scholarly journals Predictors of Mortality in Patients with Interstitial Lung Disease-Associated Pulmonary Hypertension

2020 ◽  
Vol 9 (12) ◽  
pp. 3828
Author(s):  
Esam H. Alhamad ◽  
Joseph G. Cal ◽  
Nuha N. Alrajhi ◽  
Waleed M. Alharbi

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial lung disease (ILD) patients. The aim of this study is to investigate the physiological and hemodynamic parameters that predict mortality in patients with ILD-PH. Methods: Consecutive ILD patients who underwent right heart catheterization (n = 340) were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival. Results: In total, 96 patients had PH and an additional 56 patients had severe PH. The overall survival of idiopathic pulmonary fibrosis (IPF) patients with PH was significantly worse than the survival of patients with other types of ILD with PH (p < 0.0001 by log-rank analysis). Patients with a reduced diffusing capacity of the lung for carbon monoxide (DLco) (<35% predicted), six-minute walk test final oxygen saturation by pulse oximetry (SpO2) < 88% and pulmonary vascular resistance ≥4.5 Wood units in the ILD-PH cohort had significantly worse survival. IPF diagnosis, forced vital capacity, DLco, systolic pulmonary artery pressure and cardiac index were identified as independent predictors of survival among the ILD-PH cohort. Conclusions: Patients with ILD-PH have poor prognosis. Physiological and hemodynamic parameters were important factors independently associated with outcome.

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402094411
Author(s):  
Bashar N. Alzghoul ◽  
Robert Hamburger ◽  
Thomas Lewandowski ◽  
Brandon Janssen ◽  
Daniel Grey ◽  
...  

Pulmonary hypertension in interstitial lung diseases is associated with increased mortality and hospitalizations and reduced exercise capacity. Interstitial pneumonia with autoimmune features (IPAF) is a recently described interstitial lung disease. The characteristics of pulmonary hypertension in IPAF patients are unknown. We sought to characterize patients with IPAF based on their echocardiographic probability of pulmonary hypertension and compare patients with and without pulmonary hypertension identified by right heart catheterization. We conducted a retrospective study of patients seen in the interstitial lung disease clinic from 2015 to 2018. Forty-seven patients with IPAF were identified. Patients were classified into low, intermediate and high echocardiographic pulmonary hypertension probabilities. A sub-group analysis of patients with pulmonary hypertension and without pulmonary hypertension (IPAF-PH vs. IPAF-no PH) identified by right heart catheterization was also performed. Linear regression analysis was performed to study the association between 6-min-walk-distance (6MWD) and pulmonary vascular resistance (PVR) while adjusting for age and body mass index. Right ventricular hypertrophy (>5 mm), right ventricular enlargement (>41 mm) and right ventricular systolic dysfunction defined as fractional area change% ≤35 was present in 76%, 24%, and 39% of patients, respectively. Pulmonary hypertension was identified in 12.7% of patients. IPAF-PH patients had higher mean pulmonary artery pressure and lower cardiac output compared to the IPAF-no PH group (34 mmHg vs. 19 mmHg, p = 0.002 and 4.0 vs. 5.7 L/min, p = 0.023, respectively). Lower 6MWD was associated with higher PVR on regression analysis ( p = 0.002). Pulmonologists should be aware that a significant number of IPAF patients may develop pulmonary hypertension. Reduced 6MWD may suggest the presence of pulmonary hypertension in IPAF patients.


2021 ◽  
Author(s):  
Camila Farnese Rezende ◽  
Eliane Viana Mancuzo ◽  
Maria Carmo P Nunes ◽  
Ricardo de Amorim Corrêa

