Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry

2018 ◽  
Vol 46 (2) ◽  
pp. 176-183 ◽  
Author(s):  
Vivien M. Hsu ◽  
Lorinda Chung ◽  
Laura K. Hummers ◽  
Ami Shah ◽  
Robert Simms ◽  
...  
Keyword(s):  
Risk Factors ◽  
At Risk ◽  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Oxygen Desaturation ◽  
Heart Catheterization ◽  
Pericardial Effusions ◽  
Patients At Risk ◽  
Pulmonary Arterial ◽  
Male Sex

Objective.We sought to identify predictors of mortality and cardiopulmonary hospitalizations in patients at risk for pulmonary hypertension (PH) and enrolled in PHAROS, a prospective cohort study to investigate the natural history of PH in systemic sclerosis (SSc).Methods.The at-risk population for PH was defined by the following entry criteria: echocardiogram systolic pulmonary arterial pressure > 40 mmHg, or DLCO < 55% predicted or ratio of % forced vital capacity/%DLCO > 1.6, measured by pulmonary function testing. Baseline clinical measures were evaluated as predictors of hospitalization and death between 2005 and 2014. Cox proportional hazards models were censored at date of PH onset or latest study visit and adjusted for age, sex, race, and disease duration.Results.Of the 236 at-risk subjects who were followed for a median of 4 years (range 0.4–8.5 yrs), 35 developed PH after entering PHAROS (reclassified as PH group). In the at-risk group, higher mortality was strongly associated with male sex, low %DLCO, exercise oxygen desaturation, anemia, abnormal dyspnea scores, and baseline pericardial effusion. Risks for cardiopulmonary hospitalization were associated with increased dyspnea and pericardial effusions, although PH patients with DLCO < 50% had the highest risk of cardiopulmonary hospitalizations.Conclusion.Risk factors for poor outcome in patients with SSc who are at risk for PH were similar to others with SSc-PH and SSc-pulmonary arterial hypertension, including male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions. This group should undergo right heart catheterization and receive appropriate intervention if PH is confirmed.

scholarly journals Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Verônica Silva Vilela ◽  
Marcio Macri Dias ◽  
Ângelo Antunes Salgado ◽  
Bruno Rangel Antunes da Silva ◽  
Agnaldo José Lopes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Right Heart Catheterization ◽  
Diagnostic Tools ◽  
Heart Catheterization ◽  
Systematic Screening ◽  
Mean Pulmonary Arterial Pressure ◽  
Common Group ◽  
Pulmonary Arterial

Abstract Background Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. Methods A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. Results Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. Conclusions Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

Prevalence of Pulmonary Hypertension in Systemic Sclerosis in European Caucasians and Metaanalysis of 5 Studies

2010 ◽  
Vol 37 (11) ◽  
pp. 2290-2298 ◽  
Author(s):  
JÉRÔME AVOUAC ◽  
PAOLO AIRÒ ◽  
CHRISTOPHE MEUNE ◽  
LORENZO BERETTA ◽  
PHILIPPE DIEUDE ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Function Tests ◽  
Right Heart Catheterization ◽  
Advanced Age ◽  
Noninvasive Testing ◽  
Heart Catheterization ◽  
Alveolar Volume ◽  
Different Types ◽  
Pulmonary Arterial

Objective.To measure the prevalence of different types of pulmonary hypertension (PH) and to identify patients with systemic sclerosis (SSc) at highest risk in a multicenter European sample, with a metaanalysis of relevant studies.Methods.Consecutive patients with SSc recruited at 11 French and Italian centers underwent detailed evaluations, including Doppler echocardiography, chest computed tomography, pulmonary function tests, and right-heart catheterization (RHC), to detect the presence and causes of PH. A metaanalysis was performed, including data from 4 other studies.Results.Among 206 patients in whom it was suspected, PH was confirmed by RHC in 83 patients (7%). Precapillary PH was found in 64 patients (5%), of whom 42 had pulmonary arterial hypertension (PAH) and 22 had PH secondary to interstitial lung disease (ILD). RHC identified 17 patients (1%) with postcapillary PH secondary to left-heart disease. Patients with DLCO/alveolar volume < 70% were more likely to have precapillary PH (87.5% vs 42%; p < 0.0001). Precapillary and postcapillary PH were associated with advanced age (68 ± 14 vs 59 ± 12 yrs, p < 0.0001, and 74 ± 16 vs 61.5 ± 10 yrs, p < 0.0001, respectively). The metaanalysis of 3818 patients showed a prevalence of precapillary PH of 9% (95% CI 6%–12%) and identified advanced age, longer disease duration, and limited cutaneous disease subset as risk factors for this condition.Conclusion.The prevalence of precapillary PH in our multicenter study of SSc was 5%, and in the metaanalysis 9%. Our observations support use of RHC to confirm the presence of precapillary PH suspected by noninvasive testing. We also identified patients at high risk who should be carefully monitored.

