Pulmonary Hypertension In COPD Research Articles

Incidence of pulmonary hypertension in COPD and its relation to inflammatory marker interleukin-1

BackgroundMany COPD patients present with severe PH defined by a pulmonary vascular resistance (PVR) > 5 WU as measured by right heart catheterization (RHC), and inflammation is thought to be contributing strongly to pulmonary vascular remodeling in COPD besides hypoxia. Interleukin-1 (IL-1) is thought to be a major cytokine that may be involved in development of PH in these patients.ObjectiveThis study aimed to identify the incidence of PH in COPD and its relation to inflammatory marker IL-1.Patients and methodsOne-hundred COPD patients underwent echocardiography and serum IL-1 analysis, and patients with high probability of PH underwent pulmonary artery catheterization using Swan-Ganz catheter.ResultsThe mean serum IL-1 level was 82 pg/ml ± 22 pg/ml (the normal IL-1 serum level is known to be 0:5 pg/mL), 51% of the participants were categorized as having a low probability of PH, 35% was intermediate, and 14% was high. RHC results were as follows: mild combined pre- and postcapillary PH was diagnosed in 14.3% of the patients. Mild precapillary PH was found in 42.9%, making it the most common type. Severe combined pre- and postcapillary PH was noted in 21.4% of the patients. Severe precapillary PH was present in 14.3%. A significant positive correlations were observed between serum IL-1 and tricuspid regurgitation velocity (TRV) (r = 0.409, P < 0.001), estimated systolic pulmonary artery pressure (ESPAP) (r = 0.508, P < 0.001), and mean pulmonary artery pressure (mPAP) (r = 0.410, P = 0.140).ConclusionSerum IL-1 is a potent predictor of a high probability of PH in COPD patients, and there was significant positive correlation between serum IL-1 and echocardiographic findings and PH probability and RHC findings in COPD patients.

Characterization of pulmonary vascular remodeling and MicroRNA-126-targets in COPD-pulmonary hypertension

BackgroundDespite causing increased morbidity and mortality, pulmonary hypertension (PH) in chronic obstructive pulmonary disease (COPD) patients (COPD-PH) lacks treatment, due to incomplete understanding of its pathogenesis. Hypertrophy of pulmonary arterial walls and pruning of the microvasculature with loss of capillary beds are known features of pulmonary vascular remodeling in COPD. The remodeling features of pulmonary medium- and smaller vessels in COPD-PH lungs are less well described and may be linked to maladaptation of endothelial cells to chronic cigarette smoking (CS). MicroRNA-126 (miR126), a master regulator of endothelial cell fate, has divergent functions that are vessel-size specific, supporting the survival of large vessel endothelial cells and inhibiting the proliferation of microvascular endothelial cells. Since CS decreases miR126 in microvascular lung endothelial cells, we set out to characterize the remodeling by pulmonary vascular size in COPD-PH and its relationship with miR126 in COPD and COPD-PH lungs.MethodsDeidentified lung tissue was obtained from individuals with COPD with and without PH and from non-diseased non-smokers and smokers. Pulmonary artery remodeling was assessed by ⍺-smooth muscle actin (SMA) abundance via immunohistochemistry and analyzed by pulmonary artery size. miR126 and miR126-target abundance were quantified by qPCR. The expression levels of ceramide, ADAM9, and endothelial cell marker CD31 were assessed by immunofluorescence.ResultsPulmonary arteries from COPD and COPD-PH lungs had significantly increased SMA abundance compared to non-COPD lungs, especially in small pulmonary arteries and the lung microvasculature. This was accompanied by significantly fewer endothelial cell markers and increased pro-apoptotic ceramide abundance. miR126 expression was significantly decreased in lungs of COPD individuals. Of the targets tested (SPRED1, VEGF, LAT1, ADAM9), lung miR126 most significantly inversely correlated with ADAM9 expression. Compared to controls, ADAM9 was significantly increased in COPD and COPD-PH lungs, predominantly in small pulmonary arteries and lung microvasculature.ConclusionBoth COPD and COPD-PH lungs exhibited significant remodeling of the pulmonary vascular bed of small and microvascular size, suggesting these changes may occur before or independent of the clinical development of PH. Decreased miR126 expression with reciprocal increase in ADAM9 may regulate endothelial cell survival and vascular remodeling in small pulmonary arteries and lung microvasculature in COPD and COPD-PH.

