher mortality. Whether pulmonary vessel changes quantified by CT are a marker of remodelling of the distal pulmonary vasculature requires further study.
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Affiliation(s)
- Dheyaa Alkhanfar
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,INSIGNEO, Institute for In Silico Medicine, University of Sheffield, Sheffield, UK
| | - Yousef Shahin
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,Dept of Clinical Radiology, Sheffield Teaching Hospitals, Sheffield, UK
| | - Faisal Alandejani
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK
| | - Krit Dwivedi
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK
| | - Samer Alabed
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,Dept of Clinical Radiology, Sheffield Teaching Hospitals, Sheffield, UK
| | - Chris Johns
- Dept of Clinical Radiology, Sheffield Teaching Hospitals, Sheffield, UK
| | - Allan Lawrie
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK
| | - A A Roger Thompson
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
| | - Alexander M K Rothman
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
| | | | - Johanna M Uthoff
- Dept of Computer Science, University of Sheffield, Sheffield, UK
| | - Eric Hoffman
- Dept of Radiology, University of Iowa, Iowa City, IA, USA
| | - Robin Condliffe
- Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
| | - Jim M Wild
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,INSIGNEO, Institute for In Silico Medicine, University of Sheffield, Sheffield, UK
| | - David G Kiely
- INSIGNEO, Institute for In Silico Medicine, University of Sheffield, Sheffield, UK.,Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.,These authors contributed equally
| | - Andrew J Swift
- Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.,INSIGNEO, Institute for In Silico Medicine, University of Sheffield, Sheffield, UK.,These authors contributed equally
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7
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Jiang R, Wang L, Zhao QH, Wu C, Yuan P, Wang S, Zhang R, Gong SG, Wu WH, He J, Qiu HL, Luo CJ, Liu JM, Jing ZC. Echocardiography Nomogram for Predicting Survival among Chronic Lung Disease Patients with Severe Pulmonary Hypertension. J Clin Med 2022; 11:jcm11061603. [PMID: 35329931 PMCID: PMC8955171 DOI: 10.3390/jcm11061603] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2022] [Revised: 02/12/2022] [Accepted: 03/12/2022] [Indexed: 12/03/2022] Open
Abstract
Severe pulmonary hypertension in chronic lung diseases (severe CLD-PH) differs significantly from other types of PH in physiology and prognosis. We aimed to assess whether echocardiography helps predict long-term survival in patients with severe CLD-PH. This single-centre, observational cohort study enrolled 100 patients with severe CLD-PH (mean pulmonary arterial pressure ≥35 mm Hg or ≥25 mm Hg with cardiac index <2.0 L/min/m2 or pulmonary vascular resistance ≥6 Wood units) between 2009 and 2014. The population was randomly divided into a derivation and validation cohort in a 2:1 ratio. To construct a nomogram, a multivariable logistic regression model was applied, and scores were assigned based on the hazard ratio of independent echocardiographic predictors. Multivariate Cox hazards analysis identified the strongest predictors of mortality as pulmonary arterial systolic pressure (PASP), tricuspid annular plane systolic excursion, and right ventricular end-diastolic transverse dimension. The three independent predictors were entered into the nomogram. Compared with PASP alone, the nomogram resulted in an integrated discrimination improvement of 15.5% (95% confidence interval, 5.52−25.5%, p = 0.002) with a net improvement in model discrimination (C-statistic from 0.591 to 0.746). Using echocardiographic parameters, we established and validated a novel nomogram to predict all-cause death for patients with severe CLD-PH.
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Affiliation(s)
- Rong Jiang
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Lan Wang
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Qin-Hua Zhao
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Cheng Wu
- Department of Health Statistics, Naval Medical University, 800 Xiangyin Road, Shanghai 200433, China;
| | - Ping Yuan
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Shang Wang
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Rui Zhang
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Su-Gang Gong
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Wen-Hui Wu
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Jing He
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Hong-Ling Qiu
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Ci-Jun Luo
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Jin-Ming Liu
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
| | - Zhi-Cheng Jing
- Department of Cardiopulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China; (R.J.); (L.W.); (Q.-H.Z.); (P.Y.); (S.W.); (R.Z.); (S.-G.G.); (W.-H.W.); (J.H.); (H.-L.Q.); (C.-J.L.); (J.-M.L.)
- Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, 1 Shuai-Fu-Yuan, Dongcheng District, Beijing 100730, China
- Correspondence:
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Wang L, Shen L, Zhao YL, Pudasaini B, Zhao QH, Gong SG, Zhang R, Yuan P, He J, Luo CJ, Qiu HL, Liu JM, Jiang R. Survival in severe pulmonary hypertension due to chronic lung disease: influence of in-hospital platelet distribution width. Pulm Circ 2021; 11:20458940211026484. [PMID: 34276962 PMCID: PMC8258768 DOI: 10.1177/20458940211026484] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2020] [Accepted: 05/29/2021] [Indexed: 11/22/2022] Open
Abstract
Platelet distribution width has been recognized as risk predictors of idiopathic pulmonary arterial hypertension. This study aims to investigate whether in-hospital platelet distribution width would be useful to predict all-cause death in patients with severe pulmonary hypertension due to chronic lung diseases (CLD-PH). Early in-hospital platelet distribution width was measured in 67 severe CLD-PH patients who were confirmed by right heart catheterization and followed up. Event-free survival was estimated using the Kaplan–Meier method and analyzed with the log-rank test. Cox proportional hazards models were performed to determine the association between the platelet distribution width level and all-cause death. During median of 2.4 (2.5, 3.7) years of follow-up, 44 patients died. A significant association was noted between in-hospital platelet distribution width level and the adjusted risk of all-cause mortality (hazard ratio: 1.245; 95% confidence interval: 1.117–1.386, P < 0.001). Compared with those with platelet distribution width <16.1%, the hazard ratio for all-cause death increased by 5.278 (95% confidence interval: 2.711–10.276, P < 0.0001) among patients with platelet distribution width ≥16.1%. Higher levels of platelet distribution width were also associated with increased risk of all-cause death. In-hospital platelet distribution width was independently associated with all-cause death in patients with severe CLD-PH. This potentially could be used to estimate the severity of severe CLD-PH.
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Affiliation(s)
- Lan Wang
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Li Shen
- Emergency Department, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Ya-Lin Zhao
- Department of Respiratory Critical Care Medicine, the First Hospital of Kunming, Kunming, China
| | | | - Qin-Hua Zhao
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Su-Gang Gong
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Rui Zhang
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Ping Yuan
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Jing He
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Ci-Jun Luo
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Hong-Ling Qiu
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Jin-Ming Liu
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Rong Jiang
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
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9
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Saeed ZH, Magdy MH, Abdelnaem EA, Mahmoud MM. Serum Krebs von den Lungen (KL-6) level as a marker of exacerbation of interstitial lung diseases. THE EGYPTIAN JOURNAL OF BRONCHOLOGY 2020. [DOI: 10.1186/s43168-020-00043-w] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Krebs von den Lungen (KL-6) is elevated in serum of interstitial lung disease (ILD) patients based on its leakage from the alveolar space into the blood; KL-6 is significantly higher in patients with acute exacerbation of ILDs (AE-ILD) compared with stable patients. This study aimed to determine the sensitivity and specificity of KL6 to detect AE-ILD.
Results
This is a prospective cross sectional observational study was carried out on 88 subjects at the Chest Department, Minia Cardiothoracic University Hospital, during the period from August 2018 to August 2019. This study was approved by the hospital research ethics board of Minia University and informed consent was obtained. History, examination, spirometry, ABGs, X-ray, HRCT, CBC, ESR, CRP, and KL6 levels were done to both stable and exacerbation groups of ILDs. The level of biomarkers is compared between both groups and control.
Statistical analysis done by using IBM SPSS statistical package version 20 (χ2 test and independent sample t test, ANOVA test, bivariate Pearson correlation analysis, and ROC curve analysis).
The study showed that there is a significant difference between stable and exacerbating groups regarding fever, signs of RHF, dyspnea scale, FVC, and PaO2.
Conclusion
KL-6 cutoff ≥ 187.5 U/ml could exhibit AE-ILDs with a sensitivity of 98% and a specificity of 97%. KL-6 is a more sensitive and specific marker to detect AE-ILD.
