Pulmonale Hypertonie bei chronischen Lungenerkrankungen[*]

Zusammenfassung

Die 2009 veröffentlichten Europäischen Leitlinien zur Diagnostik und Therapie der pulmonalen Hypertonie befassen sich nicht nur mit der pulmonal arteriellen Hypertonie (PAH), sondern auch mit der pulmonalen Hypertonie (PH) bei chronischen Lungenerkrankungen. Unter anderem weisen diese Leitlinien darauf hin, dass Medikamente, die derzeit für die PAH eingesetzt werden (Prostanoide, Endothelin-Rezeptor-Antagonisten und Phosphodiesterase-5-Inhibitoren) 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.
Im Juni 2010 fand in Köln eine Konsensuskonferenz 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 2009 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) in chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors) 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 pulmonary hypertension which may affect quality of life and survival of these patients in a similar manner.
In June 2010, a group of German experts met in Cologne, Germany, to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. The conference was sponsored by the German Society of Cardiology, the German Society of Respiratory Medicine and the German Society of Pediatric Cardiology. One of the working groups 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.

1 Diese Arbeit ist zuerst erschienen Dtsch Med Wochenschrift 2010; 135: S115 – S124.

Literatur

• 1 Galie N, Hoeper M M, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. The 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 the International Society of Heart and Lung Transplantation (ISHLT).  Eur Respir J. 2009;  34 1219-1263
• 2 Galie N, Hoeper M M, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The 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 the International Society of Heart and Lung Transplantation (ISHLT).  Eur Heart J. 2009;  30 2493-2537
• 3 Hoeper M M, Ghofrani H A, Gorenflo M et al. Diagnostik und Therapie der pulmonalen Hypertonie: Europäische Leitlinie 2009.  Pneumologie. 2010;  64 401-414
• 4 Hoeper M M, Barbera J A, Channick R N et al. Diagnosis, assessment, and treatment of non-pulmonary arterial hypertension pulmonary hypertension.  J Am Coll Cardiol. 2009;  54 S85-96
• 5 Kovacs G, Berghold A, Scheidl S et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review.  Eur Respir J. 2009;  34 888-894
• 6 Hoeper M M. The new definition of pulmonary hypertension.  Eur Respir J. 2009;  34 790-791
• 7 Chaouat A, Naeije R, Weitzenblum E. Pulmonary hypertension in COPD.  Eur Respir J. 2008;  32 1371-1385
• 8 Behr J, Ryu J H. Pulmonary hypertension in interstitial lung disease.  Eur Respir J. 2008;  31 1357-1367
• 9 Scharf S M, Iqbal M, Keller C et al. Hemodynamic characterization of patients with severe emphysema.  Am J Respir Crit Care Med. 2002;  166 314-322
• 10 Thabut G, Dauriat G, Stern J B et al. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation.  Chest. 2005;  127 1531-1536
• 11 Lettieri C J, Nathan S D, Barnett S D et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis.  Chest. 2006;  129 746-752
• 12 Shorr A F, Wainright J L, Cors C S et al. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant.  Eur Respir J. 2007;  30 715-721
• 13 Shorr A F, Helman D L, Davies D B et al. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics.  Eur Respir J. 2005;  25 783-788
• 14 Weitzenblum E, Hirth C, Ducolone A et al. Prognostic value of pulmonary artery pressure in chronic obstructive pulmonary disease.  Thorax. 1981;  36 752-758
• 15 Hamada K, Nagai S, Tanaka S et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis.  Chest. 2007;  131 650-656
• 16 Cottin V, Nunes H, Brillet P Y et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity.  Eur Respir J. 2005;  26 586-593
• 17 Cottin V, Le Pavec J, Prevot G et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome.  Eur Respir J. 2010;  35 105-111
• 18 Chaouat A, Bugnet A S, Kadaoui N et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease.  Am J Respir Crit Care Med. 2005;  172 189-194
