Subscribe to RSS
DOI: 10.1055/s-0042-114524
Risikostratifizierung und Verlaufskontrollen bei PAH-Patienten: Empfehlungen der Kölner Konsensus-Konferenz 2016
Risk stratification and follow-up assessment of patients with pulmonary arterial hypertension: Recommendations of the Cologne Consensus Conference 2016Publication History
Publication Date:
19 October 2016 (online)
![](https://www.thieme-connect.de/media/dmw/2016S01/lookinside/thumbnails/s19_10-1055-s-0042-114524-1.jpg)
Zusammenfassung
Die 2015 veröffentlichen Europäischen Leitlinien zur Diagnostik und Therapie der pulmonalen Hypertonie (PH) sind nunmehr auch in Deutschland gültig. Die Leitlinien befassen sich eingehend mit der Diagnostik der pulmonalen Hypertonie, und bieten darüber hinaus neue Empfehlungen zur Risikostratifizierung und Verlaufskontrollen bei Patienten mit diagnostizierter PAH. Für die praktische Umsetzung der Europäischen Leitlinien in Deutschland sind jedoch zahlreiche für Deutschland spezifische Gesichtspunkte und bereits wieder neue Daten bedeutsam, die eine ausführliche Kommentierung der Leitlinien und in einigen Punkten eine Aktualisierung notwendig machen. Im Juni 2016 fand in Köln eine Konsensuskonferenz statt, die von den Arbeitsgruppen PH der Deutschen Gesellschaften für Kardiologie (DGK), Pneumologie (DGP) und Pädiatrische Kardiologie (DGPK) organisiert wurde. Die Konferenz befasste sich mit der Umsetzung der Europäischen Leitlinien im deutschsprachigen Raum. Dazu wurden verschiedene Arbeitsgruppen eingesetzt, von denen sich eine gezielt der Risikoeinschätzung und Verlaufskontrollen bei pulmonaler Hypertonie widmete. Die Ergebnisse und Beschlüsse dieser Arbeitsgruppe werden in dem vorliegenden Manuskript detailliert beschrieben.
Abstract
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed information about the diagnosis of pulmonary hypertension, and furthermore provide novel recommendations for risk stratification and follow-up assessments. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. 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. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to risk stratification and follow-up assessment of patients with PAH. This manuscript summarizes the results and recommendations of this working group.
-
Literatur
- 1 Galie N, Humbert M, Vachiery JL 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
- 2 Hoeper MM, Huscher D, Ghofrani HA et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 2013; 168: 871-880
- 3 Humbert M, Sitbon O, Chaouat A et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023-1030
- 4 Benza RL, Miller DP, Gomberg-Maitland M et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 2010; 122: 164-172
- 5 Sitbon O, Humbert M, Nunes H et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780-788
- 6 Taichman DB, McGoon MD, Harhay MO et al. Wide variation in clinicians‘ assessment of New York Heart Association/World Health Organization functional class in patients with pulmonary arterial hypertension. Mayo Clin Proc 2009; 84: 586-592
- 7 Nickel N, Golpon H, Greer M et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2012; 39: 589-596
- 8 Barst RJ, Chung L, Zamanian RT, Turner M, McGoon MD. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest 2013; 144: 160-168
- 9 Galie N, Rubin L, Hoeper M et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008; 371: 2093-2100
- 10 McLaughlin VV, Sitbon O, Badesch DB et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244-249
- 11 Bustamante-Labarta M, Perrone S, De La Fuente RL et al. Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension. J Am Soc Echocardiogr 2002; 15: 1160-1164
- 12 McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106: 1477-1482
- 13 Raymond RJ, Hinderliter AL, Willis PW et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002; 39: 1214-1219
- 14 Wensel R, Opitz CF, Anker SD et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 2002; 106 (3) 319-324
- 15 Forfia PR, Fisher MR, Mathai SC et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006; 174: 1034-1041
- 16 Thenappan T, Palevsky HI. Long-term outcomes in pulmonary arterial hypertension. Int J Clin Pract Suppl 2009; 162: 42-44
- 17 Chandra S, Shah SJ, Thenappan T et al. Carbon monoxide diffusing capacity and mortality in pulmonary arterial hypertension. J Heart Lung Transplant 2010; 29: 181-187