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10
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Gredic M, Blanco I, Kovacs G, Helyes Z, Ferdinandy P, Olschewski H, Barberà JA, Weissmann N. Pulmonary hypertension in chronic obstructive pulmonary disease. Br J Pharmacol 2020; 178:132-151. [PMID: 31976545 DOI: 10.1111/bph.14979] [Citation(s) in RCA: 51] [Impact Index Per Article: 10.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2019] [Revised: 12/29/2019] [Accepted: 01/06/2020] [Indexed: 12/12/2022] Open
Abstract
Even mild pulmonary hypertension (PH) is associated with increased mortality and morbidity in patients with chronic obstructive pulmonary disease (COPD). However, the underlying mechanisms remain elusive; therefore, specific and efficient treatment options are not available. Therapeutic approaches tested in the clinical setting, including long-term oxygen administration and systemic vasodilators, gave disappointing results and might be only beneficial for specific subgroups of patients. Preclinical studies identified several therapeutic approaches for the treatment of PH in COPD. Further research should provide deeper insight into the complex pathophysiological mechanisms driving vascular alterations in COPD, especially as such vascular (molecular) alterations have been previously suggested to affect COPD development. This review summarizes the current understanding of the pathophysiology of PH in COPD and gives an overview of the available treatment options and recent advances in preclinical studies. LINKED ARTICLES: This article is part of a themed issue on Risk factors, comorbidities, and comedications in cardioprotection. To view the other articles in this section visit http://onlinelibrary.wiley.com/doi/10.1111/bph.v178.1/issuetoc.
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Affiliation(s)
- Marija Gredic
- Cardio-Pulmonary Institute, University of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Justus-Liebig-University, Giessen, Germany
| | - Isabel Blanco
- Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.,Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain
| | - Gabor Kovacs
- Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria.,Division of Pulmonology, Department of Internal Medicine, Medical University of Graz, Graz, Austria
| | - Zsuzsanna Helyes
- Department of Pharmacology and Pharmacotherapy, Medical School & János Szentágothai Research Centre, University of Pécs, Pécs, Hungary.,PharmInVivo Ltd, Pécs, Hungary
| | - Péter Ferdinandy
- Department of Pharmacology and Pharmacotherapy, Semmelweis University, Budapest, Hungary.,Pharmahungary Group, Szeged, Hungary
| | - Horst Olschewski
- Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria.,Division of Pulmonology, Department of Internal Medicine, Medical University of Graz, Graz, Austria
| | - Joan Albert Barberà
- Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.,Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain
| | - Norbert Weissmann
- Cardio-Pulmonary Institute, University of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Justus-Liebig-University, Giessen, Germany
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11
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Kovacs G, Agusti A, Barberà JA, Celli B, Criner G, Humbert M, Sin DD, Voelkel N, Olschewski H. Pulmonary Vascular Involvement in Chronic Obstructive Pulmonary Disease. Is There a Pulmonary Vascular Phenotype? Am J Respir Crit Care Med 2019; 198:1000-1011. [PMID: 29746142 DOI: 10.1164/rccm.201801-0095pp] [Citation(s) in RCA: 124] [Impact Index Per Article: 20.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Affiliation(s)
- Gabor Kovacs
- 1 Medical University of Graz, Graz, Austria.,2 Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria
| | - Alvar Agusti
- 3 Respiratory Institute, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute, University of Barcelona, Barcelona, Spain.,4 Centro Investigacion Biomedica en Red de Enfermedades Respiratorias, Madrid, Spain
| | - Joan Albert Barberà
- 3 Respiratory Institute, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute, University of Barcelona, Barcelona, Spain.,4 Centro Investigacion Biomedica en Red de Enfermedades Respiratorias, Madrid, Spain
| | | | - Gerard Criner
- 6 Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania
| | - Marc Humbert
- 7 Université Paris-Sud, Université Paris-Saclay; Inserm U999; Hôpital Bicêtre, Assistance Publique - Hôpitaux de Paris, Le Kremlin Bicêtre, France
| | - Don D Sin
- 8 Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, British Columbia, Canada.,9 Division of Respiratory Medicine, Department of Medicine, University of British Columbia, Vancouver, British Columbia; Canada; and
| | - Norbert Voelkel
- 10 Department of Pulmonary Medicine, Frije University, Medical Center, Amsterdam, the Netherlands
| | - Horst Olschewski
- 1 Medical University of Graz, Graz, Austria.,2 Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria
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12
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Darwiche T, Collum SD, Bi W, Reynolds JO, Wilson C, Wareing N, Hernandez AM, Mertens TCJ, Zhou Z, Pandit LM, Karmouty-Quintana H. Alterations in cardiovascular function in an experimental model of lung fibrosis and pulmonary hypertension. Exp Physiol 2019; 104:568-579. [PMID: 30663834 DOI: 10.1113/ep087321] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2018] [Accepted: 01/18/2019] [Indexed: 01/02/2023]
Abstract
NEW FINDINGS What is the central question of this study? We have evaluated changes in cardiovascular physiology using echocardiography in an experimental model of lung fibrosis. What is the main finding and its importance? Remarkably, we report changes in cardiovascular function as early as day 7, concomitant with evidence of vascular remodelling. We also report that isolated pulmonary arteries were hypercontractile in response to a thromboxane A2 agonist. These findings are significant because the development of pulmonary hypertension is one of the most significant predictors of mortality in patients with lung fibrosis, where there are no available therapies and a lack of animal models. ABSTRACT Group III pulmonary hypertension is observed in patients with chronic lung diseases such as chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) develops as a result of extensive pulmonary vascular remodelling and resultant changes in vascular tone that can lead to right ventricle hypertrophy. This eventually leads to right heart failure, which is the leading indicator of mortality in patients with idiopathic pulmonary fibrosis. Treatments for group III PH are not available, in part owing to a lack of viable animal models. Here, we have evaluated the cardiovascular changes in a model of lung fibrosis and PH. Data obtained from this study indicated that structural alterations in the right heart, such as right ventricular wall hypertrophy, occurred as early as day 14, and similar increases in right ventricle chamber size were seen between days 21 and 28. These structural changes were correlated with decreases in the systolic function of the right ventricle and right ventricular cardiac output, which also occurred between the same time points. Characterization of pulmonary artery dynamics also highlighted that PH might be occurring as early as day 21, indicated by reductions in the velocity-time integral; however, evidence for PH is apparent as early as day 7, indicated by the significant reduction in pulmonary acceleration time values. These changes are consistent with evidence of vascular remodelling observed histologically starting on day 7. In addition, we report hyperactivity of bleomycin-exposed pulmonary arteries to a thromboxane A2 receptor (Tbxa2r) agonist.
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Affiliation(s)
- Tamara Darwiche
- Department of Pharmacology, School of Biomedical Sciences, King's College London, London, UK.,Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Scott D Collum
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Weizhen Bi
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Julia O Reynolds
- Department of Medicine, Pulmonary, Critical Care and Sleep Medicine, Baylor College of Medicine, Houston, TX, USA
| | - Cory Wilson
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Nancy Wareing
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Adriana M Hernandez
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Tinne C J Mertens
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Zhen Zhou
- Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Lavannya M Pandit
- Department of Medicine, Pulmonary, Critical Care and Sleep Medicine, Baylor College of Medicine, Houston, TX, USA
| | - Harry Karmouty-Quintana
- Department of Biochemistry and Molecular Biology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA
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13
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Jeon CH. Early Detection of Pulmonary Hypertension in Connective Tissue Disease. JOURNAL OF RHEUMATIC DISEASES 2019. [DOI: 10.4078/jrd.2019.26.1.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- Chan Hong Jeon
- Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Korea
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14
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Wang L, Jin YZ, Zhao QH, Jiang R, Wu WH, Gong SG, He J, Liu JM, Jing ZC. Hemodynamic and gas exchange effects of inhaled iloprost in patients with COPD and pulmonary hypertension. Int J Chron Obstruct Pulmon Dis 2017; 12:3353-3360. [PMID: 29200842 PMCID: PMC5702173 DOI: 10.2147/copd.s141798] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
Studies have shown that vasodilators such as iloprost can be useful for treating pulmonary hypertension (PH). However, in patients with COPD, vasodilators may inhibit hypoxic pulmonary vasoconstriction and impair gas exchange. The efficacy and safety of iloprost inhalation was assessed in 67 patients with PH associated with COPD (COPD-PH), diagnosed by right heart catheterization. Of these, 37 patients had severe PH (mean pulmonary arterial pressure [mPAP] >35 mmHg or mPAP 25-35 mmHg with low cardiac index [<2.0 L⋅min-1⋅m-2]). All patients received a single 20 µg dose of iloprost via a nebulizer (4.4 µg delivered at the mouthpiece). No serious adverse events were reported. Hemodynamic and gas exchange parameters (arterial blood gas and shunt fraction [Qs/Qt]) were measured or calculated at baseline and 10 min after iloprost inhalation. mPAP decreased by 2.1 mmHg (95% CI, -3.3 to -1.0), pulmonary vascular resistance (PVR) decreased by 62.4 dyn⋅s⋅cm-5 (95% CI, -92.9 to -31.8), and cardiac output increased by 0.4 L⋅min-1 (95% CI, 0.2-0.5). There was a more significant decline in PVR in patients with severe COPD-PH than in those with nonsevere COPD-PH. Hypoxemia and intrapulmonary shunt were more extreme in patients with severe COPD-PH. However, there were no significant differences in arterial blood gas and Qs/Qt between patients with nonsevere and severe forms of COPD-PH. In conclusion, iloprost improved pulmonary hemodynamics without detrimental effects on arterial oxygenation in patients with COPD-PH, even in those with severe PH. These findings suggest that the short-term use of iloprost in patients with COPD-PH is effective and well tolerated.