Systemic sclerosis portends increased risk of conduction and rhythm abnormalities at diagnosis and during disease course: A US population-based cohort

2021 ◽  
pp. 239719832110340
Author(s):  
Yasser A Radwan ◽  
Reto D Kurmann ◽  
Avneek S Sandhu ◽  
Edward A El-Am ◽  
Cynthia S Crowson ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Confidence Interval ◽  
Cumulative Incidence ◽  
Disease Onset ◽  
Population Based ◽  
Hazard Ratio ◽  
Conduction Disorders ◽  
Increased Risk

Objectives: To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators. Methods: An incident cohort of patients with systemic sclerosis (1980–2016) from Olmsted County, MN, was compared to age- and sex-matched nonsystemic sclerosis subjects (1:2). Electrocardiograms, Holter electrocardiograms, and a need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: Seventy-eight incident systemic sclerosis cases and 156 comparators were identified (mean age 56 years, 91% female). The prevalence of any conduction disorder before systemic sclerosis diagnosis compared to nonsystemic sclerosis subjects was 15% versus 7% ( p = 0.06), and any rhythm disorder was 18% versus 13% ( p = 0.33). During a median follow-up of 10.5 years in patients with systemic sclerosis and 13.0 years in nonsystemic sclerosis comparators, conduction disorders developed in 25 patients with systemic sclerosis with cumulative incidence of 20.5% (95% confidence interval: 12.4%–34.1%) versus 28 nonsystemic sclerosis patients with cumulative incidence of 10.4% (95% confidence interval: 6.2%–17.4%) (hazard ratio: 2.57; 95% confidence interval: 1.48–4.45), while rhythm disorders developed in 27 patients with systemic sclerosis with cumulative incidence of 27.3% (95% confidence interval: 17.9%–41.6%) versus 43 nonsystemic sclerosis patients with cumulative incidence of 18.0% (95% confidence interval: 12.3%–26.4%) (hazard ratio: 1.62; 95% confidence interval: 1.00–2.64). Age, pulmonary hypertension, and smoking were identified as risk factors. Conclusion: Patients with systemic sclerosis have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to nonsystemic sclerosis patients. These findings warrant increased vigilance and screening for electrocardiogram abnormalities in systemic sclerosis patients with pulmonary hypertension.

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.

scholarly journals Prevalence and risk factors of pulmonary hypertension among adult patients with HIV infection in Ethiopia

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402097151
Author(s):  
Dawit Kebede Huluka ◽  
Desalew Mekonnen ◽  
Sintayehu Abebe ◽  
Amha Meshesha ◽  
Dufera Mekonnen ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Hypertension ◽  
Communicable Disease ◽  
Previous History ◽  
Systolic Pressure ◽  
Chronic Obstructive ◽  
Heart Catheterization ◽  
People Living With Hiv ◽  
Significant Difference ◽  
Living With Hiv

