{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T15:00:27Z","timestamp":1784818827237,"version":"3.55.0"},"reference-count":38,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T00:00:00Z","timestamp":1718323200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Objectives: Pulmonary hypertension (PH) often accompanies chronic lung diseases. Several chronic lung diseases with PH portends unfavorable outcomes. We investigated which variables in this cohort of patients with chronic lung disease and PH predicts mortality. Methods: This is a retrospective analysis of patients with chronic lung disease and PH at a single tertiary, academic center. The underlying lung disease included were COPD, IPF, other fibrotic ILD, non-fibrotic ILD, fibrotic sarcoidosis, and CPFE. All patients had right heart catheterization diagnostic of PH as well as pulmonary function testing data including 6 min walk testing. Univariable and multivariate Cox regression was performed to identify variables associated with mortality. Results: We identified 793 patients with chronic lung disease and PH. In total, 144 patients died prior to potential lung transplant. In multivariable Cox regression IPF, other fibrotic ILD, non-fibrotic ILD, and CPFE were significantly associated with an increased risk of mortality. Severe PH (PVR &gt; 5 WU), FEV1 &lt; 30% predicted, FVC &lt; 40% predicted, 6 min walk distance &lt; 150 m were also significantly associated with an increased risk of mortality. Conclusions: Carrying a diagnosis of IPF, CPFE, fibrotic ILD, or non-fibrotic ILD with PH has an increased risk of mortality as compared to COPD with PH. Hemodynamic, PVR &gt; 5 WU, 6 min walk test less than 150 m, as well as spirometric data including FEV1 &lt; 30% and FVC &lt; 40% predicted were independently associated with an increased risk of death.<\/jats:p>","DOI":"10.3390\/jcm13123472","type":"journal-article","created":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T08:02:26Z","timestamp":1718352146000},"page":"3472","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Predictors of Mortality in Pulmonary Hypertension-Associated Chronic Lung Disease"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1821-6566","authenticated-orcid":false,"given":"Jay","family":"Pescatore","sequence":"first","affiliation":[{"name":"Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University Hospital, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Bittner","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University Hospital, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilbert","family":"D\u2019Alonzo","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University Hospital, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila","family":"Weaver","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University Hospital, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0275-7416","authenticated-orcid":false,"given":"Shameek","family":"Gayen","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University Hospital, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1801914","DOI":"10.1183\/13993003.01914-2018","article-title":"Pulmonary hypertension in chronic lung disease and hypoxia","volume":"53","author":"Nathan","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1111\/resp.12530","article-title":"Multi-institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases","volume":"20","author":"Tanabe","year":"2015","journal-title":"Respirology"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Alhamad, E.H., Cal, J.G., Alrajhi, N.N., and Alharbi, W.M. (2020). Predictors of Mortality in Patients with Interstitial Lung Disease-Associated Pulmonary Hypertension. J. Clin. Med., 9.","DOI":"10.3390\/jcm9123828"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2100944","DOI":"10.1183\/13993003.00944-2021","article-title":"Elevated pulmonary vascular resistance predicts mortality in COPD patients","volume":"58","author":"Zeder","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e12297","DOI":"10.1002\/pul2.12297","article-title":"Pulmonary hemodynamics and transplant-free survival in sarcoidosis-associated pulmonary hypertension: Results from an international registry","volume":"13","author":"Gayen","year":"2023","journal-title":"Pulm. Circ."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2101483","DOI":"10.1183\/13993003.01483-2021","article-title":"Pulmonary vascular resistance predicts mortality in patients with pulmonary hypertension associated with interstitial lung disease: Results from the COMPERA registry","volume":"58","author":"Olsson","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1016\/j.chest.2023.08.013","article-title":"Impact of Systemic Sclerosis-Associated Interstitial Lung Disease With and Without Pulmonary Hypertension on Survival","volume":"165","author":"Moinzadeh","year":"2024","journal-title":"Chest"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"D109","DOI":"10.1016\/j.jacc.2013.10.036","article-title":"Pulmonary Hypertension in Chronic Lung Diseases","volume":"62","author":"Seeger","year":"2013","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1183\/09031936.00079512","article-title":"Pulmonary hypertension in COPD: Results from the ASPIRE registry","volume":"41","author":"Hurdman","year":"2013","journal-title":"Eur. Respir. J."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1016\/j.chest.2021.02.012","article-title":"Pulmonary Hypertension in Patients with COPD","volume":"160","author":"Vizza","year":"2021","journal-title":"Chest"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1164\/rccm.201801-0095PP","article-title":"Pulmonary Vascular Involvement in Chronic Obstructive Pulmonary Disease. Is There a Pulmonary Vascular Phenotype?","volume":"198","author":"Kovacs","year":"2018","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1136\/thx.36.10.752","article-title":"Prognostic value of pulmonary artery pressure in chronic obstructive pulmonary disease","volume":"36","author":"Weitzenblum","year":"1981","journal-title":"Thorax"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1378\/chest.129.3.746","article-title":"Prevalence and Outcomes of Pulmonary Arterial Hypertension in Advanced Idiopathic Pulmonary Fibrosis","volume":"129","author":"Lettieri","year":"2006","journal-title":"Chest"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Ni, H., Wei, Y., Yang, L., and Wang, Q. (2023). An increased risk of pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema: A meta-analysis. BMC Pulm. Med., 23.","DOI":"10.1186\/s12890-023-02425-4"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1186\/s12931-024-02674-9","article-title":"Elevated pulmonary vascular resistance is associated with increased lung transplant waitlist mortality among patients with chronic obstructive pulmonary disease and pulmonary hypertension: A retrospective cohort analysis","volume":"25","author":"Gayen","year":"2024","journal-title":"Respir. Res."