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Affiliation(s)
- Lan Wang
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Yuan-Zhe Jin
- Department of Cardiology, The Fourth Affiliated Hospital of China Medical University, Liaoning, China
| | - Qin-Hua Zhao
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Rong Jiang
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Wen-Hui Wu
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Su-Gang Gong
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Jing He
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Jin-Ming Liu
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China
| | - Zhi-Cheng Jing
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.,Thrombosis and Vascular Medicine Center, State Key Laboratory of Cardiovascular Disease, FuWai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China
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15
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Gall H, Felix JF, Schneck FK, Milger K, Sommer N, Voswinckel R, Franco OH, Hofman A, Schermuly RT, Weissmann N, Grimminger F, Seeger W, Ghofrani HA. The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups. J Heart Lung Transplant 2017; 36:957-967. [DOI: 10.1016/j.healun.2017.02.016] [Citation(s) in RCA: 233] [Impact Index Per Article: 29.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2016] [Revised: 02/09/2017] [Accepted: 02/15/2017] [Indexed: 11/29/2022] Open
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16
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Memon HA, Lin CH, Guha A. Chronic Thromboembolic Pulmonary Hypertension: Pearls and Pitfalls of Diagnosis. Methodist Debakey Cardiovasc J 2017; 12:199-204. [PMID: 28289494 DOI: 10.14797/mdcj-12-4-199] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic obstruction of major pulmonary arteries by organized thromboembolic material. Untreated CTEPH can result in pulmonary hypertension and eventually right heart failure, yet it is the only form of pulmonary hypertension that is potentially curable with surgical or catheter-based intervention. While early diagnosis is key to increasing the likelihood of successful treatment, CTEPH remains largely underdiagnosed. This article reviews the role of echocardiogram, ventilation/perfusion scan, and other available modalities in the diagnosis of CTEPH.
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Affiliation(s)
| | - C Huie Lin
- Houston Methodist Hospital, Houston, Texas
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17
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Jiang R, Wu C, Pudasaini B, Wang L, Zhao QH, Zhang R, Wu WH, Yuan P, Jing ZC, Liu JM. A novel scoring index by Doppler echocardiography for predicting severe pulmonary hypertension due to chronic lung diseases: a cross-sectional diagnostic accuracy study. Int J Chron Obstruct Pulmon Dis 2017; 12:1741-1751. [PMID: 28652726 PMCID: PMC5476678 DOI: 10.2147/copd.s133854] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
Background Severe pulmonary hypertension (PH) resulting from a chronic lung disease (CLD) (severe CLD-PH) requires more aggressive treatment due to its increased mortality compared with mild PH. Therefore, we developed a Doppler echocardiography scoring index (ESI) to predict severe CLD-PH. Methods A derivation cohort of 107 patients with CLD who underwent echocardiography was classified into two groups, the normal/mild PH group and the severe PH group, based on the right heart catheterization. Meanwhile, we designed the ESI by multivariate logistic regression to validate the predicted outcomes. The ESI was calculated using the following formula: ESI = ESIRVEDTD + ESIPASP + ESIPAd − ESITAPSE. Additionally, the ESI was weighted by +2 points for right ventricular end-diastolic transverse dimension ≥3.8 cm or pulmonary artery diameter ≥2.7 cm, +3 points for systolic pulmonary artery pressure (PASP) ≥61 mmHg, and −3 points for tricuspid annular plane systolic excursion ≥1.65 cm. Results In the derivation cohort, PASP ≥61 mmHg estimated by echocardiography exhibited 80.4% sensitivity and 84.3% specificity with area under receiver-operating characteristic curve of 0.823 (95% CI: 0.797–0.942, P<0.0001). Compared with PASP, ESI ≥1.0 exhibited 91.1% sensitivity and 80.4% specificity, resulting in a net improvement in model performance with a change in the c-statistic from 0.823 to 0.937 and an integrated discrimination improvement of 11.3% (95% CI: 4.5%–18.2%, P=0.001). The ESI was applied to the validation cohort, resulting in 84.2% sensitivity and 81.3% specificity with 82.9% accuracy. Conclusion The ESI showed high capacity for predicting severe CLD-PH, further implying the value of noninvasive examinations in clinic.
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Affiliation(s)
- Rong Jiang
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Cheng Wu
- Department of Health Statistics, Second Military Medical University, Shanghai, People's Republic of China
| | - Bigyan Pudasaini
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Lan Wang
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Qin-Hua Zhao
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Rui Zhang
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Wen-Hui Wu
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Ping Yuan
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Zhi-Cheng Jing
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
| | - Jin-Ming Liu
- Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine
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18
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Pulmonary Hypertension Associated with Idiopathic Pulmonary Fibrosis: Current and Future Perspectives. Can Respir J 2017; 2017:1430350. [PMID: 28286407 PMCID: PMC5327768 DOI: 10.1155/2017/1430350] [Citation(s) in RCA: 51] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2016] [Accepted: 01/19/2017] [Indexed: 12/12/2022] Open
Abstract
Pulmonary hypertension (PH) is commonly present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO). PH has been identified to be present in as much as 40% of patients with COPD or IPF and it is considered as one of the principal predictors of mortality in patients with COPD or IPF. However, despite the prevalence and fatal consequences of PH in the setting of chronic lung diseases, there are limited therapies available for patients with Group III PH, with lung transplantation remaining as the most viable option. This highlights our need to enhance our understanding of the molecular mechanisms that lead to the development of Group III PH. In this review we have chosen to focus on the current understating of PH in IPF, we will revisit the main mediators that have been shown to play a role in the development of the disease. We will also discuss the experimental models available to study PH associated with lung fibrosis and address the role of the right ventricle in IPF. Finally we will summarize the current available treatment options for Group III PH outside of lung transplantation.