• 19 Corte T J, Wort S J, Gatzoulis M A et al. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension.  Thorax. 2009;  64 883-888
• 20 Incalzi R A, Fuso L, De Rosa M et al. Electrocardiographic signs of chronic cor pulmonale: A negative prognostic finding in chronic obstructive pulmonary disease.  Circulation. 1999;  99 1600-1605
• 21 Tramarin R, Torbicki A, Marchandise B et al. Doppler echocardiographic evaluation of pulmonary artery pressure in chronic obstructive pulmonary disease. A European multicentre study. Working Group on Noninvasive Evaluation of Pulmonary Artery Pressure. European Office of the World Health Organization, Copenhagen.  Eur Heart J. 1991;  12 103-111
• 22 Torbicki A, Skwarski K, Hawrylkiewicz I et al. Attempts at measuring pulmonary arterial pressure by means of Doppler echocardiography in patients with chronic lung disease.  Eur Respir J. 1989;  2 856-860
• 23 Laaban J P, Diebold B, Zelinski R et al. Noninvasive estimation of systolic pulmonary artery pressure using Doppler echocardiography in patients with chronic obstructive pulmonary disease.  Chest. 1989;  96 1258-1262
• 24 Fisher M R, Criner G J, Fishman A P et al. Estimating pulmonary artery pressures by echocardiography in patients with emphysema.  Eur Respir J. 2007;  30 914-921
• 25 Fisher M R, Forfia P R, Chamera E et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension.  Am J Respir Crit Care Med. 2009;  179 615-621
• 26 Arcasoy S M, Christie J D, Ferrari V A et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease.  Am J Respir Crit Care Med. 2003;  167 735-740
• 27 Leuchte H H, Baumgartner R A, Nounou M E et al. Brain natriuretic Peptide is a prognostic parameter in chronic lung disease.  Am J Respir Crit Care Med. 2006;  173 744-750
• 28 Hoeper M M, Lee S H, Voswinckel R et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers.  J Am Coll Cardiol. 2006;  48 2546-2552
• 29 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
• 30 Agusti A G, Barbera J A, Roca J et al. Hypoxic pulmonary vasoconstriction and gas exchange during exercise in chronic obstructive pulmonary disease.  Chest. 1990;  97 268-275
• 31 Barbera J A, Roger N, Roca J et al. Worsening of pulmonary gas exchange with nitric oxide inhalation in chronic obstructive pulmonary disease.  Lancet. 1996;  347 4 36-440
• 32 Simonneau G, Escourrou P, Duroux P et al. Inhibition of hypoxic pulmonary vasoconstriction by nifedipine.  N Engl J Med. 1981;  304 1582-1585
• 33 Domenighetti G M, Saglini V G. Short- and long-term hemodynamic effects of oral nifedipine in patients with pulmonary hypertension secondary to COPD and lung fibrosis. Deleterious effects in patients with restrictive disease.  Chest. 1992;  102 708-714
• 34 Agostoni P, Doria E, Galli C et al. Nifedipine reduces pulmonary pressure and vascular tone during short- but not long-term treatment of pulmonary hypertension in patients with chronic obstructive pulmonary disease.  Am Rev Respir Dis. 1989;  139 120-125
• 35 Dupuis J, Hoeper M M. Endothelin receptor antagonists in pulmonary arterial hypertension.  Eur Respir J. 2008;  31 407-414
• 36 Wilkins M R, Wharton J, Grimminger F et al. Phosphodiesterase inhibitors for the treatment of pulmonary hypertension.  Eur Respir J. 2008;  32 198-209
• 37 Olschewski H, Gomberg-Maitland M. Prostacyclin therapies for the treatment of pulmonary arterial hypertension.  Eur Respir J. 2008;  31 801-901
• 38 Schulz R, Baseler G, Ghofrani H A et al. Nocturnal periodic breathing in primary pulmonary hypertension.  Eur Respir J. 2002;  19 658-663
• 39 Stolz D, Rasch H, Linka A et al. A randomised, controlled trial of bosentan in severe COPD.  Eur Respir J. 2008;  32 619-628
• 40 Blanco I, Gimeno E, Munoz P A et al. Hemodynamic and Gas Exchange Effects of Sildenafil in Patients with COPD and Pulmonary Hypertension.  Am J Respir Crit Care Med. 2009;  181 202-203
• 41 The Idiopathic Pulmonary Fibrosis Clinical Research Network . A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis.  N Engl J Med. 2010;  DOI: DOI: 10.1056/NEJMoa1002110

1 Diese Arbeit ist zuerst erschienen Dtsch Med Wochenschrift 2010; 135: S115 – S124.