nach oben

Erschienen in:

01.05.2015 | CME Zertifizierte Fortbildung

Pulmonale Hypertonie

Mögliche genetische Ursachen und therapeutische Optionen

verfasst von: Prof. Dr. E. Grünig, C.A. Eichstaedt, N. Ehlken, E. Mayer, H. Klose

Erschienen in: Herz | Ausgabe 3/2015

Einloggen, um Zugang zu erhalten

Zusammenfassung

Die pulmonale Hypertonie (PH) ist eine schwere Krankheit, die oft viel zu spät erkannt wird. Sie ist primär durch Luftnot und Rechtsherzinsuffizienz gekennzeichnet. Erbliche Faktoren spielen insbesondere für die seltene pulmonalarterielle Hypertonie (PAH) und die PAH bei angeborenen Herzfehlern eine Rolle. In diesem Artikel werden die allgemeinen und supportiven Therapien für den Lungenhochdruck sowie die gezielte medikamentöse Behandlung der PAH und der nichtoperablen chronisch thromboembolischen PH nach dem aktuellsten Stand dargestellt. Um eine gute Prognose der Patienten zu erreichen, ist eine frühe Überweisung an ein Expertenzentrum zur weiterführenden Diagnostik und Therapie essenziell.

Grünig E, Ehlken N, Hohenforst-Schmidt W et al (2014) Allgemeine Therapie der pulmonal arteriellen Hypertonie. Dtsch Med Wochenschr 139:136–141CrossRef

Opitz CF, Blindt R, Blumberg F et al (2011) Pulmonary hypertension: hemodynamic evaluation. Updated Recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 154(Suppl 1):S13–S19CrossRefPubMed

Opitz CF, Blindt R, Blumberg F et al (2010) Pulmonary hypertension: hemodynamic evaluation: hemodynamic evaluation – recommendations of the Cologne Consensus Conference 2010. Dtsch Med Wochenschr 135(Suppl 3):S78–S86CrossRefPubMed

Hoeper MM, Huscher D, Ghofrani HA et al (2013) Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 168:871–880CrossRefPubMed

Grünig E, Janssen B, Mereles D et al (2000) Abnormal pulmonary artery pressure response in asymptomatic carriers of primary pulmonary hypertension gene. Circulation 102:1145–1150CrossRefPubMed

Deng Z, Morse JH, Slager SL et al (2000) Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 67:737–744CrossRefPubMedCentralPubMed

Machado RD, Aldred MA, James V et al (2006) Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat 27:121–132CrossRefPubMed

Pfarr N, Szamalek-Hoegel J, Fischer C et al (2011) Hemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations. Respir Res 12:99CrossRefPubMedCentralPubMed

Soubrier F, Chung WK, Machado R et al (2013) Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol 62:D13–D21CrossRefPubMed

10.

Hinderhofer K, Fischer C, Pfarr N et al (2014) Identification of a new intronic BMPR2-mutation and early diagnosis of heritable pulmonary arterial hypertension in a large family with mean clinical follow-up of 12 years. PLoS One 9:e91374CrossRefPubMedCentralPubMed

11.

Galie N, Hoeper MM, Humbert M et al (2009) 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 34:1219–1263CrossRefPubMed

12.

Jais X, Olsson KM, Barbera JA et al (2012) Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 40:881–885CrossRefPubMed

13.

Mereles D, Ehlken N, Kreuscher S et al (2006) Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation 114:1482–1489CrossRefPubMed

14.

Chan L, Chin LM, Kennedy M et al (2013) Benefits of intensive treadmill exercise training on cardiorespiratory function and quality of life in patients with pulmonary hypertension. Chest 143:333–343CrossRefPubMedCentralPubMed

15.

Weinstein AA, Chin LM, Keyser RE et al (2013) Effect of aerobic exercise training on fatigue and physical activity in patients with pulmonary arterial hypertension. Respir Med 107:778–784CrossRefPubMedCentralPubMed

16.

Becker-Grünig T, Klose H, Ehlken N et al (2013) Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol 168:375–381CrossRefPubMed

17.

Man FS de, Handoko ML, Groepenhoff H et al (2009) Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 34:669–675CrossRefPubMed

18.

Ehlken N, Verduyn C, Tiede H et al (2014) Economic evaluation of exercise training in patients with pulmonary hypertension. Lung 192:359–366CrossRefPubMed

19.

Fox BD, Kassirer M, Weiss I et al (2011) Ambulatory rehabilitation improves exercise capacity in patients with pulmonary hypertension. J Card Fail 17:196–200CrossRefPubMed

20.

