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Screening auf pulmonale Hypertonie

Screening in pulmonary hypertension

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Der Pneumologe Aims and scope

Zusammenfassung

Die pulmonal arterielle Hypertonie (PAH) ist eine seltene Erkrankung, bei welcher ein generelles bevölkerungsbasiertes Screening wegen der geringen Prävalenz und fehlender einfacher Suchtests nicht empfohlen wird. Dennoch sollte bei Patienten mit Belastungsdyspnoe, welche das häufigste Symptom einer PAH ist, neben den häufigen Ursachen der Atemnot (Herz- bzw. Lungenerkrankungen, Anämie) auch an die pulmonale Hypertonie (PH) gedacht werden. Die wichtigste nichtinvasive Untersuchungsmethode ist die auf PH fokussierte Echokardiographie. Wichtige Hinweise können auch einfache Methoden geben, wie ein Rechtstyp im EKG, die rechtsseitige Verbreiterung des Herzens im Thorax-Röntgenbild oder die Erhöhung des Brain Natriuretic Peptide (BNP) bzw. des N‑terminal Pro Brain Natriuretic Peptide (NT-proBNP). Bei unklarer Symptomatik ist die Spiroergometrie sehr hilfreich. Spezialisierte Computertomographie-(CT-) und Magnetresonanztomographie (MRT-)Methoden erlauben die nichtinvasive Erfassung der pulmonalen Hämodynamik. Besonders wichtig ist es, bei Risikoerkrankungen einer PAH gezielte Untersuchungen durchzuführen. Bei der Sklerodermie wird ein regelmäßiges Screening mittels Echokardiographie empfohlen, auch wenn keine Dyspnoe vorliegt. Bei angeborenen Herzfehlern, HIV-Infektion, portaler Hypertension oder nach einer akuten Lungenembolie wird bei chronischer Belastungsdyspnoe ein aktives Screening auf PH empfohlen. Bei schweren Lungenerkrankungen soll eine Überweisung ins Expertenzentrum erfolgen, wenn sich in der Echokardiographie Hinweise auf eine schwere PH ergeben.

Abstract

Pulmonary arterial hypertension (PAH) is a rare disease and due to its low prevalence and lack of simple specific diagnostic tests, general screening is not recommended. Nevertheless, in patients with chronic exertional dyspnea, which is the main symptom of PAH, in addition to the frequent causes of dyspnea, such as left heart and lung diseases or anemia, pulmonary hypertension (PH) should also be considered. The most important non-invasive diagnostic examination method is echocardiography with the focus on PH. Furthermore, simple tools, such as right axis deviation in the electrocardiogram (ECG), right-sided cardiomegaly in chest X‑rays and increased brain natriuretic peptide (BNP) or N‑terminal pro brain natriuretic peptide (NT-proBNP) levels, may reveal important signs of PH. Cardiopulmonary exercise testing may add important diagnostic information in cases of unclear symptoms. Specialized computed tomography (CT) and magnetic resonance imaging (MRI) methods enable the non-invasive assessment of pulmonary hemodynamics. It is especially important to perform targeted investigations in patients at risk for PAH. In patients with systemic sclerosis annual screening with echocardiography is recommended even in asymptomatic patients. In patients with congenital heart disease, HIV infections, portal hypertension or after pulmonary embolism and additional exertion dyspnea, an active screening for PH is recommended. Patients with severe lung diseases and echocardiographic signs of severe PH should be referred to a PH expert center.

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Literatur

  1. Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M (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

    Article  Google Scholar 

  2. Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30:104–109

    Article  CAS  PubMed  Google Scholar 

  3. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Koerner SK (1987) Primary pulmonary hypertension. A national prospective study. Ann Intern Med 107:216–223

    Article  CAS  PubMed  Google Scholar 

  4. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, Yaici A, Weitzenblum E, Cordier JF, Chabot F, Dromer C, Pison C, Reynaud-Gaubert M, Haloun A, Laurent M, Hachulla E, Simonneau G (2006) Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 173:1023–1030

    Article  PubMed  Google Scholar 

  5. Kovacs G, Avian A, Foris V, Tscherner M, Kqiku X, Douschan P, Bachmaier G, Olschewski A, Matucci-Cerinic M, Olschewski H (2016) Use of ECG and other simple non-invasive tools to assess pulmonary hypertension. PLOS ONE 11:e0168706

