Background: Idiopathic pulmonary fibrosis (IPF) is a disease with very high mortality. Objective: We sought to characterize serial changes in pulmonary artery pressures (PAP) in patients with advanced IPF who survive to transplant. Methods: Retrospective analysis of IPF patients comparing mean PAP at the time of initial evaluation for transplan- tation (mPAPbaseline) with mPAP at the time of transplant (mPAPfollow-up). The measurements were correlated with New York Heart Association (NYHA) functional class and oxygen requirements. Results: The final cohort consisted of 44 patients with serial right heart catheterization data. The mean mPAPbaseline and mPAPfollow-up were 22.5 and 32.7 mm Hg, respectively. 38.6% (17/44) of the patients had pulmonary hypertension (PH) at baseline. The majority of the non-PH patients developed PH during the serial time interval with a subsequent incidence of 77.8%. At the time of transplant, 86.4% of the patients had PH. There was a significant association between transplant NYHA class, severity of PH and oxygen requirements. Transplant NYHA class IV patients had a higher rate of mPAP change. The severity of PH at the time of transplant did not affect transplant outcomes. Conclusion: PH is common and progressive in patients with advanced IPF who are transplant candidates. Serial change and severity of PAP elevations have a significant association with oxygen requirements and functional status, but not transplant outcomes. Whether or not progressive PH has a significant impact on outcomes without transplantation requires further study.

Keywords:
Pulmonary fibrosis, Pulmonary hypertension, Lung transplantation, Pulmonary function tests
1.
Bjoraker JA, Ryu JH, Edwin MK, et al: Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157:199–203.
2.
Latsi PI, du Bois RM, Nicholson AG, et al: Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003;168:531–537.
3.
Flaherty KR, Mumford JA, Murray S, et al: Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003;168:543–548.
4.
Collard HR, King TE, Bartelson BB, et al: Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003;168:538–542.
5.
King TE, Safrin S, Starko KM, et al: Analyses of efficacy end points in a controlled trial of interferon-gamma1b for idiopathic pulmonary fibrosis. Chest 2005;127:171–177.
6.
Martinez FJ, Safrin S, Weycker D, et al: IPF study group: the clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005;142:963–967.
7.
Zisman DA, Lynch JP, Strieter RM: Pulmonary arterial hypertension (PAH) is common in patients with idiopathic pulmonary fibrosis referred for lung transplantation. Am J Respir Crit Care Med 2005;2:A123.
8.
Gagermeier J, Dauber J, Yousem S, et al: Prevalence of secondary pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005;2:A205.
9.
Nagao T, Nagai S, Hiramoto Y, et al: Serial evaluation of high-resolution computed tomography findings in patients with idiopathic pulmonary fibrosis in usual interstitial pneumonia. Respiration 2002;69:413– 419.
10.
King TE, Costabel U, Cordier JF, et al: Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med 2000;161:646–664.
11.
Nathan SD, Shlobin OA, Ahmad S, et al: Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest 2007;131:657–663.
12.
Magro CM, Allen J, Pope-Harmon A, et al: The role of microvascular injury in the evolution of idiopathic pulmonary fibrosis. Am J Clin Pathol 2003;119:556–567.
13.
Nathan SD, Barnett SD, Urban BA, et al: Pulmonary emboli in IPF transplant recipients. Chest 2003;123:1758–1763.
14.
Ebina M, Shimizukawa M, Shibata N, et al: Heterogenous increase in CD34-positive alveolar capillaries in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2004;169:1203–1208.
15.
Cosgrove GP, Brown KK, Schiemann WP, et al: Pigment epithelial-derived factor in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2004;170:242–251.
16.
Renzoni EA, Walsh DA, Salmon M, et al: Interstitial vascularity in fibrosing alveolitis. Am J Respir Crit Care Med 2003;167:438–443.
17.
Strieter RM, Starko KM, Enelow RI, et al: Idiopathic pulmonary fibrosis biomarkers study group: effects of interferon-gamma 1b on biomarker expression in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2004;170:133–140.
18.
Burdick MD, Murray LA, Keane MP, et al: CXCL11 attenuates bleomycin-induced pulmonary fibrosis via inhibition of vascular remodeling. Am J Respir Crit Care Med 2005;171:261–268.
19.
Simler NR, Brenchley PE, Horrocks AW, et al: Angiogenic cytokines in patients with idiopathic interstitial pneumonia. Thorax 2004;59:581–585.
20.
Gagermeier J, Dauber J, Yousem S, et al: Abnormal vascular phenotypes in patients with idiopathic pulmonary fibrosis and secondary pulmonary hypertension. Chest 2005;128:601S.
21.
Barst RJ, Rubin LJ, Long WA, et al: The primary pulmonary hypertension study group. A comparison of continuous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334:296–301.
22.
Barst RJ, Langleben D, Frost A, et al; STRIDE-1 Study Group: Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;169:441–447.
23.
Simonneau G, Barst RJ, Galie N, et al; Treprostinil Study Group: Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2002;165:800–804.
24.
Kessler R, Faller M, Weitzenblum E, et al: ‘Natural history’ of pulmonary hypertension in a series of 131 patients with chronic obstructive lung disease. Am J Respir Crit Care Med 2001;164:219–224.
25.
Leuchte HH, Neurohr C, Baumgartner R, et al: Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med 2004;170:360–365.
26.
Arcasoy SM, Christie JD, Ferrari VA, et al: Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003;167:735–740.
27.
Olschewski H, Ghofrani H, Walmrath D, et al: Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir and Crit Care Med 1999;160:600–607.
28.
Ghofrani HA, Wiedemann R, Rose F, et al: Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomized controlled trial. Lancet 2002;360:895–900.
29.
Bovill JS, Boer F: Opoids in cardiac anesthesia; in Kaplan JA (ed): Cardiac Anesthesia, ed 3. Philadelphia, Saunders, 1993, pp 467–511.
30.
Gilman AK, Rall TW, Nies AS, et al: The Pharmacological Basis of Therapeutics, ed 8. New York, Macmillan, 1990.
© 2008 S. Karger AG, Basel
2008
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.