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Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Pulmonary hypertension (PH) can be diagnosed in the context of connective tissue diseases (CTD) as well as in elderly patients with multiple comorbidities. A correct clinical differential diagnosis and classification is essential before adequate therapeutic decisions can be made. Differential diagnosis of PH in CTD comprises associated pulmonary arterial hypertension (APAH), group 2 or 3 PH (PH arising from left heart or chronic lung disease), chronic thromboembolic PH (PH) and group 5 (e. g. in the context of terminal renal insufficiency). This is also true of elderly patients in whom the decision has to be made if the increasing number of coincident diseases lead to PH or have to be interpreted as comorbidities. In this manuscript, the differential diagnosis of PH is elucidated, focusing on CTD, in the context of left heart disease and chronic lung disease. Furthermore, criteria are presented facilitating an objective approach in this context., Competing Interests: Hanno Leuchte erhielt innerhalb der letzten 5 Jahre Honorar für Vorträge und Beratungen sowie Sponsoring von Vortragsveranstaltungen von Actelion, AstraZeneca, Bayer Healthcare, Boehringer Ingelheim, GSK, MSD, Novartis und OMT.Michael Halank erhielt Honorar für Fortbildungsveranstaltungen und für Beratungen, Kostenübernahme für Kongressreisen/Unterkunft und für Tagungsspesen von Acceleron, Actelion, AstraZeneca, Bayer, BERLIN CHEMIE, GSK, MSD, Novartis und OMT.Matthias Held erhielt Honorare für Vorträge, Beratung und Reisekostenunterstützung zu Veranstaltungen von Actelion, Bayer Healthcare, Berlin Chemie, Boehringer Ingelheim, GSK, MSD, Novartis, Pfizer, United Therapeutics.Ralf Ewert hat bezahlte wissenschaftliche Vorträge für Actelion, Janssen-Cilag, GSK, Bayer Vital, OMT, United Therapeutics, AstraZeneca, Pfizer gehalten. Es wurden Honorare für die Mitarbeit im Advisory Board der genannten Firmen gezahlt. Finanzielle Projektförderung erfolgte durch Actelion, OMT und Boehringer Ingelheim.Hans Klose erhielt Honorare für Vorträge und/oder Beratungstätigkeit von Actelion, Janssen-Cilag, MSD und OMT GmbH, GSK, Novartis, Astra Zeneca, Chiesi, Berlin Chemie. Tobias Lange erhielt Honorare für Vorträge und/oder Beratungstätigkeit von Acceleron Pharma, Actelion, BMS, Janssen-Cilag, MSD und OMT GmbH.Joachim Meyer: relevante Interessenkonflikte der vergangenen drei Jahre: Fa. Actelion Pharmaceutic als Referentenhonorar, 2019.Dirk Skowasch erhielt Honorare für Vorträge und Beratungstätigkeit von Actelion/Janssen-Cilag.Heinrike Wilkens erhielt Honorare für Vorträge und/oder Beratertätigkeit von Actelion, Janssen-Cilag, Bayer, Biotest, Boehringer-Ingelheim, GSK, MSD, Pfizer und Roche.Hans-Jürgen Seyfarth erhielt in den letzten 3 Jahren Vortragshonorare von Janssen/Actelion und GSK sowie Beraterhonorare von Janssen/Actelion., (Thieme. All rights reserved.)

At the 6th World Symposium on Pulmonary Hypertension (WSPH), which took place from February 27 until March 1, 2018 in Nice, scientific progress over the past 5 years in the field of pulmonary hypertension (PH) was presented by 13 working groups. The results of the discussion were published as proceedings towards the end of 2018. One of the major changes suggested by the WSPH was the lowering of the diagnostic threshold for PH from ≥ 25 to > 20 mmHg mean pulmonary arterial pressure, measured by right heart catheterization at rest. In addition, the pulmonary vascular resistance was introduced into the definition of PH, which underlines the importance of cardiac output determination at the diagnostic right heart catheterization.In this article, we discuss the rationale and possible consequences of a changed PH definition in the context of the current literature. Further, we provide a current overview on non-invasive and invasive methods for diagnosis, differential diagnosis, and prognosis of PH, including exercise tests., Competing Interests: T. J. Lange erhielt Honorare für Vorträge und/oder Beratungstätigkeit von Acceleron Pharma, Actelion, BMS, Janssen-Cilag, MSD und OMT GmbH.R. Ewert erhielt Forschungsunterstützung von OMT, Boehringer Ingelheim, Janssen. Er hat bezahlte wissenschaftliche Vorträge für Actelion, Janssen, OMT, Novartis, AstraZeneca, Boehringer Ingelheim, Bayer Vital und GSK gehalten. Teilnahme an bezahlten Advisory Boards für Actelion, OMT, GS werden angegeben.M. Halank erhielt Honorar für Fortbildungsveranstaltungen und für Beratungen, Kostenübernahme für Kongressreisen/Unterkunft undfür Tagungsspesen von Acceleron, Actelion, AstraZeneca, Bayer, Berlin Chemie, GSK, MSD, Novartis und OMT.H. Klose erhielt Honorare für Vorträge und/oder Beratungstätigkeit von Actelion, Janssen-Cilag, MSD und OMT GmbH, GSK, Novartis,Astra Zeneca, Chiesi, Berlin Chemie.H. Leuchte erhielt innerhalb der letzten 5 Jahre Honorar für Vorträge und Beratungen sowie Sponsoring von Vortragsveranstaltungen von Actelion, AstraZeneca, Bayer Healthcare, Boehringer Ingelheim, GSK, MSD, Novartis und OMT.F. J. Meyer erhielt Referentenhonorare von Actelion.H.-J. Seyfarth erhielt Honorare von Actelion für Vorträge und Beratertätigkeit sowie von GSK und Bayer für Vorträge.D. Skowasch erhielt Honorare für Vorträge und Beratungstätigkeit von Actelion/Janssen-Cilag.H. Wilkens erhielt Honorare für Vorträge und/oder Beratertätiigkeit von Actelion, Janssen-Cilag, Bayer, Biotest, Boehringer-Ingelheim, GSK, MSD, Pfizer und Roche.M. Held erhielt Honorare für Vorträge, Beratung und Reisekostenunterstützung zu Veranstaltungen von Actelion, Bayer Healthcare, Berlin Chemie, Boehringer Ingelheim, GSK, MSD, Novartis, Pfizer, United Therapeutics.Die anderen Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

