Association between Initial Treatment Strategy and Long-Term Survival in Pulmonary Arterial Hypertension

The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. A retrospective analysis of incident patients with idiopat...

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Bibliographic Details
Published in:American journal of respiratory and critical care medicine 2021-10, Vol.204 (7), p.842-854
Main Authors: Boucly, Athénaïs, Savale, Laurent, Jaïs, Xavier, Bauer, Fabrice, Bergot, Emmanuel, Bertoletti, Laurent, Beurnier, Antoine, Bourdin, Arnaud, Bouvaist, Hélène, Bulifon, Sophie, Chabanne, Céline, Chaouat, Ari, Cottin, Vincent, Dauphin, Claire, Degano, Bruno, De Groote, Pascal, Favrolt, Nicolas, Feng, Yuanchao, Horeau-Langlard, Delphine, Jevnikar, Mitja, Jutant, Etienne-Marie, Liang, Zhiying, Magro, Pascal, Mauran, Pierre, Moceri, Pamela, Mornex, Jean-François, Palat, Sylvain, Parent, Florence, Picard, François, Pichon, Jérémie, Poubeau, Patrice, Prévot, Grégoire, Renard, Sébastien, Reynaud-Gaubert, Martine, Riou, Marianne, Roblot, Pascal, Sanchez, Olivier, Seferian, Andrei, Tromeur, Cécile, Weatherald, Jason, Simonneau, Gérald, Montani, David, Humbert, Marc, Sitbon, Olivier
Format: Article
Language:English
Subjects:
Administration, Oral
Adult
Age groups
Aged
Antihypertensive Agents - therapeutic use
Clinical outcomes
Drug Therapy, Combination
Female
Follow-Up Studies
France - epidemiology
Health risks
Humans
Infusions, Parenteral
Life expectancy
Life Sciences
Male
Medical treatment
Middle Aged
Propensity Score
Pulmonary Arterial Hypertension - drug therapy
Pulmonary Arterial Hypertension - mortality
Pulmonary hypertension
Registries
Retrospective Studies
Survival Analysis
Treatment Outcome
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Summary:The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy (two oral medications and a parenteral prostacyclin). Among 1,611 enrolled patients, 984 were initiated on monotherapy, 551 were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. The survival rate was higher with the use of triple therapy (91% at 5 yr) as compared with dual therapy or monotherapy (both 61% at 5 yr) (  
ISSN:1073-449X
1535-4970
DOI:10.1164/rccm.202009-3698oc