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 Follow-up tricuspid annular plane systolic excursion predicts survival in pulmonary arterial hypertension

2017 ◽  
Vol 7 (2) ◽  
pp. 361-371 ◽  
Author(s):  
Jeremy A. Mazurek ◽  
Anjali Vaidya ◽  
Stephen C. Mathai ◽  
Justin D. Roberts ◽  
Paul R. Forfia
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Prognostic Significance ◽  
Functional Class ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
Group 2 ◽  
One Year ◽  
Group 1

Few studies have examined the utility of serial echocardiography in the evaluation, management, and prognosis of patients with pulmonary arterial hypertension (PAH). Therefore, we sought to evaluate the prognostic significance of follow-up tricuspid annular plane systolic excursion (TAPSE) in PAH. We prospectively studied 70 consecutive patients with PAH who underwent baseline right heart catheterization (RHC) and transthoracic echocardiogram, who survived to follow-up echocardiogram after initiation of PAH therapy. Baseline TAPSE was 1.6 ± 0.5 cm which increased to 2.0 ± 0.4 cm on follow-up ( P < 0.0001). The cohort was dichotomized by TAPSE at one-year follow-up: Group 1 (n = 37): follow-up TAPSE ≥ 2 cm; Group 2 (n = 33): follow-up TAPSE < 2 cm. Group 1 participants were significantly more likely to reach WHO functional class I–II status and achieve a higher six-minute walk distance on follow-up. Of the 68 patients who survived more than one year, 18 died (26.5%) over a median follow-up of 941 days (range, 3–2311 days), with significantly higher mortality in Group 2 versus Group 1 (41.9% vs. 13.5%; P = 0.003). While baseline TAPSE stratified at 2 cm did not predict survival in this cohort, TAPSE ≥ 2 cm at follow-up strongly predicted survival in bivariable models (hazard ratio, 0.21; 95% confidence interval, 0.08–0.60). In conclusion, follow-up TAPSE ≥ 2 cm is a prognostic marker and potential treatment target in a PAH population.

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 Clinical and hemodynamic benefit of macitentan and riociguat upfront combination in patients with pulmonary arterial hypertension

2019 ◽  
Vol 9 (1) ◽  
pp. 204589401982694 ◽  
Author(s):  
Roxana Sulica ◽  
Swathi Sangli ◽  
Aloke Chakravarti ◽  
David Steiger
Keyword(s):  
Survival Rate ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Score ◽  
Dual Therapy ◽  
Preliminary Evidence ◽  
Functional Class ◽  
World Health ◽  
Open Label ◽  
Pulmonary Arterial

In this open-label study, we evaluated the effect of upfront macitentan and riociguat combination in newly diagnosed pulmonary arterial hypertension (PAH) patients. In 15 consecutive PAH patients, we collected clinical and hemodynamic data at baseline, visit 1 (median 4 months) and visit 2 (median 12 months). Survival and transplantation status were analyzed over 36 months. Statistical analysis included student t-test and 95% confidence interval (CI) ( t-statistic or Clopper-Pearson). Kaplan-Meier was used to estimate survival rate. There were 11/15 women (mean age 56 years), in World Health Organization (WHO) functional class (FC) III ( n = 14) or IV ( n = 1). The 6 min walk distance increased from 281.6 m (baseline) to 315.7 m (visit 1) and visit 2 (313.9 m), representing a 34- and 32-m change ( P < 0.05), respectively, associated with Borg score improvements. Brain natriuretic peptide decreased: 318.2 pg/mL (baseline) to 122.0 pg/mL (visit 1) and 98.6 pg/mL (visit 2) ( P < 0.05). WHO FC improved in eight patients (53%, 95% CI 27%–79%). Pulmonary vascular resistance (9.2 to 5.7 Wood Units) and mean pulmonary artery pressure (47.3 to 38.9 mmHg) decreased; cardiac index increased (2.3 to 3.0 L/min/m2) (baseline to visit 2, all P < 0.05). All patients had intermediate and high risk score (baseline); at 1-year follow-up, dual therapy led to reduction to low risk score in 7/15 (47%) patients. There were no unexpected or serious side effects. Three patients died due to unrelated causes; one patient received a lung transplant. Transplant-free survival rate (36 months) was 85%. Preliminary evidence is provided for effectiveness of initial macitentan and riociguat combination therapy in PAH.

The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension

CHEST Journal ◽  
2012 ◽  
Vol 141 (2) ◽  
pp. 354-362 ◽  
Author(s):  
Raymond L. Benza ◽  
Mardi Gomberg-Maitland ◽  
Dave P. Miller ◽  
Adaani Frost ◽  
Robert P. Frantz ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Score ◽  
Newly Diagnosed ◽  
Pulmonary Arterial

ELEVATE 2: A multicenter study of rodatristat ethyl in patients with WHO Group 1 pulmonary arterial hypertension (PAH)

2021 ◽  
Author(s):  
Howard Lazarus ◽  
Jill Denning ◽  
Watiri Kamau-Kelley ◽  
Stephen Wring ◽  
Michelle Palacios ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Multicenter Study ◽  
Pulmonary Arterial ◽  
Group 1

scholarly journals Is hyponatremia associated with mortality in pulmonary arterial hypertension?

2018 ◽  
Vol 8 (2) ◽  
pp. 204589401877688 ◽  
Author(s):  
Anastasiia A. Rudkovskaia ◽  
Adriano R. Tonelli ◽  
Youlan Rao ◽  
Jeffrey P. Hammel ◽  
Gregory K. Buller ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Sodium Level ◽  
Continuous Variable ◽  
Functional Class ◽  
Right Atrial ◽  
Left Heart Failure ◽  
Tertiary Referral Center ◽  
Pulmonary Arterial ◽  
One Year

Hyponatremia is associated with poor prognosis in left heart failure and liver disease. Its prognostic role in pulmonary arterial hypertension (PAH) is not well defined. We investigated the association between hyponatremia and one-year mortality in two large cohorts of PAH. This study is a secondary analysis evaluating the association between hyponatremia and one-year mortality in patients treated with subcutaneous treprostinil (cohort 1). The results are validated using a PAH registry at a tertiary referral center (cohort 2). Eight-hundred and twenty patients were enrolled in cohort 1 (mean age = 47 ± 14 years) and 791 in cohort 2 (mean age = 55 ± 15 years). Sodium level is negatively correlated with mean right atrial pressure (r = −0.09, P = 0.018; r = −0.089, P = 0.015 in cohorts 1 and 2, respectively). In unadjusted analyses of cohort 1, the sodium level (as a continuous variable) is associated with one-year mortality (hazard ratio = 0.94; P = 0.035). Hyponatremia loses its significance (as a continuous variable and when dichotomized at ≤ 137 mmol/L; P = 0.12) when adjusted for functional class (FC), which is identified as the variable whose presence turns the effect of sodium level into non-significant. Secondary analyses using a cut-off value of < 135 mmol/L showed similar results. These results are validated in cohort 2. Although the sample size for patients with sodium < 130 mmol/L is small (n = 31), severe hyponatremia is associated with higher overall mortality (47% versus 23%; P = 0.01), even when adjusting for age, FC, and baseline 6-min walk distance ( P < 0.001). Although baseline hyponatremia is associated with one-year mortality, it loses its significance when adjusted for FC.

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