Step 1 - Basic evaluation

The basic investigation should include, in addition to the assessment of vital signs, a thorough medical history and careful clinical examination. A broad differential diagnosis is required to identify or rule out the most common causes of dyspnea. About two-thirds of cases of chronic dyspnea are caused by heart or lung diseases.

Step 2 - Targeted evaluation

Based on the overall clinical question and the findings from Step 1, targeted examinations are performed. If a diagnosis cannot be established after investigations according to Steps 1 and 2, early contact should be made with a specialist center.

Step 3 - Specialist center for PH

At this stage, the patient undergoes further evaluation to diagnose and treat PAH and CTEPH. All follow-up and medication adjustments are managed through the specialist center.

* Disease specific biomarkers, including sweat testing for cystic fibrosis, genetic testing, tissue biopsies, and the potential use of emerging and validated cardiopulmonary biomarkers of dyspnea.
** Fast-track referral as suggested by the 2022 ESC/ERS PH guidelines algorithm in case of high suspicion/likelihood of PAH or CTEPH.
a In cases of suspected PAH/CTEPH or unclear etiology after Steps 1 and 2.
bCPET is not performed routinely but can provide complementary information to the 6-minute walk test.
cShunt evaluation - stepwise measurement of O2 saturation.
d Performed in the presence of signs of precapillary pulmonary hypertension after right heart catheterization, as well as imaging findings indicating chronic pulmonary embolism.
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  2. Ahmed S., Ahmed A., Rådegran G. Structured evaluation of unclear dyspnea - An attempt to shorten the diagnostic delay in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Pulm Circ. 2024;14:e12340.
  3. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Ghofrani HA, Giannakoulas G, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Rådegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S, Group EESD. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by 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 for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). European Heart Journal. 2022;43(38):3618-3731.