Who should be referred to a specialist pulmonary -hypertension centre - a referrer's guide

Abstract

The introduction of pulmonary hypertension (PH)-specific drugs has allowed certain forms of PH to become more treatable. However, patients with these diseases can present to a number of medical specialties and can be challenging to identify, particularly in a non-specialist setting. This article provides guidance on who should be investigated and referred on to a specialist centre, highlighting the potential pitfalls during assessment.

Keywords: Pulmonary hypertension; assessment; chronic thromboembolic pulmonary hypertension; pulmonary arterial hypertension; screening.

Fig 1.

PAH prevalence across the UK. The numbers in parentheses show the number of local area teams which fall within the range. PAH = pulmonary arterial hypertension. Reproduced with permission of the Health and Social Care Information Centre.

Fig 2.

Non-specific signs of PH on CTPA. (a) Enlarged main pulmonary artery; (b) enlarged right heart; (c) hepatic reflux of contrast into IVC and hepatic veins. CTPA = computed tomography pulmonary angiography; IVC = inferior vena cava; PH = pulmonary hypertension.

Fig 3.

Specific signs of PH on CTPA. (a) Atrial septal defect; (b and c) proximal laminated thrombus in CTEPH; (d) webs in CTEPH; (e) occlusion in middle lobe and web in lingular; (f) mosaicism. CTEPH = chronic thromboembolic pulmonary hypertension; CTPA = computed tomography pulmonary angiography; PH = pulmonary hypertension.

Fig 4.

Referral algorithm. AF = atrial fibrillation; APLS = anti-phospholipid syndrome; AV = aortic valve; BMI = body mass index; CT = computed tomography; CTD = connective tissue disease; CTEPH = chronic thromboembolic pulmonary hypertension; CTPA = computed tomography pulmonary angiography; CXR = chest radiograph; DL_CO = diffusing capacity of the lung for carbon monoxide; DM = diabetes mellitus; ECG = electrocardiogram; FEV₁ = forced expiratory volume in 1 second; FVC = forced vital capacity; HIV = human immunodeficiency virus; HRCT = high resolution computed tomography; HTN = hypertension; IHD = ischemic heart disease; Ix = investigations; LA = left atrium; LAD = left anterior descending coronary artery; LVH = left ventricular hypertrophy; MV = mitral valve; NT-proBNP = N-terminal pro-brain natriuretic peptide; PE = pulmonary embolism; PH = pulmonary hypertension; PPM = pacemaker; PVOD = pulmonary veno-occlusive disease; SSc = systemic sclerosis; VA = ventriculoatrial; VQ = ventilation perfusion.