scholarly journals EXPRESS: IMPACT OF THE NEW DEFINITION FOR PULMONARY HYPERTENSION IN PATIENTS WITH LUNG DISEASE: AN ANALYSIS OF THE UNOS DATABASE

2021 ◽  
pp. 204589402199996
Author(s):  
Christopher King ◽  
Steven Nathan ◽  
Scott Barnett ◽  
Steeve Provencher ◽  
Joan Albert Barberà ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Lung Diseases ◽  
Right Heart Catheterization ◽  
Recent Change ◽  
Heart Catheterization ◽  
Copd Patients ◽  
Previous Definition ◽  
Definition Of ◽  
United Network

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.

scholarly journals Impact of Three Different Algorithms for the Screening of SSc-PAH and Comparison with the Decisions of a Multidisciplinary Team

Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1738
Author(s):  
Valentin Coirier ◽  
Céline Chabanne ◽  
Stéphane Jouneau ◽  
Nicolas Belhomme ◽  
Alice Ballerie ◽  
...  
Keyword(s):  
Multidisciplinary Team ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Tertiary Center ◽  
Previous Definition ◽  
Pulmonary Arterial ◽  
Definition Of ◽  
The Impact

Background: to compare three existing screening algorithms of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) with the results of a multidisciplinary team (MDT) meeting from a tertiary center. Methods: we conducted a monocentric longitudinal study from 2015 to 2018. All patients with SSc according to LeRoy’s classification were eligible. Patients were excluded in the case of missing data required by any of the three screening algorithms. The algorithms were applied for each patient at inclusion. Right heart catheterization (RHC) was performed based on the MDT decision. MDT members were all blinded from the results of the three algorithms regarding RHC recommendations. The RHC recommendations of each algorithm were compared with the MDT decision, and the impact on diagnosis and management was evaluated. Results: 117 SSc patients were consecutively included in the study, and 99 had follow-up data over the three-year duration of the study (10 deaths). Among the 117 patients, the MDT suggested RHC for 16 patients (14%), DETECT algorithm for 28 (24%), ASIG for 48 (41%) and ESC/ERS 2015 for 20 (17%). Among the 16 patients who had RHC, SSc-PAH was diagnosed in seven. Among patients with an initial recommendation of RHC based on at least one algorithm but not according to the MDT meeting, no SSc-PAH was diagnosed during the three-year follow-up. Results were unchanged when the new 2018 definition of PAH was applied instead of the previous definition. Conclusion: a MDT approach appears interesting for the screening of SSc-PAH, with a significant reduction of RHC performed in comparison with dedicated algorithms. The specific relevance of a MDT for the management and follow-up of patients with RHC recommended by existing algorithms but with no PAH warrants further studies.

scholarly journals Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402094411
Author(s):  
Bashar N. Alzghoul ◽  
Robert Hamburger ◽  
Thomas Lewandowski ◽  
Brandon Janssen ◽  
Daniel Grey ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Regression Analysis ◽  
Interstitial Lung Disease ◽  
Right Ventricular ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Linear Regression Analysis ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart

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.

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
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Cox Regression ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Systolic Pulmonary Artery Pressure ◽  
Hemodynamic Parameters ◽  
Rank Analysis ◽  
Minute Walk

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.

scholarly journals Combined pre- and post-capillary pulmonary hypertension defined by new criteria is worse prognosis group in patients with heart failure

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Ibe ◽  
H Wada ◽  
K Sakakura ◽  
Y Ugata ◽  
K Yamamoto ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Symptomatic Heart Failure ◽  
Kaplan Meier ◽  
Prognosis Group ◽  
Definition Of ◽  
New Criteria