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19
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Panagiotou M, Church AC, Johnson MK, Peacock AJ. Pulmonary vascular and cardiac impairment in interstitial lung disease. Eur Respir Rev 2017; 26:26/143/160053. [PMID: 28096284 PMCID: PMC9488566 DOI: 10.1183/16000617.0053-2016] [Citation(s) in RCA: 35] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2016] [Accepted: 08/02/2016] [Indexed: 12/19/2022] Open
Abstract
Pulmonary vascular and cardiac impairment is increasingly appreciated as a major adverse factor in the natural history of interstitial lung disease. This clinically orientated review focuses on the current concepts in the pathogenesis, pathophysiology and implications of the detrimental sequence of increased pulmonary vascular resistance, pre-capillary pulmonary hypertension and right heart failure in interstitial lung disease, and provides guidance on its management. Development of pulmonary hypertension is a major adverse factor in the natural history of interstitial lung diseasehttp://ow.ly/nJB0302XAmD
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Affiliation(s)
- Marios Panagiotou
- Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, UK
| | - Alistair C Church
- Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, UK
| | - Martin K Johnson
- Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, UK
| | - Andrew J Peacock
- Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, UK
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20
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Pulmonary Hypertension: Scientometric Analysis and Density-Equalizing Mapping. PLoS One 2017; 12:e0169238. [PMID: 28052133 PMCID: PMC5215006 DOI: 10.1371/journal.pone.0169238] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2015] [Accepted: 12/12/2016] [Indexed: 12/12/2022] Open
Abstract
Pulmonary hypertension (PH) is characterized by the increase of the mean pulmonary arterial pressure in the lung circulation. Despite the large number of experimental and clinical studies conducted on pulmonary hypertension, there is no comprehensive work that analyzed the global research activity on PH so far. We retrieved the bibliometric data of the publications on pulmonary hypertension for two periods from the Web of science database. Here, we set the first investigation period from 1900 to 2007 (t1) due to the cited half life of articles and the relating difficulties to interpret the citation parameters. The second evaluation period (t2) covers the time interval from 2008 onwards including the year 2015. The data were analyzed and processed to density-equalizing maps using the NewQIS platform. A total number of 18,986 publications were identified in t1 that come from 85 countries. The US published the highest number of publications (n = 7,290), followed by the UK, Germany, Japan and France. In t2 19,676 items could be found worked out by 130 countries. The raking started just the same with the USA as most publishing nation with 7,127 publications on PH, followed by the UK and Germany. Japan fell back on 6th place, whereas China came into view on the 5th position. Analyzing the average citation rate as a parameter for research quality, Mexico reached the highest value in t1 and Ireland in t2. While, the country specific h-index underlined the leading position of the US research in both evaluation periods again. The average number of international collaboration items was expanding from none in 1978 to 530 items in 2015 with the USA as the country with the highest number of collaboration articles. The present study is the first large scale density-equalizing mapping and scientometric analysis of global PH research activity. Our data draw a sketch of the global research architecture in this field, indicating a need for specific research programs in countries with a lower human development index.
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Kadmon G, Schiller O, Dagan T, Bruckheimer E, Birk E, Schonfeld T. Pulmonary hypertension specific treatment in infants with bronchopulmonary dysplasia. Pediatr Pulmonol 2017; 52:77-83. [PMID: 27333438 DOI: 10.1002/ppul.23508] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/24/2016] [Revised: 05/15/2016] [Accepted: 06/08/2016] [Indexed: 11/05/2022]
Abstract
OBJECTIVE When bronchopulmonary dysplasia (BPD) is complicated by pulmonary hypertension (PH), morbidity and mortality are significantly increased. BPD-associated PH is not included in the current indications for PH medications. However, limited data demonstrate hemodynamic improvement and decreased mortality with PH-specific treatment. This report describes our 6-year experience treating BPD-associated PH with PH medications, mainly sildenafil. STUDY DESIGN The medical records of 20 infants diagnosed with BPD-associated PH at a tertiary pediatric pulmonary hypertension clinic in 2008-2014 were reviewed. Clinical improvement was defined as a decrease in Ross functional class by at least one degree. PH severity was classified by echocardiography as mild, moderate, or severe. Hemodynamic improvement was defined as a decrease in PH severity by at least one level. RESULTS Eighteen out of 20 patients were treated with PH medications: 12 sildenafil, 5 sildenafil and bosentan, and 1 bosentan. Median follow-up time was 2 years. Mean functional class significantly decreased from 3.2 ± 0.9 at diagnosis to 1.7 ± 0.9 at the last follow-up. Improvement in functional class was observed in 15/16 children (94%). Moderate or severe PH was found in 13/18 children (72%) at diagnosis, and in three (17%, all moderate PH) at the last follow-up. Improvement in PH class by echocardiography was demonstrated in 14/18 children (78%). The survival rate was 95%. CONCLUSION Treatment of BPD complicated by PH with PH-specific medications, mainly sildenafil, is associated with improvement in both clinical and hemodynamic parameters and a low mortality rate. Pediatr Pulmonol. 2017;52:77-83. © 2016 Wiley Periodicals, Inc.
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Affiliation(s)
- Gili Kadmon
- Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Ofer Schiller
- Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Tamir Dagan
- Heart Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Elchanan Bruckheimer
- Heart Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Einat Birk
- Heart Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Tommy Schonfeld
- Pulmonary Outpatient Clinic, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
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22
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23
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Ju CR, Zhang JH, Chen M, Chen RC. Plasma myostatin levels are related to the extent of right ventricular dysfunction in exacerbation of chronic obstructive pulmonary disease. Biomarkers 2016; 22:246-252. [PMID: 27323660 DOI: 10.1080/1354750x.2016.1203999] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
OBJECTIVE To investigate the relationship between plasma myostatin levels and right ventricle (RV) dysfunction (RVD) in acute exacerbation of chronic obstructive pulmonary disease (AECOPD). METHODS The study recruited 84 patients with AECOPD. Plasma myostatin was analyzed and tricuspid annular plane systolic excursion (TAPSE) < 16 mm was used as the main indicator for RVD. RESULTS Plasma myostatin levels were significantly higher in 47 patients with RVD than 37 ones without (P < 0.005). Multivariate regression analysis revealed that myostatin levels correlated significantly with TAPSE values and RV myocardial performance index (p < 0.001) among the study patients. CONCLUSION Plasma myostatin is a potential biomarker for improving diagnosis of RVD in AECOPD.
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Affiliation(s)
- Chun-Rong Ju
- a State Key Lab of the Respiratory Disease, Guangzhou Institute of Respiratory Disease , First Affiliated Hospital of Guangzhou Medical University , Guangdong , China
| | - Jian-Heng Zhang
- a State Key Lab of the Respiratory Disease, Guangzhou Institute of Respiratory Disease , First Affiliated Hospital of Guangzhou Medical University , Guangdong , China
| | - Miao Chen
- a State Key Lab of the Respiratory Disease, Guangzhou Institute of Respiratory Disease , First Affiliated Hospital of Guangzhou Medical University , Guangdong , China
| | - Rong-Chang Chen
- a State Key Lab of the Respiratory Disease, Guangzhou Institute of Respiratory Disease , First Affiliated Hospital of Guangzhou Medical University , Guangdong , China
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24
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Hoeper MM, Behr J, Held M, Grunig E, Vizza CD, Vonk-Noordegraaf A, Lange TJ, Claussen M, Grohé C, Klose H, Olsson KM, Zelniker T, Neurohr C, Distler O, Wirtz H, Opitz C, Huscher D, Pittrow D, Gibbs JSR. Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias. PLoS One 2015; 10:e0141911. [PMID: 26630396 PMCID: PMC4667900 DOI: 10.1371/journal.pone.0141911] [Citation(s) in RCA: 75] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2015] [Accepted: 10/14/2015] [Indexed: 01/04/2023] Open
Abstract
Background Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies. Methods We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP). Results Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP. Conclusions Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival. Trial Registration clinicaltrials.gov NCT01347216
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Affiliation(s)
- Marius M. Hoeper
- Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany
- * E-mail:
| | - Juergen Behr
- Department of Internal Medicine V, University of Munich, Munich, Germany
| | - Matthias Held
- Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany
| | | | - C. Dario Vizza
- Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome, Italy
| | - Anton Vonk-Noordegraaf
- Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands
| | - Tobias J. Lange
- Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany
| | | | - Christian Grohé
- Department of Respiratory Medicine, ELK Thorax Centre, Berlin, Germany
| | - Hans Klose
- University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany
| | - Karen M. Olsson
- Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany
| | - Thomas Zelniker
- Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg, Germany
| | - Claus Neurohr
- Department of Internal Medicine V, University of Munich, Munich, Germany
| | - Oliver Distler
- Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland
| | - Hubert Wirtz
- Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany
| | - Christian Opitz
- Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany
| | - Doerte Huscher
- Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany
| | - David Pittrow
- Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany
| | - J. Simon R. Gibbs
- Department of Cardiology, National Heart & Lung Institute; Imperial College London, London, United Kingdom
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Papathanasiou A, Nakos G. Why there is a need to discuss pulmonary hypertension other than pulmonary arterial hypertension? World J Crit Care Med 2015; 4:274-277. [PMID: 26557477 PMCID: PMC4631872 DOI: 10.5492/wjccm.v4.i4.274] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2015] [Revised: 08/11/2015] [Accepted: 09/16/2015] [Indexed: 02/06/2023] Open
Abstract
Pulmonary hypertension (PH) is a condition characterized by the elevation of the mean pulmonary artery pressure above 25 mmHg and the pulmonary vascular resistance above 3 wood units. Pulmonary arterial hypertension (PAH) is an uncommon condition with severe morbidity and mortality, needing early recognition and appropriate and specific treatment. PH is frequently associated with hypoxemia, mainly chronic obstructive pulmonary disease and DPLD and/or left heart diseases (LHD), mainly heart failure with reduced or preserved ejection fraction. Although in the majority of patients with PH the cause is not PAH, a significant number of published studies are still in regard to group I PH, leading to a logical assumption that PH due to other causes is not such an important issue. So, is there a reason to discuss PH other than PAH? Chronic lung diseases, mainly chronic obstructive lung disease and DPLD, are associated with a high incidence of PH which is linked to exercise limitations and a worse prognosis. Although pathophysiological studies suggest that specific PAH therapy may benefit such patients, the results presented from small studies in regard to the safety and effectiveness of the specific PAH therapy are discouraging. PH is a common complication of left heart disease and is related to disease severity, especially in patients with reduced ejection fraction. There are two types of PH related to LHD based on diastolic pressure difference (DPD, defined as diastolic pulmonary artery pressure - mean PAWP): Isolated post-capillary PH, defined as PAWP > 15 mmHg and DPD < 7 mmHg, and combined post-capillary PH and pre-capillary PH, defined as PAWP > 15 mmHg and DPD ≥ 7 mmHg. The potential use of PAH therapies in patients with PH related to left heart disease is based on a logical pathobiological rationale. In patients with heart failure, endothelial dysfunction has been proposed as a cause of PH and hence as a target for treatment, supported by the presence of increased endothelin-1 activity and impaired nitric oxide-dependent vasodilation. Unfortunately, so far, there is no evidence supporting the use of specific PAH therapies in patients with PH related to left heart disease. In conclusion, the presence of PH in patients with conditions other than PAH contributes to the severity of the disease, affecting the outcome and quality of life. The disappointing results regarding the effectiveness of specific PAH therapies in patients with chronic lung diseases and LHD underline the need for seeking new underlying mechanisms and thus novel therapies targeting PH due to left heart disease and/or lung diseases.
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Beghetti M, Schulze-Neick I, Berger RMF, Ivy DD, Bonnet D, Weintraub RG, Saji T, Yung D, Mallory GB, Geiger R, Berger JT, Barst RJ, Humpl T. Haemodynamic characterisation and heart catheterisation complications in children with pulmonary hypertension: Insights from the Global TOPP Registry (tracking outcomes and practice in paediatric pulmonary hypertension). Int J Cardiol 2015; 203:325-30. [PMID: 26583838 DOI: 10.1016/j.ijcard.2015.10.087] [Citation(s) in RCA: 53] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2015] [Revised: 09/04/2015] [Accepted: 10/12/2015] [Indexed: 02/01/2023]
Abstract
BACKGROUND The TOPP Registry has been designed to provide epidemiologic, diagnostic, clinical, and outcome data on children with pulmonary hypertension (PH) confirmed by heart catheterisation (HC). This study aims to identify important characteristics of the haemodynamic profile at diagnosis and HC complications of paediatric patients presenting with PH. METHODS AND RESULTS HC data sets underwent a blinded review for confirmation of PH (defined as mean pulmonary arterial pressure ≥ 25 mmHg, pulmonary capillary wedge pressure ≤ 12 mmHg and pulmonary vascular resistance index [PVRI] of >3 WU × m(2)). Of 568 patients enrolled, 472 who fulfilled the inclusion criteria and had sufficient data from HC were analysed. A total of 908 diagnostic and follow-up HCs were performed and complications occurred in 5.9% of all HCs including five (0.6%) deaths. General anaesthesia (GA) was used in 53%, and conscious sedation in 47%. Complications at diagnosis were more likely to occur if GA was used (p=0.04) and with higher functional class (p=0.02). Mean cardiac index (CI) was within normal limits at diagnosis when analysed for the entire group (3.7 L/min/m(2); 95% confidence interval 3.4-4.1), as was right atrial pressure despite a severely increased PVRI (16.6 WU × m(2,) 95% confidence interval 15.6-17.76). However, 24% of the patients had a CI of <2.5L/min/m(2) at diagnosis. A progressive increase in PVRI and decrease in CI was observed with age (p<0.001). CONCLUSION In TOPP, haemodynamic assessment was remarkable for preserved CI in the majority of patients despite severely elevated PVRI. HC-related complication incidence was 5.9%, and was associated with GA and higher functional class.
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Affiliation(s)
- M Beghetti
- Pediatric Cardiology, Department of the Child and Adolescents, Hôpital des Enfants, University of Geneva, Switzerland.
| | - I Schulze-Neick
- Cardiac Unit, Great Ormond Street Hospital for Children, London, UK
| | - R M F Berger
- Center for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Netherlands
| | - D D Ivy
- Pediatrics, University of Colorado School of Medicine, Aurora, USA
| | - D Bonnet
- M3C-Paediatric Cardiology, Université Paris Descartes, Necker Enfants Malades, AP-HP, Paris, France
| | - R G Weintraub
- Royal Children's Hospital, Murdoch Children's Research Institute, Melbourne, Australia
| | - T Saji
- Toho University Medical Center Omori Hospital, Tokyo, Japan
| | - D Yung
- Seattle Children's Hospital, University of Washington School of Medicine, Seattle, USA
| | - G B Mallory
- Texas Children's Hospital, Baylor College of Medicine, Houston, USA
| | - R Geiger
- Innsbruck Medical University, Pediatric Cardiology, Innsbruck, Austria
| | - J T Berger
- Children's National Medical Center, Pediatric Critical Care and Cardiology, WA, USA
| | - R J Barst
- Pediatrics, Columbia University, New York, USA
| | - T Humpl
- Cardiology and Critical Care, University of Toronto, Toronto, ON, Canada
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Lange TJ, Baron M, Seiler I, Arzt M, Pfeifer M. Outcome of patients with severe PH due to lung disease with and without targeted therapy. Cardiovasc Ther 2015; 32:202-8. [PMID: 24909193 DOI: 10.1111/1755-5922.12084] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Abstract
INTRODUCTION Pulmonary hypertension (PH) can occur in patients with lung disease and worsen prognosis. Endothelin receptor antagonists, phosphodiesterase 5-inhibitors, and prostacyclin analogs, referred to as targeted therapy, have not been shown in a limited number of controlled clinical studies to improve exercise capacity in these patients. Possibly targeted therapy could be of benefit in patients with severe PH due to lung disease, but this subgroup is not well studied. AIMS To analyze influence of PH severity and use of targeted therapy on exercise capacity and survival in patients with PH due to lung disease. METHODS Consecutive patients with PH (mean pulmonary artery pressure ≥25 mmHg at rest) due to lung disease diagnosed by right heart catheterization between 1/2005 and 9/2011 were retrospectively included. Severe PH was defined as mean pulmonary artery pressure ≥35 mmHg. Patients were followed until 4/2012 for exercise capacity, survival, and targeted therapy use. RESULTS Patients with severe PH (n = 40) received significantly more often targeted therapy compared to the 32 patients with less severe PH (65% vs. 25%, P = 0.001). Survival was not significantly different between these groups (P = 0.310). Patients on targeted therapy were older, more often female, and had worse hemodynamic impairment, but significantly higher estimated 1-, 3-, and 5-year survival rates compared to untreated patients (97%, 81%, and 75% vs. 83%, 54%, and 19%, respectively; P = 0.002). This effect was mainly driven by the patients with severe PH, in whom the survival advantage was statistically significant on subgroup analysis (HR 0.182, P = 0.002). Exercise capacity was not significantly altered in any patient group. CONCLUSION Patients with severe PH due to lung disease may have a survival benefit from targeted therapy compared to untreated patients with less severe PH. Prospective clinical trials utilizing targeted therapy and long-term endpoints are justified in this patient group.
