Background: Although younger female patients were diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 1980s, it is now frequently encountered in elderly patients with cardiovascular comorbidities (CVCs) associated with increased risk for left heart disease (left ventricular diastolic dysfunction).
Methods: Present data until November 2019 regarding specific features and clinical outcomes of the IPAH population from the Hellenic Pulmonary Hypertension Registry (HOPE). Patients were divided into two groups based on the presence of ≥3 CVCs or <3 CVCs; arterial hypertension, diabetes mellitus, obesity, presence of coronary artery disease or atrial fibrillation.
Results: Overall, 77 patients with IPAH {55.1 [interquartile range (IQR) 24.1] years, 62.8% women} were analysed. Fifteen patients (19.2%) had ≥3 CVCs, and 25 (32%) were >65 years old. Patients with ≥3 CVCs were older [69.8 (IQR 17.8) vs. 52.1 (IQR 23.3) years, P = 0.001], presented an almost equal female-to-male ratio (1.1/1), walked less in the 6-min walk test [322 (IQR 197.8) vs. 431 (IQR 128.3) m, P = 0.003] and had lower mean arterial pulmonary pressure [39 (IQR 19) vs. 49 (IQR 16) mmHg, P = 0.011] and pulmonary vascular resistance [6.4 (4.2) vs.9.8 (5.2) Wood units, P = 0.008] at baseline than patients with fewer CVCs. Fewer patients with ≥3 CVCs received PAH-specific treatment compared with patients with fewer comorbidities [n = 11 (73.3%) vs. n = 58 (95.5%), P = 0.02]. During a median follow-up period of 3.8 (IQR 2.7) years, 18 patients died (all-cause mortality, 24.3%). Male sex, older age and worse risk status for 1-year mortality were associated with worse survival prospects, and CVCs did not have a significant impact on survival.
Conclusion: In this nationwide register-based study, the epidemiology of IPAH involves older patients with CVCs, who seem to have less haemodynamic compromise but worse functional impairment and are treated less aggressively with PAH pharmacotherapy.
KEY CONTRIBUTORS
Alexandra Arvanitaki, Elena Vrana, Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Greece Maria Boutsikou, Cardiology Department, Mediterraneo Hospital, Glyfada, Athens, Greece Anastasia Anthi, 1st Department of Critical Care and Pulmonary Hypertension Clinic, National & Kapodistrian University of Athens Medical School, Evaggelismos General Hospital, Athens Greece Sotiria Apostolopoulou, Cardiology – Pediatric Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece Aikaterini Avgeropoulou, Cardiology Department, Hippokration General Hospital, Athens, Greece Eftychia Demerouti, Cardiology – Pediatric Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece Alexandros Patrianakos, Department of Thoracic Medicine, University Hospital of Heraklion, Heraklion, Crete, Greece Panagiotis Karyofyllis, Cardiology – Pediatric Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece Ioanna Mitrouska, Department of Thoracic Medicine, University Hospital of Heraklion, Heraklion, Crete, Greece Sophia Anastasia Mouratoglou, Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Greece Katerina K. Naka, 2nd Department of Cardiology, University of Ioannina Medical School, University Hospital of Ioannina, Ioannina, Greece Stylianos E. Orfanos, 1st Department of Critical Care and Pulmonary Hypertension Clinic, National & Kapodistrian University of Athens Medical School, Evaggelismos General Hospital, Athens, Greece Evangelia Panagiotidou, Georgia Pitsiou, Respiratory Failure Unit, ‘G. Papanikolaou’ Hospital, Exohi, Thessaloniki, Greece Spyridon Rammos, Cardiology – Pediatric Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece Ioannis Stanopoulos, Respiratory Failure Unit, ‘G. Papanikolaou’ Hospital, Exohi, Thessaloniki, Greece Adina Thomaidi, Cardiology Department, Democritus University of Thrace, Alexandroupolis, Greece Alexandra Frogoudaki, Multidisciplinary Pulmonary Hypertension Center, Attikon University General Hospital, Athens, Greece Afroditi Boutou, Respiratory Failure Unit, ‘G. Papanikolaou’ Hospital, Exohi, Thessaloniki, Greece George Anastasiadis, Cardiology Department, Laiko General Hospital, Athens, Greece, on behalf of the Hellenic Society for the Study of Pulmonary Hypertension (HSSPH) Styliani Brili, Cardiology Department, Hippokration General Hospital, Athens, Greece Iraklis Tsangaris, Multidisciplinary Pulmonary Hypertension Center, Attikon University General Hospital, Athens, Greece Dimitrios Tsiapras, Vassilios Voudris, Cardiology – Pediatric Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece Athanasios Manginas, Cardiology Department, Mediterraneo Hospital, Glyfada, Athens, Greece George Giannakoulas, Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Greece