Severe Pulmonary Hypertension in COPD: Impact on Survival and Diagnostic Approach

Severe pulmonary hypertension (PH) is prognostically highly relevant in patients with COPD. The criteria for severe PH have been defined based on hemodynamic thresholds in right heart catheterization. Can noninvasive clinical tools predict severe PH in patients with COPD? How does the mortality risk change with increasing severity of airflow limitation and pulmonary vascular disease? We retrospectively analyzed all consecutive patients with COPD with suspected PH undergoing in-depth clinical evaluation, including right heart catheterization, in our PH clinic between 2005 and 2018. Clinical variables potentially indicative of severe PH or death were analyzed using univariate and stepwise multivariate logistic regression and Cox regression analysis adjusted for age and sex. We included 142 patients with median FEV1 of 55.0%predicted (interquartile range [IQR], 42.4%-69.4%predicted) and mean pulmonary arterial pressure of 35mmHg (IQR, 27-43mmHg). A multivariate model combining echocardiographic systolic pulmonary arterial pressure of≥ 56mmHg, N-terminal pro-brain natriuretic peptide (NT-proBNP) plasma levels of≥ 650 pg/mL, and pulmonary artery (PA) to ascending aorta (Ao) diameter ratio on chest CT scan of≥ 0.93 predicted severe PH with high positive and negative predictive values (both 94%). After correction for age and sex, both airflow limitation (P= .002; Global Initiative for Chronic Obstructive Lung Disease [GOLD] stages 1-2 vsstage 3: hazard ratio [HR], 1.56 [95%CI, 0.90-2.71]; GOLD stages 1-2 vsstage 4: HR, 3.45 [95%CI, 1.75-6.79]) and PH severity (P= .012; HR, 1.85 [95%CI, 1.15-2.99]) remained associated independently with survival. The combination of GOLD stages 3 and 4 airflow limitation and severe PH showed the poorest survival (HR for death, 3.26 [95%CI, 1.62-6.57; P= .001] vsGOLD stages 1-2 combined with nonsevere PH). In patients with COPD, the combination of echocardiography, NT-proBNP level, and PA to Ao diameter ratio predicts severe PH with high sensitivity and specificity. The contribution of severe PH and severe airflow limitation to impaired survival is comparable.

P15‐3: Potential amelioration of pulmonary hypertension in COPD by budesonide/glycopyrronium/formoterol via central NFκB inflammatory signaling deactivation: In vivo mouse model

P15‐3: Potential amelioration of pulmonary hypertension in COPD by budesonide/glycopyrronium/formoterol via central NFκB inflammatory signaling deactivation: In vivo mouse model

Pulmonary Hypertension in Patients With COPD: Results From the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)

Pulmonary hypertension (PH) in COPD is a poorly investigated clinical condition. Which factors determine the outcome of PH in COPD? We analyzed the characteristics and outcome of patients enrolled in the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) with moderate or severe PH in COPD as defined during the 6th PH World Symposium who received medical therapy for PH and compared them with patients with idiopathic pulmonary arterial hypertension (IPAH). The population included incident patients with moderate PH in COPD (n= 68), with severe PH in COPD (n= 307), and with IPAH (n= 489). Patients with PH in COPD were older, predominantly male, and treated mainly with phosphodiesterase-5 inhibitors. Despite similar hemodynamic impairment, patients with PH in COPD achieved a worse 6-min walking distance (6MWD) and showed a more advanced World Health Organization functional class (WHO FC). Transplant-free survival rates at 1, 3, and 5 years were higher in the IPAH group than in the PH in COPD group (IPAH: 94%, 75%, and 55%vsPH in COPD: 86%, 55%, and 38%; P= .004). Risk factors for poor outcomes in PH in COPD were male sex, low 6MWD, and high pulmonary vascular resistance (PVR). In patients with severe PH in COPD, improvements in 6MWD by≥ 30m or improvements in WHO FC after initiation of medical therapy were associated with better outcomes. Patients with PH in COPD were functionally more impaired and had a poorer outcome than patients with IPAH. Predictors of death in the PH in COPD group were sex, 6MWD, and PVR. Our data raise the hypothesis that some patients with severe PH in COPD may benefit from PH treatment. Randomized controlled studies are necessary to explore this hypothesis further. ClinicalTrials.gov; No.: NCT01347216; URL: www.clinicaltrials.gov.

Pulmonary hypertension in eosinophilic versus noneosinophilic COPD.

