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Parasitic infections by Echinococcus granulosus are rare in Germany, and predominantly affect individuals with a migration background. Liver and lungs are the most commonly affected organs. Pulmonary cysts often remain asymptomatic until rupture, at which point symptoms may manifest. The diagnostic approach typically involves a combination of imaging modalities and serological tests, occasionally supplemented by molecular genetic methods. Given the global movements of migration, considerations of the epidemiology of common diseases in the country of origin should also be taken into account in the differential diagnosis. We present the unusual case of a pneumogenic sepsis in a young man from Syria, where the combination of medical history alongside radiological, serological, and molecular genetic investigations ultimately led to the diagnosis of a severe pulmonary echinococcosis with rupture., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Acute dyspnoea is one of the most common internal medicine symptoms in the emergency department. It arises from an acute illness or from the exacerbation of a chronic illness. Symptom-related emergency structures and corresponding structural guidelines already exist in the stroke and chest pain units for dealing with the leading symptoms of acute stroke and acute chest pain. These are lacking in Germany for the key symptom of dyspnoea, although the benefits of these structures have already been proven in other countries. The German Society for Pneumology and Respiratory Medicine (DGP) has now set up a task force together with the Association of Pneumology Clinics (VPK), in order to deal with the topic and develop appropriate structural guidelines for such "dyspnoea units" in Germany. At the end of the process, the certification of such units at German hospitals is optional., Competing Interests: Dominic Dellweg: Vortragshonorar für die Firma Löwenstein Medical. Georg Nilius: Forschungsförderung von Sleep well, Löwenstein medical, Fisher & Paykel Healhtcare, Lungpacer, EllyLilly, Onera, Vortragshonorare von AstraZeneca, Boehringer Ingelheim, Berlin Chemie, Löwenstein Medical, Insmed, Reisekosten Fisher Paykel Healthcare. Achim Grünewaldt: Vortragshonorare: Boehringer Ingelheim; Honorare für Advisory Board: GSK, Forschungsunterstützung: TNI. Matthias Held: Beratungshonorare von Actelion, Bayer Healthcare, Bristol Myers Squibb, Boehringer Ingelheim, Janssen, MSD, Pfizer, Vorträge: Actelion, Astra Zeneca, Bayer HealthCare, Berlin Chemie, Boehringer Ingelheim, Bristol Myers Squibb, Daichi Sankyo, Janssen, MSD, Pfizer, Santis. Andreas Schlesinger: Vortragshonore von Chiesi, Novartis, GlaxoSmithKline, Linde, Boehringer Ingelheim, AstraZeneca, Janssen, Bristol Myers Squibb, Bayer, Berlin Chemie, medac. Winfried Randerath gibt keinen direkten Interessenkonflikt im Zusammenhang mit dem vorgelegten Beitrag an. Er hat in den vergangenen 3 Jahren Honorare für die Mitarbeit in einem Advisory Board der Firmen Bioprojet, CSL Behring, Procter & Gamble, Jazz Pharmaceuticals sowie Honorar und Reisekosten für Vortragstätigkeiten der Firmen Boehringer Ingelheim, Novartis, Bioprojet, Berlin Chemie, GSK, MSD, Westfalen Medical, Heinen und Löwenstein, Philips Respironics, Habel Medizintechnik erhalten. Die anderen Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Lung diseases and hypoventilation syndromes are often associated with pulmonary hypertension (PH). In most cases, PH is not severe. This is defined hemodynamically by a mean pulmonary arterial pressure (PAPm) > 20 mmHg, a pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance of ≤ 5 Wood units (WU). Both the non-severe (PVR ≤ 5 WU) and much more the severe PH (PVR > 5 WU) have an unfavorable prognosis.If PH is suspected, it is recommended to primarily check whether risk factors for pulmonary arterial hypertension (PAH, group 1 PH) or chronic thromboembolic pulmonary hypertension (CTEPH, group 4 PH) are present. If risk factors are present or there is a suspicion of severe PH in lung patients, it is recommended that the patient should be presented to a PH outpatient clinic promptly.For patients with severe PH associated with lung diseases, personalized, individual therapy is recommended - if possible within the framework of therapy studies. Currently, a therapy attempt with PH specific drugs should only be considered in COPD patients if the associated PH is severe and a "pulmonary vascular" phenotype (severe precapillary PH, but typically only mild to moderate airway obstruction, no or mild hypercapnia and DLCO < 45 % of predicted value) is present. In patients with severe PH associated with interstitial lung disease phosphodiesterase-5-inhibitors may be considered in individual cases. Inhaled treprostinil may be considered also in non-severe PH in this patient population., Competing Interests: M.H.: Honorare für Vortrags- und Beratertätigkeiten von AstraZeneca, Janssen