Abstract Background The 6th World Symposium on Pulmonary Hypertension suggested major revision about definition of pulmonary hypertension (PH) as mean pulmonary artery pressure (mPAP) &gt;20 mmHg. The definitions of two subsets of PH due to left heart disease (PH-LHD) also revised. The aim of this study was to investigate clinical characteristics and outcomes of subsets of PH-LHD defined by new criteria. Methods We analyzed 701 patients admitted for symptomatic heart failure (NYHA ≥2) and underwent right heart catheterization at compensated stage between 2007 and 2016. These patients were divided into 4 groups as follows: (i) Isolated post-capillary PH (Ipc-PH); mPAP &gt;20 mmHg and pulmonary artery wedge pressure (PAWP) &gt;15 mmHg and pulmonary vascular resistance (PVR) &lt;3 WU, (ii) Combined pre- and post-capillary PH (Cpc-PH); mPAP &gt;20 mmHg and PAWP &gt;15 mmHg and PVR ≥3 WU, (iii) pre-capillary PH; mPAP &gt;20 mmHg and PAWP ≤15 mmHg, (iv) no PH; mPAP ≤20 mmHg. Kaplan-Meier curves were applied to investigate whether each groups predict heart failure (HF) death or HF readmission. Results The study patients were divided into Ipc-PH (n=268), Cpc-PH (n=54), Pre-capillary PH (n=112), and no PH (n=267). Cpc-PH was significantly associated with HF death or HF readmission as compared to other groups (Figure). Conclusions Cpc-PH defined by new criteria was significantly associated with poor long-term clinical outcomes, which suggests new criteria of two subsets of PH-LHD could be strict risk stratification for symptomatic heart failure. Figure1. Kaplan-Meier curves. Funding Acknowledgement Type of funding source: None

scholarly journals THE DIFFICULTIES IN THE DIAGNOSIS OF PULMONARY HYPERTENSION ASSOCIATED WITH CHRONIC LUNG DISEASE

2020 ◽  
Vol 73 (9) ◽  
pp. 1853-1860
Author(s):  
Sylwia Łukasik ◽  
Dariusz Łukasik ◽  
Michał Tomaszewski ◽  
Weronika Topyła ◽  
Agnieszka Wojtowska ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Lung Diseases ◽  
Clinical Symptoms ◽  
Vascular Pathology ◽  
Heart Catheterization ◽  
Chronic Lung Diseases ◽  
Pulmonary Arterial ◽  
Group 3

Introduction: Chronic lung disease (WHO group 3) is the second leading cause of pulmonary hypertension (PH). In turn, the development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. The aim: To analyse the difficulties in the diagnosis of pulmonary hypertension due to chronic lung disease. Review and Discussion: According to recent literature, PH in the course of lung diseases develops as a result of both “parenchymal” and vascular pathology in patients with a genetic predisposition. Prolonged infection (especially viral) may be an additional promoting factor. Elevation of pulmonary arterial pressure (PAP) is usually moderate and correlates with severity of lung disease. In a small minority, PAP may reach that seen in WHO group 1 pulmonary arterial hypertension (PAH). Conclusions: Echocardiography and right heart catheterization are the principal tools for the diagnosis of PH in chronic lung diseases. Unfortunately, current medications for treating PAH have not shown benefit in controlled trials of group 3 PH, hence their routine use is not recommended. Patients with severe group 3 PH should be considered for referral to expert centres or entry into clinical trials.

scholarly journals Characteristics of Patients Meeting the New Definition of Pre-capillary Pulmonary Hypertension (Nice 2018) in a Single Japanese Pulmonary Hypertension Center

2021 ◽  
Author(s):  
Keiko Yamamoto ◽  
Nobuhiro Tanabe ◽  
Yukiko Takahashi ◽  
Akira Naito ◽  
Ayumi Sekine ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Right Heart Catheterization ◽  
Pulmonary Vascular Disease ◽  
Heart Catheterization ◽  
Slight Elevation ◽  
Group 3 ◽  
Definition Of ◽  
Wedge Pressure ◽  
Better Than

