The Impact of Pulmonary Hypertension on Survival in Patients With Idiopathic Pulmonary Fibrosis

Nadrous, Hassan F.; Pellikka, Patricia A.; Krowka, Michael J.; Swanson, Karen L.; Chaowalit, Nithima; Decker, Paul A.; Ryu, Jay H.

doi:10.1378/chest.128.6_suppl.616s

Cited by 92 publications

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“…7,8 PH in IPF has been associated with worse survival. 8,9 Sildenafi l improves exercise tolerance in World Health Organization (WHO) group 1 PAH 10 ; therefore, we hypothesized that PH evident on echocardiogram would predict response to sildenafi l in the STEP-IPF trial. There are no US Food and Drug Administration-approved therapies demonstrated to modify the disease course, so a treatment that could preserve exercise capacity and QOL in IPF patients with PH would be valuable.…”

Section: Resultsmentioning

confidence: 99%

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

Han

Bach

Hagan

et al. 2013

Chest

203

155

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Background: Idiopathic pulmonary fi brosis (IPF) is a progressive lung disease with pulmonary vasculopathy. Objective: The purpose of this study was to determine whether sildenafi l improves 6-min walk distance (6MWD) in subjects with IPF and right ventricular dysfunction. Methods: The IPFnet, a network of IPF research centers in the United States, conducted a randomized trial examining the effect of sildenafi l on 6MWD in patients with advanced IPF, defi ned by carbon monoxide diffusing capacity , 35% predicted. A substudy examined 119 of 180 randomized subjects where echocardiograms were available for independent review by two cardiologists. Right ventricular (RV) hypertrophy (RVH), right ventricular systolic dysfunction (RVSD), and right ventricular systolic pressure (RVSP) were assessed. Multivariable linear regression models estimated the relationship between RV abnormality, sildenafi l treatment, and changes in 6MWD, St. George's Respiratory Questionnaire (SGRQ), the EuroQol instrument, and SF-36 Health Survey (SF-36) from enrollment to 12 weeks. Results: The prevalence of RVH and RVSD were 12.8% and 18.6%, respectively. RVSP was measurable in 71 of 119 (60%) subjects; mean RVSP was 42.5 mm Hg. In the subgroup of subjects with RVSD, subjects treated with sildenafi l experienced less decrement in 6MWD (99.3 m; P 5 .01) and greater improvement in SGRQ (13.4 points; P 5 .005) and EuroQol visual analog scores (17.9 points; P 5 .04) than subjects receiving placebo. In the subgroup with RVH, sildenafi l was not associated with change in 6MWD ( P 5 .13), but was associated with greater relative improvement in SGRQ (14.8 points; P 5 .02) vs subjects receiving placebo. Sildenafi l treatment in those with RVSD and RVH was not associated with change in SF-36. Conclusions: Sildenafi l treatment in IPF with RVSD results in better preservation of exercise capacity as compared with placebo. Sildenafi l also improves quality of life in subjects with RVH and RVSD.CHEST 2013; 143(6):1699-1708Abbreviations: 6MWD 5 6-min walk distance; BNP 5 brain natriuretic peptide; D lco 5 carbon monoxide diffusing capacity; IPF 5 idiopathic pulmonary fi brosis; PAH 5 pulmonary arterial hypertension; PH 5 pulmonary hypertension; QOL 5 quality of life; RHC 5 right-sided heart catheterization; RV 5 right ventricular; RVH 5 right-sided ventricular hypertrophy; RVSD 5 right-sided ventricular systolic dysfunction; RVSP 5 right-sided ventricular systolic pressure;

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Section: Resultsmentioning

confidence: 99%

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

Han

Bach

Hagan

et al. 2013

Chest

203

155

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“…A significant number of patients with IPF have elevated pulmonary artery pressures, either at rest or with exercise (35,36). Because a significant amount of the pulmonary vasculature (40%) must be involved to account for pulmonary hypertension, this suggests that vascular involvement begins early on.…”

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confidence: 99%

State of the Art. Does Current Knowledge Explain the Pathogenesis of Idiopathic Pulmonary Fibrosis?: A Perspective

Hunninghake

Schwarz²

2007

Proceedings of the American Thoracic Society

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The cause of idiopathic pulmonary fibrosis (IPF) remains unknown. Although the observed biologic and biochemical processes associated with the disease are consistent with a fibrotic process, they are not necessarily unique to IPF. Furthermore, the importance of these observations will not be apparent until a directed therapy alters the natural history of the disease. There are essentially no studies that explain the unique histologic features of this disease. As mechanistic data accumulates, it is our opinion that these data should pass the test of explaining the clinical histologic features of the disease before it can be assumed that these features are unique for IPF.

