• Roberto Badagliacca, Michele D'Alto, Stefano Ghio, Paola Argiento, Vincenzo Bellomo, Natale Daniele Brunetti, Gavino Casu, Marco Confalonieri, Marco Corda, Michele Correale, Carlo D'Agostino, Lucrezia De Michele, Giuseppe Galgano, Alessandra Greco, Carlo Lombardi, Giovanna Manzi, Valentina Mercurio, Massimiliano Mulè, Giuseppe Paciocco, Silvia Papa, Emanuele Romeo, Laura Scelsi, Davide Stolfo, Patrizio Vitulo, Robert Naeije, and Carmine Dario Vizza.
• Department of Cardiovascular and Respiratory Sciences, Sapienza University of Rome, Rome, Italy.
• Am. J. Respir. Crit. Care Med. 2021 Feb 15; 203 (4): 484-492.

AbstractRationale: An initial oral combination of drugs is being recommended in pulmonary arterial hypertension (PAH), but the effects of this approach on risk reduction and pulmonary vascular resistance (PVR) are not known.Objectives: To test the hypothesis that a low-risk status would be determined by the reduction of PVR in patients with PAH treated upfront with a combination of oral drugs.Methods: The study enrolled 181 treatment-naive patients with PAH (81% idiopathic) with a follow-up right heart catheterization at 6 months (interquartile range, 144-363 d) after the initial combination of endothelin receptor antagonist + phosphodiesterase-5 inhibitor drugs and clinical evaluation and risk assessments by European guidelines and Registry to Evaluate Early and Long-Term PAH Disease Management scores.Measurements and Main Results: Initial combination therapy improved functional class and 6-minute-walk distance and decreased PVR by an average of 35% (median, 40%). One-third of the patients had a decrease in PVR <25%. This poor hemodynamic response was independently predicted by age, male sex, pulmonary artery pressure and cardiac index, and at echocardiography, a right/left ventricular surface area ratio of greater than 1 associated with low tricuspid annular plane systolic excursion of less than 18 mm. A low-risk status at 6 months was achieved or maintained in only 34.8% (Registry to Evaluate Early and Long-Term PAH Disease Management score) to 43.1% (European score) of the patients. Adding criteria of poor hemodynamic response improved prediction of a low-risk status.Conclusions: A majority of patients with PAH still insufficiently improved after 6 months of initial combinations of oral drugs is identifiable at initial evaluation by hemodynamic response criteria added to risk scores.

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