Abstract Purpose: Studies in the context of research have shown a significant disagreement between the echocardiographic measurement of the systolic pulmonary artery pressure (sPAP) and right atrial pressure (RAP) and that obtained by right heart catheterization (RHC). We compare measurements of sPAP and RAP, verified by transthoracic echocardiogram (TTE) with those detected by RHC in patients being investigated for pulmonary hypertension (PH). Methods: Cross-sectional study was performed in a context of usual clinical practice in the public referral center for PH, including patients with high or intermediate echocardiographic probability of pulmonary arterial hypertension and chronic thromboembolic PH. Bland-Altman test was used to assess the agreement amongthe values and ROC curve to identify sPAP and tricuspid regurgitation velocity (TRV) values ​​with better accuracy. Clinically acceptable differences of 10 mmHg for sPAP and 5 mmHg for RAP were considered.Results: Ninety-five patients were included. The Bland-Altman analysis showed a bias of 8.03 mmHg for sPAP and -3.30 mmHg for RAP. Area under the curve for sPAP and TRV measured by TTE were 0.936(95% CI: 0.836-1.0) and 0.919(95% CI: 0.837-1.0), respectively. According to the pre-defined pressure differences, only 33.4% of the echocardiographic estimate of sPAP and 55.1% of RAP were accurate, as compared to the measurements obtained by RHC. Conclusions: Real life study revealed that echocardiographic evaluation demonstrated a high discriminatory power for diagnosis of PH, but sPAP and RAP measurements showed significant disagreements in relation to hemodynamic measures. The technical improvement of diagnostic services may contribute to the earlier recognition of this condition by TTE.


2009 ◽  
Vol 36 (2) ◽  
pp. 330-336 ◽  
Author(s):  
MARGARET C. GARIN ◽  
KRISTIN B. HIGHLAND ◽  
RICHARD M. SILVER ◽  
CHARLIE STRANGE

Objective.To determine factors that influence 6-minute walk distance (6MWD) in patients with scleroderma (systemic sclerosis, SSc)-interstitial lung disease (ILD), SSc-pulmonary hypertension (PH), and idiopathic pulmonary fibrosis (IPF).Methods.We retrospectively evaluated all patients with SSc or IPF who performed a 6-minute walk test (6MWT) at a university hospital between 1999 and 2003. Chi-square, ANOVA, simple linear regression, and backwards elimination multivariable regressions were performed.Results.Forty-eight consecutive IPF patients with 6MWT were compared to 33 patients with SSc-ILD, 13 with SSc-PH, 19 with both SSc-ILD and SSc-PH (SSc-Both), and 15 with SSc without ILD or PH (SSc-Neither). Mean 6MWD did not differ between groups. Limitations to 6MWT trended toward dyspnea in IPF and lower extremity pain in SSc. SSc-Both had dyspnea limitation more than other SSc subgroups (p = 0.017). Percentage predicted forced vital capacity (FVC%) and percentage predicted carbon monoxide diffusing capacity (DLCO%) were more strongly predictive of 6MWD in IPF than in SSc; however, exclusion of SSc subjects with pain limitation improved the predictive value. Significant correlates of 6MWD in multivariable analysis differed between subgroups.Conclusion.Pain limitations confound the utility of the 6MWT, particularly in SSc. Pain may cause failure to reach a dyspnea limitation during 6MWT, especially in SSc patients without both ILD and PH. Correlates of 6MWD differ between SSc subgroups and IPF; therefore, the 6MWT distance is not always reflective of the same physiological process. 6MWT interpretation should include consideration of vascular, pulmonary, and musculoskeletal exercise limitations.


2010 ◽  
Vol 46 (3) ◽  
pp. 122-128 ◽  
Author(s):  
Isabel Blanco ◽  
Claudio Villaquirán ◽  
José Luis Valera ◽  
María Molina-Molina ◽  
Antoni Xaubet ◽  
...  

2021 ◽  
Vol 21 (02) ◽  
pp. 2150016
Author(s):  
STEPHANIE M. GEORGE ◽  
DANIEL P. PEARCE ◽  
LAQUANDA FREDERICKS ◽  
C. BOGDAN MARCU ◽  
VEERANNA MADDIPATI