Grünig E, Ehlken N, Ghofrani A et al (2011) Effect of exercise and respiratory training on clinical progression and survival in patients with severe chronic pulmonary hypertension. Respiration 81:394–401CrossRefPubMed

21.

Grünig E, Lichtblau M, Ehlken N et al (2012) Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 40:84–92CrossRefPubMed

22.

Grünig E, Maier F, Ehlken N et al (2012) Exercise training in pulmonary arterial hypertension associated with connective tissue diseases. Arthritis Res Ther 14:R148CrossRefPubMedCentralPubMed

23.

Kabitz HJ, Bremer HC, Schwoerer A et al (2014) The combination of exercise and respiratory training improves respiratory muscle function in pulmonary hypertension. Lung 192:321–328CrossRefPubMed

24.

Nagel C, Prange F, Guth S et al (2012) Exercise training improves exercise capacity and quality of life in patients with inoperable or residual chronic thromboembolic pulmonary hypertension. PLoS One 7:e41603CrossRefPubMedCentralPubMed

25.

Martinez-Quintana E, Miranda-Calderin G, Ugarte-Lopetegui A et al (2010) Rehabilitation program in adult congenital heart disease patients with pulmonary hypertension. Congenit Heart Dis 5:44–50CrossRefPubMed

26.

Raskin J, Qua D, Marks T et al (2014) A retrospective study on the effects of pulmonary rehabilitation in patients with pulmonary hypertension. Chron Respir Dis 11:153–162CrossRefPubMed

27.

Mainguy V, Maltais F, Saey D et al (2010) Effects of a rehabilitation program on skeletal muscle function in idiopathic pulmonary arterial hypertension. J Cardiopulm Rehabil Prev 30:319–323CrossRefPubMed

28.

Shoemaker MJ, Wilt JL, Dasgupta R et al (2009) Exercise training in patients with pulmonary arterial hypertension: a case report. Cardiopulm Phys Ther J 20:12–18PubMedCentralPubMed

29.

Yuan P, Yuan XT, Sun XY et al (2015) Exercise training for pulmonary hypertension: a systematic review and meta-analysis. Int J Cardiol 178:142–146CrossRefPubMed

30.

Galiè N, Corris PA, Frost A et al (2013) Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 62:D60–D72CrossRefPubMed

31.

Barst RJ, Gibbs JS, Ghofrani HA et al (2009) Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 54:S78–S84CrossRefPubMedCentralPubMed

32.

Lowe B, Grafe K, Ufer C et al (2004) Anxiety and depression in patients with pulmonary hypertension. Psychosom Med 66:831–836CrossRefPubMed

33.

Halank M, Einsle F, Lehman S et al (2013) Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung 191:337–343CrossRefPubMed

34.

Harzheim D, Klose H, Pinado FP et al (2013) Anxiety and depression disorders in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Respir Res 14:104CrossRefPubMedCentralPubMed

35.

Larisch A, Neeb C, Zwaan M de et al (2014) Mental distress and wish for psychosomatic treatment of patients with pulmonary hypertension. Psychother Psychosom Med Psychol 64:384–389CrossRefPubMed

36.

Ladwig KH, Lederbogen F, Albus C et al (2014) Position paper on the importance of psychosocial factors in cardiology: Update 2013. Ger Med Sci 12:Doc09PubMedCentralPubMed

37.

Price LC, Montani D, Jais X et al (2010) Noncardiothoracic nonobstetric surgery in mild-to-moderate pulmonary hypertension. Eur Respir J 35:1294–1302CrossRefPubMed

38.

Ramakrishna G, Sprung J, Ravi BS et al (2005) Impact of pulmonary hypertension on the outcomes of noncardiac surgery: predictors of perioperative morbidity and mortality. J Am Coll Cardiol 45:1691–1699CrossRefPubMed

39.

Meyer S, McLaughlin VV, Seyfarth HJ et al (2013) Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey. Eur Respir J 41:1302–1307CrossRefPubMed

40.

Hildenbrand FF, Bloch KE, Speich R et al (2012) Daytime measurements underestimate nocturnal oxygen desaturations in pulmonary arterial and chronic thromboembolic pulmonary hypertension. Respiration 84:477–484CrossRefPubMed

41.

Sandoval J, Aguirre JS, Pulido T et al (2001) Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 164:1682–1687CrossRefPubMed

42.

Olsson KM, Delcroix M, Ghofrani HA et al (2014) Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 129:57–65CrossRefPubMed

43.

Tongers J, Schwerdtfeger B, Klein G et al (2007) Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 153:127–132CrossRefPubMed

44.

Hoeper MM, Galie N, Murali S et al (2002) Outcome after cardiopulmonary resuscitation in patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 165:341–344CrossRefPubMed

45.

