scholarly journals Risk assessment in medically treated chronic thromboembolic pulmonary hypertension patients

2018 ◽  
Vol 52 (5) ◽  
pp. 1800248 ◽  
Author(s):  
Marion Delcroix ◽  
Gerd Staehler ◽  
Henning Gall ◽  
Ekkehard Grünig ◽  
Matthias Held ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Hypertension ◽  
High Risk ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
European Respiratory Society ◽  
Thromboembolic Pulmonary Hypertension

Abbreviated versions of the risk stratification strategy of the European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension guidelines have been recently validated in patients with pulmonary arterial hypertension. We aimed to investigate their prognostic value in medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients from the COMPERA registry, which collects six variables of interest (World Health Organization Functional Class, 6-min walk distance, brain natriuretic peptide, right atrial pressure, cardiac index and mixed venous oxygen saturation).We included patients with at least one follow-up visit, no pulmonary endarterectomy and at least three of the six variables available, and classified the patients into low-, intermediate- and high-risk groups. As a secondary analysis, the number of noninvasive low-risk criteria was counted. The association between risk assessment and survival was evaluated.Data from inclusion and follow-up (median 7 months) visits were available for 561 and 231 patients, respectively. Baseline 1- and 5-year survival estimates were significantly different (p<0.0001) in the baseline low-risk (98.6% and 88.3%, respectively), intermediate-risk (94.9% and 61.8%, respectively) and high-risk (75.5% and 32.9%, respectively) cohorts. Follow-up data were even more discriminative, with 100%, 92% and 69% 1-year survival, respectively. The number of low-risk noninvasive criteria was also associated with survival.These analyses suggest that the ESC/ERS risk assessment may be applicable in patients with medically treated CTEPH.

Single-center prognostic validation of the risk assessment of the 2015 ESC/ERS guidelines in patients with pulmonary arterial hypertension in Japan

2020 ◽  
Vol 98 (9) ◽  
pp. 653-658 ◽  
Author(s):  
Ryo Imai ◽  
Shiro Adachi ◽  
Masahiro Yoshida ◽  
Shigetake Shimokata ◽  
Yoshihisa Nakano ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Risk Group ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
Walking Distance ◽  
Pulmonary Arterial

The 2015 European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension include a multidimensional risk assessment for patients with pulmonary arterial hypertension (PAH). However, prognostic validations of this risk assessment are limited, especially outside Europe. Here, we validated the risk assessment strategy in PAH patients in our institution in Japan. Eighty consecutive PAH patients who underwent right heart catheterization between November 2006 and December 2018 were analyzed. Patients were classified as low, intermediate, or high risk by using a simplified version of the risk assessment that included seven variables: World Health Organization functional class, 6-min walking distance, peak oxygen consumption, brain natriuretic peptide, right atrial pressure, mixed venous oxygen saturation, and cardiac index. The high-risk group showed significantly higher mortality than the low- or intermediate-risk group at baseline (P < 0.001 for both comparisons), and the mortalities in the intermediate- and low-risk groups were both low (P = 0.989). At follow-up, patients who improved to or maintained a low-risk status showed better survival than those who did not (P = 0.041). Our data suggest that this risk assessment can predict higher mortality risk and long-term survival in PAH patients in Japan.

scholarly journals Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

2020 ◽  
Vol 6 (2) ◽  
pp. 00218-2019
Author(s):  
Abdullah M. Aldalaan ◽  
Sarfraz A. Saleemi ◽  
Ihab Weheba ◽  
Abeer Abdelsayed ◽  
Pekka Hämmäinen ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Disease Severity ◽  
Tertiary Care ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
World Health ◽  
Cumulative Probability ◽  
Probability Of Survival ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.MethodsThis study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry. This registry enrolled patients with pulmonary hypertension, established through right heart catheterisation, under clinical management at a specialised tertiary care centre. Patients received standard care during the period of the registry.ResultsAt the time of this analysis, 64 CTEPH patients were enrolled in the registry. Mean age at diagnosis was 39.7 years and there was a female predominance (67.6%). At baseline, most patients were in World Health Organization functional classes III or IV (70.1%). At the last follow-up visit, most patients (63.2%) had undergone endarterectomy, showing significant improvement in disease severity from baseline. Patients who underwent endarterectomy showed numerically higher (p=0.126) probability of survival at 1 year (97.5%) versus those who did not undergo endarterectomy (94.4%).ConclusionPatients were diagnosed at relatively young age, but still showed high disease severity, suggesting delay in diagnosis. Patients who underwent surgical treatment showed substantial improvements in clinical and haemodynamic parameters, while the remaining patients tended to show disease progression. The 96.6% 1-year cumulative probability of survival was high compared to previous studies.