BackgroundThe eosinophilic COPD phenotype is associated with greater airway remodelling, exacerbation risk and steroid responsiveness. However, little is known about the prevalence and characteristics of pulmonary hypertension (PH) in this patient population.MethodsWe retrospectively evaluated a cohort of COPD patients with right heart catheterisation (RHC) data at a university hospital between January 2011 and May 2019 and compared the pulmonary vascular profile and prevalence of PH between eosinophilic and noneosinophilic patients using a definition of eosinophilic COPD as at least three blood eosinophil values ≥300 cells·µL−1. We used multivariable logistic regression analyses to examine the association between eosinophilic COPD and various PH categories adjusting for age, sex, body mass index, forced expiratory volume in 1 s (%), smoking status and use of supplemental oxygen.ResultsAmong 106 COPD patients with RHC data and at least three blood eosinophil values, 25% met the definition of eosinophilic COPD. Fewer patients among the eosinophilic group required long-term oxygen therapy (69% versus 93%, p=0.001) and total lung capacity was significantly lower in the eosinophilic group (p=0.006). This group had higher mean pulmonary arterial pressure (mPAP) (median (interquartile range) 30 (27–41) mmHg versus 25 (22–30) mmHg, p=0.001) and pulmonary vascular resistance (PVR) (4 (2.8–5.1) Wood units versus 2.9 (2.1–4.1) Wood units, p=0.018). On multivariable logistic regression analyses, eosinophilic phenotype was associated with PH (adjusted (a)OR 6.5, 95% CI 1.4–30.7; p=0.018) and pre-capillary PH (aOR 3.2, 95% CI 1.1–9; p=0.027), but not severe PH (aOR 2.1, 95% CI 0.6–7.2; p=0.219).ConclusionEosinophilic COPD was associated with higher mPAP and PVR and increased likelihood of PH. More studies are needed to further explore this finding.

Post Exercise Desaturation Can Help Identifying Treatment Responders of COPD Pulmonary Hypertension in Real World: An Appraisal

Post Exercise Desaturation Can Help Identifying Treatment Responders of COPD Pulmonary Hypertension in Real World: An Appraisal

Cardiopulmonary exercise test and PaO2 in evaluation of pulmonary hypertension in COPD.

BackgroundExercise tolerance decreases as COPD progresses. Pulmonary hypertension (PH) is common in COPD and may reduce performance further. COPD patients with and without PH could potentially be identified by cardiopulmonary exercise test (CPET). However, results from previous studies are diverging, and a unified conclusion is missing. We hypothesized that CPET combined with arterial blood gases is useful to discriminate between COPD outpatients with and without PH.MethodsIn total, 93 COPD patients were prospectively included. Pulmonary function tests, right heart catheterization, and CPET with blood gases were performed. The patients were divided, by mean pulmonary artery pressure, into COPD-noPH (<25 mmHg) and COPD-PH (≥25 mmHg) groups. Linear mixed models (LMMs) were fitted to estimate differences when repeated measurements during the course of exercise were considered and adjusted for gender, age, and airway obstruction.ResultsVentilatory and/or hypoxemic limitation was the dominant cause of exercise termination. In LMM analyses, significant differences between COPD-noPH and COPD-PH were observed for PaO2, SaO2, PaCO2, ventilation, respiratory frequency, and heart rate. PaO2 <61 mmHg (8.1 kPa) during unloaded pedaling, the only load level achieved by all the patients, predicted PH with a sensitivity of 86% and a specificity of 78%.ConclusionDuring CPET, low exercise performance and PaO2 strongly indicated PH in COPD patients.

Loss of Small Vessel Volume Fraction as a Marker for Pulmonary Hypertension in COPD

