Zusammenfassung
Die pulmonale Hypertonie ist durch hämodynamische Veränderungen (Druck- bzw. Widerstandserhöhung) in der pulmonalen Strombahn gekennzeichnet, welche durch verschiedene Auslöser bedingt sein können. Folge ist eine erhöhte rechtsventrikuläre Nachlast und somit eine chronische Belastung des rechten Herzens. Im Gegensatz zum systemischen Kreislauf besitzt die pulmonale Zirkulation als sog. Niederdrucksystem aufgrund ihrer hohen Kapazität und großen Dehnbarkeit sowie des geringen Anteils an glatter Muskulatur in kleinen Arterien und Arteriolen primär keine hypertensive Prädisposition. Verringert sich jedoch der Gefäßquerschnitt durch Destruktion, Obliteration oder okklusive Läsionen in den Widerstandsgefäßen, so kommt es zu einem Anstieg des pulmonal vaskulären Widerstands und des pulmonal arteriellen Drucks. Klinische Manifestationen, natürlicher Verlauf und Reversibilität der pulmonalen Hypertonie hängen maßgeblich von der Art der pulmonalen Läsion und der Ätiologie und Schwere der hämodynamischen Veränderungen ab.
Literatur
Abenhaim L, Moride Y, Brenot F et al (1996) Appetite-suppressant drugs and the risk of primary pulmonary hypertension. N Engl J Med 335:609–616
Abraham WT, Stevenson LW, Bourge RC, Lindenfeld JA, Bauman JG, Adamson PB, CHAMPION Trial Study Group (2016) Sustained efficacy of pulmonary artery pressure to guide adjustment of chronic heart failure therapy: complete follow-up results from the CHAMPION randomised trial. Lancet 387:453–461
Alabed S, Shahin Y, Garg P, Alandejani F, Johns CS, Lewis RA et al (2021) Cardiac-MRI predicts clinical worsening and mortality in pulmonary arterial hypertension: a systematic review and meta-analysis. JACC Cardiovasc Imaging 14:931–942
Aßmus B, Angermann CE, Alkhlout B et al (2022) Treatment response to heart failure management guided by remote pulmonary-artery-pressure-monitoring depends on presence and severity of pulmonary hypertension. Eur J Heart Fail 24:2320–2330. Epub ahead of print
Boucly A, Savale L, Jaïs X et al (2021) Association between initial treatment strategy and long-term survival in pulmonary arterial hypertension. Am J Respir Crit Care Med 204:842–854
Boucly A, Weatherald J, Savale L et al (2022) External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry. Eur Respir J 59(6):2102419
Budev MM, Arroliga AC, Wiedemann HP, Matthay RA (2003) Cor pulmonale: an overview. Semin Respir Crit Care Med 24:233–244
Chin K, Sitbon O, Doelberg M et al (2021) Three- versus two-drug therapy for patients with newly diagnosed pulmonary arterial hypertension. J Am Coll Cardiol 78:1393–1403
Chin KM, Rubin LJ, Channick R, Di Scala L, Gaine S, Galiè N et al (2019) Association of N-terminal pro brain natriuretic peptide and long-term outcome in patients with pulmonary arterial hypertension. Insights From the Phase III GRIPHON Study. Circulation 139:2440–2450
Coghlan JG, Wolf M, Distler O, Denton CP, Doelberg M, Harutyunova S et al (2018) Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. Eur Respir J 51:1701197
D’Alonzo GE, Barst RJ, Ayres SM et al (1991) Survival in patients with primary pulmonary hypertension. Ann Intern Med 115:343–349
Delcroix M, Torbicki A, Gopalan D et al (2021) ERS statement on chronic thrombo-embolic pulmonary hypertension. Eur Respir J 57(6):2002828
Dimopoulos K, Inuzuka R, Goletto S et al (2010) Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 121:20–25
Dumitrescu D, Nagel C, Kovacs G, Bollmann T, Halank M, Winkler J et al (2017) Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis. Heart 103:774–782
Eyries M, Montani D, Girerd B, Perret C, Leroy A, Lonjou C et al (2014) EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet 46:65–69
Fauvel C, Raitiere O, Boucly A, de Groote P, Renard S, Bertona J et al (2022) Interest of TAPSE/sPAP ratio for noninvasive pulmonary arterial hypertension risk assessment. J Heart Lung Transplant 41:1761–1772
Gaine S (2000) Pulmonary hypertension. JAMA 284:3160–3168
