Pulmonary hypertension: Real-world data from a Portuguese expert referral centre

Abstract

Background: Pulmonary hypertension (PH) is a heterogeneous, debilitating condition with highly relevant impact on functional capacity, quality of life, and life-expectancy.

Objectives: This study aims to provide long-term data on the Portuguese PH population, by characterising the clinical presentation, evolution, and outcomes of PH patients in a specialised referral centre.

Methods: Retrospective analysis of a cohort of 101 patients with pre-capillary PH (pcPH) referenced to an expert tertiary care referral centre in northern Portugal from 2002 to 2013. Diagnosis was confirmed by right heart catheterisation (RHC). PH classification followed consensus criteria from the 5th World Symposium in Nice, 2013.

Results: The most frequent causes of pcPH were Group 1 PH - pulmonary arterial hypertension (PAH) (54.4%) and Group 4 PH - Chronic thromboembolic pulmonary hypertension (CTEPH) (25.7%); importantly, 17.8% of patients presented PH associated with multiple aetiologies. Targeted therapy was used in 91.1% of patients (48.5% combination therapy). 1-, 3-, and 5-year survival was estimated at 86.6%, 76.7%, and 64.1%, respectively. Survival was significantly better for those ≤40 years old (10.5 vs. 6.4 years; P=0.003) and for women with I/HPAH (9.3 vs. 4.5 years; P=0.039).

Conclusions: 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.

Keywords: Cohort; Portuguese population; Pulmonary arterial hypertension; Pulmonary hypertension; Real-world data; Survival.