scholarly journals Tolerability and clinical efficacy of inhaled treprostinil in patients with group 1 pulmonary arterial hypertension

2018 ◽  
Vol 9 (9) ◽  
pp. 171-177
Author(s):  
Cher Y. Enderby ◽  
Charles Burger
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Risk Score ◽  
The United States ◽  
Prognostic Indicators ◽  
Physician Assessment ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Group 1

Background: Treprostinil is a prostacyclin analogue that directly vasodilates pulmonary and systemic arterial vascular beds. The United States Food and Drug Administration approved inhaled treprostinil in July 2009 for the treatment of group 1 pulmonary arterial hypertension. Inhaled treprostinil avoids issues with continuous infusion prostanoids. This study describes a single institutional experience with inhaled treprostinil. Methods: This was a retrospective review of group 1 pulmonary arterial hypertension patients receiving inhaled treprostinil from July 2009 through September 2015. Patient demographics, vital signs, prognostic indicators, pulmonary arterial hypertension assessments, treprostinil dosing, pulmonary arterial hypertension medications, and physician assessment were collected. Prognostic indicators and the physician assessment were used to assess treatment response. A modified Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) risk score was calculated prior to and after initiation of inhaled treprostinil. Results: The mean time on inhaled treprostinil for the 16 patients was 21 ± 17 months. A total of 31% discontinued treatment. The New York Heart Association Functional Class, right ventricular size, and right ventricular function improved after inhaled treprostinil. Directional improvement in B-type natriuretic peptide, 6-minute walk distance, right arterial pressure and mean pulmonary artery pressure were also observed. The mean modified REVEAL risk score (RRS) was 7 ± 3 at baseline. The RRS decreased in 7 of the 11 patients that improved and remained stable in 2 patients. Conclusion: The majority of patients in this consecutive series receiving inhaled treprostinil tolerated treatment. Most patients remained on therapy for over 12 months. Clinical assessments of disease severity all changed directionally toward improvement and the overall risk assessment was improved or stable in 56% by the RRS.

scholarly journals 0558 Sleep Disordered Breathing and Right Ventricular Electrocardiographic and Functional Characteristics in Group 1 Pulmonary Arterial Hypertension

SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A214-A214
Author(s):  
A S Bhat ◽  
L Wang ◽  
S Kaur ◽  
R Nawabit ◽  
K Highland ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Sleep Disordered Breathing ◽  
Sleep Study ◽  
Unit Increase ◽  
Pulmonary Arterial ◽  
Group 1 ◽  
Disordered Breathing

Abstract Introduction Right ventricular (RV) electrophysiologic and functional alterations related to sleep disordered breathing (SDB) in pulmonary arterial hypertension (PAH) are not well understood. We hypothesize an association between SDB and RV electrophysiological/functional measures in World Symposium of Pulmonary Hypertension (WSPH) Group 1 PAH. Methods The NHLBI multicenter PVDOMICS study (NCT02980887) enrolls patients with PAH undergoing a battery of assessments including home sleep apnea testing(NOX-T3, Carefusion®) or with historical sleep study data. Logistic(OR,95%CI) and linear(beta coefficients,95%CI) regression models adjusted for age, sex, race, body mass index (BMI, kg/m2), PAH medications, supplemental oxygen(O2), positive airway pressure(PAP) were used to assess associations of SDB(apnea hypopnea index,(AHI), ≥3% desaturations(hypopnea), percentage recording time with SaO2<90% (TRT<90%) with electrocardiographic measures: RV hypertrophy(RVH), right bundle branch block(RBBB), and right axis deviation(RAD), echocardiographically-derived RV systolic pressure(RVSP) and RV ejection fraction(RVEF) from cardiac MRI. Analyses were performed based on an overall significance level of 0.05, using SAS software (version 9.4, Cary, NC). Results The analysis consisted of 182 PAH participants with age: 52.5±13.9 years, 71.4% female, 88.9% Caucasian, BMI:30.3±7.8 kg/m2, RVEF: 37.3±11.6, and RVSP: 67.0±23.4. None of the electrocardiographic measures were associated with AHI and only RVH was significantly associated with TRT<90% (1.25:1.09,1.43),p=0.001. Although AHI was not associated with RVSP, a 10% increase in TRT<90% was associated with a 2.60mmHg increase in RVSP (2.60:1.44,3.76),p<0.001. Each 10-unit increase in AHI was associated with a 2.72% reduction of RVEF (-2.72:-4.89,-0.56),p=0.014, and each 10-unit increase in TRT<90% was associated with a 0.72% reduction of RVEF (-0.72:-1.38,-0.06),p=0.033. Conclusion We identify nocturnal hypoxia as a predictor of RV electrophysiological and functional alterations even after consideration of confounding factors. SDB as determined by AHI was also more so associated with reduced RVEF than hypoxia. Future mechanistic studies should focus on further elucidation of SDB and nocturnal hypoxia on pathogenesis of RV dysfunction in PAH. Support U01HL125218/U01HL125205/U01HL125212/U01HL125208/U01HL125175/U01HL125215, U01HL125177/Pulmonary Hypertension Association

scholarly journals P1282 Follow-up evaluation of right chambers mechanics is associated with prognosis in group 1 pulmonary arterial hypertension