scholarly journals Balloon pulmonary angioplasty improves right atrial reservoir and conduit functions in chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 21 (8) ◽  
pp. 855-862 ◽  
Author(s):  
Yuzo Yamasaki ◽  
Kohtaro Abe ◽  
Takeshi Kamitani ◽  
Kazuya Hosokawa ◽  
Masateru Kawakubo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Feature Tracking ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Atrial ◽  
Resonance Imaging ◽  
Active Function ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Rv Function

Abstract Aims Right atrial (RA) function largely contributes to the maintenance of right ventricular (RV) function. This study investigated the effect of balloon pulmonary angioplasty (BPA) on RA functions in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) using cardiac magnetic resonance imaging (CMRI). Methods and results CMRI and RV catheterization were performed before BPA sessions and at the follow-up periods in 29 CTEPH patients. Reservoir [RA longitudinal strain (RA-LS)], passive conduit [RA early LS rate (LSR)], and active (RA late LSR) phases were assessed by using cine CMRI and a feature-tracking algorithm. The relationships between the changes in RA functions and in brain natriuretic peptide (BNP) were evaluated in both the dilated and non-dilated RA groups. RA-LS (32.4% vs. 42.7%), RA LSR (6.3% vs. 8.3%), and RA early LSR (−2.3% vs. −4.3%) were improved after BPA, whereas no significant change was seen in RA late LSR. The changes in RA peak LS and in RA early LSR were significantly correlated with the changes in BNP (ΔRA-LS: r = −0.63, ΔRA-early LSR: r = 0.65) and pulmonary vascular resistance (PVR) (ΔRA-LS: r = −0.69, ΔRA-early LSR: r = 0.66) in the nondilated RA group. Conclusion The RA reservoir and passive conduit functions were impaired in inoperable CTEPH, whereas RA active function was preserved. BPA markedly reversed these impaired functions. The improvements in RA reservoir and conduit functions were significantly correlated with the changes in BNP levels and PVR in CTEPH patients with normal RA sizes.

scholarly journals A pragmatic approach to risk assessment in pulmonary arterial hypertension using the 2015 European Society of Cardiology/European Respiratory Society guidelines

Open Heart ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. e001725
Author(s):  
Fabio Dardi ◽  
Alessandra Manes ◽  
Daniele Guarino ◽  
Elisa Zuffa ◽  
Alessandro De Lorenzis ◽  
...  
Keyword(s):  
Risk Assessment ◽  
High Risk ◽  
Risk Groups ◽  
Low Risk ◽  
Intermediate Risk ◽  
Pragmatic Approach ◽  
European Respiratory Society ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

ObjectiveTo optimise treatment of patients with pulmonary arterial hypertension (PAH), the 2015 European Society of Cardiology/European Respiratory Society guidelines recommend using risk stratification, with the aim of patients achieving low-risk status. Previous analyses of registries made progress in using risk stratification approaches, however, the focus is often on patients with a low-risk prognosis, whereas most PAH patients are in intermediate-risk or high-risk categories. Using only six parameters with high prognostic relevance, we aimed to demonstrate a pragmatic approach to individual patient risk assessment to discriminate between patients at low risk, intermediate risk and high risk of death.MethodsRisk assessment was performed combining six parameters in four criteria: (1) WHO functional class, (2) 6 min walk distance, (3) N-terminal pro-brain natriuretic peptide (BNP)/BNP plasma levels or right atrial pressure and (4) cardiac index or mixed venous oxygen saturation. Assessments were made at baseline and at first follow-up after 3–4 months.Results725 PAH treatment-naive patients were analysed. Survival estimates between risk groups were statistically significant at baseline and first follow-up (p<0.001), even when the analysis was performed within PAH etiological subgroups. Similar results were observed in 208 previously treated PAH patients. Furthermore, patients who remained at or improved to low risk had a significantly better estimated survival compared with patients who remained at or worsened to intermediate risk or high risk (p≤0.005).ConclusionThe simplified risk-assessment method can discriminate idiopathic, connective-tissue-disease-associated and congenital-heart-disease-associated PAH patients into meaningful high-risk, intermediate-risk and low-risk groups at baseline and first follow-up. This pragmatic approach reinforces targeting a low-risk profile for PAH patients.

