scholarly journals Exercise pulmonary haemodynamics predict outcome in patients with systemic sclerosis

2016 ◽  
Vol 48 (6) ◽  
pp. 1658-1667 ◽  
Author(s):  
Adriana Stamm ◽  
Stéphanie Saxer ◽  
Mona Lichtblau ◽  
Elisabeth D. Hasler ◽  
Suzana Jordan ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Artery ◽  
Cox Regression ◽  
Early Stage ◽  
Free Survival ◽  
Right Heart Catheterisation ◽  
Exercise Induced ◽  
Wedge Pressure

The aim of the present study was to investigate the prognostic value of exercise haemodynamics measured during right heart catheterisation (RHC) in patients with systemic sclerosis (SSc) referred for evaluation of pulmonary hypertension.SSc patients undergoing RHC at rest and during maximal supine incremental cycle exercise were grouped into resting precapillary pulmonary hypertension (PHrest) (mean pulmonary artery pressure (mPAP) ≥25 mmHg, pulmonary artery wedge pressure <15 mmHg), exercise-induced pulmonary hypertension (PHex) (mPAP ≥30 mmHg and mPAP/cardiac output >3 mmHg·L−1·min−1 at maximal exercise), and without pulmonary hypertension (PHnone). Patients' characteristics, haemodynamics and follow up data were compared between groups.72 SSc patients were followed for median (interquartile range) 33 (15–55) months. Mean (95% CI) survival without transplantation estimated by Kaplan-Meyer analysis was 4.4 (0.8–2.9) years in PHrest (n=17), 5.2 (4.4–6.1) years in PHex (n=28) and 9.5(8.4–10.6) years in PHnone (n=27; p<0.05 versus others). In Cox regression models, the exercise-induced increase in mPAP (hazard ratio (HR) 1.097, 95% CI 1.002–1.200) and the coefficient of pulmonary vascular distensibility alpha (HR 0.100, 95% CI 0.012–0.871) controlled for age, but not resting haemodynamics predicted transplant-free survival.Among SSc patients with normal mPAP at rest, an excessive increase in mPAP during exercise and an impaired vascular distensibility may indicate an early stage of pulmonary vasculopathy, associated with reduced survival similar to resting pulmonary hypertension patients.

P6478Resting and exercise pulmonary artery systolic pressure to rule out later development of pulmonary arterial hypertension in systemic sclerosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Gargani ◽  
V Codullo ◽  
P Argiento ◽  
A Moreo ◽  
F Pieri ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Peak Exercise ◽  
Exercise Doppler Echocardiography ◽  
Pulmonary Arterial ◽  
Further Development ◽  
Rule Out

Abstract Background Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), which is often diagnosed late when the benefits of vasoactive therapies are limited. The concept of exercise pulmonary hypertension as a possible transitional phase anticipating resting PAH has been assessed in several pathologies, but has not been endorsed by the latest European Guidelines, because not supported by sufficient data. Purpose To evaluate whether PASP values at rest and at peak exercise, estimated at echocardiography, could be predictors of further development of PAH. Methods Four hundred and twenty-nine SSc patients without a previous diagnosis of PAH, enrolled at 4 referral Centres, underwent standard exercise Doppler echocardiography with PASP estimation at rest and at peak stress. Patients were then followed-up to assess the development of PAH, as diagnosed by a complete diagnostic work-up including right heart catheterization. PAH was defined by pre-capillary pulmonary hypertension (mean pulmonary artery pressure ≥25 mmHg with pulmonary arterial wedge pressure ≤15 mmHg), without significant interstitial lung disease and/or left heart disease. Results During the median follow-up of 75 months (IQR 29–114), 16 patients developed PAH. A combined cut-off of ≥24 mmHg as resting PASP and ≥40 as peak PASP was identified as the best predictor of further development of PAH (see Figure). Both resting PASP and peak PASP were predictors of PAH at univariate analysis (resting PASP OR 1.13, 95% C.I. 1.07–1.19, p<0.0001; peak PASP OR 1.13, 95% C.I. 1.07–1.18, p<0.0001). At multivariate analysis, only peak PASP was independently associated to PAH development (OR 1.13, 95% C.I. 1.04–1.18, p<0.001). Only one patient among those with resting PASP <24 mmHg and peak PASP <40 mmHg (34.7% of the total population) developed PAH during the follow-up (after 10 years from normal exercise Doppler echocardiography). Kaplan-Meier curves Conclusions Exercise increase in PASP is an independent predictor of later development of PAH in SSc. An increase in exercise PASP is frequent and is not necessarily associated with a later development of PAH, whereas the very high negative predictive value of a normal PASP both at rest and at peak exercise can be used in the clinical practice to confidently rule out about one third of patients. Acknowledgement/Funding Italian Ministry of Health (Ricerca Finalizzata 2011-2012)

scholarly journals Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis

2018 ◽  
Vol 51 (4) ◽  
pp. 1701197 ◽  
Author(s):  
J. Gerry Coghlan ◽  
Matthias Wolf ◽  
Oliver Distler ◽  
Christopher P. Denton ◽  
Martin Doelberg ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Vena Cava ◽  
Diffusion Capacity ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Determining Factors ◽  
Pulmonary Arterial ◽  
Velocity Diffusion

