The following are key points to remember from the 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines for the diagnosis and treatment of pulmonary hypertension:

1. Pulmonary hypertension (PH) is now defined by a mean pulmonary arterial pressure >20 mm Hg at rest. The definition of pulmonary arterial hypertension (PAH) also implies a pulmonary vascular resistance (PVR) >2 Wood Units and pulmonary arterial wedge pressure ≤15 mm Hg.
2. The main diagnostic algorithm for PH has been simplified following a three-step approach, from suspicion by first-line physicians, detection by echocardiography, and confirmation with right heart catheterization in PH centers.
3. An improved recognition of computed tomography (CT) and echocardiographic signs of chronic thromboembolic pulmonary hypertension (CTEPH) at the time of an acute pulmonary embolism (PE) event, together with a systematic follow-up of patients with acute PE, should help to remediate the underdiagnosis of CTEPH.
4. The treatment algorithm for PAH has been simplified, with a clear focus on risk assessment, cardiopulmonary comorbidities, and treatment goals. Initial combination therapy and treatment escalation at follow-up when appropriate are current standards.
5. The task force has attempted to close the gap between pediatric and adult PAH care, with therapeutic and follow-up strategies based on risk stratification and treatment response, extrapolated from that in adults but adapted for age.
6. The recommendations on sex-related issues in patients with PAH, including pregnancy, have been updated, with information and shared decision making as key points. Women with PH who become pregnant or present during pregnancy with newly diagnosed PAH should be treated, whenever possible, in centers with a multidisciplinary team experienced in managing PH in pregnancy. It is recommended to stop endothelin receptor antagonists, riociguat, and selexipag because of potential or unknown teratogenicity. Despite limited evidence, calcium channel blockers, phosphodiesterase 5 (PDE5) inhibitors, and inhaled/IV/subcutaneous prostacyclin analogues are considered safe during pregnancy.
7. The recommendations for rehabilitation and exercise programs in PH have been updated following the release of additional supportive evidence. Patients with PAH should be treated with the best standard of pharmacological treatment and be in a stable clinical condition before embarking on a supervised rehabilitation program.
8. For the first time, there is a recommendation for PH medical therapy in group 3 PH, based on a single positive randomized controlled trial in patients with interstitial lung disease (ILD). The therapeutic approach to group 3 PH starts with optimizing the treatment of the underlying lung disease, including supplementary oxygen and noninvasive ventilation, where indicated, as well as enrollment into pulmonary rehabilitation programs. The new recommendation is that PDE5 inhibitors may be considered in patients with severe PH associated with ILD with individual decision-making in PH centers.
9. The concept of chronic thromboembolic pulmonary disease (CTEPD) with or without PH has been introduced, enabling further research on the natural history and management in the absence of PH. CTEPD describes symptomatic patients with mismatched perfusion defects on V/Q scan and with signs of chronic, organized, fibrotic clots on computed tomography pulmonary angiography or digital subtraction angiography, such as ring-like stenoses, webs/slits, and chronic total occlusions (pouch lesions or tapered lesions), after at least 3 months of therapeutic anticoagulation.
10. The treatment algorithm for CTEPH has been modified, including multimodal therapy with surgery, PH drugs, and balloon pulmonary angioplasty. Lifelong therapeutic anticoagulation is recommended for patients with CTEPH, as recurrent pulmonary thromboembolism accompanied by insufficient clot resolution are key pathophysiological features of this disease.

Clinical Topics: Anticoagulation Management, Cardiovascular Care Team, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Pulmonary Hypertension, Interventions and Imaging, Interventions and Vascular Medicine, Angiography, Computed Tomography, Echocardiography/Ultrasound, Nuclear Imaging, Hypertension

Keywords: Angiography, Digital Subtraction, Angioplasty, Anticoagulants, Cardiac Rehabilitation, Constriction, Pathologic, Diagnostic Imaging, Echocardiography, ESC22, ESC Congress, Hypertension, Pulmonary, Lung Diseases, Interstitial, Patient Care Team, Pregnancy, Primary Prevention, Pulmonary Arterial Hypertension, Pulmonary Embolism, Risk Assessment, Tomography, X-Ray Computed, Vascular Resistance

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