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Dtsch Med Wochenschr 2016; 141(S 01): S26-S32
DOI: 10.1055/s-0042-114525
Pulmonale Hypertonie: Empfehlungen der Kölner Konsensus-Konferenz 2016
© Georg Thieme Verlag KG Stuttgart · New York

Allgemeine und supportive Therapie der pulmonal arteriellen Hypertonie: Empfehlungen der Kölner Konsensus Konferenz 2016

General and supportive therapy of pulmonary arterial hypertension
E. Grünig
1   Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg
,
N. Benjamin
1   Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg
,
U. Krüger
2   Herzzentrum Duisburg
,
H. Kaemmerer
3   Klinik für Kinderkardiologie und angeborene Herzfehler, Deutsches Herzzentrum München, Klinik an der Technischen Universität München
,
S. Harutyunova
1   Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg
,
K. M. Olsson
4   Klinik für Pneumologie, Medizinische Hochschule Hannover
5   Deutsches Zentrum für Lungenforschung (DZL), Deutschland
,
S. Ulrich
6   Klinik für Pneumologie, Universitätsspital Zürich
,
F. Gerhardt
7   Herzzentrum der Universitätsklinik Köln
,
C. Neurohr
8   Medizinische Klinik und Poliklinik, Ludwig-Maximilians-Universität München
,
A. Sablotzki
9   Klinik für Anästhesiologie, Intensiv- und Schmerztherapie am Klinikum St. Georg, Leipzig
,
M. Halank
10   Pneumologie, Universitätsklinik Carl Gustav Carus, Dresden
,
H.-J. Kabitz
11   II. Medizinische Klinik, Klinikum Konstanz
,
G. Thimm
12   Pulmonale Hypertonie eV, Rheinstetten
,
K.-G. Fliegel
12   Pulmonale Hypertonie eV, Rheinstetten
,
H. Klose
13   Zentrum für Pulmonal-Arterielle Hypertonie Hamburg, Sektion Pneumologie, Universitätsklinikum Hamburg-Eppendorf
› Author Affiliations
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Publication History

Publication Date:
19 October 2016 (online)

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Zusammenfassung

Die 2015 veröffentlichen Europäischen Leitlinien zur Diagnostik und Therapie der pulmonalen Hypertonie sind nunmehr auch in Deutschland gültig. Die Leitlinien befassen sich eingehend mit der Therapie der pulmonal arteriellen Hypertonie (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 in Deutschland. Dazu wurden verschiedene Arbeitsgruppen eingesetzt, von denen sich eine gezielt der allgemeinen und supportiven Therapie der PAH widmete. Die Ergebnisse und Empfehlungen 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 recommendations for the targeted and supportive treatment of pulmonary arterial hypertension (PAH). 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 general and supportive therapy of PAH. This article summarizes the results and recommendations of this working group.