    Article  PubMed  PubMed Central  Google Scholar 

  6. Kovacs G, Dumitrescu D, Barner A, Greiner S, Grunig E, Hager A, Kohler T, Kozlik-Feldmann R, Kruck I, Lammers A, Mereles D, Meyer A, Meyer FJ, Pabst S, Seyfarth HJ, Sinning C, Sorichter S, Stahler G, Wilkens H, Held M (2016) Clinical classification and initial diagnosis of pulmonary hypertension: recommendations of the Cologne Consensus Conference. Dtsch Med Wochenschr 141:S10–S18

    Article  CAS  PubMed  Google Scholar 

  7. Truong QA, Massaro JM, Rogers IS, Mahabadi AA, Kriegel MF, Fox CS, O’Donnell CJ, Hoffmann U (2012) Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: the Framingham Heart Study. Circ Cardiovasc Imaging 5:147–154

    Article  PubMed  Google Scholar 

  8. Dornia C, Lange TJ, Behrens G, Stiefel J, Muller-Wille R, Poschenrieder F, Pfeifer M, Leitzmann M, Manos D, Babar JL, Stroszczynski C, Hamer OW (2012) Multidetector computed tomography for detection and characterization of pulmonary hypertension in consideration of WHO classification. J Comput Assist Tomogr 36:175–180

    Article  PubMed  Google Scholar 

  9. Pienn M, Kovacs G, Tscherner M, Avian A, Johnson TR, Kullnig P, Stollberger R, Olschewski A, Olschewski H, Balint Z (2014) Non-invasive determination of pulmonary hypertension with dynamic contrast-enhanced computed tomography: a pilot study. Eur Radiol 24:668–676

    Article  PubMed  Google Scholar 

  10. Kovacs G, Reiter G, Reiter U, Rienmuller R, Peacock A, Olschewski H (2008) The emerging role of magnetic resonance imaging in the diagnosis and management of pulmonary hypertension. Respiration 76:458–470

    Article  PubMed  Google Scholar 

  11. Reiter G, Reiter U, Kovacs G, Kainz B, Schmidt K, Maier R, Olschewski H, Rienmueller R (2008) Magnetic resonance-derived 3‑dimensional blood flow patterns in the main pulmonary artery as a marker of pulmonary hypertension and a measure of elevated mean pulmonary arterial pressure. Circ Cardiovasc Imaging 1:23–30

    Article  PubMed  Google Scholar 

  12. Reiter G, Reiter U, Kovacs G, Olschewski H, Fuchsjager M (2015) Blood flow vortices along the main pulmonary artery measured with MR imaging for diagnosis of pulmonary hypertension. Radiology 275:71. doi:10.1148/radiol.14140849

    Article  PubMed  Google Scholar 

  13. Yasunobu Y, Oudiz RJ, Sun XG, Hansen JE, Wasserman K (2005) End-tidal PCO2 abnormality and exercise limitation in patients with primary pulmonary hypertension. Chest 127:1637–1646

    Article  PubMed  Google Scholar 

  14. Dumitrescu D, Oudiz RJ, Karpouzas G, Hovanesyan A, Jayasinghe A, Hansen JE, Rosenkranz S, Wasserman K (2010) Developing pulmonary vasculopathy in systemic sclerosis, detected with non-invasive cardiopulmonary exercise testing. PLOS ONE 5:e14293

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Dumitrescu D, Nagel C, Kovacs G, Bollmann T, Halank M, Winkler J, Hellmich M, Grunig E, Olschewski H, Ewert R, Rosenkranz S (2017) Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis. Heart. doi:10.1136/heartjnl-2016-309981

    Google Scholar 

  16. Held M, Grun M, Holl R, Hubner G, Kaiser R, Karl S, Kolb M, Schafers HJ, Wilkens H, Jany B (2014) Cardiopulmonary exercise testing to detect chronic thromboembolic pulmonary hypertension in patients with normal echocardiography. Respiration 87:379–387

    Article  PubMed  Google Scholar 

  17. Leuchte HH, Holzapfel M, Baumgartner RA, Ding I, Neurohr C, Vogeser M, Kolbe T, Schwaiblmair M, Behr J (2004) Clinical significance of brain natriuretic peptide in primary pulmonary hypertension. J Am Coll Cardiol 43:764–770

    Article  CAS  PubMed  Google Scholar 

  18. Leuchte HH, Holzapfel M, Baumgartner RA, Neurohr C, Vogeser M, Behr J (2005) Characterization of brain natriuretic peptide in long-term follow-up of pulmonary arterial hypertension. Chest 128:2368–2374

    Article  CAS  PubMed  Google Scholar 

  19. Thakkar V, Stevens W, Prior D, Youssef P, Liew D, Gabbay E, Roddy J, Walker JG, Zochling J, Sahhar J, Nash P, Lester S, Rischmueller M, Proudman SM, Nikpour M (2013) The inclusion of N‑terminal pro-brain natriuretic peptide in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension: a cohort study. Arthritis Res Ther 15:R193

