Subscribe to RSS
DOI: 10.1055/a-2012-0856
Pulmonale Hypertonie bei Lungenerkrankungen und/oder Hypoxie
Pathophysiologie, Diagnose, Prognose und BehandlungPathophysiology, diagnosis, prognosis and treatment of pulmonary hypertension associated with chronic lung diseaseBasierend auf den Vorschlägen des 6. PH-Weltsymposiums 2018 wurden 2022 die aktualisierten Leitlinien zur Diagnostik und Therapie der pulmonalen Hypertonie (PH) der Europäischen Fachgesellschaften für Kardiologie und Pneumologie veröffentlicht [1]. Dieser Beitrag gibt ein Überblick über die Pathophysiologie, Diagnose, Prognose und Therapie der PH bei Lungenerkrankungen und/oder Hypoxie (PH associated with chronic Lung Disease, PH-CLD).
Abstract
The pathophysiology of pulmonary hypertension associated with chronic lung disease (PH-CLD) is complex, multifactorial, and not consistent among pulmonary diseases. However, pulmonary vasculopathy triggered by various factors, such as chronic alveolar hypoxia or cigarette smoking, seems to play a central role in the pathogenesis of PH-CLD. While the initial workup of PH-CLD is usually complicated by an overlap of symptoms of PH and the underlying lung disease, PH-CLD should be considered when there is a discrepancy between symptoms (especially exertional dyspnea) and pulmonary function tests. Clinical suspicion of PH-CLD can be strengthened by noninvasive diagnostic tools such as transthoracic echocardiography (TTE) or N-terminal pro-B-type natriuretic peptide (NT-pro-BNP). However, a right heart catheterization should only be performed in specialized centers to establish the diagnosis if therapeutic consequences for the patient were expected.
The basic treatment of PH-CLD is optimal management of the underlying lung disease. Among the existing interventional and registry-based studies, only a small number of data suggests favorable outcomes when treating PH-CLD patients with PAH-specific medications. Some publications even suggest negative effects. Nevertheless, recent data on inhaled vasoactive therapy in PH-CLD showed positive results for inhaled Treprostinil, although long-term data for this therapeutic approach are still lacking. Treatment of PH-CLD patients with PAH-specific drugs should only be performed in specialized centers and preferably in the context of clinical trials.
-
Die pathophysiologischen Mechanismen der PH-CLD sind multifaktoriell und nicht einheitlich über die verschiedenen Lungenerkrankungen und Phänotypen hinweg.
-
Die pulmonale Vaskulopathie, die durch verschiedene Faktoren wie z.B. die chronische alveoläre Hypoxie oder das Zigarettenrauchen getriggert wird, scheint eine zentrale Rolle bei der Pathogenese der PH-CLD zu spielen.
-
Die PH-CLD geht mit einer zusätzlichen Beeinträchtigung der Belastbarkeit, Lebensqualität und Prognose im Vergleich zu CLD-Patienten ohne PH einher.
-
Während die Diagnose der PH-CLD in der Regel durch die Überschneidung der Symptome von PH und Lungenerkrankung erschwert wird, sollte die PH-CLD in Betracht gezogen werden, wenn eine Diskrepanz zwischen der Einschränkung der klinischen Belastbarkeit und der Lungenfunktion besteht.
-
Der klinische Verdacht auf PH-CLD kann durch nicht invasive Diagnostik wie TTE oder NT-pro-BNP erhärtet werden, allerdings sollte eine invasive RHK-Untersuchung nur dann in erfahrenen Zentren zur Diagnosestellung durchgeführt werden, wenn sich hieraus für den Patienten therapeutische Konsequenzen ergeben.
-
Die Behandlung von PH-CLD-Patienten mit PAH-spezifischen Medikamenten sollte nur in spezialisierten Zentren und möglichst im Rahmen klinischer Studien durchgeführt werden.
Schlüsselwörter
pulmonale Hypertonie - chronische Lungenerkrankung - pulmonale Vaskulopathie - COPD - interstitielle LungenerkrankungKeywords
pulmonary hypertension - chronic lung disease - pulmonary vasculopathy - chronic obstructive pulmonary disease - interstitial lung diseasePublication History
Article published online:
10 November 2023
© 2023. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
-
Literatur
- 1 Humbert M, Kovacs G, Hoeper MM. et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43: 3618-3731 DOI: 10.1093/eurheartj/ehac237. (PMID: 36017548)
- 2 Nathan SD, Barbera JA, Gaine SP. et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J 2019; 53: 1801914 DOI: 10.1183/13993003.01914-2018. (PMID: 30545980)
- 3 Hopkins N, McLoughlin P. The structural basis of pulmonary hypertension in chronic lung disease: remodelling, rarefaction or angiogenesis?. J Anat 2002; 201: 335-348 DOI: 10.1046/j.1469-7580.2002.00096.x. (PMID: 12430958)
- 4 Seimetz M, Parajuli N, Pichl A. et al. Inducible NOS inhibition reverses tobacco-smoke-induced emphysema and pulmonary hypertension in mice. Cell 2011; 147: 293-305 DOI: 10.1016/j.cell.2011.08.035. (PMID: 22000010)
- 5 Kovacs G, Agusti A, Barberà JA. et al. Pulmonary Vascular Involvement in Chronic Obstructive Pulmonary Disease. Is There a Pulmonary Vascular Phenotype?. Am J Respir Crit Care Med 2018; 198: 1000-1011 DOI: 10.1164/rccm.201801-0095PP.