Schlüsselwörter

allgemeine Maßnahmen - supportive Therapie - pulmonal arterielle Hypertonie

Keywords

general measures - supportive therapy - pulmonary arterial hypertension
 

  • Literatur

  • 1 Galie N, Humbert M, Vachiery JL et al. 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
    CrossrefPubMedGoogle Scholar
  • 2 Mereles D, Ehlken N, Kreuscher S et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006; 114: 1482-1489
    CrossrefPubMedGoogle Scholar
  • 3 de Man FS, Handoko ML, Groepenhoff H et al. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2009; 34: 669-675
    CrossrefPubMedGoogle Scholar
  • 4 Becker-Grunig T, Klose H, Ehlken N et al. Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. International journal of cardiology 2013; 168: 375-381
    CrossrefPubMedGoogle Scholar
  • 5 Grunig E, Maier F, Ehlken N et al. Exercise training in pulmonary arterial hypertension associated with connective tissue diseases. Arthritis research & therapy. 2012; 14: R148
    CrossrefPubMedGoogle Scholar
  • 6 Nagel C, Prange F, Guth S et al. Exercise training improves exercise capacity and quality of life in patients with inoperable or residual chronic thromboembolic pulmonary hypertension. PLoS One 2012; 7: e41603
    CrossrefPubMedGoogle Scholar
  • 7 Grunig E, Lichtblau M, Ehlken N et al. Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 2012; 40: 84-92
    CrossrefPubMedGoogle Scholar
  • 8 Chan L, Chin LM, Kennedy M et al. Benefits of intensive treadmill exercise training on cardiorespiratory function and quality of life in patients with pulmonary hypertension. Chest 2013; 143: 333-343
    CrossrefPubMedGoogle Scholar
  • 9 Weinstein AA, Chin LM, Keyser RE et al. Effect of aerobic exercise training on fatigue and physical activity in patients with pulmonary arterial hypertension. Respir Med 2013; 107: 778-784
    CrossrefPubMedGoogle Scholar
  • 10 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
    CrossrefPubMedGoogle Scholar
  • 11 Yuan P, Yuan XT, Sun XY et al. Exercise training for pulmonary hypertension: A systematic review and meta-analysis. International journal of cardiology 2014; 178c: 142-146
    PubMedGoogle Scholar
  • 12 Pandey A, Garg S, Khunger M et al. Efficacy and Safety of Exercise Training in Chronic Pulmonary Hypertension: Systematic Review and Meta-Analysis. Circulation Heart failure. 2015; 8: 1032-1043
    PubMedGoogle Scholar
  • 13 Buys R, Avila A, Cornelissen VA. Exercise training improves physical fitness in patients with pulmonary arterial hypertension: a systematic review and meta-analysis of controlled trials. BMC Pulm Med 2015; 15: 40
    CrossrefPubMedGoogle Scholar
  • 14 Jais X, Olsson KM, Barbera JA et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 2012; 40: 881-885
    CrossrefPubMedGoogle Scholar
  • 15 Duarte AG, Thomas S, Safdar Z et al. Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience. Chest 2013; 143: 1330-1336
    CrossrefPubMedGoogle Scholar
  • 16 Thorne S, Nelson-Piercy C et al. Pregnancy and contraception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care 2006; 32: 75-81
    CrossrefPubMedGoogle Scholar
  • 17 Bendayan D, Hod M, Oron G et al. Pregnancy outcome in patients with pulmonary arterial hypertension receiving prostacyclin therapy. Obstet Gynecol 2005; 106: 1206-1210
    CrossrefPubMedGoogle Scholar
  • 18 Bonnin M, Mercier FJ, Sitbon O et al. Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology 2005; 102: 1133-1137
    CrossrefPubMedGoogle Scholar
  • 19 Zengin E, Sinning C, Schrage B et al. Right heart failure in pregnant women with cyanotic congenital heart disease – The good, the bad and the ugly. International journal of cardiology 2016; 202: 773-775
    CrossrefPubMedGoogle Scholar
  • 20 Olsson KM, Jais X. Birth control and pregnancy management in pulmonary hypertension. Seminars in respiratory and critical care medicine 2013; 34: 681-688