Luesebrink U, Fischer D, Gezgin F et al (2012) Ablation of typical right atrial flutter in patients with pulmonary hypertension. Heart Lung Circ 21:695–699CrossRefPubMed

46.

Ghofrani HA, Distler O, Gerhardt F et al (2010) Treatment of pulmonary arterial hypertension (PAH): recommendations of the Cologne Consensus Conference 2010. Dtsch Med Wochenschr 135(Suppl 3):S87–S101CrossRefPubMed

47.

Ruiter G, Lankhorst S, Boonstra A et al (2011) Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 37:1386–1391CrossRefPubMed

48.

Galie N, Manes A, Negro L et al (2009) A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 30:394–403CrossRefPubMedCentralPubMed

49.

Galie N, Rubin L, Hoeper M et al (2008) Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 371:2093–2100CrossRefPubMed

50.

Simonneau G, Rubin LJ, Galie N et al (2008) Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 149:521–530CrossRefPubMed

51.

Pulido T, Adzerikho I, Channick RN et al (2013) Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 369:809–818CrossRefPubMed

52.

Olschewski H, Simonneau G, Galie N et al (2002) Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 347:322–329CrossRefPubMed

53.

Hoeper MM, Gall H, Seyfarth HJ et al (2009) Long-term outcome with intravenous iloprost in pulmonary arterial hypertension. Eur Respir J 34:132–137CrossRefPubMed

54.

Rubin LJ, Mendoza J, Hood M et al (1990) Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 112:485–491CrossRefPubMed

55.

Barst RJ (1996) Diagnosis and treatment of pulmonary artery hypertension. Curr Opin Pediatr 8:512–519CrossRefPubMed

56.

Badesch DB, Tapson VF, McGoon MD et al (2000) Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 132:425–434CrossRefPubMed

57.

Hiremath J, Thanikachalam S, Parikh K et al (2010) Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant 29:137–149CrossRefPubMed

58.

Simonneau G, Torbicki A, Hoeper MM et al (2012) Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 40:874–880CrossRefPubMed

59.

Ghofrani HA, Galie N, Grimminger F et al (2013) Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 369:330–340CrossRefPubMed

60.

Sitbon O, Jais X, Savale L et al (2014) Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 43:1691–1697CrossRefPubMed

61.

Behr J, Borst MM, Winkler J et al (2005) A role for combination therapy in pulmonary arterial hypertension. Pneumologie 59:730–735CrossRefPubMed

62.

Mayer E, Jenkins D, Lindner J et al (2011) Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg 141:702–710CrossRefPubMed

63.

Madani MM, Auger WR, Pretorius V et al (2012) Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg 94:97–103CrossRefPubMed

64.

Ghofrani HA, D’Armini AM, Grimminger F et al (2013) Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 369:319–329CrossRefPubMed

Titel: Pulmonale Hypertonie
Mögliche genetische Ursachen und therapeutische Optionen
verfasst von: Prof. Dr. E. Grünig
C.A. Eichstaedt
N. Ehlken
E. Mayer
H. Klose
Publikationsdatum: 01.05.2015
Verlag: Urban & Vogel
Erschienen in: Herz / Ausgabe 3/2015
Print ISSN: 0340-9937
Elektronische ISSN: 1615-6692
DOI: https://doi.org/10.1007/s00059-015-4230-6

Neu im Fachgebiet Kardiologie

25.04.2024 | Hypotonie | Nachrichten

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Pulmonale Hypertonie

Mögliche genetische Ursachen und therapeutische Optionen

Zusammenfassung

Neu im Fachgebiet Kardiologie

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

Therapiestart mit Blutdrucksenkern erhöht Frakturrisiko

Adipositas-Medikament auch gegen Schlafapnoe wirksam

Komplette Revaskularisation bei Infarkt: Neue Studie setzt ein Fragezeichen

Update Kardiologie

Springer Medizin

Zusammenfassung

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 3/2015

Sport und Herz – keine ganz problemlose Beziehung

Predictive value of plasma asymmetric dimethylarginine, homocysteine, and high-sensitive CRP levels in occult coronary artery disease

Terminal QRS distortion and severity of coronary artery disease in ST-elevation myocardial infarction

Link between aortic valve sclerosis and myocardial no-reflow in ST-segment elevation myocardial infarction

Impact of D-dimer level on postinterventional coronary flow and in-hospital MACE in ST-segment elevation myocardial infarction

Isolated interrupted aortic arch in adulthood

Neu im Fachgebiet Kardiologie

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

Therapiestart mit Blutdrucksenkern erhöht Frakturrisiko

Adipositas-Medikament auch gegen Schlafapnoe wirksam

Komplette Revaskularisation bei Infarkt: Neue Studie setzt ein Fragezeichen

Update Kardiologie