Diagnostikalgorithmus der pulmonalen Hypertonie

Zusammenfassung

Für die Diagnose der pulmonalen Hypertonie (PH) wird ein schrittweiser diagnostischer Algorithmus empfohlen, um die Patient*innen, die an ein PH-Zentrum überwiesen werden müssen und eine invasive hämodynamische Bewertung benötigen, zu identifizieren. Bei hoher Wahrscheinlichkeit für eine pulmonalarterielle Hypertonie oder chronisch thromboembolische PH wird eine schnelle Überweisung an ein PH-Zentrum zu jedem Zeitpunkt während der diagnostischen Abklärung empfohlen. Auch Patienten mit interstitieller Lungenerkrankung und Verdacht auf PH sowie Patienten mit pulmonalen Erkrankungen oder Erkrankungen des linken Herzens und Verdacht auf schwere PH sollten an ein PH-Expertenzentrum überwiesen werden.

Abstract

For the diagnosis of pulmonary hypertension (PH), a stepwise diagnostic algorithm is recommended to identify patients who require referral to a PH center and who require invasive hemodynamic assessment. In cases of high probability of pulmonary arterial hypertension or chronic thrombo-embolic PH, prompt referral to a PH center is recommended at any time during the diagnostic workup. Patients with interstitial lung disease and suspected PH, as well as patients with pulmonary disease or left heart disease and suspected severe PH, should also be referred to a PH expert center.

Schlüsselwörter

pulmonale Hypertonie - Echokardiografie - Diagnostik - Rechtsherzkatheter

Keywords

pulmonary hypertension - echocardiography - diagnostics - right heart catherization