    Article in Thieme ConnectPubMedGoogle Scholar
  • 21 Meyer S, McLaughlin VV, Seyfarth HJ et al. Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey. Eur Respir J 2013; 41: 1302-1307
    CrossrefPubMedGoogle Scholar
  • 22 Olofsson C, Bremme K, Forssell G et al. Cesarean section under epidural ropivacaine 0.75 % in a parturient with severe pulmonary hypertension. Acta Anaesthesiol Scand 2001; 45: 258-260
    PubMedGoogle Scholar
  • 23 Raines DE, Liberthson RR, Murray JR. Anesthetic management and outcome following noncardiac surgery in nonparturients with Eisenmenger’s physiology. J Clin Anesth 1996; 8: 341-347
    CrossrefPubMedGoogle Scholar
  • 24 Rich S, Dantzker DR, Ayres SM et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107: 216-223
    CrossrefPubMedGoogle Scholar
  • 25 Guillevin L, Armstrong I, Aldrighetti R et al. Understanding the impact of pulmonary arterial hypertension on patients’ and carers’ lives. Eur Respir Rev 2013; 22: 535-542
    CrossrefPubMedGoogle Scholar
  • 26 Lowe B, Grafe K, Ufer C et al. Anxiety and depression in patients with pulmonary hypertension. Psychosom Med 2004; 66: 831-836
    CrossrefPubMedGoogle Scholar
  • 27 Halank M, Speich R, Petkova D et al. Lebensqualität bei pulmonal arterieller und chronisch thromboembolischer pulmonaler Hypertonie. Dtsch med Wochenschr 2014; 139: S126-135
    Article in Thieme ConnectPubMedGoogle Scholar
  • 28 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
    CrossrefPubMedGoogle Scholar
  • 29 Whooley MA, Simon GE. Managing depression in medical outpatients. N Engl J Med 2000; 343: 1942-1950
    CrossrefPubMedGoogle Scholar
  • 30 Ladwig KH, Lederbogen F, Albus C et al. Position paper on the importance of psychosocial factors in cardiology: Update 2013. Ger Med Sci 2014; 12: Doc09
    PubMedGoogle Scholar
  • 31 Larisch A, Neeb C, de Zwaan M et al. Psychische Belastung und Psychosomatischer Behandlungswunsch von Patienten mit Pulmonaler Hypertonie. Psychother Psych Med 2014; 64: 384-389
    Article in Thieme ConnectPubMedGoogle Scholar
  • 32 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
    PubMedGoogle Scholar
  • 33 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
    CrossrefPubMedGoogle Scholar
  • 34 Fuster V, Steele PM, Edwards WD et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-587
    CrossrefPubMedGoogle Scholar
  • 35 Huber K, Beckmann R, Frank H et al. Fibrinogen, t-PA, and PAI-1 plasma levels in patients with pulmonary hypertension. Am J Respir Crit Care Med 1994; 150: 929-933
    CrossrefPubMedGoogle Scholar
  • 36 Herve P, Humbert M, Sitbon O et al. Pathobiology of pulmonary hypertension. The role of platelets and thrombosis. Clinics in chest medicine. 2001; 22: 451-458
    CrossrefPubMedGoogle Scholar
  • 37 Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446-1449
    CrossrefPubMedGoogle Scholar
  • 38 Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76-81
    CrossrefPubMedGoogle Scholar
  • 39 Olsson KM, Delcroix M, Ghofrani HA 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
    CrossrefPubMedGoogle Scholar
  • 40 Galiè ND, Ghofrani HA, Jansa P et al. Anticoagulant therapy does not influence long-term outcomes in patients with pulmonary arterial hypertension (PAH): insights from the randomised controlled SERAPHIN trial of macitentan. Eur Heart J 2014; 35 (Suppl 1) 10
    CrossrefPubMedGoogle Scholar
  • 41 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
    CrossrefPubMedGoogle Scholar
  • 42 Cohn JN. Optimal diuretic therapy for heart failure. Am J Med 2001; 111: 577
    CrossrefPubMedGoogle Scholar
  • 43 Maron BA, Waxman AB, Opotowsky AR et al. Effectiveness of spironolactone plus ambrisentan for treatment of pulmonary arterial hypertension (from the [ARIES] study 1 and 2 trials). Am J Cardiol 2013; 112: 720-725
    CrossrefPubMedGoogle Scholar
