Allgemeine Therapie der pulmonalarteriellen Hypertonie nach den neuen Leitlinien

Zusammenfassung

In den neuen Leitlinien (LL) für pulmonalarterielle Hypertonie (PAH) sind die allgemeinen Maßnahmen ein integraler Bestandteil der Behandlung der Patienten. Auch die systemischen Auswirkungen der pulmonalen Hypertonie und Rechtsherzinsuffizienz sollten angemessen berücksichtigt und behandelt werden. Im folgenden Artikel werden die in den LL genannten Maßnahmen unter Berücksichtigung des bestehenden Empfehlungsgrads und der Evidenzen beschrieben. Leider sind die meisten Allgemeinmaßnahmen, wie die Gabe von Diuretika, Sauerstoff, psychosozialer Support und Impfungen, nicht oder unzureichend in randomisierten, kontrollierten Studien untersucht worden. So haben sie zwar einen hohen I-Empfehlungsgrad, aber einen niedrigen Evidenzgrad C. Nur bei dem spezialisierten körperlichen Training liegen bislang insgesamt 7 randomisierte, kontrollierte Studien und 5 Metaanalysen vor, die eine Verbesserung der Sauerstoffaufnahme, körperlichen Belastbarkeit, der Beschwerden (WHO-Funktionsklasse), Lebensqualität und Hämodynamik nachgewiesen haben (daher neu IA-Empfehlung). Auch weitere Maßnahmen wie die Antikoagulation, Eisensubstitution und andere werden im Folgenden besprochen.

Abstract

Treatment of PAH involves a multidisciplinary approach that includes general measures. Supervised exercise and rehabilitation, diuretics, vaccination against influenza, pneumococcus and SARS-CoV-2, iron administration for iron deficiency anaemia, and in-flight O₂ administration for low oxygen saturation are now clearly recommended. No general recommendation can be made for the use of anticoagulation, the choice of an anaesthetic procedure, the use of cardiovascular drugs or the administration of long-term oxygen administration. Treatment choices should be based on individual decisions involving a PH expert centre. The management of women of childbearing potential with PAH is complex and requires comprehensive counselling, psychological support and close medical care regarding risks of and alternatives to pregnancy, contraception, as well as multidisciplinary support for existing or desired pregnancy and abortion.

Schlüsselwörter

allgemeine Maßnahmen - supportive Therapie - Leitlinien - Empfehlungen - pulmonalarterielle Hypertonie

Key words

general measures - supportive treatment - guidelines - recommendations - pulmonary arterial hypertension