Globally, non-communicable diseases are increasing in people living with HIV. Pulmonary hypertension is a rare non-communicable disease in people living with HIV with a reported prevalence of <1%. However, data on pulmonary hypertension in people living with HIV from Africa are scarce and are non-existent from Ethiopia. This study aimed to examine the prevalence and severity of echocardiographic pulmonary hypertension and risk factors associated with pulmonary hypertension in people living with HIV in Ethiopia. A total of 315 consecutive adult people living with HIV followed at the Tikur Anbessa Specialized Hospital HIV Referral Clinic were enrolled from June 2018 to February 2019. Those with established pulmonary hypertension of known causes were excluded. A structured questionnaire was used to collect data on demographics, respiratory symptoms, physical findings, physician-diagnosed lung disease, and possible risk factors. Pulmonary hypertension was defined by a tricuspid regurgitant velocity of ≥2.9 m/sec on transthoracic echocardiography. A tricuspid regurgitant velocity ≥3.5, which translates into a pulmonary arterial pressure/right ventricular systolic pressure of ≥50 mmHg, was considered moderate-to-severe pulmonary hypertension. The mean age of the participants was 44.5 ± 9.8 years and 229 (72.7%) were females. Pulmonary hypertension was diagnosed in 44 (14.0%) of participants, of whom 9 (20.5%) had moderate-to-severe disease. In those with pulmonary hypertension, 17 (38.6%) were symptomatic: exertional dyspnea, cough, and leg swelling were seen in 12 (27.3%), 9 (20.5%), and 4 (9.1%), respectively. There was no significant difference in those with pulmonary hypertension compared to those without the disease by gender, cigarette smoking, previous history of pulmonary tuberculosis treatment, physician-diagnosed chronic obstructive pulmonary disease or bronchial asthma, duration of anti-retroviral therapy therapy or anti-retroviral regimen type. Pulmonary hypertension looks to be a frequent complication in people living with HIV in Ethiopia and is often associated with significant cardiopulmonary symptoms. Further studies using right heart catheterization are needed to better determine the etiology and prevalence of pulmonary hypertension in people living with HIV in Ethiopia compared to other countries.

Differential expression of hepatocyte growth factor in patients with systemic sclerosis-associated pulmonary arterial hypertension

2017 ◽  
Vol 2 (3) ◽  
pp. 225-230
Author(s):  
Yon K. Sung ◽  
Roham T. Zamanian ◽  
Catriona A. Wagner ◽  
William Robinson ◽  
Virginia Steen ◽  
...  
Keyword(s):  
At Risk ◽  
Systemic Sclerosis ◽  
Growth Factor ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Hepatocyte Growth Factor ◽  
Arterial Hypertension ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
Hepatocyte Growth

Introduction Non-invasive biomarkers are needed to identify pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) patients who may benefit from early intervention. We sought to identify novel cytokines that differentiate patients with incident SSc-PAH from those at high risk for PAH. Methods The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry is a multicenter registry of SSc patients at high risk for PAH (at-risk) or with incident right-heart catheterization-confirmed PAH (definite PAH). Serum from 10 at-risk and 9 definite PAH patients were profiled with Bio-PlexTM bead arrays for 48 cytokines and chemokines. We also evaluated the longitudinal change in cytokine profiles from 3 at-risk patients who subsequently developed definite PAH. Results Clinical features of at-risk versus definite PAH patients were not significantly different except for right-ventricular systolic pressure on echocardiogram (34 ± 7 vs. 45 ± 8 mmHg, p = 0.006), left atrial diameter (2.9 ± 0.5 vs. 3.7 ± 0.4 cm, p = 0.02), 6-minute walk distance (508 ± 115 vs. 393 ± 70 m, p = 0.02), mean pulmonary artery pressure (18 ± 4 vs. 32 ± 6 mmHg, p = 0.01), and pulmonary vascular resistance (111 ± 48 vs. 272 ± 109 dyn/s/cm5, p = 0.009). Serum cytokine profiling identified hepatocyte growth factor (HGF) as the only cytokine significantly different between the at-risk and definite PAH groups (225.8 ± 55.0 vs. 361.6 ± 164.5 pg/mL, q<0.1%). Profiling of longitudinal samples of at-risk to definite PAH patients did not identify any significant changes in HGF or other cytokines over time. Conclusions Definite PAH patients expressed higher levels of HGF than at-risk patients. Further studies are needed to clarify the utility of HGF as a predictive biomarker for SSc-PAH.

Abstract 19944: Targeted Therapy of Pulmonary Hypertension in Patients With Heart Failure and Preserved Ejection Fraction: Safety and Efficacy in Comparison to IPAH in Compera

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Stephan Rosenkranz ◽  
Marius M Hoeper ◽  
Doerte Huscher ◽  
David Pittrow ◽  
Christian F Opitz
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Ejection Fraction ◽  
Preserved Ejection Fraction ◽  
Evidence Based Treatment ◽  
Patients With Heart Failure ◽  
Venous Oxygen Saturation ◽  
Initial Ph ◽  
Pulmonary Arterial

