Pulmonale Hypertonie bei Lungenkrankheiten: Empfehlungen der Kölner Konsensus-Konferenz 2016

Zusammenfassung

Die 2015 veröffentlichen Europäischen Leitlinien zur Diagnostik und Therapie der Pulmonalen Hypertonie (PH) befassen sich nicht nur mit der pulmonal arteriellen Hypertonie (PAH), sondern auch mit der PH bei chronischen Lungenerkrankungen. Unter anderem weisen diese Leitlinien darauf hin, dass Medikamente, die derzeit für die PAH eingesetzt werden (Prostanoide, Endothelin-Rezeptor-Antagonisten, Phosphodiesterase-5 Inhibitoren, sGC-Stimulatoren) bei anderen Formen der PH bislang unzureichend untersucht sind. Daher wird der Einsatz dieser Medikamente bei Patienten mit chronischen Lungenerkrankungen und PH nicht empfohlen. Diese Empfehlung steht allerdings in einigen Fällen dem ärztlichen und ethischen Anspruch entgegen, auch andere Formen des Lungenhochdrucks zu behandeln, die die Lebensqualität und die Lebenserwartung der Betroffenen in vergleichbarer Weise einschränken. Tatsächlich müssen hierbei die Schwere der PH und die Ausprägung der Lungenerkrankung berücksichtigt werden. Im Juni 2016 fand in Köln eine Konsensus-Konferenz statt, die von den Deutschen Gesellschaften für Kardiologie, Pneumologie und Pädiatrische Kardiologie (DGK, DGP und DGPK) organisiert wurde, und die sich mit strittigen Themen in der praktischen Umsetzung der Europäischen Leitlinien in Deutschland befasste. Dazu wurden verschiedene Arbeitsgruppen eingesetzt, von denen eine sich gezielt der Diagnostik und Therapie der PH bei chronischen Lungenerkrankungen widmete. Die Ergebnisse und Beschlüsse dieser Arbeitsgruppe werden in dem vorliegenden Manuskript detailliert beschrieben.

Abstract

The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) associated with chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, sGC stimulators) 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 PH which may affect quality of life and survival of these patients in a similar manner. To this end, it is crucial to consider the severity of both PH and the underlying lung disease. 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 PH in patients with chronic lung disease. The recommendations of this working group are summarized in the present paper.

Schlüsselwörter

Pulmonale Hypertonie bei Lungenkrankheit - Pulmonale Hypertonie bei interstitieller Lungenkrankheit - Pulmonale Hypertonie bei CPFE - Pulmonale Hypertonie bei COPD - Atypische IPAH

Keywords

PH-Lung - PH-ILD - PH-CPFE - PH-COPD - Atypical IPAH

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