Abstract
RATIONALE: Pulmonary arterial hypertension (PAH) is characterized in part by increased dead space ventilation, which can be estimated noninvasively at the bedside by measurement of end-tidal CO(2) (ETco(2)). OBJECTIVES: Prior work has demonstrated that ETco(2) is lower in patients with PAH than in control patients, but whether ETco(2) has prognostic value is unknown. We hypothesized that lower measurements of ETco(2) in patients with PAH correlate with worse long-term outcomes. METHODS: Patients with PAH seen in our referral clinic were prospectively recruited for ETco(2) measurement between September 2009 and February 2010. Vital status as of July 2015 was documented using medical records and the Social Security Death Index. RESULTS: Eighty-two patients were followed for a median of 60 months. Twenty-six patients died, and two were lost to follow-up. Patients who died were more likely to be older (58.5 ± 14.9 vs. 47.6 ± 12.2 yr; P < 0.05) and to have shorter 6-minute walk distance (296 ± 127 vs. 401 ± 92 m; P < 0.05). Mean ETco(2) in survivors was 30.5 ± 4.8 mm Hg, whereas mean ETco(2) in patients who died was 27.1 ± 4.2 mm Hg (P = 0.004). After stratification by median baseline ETco(2) of 29 mm Hg, survival in each group was analyzed. Patients with lower ETco(2) had shorter survival (P = 0.006). Cox regression analysis with ETco(2) as a continuous variable revealed the hazard ratio to be 0.88 (95% confidence interval, 0.80-0.97; P = 0.006). In 52 patients with more than one measurement a median of 17 months apart, ETco(2) was unchanged. CONCLUSIONS: Our single-center data suggest that lower ETco(2) is associated with shorter survival and that ETco(2) is stable over time in patients with PAH.