The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide (DLCO) <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses.96 patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at baseline were followed for 2.95±0.7 years (median 3 years). Of these, 71 had a second RHC; 18 of these 71 patients (25.3%) developed PH, and five (7%) developed SSc-associated pulmonary arterial hypertension. For patients with an mPAP of 21–24 mmHg at baseline, the likelihood of presenting with PH as opposed to normal pressures on follow-up was significantly higher (p=0.026). Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up.In a selected cohort of SSc patients with a DLCO <60%, pulmonary pressures appeared to rise progressively during follow-up. In this population, it was possible to identify manifest PH in almost 25% of patients using prospective RHC during follow-up. Therefore, regular clinical assessment including RHC might be useful in patients with SSc.

scholarly journals Prognostic relevance of exercise pulmonary hypertension for new-onset atrial fibrillation in primary mitral regurgitation

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Teramoto ◽  
M Izumo ◽  
S Kuwata ◽  
R Kamijima ◽  
T Suzuki ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Pulmonary Hypertension ◽  
Mitral Regurgitation ◽  
Pulmonary Artery ◽  
Systolic Pulmonary Artery Pressure ◽  
Exercise Echocardiography ◽  
Primary Mitral Regurgitation ◽  
Exercise Induced ◽  
New Onset

Abstract Background/Introduction New-onset of atrial fibrillation (AF) portends poor prognosis in patients with primary mitral regurgitation (MR). However less is known about prognostic indicator for new-onset AF. Purpose The purpose of this study was to identify the prognostic relevance of exercise pulmonary hypertension for the new-onset AF in patients with primary MR. Methods Total of 114 consecutive patients with primary MR who underwent symptom-limited exercise echocardiography using supine-cycle ergometer were followed for new-onset AF over mean follow-up time of 3.6±2.6 years. Those with prevalent AF and pulmonary hypertension (estimated systolic pulmonary artery pressure ≥50mmHg) prior to exercise echocardiography were excluded from our analysis. We defined exercise-induced pulmonary hypertension (ExPHT) as those with peak estimated systolic pulmonary artery pressure (SPAP) ≥60mmHg or delta SPAP defined as differences between rest and peak SPAP ≥20mmHg. Results The mean age was 61±15 years old and 70 (61%) were male. Of those, 8 (7.8%) had mild MR, 32 (31.1%) had moderate MR, and 63 (61.2%) had severe MR. 60 (52.2%) patients had ExPHT. A total of 27 cases of new-onset AF were found during follow-up where the ExPHT group had higher prevalence of new-onset AF than the non-ExPHT group (35% vs. 11%, p=0.002). Those with ExPHT had significantly stronger association with shorter event-free survival time of new-onset AF (log-rank p&lt;0.001, Figure). When adjusted for age, sex, body surface area, the American Society of Echocardiography MR grade, left atrial dimension, peak systolic blood pressure and heart rate, the multivariable Cox regression analysis showed that those with ExPHT had a hazard risk of 3.1 ([95% CI 1.1–9.1], p=0.039) for new-onset of AF. Conclusions Exercise-induced pulmonary hypertension predicted incident of new-onset AF in those with primary MR. Exercise echocardiography is expected to play an important role in decision making with regards to the optimal timing for surgical intervention in primary MR. Figure 1 Funding Acknowledgement Type of funding source: None

scholarly journals Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry

2020 ◽  
Vol 55 (5) ◽  
pp. 1901747 ◽  
Author(s):  
Oksana A. Shlobin ◽  
Vasilis Kouranos ◽  
Scott D. Barnett ◽  
Esam H. Alhamad ◽  
Daniel A. Culver ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Cox Regression ◽  
Forced Expiratory Volume ◽  
Free Survival ◽  
International Registry ◽  
Kaplan Meier ◽  
Right Heart Catheterisation ◽  
Long Term Follow Up ◽  
Independent Risk Factors

IntroductionSarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients.MethodsReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival.ResultsData from 215 patients followed for a mean±sd 2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (DLCO) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.ConclusionReduced DLCO (<35% pred) and 6MWD (<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was identified as an independent risk factor for worsened outcomes.

