A Gomes1, C Cruz1, J Rocha1, M Ricardo1, M Vicente2, A Melo3, M Santos4, L Carvalho5, F Gonçalves5, A Reis6. 1. Internal Medicine Service, Medicine Department, Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal. 2. Department of Health Sciences, University of Aveiro, Aveiro, Portugal. 3. Pulmonary Vascular Disease Unit, Medicine Department, Centro Hospitalar do Porto Hospital de Santo António, Porto, Portugal. 4. Pulmonary Vascular Disease Unit, Medicine Department, Centro Hospitalar do Porto Hospital de Santo António, Porto, Portugal; Cardiology Service, Medicine Department, Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal. 5. Internal Medicine Service, Medicine Department, Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal; Pulmonary Vascular Disease Unit, Medicine Department, Centro Hospitalar do Porto Hospital de Santo António, Porto, Portugal. 6. Pulmonary Vascular Disease Unit, Medicine Department, Centro Hospitalar do Porto Hospital de Santo António, Porto, Portugal. Electronic address: reisabilio@gmail.com.
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.
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.
Authors: Sophia Emmons-Bell; Catherine Johnson; Alexandra Boon-Dooley; Paul A Corris; Peter J Leary; Stuart Rich; Magdi Yacoub; Gregory A Roth Journal: Pulm Circ Date: 2022-01-18 Impact factor: 2.886
Authors: Abdullah M Aldalaan; Sarfraz A Saleemi; Ihab Weheba; Abeer Abdelsayed; Pekka Hämmäinen; Maha M Aleid; Fatima Alzubi; Hamdeia Zaytoun; Nadeen Alharbi Journal: ERJ Open Res Date: 2020-05-11