SESSION TITLE: Pulmonary Hypertension SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, November 1, 2017 at 01:30 PM - 02:30 PM PURPOSE: Pulmonary hypertension develops in a subset of subjects with COPD, and when present is accompanied by increasing morbidity and mortality. Early diagnosis, prognostication and selection of subjects that may benefit from pulmonary vasodilatory therapy remains challenging. In prior work it has been shown that smokers show evidence of pulmonary vascular pruning in 3D reconstruction of the pulmonary vasculature. In this study we examine changes to the pulmonary vascular tree in patients with COPD and pre-capillary pulmonary hypertension proven by right heart catheterization (PAH). METHODS: A retrospective database query of subjects with diagnosis of COPD and PH possessing ct angiograms yielded approximately 200 subjects. From these, 21 subjects with non-elevated PCWP (<15mmHg), PH (elevated PVR and mPAP) and FEV1/FVC of less than 70% by PFT were selected. Of these, seven had 1mm cuts appropriate for reconstruction. A cohort of 15 subjects having undergone right heart catheterization, imaging and exercise testing with no evidence of cardiopulmonary disease was used as control subjects. Vascular reconstruction was performed as previously described. Small vessel volume fraction, defined as the vascular volume in small vessels (less than 5mm2 in cross-sectional area) divided by total vascular volume, was used to measure pruning. Association was tested using a two-tailed t-test, assuming unequal variances. RESULTS: Seven patients (Age 65.7±10.5) with COPD (FEV1pp 61.7±6.8%) were identified with precapillary PAH (mPAP 45±14.3mmHg; PVR 8.6±6WU; mPCWP 10.8±2.2mmHg, max 14mmHg). Fifteen subjects (Age 46 ; mPAP 15mmHg; PVR 1.1 WU; mPCWP 9mmHg) were used as controls. Subjects with PAH and COPD had significant proximal dilation and distal loss of vasculature, which was quantified by dividing the volume in small vessels (defined as those with cross sectional area of less than 5mm2 ) by the total volume of the vasculature detected. Small vessel volume fraction in the COPD group was lower (0.37 ± 0.12) as compared to control subjects (0.55 ±0.11) with a p-value of 0.006 for the comparison. CONCLUSIONS: The vascular pruning previously described in smokers is quantifiable from CT angiography in subjects with precapillary pulmonary arterial hypertension in the context of significant COPD burden. CLINICAL IMPLICATIONS: Measurement of pulmonary vascular morphology in smokers may be useful in detecting and subtyping the extent of pulmonary vascular disease in smokers. DISCLOSURE: The following authors have nothing to disclose: Farbod Rahaghi, Viviana Paz, James Ross, Raúl San José Estépar, George Washko, Franck Rahaghi Image processing techniques for reconstruction of the vasculature used are currently in exploratory stage and appropriate only for research purposes.

Dysregulation of microRNA-214 and PTEN contributes to the pathogenesis of hypoxic pulmonary hypertension.

Hypoxia-induced pulmonary hypertension, which is characterized by vascular remodeling of blood vessels, is an important complication in COPD. In this study, we found that the expression of miR-214 was differentially expressed by screening 13 candidate miRNAs in pulmonary artery smooth muscle cells (PASMCs). Additionally, using luciferase assay in PASMCs, we found that phosphatase-and-tensin homolog (PTEN) was a target of miR-214. Furthermore, the expression of PTEN was found to be substantially downregulated in PASMCs from COPD patients with pulmonary hypertension (PH) compared with normal controls by using real-time polymerase chain reaction (PCR), immunohistochemistry, and Western blot. In addition, we transfected PASMCs with miR-214 mimics, using real-time PCR and Western blotting, to confirm the miRNA/mRNA relationship. Furthermore, the introduction of miR-214 significantly promoted the proliferation of PASMCs by suppressing apoptosis of the cells, which was mediated by the downregulation of PTEN. Exposure to hypoxia significantly increased the expression of miR-214 and decreased the expression of PTEN in PASMCs, and its proliferation was significantly promoted. Such effects could be significantly attenuated by the introduction of miR-214 inhibitors, which significantly downregulated miR-214 expression and upregulated the expression of PTEN. In conclusion, hypoxia-induced upregulation of miR-214 was found to promote PH development by targeting PTEN in PASMCs, and miR-214 could be a promising diagnostic tool and novel therapeutic target in the management of hypoxia-induced PH in COPD.

The Use of Macitentan in the Treatment of Out-of-Proportion Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease: Case Report

Pulmonary hypertension (PH) is an important complication of chronic pulmonary disease, especially of chronic obstructive lung disease (COPD). The exact prevalence of PH in COPD is unknown. PH is defined as an increase in mean pulmonary arterial pressure (PAPm) > 25 mmHg at rest as assessed by right heart catheterization . In most cases, PH in COPD patients is usually mild to moderate.

