Risk stratification and medical therapy of pulmonary arterial hypertension

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Abstract

Pulmonary arterial hypertension (PAH) remains a severe clinical condition despite the availability over the past 15 years of multiple drugs interfering with the endothelin, nitric oxide and prostacyclin pathways. The recent progress observed in medical therapy of PAH is not, therefore, related to the discovery of new pathways, but to the development of new strategies for combination therapy and on escalation of treatments based on systematic assessment of clinical response. The current treatment strategy is based on the severity of the newly diagnosed PAH patient as assessed by a multiparametric risk stratification approach. Clinical, exercise, right ventricular function and haemodynamic parameters are combined to define a low-, intermediate- or high-risk status according to the expected 1-year mortality. The current treatment algorithm provides the most appropriate initial strategy, including monotherapy, or double or triple combination therapy. Further treatment escalation is required in case low-risk status is not achieved in planned follow-up assessments. Lung transplantation may be required in most advanced cases on maximal medical therapy.

Abstract

State of the art and research perspectives on medical therapy of pulmonary arterial hypertension, including treatment algorithm http://ow.ly/4UkJ30md5GS

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Most cited references 61

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Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.

G D'Alonzo, R Barst, S. Ayres … (1991)

To characterize mortality in persons diagnosed with primary pulmonary hypertension and to investigate factors associated with survival. Registry with prospective follow-up. Thirty-two clinical centers in the United States participating in the Patient Registry for the Characterization of Primary Pulmonary Hypertension supported by the National Heart, Lung, and Blood Institute. Patients (194) diagnosed at clinical centers between 1 July 1981 and 31 December 1985 and followed through 8 August 1988. At diagnosis, measurements of hemodynamic variables, pulmonary function, and gas exchange variables were taken in addition to information on demographic variables, medical history, and life-style. Patients were followed for survival at 6-month intervals. The estimated median survival of these patients was 2.8 years (95% Cl, 1.9 to 3.7 years). Estimated single-year survival rates were as follows: at 1 year, 68% (Cl, 61% to 75%); at 3 years, 48% (Cl, 41% to 55%); and at 5 years, 34% (Cl, 24% to 44%). Variables associated with poor survival included a New York Heart Association (NYHA) functional class of III or IV, presence of Raynaud phenomenon, elevated mean right atrial pressure, elevated mean pulmonary artery pressure, decreased cardiac index, and decreased diffusing capacity for carbon monoxide (DLCO). Drug therapy at entry or discharge was not associated with survival duration. Mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index. Such an equation, once validated prospectively, could be used as an adjunct in planning treatment strategies and allocating medical resources.

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Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).

Raymond Benza, Dave Miller, Mardi Gomberg-Maitland … (2010)

Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use. Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique. We identified key predictors of survival based on the patient's most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.

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A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension.

R Barst, L Rubin, W. A. Long … (1996)

Primary pulmonary hypertension is a progressive disease for which no treatment has been shown in a prospective, randomized trial to improve survival. We conducted a 12-week prospective, randomized, multicenter open trial comparing the effects of the continuous intravenous infusion of epoprostenol (formerly called prostacyclin) plus conventional therapy with those of conventional therapy alone in 81 patients with severe primary pulmonary hypertension (New York Heart Association functional class III or IV). Exercise capacity was improved in the 41 patients treated with epoprostenol (median distance walked in six minutes, 362 m at 12 weeks vs. 315 m at base line), but it decreased in the 40 patients treated with conventional therapy alone (204 m at 12 weeks vs. 270 m at base line; P < 0.002 for the comparison of the treatment groups). Indexes of the quality of life were improved only in the epoprostenol group (P < 0.01). Hemodynamics improved at 12 weeks in the epoprostenol-treated patients. The changes in mean pulmonary-artery pressure for the epoprostenol and control groups were -8 percent and +3 percent, respectively (difference in mean change, -6.7 mm Hg; 95 percent confidence interval, -10.7 to -2.6 mm Hg; P < 0.002), and the mean changes in pulmonary vascular resistance for the epoprostenol and control groups were -21 percent and +9 percent, respectively (difference in mean change, -4.9 mm Hg/liter/min; 95 percent confidence interval, -7.6 to -2.3 mm Hg/liter/min; P < 0.001). Eight patients died during the study, all of whom had been randomly assigned to conventional therapy (P = 0.003). Serious complications included four episodes of catheter-related sepsis and one thrombotic event. As compared with conventional therapy, the continuous intravenous infusion of epoprostenol produced symptomatic and hemodynamic improvement, as well as improved survival in patients with severe primary pulmonary hypertension.

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Journal

Journal ID (nlm-ta): Eur Respir J

Journal ID (iso-abbrev): Eur. Respir. J

Journal ID (publisher-id): ERJ

Journal ID (hwp): erj

Title: The European Respiratory Journal

Publisher: European Respiratory Society

ISSN (Print): 0903-1936

ISSN (Electronic): 1399-3003

Publication date Collection: January 2019

Publication date (Electronic): 24 January 2019

Volume: 53

Issue: 1

Electronic Location Identifier: 1801889

Affiliations

[1 ]Dept of Experimental, Diagnostic and Specialty Medicine (DIMES), Alma Mater Studiorum, University of Bologna, Bologna, Italy

[2 ]Pulmonary and Critical Care Division, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

[3 ]Dept of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA

[4 ]Pulmonary Hypertension Center, Thoraxklinic at Heidelberg University Hospital, Heidelberg, Germany

[5 ]State Key Lab of Cardiovascular Disease, FuWai Hospital and Key Lab of Pulmonary Vascular Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

[6 ]Non-Coronary Heart Disease Dept, Almazov National Medical Research Centre, St Petersburg, Russian Federation

[7 ]Tufts University School of Medicine, Pulmonary, Critical Care and Sleep Division, Tufts Medical Center, Boston, MA, USA

[8 ]Cardiopulmonary Dept, National Heart Institute, La Salle University, Mexico City, Mexico

[9 ]Pulmonary, Critical Care Division, Houston Methodist Hospital, Weill Cornell College of Medicine, Houston, TX, USA

[10 ]Dept of Cardiology, International University of Health and Welfare School of Medicine, Tokyo, Japan

[11 ]Cardiovascular Medicine, The University of Michigan, Ann Arbor, MI, USA

Author notes

Nazzareno Galiè, Dept of Experimental, Diagnostic and Specialty Medicine (DIMES), Alma Mater Studiorum, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy. E-mail: nazzareno.galie@ 123456unibo.it

Article

Publisher ID: ERJ-01889-2018

DOI: 10.1183/13993003.01889-2018

PMC ID: 6351343

PubMed ID: 30545971

SO-VID: 5b2f4757-e260-42b2-bd31-c221e595be5e

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This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

Risk stratification and medical therapy of pulmonary arterial hypertension

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Most cited references 61

Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.

Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).

A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension.

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