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 Improvements in French risk stratification score were correlated with reductions in mean pulmonary artery pressure in pulmonary arterial hypertension: a subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuichi Tamura ◽  
◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract Background Since there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort. Methods We enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements. Results The ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p = 0.03) and with the improvements in mPAP (p < 0.001). Conclusion The improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

scholarly journals Improvements in French Risk Stratification Score were Correlated with Reductions in Mean Pulmonary Artery Pressure in Pulmonary Arterial Hypertension: A Subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2020 ◽  
Author(s):  
Yuichi Tamura ◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
Hiroaki Miyata ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract BackgroundSince there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort.MethodsWe enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements.ResultsThe ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p=0.03) and with the improvements in mPAP (p<0.001).ConclusionThe improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

scholarly journals Improvements in French Risk Stratification Score were Correlated with Reductions in Mean Pulmonary Artery Pressure in Pulmonary Arterial Hypertension: A Subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2020 ◽  
Author(s):  
Yuichi Tamura ◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
Hiroaki Miyata ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract Background Since there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort.Methods We enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements.Results The ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p = 0.03) and with the improvements in mPAP (p < 0.001).Conclusion The improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

scholarly journals Improvements in French Risk Stratification Score were Correlated with Reductions in Mean Pulmonary Artery Pressure in Pulmonary Arterial Hypertension: A Subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2020 ◽  
Author(s):  
Yuichi Tamura ◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
Hiroaki Miyata ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract BackgroundSince there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort.MethodsWe enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements.ResultsThe ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p=0.03) and with the improvements in mPAP (p<0.001).ConclusionThe improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

scholarly journals Improvements in French Risk Stratification Score were Correlated with Reductions in Mean Pulmonary Artery Pressure in Pulmonary Arterial Hypertension: A Subanalysis of the Japan Pulmonary Hypertension Registry (JAPHR)

2021 ◽  
Author(s):  
Yuichi Tamura ◽  
Hiraku Kumamaru ◽  
Kohtaro Abe ◽  
Toru Satoh ◽  
Hiroaki Miyata ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arterial ◽  
The Relationship ◽  
Risk Stratification Score

Abstract BackgroundSince there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort.MethodsWe enrolled 108 patients with PAH from JAPHR from previous reported cohort and analyzed the relations between French Risk Stratification scores and hemodynamic improvements.ResultsThe ratio meeting 0 to 4 French Risk Stratification score was 21.3%, 31.5%, 32.4%, 13.0%, and 1.9% at baseline, and 6.5%, 23.2%, 33.3%, 23.2%, 13.9% at follow-up, respectively. The improvements in the number of criteria met were associated both with mPAP at follow-up (p=0.03) and with the improvements in mPAP (p<0.001).ConclusionThe improvements in French Risk Stratification may become a marker of improved hemodynamics including mPAP.

Pulmonary Hypertension

2013 ◽  
Author(s):  
George K Istaphanous ◽  
Andreas W Loepke
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Pulmonary Artery Pressure ◽  
Disease Process ◽  
Underlying Disease ◽  
Artery Pressure ◽  
Pulmonary Arterial ◽  
Made In

Pediatric pulmonary arterial hypertension (PAH) is characterized by a pathologically elevated pulmonary artery pressure in children. The etiology of PAH is multifactorial, and while its prognosis is closely related to the reversibility of the underlying disease process, much progress has recently been made in its diagnosis and treatment, significantly decreasing the associated morbidity and mortality.

P6479Diagnostic and prognostic significance of serum levels of selenoprotein P in patients with pulmonary arterial hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
N Kikuchi ◽  
K Satoh ◽  
T Satoh ◽  
J Omura ◽  
R Kurosawa ◽  
...  
Keyword(s):  
Experimental Study ◽  
Clinical Study ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Composite Endpoint ◽  
Serum Levels ◽  
Selenoprotein P ◽  
Pulmonary Arterial ◽  
Novel Therapeutic

Abstract Background Despite the recent progress in upfront combination therapy for pulmonary arterial hypertension (PAH), a useful biomarker for the disorder still remains to be developed. Selenoprotein P (SeP) is a glycoprotein secreted mainly from hepatocytes but also from other various kinds of cells, including pulmonary artery smooth muscle cells (PASMCs), to maintain selenium homeostasis and cellular energy metabolism. We have recently demonstrated that SeP expression in PASMCs is markedly up-regulated in PAH patients and plays crucial roles in the pathogenesis of the disorder. In this study, we thus examined whether serum levels of SeP could be a useful biomarker for the disorder. Methods In the experimental study, we performed gene expression microarray and in silico analyses to identify a novel therapeutic target for PAH. We also used the lung, serum, and cultured PAMSCs derived from patients with PAH for mechanistic experiments. In the clinical study, we enrolled a total of 65 consecutive patients with PAH who underwent right heart catheterization for hemodynamic assessment. We measured serum SeP levels and evaluated their prognostic impacts during follow-up (mean 1,520 days, IQR: 1,393–1,804 days). Serum SeP level was measured using a newly developed sol particle homogeneous immunoassay. As controls, we collected serum samples from 20 controls without any known cardiac disorders evaluated by hematological examination, echocardiography, and coronary angiography. In PAH patients, we examined the relationship between baseline SeP levels and composite endpoint of all-cause death and lung transplantation. The correlation between the absolute changes in SeP and those in hemodynamic parameters during follow-up were also examined. Results In the experimental study, SeP promoted PASMC proliferation through increased oxidative stress and mitochondrial metabolic dysfunction, which were associated with activated HIF-1α and dysregulated glutathione metabolism. In the clinical study, PAH patients showed significantly higher levels of serum SeP compared with controls (3.07±0.57 vs. 2.43±0.25 mg/L, P<0.0001). Higher SeP levels (cut-off point, 3.47 mg/L) were significantly associated with the composite endpoint of all-cause death and lung transplantation in PAH patients [HR: 4.85 (1.42 to 16.6), P<0.01]. Importantly, we found that absolute changes in SeP levels in PAH patients significantly correlated with those in mean pulmonary artery pressure, pulmonary vascular resistance, and cardiac index in response to PAH-specific therapy (R=0.78, 0.76, and −0.71, respectively, all P<0.0001). Furthermore, the increases in SeP levels during follow-up predicted the poor outcome in PAH patients [Figure, HR: 4.29 (1.27 to 14.4), P<0.05]. Figure 1 Conclusions These results indicate that SeP is a novel therapeutic target of PAH and that serum SeP levels are a novel biomarker for diagnosis and assessment of treatment efficacy and long-term prognosis in PAH patients. Acknowledgement/Funding Grants-in-aid for Scientific Research from the Japan Agency for Medical Research and Development, Tokyo, Japan (16ek0109176h0001, 17ek0109227h0001).

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