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Affiliation(s)
- Tobias J Lange
- Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany
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Bandorski D, Erkapic D, Stempfl J, Höltgen R, Grünig E, Schmitt J, Chasan R, Grimminger J, Neumann T, Hamm CW, Seeger W, Ghofrani HA, Gall H. Ventricular tachycardias in patients with pulmonary hypertension: an underestimated prevalence? A prospective clinical study. Herzschrittmacherther Elektrophysiol 2015; 26:155-162. [PMID: 26031512 DOI: 10.1007/s00399-015-0364-8] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2015] [Accepted: 03/10/2015] [Indexed: 06/04/2023]
Abstract
BACKGROUND Sudden cardiac death (SCD) accounts for approximately 30 % in patients with pulmonary arterial hypertension (PAH). The exact circumference for SCD in this patient population is still unclear. Malignant cardiac arrhythmias are reported to be rarely present. There are no systematic data concerning long-term electrocardiographic (ECG) recording in patients with PAH. OBJECTIVES We sought to investigate the rate of potentially relevant arrhythmias in patients with pulmonary hypertension (PH). METHODS Consecutive patients without diagnosis of known cardiac arrhythmias followed in our outpatient clinic for PH were enrolled in the study. All patients underwent a 72-h Holter ECG. Clinical data, 6-min walk distance, laboratory values, and echocardiography were collected/performed. RESULTS Ninety-two consecutive patients (New York Heart Association class (NYHA) III/IV: 65.2 %/5.4 %, PH Group 1: 35.9 %, Group 3: 10.9 %, Group 4: 28.3 %, Group 5: 2.2 %) were investigated. Relevant arrhythmias were newly detected in 17 patients: non-sustained ventricular tachycardia (n = 12), intermittent second-degree heart block (n = 1), intermittent third-degree heart block (n= 3), and atrial flutter (n = 1). Echocardiographic systolic pulmonary pressure and diameter of the right heart were elevated in patients with relevant arrhythmias. Right heart catheterization revealed higher pulmonary vascular resistance (672 vs. 542 dyn · s · cm(-5), p = 0.247) and lower cardiac index (2.46 vs. 2.82 l/min/m(2), p = 0.184). CONCLUSIONS Ventricular tachycardias occur more often in PH patients than previously reported. However, the prognostic relevance of non-sustained ventricular tachycardias in this cohort remains unclear. As a large number of PH patients die from SCD, closer monitoring, e.g., using implantable event recorders, might be useful to identify patients at high risk.
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Affiliation(s)
- Dirk Bandorski
- University of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany,
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Behr J. The diagnosis and treatment of idiopathic pulmonary fibrosis. DEUTSCHES ARZTEBLATT INTERNATIONAL 2015; 110:875-81. [PMID: 24529303 DOI: 10.3238/arztebl.2013.0875] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/29/2013] [Revised: 10/10/2013] [Accepted: 10/10/2013] [Indexed: 01/12/2023]
Abstract
BACKGROUND Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial disease of the lung and has the worst prognosis of all such diseases, with a median survival time of three to four years. Its prevalence is 2-29 per 100,000 persons and its incidence approximately 10 per 100,000 persons per year, with an upward trend. METHOD Selective literature search in the EMBASE and PubMed databases for pertinent publications from 1996 to 2012, with special attention to randomized controlled trials. RESULTS IPF manifests itself clinically with exertional dyspnea, dry cough, and inspiratory crepitations (sclerosiphonia). The diagnosis is confirmed by the demonstration of a usual interstitial pneumonia (UIP) pattern in a high-resolution thin-slice CT (HRCT) of the lungs, or else histologically by lung biopsy, along with the exclusion of other causes such as asbestosis or connective tissue disease. In 15 randomized controlled therapeutic trials carried out since 2004, most of the drugs that were tested, including immune suppressants, were found to be ineffective against IPF or even harmful. Only pirfenidone lessens the annual reduction of pulmonary volume (FVC, forced expiratory vital capacity) and of the distance walked in 6 minutes by about 30%, with corresponding improvement of progression-free survival, but without any significant lessening of overall mortality (placebo, 10%; pirfenidone, 8%). Pirfenidone also commonly causes gastrointestinal and cutaneous side effects. The efficacy of N-acetyldysteine and nintedanib has not yet been definitively demonstrated. Lung transplantation is the only current treatment that enables long-term survival. CONCLUSION IPF has a worse prognosis than many types of cancer. Drugs can delay the progression of the disease but probably cannot bring it to a permanent standstill.
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Affiliation(s)
- Jürgen Behr
- Medizinische Klinik V, Klinikum der Universität München
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30
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Rosenkranz S. Pulmonary hypertension 2015: current definitions, terminology, and novel treatment options. Clin Res Cardiol 2014; 104:197-207. [PMID: 25479818 DOI: 10.1007/s00392-014-0765-4] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/21/2014] [Accepted: 09/26/2014] [Indexed: 12/12/2022]
Abstract
Pulmonary hypertension (PH) is a common phenomenon that may occur as a consequence of various diseases (e.g., heart failure, chronic lung diseases, and pulmonary embolism), as a distinct disease of the small pulmonary arterioles, or a combination of both. Independently from the origin, PH has important impact on patient´s symptoms and life expectancy. The establishment of an exact diagnosis and classification, as well as the understanding of the hemodynamic interrelations, provides the basis for often challenging treatment decisions. Recently, the 5th World Symposium on PH took place in Nice, France, where important standards and definitions were specified. Furthermore, the results of recent phase III trials have led to the approval of new targeted therapies. The most relevant developments including the rating of novel treatment options are summarized in this article.
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Affiliation(s)
- Stephan Rosenkranz
- Clinic III for Internal Medicine, Center for Molecular Medicine Cologne (CMMC), Cologne Cardiovascular Research Center (CCRC), Heart Center at the University of Cologne, Kerpener Str. 62, 50937, Cologne, Germany,
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Eberhardt R, Gerovasili V, Kontogianni K, Gompelmann D, Ehlken N, Herth FJF, Grünig E, Nagel C. Endoscopic lung volume reduction with endobronchial valves in patients with severe emphysema and established pulmonary hypertension. ACTA ACUST UNITED AC 2014; 89:41-8. [PMID: 25502235 DOI: 10.1159/000368369] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2014] [Accepted: 08/26/2014] [Indexed: 11/19/2022]
Abstract
BACKGROUND One of the most common forms of pulmonary hypertension (PH) is that associated with chronic obstructive pulmonary disease (COPD). So far, patients with severe emphysema and established PH have been excluded from endoscopic lung volume reduction (ELVR) therapy due to the risk of right heart decompensation. OBJECTIVE The aim of this pilot study was to evaluate the feasibility and efficacy of ELVR using one-way endobronchial valves (EBV) in this specific group of patients. METHODS We prospectively included 6 patients with COPD, severe heterogeneous emphysema, and established PH who underwent right heart catheterization and clinical assessments before and 90 days after ELVR with unilateral EBV placement. RESULTS This study was not powered to measure any statistical differences in endpoints. Ninety days after ELVR, the symptoms, lung function, and hemodynamics improved in 5 out of 6 patients (1 patient normalized and 1 slightly worsened). The mean hemodynamics improved from baseline to 90 days after ELVR as follows: mean pulmonary artery pressure, -2.5 ± 3.5 mm Hg; pulmonary arterial wedge pressure, -4.3 ± 8.3 mm Hg; cardiac index, +0.3 ± 0.6 l/min/m(2), and 6-min walk distance, +59 ± 99 m. ELVR was performed without PH-related complications in all patients. CONCLUSION To our knowledge, this is the first prospective, single-center pilot study to evaluate the feasibility and efficacy of ELVR in patients with established PH. ELVR was feasible and resulted in an improvement of clinical and hemodynamic parameters in 5 out of 6 patients. These results have to be further confirmed in larger-scale controlled studies.
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Affiliation(s)
- Ralf Eberhardt
- Department of Pulmonology and Respiratory Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
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Pulmonary hypertension and right heart dysfunction in chronic lung disease. BIOMED RESEARCH INTERNATIONAL 2014; 2014:739674. [PMID: 25165714 PMCID: PMC4140123 DOI: 10.1155/2014/739674] [Citation(s) in RCA: 64] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/18/2014] [Revised: 06/24/2014] [Accepted: 06/29/2014] [Indexed: 11/30/2022]
Abstract
Group 3 pulmonary hypertension (PH) is a common complication of chronic lung disease (CLD), including chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep-disordered breathing. Development of PH is associated with poor prognosis and may progress to right heart failure, however, in the majority of the patients with CLD, PH is mild to moderate and only a small number of patients develop severe PH. The pathophysiology of PH in CLD is multifactorial and includes hypoxic pulmonary vasoconstriction, pulmonary vascular remodeling, small vessel destruction, and fibrosis. The effects of PH on the right ventricle (RV) range between early RV remodeling, hypertrophy, dilatation, and eventual failure with associated increased mortality. The golden standard for diagnosis of PH is right heart catheterization, however, evidence of PH can be appreciated on clinical examination, serology, radiological imaging, and Doppler echocardiography. Treatment of PH in CLD focuses on management of the underlying lung disorder and hypoxia. There is, however, limited evidence to suggest that PH-specific vasodilators such as phosphodiesterase-type 5 inhibitors, endothelin receptor antagonists, and prostanoids may have a role in the treatment of patients with CLD and moderate-to-severe PH.