Pulmonary hypertension (PH) is diagnosed invasively by right heart catheterization (RHC), which determines patient’s mean and systolic pulmonary artery pressure (mPAP, sPAP) and pulmonary vascular resistance (PVR). This study sought to identify non-invasive echocardiography parameters useful for screening PH. Patients ([Formula: see text]; 19 normotensive; 17 pre-capillary PH; 48 post-capillary PH) who had undergone transthoracic Doppler echocardiography and RHC within 60 days of each other were identified. Tricuspid regurgitant (TR) jet velocities, velocity spectral densities, average flow rates, and Fourier transforms (FFT) of velocity waveforms were calculated via an in-house MATLAB code. Correlations were found between the FFT magnitude at 0 Hz and sPAP and mPAP for normotensive patients; between the MATLAB-calculated TR jet and sPAP and PVR for all PH patients; and between the sum of FFT magnitudes [Formula: see text][Formula: see text]Hz and mPAP, sPAP, and PVR for post-capillary PH patients. Statistical difference was found between the FFT magnitudes at 2 Hz of pre- and post-capillary PH patients ([Formula: see text]). These results suggest non-invasive parameters with clinical utility for estimating RHC measurements and discriminating between PH types, offering a path forward for less invasive and more accessible PH screening protocol.


2021 ◽  
pp. 204589402199996
Author(s):  
Christopher King ◽  
Steven Nathan ◽  
Scott Barnett ◽  
Steeve Provencher ◽  
Joan Albert Barberà ◽  
...  

The implications of the recent change in the definition of pulmonary hypertension (PH) on epidemiology and outcomes are not known. We sought to determine the percentage of patients with the two most common lung diseases that would be reclassified regarding the presence/absence of PH with the revised definition. A query of the United Network for Organ Sharing database was performed. The percentage of patients meeting the current and previous definition of PH were described. Outcomes of patients stratified by the current and previous definitions were compared. There were 15,563 patients with right heart catheterization data analyzed. PH was more prevalent in both COPD and IPF under the new definition at 52.4% versus 82.4%, and 47.6% versus 73.6%, respectively. “Pre-capillary” PH by the new definition was lower at 28.1% for COPD and 36.8% for IPF. Of the patients with PH by the old definition, 23.9% of COPD patients and 18.7% of IPF patients were not classified as PH by the new definition. Conversely, 15.9% of COPD patients and 15.1% of IPF patients who did not meet diagnostic criteria for PH by the old definition, did have PH by the new definition. Patients in both disease categories had shorter transplant-free waitlist survival in the presence of PH by both the new and old definitions. There was a trend toward the new definition of pre-capillary PH better discerning outcomes compared to the old definition of PH in IPF patients. Most patients with advanced lung disease who are listed for lung transplantation have PH, but fewer have pre-capillary PH than PH by the old definition. Both the old and new definition of precapillary PH appear to discern outcomes among the two groups of lung disease analyzed, with some evidence to suggest that the new definition performs slightly better in the IPF population.


2021 ◽  
Vol 12 ◽  
Author(s):  
Xianqiu Chen ◽  
Jian Guo ◽  
Dong Yu ◽  
Bing Jie ◽  
Ying Zhou

Background: Progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) share similar progression phenotype but with different pathophysiological mechanism. The purpose of this study was to assess clinical characteristics and outcomes of patients with PF-ILD in a single-center cohort.Methods: Patients with PF-ILD treated in Shanghai Pulmonary Hospital from Jan. 2013 to Dec. 2014 were retrospectively analyzed. Baseline characteristics and clinical outcomes were collected for survival analysis to identifying clinical predictors of mortality.Results: Among 608 patients with ILD, 132 patients met the diagnostic criteria for PF-ILD. In this single-center cohort, there were 51 (38.6%) cases with connective tissue disease-associated interstitial lung disease (CTD-ILD) and 45 (34.1%) with unclassifiable ILDs. During follow-up, 83 patients (62.9%) either died (N = 79, 59.8%) or underwent lung transplantations (N = 4, 3.0%) with a median duration follow-up time of 53.7 months. Kaplan-Meier survival curves revealed that the 1, 3 and 5-years survival of PF-ILD were 90.9, 58.8 and 48.1%, respectively. In addition, the prognosis of patients with PF-ILD was similar to those with IPF, while it was worse than non-PF-ILD ones. Multivariate Cox regression analysis demonstrated that high-resolution computed tomography (HRCT) scores (HR 1.684, 95% CI 1.017–2.788, p = 0.043) and systolic pulmonary artery pressure (SPAP) &gt; 36.5 mmHg (HR 3.619, 95%CI 1.170–11.194, p = 0.026) were independent risk factors for the mortality of PF-ILD.Conclusion: Extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
N Seidova ◽  
A Manouras ◽  
L H Lund ◽  
A Venkateshvaran

