Pulmonary hypertension - the latest updates for physicians

Abstract

Pulmonary hypertension (PH) is common, with an estimated prevalence of approximately 1% that increases with age. Prompt and accurate diagnosis is key to institute timely and appropriate therapy to improve symptoms and prognosis. The international guidelines for the diagnosis and management of PH have recently been updated, with a lowering of the haemodynamic threshold for diagnosis to a mean pulmonary artery pressure >20 mmHg. New diagnostic algorithms and revised indications for screening in at-risk groups have been developed to facilitate early referral to specialist PH centres. This includes fast-track referral pathways for patients who are either clinically high-risk or are at-risk for pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). This review summarises key changes in the PH guidelines for general physicians who are, most often, the first healthcare professionals to encounter these patients and consequently have a key role as referrers into specialist PH services.

Keywords: Pulmonary hypertension; chronic thromboembolic pulmonary hypertension; pulmonary arterial hypertension; screening; systemic sclerosis.

Fig 1.

Diagnostic algorithm for patients with unexplained dyspnoea and/or suspected pulmonary hypertension. Adapted from the 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. ABG = arterial blood gas; BNP = brain natriuretic peptide; CPET = cardiopulmonary exercise testing; CT = computed tomography; CTEPH = chronic thromboembolic pulmonary hypertension; ECG = electrocardiogram; HIV = human immunodeficiency virus; N = no; NT-proBNP = N-terminal pro-brain natriuretic peptide; PAH = pulmonary arterial hypertension; PE = pulmonary embolism; PFT = pulmonary function tests; PH = pulmonary hypertension; Y = yes.

Fig 2.

When to refer patients with possible HFpEF-associated pulmonary hypertension (PH) to specialist centres for further evaluation by right-heart catheterisation (RHC). RHC is recommended in cases with an intermediate probability of HFpEF-associated PH when risk factors of pulmonary arterial hypertension / chronic thromboembolic pulmonary hypertension (PAH/CTEPH) are present and/or if there is evidence of right ventricular (RV) abnormality. If the probability of HFpEF-associated PH is high, management should focus on the underlying left heart disease (LHD). Adapted from Vachiery et al 2019. AF = atrial fibrillation; CMR = cardiovascular magnetic resonance; CPET = cardiopulmonary exercise testing; ECG = electrocardiogram; HFpEF = heart failure with preserved ejection fraction; LBBB = left bundle branch block; LVH: left ventricular hypertrophy; SSc: systemic sclerosis.