Insights From the Fifth World Health Organization Symposium Working Group: Definitions and Diagnosis of Pulmonary Hypertension

2014 ◽  
Vol 13 (1) ◽  
pp. 27-30
Author(s):  
Fernando Torres
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Working Group ◽  
Venous Hypertension ◽  
World Health ◽  
Pulmonary Capillary ◽  
Exercise Induced ◽  
Definition Of ◽  
Health Organization ◽  
Wedge Pressure

During the Fifth World Symposium on Pulmonary Hypertension, the working group on diagnosis and assessment was charged with evaluating the definition of pulmonary arterial hypertension (PAH) as it was established at the Fourth World Symposium. The group also covered related topics such as “borderline PAH,” exercise-induced PAH, and issues surrounding the measurement of pulmonary capillary wedge pressure (PCWP). The working group's discussion specifically addressed the following questions:Should pulmonary hypertension (PH) continue to be defined by a resting mean pulmonary artery pressure (MPAP) ≥25 mm Hg, and should the term “borderline PH” be introduced?Should exercise-induced PH be included as a subset of PH?Should pulmonary vascular resistance (PVR) be reintroduced in the definition of PAH?Is pulmonary artery wedge pressure (PAWP) of 15 mm Hg adequate to distinguish between pre- and post-capillary PH, and how should it be measured?Should fluid or exercise challenge be used to distinguish patients with PAH from pulmonary venous hypertension (PVH)?Should exercise hemodynamics be used to unmask left sided heart failure?

Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

2014 ◽  
Vol 12 (4) ◽  
pp. 186-192 ◽  
Author(s):  
David Poch ◽  
Victor Pretorius
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Artery Wedge Pressure ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Wedge Pressure

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

scholarly journals SAT0339 NERVOUS SYSTEM INVOLVEMENT IN SYSTEMIC SCLEROSIS: A COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1115.2-1115
Author(s):  
T. Porel ◽  
S. DE Almeida Chaves ◽  
D. Adoue ◽  
L. Astudillo ◽  
D. Ribes ◽  
...  
Keyword(s):  
Nervous System ◽  
Systemic Sclerosis ◽  
Associated Factors ◽  
Cox Regression ◽  
Nervous System Disease ◽  
Cns Disease ◽  
System Involvement ◽  
System Disease ◽  
Disease Occurrence

Background:Nervous system involvement is considered to be rare in systemic sclerosis (SSc). Its prevalence is highly variable in SSc cohort studies and its prognosis is not well established.Objectives:To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) and central nervous system (CNS) disease in a cohort of systemic sclerosis patients.Methods:We have carried out a retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital, south west France. We included patients who met the following inclusion criteria: being over 18 years of age on diagnosis, meeting the ACR /EULAR 2013 classification criteria, being diagnosed after 01/01/1966 and before 31/12/2018, at least 12 months of follow-up. Patients were followed until 31/12/2019. Nervous system involvement associated with SSc was included when there was involvement on or after diagnosis and after exclusion of all other causes. Only symptomatic clinical involvement was included. Ischemic or hemorrhagic strokes were excluded. We calculated the incidence of CNS and/or PNS disease during the follow-up period per 1,000 person-years. Kaplan-Meier curves were plotted to determine the cumulative incidence of nervous system disease. We evaluated associated factors of CNS and/or PNS disease using multivariable Cox regression.Results:Of 447 SSc patients, 79.8% were female, 68 (15%) were diffuse cutaneous SSc, 342 (77%) were limited cutaneous SSc and 37 (8%) were sine scleroderma SSc. The mean ± SD age at diagnosis was 52.9 ± 14.3 years.During the study period, 82 (18%) patients experienced a PNS disease, 29 (6%) a CNS disease. The incidence was 28 per 1,000 patient-years of any nervous system disease, with 22 per 1,000 patient-years and 6 per 1,000 patient-years of PNS disease and CNS disease, respectively. The most frequent were carpal tunnel syndrome (63%) and polyneuropathies (12%) for PNS disease, and headache (45%) and seizures (10%) for CNS disease.Three significant independent associated factors with PNS disease occurrence were identified using multivariable Cox regression: BMI>23.1kg/m2(HR = 1.06 [1.01-1.12]), joint involvement (HR = 2.7 [1.3-5.5]), and an alteration in the left ventricular ejection fraction (HR = 3.8 [1.4-10.3]).Four significant independent associated factors with CNS disease occurrence were identified: age > 54 years (HR = 2.5 [1.1-6.0]), positive anti-PmScl testing (HR = 6.4 [1.5-28.2]), Caucasian origin (HR = 0.2 [0.1-0.5]) and hemoglobin < 12g/dl (HR = 0.2 [0.04-0.8]).Nervous system disease occurrence did not appear to have a negative impact on the survival of SSc patients (log-rank p=0.56).Conclusion:This study shows that specific nervous system disease in SSc is not uncommon and does not appear to increase mortality, but it could have an impact on functional prognosis and needs to be monitored.Disclosure of Interest:None declared

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