  • 44 Rabinovitz A, Raiszadeh F, Zolty R. Association of hyponatremia and outcomes in pulmonary hypertension. Journal of cardiac failure 2013; 19: 550-556
    CrossrefPubMedGoogle Scholar
  • 45 Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682-1687
    CrossrefPubMedGoogle Scholar
  • 46 Hildenbrand FF, Bloch KE, Speich R et al. Daytime measurements underestimate nocturnal oxygen desaturations in pulmonary arterial and chronic thromboembolic pulmonary hypertension. Respiration; international review of thoracic diseases. 2012; 84: 477-484
    CrossrefPubMedGoogle Scholar
  • 47 Ulrich S, Keusch S, Hildenbrand FF et al. Effect of nocturnal oxygen and acetazolamide on exercise performance in patients with pre-capillary pulmonary hypertension and sleep-disturbed breathing: randomized, double-blind, cross-over trial. Eur Heart J 2013; 36: 615-623
    PubMedGoogle Scholar
  • 48 Rich S, Seidlitz M, Dodin E et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998; 114: 787-792
    CrossrefPubMedGoogle Scholar
  • 49 Ghofrani HA, Distler O, Gerhardt F et al. Treatment of pulmonary arterial hypertension (PAH): updated Recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 2011; 154: S20-33
    CrossrefPubMedGoogle Scholar
  • 50 Ruiter G, Lankhorst S, Boonstra A et al. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J. 2011; 37: 1386-1391
    CrossrefPubMedGoogle Scholar
  • 51 Ruiter G, Lanser IJ, de Man FS et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology 2014; 53: 285-292
    CrossrefPubMedGoogle Scholar
  • 52 Broberg CS, Jayaweera AR, Diller GP et al. Seeking optimal relation between oxygen saturation and hemoglobin concentration in adults with cyanosis from congenital heart disease. Am J Cardiol 2011; 107: 595-599
    CrossrefPubMedGoogle Scholar
  • 53 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
    CrossrefPubMedGoogle Scholar
  • 54 Van De Bruaene A, Delcroix M, Pasquet A et al. Iron deficiency is associated with adverse outcome in Eisenmenger patients. Eur Heart J 2011; 32: 2790-2799
    CrossrefPubMedGoogle Scholar
  • 55 Viethen T, Gerhardt F, Dumitrescu D et al. Ferric carboxymaltose improves exercise capacity and quality of life in patients with pulmonary arterial hypertension and iron deficiency: a pilot study. Int J Cardiol 2014; 175: 233-239
    CrossrefPubMedGoogle Scholar
  • 56 Kaemmerer H, Fratz S, Braun SL et al. Erythrocyte indexes, iron metabolism, and hyperhomocysteinemia in adults with cyanotic congenital cardiac disease. Am J Cardiol 2004; 94: 825-828
    CrossrefPubMedGoogle Scholar
  • 57 Tongers J, Schwerdtfeger B, Klein G et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 2007; 153: 127-132
    CrossrefPubMedGoogle Scholar
  • 58 Hoeper MM, Galie N, Murali S et al. Outcome after cardiopulmonary resuscitation in patients with pulmonary arterial hypertension. Am J Respir Crit Care Med. 2002; 165: 341-344
    CrossrefPubMedGoogle Scholar
  • 59 Showkathali R, Tayebjee MH, Grapsa J et al. Right atrial flutter isthmus ablation is feasible and results in acute clinical improvement in patients with persistent atrial flutter and severe pulmonary arterial hypertension. Int J Cardiol 2011; 149: 279-280
    CrossrefPubMedGoogle Scholar
  • 60 Bandorski D, Bogossian H, Braun O et al. Patients with atrial fibrillation complicated by coronary artery disease. Is a single value of sensitive cardiac troponin I on admission enough? Herzschrittmacherther Elektrophysiol 2015; 26: 39-44
    PubMedGoogle Scholar
  • 61 Rajdev A, Garan H, Biviano A. Arrhythmias in pulmonary arterial hypertension. Prog Cardiovasc Dis 2012; 55: 180-186
    CrossrefPubMedGoogle Scholar
  • 62 Luesebrink U, Fischer D, Gezgin F et al. Ablation of typical right atrial flutter in patients with pulmonary hypertension. Heart Lung Circ 2012; 21: 695-699
    CrossrefPubMedGoogle Scholar