• Literatur

• 1 Humbert M, Kovacs G, Hoeper MM. et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2022;
• 2 Gonzalez-Saiz L, Fiuza-Luces C, Sanchis-Gomar F. et al. Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial. Int J Cardiol 2017; 231: 277-283
• 3 Grunig E, MacKenzie A, Peacock AJ. 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 Ehlken N, Lichtblau M, Klose H. et al. Exercise training improves peak oxygen consumption and haemodynamics in patients with severe pulmonary arterial hypertension and inoperable chronic thrombo-embolic pulmonary hypertension: a prospective, randomized, controlled trial. Eur Heart J 2016; 37: 35-44
• 5 Harzheim D, Klose H, Pinado FP. et al. Anxiety and depression disorders in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Respir Res 2013; 14: 104
• 6 Lowe B, Grafe K, Ufer C. et al. Anxiety and depression in patients with pulmonary hypertension. Psychosom Med 2004; 66: 831-836
• 7 Olsson KM, Meltendorf T, Fuge J. et al. Prevalence of Mental Disorders and Impact on Quality of Life in Patients With Pulmonary Arterial Hypertension. Front Psychiatry 2021; 12: 667602
• 8 Wieteska-Milek M, Szmit S, Florczyk M. et al. COVID-19 Vaccination in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: Safety Profile and Reasons for Opting against Vaccination. Vaccines (Basel) 2021; 9: 1395
• 9 Stickel S, Gin-Sing W, Wagenaar M. et al. The practical management of fluid retention in adults with right heart failure due to pulmonary arterial hypertension. Eur Heart J Suppl 2019; 21: K46-K53
• 10 Kjellstrom B, Sandqvist A, Hjalmarsson C. 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
• 11 Osterberg L, Blaschke T. Adherence to medication. N Engl J Med 2005; 353: 487-497
• 12 Ulrich S, Saxer S, Hasler ED. et al. Effect of domiciliary oxygen therapy on exercise capacity and quality of life in patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension: a randomised, placebo-controlled trial. Eur Respir J 2019; 54: 1900276
• 13 Weitzenblum E, Sautegeau A, Ehrhart M. et al. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1985; 131: 493-498
• 14 Ulrich S, Hasler ED, Saxer S. et al. Effect of breathing oxygen-enriched air on exercise performance in patients with precapillary pulmonary hypertension: randomized, sham-controlled cross-over trial. Eur Heart J 2017; 38: 1159-1168
• 15 Adir Y, Humbert M, Chaouat A. Sleep-related breathing disorders and pulmonary hypertension. Eur Respir J 2021; 57: 2002258
• 16 Sonnweber T, Nairz M, Theurl I. et al. The crucial impact of iron deficiency definition for the course of precapillary pulmonary hypertension. PLoS One 2018; 13: e0203396
• 17 Ruiter G, Manders E, Happe CM. et al. Intravenous iron therapy in patients with idiopathic pulmonary arterial hypertension and iron deficiency. Pulm Circ 2015; 5: 466-472
• 18 McDonagh T, Damy T, Doehner W. 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
• 19 Rhodes CJ, Howard LS, Busbridge M. et al. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011; 58: 300-309
• 20 Ruiter G, Lankhorst S, Boonstra A. et al. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 2011; 37: 1386-1391
• 21 Olsson KM, Fuge J, Brod T. et al. Oral iron supplementation with ferric maltol in patients with pulmonary hypertension. Eur Respir J 2020; 56: 2000616
• 22 Howard L, He J, Watson GMJ. et al. Supplementation with Iron in Pulmonary Arterial Hypertension. Two Randomized Crossover Trials. Ann Am Thorac Soc 2021; 18: 981-988
• 23 Johnson SR, Granton JT, Mehta S. Thrombotic arteriopathy and anticoagulation in pulmonary hypertension. Chest 2006; 130: 545-552
• 24 Preston IR, Roberts KE, Miller DP. 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
• 25 Olsson KM, Meyer B, Hinrichs J. et al. Chronic thromboembolic pulmonary hypertension. Dtsch Arztebl Int 2014; 111: 856-862
• 26 Wang P, Hu L, Yin Y. 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
• 27 Burns RM, Peacock AJ, Johnson MK. et al. Hypoxaemia in patients with pulmonary arterial hypertension during simulated air travel. Respir Med 2013; 107: 298-304
• 28 Kylhammar D, Radegran G. The principal pathways involved in the in vivo modulation of hypoxic pulmonary vasoconstriction, pulmonary arterial remodelling and pulmonary hypertension. Acta Physiol (Oxf) 2017; 219: 728-756
• 29 Schneider SR, Mayer LC, Lichtblau M. et al. Effect of Normobaric Hypoxia on Exercise Performance in Pulmonary Hypertension: Randomized Trial. Chest 2021; 159: 757-771
• 30 Roubinian N, Elliott CG, Barnett CF. et al. Effects of commercial air travel on patients with pulmonary hypertension air travel and pulmonary hypertension. Chest 2012; 142: 885-892
• 31 Kamp JC, von Kaisenberg C, Greve S. et al. Pregnancy in pulmonary arterial hypertension: Midterm outcomes of mothers and offspring. J Heart Lung Transplant 2021; 40: 229-233
• 32 Corbach N, Berlier C, Lichtblau M. et al. Favorable Pregnancy Outcomes in Women With Well-Controlled Pulmonary Arterial Hypertension. Front Med (Lausanne) 2021; 8: 689764