- 6 Hoeper MM, Pausch C, Grünig E. et al. Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry. J Heart and Lung Transplant 2020; 39: 1435-1444 DOI: 10.1016/j.healun.2020.09.011.
- 7 Hilde JM, Skjørten I, Grøtta OJ. et al. Right ventricular dysfunction and remodeling in chronic obstructive pulmonary disease without pulmonary hypertension. J Am Coll Cardiol 2013; 62: 1103-1111 DOI: 10.1016/j.jacc.2013.04.091.
- 8 Chaouat A, Bugnet AS, Kadaoui N. et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2005; 172: 189-194 DOI: 10.1164/rccm.200401-006OC. (PMID: 15831842)
- 9 Raghu G, Nathan SD, Behr J. et al. Pulmonary hypertension in idiopathic pulmonary fibrosis with mild-to-moderate restriction. Eur Respir J 2015; 46: 1370-1377 DOI: 10.1183/13993003.01537-2014.
- 10 Shorr AF, Wainright JL, Cors CS. et al. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J 2007; 30: 715-721 DOI: 10.1183/09031936.00107206.
- 11 Cottin V, Le Pavec J, Prévot G. et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J 2010; 35: 105-111 DOI: 10.1183/09031936.00038709.
- 12 Kessler R, Chaouat A, Schinkewitch P. et al. The obesity-hypoventilation syndrome revisited: a prospective study of 34 consecutive cases. Chest 2001; 120: 369-376 DOI: 10.1378/chest.120.2.369. (PMID: 11502631)
- 13 Thurnheer R, Ulrich S, Bloch KE. Precapillary Pulmonary Hypertension and Sleep-Disordered Breathing: Is There a Link?. Respiration 2017; 93: 65-77 DOI: 10.1159/000452957.
- 14 Cuttica MJ, Kalhan R, Shlobin OA. et al. Categorization and impact of pulmonary hypertension in patients with advanced COPD. Respir Med 2010; 104: 1877-1882 DOI: 10.1016/j.rmed.2010.05.009.
- 15 Olsson KM, Hoeper MM, Pausch C. et al. Pulmonary vascular resistance predicts mortality in patients with pulmonary hypertension associated with interstitial lung disease: results from the COMPERA registry. Eur Respir J 2021; 58: 2101483 DOI: 10.1183/13993003.01483-2021.
- 16 Hoeper MM, Dwivedi K, Pausch C. et al. Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis. Lancet Respir Med 2022; 10: 937-948 DOI: 10.1016/s2213-2600(22)00097-2. (PMID: 35777416)
- 17 Nadrous HF, Pellikka PA, Krowka MJ. et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005; 128: 2393-2399 DOI: 10.1378/chest.128.4.2393.
- 18 Singh N, Dorfmüller P, Shlobin OA. et al. Group 3 Pulmonary Hypertension: From Bench to Bedside. Circ Res 2022; 130: 1404-1422 DOI: 10.1161/circresaha.121.319970.
- 19 Barnett CF, Marco TD. et al. Pulmonary hypertension due to lung disease. In: Broaddus VC, Ernst JD, King Jr TE. Murray & Nadel’s Textbook of Respiratory Medicine. 6th ed. Philadelphia: Elsevier Saunders; 2016
- 20 Blanco I, Gimeno E, Munoz PA. et al. Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease and pulmonary hypertension. Am J Respir Crit Care Med 2010; 181: 270-278 DOI: 10.1164/rccm.200907-0988OC.
- 21 Seeger W, Adir Y, Barberà JA. et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 2013; 62: D109-116 DOI: 10.1016/j.jacc.2013.10.036. (PMID: 24355635)
- 22 Waxman A, Restrepo-Jaramillo R, Thenappan T. et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med 2021; 384: 325-334 DOI: 10.1056/NEJMoa2008470. (PMID: 33440084)
- 23 Zisman DA, Schwarz M, Anstrom KJ. et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010; 363: 620-628 DOI: 10.1056/NEJMoa1002110. (PMID: 20484178)
- 24 Goudie AR, Lipworth BJ, Hopkinson PJ. et al. Tadalafil in patients with chronic obstructive pulmonary disease: a randomised, double-blind, parallel-group, placebo-controlled trial. Lancet Respir Med 2014; 2: 293-300 DOI: 10.1016/s2213-2600(14)70013-x.
- 25 Nathan SD, Behr J, Collard HR. et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med 2019; 7: 780-790 DOI: 10.1016/s2213-2600(19)30250-4. (PMID: 31416769)
- 26 Corte TJ, Keir GJ, Dimopoulos K. et al. Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2014; 190: 208-217 DOI: 10.1164/rccm.201403-0446OC.