und MSD. Reisekosten von Janssen. Alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.K.E.Z.: Reisekosten und Vortragstätigkeiten von Fa. MSD, Janssen und Ferrer. Alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.N.S.: Honorare für Berater- und Vortragstätigkeiten von MSD und Janssen. Alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.S.U.: Forschungsgelder vom Schweizerischen Nationalfond, der Lungenliga Schweiz, der Lunge Zürich, der Emdo Foundation, von Janssen SA, Orpha Swiss. Reisekosten und Vortragstätigkeiten von Janssen SA, Orpha Swiss, MSD und Novartis, alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.M.H.: Beratungshonorare: Actelion, Bayer Healthcare, Bristol Myers Squibb, Boehringer Ingelheim, Janssen, MSD, Pfizer. Honorare für Vorträge von: Actelion, AstraZeneca, Bayer HealthCare, Berlin Chemie, Boehringer Ingelheim, Bristol Myers Squibb, Daichi Sankyo, Janssen, MSD, Pfizer, Santis.T.K.: Honorare für Vortragstätigkeiten und Reisekostenübernahme von AstraZeneca, Berlin Chemie, Janssen und MSD. Alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.V.F.: Honorare für Vortragstätigkeiten und Reisekostenübernahme von Boehringer Ingelheim, BMS, Chiesi, Janssen, MSD, alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.M.H.: keineC.N.: Honorare für Vortrags- und Beratertätigkeiten von AstraZeneca, Janssen und MSD. Alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.J.R.: keineS.H.: Zuwendungen von Janssen, Bayer und MSD, die in keinem Zusammenhang mit dieser Arbeit stehen.D.S.: Vortrags- und Beratungshonorare und/oder Forschungsunterstützung von AstraZeneca, Bayer, Boehringer Ingelheim, Chiesi, GSK, Janssen, MSD, Sanofi, DFG, BMBF. Alle nicht im Zusammenhang mit der aktuellen Übersichtsarbeit.N.K.: erhielt finanzielle Zuwendungen von Janssen, Ferrer, Bayer und MSD, die in keinem Zusammenhang mit dieser Arbeit stehen.J.B.: Honorare für Vorträge und Beratertätigkeit von AstraZeneca, Biogen, Boehringer-Ingelheim, BMS, Ferrer, Novartis, Roche, and Sanofi-Genzyme. Für Tätigkeiten im Rahmen von Data Monitoring and Safety Boards erhielt er Honorare von Actelion und Galapagos. Es besteht kein Zusammenhang mit der vorliegenden Publikation., (Thieme. All rights reserved.)

Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue, become integrated into the vessel wall, and lead to chronic obstructions. CTEPD is differentiated into cases without pulmonary hypertension (PH), characterized by a mean pulmonary arterial pressure up to 20 mmHg and a form with PH. Then, it is still referred to as chronic thromboembolic pulmonary hypertension (CTEPH).When there is suspicion of CTEPH, initial diagnostic tests should include echocardiography and ventilation/perfusion scan to detect perfusion defects. Subsequently, referral to a CTEPH center is recommended, where further imaging diagnostics and right heart catheterization are performed to determine the appropriate treatment.Currently, three treatment modalities are available. The treatment of choice is pulmonary endarterectomy (PEA). For non-operable patients or patients with residual PH after PEA, PH-targeted medical therapy, and the interventional procedure of balloon pulmonary angioplasty (BPA) are available. Increasingly, PEA, BPA, and pharmacological therapy are combined in multimodal concepts.Patients require post-treatment follow-up, preferably at (CTE)PH centers. These centers are required to perform a minimum number of PEA surgeries (50/year) and BPA interventions (100/year)., Competing Interests: S.G. erhielt Redner- und/oder Beraterhonorare von Actelion/Janssen, Bayer, MSD.M. Halank erhielt Honorare für Vorträge von AstraZeneca, Janssen-Cilag und MSD, Honorare für Beratungen im Rahmen von AdBoards von Janssen-Cilag und MSD und finanzielle Unterstützung für die Teilnahme an Kongressen von Janssen-Cilag.M. Held erhielt Redner- und/oder Beraterhonorare von AstraZeneca, Bayer Healthcare, Berlin Chemie, Bristol Myers Squibb, Boehringer Ingelheim, Janssen, MSD, OMT, Pfizer, Santis.L.H. erhielt Redner- und/oder Beraterhonorare von Johnson&Johnson,MSD, INARI und Boston Scientific.S.K. erhielt institutionelle Zuschüsse und Redner- und/oder Beraterhonorare von Bayer AG, Daiichi Sankyo, and Boston Scientific; institutionelle Zuschüsse von Inari Medical; und Redner- und/oder Beraterhonorare von MSD and Bristol Myers Squibb/Pfizer.E.M. erhielt Redner- und/oder Beraterhonorare von Actelion/Janssen, Bayer, MSD.A.O. erhielt Rednerhonorare und Kongressreiseunterstützung von AOP, CSL Behring, Berlin Chemie, Novartis, Pfizer.H.-J. Schäfers hat keinen Interessenkonflikt.H.-J. Seyfarth erhielt Redner- und/oder Beraterhonorare Janssen.H.W. erhielt Redner- und/oder Beraterhonorare von Bayer, Biotest, Boehringer Ingelheim, Daiichi Sanchyo, Ferrer, GSK, Janssen, MSD und Roche.C.B.W. erhielt Redner- und/oder Beraterhonorare von Actelion, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD und Pfizer., (Thieme. All rights reserved.)

