scholarly journals P167 Pulmonary hypertension in patients with systemic sclerosis: an audit of screening practices, incidence and cost over 10 years

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Luke Spray ◽  
Josephine Vila ◽  
Bridget Griffiths
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Annual Cost ◽  
Screening Programme ◽  
Total Annual Cost ◽  
Electronic Patient Records ◽  
Right Heart Catheterisation ◽  
Annual Screening ◽  
Pulmonary Arterial ◽  
Low Incidence

Abstract Background Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), a subtype of pulmonary hypertension (PH) which is not due to left-heart disease, chronic hypoxia or pulmonary arterial thrombus. The European Society of Cardiology recommends annual screening of patients with SSc due to the insidious presentation of PAH, poor outcomes, and the availability of effective treatments. Transthoracic echocardiography is the standard screening investigation, but NT pro-BNP, a biochemical marker of ventricular stretch, may be a cost-effective initial test with echocardiography reserved for patients with new or worsening symptoms or increasing NT pro-BNP. Our centre introduced NT pro-BNP as an adjunct to echocardiography in 2014. Methods We audited our SSc-PAH screening programme from 2009-2018 against the standard that every SSc patient should be screened annually with echocardiography or NT pro-BNP. Patients seen by the regional PH service prior to the first rheumatology clinic were excluded. We used our centre’s database of SSc patients and electronic patient records to determine if a patient had undergone PH screening. We calculated cost estimates from our hospital’s biochemistry and echocardiography departments. Results From 2009 to 2018, the number of SSc patients requiring annual screening rose from 81 to 215. In 2009, 65% of patients were screened - all with echocardiography. In 2018, 88% of patients were screened - 25% had an echocardiogram and 83% had a NT pro-BNP. 63% of patients were screened only through NT pro-BNP. Across the 1476 patient-years studied, only 6 new cases of PH were identified. PH was secondary to ILD in two cases, and true PAH in three cases (one patient refused diagnostic right-heart catheterisation). All three PAH diagnoses came from echocardiograms requested for worsening dyspnoea, so are not attributable to the screening programme. On 31 December 2018, 55% of these patients were taking phosphodiesterase 5 inhibitors (PDE-5i) for severe Raynaud’s phenomenon (50% on sildenafil and 5% on tadalafil). 4% of patients were prescribed bosentan and 3% of patients were prescribed dual therapy with a PDE5i and bosentan. This may explain our centre’s low incidence of PAH. The annual cost of screening per patient has dropped from £82 in 2014 to £59 in 2018, and the total annual cost has plateaued since 2014, despite rising patient numbers and improved screening rates. Conclusion Since introducing NT pro-BNP alongside echocardiography as a screening tool for PAH in SSc patients, we spend less on our screening programme per patient and achieve higher screening rates. However, in 10 years, our screening programme has not detected any asymptomatic cases of PAH, raising questions about the necessity of screening asymptomatic SSc patients. Widespread PDE-5i use may contribute to the low incidence of PAH in our cohort. Disclosures L. Spray None. J. Vila None. B. Griffiths None.

P3676Hemodynamic phenotypes in systemic sclerosis patients screened for pulmonary hypertension (PH): impact of the new definition of PH

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Xanthouli ◽  
N Milde ◽  
A M Marra ◽  
N Benjamin ◽  
C Nagel ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Functional Class ◽  
Walking Distance ◽  
Pulmonary Vascular Disease ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial ◽  
Definition Of ◽  
The Impact

