Echocardiographic and Hemodynamic Predictors of Mortality in Idiopathic Pulmonary Fibrosis

Rivera‐Lebron, Belinda; Forfia, Paul R.; Kreider, Maryl; Lee, James C.; Holmes, John H.; Kawut, Steven M.

doi:10.1378/chest.12-2298

Cited by 105 publications

(102 citation statements)

References 18 publications

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“…In general, the prevalence of PH was similar in studies from Europe [18,25,[40][41][42][43][44][45][47][48][49], North America [5,6,26,28,46,[50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65][66][67], Asia and Africa [37,[68][69][70][71][72][73][74][75], although the majority of evidence was reported from North America (n=23, 53%). Across studies, the prevalence of PH among IPF patients ranged from 3% to 86%; most estimates tended to be between 30% and 50% (figure 3) [25, 43, 44, 47, 50, 52-66, 68, 71-73].…”

Section: Pulmonary Hypertensionmentioning

confidence: 85%

“…Presence of PH was found to be associated with an increased risk for death (HR for death, PH versus no PH 4.03, 95% CI 1.17-27.9; p=0.03 (study population was CPFE patients) [76]; HR 3.6, 95% CI not reported; p=0.0001 [44]). However, an analysis of data from 121 IPF patients from a hospital in Denmark did not find a significant association between PH and mortality, although a numerical trend remained after adjustment for age, gender and forced vital capacity (FVC) (HR 2.2, 95% CI 0.94-5.2; p=0.068) [48]; similarly, a review of IPF patients evaluated for lung transplantation at a US university hospital did not find a significant association between mean pulmonary artery pressure (mPAP) and mortality [67]. Among a review based on existing data of 25 IPF patients from the USA with PH, 1-year mortality was 29% [57].…”

Section: Pulmonary Hypertensionmentioning

confidence: 96%

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Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies published in English from January 1990 to January 2015 identified via MEDLINE and EMBASE were included; bibliographies of articles were also searched.Among the 126 studies included, prevalence of pulmonary hypertension (PH) was 3-86%, 6-91% for obstructive sleep apnoea, 3-48% for lung cancer and 6-67% for chronic obstructive pulmonary disease (COPD). Nonrespiratory comorbidities included ischaemic heart disease (IHD) (3-68%) and gastrooesophageal reflux (GER) (0-94%). Mortality was highest among patients with IPF and lung cancer. Most studies assessed relatively small samples of patients with IPF.PH, COPD, lung cancer, GER and IHD are significant comorbidities; differences in IPF severity, case definitions and patient characteristics limited the comparability of findings. The identification and prompt treatment of comorbidities may have a clinically significant impact on overall outcome that is meaningful for patients with IPF. @ERSpublications Identification and prompt treatment of comorbidities has a clinically significant impact on outcome for IPF patients

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Section: Pulmonary Hypertensionmentioning

confidence: 85%

Section: Pulmonary Hypertensionmentioning

confidence: 96%

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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“…Other echocardiographic indices may be useful for the identification of right ventricle dysfunction and PH. A right ventricle to left ventricle diameter ratio >1 is associated with a 5.4 increase in the HR for mortality in IPF [90], while moderate-to-severe right ventricle dysfunction, identified by tricuspid annular plane systolic excursion <1.6 cm, is associated with a 7.5 increase, suggesting that echocardiography may indeed have a role for the identification of IPF patients with clinically significant pulmonary vascular impairment [90]. HRCT measurements of the diameter of the pulmonary artery do not accurately indicate PH in IPF, possibly due to the confounding effect of reduced pleural pressure causing the dilation of cavitary intrathoracic organs [76].…”

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 98%

“…PH is associated with increased mortality in IPF. In a series of 135 patients, there was a 2.4 increase in the hazard ratio (HR) for death per 10 mmHg increase in mPAP, while an increase in PVR of 1 Wood unit was associated with a 1.4 increase in the HR for mortality [90].…”