The new guidelines for the diagnosis and treatment of pulmonary hypertension include a new diagnostic algorithm and provide specific recommendations for the required diagnostic procedures, including screening methods. These recommendations are commented on by national experts under the auspices of the DACH. These comments provide additional decision support and background information, serving as a further guide for the complex diagnosis of pulmonary hypertension., Competing Interests: K.T.: Vortrags- und/oder Beratungshonorare von Janssen.N.K.: Vortrags- und/oder Beratungshonorare von Janssen.M.J.R.: Vortrags- und/oder Beratungshonorare von Bayer vital, Janssen, MSD.J.B.: Honorare für Vorträge und Beratertätigkeit von AstraZeneca, Biogen, Boehringer-Ingelheim, BMS, Ferrer, Novartis, Roche, and Sanofi-Genzyme. Für Tätigkeiten im Rahmen von Data Monitoring and Safety Boards erhielt er Honorare von Actelion und Galapagos. Es besteht kein Zusammenhang mit der vorliegenden Publikation.B.E. hat von folgenden Firmen Vortragshonorare bzw. Honorare für Advisory Boards innerhalb der letzten 3 Jahre erhalten: Janssen, OMT, MSD, Bayer, AOP. Alle nicht in Zusammenhang mit dieser Arbeit.A.H. erhielt Fahrtkostenerstattungen von Actelion, Pfizer, GlaxoSmithKline, Lilly und OMT; er erhielt Rednerhonorare von Encysive, Pfizer, Actelion, Medtronic, Schiller, GlaxoSmithKline, OMT, AOP Orphan und Janssen; er erhielt Autorenhonorare von Actelion; er erhielt Beraterhonorare von Actelion, Bayer, Ethypharm und GlaxoSmithKline; er besitzt Aktien von Gilead, Merck, Merck KGAA, Johnson & Johnson, Pfizer, Abbott, Siemens und Takeda. Seine Klinik beteiligte sich an Studien von Actelion, Medtronic, Edwards, Occlutec, Novartis, Lilly, Bayer und Bristol Myers Squibb; seine Klinik erhielt Forschungsgelder von Pfizer, GlaxoSmithKline, Abbott, Actelion und Medtronic. Alle nicht in Zusammenhang mit dieser Arbeit.H.G.: Vortrags-/Beraterhonorare von Amgen, Actelion, AstraZeneca, Bayer, BMS, Gossamer Bio, GSK, Janssen Cilag, Lilly, MSD, Novartis, OMT, Pfizer, United Therapeutics.M.H. erhielt Beratungshonorare von Bayer Healthcare, Bristol Myers Squibb, Boehringer Ingelheim, Janssen, MSD, Pfizer; Honorare für Vorträge von AstraZeneca, Bayer HealthCare, Berlin Chemie, Boehringer Ingelheim, Bristol Myers Squibb, Daichi Sankyo, Janssen, MSD, Pfizer, Santis.Alle anderen Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Pneumomediastinum, defined as abnormal presence of air in the mediastinum, is a rare cause of acute chest pain. The condition may occur spontaneously as well as a secondary consequence of trauma or medical interventions. The spontaneous pneumomediastinum (Hamman's syndrome) is associated with a good prognosis, even without intervention. However, undelying severe conditions such as gastrointestinal perforations should be excluded. Diagnosis might be made using conventionell chest x-ray; a CT scan may give additional useful information. A subcutanous emphysema is a common finding in patients with pneumomediastinum. The presence of air in the epidural space of the spinal canal (pneumorrhachis) is a rarely seen but likewise mostly benign complication. We report a case of a young man with Hamman's syndrome and pneumorrhachis, provoked by acute asthma exacerbation; despite pronounced symptoms, his condition could be treated conservatively., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Background:  The World Conference on PH recommended differentiation of isolated postcapillary (Ipc) and combined post- and precapillary (Cpc) PH according to pulmonary vascular resistance alone. The aim of this study was the haemodynamic and functional characterization of patients diagnosed IpcPH and CpcPH according to the current recommendation of the latest World Symposium on Pulmonary Hypertension (PH) with an exploratory data analysis., Methods:  We evaluated all consecutive patients presenting at the PH outpatient clinic of Mission Medical Hospital from 2008-2015. All received a complete diagnostic work-up according to the