Abstract Background Patients with systemic sclerosis (SSc) are at high risk of developing concomitant pulmonary hypertension (PH) which has a crucial impact on the patients' symptoms, quality of life and prognosis. Purpose The aim of this study was to analyse the impact of the new hemodynamic definition of precapillary PH as proposed at the 2018 World Symposium on PH in Nice in patients with SSc. Although recent data suggest that PVR >2 WU could be considered as abnormal in the new definition a conservative cut-off value of PVR ≥3 WU has been used. Methods SSc-patients were screened for PH using clinical assessments as WHO-functional class, 6 minute walking distance and right heart catheterisation. Patients were divided into hemodynamic subgroups according to their mean pulmonary arterial pressure (mPAP) values with mPAP ≤20 mmHg, 21–24 mmHg and ≥25 mmHg. These subgroups were further divided according to their pulmonary vascular resistance (PVR) with PVR <3 WU or ≥3 WU. Results One-hundred-twenty-two patients (79% female, age 57.6±12.7 years, 6MWD 439.5±98.1 meters, 70% diffuse cutaneous SSc, 30% limited cutaneous SSc, 53% WHO-FC II, 25% WHO-FC III) who were prospectively screened for PH were included in the analysis (Figure 1). Among them 26 had a symptomatic manifest PH using the cut-off value of mPAP ≥25 mmHg. Only half of this group presented with PVR ≥3 WU, the others had PVR <3 WU. Eight of these 26 PH-patients presented with PH due to left heart disease. Out of 21 patients with mildly elevated mPAP 21–24 mmHg, two (10%) met the new definition criteria of PH (pulmonary arterial wedge pressure <15 mmHg, mPAP 23 and 24 mmHg, PVR 3.0 and 3.2 WU, CI 2.2 L/min/m2 both, WHO-FC II both, respectively). Out of 75 patients with mPAP <21 mmHg, three presented with PVR ≥3 WU. Overview of systemic sclerosis patients Conclusions The new definition of precapillary pulmonary hypertension may on the one hand allow detecting an additional 10% of PH patients with mild elevated mPAP. On the other hand, eight of 13 patients (62%), who met the former definition of pulmonary arterial hypertension, would be classified as “normal” due to a lack of increase in PVR according to the new definition. The data of this study suggest that for SSc-patients the cut-off value of mPAP >20 mmHg is useful, but the criteria of PVR ≥3 WU may be too strict. Further studies with larger sample sizes will be needed to better characterise these hemodynamic subgroups and to define the extent of pulmonary vascular disease and treatability.

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.

Abstract 4383: Incidence of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis: A 3-Year Longitudinal Study

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Pascal de Groote ◽  
Eric Hachulla ◽  
Virginie Gressin ◽  
Jean Sibilia ◽  
Elisabeth Diot ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Doppler Echocardiography ◽  
Large Population ◽  
Pulmonary Pressure ◽  
Right Heart Catheterisation ◽  
Normal Mean ◽  
Pulmonary Arterial

We have previously determined a prevalence of 7.85% [95%CI: 5.70–10.00] of pulmonary arterial hypertension (PAH) in a large multicentre systemic sclerosis (SSc) population (the ItinerAIR Scleroderma registry). We used a screening algorithm based on dyspnea, Doppler echocardiographic evaluation of the peak velocity of tricuspid regurgitation (VTR) and right heart catheterisation (RHC). No study has determined the incidence of PAH in a large population at risk. To determine the incidence of PAH over 3 years of follow-up in the ItinerAIR SSc registry. Patients underwent regular screening for PAH with Doppler echocardiography confirmed by RHC in patients with VTR of 2.8–3 m/s and unexplained dyspnea, or VTR >3 m/s. 384 patients were followed for a mean of 41±5.6 months (median 41 months). The baseline characteristics of this population were: 87% female, mean age 53±12 years, mean duration of SSc at study entry 8.7±7.6 years, and 24.0% with diffuse SSc. Doppler echocardiography detected PAH in 18 patients (incidence: 1.37 per 100 patient-years). Among these, RHC identified 8 patients with pre-capillary PAH (incidence: 0.61 per 100 patient-years [95%CI: 0.26–1.20]), 8 with post-capillary pulmonary hypertension (PH) and 2 with PH due to severe interstitial lung disease. The 8 patients with pre-capillary PAH had moderate PAH (mean pulmonary pressure: 36±14.6 mmHg). Among the 8 patients with post-capillary PH, echocardiography was always considered normal by the expert cardiologist, except in 2 cases (moderate left atrial dilatation in one and mild aortic sclerosis in the other). These 8 patients had mild post-capillary PH, 5 of them having normal mean pulmonary pressure at rest but a significant increase during exercise with a pulmonary wedge pressure > 15 mmHg. In this large multicentre SSc cohort we prospectively found an incidence of PAH of 0.61 per 100 patient-years. We also demonstrated that RHC is still mandatory to precisely define the type of pulmonary hypertension since mild post-capillary PH without echocardiographic abnormalities are frequent.

