TY - JOUR T1 - Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0050-2019 VL - 28 IS - 154 SP - 190050 AU - Katleen Swinnen AU - Rozenn Quarck AU - Laurent Godinas AU - Catharina Belge AU - Marion Delcroix Y1 - 2019/12/31 UR - http://err.ersjournals.com/content/28/154/190050.abstract N2 - Pulmonary arterial hypertension is a rare and incurable chronic disease characterised by a progressive increase in pulmonary vascular resistance and right heart failure. Patient registries collecting observational data can be of great value in the understanding of clinical problems. While clinical trials provide data in selected patient populations, registries better depict real-life practice. This review aims to reflect the input of patient registries in the general knowledge of the disease. Advances in epidemiology of the different subgroups, including data on incidence and/or prevalence, increasing age at presentation and stagnating diagnostic delay are reported. The importance of haemodynamic definition criteria and cardiac comorbidities are underscored. The review also shows the major transformation that pulmonary arterial hypertension therapeutic management has undergone, with still insufficient use of combination therapies; consecutive improvement in outcome; upcoming evidence in disfavour of anticoagulation; and validity of the available risk-stratification tools derived from large registries. Product registries are also briefly presented. Finally, the benefits of registries and methodological aspects are discussed, including immortal time bias, registry data quality and recommendations from EU organisations (EUCERD and PARENT).Registries are essential for tracing clinical evolution in PAH and implementing recommendations for practice. Modern management of registries should be further developed and extended, since many questions remain unanswered. http://bit.ly/2Y1umZK ER -