guidelines. We analyzed data of patients with mPAP ≥ 25 mmHg and pulmonary capillary wedge pressure (PCWP  > 15 mmHg. We compared anthropometric, hemodynamic and functional data of six-minute walking test (6 MWT), cardiopulmonary exercise testing (CPET) and echocardiography of patients with IpcPH and CpcPH., Results:  Out of 726 patients 58 showed a postcapillary PH: IpcPH: n = 20; CpcPH: n = 38. Patients with IpcPH had a significantly lower mPAP and PVR than patients with CpcPH. Cardiac index was lower in the Cpc-PH group compared to the IpcPH group. Functional capacity did not differ. CpcPH patients showed a higher right/left atrial area (RA/LA)-ratio., Discussion and Conclusion: Although CpcPH patients showed higher values of mPAP and PVR functional capacity was not worse than in patients with IpcPH. In patients with PH due to left heart disease an elevated RA/LA ratio may indicate CpcPH and invasive diagnostic work-up should be considered., Competing Interests: Matthias Held erklärt, dass er Beraterhonorare von Bayer Healthcare, Boehringer Ingelheim, Janssen, und MSD erhalten hat und Vortragshonorare von Astra Zeneca, Bayer HealthCare, Berlin Chemie, Boehringer Ingelheim, Bristol Myers Squibb, Daichi Sankyo, Janssen, MSD, Pfizer, Santis erhalten hat.Gülmisal Güder erklärt, dass sie Fördermittel des Bundesministeriums für Bildung und Forschung, der Deutschen Herzstiftung und der Deutschen Gesellschaft für Kardiologie erhalten und war beratend bzw. als Referentin für die Firmen AstraZeneca, Abiomed, Bayer, Boehringer Ingelheim, Novartis, Orion GmbH, Pfizer und Vifor Pharma Germany tätig. Mit der aktuellen Studie gibt es keine Interessenkonflikte.Franziska Joa erklärt, dass sie Vortragshonorare von Berlin-Chemie, Bayer, Boehringer Ingelheim, Actelion, Johnson and Johnson erhalten hat.Elena Pfeuffer-Jovic erklärt, Reisekostenunterstützung von Actelion, Boehringer Ingelheim, Novartis und OMT erhalten zu haben.Simon Weiner, Jörg Hoffmann und Johanna Walthelm-Hösel erklären keine Interessenkonflikte zu haben., (Thieme. All rights reserved.)

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

Despite growing experience due to increasing patient numbers, the intensive care treatment of patients with severe COVID-19 pneumonia continues to be a particular challenge in individual cases, which may also therefore legitimize individualized therapeutic attempts. In this context, the so-called hyperinflammation syndrome characterized by a cytokine storm accompanied by a massive increase in inflammatory markers such as interleukin(IL)-6, represents such a situation. This case report describes the therapeutic approach of using the IL-6-specific antibody tocilizumab in combination with hemoadsorption therapy (CytoSorb) in a 58-year-old male patient with severe COVID-19 pneumonia. The patient suffered a massive clinical deterioration with concomitant Horovitz index of 127 mmHg that occurred on the 6 th day of ventilation. After combined application of the above-mentioned therapeutic approaches, the patient stabilized rapidly paralleled by a significant increase in the Horovitz index, and the possibility of de-escalating the ventilation regimen, which ultimately enabled successful extubation after only 13 days of ventilation. Moreover, the combined treatment was associated with significant hemodynamic stabilization and a consecutive reduction in vasopressor doses, while hyperinflammation could be kept well under control. The incorporation of the hemoadsorber into the therapeutic regimen proved to be safe and straightforward. In conclusion, the combination of CytoSorb therapy and IL-6 blockade by tocilizumab appeared, at least in this case, to be an effective measure to modulate an overshooting immune response in COVID-19 pneumonia with a concomitant clinical improvement in both respiratory and hemodynamic function, and thus could be used as a potential therapeutic option in this clinical picture., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

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.)