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
F Biondi ◽  
S Albani ◽  
F Lo Giudice ◽  
L Howard ◽  
A De Luca ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Longitudinal Strain ◽  
Specific Treatment ◽  
Right Atrial ◽  
Echocardiographic Parameters ◽  
Pulmonary Arterial ◽  
Group 1

Abstract Background Pulmonary arterial hypertension (PAH) is a severe disease that progressively leads to right ventricular (RV) failure and cardiovascular death. Evaluation of right heart mechanics by means of 2-dimensional speckle tracking echocardiography (2D-STE) has displayed to be a promising tool to estimate prognosis in PAH patients. Purpose To evaluate the association between right ventricular free wall longitudinal strain (RVFWLS) and right peak atrial longitudinal strain (RPALS) at follow-up after initiation of specific vasodilator therapy in PAH patients, with outcomes. Methods 83 subjects diagnosed with PAH Group 1 at three University Hospitals (Hammersmith Hospital, London, United Kingdom; Trieste University Hospital, Trieste, Italy; FTGM, Pisa, Italy), who were naive from specific treatment for PAH at the time of diagnosis, were retrospectively enrolled in this study. Standard echocardiographic parameters were collected. Outcomes were defined as the combination of all-cause mortality, hospitalization for PAH and first prostanoid administration. We investigated the correlation between RVFWLS and RPALS with outcomes adjusting for validated echocardiographic parameters strongly associated with prognosis in PAH (right atrial area – RAA and pericardial effusion – PE) and patients’ haemodynamics. Results 30 patients experienced outcomes during a median follow-up time of 33 months. Median RVFWLS at follow-up was -15.8% (IQR: -12.1%/- 21.1%). Median RPALS at follow-up was 25% (IQR: 17.9%/36.6%). In the multivariate analysis, RVFLWS at follow-up was independently associated with outcomes (95% confidence interval [CI]: 1.01 – 1.24, p = 0.04), irrespectively from RAA (95% CI: 0.98 – 1.20, p = 0.12) and pulmonary arterial systolic pressure (PASP, 95% CI: 0.99 – 1.06, p = 0.12). We also observed a trend towards superiority of RPALS at follow-up (95% CI 0.90 – 1.00, p = 0.07) over RAA (95% CI: 0.98 – 1.20, p = 0.14) and PASP (95% CI: 0.99 - 1.05, p= 0.31). Finally, RPALS (95% CI: 0.88 – 1.00, p = 0.05) was independently associated with outcomes over PE (95% CI: 0.14 – 1.89, p = 0.32) and RVFWLS (95% CI: 0.97 – 1.20, p = 0.16). Conclusions In PAH group 1, assessment of both RVFWLS and RPALS at follow-up is associated with outcomes, independently from standard echocardiographic parameters.

scholarly journals A modified risk score in one-year survival rate assessment of group 1 pulmonary arterial hypertension

2018 ◽  
Vol 18 (1) ◽  
Author(s):  
Wei Xiong ◽  
Yunfeng Zhao ◽  
Mei Xu ◽  
Bigyan Pudasaini ◽  
Xuejun Guo ◽  
...  
Keyword(s):  
Survival Rate ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Score ◽  
Pulmonary Arterial ◽  
One Year ◽  
Group 1

scholarly journals Non-Invasive Risk Prediction Based on Right Ventricular Function in Patients with Pulmonary Arterial Hypertension

2021 ◽  
Vol 10 (21) ◽  
pp. 5130
Author(s):  
Vazhma Qaderi ◽  
Jessica Weimann ◽  
Lars Harbaum ◽  
Benedikt N. Schrage ◽  
Dorit Knappe ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Right Ventricular ◽  
Risk Score ◽  
Right Atrial ◽  
Conventional Model ◽  
Right Heart ◽  
Non Invasive ◽  
Pulmonary Arterial

Background: Right ventricular dysfunction is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to identify echocardiographic right heart parameters associated with adverse outcome and to develop a non-invasive, echocardiography-based risk score for PAH patients. Methods and Results: In 254 PAH patients we analyzed functional status, laboratory results, and echocardiographic parameters. We included these parameters to estimate all-cause death or lung transplantation using Cox regression models. The analyses included a conventional model using guideline-recommended variables and an extended echocardiographic model. Based on the final model a 12-point risk score was derived, indicating the association with the primary outcome within five years. During a median follow-up time of 4.2 years 74 patients died or underwent lung transplantation. The conventional model resulted in a C-Index of 0.539, whereas the extended echocardiographic model improved the discrimination (C-index 0.639, p-value 0.017). Ultimately, the newly developed risk score included WHO functional class, 6-min walking distance, N-terminal brain natriuretic peptide concentrations, pericardial effusion, right atrial area, tricuspid annular plane systolic excursion, and fractional area change. Conclusion: Integrating right heart function assessed by echocardiography improves prediction of death or lung transplantation in PAH patients. Independent validation of this finding is warranted.

scholarly journals preoperative right ventricular overwork is a major determinant of residual pulmonary arterial hypertension in patients with repaired arterial septal defect

