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Canadian Respiratory Journal
Volume 2017 (2017), Article ID 1430350, 12 pages
Review Article

Pulmonary Hypertension Associated with Idiopathic Pulmonary Fibrosis: Current and Future Perspectives

1Department of Biochemistry and Molecular Biology, University of Texas Medical School at Houston, Houston, TX 77030, USA
2Methodist DeBakey Heart and Vascular Center, The Methodist Hospital, Houston, TX 77030, USA
3Methodist J.C. Walter Jr. Transplant Center, The Methodist Hospital, Houston, TX 77030, USA

Correspondence should be addressed to Harry Karmouty-Quintana; ude.cmt.htu@ytuomrak.yrrah

Received 18 November 2016; Accepted 19 January 2017; Published 13 February 2017

Academic Editor: Elie El Agha

Copyright © 2017 Scott D. Collum et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.