Plasma brain natriuretic peptide levels in COPD without pulmonary hypertension

IntroductionIt has long been known that COPD causes elevation in BNP level caused by hypoxia present in cases of pulmonary hypertension in COPD. The aim of this studyThis study was carried out to evaluate the changes in BNP level in COPD patients without pulmonary hypertension or cor pulmonale during exacerbation and after remission. Subjects and methodsThis study was created on 50 subjects, 30 COPD patients according to inclusion and exclusion criteria (BNP level will be measured during exacerbation and after remission) plus 20 age matched apparently healthy control subjects, ten of them are non smokers and ten are asymptomatic smokers. For all subjects, history taking, full clinical exam done. PFT (spirometry), BNP level measurement on human serum by ELIZA, routine labs (CBC, liver and renal function). ECG. echocardiography. ResultsLevels of BNP were significantly higher in COPD patients with mean (60.52±30.98pg/mL) than control with mean (21.13±4.61pg/mL) and higher during exacerbation (60.52±30.9pg/mL) than during remission (35.65±16.54pg/mL), BNP was significantly higher in grade (III, IV) with mean (86.94±40.19, pg/mL) than grade (II) (56.76±6.2pg/mL) and grade (II) was significantly higher than grade (I) with mean (37.86±8.81pg/mL) and it was a significantly inversely related to post FEV1% and post FEF 25–75% and significantly direct correlated to paco2 and non significant negative correlation to pao2. ConclusionPlasma BNP can be used as a useful prognostic biomarker of COPD and a good predictor of exacerbation, As BNP level was significantly higher in COPD patients than in control groups, (p<0.005) and also significantly higher in grade (IV, III) than grade (II) and was significantly higher in grade (II) than grade (I) COPD patients, BNP level significantly higher (p<0.005) during exacerbation than during remission of COPD patients.

Usefulness of the 6-minute walk test as a screening test for pulmonary arterial enlargement in COPD.

PurposePulmonary hypertension and exercise-induced oxygen desaturation (EID) influence acute exacerbation of COPD. Computed tomography (CT)-detected pulmonary artery (PA) enlargement is independently associated with acute COPD exacerbations. Associations between PA to aorta (PA:A) ratio and EID in patients with COPD have not been reported. We hypothesized that the PA:A ratio correlated with EID and that results of the 6-minute walk test (6MWT) would be useful for predicting the risk associated with PA:A >1.Patients and methodsWe retrospectively measured lung function, 6MWT, emphysema area, and PA enlargement on CT in 64 patients with COPD. The patients were classified into groups with PA:A ≤1 and >1. Receiver-operating characteristic curves were used to determine the threshold values with the best cutoff points to predict patients with PA:A >1.ResultsThe PA:A >1 group had lower forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1:FVC ratio, diffusion capacity of lung carbon monoxide, 6MW distance, and baseline peripheral oxygen saturation (SpO2), lowest SpO2, highest modified Borg scale results, percentage low-attenuation area, and history of acute COPD exacerbations ≤1 year, and worse BODE (Body mass index, airflow Obstruction, Dyspnea, and Exercise) index results (P<0.05). Predicted PA:A >1 was determined for SpO2 during 6MWT (best cutoff point 89%, area under the curve 0.94, 95% confidence interval 0.88–1). SpO2 <90% during 6MWT showed a sensitivity of 93.1, specificity of 94.3, positive predictive value of 93.1, negative predictive value of 94.3, positive likelihood ratio of 16.2, and negative likelihood ratio of 0.07.ConclusionLowest SpO2 during 6MWT may predict CT-measured PA:A, and lowest SpO2 <89% during 6MWT is excellent for detecting pulmonary hypertension in COPD.

&lt;em&gt;TRPM8&lt;/em&gt;&amp;nbsp;genetic variations associated with COPD risk in the Chinese Han population

TRPM8 plays a key role in COPD. The development of pulmonary hypertension (PH) in COPD adversely affects survival and exercise capacity. Thus, the aim of this study was to evaluate the possible association between single nucleotide polymorphisms (SNPs) in TRPM8 and COPD or PH in COPD among the Chinese Han population. A total of 513 COPD patients and 506 controls were enrolled in the study. Six tag SNPs (tSNPs) were genotyped. The relationship between COPD or PH in COPD and the six tSNPs was evaluated using the χ2 test and genetic model analysis. In the rs9789398 polymorphism, the T/C genotype was associated with an increased risk for COPD (P=0.005). Under the assumption of models of inheritance, there was an association between the rs9789398 polymorphism and COPD. In the rs9789675 polymorphism, the G/A genotype was associated with an increased risk for COPD (P=0.021). Furthermore, by the χ2 test, we found that the minor allele “A” of rs9789675 (odds ratio [OR] =0.63, 95% confidence interval [CI], 0.42–0.97, P=0.034) and the minor allele “C” of rs9789398 (OR =1.59, 95% CI, 1.03–2.44, P=0.034) were associated with a decreased risk of PH in COPD in allele models. In genetic models, the genotypes “GA” and “AA” of rs9789675 were associated with a decreased risk of PH in COPD. The genotypes “TC” and “CC” of rs9789398 were associated with a decreased risk of PH in COPD. Moreover, “CG” of rs1004478 was significantly associated with a decreased risk of PH in COPD. There was a significant association between the five SNPs (rs2362290, rs9789675, rs9789398, rs1003540, and rs104478) in the TRPM8 gene and the risk of PH in COPD. Our findings indicated that rs9789398 in the TRPM8 gene was significantly associated with the risk of COPD in the Chinese Han population. Moreover, rs9789675, rs9789398, and rs1004478 were significantly associated with the risk of PH in COPD. This study provides a novel insight into COPD and PH in the development of COPD.

