Christian F Opitz1, Marius M Hoeper2, J Simon R Gibbs3, Harald Kaemmerer4, Joanna Pepke-Zaba5, J Gerry Coghlan6, Laura Scelsi7, Michele D'Alto8, Karen M Olsson2, Silvia Ulrich9, Werner Scholtz10, Uwe Schulz11, Ekkehard Grünig12, Carmine D Vizza13, Gerd Staehler14, Leonhard Bruch15, Doerte Huscher16, David Pittrow17, Stephan Rosenkranz18. 1. Department of Cardiology, DRK Kliniken Berlin, Berlin, Germany; Department of Cardiology, Medical University of Greifswald, Greifswald, Germany. Electronic address: c.opitz@drk-kliniken-berlin.de. 2. Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany. 3. Department of Cardiology, National Heart & Lung Institute, Imperial College London, London, United Kingdom. 4. Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center, München, Technical University Munich, Munich, Germany. 5. Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge, United Kingdom. 6. Cardiology Department, Royal Free Hospital, London, United Kingdom. 7. Division of Cardiology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. 8. Department of Cardiology, Second University of Naples-Monaldi Hospital, Naples, Italy. 9. Clinic of Pulmonology, University Hospital Zurich, Zurich, Switzerland. 10. Department of Cardiology, Heart and Diabetes Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany. 11. Clinic for Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, Ruhr University Bochum, Bad Oeynhausen, Germany. 12. Centre for Pulmonary Hypertension, University Hospital, Heidelberg, Germany. 13. Pulmonary Hypertension Unit Department of Cardiovascular and Respiratory Disease, University of Rome 'La Sapienza,' Rome, Italy. 14. Medical Clinic I, Pneumology, Loewenstein Clinic, Loewenstein, Germany. 15. Department of Internal Medicine, Unfallkrankenhaus Berlin, Berlin, Germany. 16. Epidemiology Unit, German Rheumatism Research Centre, Berlin; Department of Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany. 17. Institute for Clinical Pharmacology, Medical Faculty, Technical University Carl Gustav Carus, Dresden, Germany. 18. Clinic III for Internal Medicine (Cardiology), and Center for Molecular Medicine Cologne (CMMC), University of Cologne, Cologne, Germany; Cologne Cardiovascular Research Center (CCRC), University of Cologne, Cologne, Germany.
Abstract
BACKGROUND: Pulmonary hypertension (PH) is hemodynamically classified as pre-capillary (as seen in idiopathic pulmonary arterial hypertension [IPAH]) or post-capillary (as seen in heart failure with preserved ejection fraction [HFpEF]). Overlaps between these conditions exist. Some patients present with risk factors for left heart disease but pre-capillary PH, whereas patients with HFpEF may have combined pre- and post-capillary PH. OBJECTIVES: This study sought to further characterize similarities and differences among patient populations with either PH-HFpEF or IPAH. METHODS: We used registry data to analyze clinical characteristics, hemodynamics, and treatment responses in patients with typical IPAH (<3 risk factors for left heart disease; n = 421), atypical IPAH (≥3 risk factors for left heart disease; n = 139), and PH-HFpEF (n = 226) receiving PH-targeted therapy. RESULTS: Compared with typical IPAH, patients with atypical IPAH and PH-HFpEF were older, had a higher body mass index, had more comorbidities, and had a lower 6-min walking distance, whereas mean pulmonary artery pressure (46.9 ± 13.3 mm Hg vs. 43.9 ± 10.7 mm Hg vs. 45.7 ± 9.4 mm Hg, respectively) and cardiac index (2.3 ± 0.8 l/min/m(2) vs. 2.2 ± 0.8 l/min/m(2) vs. 2.2 ± 0.7 l/min/m(2), respectively) were comparable among groups. After initiation of targeted PH therapies, all groups showed improvement in exercise capacity, functional class, and natriuretic peptides from baseline to 12 months, but treatment effects were less pronounced in patients with PH-HFpEF than typical IPAH; with atypical IPAH in between. Survival rates at 1, 3, and 5 years were almost identical for the 3 groups. CONCLUSIONS: Patients with atypical IPAH share features of both typical IPAH and PH-HFpEF, suggesting that there may be a continuum between these conditions.
BACKGROUND: Pulmonary hypertension (PH) is hemodynamically classified as pre-capillary (as seen in idiopathic pulmonary arterial hypertension [IPAH]) or post-capillary (as seen in heart failure with preserved ejection fraction [HFpEF]). Overlaps between these conditions exist. Some patients present with risk factors for left heart disease but pre-capillary PH, whereas patients with HFpEF may have combined pre- and post-capillary PH. OBJECTIVES: This study sought to further characterize similarities and differences among patient populations with either PH-HFpEF or IPAH. METHODS: We used registry data to analyze clinical characteristics, hemodynamics, and treatment responses in patients with typical IPAH (<3 risk factors for left heart disease; n = 421), atypical IPAH (≥3 risk factors for left heart disease; n = 139), and PH-HFpEF (n = 226) receiving PH-targeted therapy. RESULTS: Compared with typical IPAH, patients with atypical IPAH and PH-HFpEF were older, had a higher body mass index, had more comorbidities, and had a lower 6-min walking distance, whereas mean pulmonary artery pressure (46.9 ± 13.3 mm Hg vs. 43.9 ± 10.7 mm Hg vs. 45.7 ± 9.4 mm Hg, respectively) and cardiac index (2.3 ± 0.8 l/min/m(2) vs. 2.2 ± 0.8 l/min/m(2) vs. 2.2 ± 0.7 l/min/m(2), respectively) were comparable among groups. After initiation of targeted PH therapies, all groups showed improvement in exercise capacity, functional class, and natriuretic peptides from baseline to 12 months, but treatment effects were less pronounced in patients with PH-HFpEF than typical IPAH; with atypical IPAH in between. Survival rates at 1, 3, and 5 years were almost identical for the 3 groups. CONCLUSIONS:Patients with atypical IPAH share features of both typical IPAH and PH-HFpEF, suggesting that there may be a continuum between these conditions.
Authors: Marius M Hoeper; Tilmann Kramer; Zixuan Pan; Christina A Eichstaedt; Jens Spiesshoefer; Nicola Benjamin; Karen M Olsson; Katrin Meyer; Carmine Dario Vizza; Anton Vonk-Noordegraaf; Oliver Distler; Christian Opitz; J Simon R Gibbs; Marion Delcroix; H Ardeschir Ghofrani; Doerte Huscher; David Pittrow; Stephan Rosenkranz; Ekkehard Grünig Journal: Eur Respir J Date: 2017-08-03 Impact factor: 16.671
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