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 Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. 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. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the targeted therapy of PAH. This article summarizes the results and recommendations of the working group on targeted treatment of PAH., (© Georg Thieme Verlag KG Stuttgart · New York.)

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed information about the diagnosis of pulmonary hypertension, and furthermore provide novel recommendations for risk stratification and follow-up assessments. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. 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. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to risk stratification and follow-up assessment of patients with PAH. This manuscript summarizes the results and recommendations of this working group., (© Georg Thieme Verlag KG Stuttgart · New York.)

Riociguat is the first clinically available soluble Guanylate-cyclase stimulator (sGC) and representative of a completely new class of drugs. Riociguat is approved for pulmonary arterial hypertension (PAH) and non-operable or recurrent/persistent chronic thromboembolic pulmonary hypertension (CTEPH). Moreover, Riociguat is currently under investigation for a wider spectrum of diseases. This article focusses on its mode of action and clinical trial data. Finally, based on these data, the status of approval, as well as the costs a proposal is given how Riociguat can be integrated in the current treatment of PAH and CTEPH.

An 87-year-old woman presented with a left-sided pleural effusion. The milky aspirate fulfilled the criteria of a chylothorax. Thorax computed tomography (CT) showed characteristic multiple cysts and consequently the rare diagnosis of post-menopausal pulmonary lymphangioleiomyomatosis was made. In the diagnostic work-up of pleural effusion the determination of triglycerides should be considered to confirm the diagnosis of chylothorax. In the presence of a chylothorax the differential diagnosis of lymphangioleiomyomatosis should be included even in advanced age.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension have been adopted for Germany. The guidelines contain detailed recommendations for the diagnosis of pulmonary hypertension. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update y appears necessary. In June 2010, 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. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to non-invasive diagnosis of PH. This commentary summarizes the results and recommendations of the working group on treatment of PAH., (© Georg Thieme Verlag KG Stuttgart · New York.)

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) have been adopted for Germany. Invasive hemodynamic data obtained by right heart catheterization are essential to confirm the diagnosis, test vasoreactivity, assess severity and guide therapy in PH patients. The definition of PH is resting on a mean pulmonary artery pressure ≥ 25 mm Hg obtained by right heart catheterization. Furthermore, a pulmonary capillary wedge pressure > 15 mm Hg excludes pre-capillary PH. Vasoreactivity testing is part of the diagnostic work-up in pulmonary arterial hypertension. Recent data on the use of inhaled iloprost update these guidelines and are of special importance due to the frequent diagnostic use of iloprost in Germany. Other aspects of invasive hemodynamic data in certain PH subgroups as well as their measurement and interpretation in children are discussed. Several aspects of right heart catheterization in PH justify a detailed commentary, and in some areas an update already appears necessary. In June 2010, 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 Paediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups were initiated, one of which was specifically addressing the invasive hemodynamic evaluation of patients with PH. This commentary summarizes the results and recommendations of this working group., (© Georg Thieme Verlag KG Stuttgart · New York.)

In suspected acute pulmonary embolism, the measurement of the d-dimer is now an established screening method. Further diagnostic measures include the electrocardiogram, echocardiography and conventional chest radiography, as well as the analysis of blood gases. The specific demonstration of a pulmonary embolism is achieved with a helical CT of the thorax, pulmonary angiography and ventilation/perfusion scanning. Apart from continuous (infusion) of unfractionated heparin, the low molecular weight heparins also are of potential value in the treatment of pulmonary embolism. A larger percentage of patients benefit from the use of fibrinolytic agents than was previously thought, so that the indication for fibrinolytic therapy should not be made dependent on the presence of hemodynamic instability.