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

A significant proportion of the current technological developments in pneumology originate from the various areas of information technology. The spectrum ranges from smartphone apps to be used in daily life or in patient care to the use of artificial intelligence in screening and early detection of diseases. The diagnostic accuracy of apps for symptom analysis is currently very limited. Research projects are performed on the integration of symptoms and functional parameters into early detection, but also on mobility measurements as a prognostic marker in COPD. Lung cancer screening using computed tomography represents a major challenge. Here, artificial intelligence can help radiologists to cope with huge amounts of data. However, the quality of the software depends on the sufficient training of the system. Technological developments shape all fields of pneumology. For diagnostic and interventional endoscopy, they offer improved biopsy techniques and microstructural imaging. Advances in lung function measurements allow the differentiated analysis of respiratory mechanical disorders, and they could be transferred to ventilation technology. The translation of basic findings about the lung microbiome into patient care may perspectively help to better understand and treat COPD exacerbations., Competing Interests: Michael Dreher erhielt Honorare für Vorträge von Actelion, Astra Zeneca, Bayer, Berlin Chemie, Boehringer Ingelheim, Chiesi, GSK, Hamilton, Heinen und Löwenstein, Intermune, Linde, Novartis, Pfizer, Philips Respironics, ResMed, Roche, Weinmann. Beratungshonorare von Almirall, Boehringer Ingelheim, Hamilton, Linde, Novartis, Pfizer, Philips Respironics, ResMed, Roche. Research Grants von Linde, Philips Respironics, ResMedRembert Koczulla erhielt Honorare für Vorträge/Advisory Boards von Sanofi, Boehringer Ingelheim, Astra Zeneca, Chiesi, Novartis, Berlin Chemie, GSK, CSL Behring, Grifols, Streamed up, Schön Klinik, Produktunterstützung von Novotec, Berater bei KaiaGernot Rohde erhielt Honorare für Vorträge und Beratungstätigkeiten von Astra Zeneca, Boehringer Ingelheim, GSK, Novartis, Roche, Berlin Chemie, Grifols, Vertex, Pfizer, MSD, Chiesi, Bayer, Insmed, BMS, Sanofi, PulmonX.Winfried Randerath erhielt Honorare für Vorträge und Beratertätigkeit von Bayer, Berlin Chemie, Boehringer Ingelheim, Heinen und Löwenstein, Novartis, Philips Respironics, ResMed.Gratiana Steinkamp erhielt Honorare für medizinisch-wissenschaftliches Publizieren von Boehringer Ingelheim, InfectoPharm, Pari GmbH und Gilead.Julia Wälscher erhielt Vortragshonorare und Reisekostenunterstützungen von Boehringer Ingelheim und Roche.Henrik Watz erhielt Honorare für Vorträge oder Beratungstätigkeit der Firmen Astra Zeneca, Boehringer Ingelheim, Bayer, BerlinChemie, Chiesi, GSK, Novartis, Takeda., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background:  Pulmonary hypertension (PH) is defined as an elevation of mean pulmonary-arterial pressure by > 20 mmHg at rest, which may lead to right heart failure. Physical exercise has not been regularly recommended for PH patients for fear of symptom deterioration or occurrence of exercise-induced adverse events., Methods:  Three electronic databases were searched for randomized, controlled trials investigating exercise training in PH patients using the following keywords: "pulmonary hypertension" OR "pulmonary arterial hypertension" AND "exercise" OR "pulmonary rehabilitation" AND "randomized"., Results:  Five studies involving 187 PH patients were included in this systematic review. Exercise programs lasted for 3 - 12 weeks (e. g. endurance training for 10 - 45 minutes; 60 - 80 % of the peak heart rate). PH patients significantly improved exercise capacity compared to controls in 6-minute walk distance (+ 45 m; 95 % CI: 26 m - 64 m) or peak oxygen consumption (+ 2.3 ml/kg/min; 95 % CI: 1.8 - 2.9 ml/kg/min), both p < 0.001. Also, physical and mental quality of life improved significantly by exercise training. No exercise-induced adverse events were observed., Conclusion:  Supervised exercise training can safely and significantly improve physical performance and quality of life in clinically stable PH patients with optimal drug treatment. However, larger studies including a wider range of PH are mandatory., Competing Interests: Die Autoren erklären, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

When caring for patients with respiratory diseases, always think of the heart! This is especially important for COPD patients, but also for a variety of other disorders of the respiratory system. At the workshop "Luftschlösser", held once more at Wiesbaden, Germany in February 2019 the many and important interactions of the lungs and the heart as well as the therapeutic implications were discussed. Based on pathophysiology, the psycho-social consequences of dyspnea, the leading symptom in patients with lung and heart disease became apparent. A particularly demanding diagnostic and therapeutic situation occurs in patients suffering simultaneously of lung and heart disease. It has been shown how frequently the diagnosis myocardial infarction is missed in COPD patients - and vice versa. Surprisingly, this is also the case in asthmatics with coronary heart disease or heart failure, a fact not readily known in clinical practice. In patients with emphysema and no apparent heart disease, hyperinflation leads to significantly restricted heart function. Reducing hyperinflation by inhaling broncholytics thus improves heart function. Biomarkers are increasingly being used for diagnostic purposes. Their role is being investigated in the large German COPD cohort COSYCONET. Lung