    Article  PubMed  PubMed Central  Google Scholar 

  20. Coghlan JG, Denton CP, Grunig E, Bonderman D, Distler O, Khanna D, Muller-Ladner U, Pope JE, Vonk MC, Doelberg M, Chadha-Boreham H, Heinzl H, Rosenberg DM, McLaughlin VV, Seibold JR, DETECT study group (2014) Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 73:1340–1349

    Article  PubMed  Google Scholar 

  21. Bonderman D, Wexberg P, Martischnig AM, Heinzl H, Lang MB, Sadushi R, Skoro-Sajer N, Lang IM (2011) A noninvasive algorithm to exclude pre-capillary pulmonary hypertension. Eur Respir J 37:1096–1103

    Article  CAS  PubMed  Google Scholar 

  22. Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, Kahan A, Cabane J, Frances C, Launay D, Mouthon L, Allanore Y, Tiev KP, Clerson P, de Groote P, Humbert M (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800

    Article  PubMed  Google Scholar 

  23. Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J, Launay D, Mouthon L, Jego P, Cabane J, de Groote P, Chabrol A, Lazareth I, Guillevin L, Clerson P, Humbert M (2009) Risk factors for death and the 3‑year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology (Oxford) 48:304–308

    Article  Google Scholar 

  24. Khanna D, Gladue H, Channick R, Chung L, Distler O, Furst DE, Hachulla E, Humbert M, Langleben D, Mathai SC, Saggar R, Visovatti S, Altorok N, Townsend W, FitzGerald J, McLaughlin VV, Scleroderma Foundation and Pulmonary Hypertension Association (2013) Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension. Arthritis Rheum 65:3194–3201

    Article  PubMed  Google Scholar 

  25. Avouac J, Airo P, Meune C, Beretta L, Dieude P, Caramaschi P, Tiev K, Cappelli S, Diot E, Vacca A, Cracowski JL, Sibilia J, Kahan A, Matucci-Cerinic M, Allanore Y (2010) Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 37:2290–2298

    Article  PubMed  Google Scholar 

  26. Hachulla E, de Groote P, Gressin V, Sibilia J, Diot E, Carpentier P, Mouthon L, Hatron PY, Jego P, Allanore Y, Tiev KP, Agard C, Cosnes A, Cirstea D, Constans J, Farge D, Viallard JF, Harle JR, Patat F, Imbert B, Kahan A, Cabane J, Clerson P, Guillevin L, Humbert M (2009) The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 60:1831–1839

    Article  PubMed  Google Scholar 

  27. Nihtyanova SI, Schreiber BE, Ong VH, Rosenberg D, Moinzadeh P, Coghlan JG, Wells AU, Denton CP (2014) Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 66:1625–1635

    Article  PubMed  Google Scholar 

  28. Meune C, Avouac J, Airo P, Beretta L, Dieude P, Wahbi K, Caramaschi P, Tiev K, Cappelli S, Diot E, Vacca A, Cracowski JL, Sibilia J, Kahan A, Matucci-Cerinic M, Allanore Y (2011) Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations. Arthritis Rheum 63:2790–2796

    Article  PubMed  Google Scholar 

  29. Codullo V, Caporali R, Cuomo G, Ghio S, D’Alto M, Fusetti C, Borgogno E, Montecucco C, Valentini G (2013) Stress doppler echocardiography in systemic sclerosis: evidence for a role in the prediction of pulmonary hypertension. Arthritis Rheum 65:2403–2411

    Article  PubMed  Google Scholar 

  30. Reichenberger F, Voswinckel R, Schulz R, Mensch O, Ghofrani HA, Olschewski H, Seeger W (2009) Noninvasive detection of early pulmonary vascular dysfunction in scleroderma. Respir Med 103:1713–1718

    Article  CAS  PubMed  Google Scholar 

  31. Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Muller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumanovics G, Coleiro B, Feierl E, Szucs G, Von Muhlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kotter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69:1809–1815

    Article  PubMed  Google Scholar 

  32. Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB, Miller DP, Nicolls MR, Zamanian RT (2010) Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 138:1383–1394

    Article  PubMed  PubMed Central  Google Scholar 

  33. Chung L, Farber HW, Benza R, Miller DP, Parsons L, Hassoun PM, McGoon M, Nicolls MR, Zamanian RT (2014) Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. Chest 146:1494–1504