scholarly journals Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 53 (6) ◽  
pp. 1802004 ◽  
Author(s):  
Marc Humbert ◽  
Harrison W. Farber ◽  
Hossein-Ardeschir Ghofrani ◽  
Raymond L. Benza ◽  
Dennis Busse ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Risk Stratification ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Low Risk ◽  
Risk Stratification Model ◽  
Risk Stratum ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Clinical Worsening

BackgroundCurrent pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension (PAH) in the French, Swedish and COMPERA registries. Our objective was to investigate the three abbreviated risk stratification methods for patients with mostly prevalent PAH and chronic thromboembolic pulmonary hypertension (CTEPH), in patients from the PATENT-1/2 and CHEST-1/2 studies of riociguat.MethodsRisk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.ResultsWith all three methods, riociguat improved risk group/strata in patients with PAH after 12 weeks. The French non-invasive and Swedish/COMPERA methods discriminated prognosis for survival and clinical worsening-free survival at both baseline and follow-up. Furthermore, patients achieving one or more low-risk criteria or a low-risk stratum at follow-up had a significantly reduced risk of death and clinical worsening compared with patients achieving no low-risk criteria or an intermediate-risk stratum. Similar results were obtained in patients with inoperable or persistent/recurrent CTEPH.ConclusionsThis analysis confirms and extends the results of the registry analyses, supporting the value of goal-oriented treatment in PAH. Further assessment of these methods in patients with CTEPH is warranted.

scholarly journals Risk assessment in precapillary pulmonary hypertension: a comparative analysis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Thomas Sonnweber ◽  
Eva-Maria Schneider ◽  
Manfred Nairz ◽  
Igor Theurl ◽  
Günter Weiss ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Hypertension ◽  
High Risk ◽  
Risk Stratification ◽  
Risk Prediction ◽  
Mortality Risk ◽  
Assessment Tool ◽  
Risk Models ◽  
Non Invasive

Abstract Background Risk stratification is essential to assess mortality risk and guide treatment in patients with precapillary pulmonary hypertension (PH). We herein compared the accuracy of different currently used PH risk stratification tools and evaluated the significance of particular risk parameters. Methods We conducted a retrospective longitudinal observational cohort study evaluating seven different risk assessment approaches according to the current PH guidelines. A comprehensive assessment including multi-parametric risk stratification was performed at baseline and 4 yearly follow-up time-points. Multi-step Cox hazard analysis was used to analyse and refine risk prediction. Results Various available risk models effectively predicted mortality in patients with precapillary pulmonary hypertension. Right-heart catheter parameters were not essential for risk prediction. Contrary, non-invasive follow-up re-evaluations significantly improved the accuracy of risk estimations. A lack of accuracy of various risk models was found in the intermediate- and high-risk classes. For these patients, an additional evaluation step including assessment of age and right atrium area improved risk prediction significantly. Discussion Currently used abbreviated versions of the ESC/ERS risk assessment tool, as well as the REVEAL 2.0 and REVEAL Lite 2 based risk stratification, lack accuracy to predict mortality in intermediate- and high-risk precapillary pulmonary hypertension patients. An expanded non-invasive evaluation improves mortality risk prediction in these individuals.

scholarly journals Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study)

2021 ◽  
Vol 1 (2) ◽  
pp. 105-113
Author(s):  
Javier Enrique Fajardo-Rivero ◽  
Melissa Mogollón ◽  
Diego Fernando García-Bohórquez ◽  
Andrés Villabona-Rueda ◽  
Tania Mendoza-Herrera ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Thrombotic Event ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pharmacological Therapy ◽  
Functional Class ◽  
World Health ◽  
Class Iii ◽  
European Respiratory Society ◽  
Cross Sectional ◽  
Number Of Patients