Abstract Background: The 6th World Symposium on Pulmonary Hypertension (Nice 2018) proposed a new definition of pre-capillary pulmonary hypertension (PH) as a condition with mean pulmonary artery pressure (mPAP) > 20 mmHg, pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU). The characteristics and prognosis of patients with pre-capillary PH, according to this new definition, is unclear. Therefore, we determined the characteristics of patients with pre-capillary PH who had pulmonary vascular disease with slight elevation in mPAP.Methods: We retrospectively enrolled 683 patients who underwent their first right heart catheterization (RHC) at Chiba University, Japan. Among them, 489 patients met the pre-capillary PH requirement with mPAP ≥ 25 mmHg (conventional pre-capillary PH group), while 22 patients met the borderline pre-capillary PH criteria (borderline pre-capillary PH group). Results: The borderline pre-capillary PH group comprised 4.3% of the total pre-capillary PH patients, and the majority was in Group 3 (40.9%) or 4 (45.5%). The survival of the borderline pre-capillary PH group tended to be better than that of the conventional pre-capillary PH group. Conclusions: Pre-capillary PH patients, according to the Nice 2018 definition, comprised 4.3 % of the population with total pre-capillary PH and had better survival than the conventional PH pre-capillary group; moreover, the majority of the pre-capillary PH cases were either Group 3 and 4.

Sarcoidosis-Associated Pulmonary Hypertension

2017 ◽  
Vol 38 (04) ◽  
pp. 450-462 ◽  
Author(s):  
Oksana Shlobin ◽  
Robert Baughman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Lung Disease ◽  
Pulmonary Artery Pressure ◽  
Ct Scanning ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Double Blind ◽  
Artery Pressure ◽  
Fibrotic Lung Disease

AbstractSarcoidosis-associated pulmonary hypertension (SAPH) is found in 5 to 20% of sarcoidosis patients. Elevated pulmonary artery pressure may be due to multiple factors, including vasculocentric, parenchymal, and mechanical, as well as comorbidities such as cardiac sarcoidosis and sleep apnea. Most SAPH patients have fibrotic lung disease, but SAPH may be present in those without advanced parenchymal lung disease. Several features have been shown to suggest SAPH, including reduced DLCO, shortened 6-minute walk distance, with or without desaturation, and the presence of increased pulmonary artery to aorta ratio on CT scanning. Echocardiography remains an important tool for the evaluation of SAPH but may both over- or underestimate the severity of pulmonary artery pressure. Right heart catheterization remains the definitive test to make the diagnosis. There have been several reports on the value of different modalities of treating SAPH. These include prospective clinical trials and one double-blind placebo-controlled randomized trial. Evidence-based guidelines for treatment of SAPH are discussed in this review.

scholarly journals Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401983221
Author(s):  
Vasile Foris ◽  
Luka Brcic ◽  
Philipp Douschan ◽  
Gabor Kovacs ◽  
Elvira Stacher-Priehse ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Lung Fibrosis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Lung Function Test ◽  
Right Heart Catheterization ◽  
Smoking History ◽  
Heart Catheterization ◽  
Lung Pathology ◽  
Function Test

The diagnosis of idiopathic pulmonary arterial hypertension (iPAH) is complex and, besides invasive hemodynamic evaluation, includes several diagnostic steps to exclude any underlying diseases. The role of a decreased diffusion capacity of the lung for carbon monoxide (DLCO) is a matter of discussion. Here, we present a 76-year-old man with a smoking history of 30 pack-years who was diagnosed with iPAH after chronic thromboembolic pulmonary hypertension was excluded based on a negative perfusion scan, an underlying heart disease was excluded based on echocardiography and right heart catheterization, and a significant lung disease was excluded based on lung function test (FVC = 101% predicted, FEV1 = 104% predicted, FEV1/FVC = 77, TLC = 97% predicted) and thin-slice computed tomography (CT) scan. Just DLCO was reduced to 40% predicted, suggesting a possible structural lung disease. Postmortem examination demonstrated severe interstitial lung fibrosis combined with microscopic emphysema. This indicates that both CT imaging and pulmonary function test may be insensitive to a diffuse peripheral combined pattern of fibrosis and emphysema and that DLCO may be the only sensitive marker of this significant lung pathology.