    Article  PubMed  PubMed Central  Google Scholar 

  34. Ruiz-Cano MJ, Escribano P, Alonso R, Delgado J, Carreira P, Velazquez T, Sanchez MA, de la Saenz Calzada C (2009) Comparison of baseline characteristics and survival between patients with idiopathic and connective tissue disease-related pulmonary arterial hypertension. J Heart Lung Transplant 28:621–627

    Article  PubMed  Google Scholar 

  35. Humbert M, Yaici A, de Groote P, Montani D, Sitbon O, Launay D, Gressin V, Guillevin L, Clerson P, Simonneau G, Hachulla E (2011) Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63:3522–3530

    Article  PubMed  Google Scholar 

  36. Allanore Y, Borderie D, Avouac J, Zerkak D, Meune C, Hachulla E, Mouthon L, Guillevin L, Meyer O, Ekindjian OG, Weber S, Kahan A (2008) High N‑terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum 58:284–291

    Article  CAS  PubMed  Google Scholar 

  37. Hsu VM, Chung L, Hummers LK, Wigley F, Simms R, Bolster M, Silver R, Fischer A, Hinchcliff ME, Varga J, Goldberg AZ, Derk CT, Schiopu E, Khanna D, Shapiro LS, Domsic RT, Medsger T, Mayes MD, Furst D, Csuka ME, Molitor JA, Alkassab F, Steen VD (2014) Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. Semin Arthritis Rheum 44:55–62

    Article  PubMed  Google Scholar 

  38. Kusunose K, Yamada H, Hotchi J, Bando M, Nishio S, Hirata Y, Ise T, Yamaguchi K, Yagi S, Soeki T, Wakatsuki T, Kishi J, Sata M (2015) Prediction of future overt pulmonary hypertension by 6‑min walk stress echocardiography in patients with connective tissue disease. J Am Coll Cardiol 66:376–384

    Article  PubMed  Google Scholar 

  39. Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, Das C, Elliot CA, Johnson M, DeSoyza J, Torpy C, Goldsmith K, Hodgkins D, Hughes RJ, Pepke-Zaba J, Coghlan JG (2009) Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 179:151–157

    Article  PubMed  Google Scholar 

  40. Nagel C, Henn P, Ehlken N, D’Andrea A, Blank N, Bossone E, Bottger A, Fiehn C, Fischer C, Lorenz HM, Stockl F, Grunig E, Egenlauf B (2015) Stress doppler echocardiography for early detection of systemic sclerosis-associated pulmonary arterial hypertension. Arthritis Res Ther 17(165-015):0673–0677

    Google Scholar 

  41. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB (2010) Guidelines for the echocardiographic 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 (quiz 786–788)

    Article  PubMed  Google Scholar 

  42. Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P (2004) Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350:2257–2264

    Article  CAS  PubMed  Google Scholar 

  43. Wilkens H, Konstantinides S, Lang I, Bunck AC, Gerges M, Gerhardt F, Grgic A, Grohe C, Guth S, Held M, Hinrichs J, Hoeper MM, Klepetko W, Kramm T, Kruger U, Lankeit M, Meyer BC, Olsson KM, Schafers HJ, Schmidt M, Seyfarth HJ, Ulrich S, Wiedenroth CB, Mayer E (2016) Chronic thromboembolic pulmonary hypertension: recommendations of the Cologne Consensus Conference. Dtsch Med Wochenschr 141:S62–S69

    Article  CAS  PubMed  Google Scholar 

  44. Held M, Hesse A, Gott F, Holl R, Hubner G, Kolb P, Langen HJ, Romen T, Walter F, Schafers HJ, Wilkens H, Jany B (2014) A symptom-related monitoring program following pulmonary embolism for the early detection of CTEPH: a prospective observational registry study. BMC Pulm Med:. doi:10.1186/1471-2466-14-141

  45. Olschewski H, Behr J, Bremer H, Claussen M, Douschan P, Halank M, Held M, Hoeper MM, Holt S, Klose H, Kruger S, Lange TJ, Reichenberger F, Skowasch D, Ulrich S, Wilkens H, Seeger W (2016) Pulmonary hypertension due to chronic lung disease: Recommendations of the Cologne Consensus Conference. Dtsch Med Wochenschr 141(2016):S57–S61

    CAS  PubMed  Google Scholar 

  46. Chaouat A, Bugnet AS, Kadaoui N, Schott R, Enache I, Ducolone A, Ehrhart M, Kessler R, Weitzenblum E (2005) Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 172:189–194

    Article  PubMed  Google Scholar 

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Kovacs, G., Olschewski, H. Screening auf pulmonale Hypertonie. Pneumologe 14, 153–159 (2017). https://doi.org/10.1007/s10405-017-0105-2

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