Section: Alterations In Pulmonary Haemodynamicsmentioning

confidence: 98%

Physiology of the lung in idiopathic pulmonary fibrosis

et al. 2018

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The clinical expression of idiopathic pulmonary fibrosis (IPF) is directly related to multiple alterations in lung function. These alterations derive from a complex disease process affecting all compartments of the lower respiratory system, from the conducting airways to the lung vasculature. In this article we review the profound alterations in lung mechanics (reduced lung compliance and lung volumes), pulmonary gas exchange (reduced diffusing capacity, increased dead space ventilation, chronic arterial hypoxaemia) and airway physiology (increased cough reflex and increased airway volume), as well as pulmonary haemodynamics related to IPF. The relative contribution of these alterations to exertional limitation and dyspnoea in IPF is discussed.

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“…An additional possibility is that 6MWD measures the prognostic importance of concurrent emphysema or pulmonary hypertension. Although the impact of emphysema is unclear [7], abnormal pulmonary haemodynamics predict increased mortality in IPF and the presence of pulmonary hypertension could account for the independent relationship of 6MWD with mortality [8]. Finally, 6MWD is influenced by extrapulmonary disease and a reduced 6MWD could indicate the presence of other comorbidities or frailty that increase mortality independent of IPF severity.…”

Section: Defining Mortality Risk In Ipfmentioning

confidence: 99%

Risk prediction and end-points in idiopathic pulmonary fibrosis: one step at a time

Ryerson

2014

Eur Respir J

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@ERSpublicationsPredicting mortality in IPF: a commentary on new data showing the prognostic importance of 6-min walking distance http:// http://ow.ly/tCD0NIdiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a median survival of approximately 3 years from diagnosis. Accurate prediction of mortality is important in IPF, as it helps determine the urgency of lung transplantation, guides other management and end-of-life decisions, and facilitates enrichment of clinical trial populations. Although most patients with IPF die from progressive respiratory failure [1], predicting outcomes is challenging due to the heterogeneity of disease progression.In this issue of the European Respiratory Journal, DU BOIS et al.[2] use the INSPIRE (International Study of Survival Outcomes in Idiopathic Pulmonary Fibrosis with Interferon-c-1b) dataset to describe a new prediction model that estimates mortality in IPF. The INSPIRE study was a multicentre double-blind randomised trial comparing interferon-c-1b to placebo in 826 patients with mild to moderate IPF [3]. This trial did not meet its primary end-point but has generated high-quality data that continue to provide valuable insight into the progression of IPF. DU BOIS et al. [4] have previously used the INSPIRE data to show that age, baseline forced vital capacity (FVC), 24-week change in FVC, and recent respiratory hospitalisation independently predict 1-year mortality in patients with IPF [4]. These authors have now extended their findings by showing that mortality prediction is improved by adding both baseline and 24-week change in 6-min walking distance (6MWD) to this multivariate model [2]. This new study is a noteworthy addition to the literature, providing important data on the prediction of mortality in IPF and suggesting potential options for clinical trial end-points. Defining mortality risk in IPFThe model developed by DU BOIS et al.[2] accurately estimates 1-year mortality in patients with IPF with a C-statistic of 0.80. The C-statistic, or C-index, is a measure of how well a model predicts the risk of an outcome (e.g. 1-year mortality) in any two individuals randomly selected from the cohort of interest. A Cstatistic of 1.0 indicates that the model appropriately identifies the individual at higher risk of the outcome in every paired comparison. A C-statistic above 0.70 is generally considered acceptable, and a C-statistic of 0.50 indicates that the prediction model performs no better than chance. The C-statistic of 0.80 in this study compares favourably with models that are in routine clinical use in other fields and other validated models in IPF [5]. It is therefore tempting to use this new model in the clinical setting; however, there are some questions that require further study.

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Echocardiographic and Hemodynamic Predictors of Mortality in Idiopathic Pulmonary Fibrosis

Cited by 105 publications

References 18 publications

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

Physiology of the lung in idiopathic pulmonary fibrosis

Risk prediction and end-points in idiopathic pulmonary fibrosis: one step at a time

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