patients suffering from more severe heart diseases pose a challenge for therapy in intensive care, especially when ventilated, and weaning from the ventilator is prolonged. Lung vessel diseases are "classic" examples of the intimate interaction of the lungs and the heart. In pulmonary arterial hypertension as well as in chronic thrombo-embolic pulmonary hypertension the lag time between the first symptoms and the definite diagnosis is often unacceptably long. For both diseases of the lung vessels therapeutic options have improved significantly over the last years. Pulmonologists should take care of this increasingly important patient group. Sleep-related breathing disorders and heart function are closely intertwined. Both conditions need special attention after the results of the SERVE-HF trial have been published. But there is no doubt that obstructive sleep apnea represents an independent and important risk factor for cardiovascular disease and needs to be treated according to existing guidelines.This workshop demonstrated impressively the multiple interactions of the respiratory system with cardiac function, resulting diagnostic and therapeutic problems, and means to overcome these problems. Guidelines for respiratory diseases should appropriately address cardiac comorbidity., Competing Interests: B. Jany erhielt Vortragshonorare und Reisekostenerstattung von Boehringer Ingelheim.R. Bals erhielt Forschungsmittel oder persönliche Bezüge von AstraZeneca, CSL Behring, Boehringer Ingelheim, Novartis, German Federal Ministry of Education and Research (BMBF), Competence Network Asthma and COPD (ASCONET), persönliche Bezüge von GlaxoSmithKline, Grifols. Weitere Förderung außerhalb dieser Publikation erfolgte durch die Wilhelm-Sander Stiftung, die Schwiete Stiftung, die Deutsche Krebshilfe und den Mukoviszidose e. V. M. Dreher erhielt Vortragshonorare von Actelion, Astra Zeneca, Bayer, Berlin Chemie, Boehringer, Chiesi, Hamilton, Heinen und Löwenstein, Intermune, Linde, Novartis, Pfizer, Philips Respironics, ResMed, Roche und Weinmann, Beraterhonorare von Almirall, Boehringer, Hamilton, Linde, Novartis, Pfizer, Philips Respironics, ResMed und Roche, Forschungsmittel von Linde, Philips Respironics und ResMed.M. Held gibt an Vortrags- und Beraterhonorare von folgenden Firmen erhalten zu haben: Actelion, Astra Zeneca, Bayer Healthcare, BMS, Boehringer Ingelheim, Berlin Chemie, Daichii Sanyo, MSD, OMT, Pfizer und Forschungsbeihilfen von der Firma Actelion.R. Koczulla hat Honorare von Grifols, CSL, Novartis, Roche, GSK, Boehringer, Astra Zeneca, Berlin Chemie, TEVA, Sanofi, Mundipharma, GSK, Chiesi erhalten.W. Randerath erhielt Vortragshonorare und Reisekosten der Firmen Boehringer Ingelheim, Berlin Chemie, Bayer, Novartis und Roche.H. Watz erhielt Honorare für Vortrags- und Beratungstätigkeit sowie die Durchführung von klinischen Studien und Reisekostenerstattungen von den Firmen AstraZeneca, Bayer, Boehringer Ingelheim, BerlinChemie, Chiesi, Novartis, GlaxoSmithKline, Takeda und SanofiAventis.H. Wilkens erhielt Honorare für Vorträge und/oder Beratertätigkeiten von Actelion, Bayer Health Care, Boehringer Ingelheim, MSD, Pfizer und Roche.G. Steinkamp erhielt Honorare für Medizinisch-wissenschaftliches Publizieren von Boehringer Ingelheim, Gilead Sciences, InfectoPharm, Novartis, Pari GmbH und Alpha1 Deutschland e. V. L. Jany und M. Pfeifer geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

Patients with intermediate-risk pulmonary embolism (PE) may, depending on the method and cut-off values used for definition, account for up to 60% of all patients with PE and have an 8% or higher risk of short-term adverse outcome. Although four non-vitamin K-dependent direct oral anticoagulants (NOACs) have been approved for the treatment of venous thromboembolism, their safety and efficacy as well as the optimal anticoagulation regimen using these drugs have not been systematically investigated in intermediate-risk PE. Moreover, it remains unknown how many patients with intermediate-high-risk and intermediate-low-risk PE were included in most of the phase III NOAC trials. The ongoing Pulmonary Embolism International Thrombolysis 2 (PEITHO-2) study is a prospective, multicentre, multinational, single-arm trial investigating whether treatment of acute intermediate-risk PE with parenteral heparin anticoagulation over the first 72 hours, followed by the direct oral thrombin inhibitor dabigatran over 6 months, is effective and safe. The primary efficacy outcome is recurrent symptomatic venous thromboembolism or death related to PE within the first 6 months. The primary safety outcome is major bleeding as defined by the International Society on Thrombosis and Haemostasis. Secondary outcomes include all-cause mortality, the overall duration of hospital stay (index event and repeated hospitalizations) and the temporal pattern of recovery of right ventricular function over the 6-month follow-up period. By applying and evaluating a contemporary risk-tailored treatment strategy for acute PE, PEITHO-2 will implement the recommendations of current guidelines and contribute to their further evolution., Competing Interests: Financial Support: PEITHO-2 is an independent, investigator-initiated trial. The study has an academic sponsor (University Medical Center Mainz, Germany) and is supported by public funding. In addition, the sponsor has obtained the study drug (dabigatran) and a grant from the market authorization holder of dabigatran, Boehringer Ingelheim. The authors are solely responsible for the design and conduct of the PEITHO-2 trial study, for all study analyses and for the drafting and editing of reports and publications and their final contents.