Publication History

Article published online:
06 October 2025

• Literatur

• 1 Harbaum L, Fuge J, Kamp JC. et al. Blood carbon dioxide tension and risk in pulmonary arterial hypertension. Int J Cardiol 2020; 318: 131-137
• 2 Meyer FJ. Peripheral airway obstruction in primary pulmonary hypertension. Thorax 2002; 57: 473-476
• 3 Humbert M, Kovacs G, Hoeper MM. et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2023; 61: 2200879
• 4 He J, Fang W, Lv B. et al. Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography. Nucl Med Commun 2012; 33: 459-463
• 5 Mukherjee M, Rudski LG, Addetia K. et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults and Special Considerations in Pulmonary Hypertension: Recommendations from the American Society of Echocardiography. J Am Soc Echocardiogr 2025; 38: 141-186
• 6 Jankowich M, Maron BA, Choudhary G. Mildly elevated pulmonary artery systolic pressure on echocardiography: bridging the gap in current guidelines. Lancet Respir Med 2021; 9: 1185-1191
• 7 Held M, Linke M, Jany B. Echokardiographie und Rechtsherzkatheterisierung bei pulmonaler Hypertonie. Dtsch Med Wochenschr 2014; 139: 1511-1517
• 8 Kim BS, Heo R, Shin J. et al. E/E′ and D-shaped Left Ventricle Severity in Patients with Increased Pulmonary Artery Pressure. J Cardiovasc Imaging 2018; 26: 85
• 9 Nabeshima Y, Kitano T, Node K. et al. Prognostic value of right ventricular free-wall longitudinal strain in patients with pulmonary hypertension: systematic review and meta-analyses. Open Heart 2024; 11: e002561
• 10 Hosseinsabet A, Mahmoudian R, Jalali A. et al. Normal Ranges of Right Atrial Strain and Strain Rate by Two-Dimensional Speckle-Tracking Echocardiography: A Systematic Review and Meta-Analysis. Front Cardiovasc Med 2021; 8: 771647
• 11 Hasselberg NE, Kagiyama N, Soyama Y. et al. The Prognostic Value of Right Atrial Strain Imaging in Patients with Precapillary Pulmonary Hypertension. J Am Soc Echocardiogr 2021; 34: 851-861.e1
• 12 Brida M, Chessa M, Celermajer D. et al. Atrial septal defect in adulthood: a new paradigm for congenital heart disease. Eur Heart J 2022; 43: 2660-2671
• 13 Silvestry FE, Cohen MS, Armsby LB. et al. Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. J Am Soc Echocardiogr 2015; 28: 910-958
• 14 Khalil M. Herz und Gefäße. In: Speer CP, Gahr M, Dötsch J. Hrsg. Pädiatrie. Berlin, Heidelberg: Springer; 2019: 453-483
• 15 Engelfriet P, Tijssen J, Kaemmerer H. et al. Adherence to guidelines in the clinical care for adults with congenital heart disease: The Euro Heart Survey on Adult Congenital Heart Disease. Eur Heart J 2006; 27: 737-745
• 16 Gabriels C, De Meester P, Pasquet A. et al. A different view on predictors of pulmonary hypertension in secundum atrial septal defect. Int J Cardiol 2014; 176: 833-840
• 17 Duffels MGJ, Engelfriet PM, Berger RMF. et al. Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry. Int J Cardiol 2007; 120: 198-204
• 18 Hoeper MM, Oerke B, Wissmüller M. et al. Tadalafil for Treatment of Combined Postcapillary and Precapillary Pulmonary Hypertension in Patients With Heart Failure and Preserved Ejection Fraction: A Randomized Controlled Phase 3 Study. Circulation 2024; 150: 600-610
• 19 Bermejo J, Yotti R, García-Orta R. et al. Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial. Eur Heart J 2018; 39: 1255-1264
• 20 Vitulo P, Stanziola A, Confalonieri M. et al. Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A randomized controlled multicenter clinical trial. J Heart Lung Transplant 2017; 36: 166-174
• 21 Elkhapery A, Hammami MB, Sulica R. et al. Pulmonary Vasodilator Therapy in Severe Pulmonary Hypertension Due to Chronic Obstructive Pulmonary Disease (Severe PH-COPD): A Systematic Review and Meta-Analysis. JCDD 2023; 10: 498
• 22 Nathan SD, Behr J, Collard HR. et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med 2019; 7: 780-790
• 23 Held M, Weiner S, Walthelm J. et al. Funktionelle Charakterisierung von Patienten mit isoliert postkapillärer oder kombiniert post- und präkapillärer pulmonaler Hypertonie. Dtsch Med Wochenschr 2021; 146: e88-e94
• 24 Chebib N, Mornex J, Traclet J. et al. Pulmonary hypertension in chronic lung diseases: comparison to other pulmonary hypertension groups. Pulm Circ 2018; 8: 1-10
• 25 Held M, Grün M, Holl R. et al. Cardiopulmonary Exercise Testing to Detect Chronic Thromboembolic Pulmonary Hypertension in Patients with Normal Echocardiography. Respiration 2014; 87: 379-387
• 26 Yasunobu Y, Oudiz RJ, Sun X-G. et al. End-tidal Pco 2 Abnormality and Exercise Limitation in Patients With Primary Pulmonary Hypertension. Chest 2005; 127: 1637-1646
• 27 Dumitrescu D, Nagel C, Kovacs G. et al. Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis. Heart 2017; 103: 774-782
• 28 Oswald-Mammosser M, Albert RK, Weitzenblum E. et al. AaDO2 as a predictor of pulmonary hypertension resulting from pulmonary emboli. Cor Vasa 1991; 33: 235-243
• 29 Kovacs G, Avian A, Olschewski A. et al. Zero reference level for right heart catheterisation. Eur Respir J 2013; 42: 1586-1594
• 30 Hoeper MM, Bogaard HJ, Condliffe R. et al. Definitions and Diagnosis of Pulmonary Hypertension. J Am Coll Cardiol 2013; 62: D42-D50
• 31 Kovacs G, Avian A, Pienn M. et al. Reading Pulmonary Vascular Pressure Tracings. How to Handle the Problems of Zero Leveling and Respiratory Swings. Am J Respir Crit Care Med 2014; 190: 252-257
• 32 Kovacs G, Herve P, Barbera JA. et al. An official European Respiratory Society statement: pulmonary haemodynamics during exercise. Eur Respir J 2017; 50: 1700578
• 33 Sitbon O, Humbert M, Jaïs X. et al. Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension. Circulation 2005; 111: 3105-3111
• 34 Reddy YNV, Kaye DM, Handoko ML. et al. Diagnosis of Heart Failure With Preserved Ejection Fraction Among Patients With Unexplained Dyspnea. JAMA Cardiol 2022; 7: 891
• 35 Eisman AS, Shah RV, Dhakal BP. et al. Pulmonary Capillary Wedge Pressure Patterns During Exercise Predict Exercise Capacity and Incident Heart Failure. Circ Heart Fail 2018; 11: e004750
• 36 D’Alto M, Romeo E, Argiento P. et al. Clinical Relevance of Fluid Challenge in Patients Evaluated for Pulmonary Hypertension. Chest 2017; 151: 119-126
• 37 Montani D, Girerd B, Jaïs X. et al. Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation. Eur Respir J 2021; 58: 2004229
• 38 Coghlan JG, Denton CP, Grünig E. et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014; 73: 1340-1349