scholarly journals Impact of the revised haemodynamic definition on the diagnosis of pulmonary hypertension in patients with systemic sclerosis

2019 ◽  
Vol 54 (2) ◽  
pp. 1900586 ◽  
Author(s):  
Sara Jaafar ◽  
Scott Visovatti ◽  
Amber Young ◽  
Suiyuan Huang ◽  
Paul Cronin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Lung Disease ◽  
Single Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Causes Of Mortality ◽  
Pulmonary Arterial ◽  
Definition Of ◽  
The Impact

IntroductionPulmonary arterial hypertension (PAH) is one of the leading causes of mortality in systemic sclerosis (SSc). We explored the impact of the updated haemodynamic definition of pulmonary hypertension (PH), as proposed by the 6th World Symposium on Pulmonary Hypertension.MethodsIn this single-centre retrospective analysis, patients with SSc who had right heart catheterisation (RHC) were included. We compared the prior PH definition to the updated PH definition. The prior definition classified PH as mean pulmonary arterial pressure (mPAP) ≥25 mmHg and further divided into pre-capillary PH (PAH and PH due to lung disease and/or hypoxia), post-capillary PH, and combined pre- and post-capillary PH groups. For the updated definition, PH was classified as mPAP >20 mmHg and further divided into the different groups. We validated our findings in the DETECT cohort.ResultsBetween 2005 and March 2019, 268 RHCs were performed in this single-centre cohort. Using the prior definition, 137 (51%) were diagnosed with PH, with 89 classified as pre-capillary PH (56 with PAH and 33 with PH due to lung disease and/or hypoxia), 29 as post-capillary PH, and 19 as combined pre- and post-capillary PH. When the updated definition was applied to the cohort, seven out of 131 (5%) with no PH were reclassified to pre-capillary PH (PAH (n=1), PH due to lung disease (n=3) and post-capillary PH (n=3)). In those with mPAP 21–24 mmHg, with no left heart or significant lung disease, one out of 28 (4%) in our cohort and four out of 36 (11%) in the DETECT cohort were reclassified as PAH.ConclusionThe updated PH definition does not appear to have a significant impact on the diagnosis of PH in two different screening cohorts.

scholarly journals Effect of Coronavirus Disease 2019 in Pulmonary Circulation. The Particular Scenario of Precapillary Pulmonary Hypertension

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 548 ◽  
Author(s):  
Jorge Nuche ◽  
Teresa Segura de la Cal ◽  
Carmen Jiménez López Guarch ◽  
Francisco López-Medrano ◽  
Carmen Pérez-Olivares Delgado ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Respiratory Infections ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The Elderly ◽  
Angiotensin Converting Enzyme 2 ◽  
Common Features ◽  
Pulmonary Vasodilators ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Low Incidence

The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease’s specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management.

scholarly journals Under pressure: pulmonary hypertension associated with left heart disease

2015 ◽  
Vol 24 (138) ◽  
pp. 665-673 ◽  
Author(s):  
Harrison W. Farber ◽  
Simon Gibbs
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Survival Rates ◽  
Left Ventricular ◽  
Left Heart ◽  
Mean Pulmonary Arterial Pressure ◽  
Left Heart Disease ◽  
Right Heart Catheterisation ◽  
Pulmonary Capillary ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) associated with left heart disease (PH-LHD) is the most common type of PH, but its natural history is not well understood. PH-LHD is diagnosed by right heart catheterisation with a mean pulmonary arterial pressure ≥25 mmHg and a pulmonary capillary wedge pressure >15 mmHg. The primary causes of PH-LHD are left ventricular dysfunction of systolic and diastolic origin, and valvular disease. Prognosis is poor and survival rates are low. Limited progress has been made towards specific therapies for PH-LHD, and management focuses on addressing the underlying cause of the disease with supportive therapies, surgery and pharmacological treatments. Clinical trials of therapies for pulmonary arterial hypertension in patients with PH-LHD have thus far been limited and have provided disappointing or conflicting results. Robust, long-term clinical studies in appropriate target populations have the potential to improve the outlook for patients with PH-LHD. Herein, we discuss the knowledge gaps in our understanding of PH-LHD, and describe the current unmet needs and challenges that are faced by clinicians when identifying and managing patients with this disease.

