Pulmonary hypertension in idiopathic pulmonary fibrosis: A review

Department of Thoracic Medicine Royal Brompton Hospital, London, UK.
Sarcoidosis, vasculitis, and diffuse lung diseases: official journal of WASOG / World Association of Sarcoidosis and Other Granulomatous Disorders (Impact Factor: 1.17). 07/2009; 26(1):7-19.
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Pulmonary hypertension (PH) is a common in patients with idiopathic pulmonary fibrosis (IPF) referred for transplantation. When present, PH is associated with increased mortality, and may explain the deterioration of some patients with preserved pulmonary function. PH in IPF may develop as a consequence of, or disproportionate to the underlying fibrotic lung disease. The distinction between these two 'stages' of PH is essential as there are key differences in their pathophysiology, identification, and potential treatment options. Treatment advances in idiopathic pulmonary artery hypertension have focused attention on PH associated with underlying lung disease. We focus on pathogenetic mechanisms, identification of PH, and the potential for therapeutic intervention for PH in IPF. Although vascular ablation, and chronic hypoxia are both important in the aetiology of secondary PH, these mechanisms do not explain the development of disproportionate PH. In these patients, the early development of PH may be associated with increased fibrotic cell mediators, abnormal vasculature or response to hypoxia, seen in IPF. Nocturnal and exercise desaturation are common in IPF, and may precede and contribute to the development PH. Therapeutic options for PH in IPF are limited, and there have been no controlled trials. Successful therapeutic intervention in pulmonary arterial hypertension, has led to suggestions that therapeutic intervention with PH specific therapy may be useful. However, controlled trials are warranted before therapy can be recommended. In the design of such trials, the distinction between secondary and disproportionate PH is essential.

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Available from: Tamera J Corte
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    • "Rapid progression of PH was reported in latestage diffuse parenchymal lung disease (DPLD)/IPF patients [12]. In some studies, the prognosis of PH in lung fibrosis is not linked to the mPAP values but to pulmonary vascular resistance [13] or cardiac index (CI), with CI values < 2.4 L/min/m 2 correlated to survival of only a few months [14]. Assessment and definition of PH due to chronic lung diseases is a fundamental step in end-stage lung diseases (Group 3). "
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    ABSTRACT: Introduction: Single or bilateral lung transplantation is a therapeutic procedure for end-stage lung diseases. In particular, in cases of chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis, patients can be referred to the transplant center late and with important comorbilities. Pulmonary hypertension (PH) associated with lung diseases not only is an index of poor outcome but also is an indication for bilateral procedure. Methods: We conducted a retrospective observational study. We analyzed right heart catheterization in a consecutive series of patients who underwent lung transplantation from 2006 to 2014 for end-stage COPD and pulmonary fibrosis. Results: We included in the study 73 patients (35 with fibrosis and 38 with COPD); prevalence of PH was higher in the COPD group (84.3% vs 31.4%), and with worse hemodynamic parameters (mean pulmonary artery pressure [30.3 mm Hg vs 24.1 mm Hg]). The majority of COPD patients presented mild or moderate PH, and fibrosis patients showed normal pulmonary arterial pressures. Conclusions: COPD patients are referred to the Transplant Center with a higher prevalence of PH because of an echocardiographic screening or a late referral, but many patients survive on the waiting list and undergo the procedure. On the other hand, patients transplanted with interstitial diseases have a lower prevalence of PH; this can be explained by an earlier referral or a higher mortality on the waiting list and a more aggressive and rapidly progressing disease.
    Full-text · Article · Sep 2015 · Transplantation Proceedings
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    • "Genetic associations with the disease include pulmonary surfactant-associated proteins (SFTPA-1 and SFTPA-2), telomerase reverse transcriptase (TERT), and telomerase RNA component (TERC) [119, 120]. It is of interest that statistically significant association in survival has been reported between IPF patients with and without PH at the time of initial IPF diagnosis [121]; PH in IPF can develop either as consequence of the fibrotic process or disproportionate to the degree of fibrotic lung damage [122]. Although chronic hypoxia and its subsequent pulmonary arterial vasoconstriction are thought to have a major role in secondary IPF-PH, studies that showed the existence of PH in such patients even with arterial pO2 levels within normal range (normoxic) led the investigators to partly relinquish this concept and redirect to other possible underlying mechanisms [123–125]. "
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    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.
    Full-text · Article · Oct 2012 · Pulmonary Medicine
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