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Iyer AS, Wells JM, Vishin S, Bhatt SP, Wille KM, Dransfield MT. CT scan-measured pulmonary artery to aorta ratio and echocardiography for detecting pulmonary hypertension in severe COPD. Chest 2014; 145:824-832. [PMID: 24114440 DOI: 10.1378/chest.13-1422] [Citation(s) in RCA: 143] [Impact Index Per Article: 13.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023] Open
Abstract
BACKGROUND COPD is associated with significant morbidity primarily driven by acute exacerbations. Relative pulmonary artery (PA) enlargement, defined as a PA to ascending aorta (A) diameter ratio greater than one (PA:A>1) identifies patients at increased risk for exacerbations. However, little is known about the correlation between PA:A, echocardiography, and invasive hemodynamics in COPD. METHODS A retrospective observational study of patients with severe COPD being evaluated for lung transplantation at a single center between 2007 and 2011 was conducted. Clinical characteristics, CT scans, echocardiograms, and right-sided heart catheterizations were reviewed. The PA diameter at the bifurcation and A diameter from the same CT image were measured. Linear and logistic regression were used to examine the relationships between PA:A ratio by CT scan and PA systolic pressure (PASP) by echocardiogram with invasive hemodynamics. Receiver operating characteristic analysis assessed the usefulness of the PA:A ratio and PASP in predicting resting pulmonary hypertension (PH) (mean pulmonary artery pressure [mPAP]>25 mm Hg). RESULTS Sixty patients with a mean predicted FEV1 of 27%±12% were evaluated. CT scan-measured PA:A correlated linearly with mPAP after adjustment for multiple covariates (r=0.30, P=.03), a finding not observed with PASP. In a multivariate logistic model, mPAP was independently associated with PA:A>1 (OR, 1.44; 95% CI, 1.02-2.04; P=.04). PA:A>1 was 73% sensitive and 84% specific for identifying patients with resting PH (area under the curve, 0.83; 95% CI, 0.72-0.93; P<.001), whereas PASP was not useful. CONCLUSIONS A PA:A ratio>1 on CT scan outperforms echocardiography for diagnosing resting PH in patients with severe COPD.
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Affiliation(s)
- Anand S Iyer
- Department of Internal Medicine, University of Alabama at Birmingham
| | - J Michael Wells
- Department of Internal Medicine, University of Alabama at Birmingham; Division of Pulmonary, Allergy, and Critical Care, Department of Medicine and University of Alabama at Birmingham Lung Health Center, University of Alabama at Birmingham; Birmingham Veterans Affairs Medical Center, Birmingham, AL.
| | - Sonia Vishin
- Department of Internal Medicine, University of Alabama at Birmingham; Division of Pulmonary, Allergy, and Critical Care, Department of Medicine and University of Alabama at Birmingham Lung Health Center, University of Alabama at Birmingham
| | - Surya P Bhatt
- Department of Internal Medicine, University of Alabama at Birmingham
| | - Keith M Wille
- Department of Internal Medicine, University of Alabama at Birmingham; Division of Pulmonary, Allergy, and Critical Care, Department of Medicine and University of Alabama at Birmingham Lung Health Center, University of Alabama at Birmingham
| | - Mark T Dransfield
- Department of Internal Medicine, University of Alabama at Birmingham; Division of Pulmonary, Allergy, and Critical Care, Department of Medicine and University of Alabama at Birmingham Lung Health Center, University of Alabama at Birmingham; Birmingham Veterans Affairs Medical Center, Birmingham, AL
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Zimmermann GS, von Wulffen W, Huppmann P, Meis T, Ihle F, Geiseler J, Leuchte HH, Tufman A, Behr J, Neurohr C. Haemodynamic changes in pulmonary hypertension in patients with interstitial lung disease treated with PDE-5 inhibitors. Respirology 2014; 19:700-6. [DOI: 10.1111/resp.12294] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2013] [Revised: 12/11/2013] [Accepted: 01/29/2014] [Indexed: 01/30/2023]
Affiliation(s)
- Gregor S. Zimmermann
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
| | - Werner von Wulffen
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
| | - Patrick Huppmann
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
| | - Tobias Meis
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
| | - Franziska Ihle
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
| | | | - Hanno H. Leuchte
- Department of Internal Medicine II; Neuwittelsbach Hospital; Munich
| | - Amanda Tufman
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
| | - Juergen Behr
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
- AsklepiosKlinik; Gauting Germany
| | - Claus Neurohr
- Department of Internal Medicine V; Comprehensive Pneumology Center; Member of the German Center for Lung Research; University of Munich
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Rosenkranz S. [Cor pulmonale and pulmonary hypertension. Update after the world conference in Nice]. Herz 2014; 39:58-65. [PMID: 24638159 DOI: 10.1007/s00059-014-4058-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Pulmonary hypertension (PH) is a common phenomenon which may occur as a consequence of various diseases (e.g. heart failure, chronic lung diseases and pulmonary embolism), as a distinct disease of the small pulmonary arterioles or a combination of both. Independent from the origin, PH has an important impact on patient symptoms and prognosis. Establishment of an exact diagnosis and classification as well as an understanding of the hemodynamic interrelationships provide the basis for often challenging treatment decisions. Recently, the fifth World Symposium on PH took place in Nice, France, where important standards and definitions were specified. The most relevant results including the rating of novel treatment options are summarized in this article.
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Affiliation(s)
- S Rosenkranz
- Klinik III für Innere Medizin, Zentrum für Molekulare Medizin Köln (ZMMK), Cologne Cardiovascular Research Center (CCRC), Klinikum der Universität zu Köln, Kerpener Str. 62, 50937, Köln, Deutschland,
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37
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Abstract
Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation. The task force encompassing the authors of this article provided criteria for this discrimination and suggested using the following definitions for group 3 patients, as exemplified for COPD, IPF, and CPFE: COPD/IPF/CPFE without PH (mean pulmonary artery pressure [mPAP] <25 mm Hg); COPD/IPF/CPFE with PH (mPAP ≥25 mm Hg); PH-COPD, PH-IPF, and PH-CPFE); COPD/IPF/CPFE with severe PH (mPAP ≥35 mm Hg or mPAP ≥25 mm Hg with low cardiac index [CI <2.0 l/min/m(2)]; severe PH-COPD, severe PH-IPF, and severe PH-CPFE). The "severe PH group" includes only a minority of chronic lung disease patients who are suspected of having strong general vascular abnormalities (remodeling) accompanying the parenchymal disease and with evidence of an exhausted circulatory reserve rather than an exhausted ventilatory reserve underlying the limitation of exercise capacity. Exertional dyspnea disproportionate to pulmonary function tests, low carbon monoxide diffusion capacity, and rapid decline of arterial oxygenation upon exercise are typical clinical features of this subgroup with poor prognosis. Studies evaluating the effect of pulmonary arterial hypertension drugs currently not approved for group 3 PH patients should focus on this severe PH group, and for the time being, these patients should be transferred to expert centers for individualized patient care.