Abstract Background Both the ratio between tricuspid annular plane systolic excursion (TAPSE) and doppler estimated systolic pulmonary artery pressure (SPAP) and right ventricular longitudinal strain (RVSL) have been proposed as sensitive measures of RV contractile function and demonstrate important prognostic implications in heart failure (HF). However, a comparison between measurements to discern pulmonary hypertension (PH) in heart failure has not been adequately explored. Methods Consecutive subjects undergoing right heart catheterization (RHC) and simultaneous echocardiography to evaluate HF were enrolled. Subjects with atrial fibrillation, recent acute coronary syndrome and poor diagnostic images were excluded. RV-PA coupling was assessed based on the ratio between invasively derived RV elastance and arterial elastance (Ees/Ea) and correlated with TAPSE/RVSP and speckle-tracking derived RVSL. ROC analysis was performed to identify PH, defined as invasive PA mean pressure ≥25mmHg. Results 99 subjects were analysed (age: 59 ± 14; 49% female). Both TAPSE/RVSP (r = 0.47; p &lt; 0.001) and RVSL (r = 0.48; p &lt; 0.001) demonstrated significant associations with invasive Ees/Ea. TAPSE/RVSP was significantly lower in PH when compared with non-PH subjects (0.42 ± 0.27 vs 0.65 ± 0.34mm/mmHg; p &lt; 0.001) and demonstrated strong differentiating ability (AUC = 0.80) for PH. RVSL, however, did not significantly differ between groups (p &gt; 0.05). Conclusions Both TAPSE/RVSP and RVSL demonstrate significant association with invasive measures of RV-PA coupling. The stronger ability of TAPSE/RVSP to distinguish PH as compared with RVSL may be attributed to the lower load-dependency of this measure.


2018 ◽  
Vol 2018 ◽  
pp. 1-10 ◽  
Author(s):  
Marcin Waligóra ◽  
Anna Tyrka ◽  
Piotr Podolec ◽  
Grzegorz Kopeć

Introduction. Several diagnostic tests have been recommended for risk assessment in pulmonary hypertension (PH), but the role of electrocardiography (ECG) in monitoring of PH patients has not been yet established. Therefore the aim of the study was to evaluate which ECG patterns characteristic for pulmonary hypertension can predict hemodynamic improvement in patients treated with targeted therapies. Methods. Consecutive patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) were eligible to be included if they had had performed two consecutive right heart catheterization (RHC) procedures before and after starting of targeted therapies. Patients were followed up from June 2009 to July 2017. ECG patterns of right ventricular hypertrophy according to American College of Cardiology Foundation were assessed. Results. We enrolled 80 patients with PAH and 11 patients with inoperable CTEPH. The follow-up RHC was performed within 12.6±10.0 months after starting therapy. Based on median change of pulmonary vascular resistance, we divided our patients into two subgroups: with and without significant hemodynamic improvement. RV1, max⁡RV1,2 + max⁡SI,aVL-SV1, and PII improved along with the improvement of hemodynamic parameters including PVR. They predicted hemodynamic improvement with similarly good accuracy as shown in ROC analysis: RV1 (AUC: 0.75; 95% CI: 0.63–0.84), PII (AUC: 0.67, 95% CI: 0.56–0.77), and max⁡RV1,2+max⁡SI,aVL-SV1 (0.73; 95% CI: 0.63–0.82). In Cox regression only change in RV1 remained significant mortality predictor (HR: 1.12, 95% CI: 1.01–1.24). Conclusion. Electrocardiogram may be useful in predicting hemodynamic effects of targeted therapy in precapillary pulmonary hypertension. Decrease of RV1, max⁡RV1,2+max⁡SI,aVL-SV1, and PII corresponds with hemodynamic improvement after treatment. Of these changes a decrease of R wave amplitude in V1 is associated with better survival.


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