Follow-Up Functional Class and 6-Minute Walk Distance Identify Long-Term Survival in Pulmonary Arterial Hypertension
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PULMONARY HYPERTENSION
Follow‑Up Functional Class and 6‑Minute Walk Distance Identify Long‑Term Survival in Pulmonary Arterial Hypertension Gustavo A. Heresi1 · Youlan Rao2 Received: 24 June 2020 / Accepted: 29 October 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose Guidelines recommend specific treatment goals for pulmonary arterial hypertension (PAH) patients: functional class I or II, 6-min walk distance (6MWD) ≥ 380 to 440 m, normal natriuretic peptide levels, and normal right-sided invasive hemodynamics. Only observational registry data support this recommendation. Our aim was to test these goals in a large group 1 PAH cohort against long-term survival. Methods We analyzed the PHIRST and TRIUMPH populations (n = 563, age 53.5 ± 14.7 years, female sex 79%). The predictor variables were the treatment goals measured at the end of the placebo-controlled phase (16 and 12 weeks, respectively). The primary outcome was all-cause mortality at the end of follow-up during the open-label extension phase. Results There were 73 deaths during median follow of 1072 days (range 27 to 2177). Patients who achieved a functional class I or II had better survival. Both a 6MWD ≥ 380 m and ≥ 440 m were associated with lower mortality, but survival was better in patients able to walk ≥ 440 m. The best long-term survival was achieved with functional class I or II and 6MWD ≥ 440 m. In a subset of the population, cardiac index > 2.5 L/min/m2 was weakly associated with better survival. Conclusion WHO functional class I or II, 6MWD ≥ 440 m and CI > 2.5 L/min/m2 measured 12–16 weeks after the introduction of PAH-targeted therapy are associated with better long-term survival in PAH. Best survival is observed by reaching both the functional class and the 6MWD goals. Keywords Pulmonary hypertension · Survival · Treatment goals
Introduction Pulmonary arterial hypertension (PAH) is characterized by progressive remodeling of the resistance pulmonary arteries which leads to elevated pulmonary vascular resistance, right ventricular failure and premature death. Over the past 30 years, several medications that target pathways involved in the disease process have become available. These PAH-targeted therapies have improved the quality of life Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00408-020-00402-w) contains supplementary material, which is available to authorized users. * Gustavo A. Heresi [email protected] 1
Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, A90, Cleveland, OH 44195, USA
United Therapeutics, Durham, NC, USA
2
and clinical outcomes of PAH patients [1]. Despite treatment advances, 5-year survival of newly diagnosed patients remains poor at 61.2% [2]. To improve outcomes, current guidelines recommend escalating medical therapy to achieve treatment goals such as the functional class, 6-min walk distance (6MWD), natriuretic peptide blood levels, and right at
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