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Suzuki ◽  
Y Tanaka ◽  
K Yamashita ◽  
A Shono ◽  
K Sumimoto ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Septal Defect ◽  
Hazard Ratio ◽  
Coupling Index ◽  
Pulmonary Arterial ◽  
Asd Closure ◽  
The Right ◽  
Postoperative Residual

Abstract Funding Acknowledgements Type of funding sources: None. Background The haemodynamic effect of atrial septal defect (ASD) is a chronic volume overload of the right heart and pulmonary vasculature. Pulmonary overcirculation is generally compensated for by the right ventricular (RV) and pulmonary arterial (PA) reserve. However, in a subset of patients, prolonged pulmonary overcirculation insidiously induces obstructive pulmonary vasculopathy, which results in postoperative residual pulmonary arterial hypertension (PAH) after ASD closure. Postoperative PAH is a major concern because it is closely associated with poor outcomes and impaired quality of life. However, to date, no clinically robust predictors of postoperative residual PAH have been clearly identified. Purpose This study sought to assess the haemodynamic characteristics of ASD patients in terms of mechano-energetic parameters and to identify the predictors of postoperative residual PAH in these patients. Methods A total of 120 ASD patients (age: 58 ± 17 years) and 46 normal controls were recruited. As previously reported, the simplified RV contraction pressure index (sRVCPI) was calculated as an index of RV external work by multiplying the tricuspid annular plane systolic excursion (TAPSE) by the pressure gradient between the RV and right atrium. RV- PA coupling was evaluated using TAPSE divided by PA systolic pressure as an index of the RV length-force relationship. These parameters were measured both at baseline and 6 months after ASD closure. Results As expected, baseline sRVCPI was significantly greater in patients with ASD than in controls (775 ± 298 vs. 335 ± 180 mm Hg • mm, P < 0.01), which indicated significant "RV overwork". As a result, RV-PA coupling in ASD patients was significantly impaired compared to that in controls (0.9 ± 0.8 vs. 3.5 ± 1.7 mm/mm Hg, P < 0.01). All 120 ASD patients underwent transcatheter or surgical shunt closure; 15 of them had residual PAH after closure. After 6 months, RV-PA coupling index significantly improved in patients without residual PAH, from 0.96 ± 0.81 to 1.27 ± 1.24 mm/mm Hg (P = 0.02). Furthermore, RV load was markedly reduced, with sRVCPI falling from 691 ± 258 to 434 ± 217 mm Hg • mm, P < 0.01). However, in patients with residual PAH, RV-PA coupling index deteriorated from 0.64 ± 0.23 to 0.53 ± 0.12 mm/mm Hg (P < 0.01). As a result, RV overload was not significantly relieved (sRVCPI; from 971 ± 382 to 783 ± 166 mm Hg • mm, P = 0.22). In a multivariate analysis, baseline pulmonary vascular resistance (hazard ratio 1.009; P < 0.01) and preoperative sRVPCI (hazard ratio 1.003; P < 0.01) revealed to be independent predictors of residual PAH. Conclusion In terms of mechano-energetic function, preoperative "RV overwork" can be used as a robust predictor of an impaired RV-PA relationship in ASD patients. Moreover, periodic assessment of sRVPCI may contribute to the better management for patients with unrepaired ASD. Abstract Figure.

scholarly journals Comparative analysis on the anti-inflammatory/immune effect of mesenchymal stem cell therapy for the treatment of pulmonary arterial hypertension

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Seyeon Oh ◽  
Albert Y. Jang ◽  
Sehyun Chae ◽  
Seungbum Choi ◽  
Jeongsik Moon ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Right Ventricular Function ◽  
Treated Group ◽  
Therapeutic Effects ◽  
Mesenchymal Stem Cell Therapy ◽  
Adaptive Immune Cells ◽  
Pulmonary Arterial

AbstractDespite the advancement of targeted therapy for pulmonary arterial hypertension (PAH), poor prognosis remains a reality. Mesenchymal stem cells (MSCs) are one of the most clinically feasible alternative treatment options. We compared the treatment effects of adipose tissue (AD)-, bone marrow (BD)-, and umbilical cord blood (UCB)-derived MSCs in the rat monocrotaline-induced pulmonary hypertension (PH) model. The greatest improvement in the right ventricular function was observed in the UCB-MSCs treated group. The UCB-MSCs treated group also exhibited the greatest improvement in terms of the largest decrease in the medial wall thickness, perivascular fibrosis, and vascular cell proliferation, as well as the lowest levels of recruitment of innate and adaptive immune cells and associated inflammatory cytokines. Gene expression profiling of lung tissue confirmed that the UCB-MSCs treated group had the most notably attenuated immune and inflammatory profiles. Network analysis further revealed that the UCB-MSCs group had the greatest therapeutic effect in terms of the normalization of all three classical PAH pathways. The intravenous injection of the UCB-MSCs, compared with those of other MSCs, showed superior therapeutic effects in the PH model for the (1) right ventricular function, (2) vascular remodeling, (3) immune/inflammatory profiles, and (4) classical PAH pathways.

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