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“…(10) These findings were confirmed in a study in which pulmonary hypertension was defined as an mPAP >25 mmHg: in patients with pulmonary hypertension, the 1-year mortality rate was 28%, whereas it was 5.5% in those without pulmonary hypertension.…”

Section: F P Machadomentioning

confidence: 70%

Hipertensão pulmonar em pneumopatias crônicas: temos que aprender mais

Machado¹

2008

J. bras. pneumol.

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Pulmonary hypertension is a common complication of chronic respiratory diseases such as chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis and sleep apnea. It is estimated that the prevalence of pulmonary hypertension in patients hospitalized for respiratory disorders is 28%.(1) In patients with COPD or idiopathic pulmonary fibrosis who are referred for lung transplantation or lung volume reduction surgery, the prevalence of pulmonary hypertension -defined as mean pulmonary artery pressure (mPAP) >25 mmHg -approaches 30%.(2-4) In general, the pulmonary hypertension seen in such patients tends to be mild, with an mPAP ranging from 25 to 35 mmHg. In the majority of cases, the severity of pulmonary hypertension is directly correlated with the severity of the respiratory disease and with the degree of hypoxemia. However, these correlations are weak, and some patients present severe pulmonary hypertension out of proportion to the severity of the lung disease. For instance, Thabut et al. studied 215 patients with advanced COPD and identified a subgroup of 16 patients that had severe pulmonary hypertension (mPAP, 39.8 ± 10.2 mmHg) and yet presented a more moderate reduction in forced expiratory volume in one second (FEV 1 ; 48.5 ± 11.8% of predicted).(5) In a group of 28 patients with idiopathic pulmonary fibrosis, Leuchte et al. identified 6 patients with an mPAP > 35 mmHg. (6) It seems clear that hypoxic vasoconstriction is not the sole mechanism associated with the pathogenesis of pulmonary hypertension in patients with chronic respiratory disorders. Individuals living at high altitude develop pulmonary hypertension and pulmonary artery medial hypertrophy that are reversible at sea level. In addition, chronic oxygen use does not reverse pulmonary hypertension in patients with COPD or pulmonary fibrosis, suggesting that remodeling of the pulmonary arterial circulation occurs in such patients. In contrast to that seen in individuals living at high altitude, pulmonary hypertension in patients with COPD or pulmonary fibrosis affects all layers of the arterial wall.(7) These changes are more prominent in the intima, which is thickened by the presence of smooth muscle fibers, together with the deposition of collagen and elastin, in the extracellular space. Furthermore, in patients with pulmonary fibrosis, intimal lesions can lead to acellular fibrosis and cause luminal obstruction, which is more prominent within the fibroblast layer, where the vascular density is extremely low.(8) Although the pathobiological mechanisms involved are not well defined, these histological changes appear to be related to abnormalities in the same mediators implicated in the pathogenesis of idiopathic pulmonary hypertension. Such mediators include nitric oxide, endothelin and prostacyclins.Pulmonary hypertension is associated with worse survival in patients with chronic respiratory disorders. In one cohort of patients with COPD, the 5-year survival rate was 36% for individuals with pulmonary hypertension and 62% for th...

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The Impact of Pulmonary Hypertension on Survival in Patients With Idiopathic Pulmonary Fibrosis

Cited by 92 publications

References 0 publications

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

State of the Art. Does Current Knowledge Explain the Pathogenesis of Idiopathic Pulmonary Fibrosis?: A Perspective

Hipertensão pulmonar em pneumopatias crônicas: temos que aprender mais

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