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Targeted therapy of pulmonary arterial hypertension: Updated recommendations from the Cologne Consensus Conference 2018.

  • Hoeper MM Department of Pneumology, Hannover Medical School, Hanover, Germany; German Centre for Lung Research (DZL), Germany. Electronic address: hoeper.marius@mh-hannover.de.
  • Apitz C Department of Pediatric Cardiology, University Hospital for Pediatric and Adolescent Medicine, Ulm, Germany.
  • Grünig E German Centre for Lung Research (DZL), Germany; Centre for Pulmonary Hypertension, Chest Clinic at Heidelberg University Hospital, Germany.
  • Halank M Medical Department I, Carl Gustav Carus University Hospital at the TU Dresden, Dresden, Germany.
  • Ewert R University Medicine Greifswald, Department and Outpatient Department for Internal Medicine, Pneumology/Infectiology, Greifswald, Germany.
  • Kaemmerer H Department of Pediatric Cardiology and Congenital Heart Defects, German Heart Centre Munich, Hospital at the Technical University of Munich, Germany.
  • Kabitz HJ Medical Department II, Pneumology, Cardiology and Internal Intensive Medicine, Constance Hospital, Germany.
  • Kähler C Department of Pneumology, Critical Care and Allergology, Lung Centre South-West, Wangen im Allgäu, Germany.
  • Klose H Centre for Pulmonary Hypertension Hamburg, Pneumology Department, Hamburg-Eppendorf University Hospital, Germany.
  • Leuchte H Neuwittelsbach, Hospital of the Compassionate Sisters and Academic Teaching Hospital at the LMU, Munich, Germany.
  • Ulrich S Clinic for Pneumology, Zurich University Hospital, Switzerland.
  • Olsson KM Department of Pneumology, Hannover Medical School, Hanover, Germany; German Centre for Lung Research (DZL), Germany.
  • Distler O Clinic for Rheumatology, Zurich University Hospital, Switzerland.
  • Rosenkranz S Department of Internal Medicine III, Cardiovascular Research Centre (CCRC), Heart Centre of the University of Cologne, Germany.
  • Ghofrani HA German Centre for Lung Research (DZL), Germany; Universities of Giessen and Marburg Lung Center (UGMLC), Gießen, Germany; Department for Pneumology, Kerckhoff Hospital Bad Nauheim, Germany; Department of Medicine, Imperial College, London, UK.
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  • 2018-09-08
Published in:
  • International journal of cardiology. - 2018
Endothelin receptor antagonists
Phosphodiesterase-5 inhibitors
Prostacyclin analogues
Pulmonary arterial hypertension
Pulmonary hypertension
Therapy
sGC stimulators
Combined Modality Therapy
Consensus Development Conferences as Topic
Drug Delivery Systems
Endothelin Receptor Antagonists
Germany
Humans
Hypertension, Pulmonary
Phosphodiesterase 5 Inhibitors
Practice Guidelines as Topic
English In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines and included new evidence, where available. The treatment algorithm for PAH was modified based on the observation that there are now many patients diagnosed with IPAH who are at an advanced age and have significant cardiopulmonary comorbidities. For patients newly diagnosed with classic forms of PAH, i.e. younger patients without significant cardiopulmonary comorbidities, the consensus-based recommendation was to use initial combination therapy as the standard approach. The use of monotherapies was no longer considered appropriate in such patients. The choice of treatment strategies should be based on the risk assessment as proposed in the European guidelines. In patients presenting with a low or intermediate risk, oral combination therapy with endothelin receptor antagonists and phosphodiesterase-5 inhibitors or soluble guanylate cyclase stimulators, respectively, should be used. In high-risk patients, triple combination therapy including a subcutaneous or intravenous prostacyclin analogue should be considered. For patients who suffer from PAH and significant cardiopulmonary comorbidities, initial monotherapy is recommended and the use of combination therapies should be considered on an individual basis. The latter recommendations are based on the scarcity of evidence supporting the use of combination therapy and the higher risk of drug-related adverse events in such patients.
Language
  • English
Open access status
bronze
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https://sonar.ch/global/documents/260501
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