Pulmonary embolism (PE) is a potentially life-threatening acute cardiovascular syndrome. However, more than 95 % of patients are haemodynamically stable at presentation, and among them are patients at truly low risk who may qualify for immediate or early discharge. The Home Treatment of Pulmonary Embolism (HoT-PE) study is a prospective international multicentre single-arm phase 4 management (cohort) trial aiming to determine whether home treatment of acute low-risk PE with the oral factor Xa inhibitor rivaroxaban is feasible, effective, and safe. Patients with confirmed PE, who have no right ventricular dysfunction or free floating thrombi in the right atrium or ventricle, are eligible if they meet none of the exclusion criteria indicating haemodynamic instability, serious comorbidity or any condition mandating hospitalisation, or a familial/social environment unable to support home treatment. The first dose of rivaroxaban is given in hospital, and patients are discharged within 48 hours of presentation. Rivaroxaban is taken for at least three months. The primary outcome is symptomatic recurrent venous thromboembolism or PE-related death within three months of enrolment. Secondary outcomes include quality of life and patient satisfaction, and health care resource utilisation compared to existing data on standard-duration hospital treatment. HoT-PE is planned to analyse 1,050 enrolled patients, providing 80 % power to reject the null hypothesis that the recurrence rate of venous thromboembolism is >3 % with α≤0.05. If the hypothesis of HoT-PE is confirmed, early discharge and out-of-hospital treatment may become an attractive, potentially cost-saving option for a significant proportion of patients with acute PE.

Dyspnoea is the predominant symptom in patients with pulmonary hypertension (PH) at diagnosis. However, since dyspnoea is nonspecific and often occurs in a number of common diseases, the presence of PH can easily be underdiagnosed.In addition, this symptom underlies a high variability in the subjective perception, therefore further diagnostic procedures are often delayed by the patients.A survey of the incidence and severity of dyspnoea in 372 patients with PAH was conducted by questionnaire in German centres. Age, sex distribution and the range of comorbidities corresponded to the findings of national and international registries.Approximately 99 % of patients reported the presence of dyspnoea on exertion, even at low loads.Remarkably, in 13 % of patients dyspnoea occurs as a paroxysmal symptom, which may lead to the differential diagnosis of bronchial asthma. In addition, the patients who were being followed in specialized PH centres reported an increase in dyspnoea during the last year.The results of the survey on the incidence of dyspnoea in patients with PAH are consistent with the findings of international studies., (© 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.