- 18 Sachdev A, Villarraga HR, Frantz RP et al. Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension. Chest 2011; 139: 1299-1309
- 19 Fine NM, Chen L, Bastiansen PM et al. Outcome prediction by quantitative right ventricular function assessment in 575 subjects evaluated for pulmonary hypertension. Circ Cardiovasc Imaging 2013; 6: 711-721
- 20 Shimada YJ, Shiota M, Siegel RJ et al. Accuracy of right ventricular volumes and function determined by three-dimensional echocardiography in comparison with magnetic resonance imaging: a meta-analysis study. J Am Soc Echocardiogr 2010; 23: 943-953
- 21 Smith BC, Dobson G, Dawson D et al. Three-dimensional speckle tracking of the right ventricle: toward optimal quantification of right ventricular dysfunction in pulmonary hypertension. J Am Coll Cardiol 2014; 64: 41-51
- 22 Grunig E, Tiede H, Enyimayew EO et al. Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension. Circulation 2013; 128: 2005-2015
- 23 van Wolferen SA, Marcus JT, Boonstra A et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 2007; 28: 1250-1257
- 24 van de Veerdonk MC, Kind T, Marcus JT et al. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol 2011; 58: 2511-2519
- 25 Swift AJ, Rajaram S, Marshall H et al. Black blood MRI has diagnostic and prognostic value in the assessment of patients with pulmonary hypertension. Eur Radiol 2012; 22: 695-702
- 26 Swift AJ, Rajaram S, Campbell MJ et al. Prognostic value of cardiovascular magnetic resonance imaging measurements corrected for age and sex in idiopathic pulmonary arterial hypertension. Circ Cardiovasc Imaging 2014; 7: 100-106
- 27 Sitbon O, McLaughlin VV, Badesch DB et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax 2005; 60: 1025-1030
- 28 Hoeper MM, Maier R, Tongers J et al. Determination of cardiac output by the Fick method, thermodilution, and acetylene rebreathing in pulmonary hypertension. Am J Respir Crit Care Med. 1999; 160: 535-541
- 29 Rich JD, Archer SL, Rich S. Noninvasive cardiac output measurements in patients with pulmonary hypertension. Eur Respir J 2013; 42: 125-133
- 30 Gabler NB, French B, Strom BL et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 2012; 126: 349-356
- 31 Savarese G, Paolillo S, Costanzo P et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol 2012; 60: 1192-1201
- 32 Fritz JS, Blair C, Oudiz RJ et al. Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest 2013; 143: 315-323
- 33 Paciocco G, Martinez FJ, Bossone E et al. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur Respir J 2001; 17: 647-652
- 34 Provencher S, Chemla D, Herve P et al. Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension. Eur Respir J 2006; 27: 114-120
- 35 Sun XG, Hansen JE, Oudiz RJ et al. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation 2001; 104: 429-435
- 36 Diller GP, Dimopoulos K, Okonko D et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005; 112: 828-835
- 37 Arena R, Lavie CJ, Milani RV et al. Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: an evidence-based review. J Heart Lung Transplant 2010; 29: 159-173
- 38 Blumberg FC, Arzt M, Lange T et al. Impact of right ventricular reserve on exercise capacity and survival in patients with pulmonary hypertension. Eur J Heart Fail 2013; 15: 771-775
- 39 Wensel R, Francis DP, Meyer FJ et al. Incremental prognostic value of cardiopulmonary exercise testing and resting haemodynamics in pulmonary arterial hypertension. Int J Cardiol 2013; 167: 1193-1198
- 40 Scheidl SJ, Englisch C, Kovacs G et al. Diagnosis of CTEPH versus IPAH using capillary to end-tidal carbon dioxide gradients. Eur Respir J 2012; 39: 119-124
- 41 Shi X, Guo J, Gong S et al. Oxygen uptake is more efficient in idiopathic pulmonary arterial hypertension than in chronic thromboembolic pulmonary hypertension. Respirology 2016; 21: 149-156
- 42 Humbert M, Monti G, Brenot F et al. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am J Respir Crit Care Med 1995; 151: 1628-1631
- 43 Balabanian K, Foussat A, Dorfmuller P et al. CX(3)C chemokine fractalkine in pulmonary arterial hypertension. Am J Respir Crit Care Med 2002; 165: 1419-1425
- 44 Dorfmuller P, Zarka V, Durand-Gasselin I et al. Chemokine RANTES in severe pulmonary arterial hypertension. Am J Respir Crit Care Med 2002; 165: 534-539
- 45 Quarck R, Nawrot T, Meyns B et al. C-reactive protein: a new predictor of adverse outcome in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 53: 1211-1218