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1183\/09031936.00038709","article-title":"Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome","volume":"35","author":"Cottin","year":"2010","journal-title":"Eur. Respir. J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"Galie","year":"2022","journal-title":"Eur. Heart J."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"37","author":"Humbert","year":"2016","journal-title":"Eur. Heart J."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1164\/rccm.200703-456SO","article-title":"Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Pulmonary Disease","volume":"176","author":"Rabe","year":"2007","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_20","first-page":"1","article-title":"Pulmonary hypertension in chronic lung diseases: Comparison to other pulmonary hypertension groups","volume":"8","author":"Chebib","year":"2018","journal-title":"Pulm. Circ."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1378\/chest.08-2306","article-title":"Idiopathic Pulmonary Fibrosis and Emphysema","volume":"136","author":"Carrillo","year":"2009","journal-title":"Chest"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2794","DOI":"10.1183\/09031936.97.10122794","article-title":"Co-morbidity contributes to predict mortality of patients with chronic obstructive pulmonary disease","volume":"10","author":"Incalzi","year":"1997","journal-title":"Eur. Respir. J."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1056\/NEJMoa021322","article-title":"The Body-Mass Index, Airflow Obstruction, Dyspnea, and Exercise Capacity Index in Chronic Obstructive Pulmonary Disease","volume":"350","author":"Celli","year":"2004","journal-title":"N. Engl. J. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1164\/rccm.201011-1790OC","article-title":"Ascertainment of Individual Risk of Mortality for Patients with Idiopathic Pulmonary Fibrosis","volume":"184","author":"Weycker","year":"2011","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1186\/s12931-019-0980-7","article-title":"Interstitial lung disease associated with systemic sclerosis (SSc-ILD)","volume":"20","author":"Cottin","year":"2019","journal-title":"Respir. Res."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"2002718","DOI":"10.1183\/13993003.02718-2020","article-title":"Progressive fibrosing interstitial lung disease: A clinical cohort (the PROGRESS study)","volume":"57","author":"Nasser","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"2071","DOI":"10.1056\/NEJMoa1402584","article-title":"Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis","volume":"370","author":"Richeldi","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"163","DOI":"10.5603\/CJ.a2021.0159","article-title":"Pulmonary arterial hypertension and right ventricular systolic dysfunction in COVID-19 survivors","volume":"29","author":"Rossi","year":"2022","journal-title":"Cardiol. J."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1183\/09031936.00131813","article-title":"6-minute walk distance is an independent predictor of mortality in patients with idiopathic pulmonary fibrosis","volume":"43","author":"Albera","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"106801","DOI":"10.1016\/j.rmed.2022.106801","article-title":"The six-minute walk test in sarcoidosis associated pulmonary hypertension: Results from an international registry","volume":"196","author":"Gupta","year":"2022","journal-title":"Respir. Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1778","DOI":"10.1378\/chest.07-2050","article-title":"The 6-Min Walk Distance, Peak Oxygen Uptake, and Mortality in COPD","volume":"132","author":"Cote","year":"2007","journal-title":"Chest"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1164\/ajrccm.161.2.9906015","article-title":"Clinical Correlates and Prognostic Significance of Six-minute Walk Test in Patients with Primary Pulmonary Hypertension","volume":"161","author":"Miyamoto","year":"2000","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1183\/09031936.01.17406470","article-title":"Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension","volume":"17","author":"Paciocco","year":"2001","journal-title":"Eur. Respir. J."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1056\/NEJMoa2008470","article-title":"Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease","volume":"384","author":"Waxman","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1901747","DOI":"10.1183\/13993003.01747-2019","article-title":"Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: Results from an international registry","volume":"55","author":"Shlobin","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1186\/s40001-023-01130-6","article-title":"FVC\/DLCO identifies pulmonary hypertension and predicts 5-year all-cause mortality in patients with COPD","volume":"28","author":"Li","year":"2023","journal-title":"Eur. J. Med. Res."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1016\/j.healun.2020.06.014","article-title":"Risk assessment in severe pulmonary hypertension due to interstitial lung disease","volume":"39","author":"Yogeswaran","year":"2020","journal-title":"J. Heart Lung Transplant."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"A5862","DOI":"10.1016\/j.chest.2023.07.3779","article-title":"Derivation of a simple risk calculator for predicting clinical worsening in patients with pulmonary hypertension due to interstitial lung disease","volume":"164","author":"Bag","year":"2023","journal-title":"Chest"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/12\/3472\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:58:33Z","timestamp":1760108313000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/12\/3472"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,14]]},"references-count":38,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2024,6]]}},"alternative-id":["jcm13123472"],"URL":"https:\/\/doi.org\/10.3390\/jcm13123472","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,14]]}}}