The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire scores predict mortality and hospitalizations in patients with pulmonary hypertension

Abstract Introduction Pulmonary hypertension (PH) impacts patients' health-related quality of life (HRQoL). The prognostic value of HRQoL, using the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire, is underdetermined. Aim To evaluate whether the CAMPHOR scales can independently predict adverse outcomes. Methods We prospectively enrolled PH patients. Clinical and performance parameters, along with HRQoL assessed with the CAMPHOR, were evaluated. Adverse outcomes, defined as PH-related hospitalizations or mortality, were recorded. Data were analyzed in two stages: univariate analyses (including Kaplan-Meier curves and log-rank tests) and Cox regression models grouped into blocks: 1) basic patient characteristics: sex, age; 2) NTproBNP, mPAP and echocardiographic measurements; 3) physical performance: WHO-FC, 6MWT, Borg dyspnea score, SpO2. Results 117 PH patients (mean age 66±14 years, 56% female) were included. The median CAMPHOR values were -1.18 logits for symptoms (IQR -2.19;0.36), -2.26 for activities (IQR -4.13;0.71) and -1.08 for QoL (IQR -2.24;0.32). Over a median follow-up of 24 months (range: 0.3-50 months), 55 (47%) patients experienced an adverse event. Kaplan-Meier curves showed an increased risk for adverse outcomes in patients with CAMPHOR scores above the median in all three subscales (Figure 1). Higher scores were associated with adverse outcomes (symptoms: HR=1.29, 95%CI=1.11–1.49, p=0.001; activities: HR=1.16, 95%CI=1.06-1.27, p=0.001; QoL: HR=1.28, 95%CI=1.11-1.47, p=0.001). High scores remained predictive of adverse outcomes when adjusted for variables in the three thematic blocks. Conclusion High CAMPHOR scores, indicating worse HRQoL, identified PH patients at increased risk of adverse outcome.