The ‘Ten Commandments’ of the 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

The 10 main messages of the new guidelines for the diagnosis and treatment of pulmonary hypertension (Figure 1) are: Pulmonary hypertension (PH) is now defined by a mean pulmonary artery pressure >20 mmHg at rest, as assessed by right heart catheterization. A pre-capillary component is defined by a pulmonary vascular resistance (PVR) >2 Wood units. Exercise PH is defined by a mean pulmonary artery pressure/cardiac output slope between rest and exercise >3 mmHg/L/min. The clinical classification of PH distinguishes five main groups: (i) Pulmonary arterial hypertension (PAH); (ii) PH associated with left heart disease; (iii) PH associated with lung diseases and/or hypoxia; (iv) PH associated with pulmonary artery obstructions (mainly chronic thromboembolic PH; CTEPH); (v) PH with unclear and/or multi-factorial mechanisms. The differential diagnosis is crucial, because the therapeutic strategies for each of these five groups are fundamentally different. The diagnostic strategy aims at raising early suspicion of PH and ensuring fast-track referral to PH centres in patients with suspected PAH, CTEPH, or any other form of severe PH. It is recommended to assign an ‘echocardiographic probability’ of PH, based on the tricuspid regurgitation velocity and other echocardiographic signs of PH, including the TAPSE/sPAP ratio. Right heart catheterization—comprising a complete set of haemodynamics—is required to establish a diagnosis of PH. In patients with PAH (without cardiopulmonary comorbidities and non-vasoresponders), risk assessment utilizing a three-strata approach is recommended at the time of diagnosis. For patients presenting with low or intermediate risk, initial combination therapy with a phosphodiesterase type 5 inhibitor (PDE5i) and an endothelin receptor antagonist (ERA) is recommended. In patients at high risk, initial combination therapy with a PDE5i, an ERA, and s.c./i.v. prostacyclin analogues should be considered. During follow-up, PAH patients should be re-assessed using the refined four-strata risk assessment model. In patients at intermediate-low-risk, therapy should be intensified by adding a prostacyclin receptor agonist, or by switching from PDE5i to a soluble guanylate cyclase stimulator. For patients at intermediate-high or high risk, addition of s.c./i.v. prostacyclin analogues and evaluation for lung transplantation should be considered. In patients with ‘PAH with comorbidities’, initial monotherapy with a PDE5i or an ERA should be considered. For patients presenting at intermediate or high risk during follow-up, additional PAH medications may be considered on an individual basis. PH associated with left heart disease is sub-categorized into isolated post-capillary PH (PVR ≤2 WU), and combined post- and pre-capillary PH (PVR >2 WU). In patients with a severe pre-capillary component (e.g. PVR >5 WU), an individualized approach to treatment is recommended. For PH associated with lung diseases and/or hypoxia, ‘severe PH’ is defined by a PVR >5 WU. In such patients, an individualized approach to treatment is recommended. Inhaled treprostinil may be considered in patients with PH associated with interstitial lung disease. For patients with CTEPH, a multi-modality approach to treatment is recommended, considering pulmonary endarterectomy, balloon pulmonary angioplasty, and medical therapy. It is recommended for PH centres to provide care by a multi-disciplinary team, which collaborates with patient associations, and is involved in research, teaching, and education. ‘Ten Commandments’ for the 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. 6MWD, 6 min walking distance; BPA, pulmonary balloon angioplasty; CpcPH, combined post- and pre-capillary pulmonary hypertension; CTEPH, chronic thromboembolic pulmonary hypertension; ERA, endothelin receptor antagonist; LHD, left heart disease; ILD, interstitial lung disease; IpcPH, isolated post-capillary pulmonary hypertension; MDT, multi-disciplinary team; mPAP, mean pulmonary artery pressure; PAH, pulmonary arterial hypertension; PCA, prostacyclin analogue; PDE5i, phosphodiesterase type 5 inhibitor; PEA, pulmonary endarterectomy; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterization; WU, Wood unit.