Five-Year Outcomes of Patients Enrolled in the REVEAL Registry

作者：Harrison W. Farber, Dave P. Miller, Abby Poms, David B. Badesch, Adaani Frost, Erwan Muros‐Le Rouzic, Alain Romero, Wade W. Benton, C. Gregory Elliott, Michael D. McGoon, Raymond L. Benza · 发表于：CHEST Journal · 年份：2015 · DOI：10.1378/chest.15-0300 · 被引用次数：528 · 研究领域：Pulmonary Hypertension Research and Treatments、Cancer Research and Treatment、Cardiovascular Function and Risk Factors

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare, severe disease characterized by worsening right-sided heart failure, decreasing functional status, and poor survival. The present study characterizes the 5-year survival in the United States of a new and previous diagnosis of PAH in patients stratified by baseline functional class (FC). The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) is a 55-center observational US registry of the demographics, disease course, and management of patients with World Health Organization (WHO) group 1 PAH. METHODS: The REVEAL Registry enrolled newly and previously diagnosed patients aged ≥ 3 months with WHO group 1 PAH consecutively from March 2006 to December 2009. Demographics, disease characteristics, and hemodynamic data were collected at enrollment. Survival analysis was conducted by FC and other subgroups in patients aged ≥ 18 years. RESULTS: Survival differences between previously diagnosed and newly diagnosed patients at 1 year (90.4% vs 86.3%) were maintained to 5 years; 5-year survival for previously diagnosed patients was 65.4% compared with 61.2% for newly diagnosed patients. Previously diagnosed patients in FC I, II, III, and IV had an estimated 5-year survival rate of 88.0%, 75.6%, 57.0%, and 27.2%, respectively, compared with 72.2%, 71.7%, 60.0%, and 43.8% for newly diagnosed patients in FC I, II, III, and IV, respectively. CONCLUSIONS: Patient survival of advanced PAH remains poor at...