- 46 Nagaya N, Nishikimi T, Uematsu M et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000; 102: 865-870
- 47 Torbicki A, Kurzyna M, Kuca P et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation 2003; 108: 844-848
- 48 Fijalkowska A, Kurzyna M, Torbicki A et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 2006; 129: 1313-1321
- 49 Leuchte HH, El Nounou M, Tuerpe JC et al. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest 2007; 131: 402-409
- 50 Nagaya N, Uematsu M, Satoh T et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160: 487-492
- 51 Hoeper MM, Pletz MW, Golpon H et al. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2007; 29: 944-950
- 52 Nickel N, Kempf T, Tapken H et al. Growth differentiation factor-15 in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med 2008; 178: 534-541
- 53 Lorenzen JM, Nickel N, Kramer R et al. Osteopontin in patients with idiopathic pulmonary hypertension. Chest 2011; 139: 1010-1017
- 54 Warwick G, Thomas PS, Yates DH. Biomarkers in pulmonary hypertension. Eur Respir J 2008; 32: 503-512
- 55 Leuchte HH, Baumgartner RA, Nounou ME et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 2006; 173: 744-750
- 56 Mathai SC, Bueso M, Hummers LK et al. Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension. Eur Respir J 2010; 35: 95-104
- 57 Olsson KM, Nickel NP, Tongers J, Hoeper MM. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int J Cardiol 2013; 167: 2300-2305
- 58 Fox BD, Shimony A, Langleben D et al. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur Respir J 2013; 42: 1083-1091
- 59 Rosenkranz S, Gibbs JS, Wachter R et al. Left ventricular heart failure and pulmonary hypertensiondagger. Eur Heart J 2016; 37: 942-954
- 60 D‘Alonzo GE, Barst RJ, Ayres SM et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343-349
- 61 Humbert M, Sitbon O, Yaici A et al. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010; 36: 549-555
- 62 Leuchte HH, Baezner C, Baumgartner RA et al. Residual pulmonary vasodilative reserve predicts outcome in idiopathic pulmonary hypertension. Heart 2015; 101: 972-976
- 63 Hasler ED, Muller-Mottet S, Furian M et al. Pressure-Flow During Exercise Catheterization Predicts Survival in Pulmonary Hypertension. Chest 2016; 150: 57-67
- 64 Benza RL, Gomberg-Maitland M et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 2012; 141: 354-362
- 65 Sitbon O, Benza RL, Badesch DB et al. Validation of two predictive models for survival in pulmonary arterial hypertension. Eur Respir J 2015; 46: 152-164
- 66 Hoeper MM, Markevych I, Spiekerkoetter E et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26: 858-863
- 67 McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013; 62: D73-81
- 68 Farber HW, Miller DP, McGoon MD et al. Predicting outcomes in pulmonary arterial hypertension based on the 6-minute walk distance. J Heart Lung Transplant 2015; 34: 362-368
- 69 Pulido T, Adzerikho I, Channick RN et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809-818
- 70 Sitbon O, Channick R, Chin KM et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med 2015; 373: 2522-2533
- 71 Rival G, Lacasse Y, Martin S. Effect of Pulmonary Arterial Hypertension Specific Therapies on Health-Related Quality of Life: A Systematic Review. Chest 2014; 146: 686-708
- 72 Somaini G, Hasler ED, Saxer S et al. Prevalence of Anxiety and Depression in Pulmonary Hypertension and Changes during Therapy. Respiration 2016; 91: 359-366
- 73 Swetz KM, Shanafelt TD, Drozdowicz LB et al. Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: results from a cross-sectional patient survey. J Heart Lung Transplant 2012; 31: 1102-1108
- 74 Cenedese E, Speich R, Dorschner L et al. Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J 2006; 28: 808-815
- 75 Mathai SC, Suber T, Khair RM, Kolb TM, Damico RL, Hassoun PM. Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension. Annals of the American Thoracic Society 2016; 13: 31-39
- 76 Blok IM, van Riel AC, Schuuring MJ et al. Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease. Neth Heart J 2015; 23: 278-284
- 77 Halank M, Speich R, Petkova D et al. [Quality of life in pulmonal arterial hypertension and in chronic thromboembolic pulmonary hypertension]. Dtsch Med Wochenschr 2014; 139: S126-135
- 78 Cima K, Twiss J, Speich R et al. The German adaptation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). Health Qual Life Outcomes 2012; 10: 110