Galiè N, Ghofrani HA, Torbicki A et al, for the Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group (2005) Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353: 21482157
Galiè N, Brundage BH, Ghofrani HA et al, for the PHIRST Investigators (2009) Tadalafil therapy for pulmonary arterial hypertension. Circulation 119:2894–2903
Galiè N, Barbera JA, Frost A et al (2015) Initial use of Ambrisentan plus Tadalafil in pulmonary arterial hypertension. New Engl J Med 379:834–844
Galiè N, Humbert M, Vachiery JL et al (2016) 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). Eur Heart J 37:67–119
Ghofrani HA, D’Armini AM, Grimminger F et al, for the CHEST Investigators (2013a) Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 369:319–329
Ghofrani HA, Galiè N, Grimminger F et al, for the PATENT Investigators (2013b) Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 369:330–340
Guazzi M, Naeije R (2017) Pulmonary hypertension in heart failure: pathophysiology, pathobiology, and emerging clinical perspectives. J Am Coll Cardiol 69:1718–1734
Guazzi M, Vicenzi M, Arena R, Guazzi MD (2011) Pulmonary hypertension in heart failure with preserved ejection fraction: a target of phosphodiesterase-5 inhibition in a 1-year study. Circulation 124:164–174
Hachulla E, Jais X, Cinquetti G, Clerson P, Rottat L, Launay D et al (2018) Pulmonary arterial hypertension associated with systemic lupus erythematosus: results from the French Pulmonary Hypertension Registry. Chest 153:143–151
Harris P, Heath D (1986) The structure of the normal pulmonary blood vessels after infancy. In: Harris P, Heath D (Hrsg) The human circulation: its form and function in health and disease. Churchill-Livingstone, Edinburgh, S 30–47
Hoendermis E, Liu LCY, Hummel YM et al (2015) Effects of sildenafil on invasive hemodynamics and exercise capacity in heart failure patients with preserved ejection fraction and pulmonary hypertension: a randomized controlled trial. Eur Heart J 36:2565–2573
Hoeper MM, Pletz MW, Golpon H, Welte T (2007) Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 29:944–950
Hoeper MM, Ghofrani HA, Al-Hiti H et al, on behalf of the REPLACE investigators (2021) Switching from phosphodiesterase-5 inhibitors to riociguat in patients with pulmonary arterial hypertension: results from the REPLACE study. Lancet Respir Med 9:573–584
Hoeper MM, Dwivedi K, Pausch C et al (2022a) Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis. Lancet Respir Med 10:937–948
Hoeper MM, Pausch C, Olsson KM et al (2022b) COMPERA 2.0: a refined 4-strata risk assessment model for pulmonary arterial hypertension. Eur Respir J 60(1):2102311
Hoeper MM, Badesch DB, Ghofrani HA, Gibbs JSR, Gomberg-Maitland M, McLaughlin VV et al, for the STELLAR Trial Investigators (2023) A phase 3 trial of Sotatercept for the treatment of pulmonary arterial hypertension. N Eng J Med 388:1478–1490
Humbert M, Guignabert C, Bonnet S et al (2019) Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J 53(1):pii: 1801887
Humbert M, McLaughlin V, Gibbs JSR, Gomberg-Maitland M, Hoeper MM, Preston IR et al, PULSAR Trial Investigators (2021) Sotatercept for the treatment of pulmonary arterial hypertension. N Engl J Med 384:1204–1215
Humbert M, Kovacs G, Hoeper MM et al (2022) 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 43:3618–3731
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C et al (2022) Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med 10:961–971
Kim NH, Delcroix M, Jais X et al (2019) Chronic thromboembolic pulmonary hypertension. Eur Respir J 53(1). https://doi.org/10.1183/13993003.01915-2018
Kinsella JP, Abman SH (1995) Recent developments in the pathophysiology and treatment of persistent pulmonary hypertension of the newborn. J Pediatr 126:853–864
Kovacs G, Berghold A, Scheidl S, Olschewski H (2009) Pulmonary arterial pressure during rest and exercise in healthy subjects. A systematic review. Eur Respir J 34:888–894
Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA et al (2016) International Liver Transplant Society Practice Guidelines: diagnosis and management of hepatopulmonary syndrome and portopulmonary hypertension. Transplantation 100:1440–1452
Lammers AE, Bauer LJ, Diller GP, Helm PC, Abdul-Khaliq H, Bauer UMM et al (2020) Pulmonary hypertension after shunt closure in patients with simple congenital heart defects. Int J Cardiol 308:28–32
Lau EMT, Giannoulatou E, Celermajer DS, Humbert M (2017) Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol 14:603–614
Launay D, Montani D, Hassoun PM, Cottin V, Le Pavec J, Clerson P et al (2018) Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis. PLoS One 13:e0197112
Leber L, Beaudet A, Muller A (2021) Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulm Circ 11:2045894020977300
Lewis RA, Johns CS, Cogliano M, Capener D, Tubman E, Elliot CA et al (2020) Identification of cardiac magnetic resonance imaging thresholds for risk stratification in pulmonary arterial hypertension. Am J Respir Crit Care Med 201:458–468
Lopez-Sendon J, Sanchez MAG, De Juan MJM, Coma-Canella I (1990) Pulmonary hypertension in the toxic oil syndrome. In: Fishman AP (Hrsg) The pulmonary circulation: normal and abnormal. University of Pennsylvania Press, Philadelphia, S 385–396
Mahmut E, Madani MM, Kim NH et al (2018) Chronic thromboembolic pulmonary hypertension: evolving therapeutic approaches for operable and inoperable disease. J Am Coll Cardiol 71:2468–2486
Marecki JC, Cool CD, Parr JE et al (2006) HIV-1 Nef is associated with complex pulmonary vascular lesions in SHIV-nef-infected macaques. Am J Respir Crit Care Med 174:437–445
Maron BA, Hess E, Maddox TM et al (2016) Association of borderline pulmonary hypertension with mortality and hospitalization in a large patient cohort: insights from the Veterans Affairs clinical assessment, reporting, and tracking program. Circulation 133:1240–1248
Maron BA, Brittain EL, Hess E et al (2020) Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study. Lancet Respir Med 8:873–884
McLaughlin VV, Vachiery JL, Oudiz R et al (2019) Patients with pulmonary arterial hypertension with and without cardiovascular risk factors: results from the AMBITION trial. J Heart Lung Transplant 38:1286–1295
Montani D, Lau EM, Dorfmuller P, Girerd B, Jais X, Savale L et al (2016) Pulmonary veno-occlusive disease. Eur Respir J 47:1518–1534
Morrell NW, Aldred MA, Chung WJ et al (2019) Genetics and genomics in pulmonary arterial hypertension. Eur Respir J 58:1801899
Moser B, Jaksch P, Taghavi S, Murakozy G, Lang G, Hager H et al (2018) Lung transplantation for idiopathic pulmonary arterial hypertension on intraoperative and postoperatively prolonged extracorporeal membrane oxygenation provides optimally controlled reperfusion and excellent outcome. Eur J Cardiothorac Surg 53:178–185
Nathan SD, Barbera JA, Gaine SP et al (2019) Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J 53(1):1801914
Olschewski A, Weir EK (2004) Hypoxic pulmonary vasoconstriction and hypertension. In: Peacock AJ, Rubin LJ (Hrsg) Pulmonary circulation. Arnold/Oxford University Press, London, S 33–44
Olschewski A, Berghausen EM, Eichstaedt CA et al (2018) Pathobiology, pathology and genetics of pulmonary hypertension: update from the Cologne Consensus Conference 2018. Int J Cardiol 272S:4–10
Opitz CF, Hoeper MM, Gibbs JSR et al (2016) Pre-capillary, combined, and post-capillary pulmonary hypertension: a pathophysiological continuum. J Am Coll Cardiol 68:368–378
Pulido T, Adzerikho I, Channick RN et al, for the SERAPHIN Investigators (2013) Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 369:809–818
Rabinovitch M (2012) Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest 122:4306–4313
Rich S, Dantzker DR, Ayres SM et al (1987) Primary pulmonary hypertension: a national prospective study. Ann Intern Med 107:216–223
Riel AC van, Schuuring MJ, van Hessen ID, Zwinderman AH, Cozijnsen L, Reichert CL et al (2014) Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification. Int J Cardiol 174:299–305