scholarly journals Characteristics of patients meeting the new definition of pre-capillary pulmonary hypertension (Nice 2018) in a single Japanese pulmonary hypertension center

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Keiko Yamamoto ◽  
Nobuhiro Tanabe ◽  
Yukiko Takahashi ◽  
Akira Naito ◽  
Ayumi Sekine ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Right Heart Failure ◽  
Asian Country ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Significant Difference ◽  
Definition Of ◽  
Underlying Diseases

Abstract Background The 6th World Symposium on Pulmonary Hypertension (Nice 2018) proposed a new definition of pre-capillary pulmonary hypertension (PH) as a condition with mean pulmonary artery pressure (mPAP) > 20 mmHg, pulmonary artery wedge pressure  ≤ 15 mmHg, and pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU). The characteristics and prognosis of patients with pre-capillary PH, according to this new definition, is unclear. Therefore, we determined the characteristics and survival of patients with borderline pre-capillary PH. Methods We retrospectively enrolled 683 patients who underwent their first right heart catheterization at Chiba University, Japan. Among them, 489 patients met the pre-capillary PH requirement with mPAP ≥ 25 mmHg (conventional pre-capillary PH group), while 22 patients met the borderline pre-capillary PH criteria (borderline pre-capillary PH group). Additionally, 16 patients with a mean PAP of 20–25 and PVR of 2–3 WU were also examined. Results The borderline pre-capillary PH group comprised 4.3% of the total patients with pre-capillary PH, and the majority was in Group 3 (40.9%) or 4 (45.5%). The survival of the borderline pre-capillary PH group tended to be better than that of the conventional pre-capillary PH group. The prognosis of Group3 PH was the worst among the patients with borderline precapillary PH. There was no significant difference in survival between the borderline pre-capillary PH group with PVR ≥ 3 WU and that with PVR of 2–3 2WU, although none of the patients in the latter group died due to right heart failure. Conclusions This is the first study conducted in a PH center in an Asian country to reveal the characteristics of patients with pre-capillary PH, according to the Nice 2018 definition. They comprised 4.3% of the total population with pre-capillary PH, and the majority of the pre-capillary PH cases were in either Group3 or 4. The prognosis may be affected by the patients’ underlying diseases. Further prospective studies are needed to determine whether the new definition, including the PVR cut-off, is beneficial in clinical practice.

Pathology and Pathobiology of Pulmonary Hypertension

2017 ◽  
Vol 38 (05) ◽  
pp. 571-584 ◽  
Author(s):  
Peter Dorfmüller ◽  
Christophe Guignabert
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Inflammation ◽  
Right Heart Catheterization ◽  
Point Of View ◽  
Heart Catheterization ◽  
Pulmonary Endothelium ◽  
Hemodynamic State ◽  
Pulmonary Arterial ◽  
Genetic And Environmental Factors ◽  
And Migration

Pulmonary hypertension (PH) is a hemodynamic state defined by a mean pulmonary artery pressure ≥ 25 mm Hg during resting right heart catheterization. PH can result from precapillary (arterial) or postcapillary (venous) pathophysiological mechanisms. Interestingly, recent PH pathology has shown that pulmonary arterial or pulmonary venous remodelling are rarely independent phenomena, but frequently occur in combined fashion in lungs from patients suffering from different forms of PH, including pulmonary arterial hypertension (PAH). In PAH, it is now becoming clear that aberrant signals present in vessel wall microenvironment, which is largely orchestrated by dysfunctional pulmonary endothelial cells, are key contributors of the pulmonary vascular remodeling process, fostering proliferation, and survival and migration of resident pulmonary vascular cells such as smooth muscle cells, myofibroblasts, and pericytes. In addition, both genetic and environmental factors are also critical in the development of pulmonary vascular inflammation and chronic impairment of the pulmonary endothelium. This article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

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