Background: While targeted therapies are available for idiopathic pulmonary arterial hypertension (IPAH), evidence based treatment recommendations for pulmonary hypertension (PH) associated with heart failure and preserved ejection fraction (HFpEF) are lacking. Methods and Results: Out of 5,935 patients in the prospective COMPERA registry, we analyzed patients with “typical” IPAH (n=421, ≤2 of the following risk factors: BMI >30 kg/m2, hypertension, CAD, diabetes and atrial fibrillation at the time of diagnosis), “atypical” IPAH (n=139, >2 risk factors) or PH-HFpEF (n=226) who received targeted PH therapies. Patients with PH-HFpEF, when compared to “typical” and “atypical” IPAH were older (73±8 vs. 62±17 and 71±9 years), had a higher BMI (30 vs. 26 and 32 kg/m2), and more comorbidities (98% vs. 73% and 100%, all p<0.001), respectively. However, mean PAP (46±9 vs. 47±13 and 44±11 mmHg), cardiac index (2.2±0.7 vs. 2,3±0,8 and 2,2±0,8 l/min), and mixed venous oxygen saturation (62±7 vs. 62±10 and 63±9%, all ns) were almost identical. As compared to “typical” and “atypical” IPAH, PH-HFpEF patients had a higher PAWP (20±4 vs. 9±3 and 10±4 mmHg), resulting in a lower calculated PVR (559±270 vs. 861±477 and 784±844 dyn.s.cm-5). Survival at 1, 2 and 3 years post diagnosis was not different between groups. PDE-5 inhibitors were the most common form of initial PH treatment in PH-HFpEF (94%), and combination therapy was less common compared to “typical” or “atypical” IPAH at 1 year (7% vs. 44% and 26%). All 3 groups responded to targeted PH therapies at 12 months, while treatment effects were less pronounced in PH-HFpEF: Compared to baseline, the median increase of the 6MWD at 1 year was 29, 50, and 60 m, respectively. Treatment discontinuations occurred more frequently in patients with PH-HFpEF than in IPAH, either because of side effects or lack of improvement. Conclusions: Despite almost identical alterations of pulmonary artery pressure and cardiac output, patients with PH-HFpEF differed with respect to age, comorbidities and certain hemodynamic features when compared to “typical” or “atypical” IPAH. All groups responded to targeted PH therapy, however tolerability and efficacy of PH drugs were reduced in patients with PH-HFpEF while survival was not different.

Diagnosis of pulmonary hypertension associated whith systemic sclerosis

2020 ◽  
Vol 5-6 (215-216) ◽  
pp. 15-23
Author(s):  
Nazym Junusbayeva ◽  
◽  
Bakytsholpan Issayeva ◽  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Clinical Symptoms ◽  
Survival Rates ◽  
Early Death ◽  
Daily Practice ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
The Right

Systemic sclerosis is the most common autoimmune disease associated with pulmonary hypertension. Pulmonary hypertension is a potential predictor of early death, and therefore, recently, interest in a comprehensive study of the diagnosis of pulmonary hypertension associated with systemic sclerosis has been increasing among researchers. Aim. Consideration of current diagnostic issues, as well as the principles of using diagnostic algorithms to verify pulmonary hypertension associated with systemic sclerosis, applied in daily clinical practice. Material and methods. In order to study the literature data, a search was carried out for information on this problem up to 10 years in depth in the Web of Science, PubMed/MEDLINE. During the search, the following terms were used individually and in combination: "systemic sclerosis", "pulmonary hypertension", "DETECT algorithm", "catheterization of the right heart chambers". The main search criteria were studies based on the study of patients with pulmonary hypertension associated with systemic sclerosis: meta-analyzes, original studies, retrospective and cohort studies. Results and discussion. The clinical symptoms of pulmonary hypertension can be nonspecific, which greatly complicates the diagnosis of the disease in the early stages. The absence of a specific clinical picture before the development of an advanced stage of pulmonary hypertension leads to late verification of the diagnosis. Key algorithms used in the daily practice of a doctor make it possible to minimize the number of undiagnosed cases of pulmonary hypertension. Conclusions. Diagnosis of PH associated with systemic sclerosis is often challenging for clinicians to practice. Early diagnosis and therefore treatment of PH are of paramount importance as they improve survival rates in patients with systemic sclerosis. Keywords: systemic sclerosis, pulmonary hypertension, screening, DETECT algorithm, right heart catheterization.

Diagnosis and clinical investigation of patients presenting with pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Investigation ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Reference Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Invasive Test ◽  
Pulmonary Arterial

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.

Sign in / Sign up

Export Citation Format

Share Document