scholarly journals Right heart catheterisation: best practice and pitfalls in pulmonary hypertension

2015 ◽  
Vol 24 (138) ◽  
pp. 642-652 ◽  
Author(s):  
Stephan Rosenkranz ◽  
Ioana R. Preston
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Best Practice ◽  
Data Interpretation ◽  
Volume Measurement ◽  
Right Heart ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial

Right heart catheterisation (RHC) plays a central role in identifying pulmonary hypertension (PH) disorders, and is required to definitively diagnose pulmonary arterial hypertension (PAH). Despite widespread acceptance, there is a lack of guidance regarding the best practice for performing RHC in clinical practice. In order to ensure the correct evaluation of haemodynamic parameters directly measured or calculated from RHC, attention should be drawn to standardising procedures such as the position of the pressure transducer and catheter balloon inflation volume. Measurement of pulmonary arterial wedge pressure, in particular, is vulnerable to over- or under-wedging, which can give rise to false readings. In turn, errors in RHC measurement and data interpretation can complicate the differentiation of PAH from other PH disorders and lead to misdiagnosis. In addition to diagnosis, the role of RHC in conjunction with noninvasive tests is widening rapidly to encompass monitoring of treatment response and establishing prognosis of patients diagnosed with PAH. However, further standardisation of RHC is warranted to ensure optimal use in routine clinical practice.

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)

P4689How is the impact of updated hemodynamic definitions on frequencies of overall pulmonary hypertension and pre-capillary pulmonary hypertension as compared to those with previous criteria

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Tanyeri ◽  
B Keskin ◽  
O Y Akbal ◽  
A Hakgor ◽  
A Karagoz ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Critical Role ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Mean Pressure ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
The Impact ◽  
Wedge Pressure

Abstract Background and aim In this study we evaluated the impact of the updated pulmonary hypertension (PH) definitive criteria proposed in 6th World PH Symposium (WSPH) on numbers and frequencies of and pre- versus post-capillary PH as compared to those in European Society of Cardiology (ESC) 2015 PH Guidelines. Methods Study group comprised the retrospectively evaluated 1299 patients (pts) (age 53.1±18.8 years, female 807, 62.1%) who underwent right heart catheterisation (RHC) with different indications between 2006 and 2018. For ESC and WSPH PH definitions, pulmonary arterial mean pressure (PAMP) ≥25 mmHg (definition-A) and PAMP >20 mmHg (definition-B) RHC criteria were used, respectively. For pre-capillary PH definitions, pulmonary artery wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) ≥3 Wood units criteria were included in the both definitions. Results In RHC assessments, PAMP ≥25 mmHg and >20 mmHg were noted in 891 (68.6%) and 1051 (80.9%) of overall pts, respectively. Moreover, pre-capillary PH was diagnosed in 284 (21.8%) and 298 (22.9%) with definition-A and B, respectively. Although updated WSPH definition was associated with a net 12.3% and a relative 18% increase in the overall PH diagnosis, net and relative changes in the frequency of the pre-capillary PH were only 1% and 4.9%. Increase in the overall PH with updated WSPH criterias compared to previous ESC definitions was associated with increase in the number of pre-capillary PH (n=298, 22.9%) but not in the overall frequency of post-capillary PH (688, 52.9%). Because PVR was the product of the transpulmonary gradient (PAMP minus PAWP) divided by cardiac output, this measure was found to keep specificity for distinction between pre- versus post-capillary PH even after lowering thetreshold diagnostic for PAMP from 25 to 20 mmHg. Conclusions Although updated WSPH definition was associated with net 12.3% and relative 18% increase in the overall PH diagnosis, its impact on frequencies of pre- versus post-capillary PH within overall PH population was negligible.These seem to be due to critical role of PVR ensuring specificity in pre-capillary PH diagnosis even after lowering the definitive PAMP treshold to 20 mmHg.

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