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The ESC/ERS guidelines (published at the end of 2015) and other international recommendations defined pulmonary hypertension (PH) by an invasively measured mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest. At the 6 th World Symposium on Pulmonary Hypertension in Nice a modification of this hemodynamic definition in the sense of lowering the threshold to > 20 mmHg was proposed. A pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU) is additionally required for the diagnosis of pre-capillary PH. This modification must be critically reviewed with regard to the underlying rationale and possible consequences. Therefore, a detailed explanation is required. In particular, it must be made clear that this change currently has no influence on the evidence-based and approval-compliant prescription of drugs for the targeted therapy of pulmonary arterial hypertension (PAH)., Competing Interests: S. Rosenkranz: Honorare für Vorträge und/oder Beratertätigkeiten von Abbott, Actelion, Arena, Bayer, Ferrer, GSK, MSD, Novartis, Pfizer und United Therapeutics. Forschungsunterstützung an Institutionen von Actelion, Bayer, Novartis, Pfizer und United Therapeutics. G.P. Diller: Forschungsunterstützung und/oder Konferenz-Teilnahme und/oder Honorare für Vorträge und/oder Beratertätigkeit von Actelion, Pfizer, Daiichi-Sankyo, AOP Orphan Therapeuticals. D. Dumitrescu: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, GSK, MSD, Novartis, Pfizer, Servier. Forschungsunterstützung an Institution von Actelion. R. Ewert: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, GSK, Merck, OMT, Pfizer, United Therapeutics. H.A. Ghofrani: Honorare für Vorträge und/oder Konsultationen von Actelion, Bayer, Gilead, GSK, MSD, Novartis, Pfizer und United Therapeutics. E. Grünig: Honorare für Beratertätigkeiten und/oder Vorträge von Actelion, Bayer, GSK, Novartis, Pfizer und United Therapeutics. M. Halank: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, AOP orphan/OMT, Bayer, Gilead, GSK, MSD, Novartis und Pfizer. M. Held: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, Berlin Chemie, Boehringer, GSK, MSD, Novartis, Pfizer, United Therapeutics. H. Kaemmerer: Forschungsunterstützung und/oder Konferenz-Teilnahme und/oder Honorare für Vorträge und/oder Beratertätigkeit von Actelion, Bayer, Pfizer. H. Klose: Honorare für Vorträge und/oder Beratertätigkeiten und Kongressteilnahmeunterstützung von Actelion, Bayer, MSD, GSK, UT, Novartis, OMT und Pfizer; Forschungsunterstützung von GSK, Actelion und Bayer. G. Kovacs: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, GSK, MSD, Novartis, Pfizer, Chiesi, Boehringer-Ingelheim. S. Konstantinides: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, Boehringer. I.M. Lang: Honorare für Beratertätigkeiten und/oder Vorträge von Actelion, AOP Orphan, Astra-Zeneca, Bayer-Schering, Cordis, Daiichi-Sankyo, Edwards, GSK, Medtronic, Novartis, Pfizer, Servier, United Therapeutics. T.J. Lange: Honorare für Vorträge und/oder Beratertätigkeiten und/oder Forschungsunterstützung an Institutionen von Actelion, AOP orphan/OMT, Bayer, GSK, Pfizer and United Therapeutics. H. Leuchte: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, GSK, Merck, und Pfizer. E. Mayer: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, MSD und Pfizer. A. Olschewski: Honorare für Vorträge und/oder Beratertätigkeiten und/oder Forschungsunterstützung an Institutionen von Bayer, GSK, Pfizer. H. Olschewski: Honorare für Vorträge und/oder Beratertätigkeiten und/oder Forschungsunterstützung an Institutionen von Actelion, Bayer, Belerophon, Boehringer, GSK, Menarini, MSD, Novartis, Pfizer, Roche. K.M. Olsson: Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer, GSK, Pfizer und United Therapeutics. C. Opitz: Keine persönlichen Einkünfte als mögliche Interessenkonflikte. R.T. Schermuly: Honorare für Vorträge und/oder Beratertätigkeiten und/oder Forschungsunterstützung an Institutionen von Bayer, Gilead, MSD, Novartis, United Therapeutics. W. Seeger: Honorare für Vorträge und/oder Beratertätigkeiten und/oder Forschungsunterstützung an Institutionen von Actelion, Bayer, Pfizer, Novartis. H. Wilkens: Honorare für Beratertätigkeiten und/oder Vorträge von Actelion, Bayer, Boehringer-Ingelheim, GSK, MSD, Novartis, Pfizer und Roche. M.M. Hoeper: Honorare für Beratertätigkeiten und/oder Vorträge von Actelion, Bayer, Gilead, GSK, Merck und Pfizer., (© Georg Thieme Verlag KG Stuttgart · New York.)

The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH), but also other significant subgroups of pulmonary hypertension (PH). In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. Several working groups were initiated, one of which was dedicated to the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). In every patient with PH of unknown cause CTEPH should be excluded. The primary treatment option is surgical pulmonary endarterectomy (PEA) in a specialized multidisciplinary CTEPH center. Inoperable patients or patients with persistent or recurrent CTEPH after PEA are candidates for targeted drug therapy. For balloon pulmonary angioplasty (BPA), there is currently only limited experience. This option - as PEA - is reserved to specialized centers with expertise for this treatment method. In addition, a brief overview is given on pulmonary artery sarcoma, since its surgical treatment is often analogous to PEA. The recommendations of this working group are summarized in the present paper., (© Georg Thieme Verlag KG Stuttgart · New York.)

This position paper summarises current developments in chronic thromboembolic pulmonary hypertension (CTEPH) including diagnostic approaches and treatment options. Based on the guidelines of the task force of CTEPH experts at the 5th World Symposium on Pulmonary Hypertension in Nice 2013. Open questions arising during the treatment of patients with CTEPH are addressed. Patients with suspected CTEPH should undergo echocardiography and cardiopulmonary exercise testing. A ventilation/perfusion scan is the recommended imaging test for screening in the diagnostic algorithm for the evaluation of CTEPH. CTEPH-patients should be discussed in an expert center with an interdisciplinary team and an experienced PEA surgeon to decide the further treatment. Pulmonary endarterectomy (PEA) is the treatment of choice for patients with CTEPH. Medical therapy with PH-targeted medications for inoperable CTEPH and residual disease after PEA should only be initiated if evaluation reveals that the patient is no candidate for a PEA. Current data suggest that CTEPH patients treated with PEA have a better long-term survival rate and quality of life than patients treated with medical therapy., (© Georg Thieme Verlag KG Stuttgart · New York.)