Exercise hypoxaemia as a predictor of pulmonary hypertension in COPD patients without severe resting hypoxaemia.

Pulmonary hypertension (PH) in COPD is associated with morbidity and mortality. Previous studies showed a relationship between resting hypoxaemia and PH, but little is known about the relationship between exercise hypoxaemia and PH in COPD without resting hypoxaemia. A retrospective observational study of COPD patients without resting hypoxaemia was conducted to evaluate the relationships between exercise hypoxaemia and pulmonary haemodynamics. Clinical characteristics, pulmonary function, blood gas analysis, 6-min walk distance (6MWD) and oxygen saturation of peripheral artery (SpO2 ) at the end of the 6-min walk test (6MWT) were reviewed. Correlation analysis and stepwise regression analysis were performed to identify the predictor of mean pulmonary artery pressure (mPAP). Eighty-four consecutive patients with a mean predicted forced expiratory volume in 1 s (FEV1 ) of 47 ± 21% were evaluated. In univariate analysis, mPAP had negative correlations with age (r = -0.27, P < 0.05), arterial partial pressure of oxygen (PaO2 , r = -0.24, P < 0.05), % predicted forced vital capacity (FVC, r = -0.28, P < 0.05), % predicted FEV1 (r = -0.40, P < 0.001), FEV1 /FVC ratio (r = -0.33, P < 0.005), % predicted diffusion capacity for carbon monoxide (DLCO , r = -0.40, P < 0.001), 6MWD (r = -0.40, P < 0.001) and SpO2 at the end of the 6MWT (r = -0.74, P < 0.001). In stepwise regression analysis, SpO2 at the end of the 6MWT and 6MWD remained as independent predictors of mPAP (R2 = 0.60). In receiver operating characteristic (ROC) analysis, SpO2 at the end of the 6MWT presented an area under the curve of 0.896 for the prediction of PH, with a sensitivity of 0.86 and specificity of 0.84 for the cut-off point of 81%. In addition to 6MWD, exercise hypoxaemia indicates PH in patients with COPD without resting hypoxaemia.

Pulmonary hypertension associated with lung diseases and hypoxemia.

Pulmonary hypertension that develops in the setting of underlying lung diseases such as COPD or idiopathic pulmonary fibrosis (IPF) is associated with decreased functional status, worsening hypoxemia and quality of life, and increased mortality. This complication of lung disease is complex in its origin and carries a unique set of diagnostic and therapeutic issues. This review attempts to provide an overview of mechanisms associated with the onset of pulmonary hypertension in COPD and IPF, touches on appropriate evaluation, and reviews the state of knowledge on treating pulmonary hypertension related to underlying lung disease.

(839) - Pulmonary Hypertension in COPD: A Survival Analysis of Patients Listed for Lung Transplantation

(839) - Pulmonary Hypertension in COPD: A Survival Analysis of Patients Listed for Lung Transplantation

(391) - SPHERIC-1 (Sildenafil and Pulmonary HypERtension in COPD): Intention-to-Treat (ITT) Analysis of Safety and Efficacy Data

PHERIC-1 is an italian multicenter, double-blind, placebo-controlled trial aimed to investigate the safety and the hemodynamic effects of sustained sildenafil (Sld) administration in COPD-associate out-of-proportion pulmonary hypertension (COPD-OoPPH) (http://clinicaltrials.gov/ct2/show/NCT01441934). Primary end-points: reduction in pulmonary vascular resistance (PVR), and drop in PaO2. ITT analysis of the results is presented.

Pulmonary hypertension in COPD; the importance of PaO2

Pulmonary hypertension in COPD; the importance of PaO2

Treatment of Pulmonary Hypertension in COPD: Implications for Exercise Tolerance and Mortality

Treatment of Pulmonary Hypertension in COPD: Implications for Exercise Tolerance and Mortality