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Background Health-related quality of life (HRQoL) impairment is common in pulmonary hypertension (PH), but its clinical predictors are not well established. This study aims to characterize the HRQoL of patients with pulmonary arterial hypertension (PAH) and other precapillary forms of PH (pcPH) and to explore its clinical correlates. Materials and Methods A cross-sectional, observational study of patients with documented PAH and other forms of pcPH. Patients completed two patient-reported outcome measures (PROM): Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and Nottingham Health Profile (NHP). Clinical characteristics were retrieved from electronic medical records. Results Mean CAMPHOR and NHP scores for the study population were indicative of a moderate HRQoL impairment. Patients in World Health Organisation Functional Classes (WHO FC) III/IV showed significantly worse HRQoL. The main clinical correlates of HRQoL were WHO FC, 6-minute walking distance (6MWD), and Borg dyspnoea index. Overall quality of life (QoL), assessed through CAMPHOR's QoL domain, showed patterns comparable to HRQoL measured by both instruments. Conclusions HRQoL, measured by two different PROMs, is impaired in Portuguese patients with PAH and other forms of pcPH, particularly in patients with increased disease severity. WHO FC, 6MWD, and Borg dyspnoea index are highly correlated with HRQoL and QoL.

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This study provides long-term, real-world data for the management of PAH and CTEPH in Portugal and demonstrates the importance of dedicated electronic medical records and well defined clinical management protocols for better patient outcomes. Patients presented mostly with intermediate or high risk of mortality, which suggests delayed diagnosis and highlights the need to increase awareness among clinicians.

Our data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis.