Pneumologie 2023; 77(11): 907-915
DOI: 10.1055/a-2145-4741
Übersicht

Allgemeine Maßnahmen und Management der pulmonalarteriellen Hypertonie

General measures and management of pulmonary arterial hypertension
Mona Lichtblau
1   Klinik für Pneumologie, Universitätsspital Zürich, Zürich, Schweiz
,
Satenik Harutyunova
2   Zentrum für pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
,
Robert Nechwatal
3   Rehaklinik Heidelberg Königstuhl, Heidelberg, Deutschland
,
Laura Mayer
1   Klinik für Pneumologie, Universitätsspital Zürich, Zürich, Schweiz
,
Hans-Joachim Kabitz
4   Klinik für Pneumologie und Schlafmedizin, Kantonsspital Aarau (KSA), Schweiz
,
Finn Moritz Wilkens
2   Zentrum für pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
,
Sören Uiker
3   Rehaklinik Heidelberg Königstuhl, Heidelberg, Deutschland
,
Ekkehard Grünig
2   Zentrum für pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
,
Daniel Ritter
5   Klinikum Würzburg Mitte, Standort Missionsärztliche Klinik, Würzburg, Deutschland
,
Andreea Florea
2   Zentrum für pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
,
Nicola Benjamin
2   Zentrum für pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
› Author Affiliations

Zusammenfassung

Die Betreuung von Patienten mit pulmonalarterieller Hypertonie (PAH) erfordert ein multimodales Konzept und Management vielschichtiger Maßnahmen, die die medikamentöse Therapie für das Management von Nebenwirkungen und der Rechtsherzinsuffizienz umfassen sowie die Aufklärung u. a. über Schwangerschaft und Flugreisen, psychosoziale Unterstützung, körperliches Training und Prophylaxe durch Impfungen.

Positive Studienergebnisse haben zu einer gesteigerten Empfehlung eines spezifischen Trainings bei pulmonaler Hypertonie geführt. Auch wurde die Empfehlung zur Eisengabe auf die aktuelle Evidenzlage angepasst.

Besonderen Fokus hat in den aktuellen Leitlinien die Ausarbeitung der Empfehlungen zu Schwangerschaftsaufklärung und Verhütung sowie dem Management einer Schwangerschaft erhalten.

In diesem Artikel soll ein Überblick über die Empfehlungen zu allgemeinen Maßnahmen und zum Management der PAH gemäß ESC/ERS-Leitlinien gegeben werden. Ergänzungen der Leitlinienempfehlungen wurden durch die Autoren als Kommentare gekennzeichnet.

Abstract

Care of patients with pulmonary arterial hypertension (PAH) needs a multi-facetet concept and measures, including management of adverse reactions, right heart insufficiency as well as information on pregnancy, travels by air, psychosocial support, physical exercise training and prophylaxis by vaccination.

Positive study results led to an higher recommendation of specialized exercise training in pulmonary hypertension. Also, the recommendation on iron substitution was amended according to the current evidence.

In the current guidelines, special focus was given to the elaboration of recommendations regarding pregnancy, including patient information, contraception and patient management in case of pregnancy.

This article aims to provide an overview on the recommendations of general measuremes, special circumstances and patient management according to the ESC/ERS guidelines. Amendments to the guideline recommendations are given as comments from the authors of this article.