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Kauppert CA, Dvorak I, Kollert F, Heinemann F, Jörres RA, Pfeifer M, Budweiser S. Pulmonary hypertension in obesity-hypoventilation syndrome. Respir Med 2013; 107:2061-70. [DOI: 10.1016/j.rmed.2013.09.017] [Citation(s) in RCA: 64] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2013] [Revised: 09/10/2013] [Accepted: 09/19/2013] [Indexed: 12/20/2022]
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Minai OA, Fessler H, Stoller JK, Criner GJ, Scharf SM, Meli Y, Nutter B, DeCamp MM. Clinical characteristics and prediction of pulmonary hypertension in severe emphysema. Respir Med 2013; 108:482-90. [PMID: 24290900 DOI: 10.1016/j.rmed.2013.11.006] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2013] [Revised: 10/28/2013] [Accepted: 11/08/2013] [Indexed: 10/26/2022]
Abstract
BACKGROUND We explored the prevalence, clinical and physiologic correlates of pulmonary hypertension (PH), and screening strategies in patients with severe emphysema evaluated for the National Emphysema Treatment Trial (NETT). METHODS Patients undergoing Doppler echocardiography (DE) and right heart catheterization were included. Patients with mean pulmonary arterial pressure ≥ 25 mmHg (PH Group) were compared to the remainder (non-PH Group). RESULTS Of 797 patients, 302 (38%) had PH and 18 (2.2%) had severe PH. Compared to the non-PH Group, patients with PH had lower % predicted FEV1 (p < 0.001), % predicted diffusion capacity for carbon monoxide (p = 0.006), and resting room air PaO2 (p < 0.001). By multivariate analysis, elevated right ventricular systolic pressure, reduced resting room air PaO2, reduced post-bronchodilator % predicted FEV1, and enlarged pulmonary arteries on computed tomographic scan were the best predictors of PH. A strategy using % predicted FEV1, % predicted DLCO, PaO2, and RVSP was predictive of the presence of pre-capillary PH and was highly predictive of its absence. CONCLUSIONS Mildly elevated pulmonary artery pressures are found in a significant proportion of patients with severe emphysema. However, severe PH is uncommon in the absence of co-morbidities. Simple non-invasive tests may be helpful in screening patients for pre-capillary PH in severe emphysema but none is reliably predictive of its presence.
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Affiliation(s)
- Omar A Minai
- Department of Pulmonary, Allergy, and Critical Care, Cleveland Clinic, USA.
| | - Henry Fessler
- Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, USA.
| | - James K Stoller
- Department of Pulmonary, Allergy, and Critical Care, Cleveland Clinic, USA.
| | - Gerard J Criner
- Division of Pulmonary and Critical Care Medicine, Temple University, USA.
| | - Steven M Scharf
- Division of Pulmonary and Critical Care Medicine, University of Maryland, USA.
| | - Yvonne Meli
- Department of Pulmonary, Allergy, and Critical Care, Cleveland Clinic, USA.
| | - Benjamin Nutter
- Department of Quantitative Health Sciences, Cleveland Clinic, USA.
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Ganga HV, Nair SU, Puppala VK, Miller WL. Risk of new-onset atrial fibrillation in elderly patients with the overlap syndrome: a retrospective cohort study. J Geriatr Cardiol 2013; 10:129-34. [PMID: 23888171 PMCID: PMC3708051 DOI: 10.3969/j.issn.1671-5411.2013.02.001] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2013] [Revised: 04/02/2013] [Accepted: 04/22/2013] [Indexed: 12/31/2022] Open
Abstract
OBJECTIVE Co-existence of obstructive sleep apnea (OSA) and chronic obstructive pulmonary disease (COPD) is referred to as overlap syndrome. Overlap patients have greater degree of hypoxia and pulmonary hypertension than patients with OSA or COPD alone. Studies showed that elderly patients with OSA alone do not have increased risk of atrial fibrillation (AF) but it is not known if overlap patients have higher risk of AF. To determine whether elderly patients with overlap syndrome have an increased risk of AF. METHODS In this single center, community-based retrospective cohort analysis, data were collected on 2,873 patients > 65 years of age without AF, presenting in the year 2006. Patients were divided into OSA group (n = 60), COPD group (n = 416), overlap syndrome group (n = 28) and group with no OSA or COPD (n = 2369). The primary endpoint was incidence of new-onset AF over the following two years. Logistic regression was performed to adjust for heart failure (HF), coronary artery disease, hypertension (HTN), cerebrovascular disease, cardiac valve disorders, diabetes mellitus, hyperlipidemia, chronic kidney disease (CKD) and obesity. RESULTS The incidence of AF was 10% in COPD group, 6% in OSA group and 21% in overlap syndrome group (P < 0.05). After adjusting for age, sex, HF, CKD, and HTN, patients with overlap syndrome demonstrated a significant association with new-onset AF (OR = 3.66, P = 0.007). HF, CKD and HTN were also significantly associated with new-onset AF (P < 0.05). CONCLUSION Among elderly patients, the presence of overlap syndrome is associated with a marked increase in risk of new-onset AF as compared to the presence of OSA or COPD alone.
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Affiliation(s)
- Harsha V Ganga
- The Henry Low Heart Center, Hartford Hospital, B-1010, 80 Seymour Street, Hartford, CT 06102, USA
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Four cases with group 3 out-of-proportion pulmonary hypertension with a favorable response to vasodilators. Respir Med Case Rep 2013; 9:4-7. [PMID: 26029619 PMCID: PMC3949553 DOI: 10.1016/j.rmcr.2013.03.002] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2013] [Accepted: 03/08/2013] [Indexed: 11/20/2022] Open
Abstract
Some patients with group 3 pulmonary hypertension (PH) (PH due to lung disease and/or hypoxia) exhibit disproportionately advanced or “out-of-proportion” PH. In the present case series, we document four consecutive patients with progressive out-of-proportion group 3 PH. All patients exhibited progressive dyspnea or peripheral edema and were treated by pulmonary artery hypertension (PAH)-specific vasodilator(s). At the follow-up assessment 3–4 months later, symptoms/signs and pulmonary hemodynamic measurements improved in all four patients (45 ± 8% decrease in pulmonary vascular resistance). Pulmonary oxygenation deteriorated in one patient but improved or did not significantly change in the remaining three cases. Importantly, the background lung parenchymal disease (early-onset chronic obstructive pulmonary disease, rheumatoid arthritis-associated interstitial pneumonia, and combined pulmonary fibrosis and emphysema) was stable upon progression of the right heart failure symptoms/signs, and also during the 3–4-month follow-up period in all cases. We herein describe the clinical features of the four cases and discuss the potential benefits and risks of PAH-specific treatment in this emerging population.
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Abstract
Pulmonary hypertension (PH) is found in many clinical conditions and is associated with increased morbidity and mortality. Pulmonary arterial hypertension (PAH) is a clinical condition characterized by precapillary PH without causes such as lung disease, chronic thromboembolic PH, or other rare conditions. Evaluating a patient with suspected PH requires a series of investigations intended to confirm the diagnosis, determine the clinical PH group (and, in the case of PAH group 1, the specific etiology), and evaluate the functional and hemodynamic impairment. The workup should identify the risk factors for PH (e.g., left heart disease, lung diseases associated with alveolar hypoxia, and chronic thromboembolism) versus the conditions associated with PAH group 1 (e.g., scleroderma, human immunodeficiency virus, anorexigen use, liver disease). A detailed algorithm is presented to help physicians determine the appropriate PH category. Because the presence of one condition associated with PH does not exclude another etiologies, clinicians are strongly encouraged to follow the entire algorithm. Discussions and case studies are presented describing the differentiation of PAH group 1 from PH group 2 and PAH group 1 from PH group 3; diagnosing PH group 4; determining the long-term calcium channel blocker response in those with idiopathic PAH; and determining the severity of PH.
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Abstract
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with a variable natural history. Pulmonary hypertension (PH) is frequently found in patients with IPF and is associated with an almost 3-fold increase in the risk of death. Pulmonary hypoxic vasoconstriction plays an important role in the pathogenesis of PH in IPF (PH-IPF), although it has become clear that it is not the only mechanism involved. While invasive right heart catheterization is the gold standard modality of hemodynamic assessment, there has been increasing interest in noninvasive testing, such as Doppler echocardiogram, as complementary methods of assessing right ventricular function in these patients. While the expanding array of pharmacologic options for the treatment of pulmonary arterial hypertension has engendered increased interest in the application of these therapies for PH-IPF, supportive evidence for benefit is lacking.
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Pulmonary hypertension in parenchymal lung disease. Pulm Med 2012; 2012:684781. [PMID: 23094153 PMCID: PMC3474989 DOI: 10.1155/2012/684781] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2012] [Accepted: 09/07/2012] [Indexed: 01/23/2023] Open
Abstract
Idiopathic pulmonary arterial hypertension (IPAH) has been extensively investigated, although it represents a less common form of the pulmonary hypertension (PH) family, as shown by international registries. Interestingly, in types of PH that are encountered in parenchymal lung diseases such as interstitial lung diseases (ILDs), chronic obstructive pulmonary disease (COPD), and many other diffuse parenchymal lung diseases, some of which are very common, the available data is limited. In this paper, we try to browse in the latest available data regarding the occurrence, pathogenesis, and treatment of PH in chronic parenchymal lung diseases.
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