The benefits of surgical therapy of locally advanced non-small cell lung cancer (NSCLC) with infiltration of the superior vena cava (SVC) remains controversial. Here we describe our therapeutic approach and results of our intervention.A retrospective analysis of 22 patients with NSCLC who underwent SVC replacement (n = 17) or reconstruction (n = 5) between 1998 and 2013 was performed. Pneumonectomy was necessary in 16 patients, lobectomy in 8. Preoperative chemotherapy was administered to 3 patients, 16 received postoperative radiation treatment. The clinical course and survival were analyzed.Major postoperative morbidities were found in 13 patients. Graft thrombosis did not occur. Thirty-day mortality was 7 % in pneumonectomy patients and 0 % following lobectomy. Local recurrence was found in 4.5 %, distant metastases developed in 54.5 % of the patients (p = 0.0008). One- and five-year survival probabilities for all patients were 63.6 and 27.9 %. Five-year survival probability was 33 % for patients with SVC reconstruction and 25 % for patients with SVC replacement (p = 0.22). Five-year survival rates after pneumonectomy and lobectomy were 21.4 % and 37.5 %, respectively (p = 0.18).Radical resection involving the SVC in carefully selected patients with NSCLC results in excellent local tumor control. Due to the high rate of distant metastases, application of induction and adjuvant chemotherapy should be carefully assessed., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: The reported prevalence of pulmonary hypertension (PH) in sarcoidosis is variable and depends on the ethnic background, the underlying pathophysiology and the diagnostic methods used. We aimed to analyse the prevalence and the severity of PH and its relationship to ventilation and gas exchange in sarcoidosis., Methods: We performed a retrospective analysis of echocardiography, pulmonary function tests, blood gases and right heart catheterisation of 123 patients with sarcoidosis., Results: 5.6 % of all patients showed pulmonary hypertension. Parenchymal lung abnormalities (seen in 59.4 % of all patients) had no influence on PH. In patients with PH VC (p < 0.001), FEV1 (p < 0.001), FEV1/VC (p = 0.01), TLCO/VA (p < 0.001), pO2 (p = 0.04) and SO2 (p < 0.01), but not ITGV, TLC and RV were significantly lower. We found significant negative correlations for right ventricular systolic pressure (RVSP) and VC (R = -0.52, p < 0.01), FEV1 (R = -0.53, p < 0.01), DLCO-VA (R = -0.55, p = 0.02), pO2 (R = -0.69, p = 0.04) and SO2 (R = -0.91, p < 0.001)., Conclusion: Prevalence and severity of PH were similar to published Japanese data, but were less than reported in African-Americans. PH was associated with decreased vital capacity, forced exspiratory volume at one second and oxygenation; however, it was independent from parenchymal abnormalities. The influence of power of breathing on VC and FEV1 and its correlation with RVSP should be evaluated in a prospective trial., (© Georg Thieme Verlag KG Stuttgart · New York.)

The 2009 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) in chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH. This recommendation, however, is not always in agreement with medical ethics as physicians feel sometimes inclined to treat other form of pulmonary hypertension which may affect quality of life and survival of these patients in a similar manner. In June 2010, a group of German experts met in Cologne, Germany, to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. The conference was sponsored by the German Society of Cardiology, the German Society of Respiratory Medicine and the German Society of Pediatric Cardiology. One of the working groups was dedicated to the diagnosis and treatment of PH in patients with chronic lung disease. The recommendations of this working group are summarized in the present paper., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: A sudden unilateral hearing loss in childhood is commonly regarded as an unusual event., Case-Report: In this paper, we report of a 10 year old girl suffering from a unilateral right-sided sudden hearing loss following an acute infection with Mycoplasma pneumoniae, which is also rarely observed. Maximum hearing loss was estimated around 30 dB (0.25-6 kHz), which resolved after infusions using plasma expanders. Both transitory and distortion product otoacoustic emissions remained absent, while latencies of acoustically evoked potentials were normal., Discussion: This suggests a damage of outer hair cell function as an explanation for the observed hearing loss. The coincidence of two rare events for children such as a spontaneous sudden unilateral hearing loss combined with an infection with Mycoplasma pneumoniae gives rise to the hypothesis of a causal relationship that is discussed according to the literature.

Since 1987 233 HIV-infected patients have been treated at the Department of Otorhinolaryngology, Head and Neck Surgery of the Ludwig-Maximilians-University of Munich. 70% of these patients had advanced immunodeficiency disease (ARC and AIDS). 46 presented a Kaposi's sarcoma (KS) in the head and neck region. 91% were homosexual men. KS was most often located in the mouth (67%), oropharynx (65%) and skin (39.1%), while the larynx (10.9%), hypopharynx (8.7%), lymph nodes (6.5%) and nasopharynx (4.3%) were rarely involved. In 15 patients, a KS of the head and neck region was the initial symptom for the HIV-infection. Although the clinical features of this disease are typical, histological examination is required because differential diagnosis can show other rare diseases, such as bacillary angiomatosis, which are easily cured. The morphology of early plain or elevated KS exhibits more irregular vascular components while the nodular KS is dominated by sarcomatous cell lines. Immunohistochemical studies with antibodies to viral components revealed no reactivity to HIV-, HPV-, HSV-, EBV- and CMV-antigens. The best local treatment proved to be CO2- or ND:YAG-laser therapy. Cutaneous lesions were treated with camouflage or by fractionated radiotherapy. Advanced disease showed best response to systemic chemotherapy. Despite the advanced stage of immunodeficiency syndrome, an adequate local or systemic therapy can obviously improve the quality of life in HIV-infected patients.