Publication History

Article published online:
14 November 2023

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  • Literatur

  • 1 Humber M. et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43: 3618-3731
  • 2 Galie N. et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46: 903-975
  • 3 Grunig E. et al. Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial. Eur Heart J 2021; 42: 2284-2295
  • 4 Grunig E. et al. ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension. Eur Respir J 2019; 53: 1800332
  • 5 Kingman M. et al. Living with pulmonary hypertension: unique insights from an international ethnographic study. BMJ Open 2014; 4: e004735
  • 6 Olsson KM. et al. Prevalence of Mental Disorders and Impact on Quality of Life in Patients With Pulmonary Arterial Hypertension. Front Psychiatry 2021; 12: 667602
  • 7 Kjellstrom B. et al. Adherence to disease-specific drug treatment among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. ERJ Open Res 2020; 6: 00299-2020
  • 8 Hoeper MM. et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J 2017; 50: 1700740
  • 9 Olsson KM. et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014; 129: 57-65
  • 10 Preston IR. et al. Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL). Circulation 2015; 132: 2403-2411
  • 11 Khan MS. et al. Is Anticoagulation Beneficial in Pulmonary Arterial Hypertension?. Circ Cardiovasc Qual Outcomes 2018; 11: e004757
  • 12 Wang P. et al. Can anticoagulants improve the survival rate for patients with idiopathic pulmonary arterial hypertension? A systematic review and meta-analysis. Thromb Res 2020; 196: 251-256
  • 13 Garry JD. et al. Anticoagulation in pulmonary arterial hypertension – association with mortality, healthcare utilization, and quality of life: The Pulmonary Hypertension Association Registry (PHAR). J Heart Lung Transplant 2022; 41: 1808-1818
  • 14 Sandoval J. et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682-1687
  • 15 Adir Y, Humbert M, Chaouat A. Sleep-related breathing disorders and pulmonary hypertension. Eur Respir J 2021; 57: 2002258
  • 16 Sonnweber T. et al. The crucial impact of iron deficiency definition for the course of precapillary pulmonary hypertension. PLoS One 2018; 13: e0203396
  • 17 McDonagh T. et al. Screening, diagnosis and treatment of iron deficiency in chronic heart failure: putting the 2016 European Society of Cardiology heart failure guidelines into clinical practice. Eur J Heart Fail 2018; 20: 1664-1672
  • 18 Ruiter G. et al. Intravenous iron therapy in patients with idiopathic pulmonary arterial hypertension and iron deficiency. Pulm Circ 2015; 5: 466-472
  • 19 Kramer T. et al. Ferric carboxymaltose in patients with pulmonary arterial hypertension and iron deficiency: a long-term study. J Cachexia Sarcopenia Muscle 2021; 12: 1501-1512
  • 20 Olsson KM. et al. Oral iron supplementation with ferric maltol in patients with pulmonary hypertension. Eur Respir J 2020; 56: 2000616
  • 21 Howard L. et al. Supplementation with Iron in Pulmonary Arterial Hypertension. Two Randomized Crossover Trials. Ann Am Thorac Soc 2021; 18: 981-988
  • 22 Ruiter G. et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014; 53: 285-292
  • 23 Xanthouli P. et al. Hypochromic red cells as a prognostic indicator of survival among patients with systemic sclerosis screened for pulmonary hypertension. Arthritis Res Ther 2023; 25: 38
  • 24 Kiely DG. et al. Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach. BJOG 2010; 117: 565-574
  • 25 Corbach N. et al. Favorable Pregnancy Outcomes in Women With Well-Controlled Pulmonary Arterial Hypertension. Front Med (Lausanne) 2021; 8: 689764
  • 26 Meyer S. et al. Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey. Eur Respir J 2013; 41: 1302-1307
  • 27 Hassan HJ. et al. A novel approach to perioperative risk assessment for patients with pulmonary hypertension. ERJ Open Res 2021; 7: 00257-2021
  • 28 Halvorsen S. et al. 2022 ESC Guidelines on cardiovascular assessment and management of patients undergoing non-cardiac surgery. Eur Heart J 2022; 43: 3826-3924
  • 29 Coker RK. et al. BTS Clinical Statement on air travel for passengers with respiratory disease. Thorax 2022; 77: 329-350
  • 30 Schneider SR. et al. Effect of a day-trip to altitude (2500 m) on exercise performance in pulmonary hypertension: randomised crossover trial. ERJ Open Res 2021; 7: 00314-2021
  • 31 Schneider SR. et al. Cardiorespiratory Adaptation to Short-Term Exposure to Altitude vs. Normobaric Hypoxia in Patients with Pulmonary Hypertension. J Clin Med 2022; 11: 2769