• Athénaïs Boucly, Laurent Savale, Xavier Jaïs, Fabrice Bauer, Emmanuel Bergot, Laurent Bertoletti, Antoine Beurnier, Arnaud Bourdin, Hélène Bouvaist, Sophie Bulifon, Céline Chabanne, Ari Chaouat, Vincent Cottin, Claire Dauphin, Bruno Degano, Pascal De Groote, Nicolas Favrolt, Yuanchao Feng, Delphine Horeau-Langlard, Mitja Jevnikar, Etienne-Marie Jutant, Zhiying Liang, Pascal Magro, Pierre Mauran, Pamela Moceri, Jean-François Mornex, Sylvain Palat, Florence Parent, François Picard, Jérémie Pichon, Patrice Poubeau, Grégoire Prévot, Sébastien Renard, Martine Reynaud-Gaubert, Marianne Riou, Pascal Roblot, Olivier Sanchez, Andrei Seferian, Cécile Tromeur, Jason Weatherald, Gérald Simonneau, David Montani, Marc Humbert, and Olivier Sitbon.
• Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
• Am. J. Respir. Crit. Care Med. 2021 Oct 1; 204 (7): 842-854.

AbstractRationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: 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). Measurements and Main Results: 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) (P < 0.001). Propensity score matching of age, sex, and pulmonary vascular resistance also showed significant differences between triple therapy and dual therapy (10-yr survival, 85% vs. 65%). In high-risk patients (n = 243), the survival rate was higher with triple therapy than with monotherapy or dual therapy, whereas there was no difference between monotherapy and double therapy. In intermediate-risk patients (n = 1,134), survival improved with an increasing number of therapies. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death (hazard ratio, 0.29; 95% confidence interval, 0.11-0.80; P = 0.017). Among the 148 patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients.

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