Rosenkranz S, Gibbs JSR, Wachter R, De Marco T, Vonk-Noordegraaf A, Vachiéry JL (2016) Left ventricular heart failure and pulmonary hypertension. Eur Heart J 37:942–954
Rosenkranz S, Howard LS, Gomberg-Maitland M, Hoeper MM (2020) Systemic consequences of pulmonary hypertension and right heart failure. Circulation 141:678–693
Rosenkranz S, Channick R, Chin KM, Jenner B, Gaine S, Galiè N et al (2022) Impact of comorbidities on selexipag treatment effect in patients with pulmonary arterial hypertension: insights from the GRIPHON study. Eur J Heart Fail 24:205–214
Rosenkranz S, Pausch C, Coghlan JG et al (2023) Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: a COMPERA analysis. J Heart Lung Transplant 42:102–114
Rudski LG, Lai WW, Afilalo J et al (2010) Guidelines for the échocardiographie assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 23:685–713
Sadushi-Kolici R, Jansa P, Kopec G, Torbicki A, Skoro-Sajer N, Campean IA et al (2019) Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. Lancet Respir Med 7:239–248
Savale L, Guimas M, Ebstein N, Fertin M, Jevnikar M, Renard S et al (2020) Portopulmonary hypertension in the current era of pulmonary hypertension management. J Hepatol 73:130–139
Simonneau G, Montani D, Celermayer DS et al (2019) Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 53(1). https://doi.org/10.1183/13993003.01913-2018
Sitbon O, Humbert M, Jais X et al (2005) Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:3105–3111
Sitbon O, Lascoux-Combe C, Delfraissy JF et al (2008) Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med 177:108–113
Sitbon O, Channick R, Chin KM et al, GRIPHON Investigators (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373:2522–2533
Tongers J, Schwerdtfeger B, Klein G et al (2007) Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 153:127–132
Tunariu N, Gibbs SJ, Win Z et al (2007) Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med 48:680–684
Vachiéry JL, Tedford RJ, Rosenkranz S, Palazzini M, Lang I, Guazzi M, Coghlan G, Chazova I, De Marco T (2019) Pulmonary hypertension due to left heart disease. Eur Respir J 53(1). https://doi.org/10.1183/13993003.01897-2018
Valerio L, Mavromanoli AC, Barco S, et al, on behalf of the FOCUS Investigators (2022) Implications of persistent functional impairment after acute pulmonary embolism: FOCUS, a prospective multicentre observational cohort study. Eur Heart J 43:3387–3398
Vanderpool RR, Saul M, Nouraie M et al (2018) Association between hemodynamic markers of pulmonary hypertension and outcomes in patients with heart failure and preserved ejection fraction. JAMA Cardiol 3:298–306
Wasserman K, Hansen JE, Sue DY et al (Hrsg) (2004) Principles of exercise testing and interpretation, 4. Aufl. Lippincott Williams & Wilkins, Philadelphia
Waxman A, Restrepo-Jaramillo R, Thenappan T et al (2021) Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med 384:325–334
Weatherald J, Montani D, Jevnikar M, Jais X, Savale L, Humbert M (2019) Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev 28:190023
Yasunobu Y, Oudiz RJ, Sun XG et al (2005) End-tidal PCO2 abnormality and exercise limitation in patients with primary pulmonary hypertension. Chest 127:1637–1646
Yusen RD, Edwards LB, Kucheryavaya AY, Benden C, Dipchand AI, Goldfarb SB et al (2015) The Registry of the International Society for Heart and Lung Transplantation: thirty-second official adult lung and heart-lung transplantation report – 2015; focus theme: early graft failure. J Heart Lung Transplant 34:1264–1277
Zeder K, Banfi C, Steinrisser-Allex G et al (2022) Diagnostic, prognostic and differential-diagnostic relevance of pulmonary haemodynamic parameters during exercise: a systematic review. Eur Respir J 60(4):2103181
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Rosenkranz, S. (2023). Pulmonale Hypertonie. In: Marx, N., Erdmann, E. (eds) Klinische Kardiologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-62939-0_30-1
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