Chronic thromboembolic disease (CTEPH) is one of the causes for developing pulmonary hypertension (PH). PH is characterized by an increase in pulmonary vascular pressure and resistance, ultimately leading to chronic overload. This study describes the clinical, functional, and hemodynamic characteristics as well as the established treatment strategy for a cohort of patients diagnosed with CTEPH in Bucaramanga, Colombia. In Colombia, PH is considered as an orphan disease with limited epidemiological data. We aim to provide useful information in order to help guide future clinical decisions for PH treatment and prevention. We conducted a cross-sectional study, obtaining clinical data from patients under follow-up, over 18 years of age, with hemodynamic confirmation of CTEPH in two pulmonary outpatient centers in Bucaramanga, Colombia between 2012 and 2018. 35 patients with diagnosis of CTEPH were included. Mean age was 52.3 ± 17.9 years. The mean time between the onset of symptoms to diagnosis was 14 months. 71% had a previous thrombotic event and 69% had functional class III and IV according to the world health organization (WHO) criteria. Most of the patients were classified as at high risk of mortality according to the European Society of Cardiology (ESC) and the European Respiratory Society (ERS/ESC) criteria and 60% were referred to undergo thromboendarterectomy. Most of the patients were under monotherapy treatment with Bosentan, the most prescribed medication in both monotherapy and dual therapy. This study identified a high number of patients in advanced stages of CETPH due to late diagnosis, related to health care limitations. This resulted in worse prognosis and quality of life. In addition, low adherence to non-pharmacological interventions was evidenced in patients who were not candidates for thromboendarterectomy despite the onset of pharmacological therapy.

Changes in Estimated Glomerular Filtration after Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

2019 ◽  
Vol 10 (1) ◽  
pp. 22-31 ◽  
Author(s):  
Szymon Darocha ◽  
Marta Banaszkiewicz ◽  
Arkadiusz Pietrasik ◽  
Anna Siennicka ◽  
Mateusz Piorunek ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Renal Function ◽  
Glomerular Filtration ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Kidney Injury ◽  
Right Atrial ◽  
Minimal Risk ◽  
Mixed Venous Saturation ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

Background: Balloon pulmonary angioplasty (BPA) is a novel treatment option for inoperable or persistent chronic thromboembolic pulmonary hypertension (CTEPH). Little is known about renal function in CTEPH patients undergoing BPA. Objectives: The aim of this study was to assess the frequency of contrast-induced acute kidney injury (CI-AKI) in patients with CTEPH undergoing BPA and to evaluate the relationship between hemodynamic and renal function. Methods: A total of 41 CTEPH patients were included and 250 consecutive BPA sessions were analyzed for frequency of CI-AKI. The serum creatinine (SC) concentration was measured and the glomerular filtration rate (GFR) was estimated using the Modification of Diet in Renal Disease equation before and 72 h after each BPA procedure. CI-AKI was defined as an increase of 25% or 0.5 mg/dL in SC from the baseline value within 48–72 h of contrast administration. SC and GFR were assessed before and after 3–6 months of completing the BPA treatment and correlated with hemodynamic parameters. Results: The SC concentration and GFR did not change significantly within 72 h after BPA (+1%, p = 0.921, and +4%, p = 0.112, respectively). CI-AKI was noted in 2 cases (0.8%). Significant improvement was noted in GFR (75.4 ± 21.2 vs. 80.9 ± 22.4 mL/min/1.73 m2; p = 0.012) in addition to improvement in right atrial pressure (RAP; 9.1 ± 4.1 to 5.0 ± 2.2 mm Hg; p < 0.001), mean pulmonary artery pressure (49.1 ± 10.7 to 29.8 ± 8.3 mm Hg; p < 0.001), cardiac index (CI; 2.42 ± 0.6 to 2.70 ± 0.6 L/min/m2; p = 0.004), and pulmonary vascular resistance (9.42 ± 3.6 to 4.4 ± 2.3 Wood units; p < 0.001). In a subpopulation of 12 patients with impaired renal function at baseline, the relative increase in GFR was significantly correlated with relative improvement in CI (r = 0.060; p = 0.037), RAP (r = –0.587; p = 0.044), and mixed venous saturation (r = 0.069; p = 0.012). Conclusions: Hemodynamically effective BPA procedures improve renal function in patients with CTEPH with a minimal risk of CI-AKI in the course of treatment.

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