From the microscopic to the macroscopic: clinical–radiological–pathological correlation in pulmonary hypertension

Extract

Pulmonary hypertension (PH) is defined as the presence of a mean pulmonary arterial pressure >20 mmHg [1]. This simple haemodynamic definition encompasses a heterogenous collection of conditions. It is now appreciated that although treatable forms of PH are relatively rare, PH itself is not an uncommon entity, affecting ~1% of the global population [1]. Current international guidelines describe five classification groups: group 1 (pulmonary arterial hypertension (PAH)), group 2 (PH associated with left heart disease), group 3 (PH associated with lung disease), group 4 (PH associated with pulmonary arterial obstructions) and group 5 (PH with unclear and/or multifactorial causes) figure 1 [1]. These groups are characterised by shared clinical, haemodynamic and radiological features which are underpinned by common histopathological changes.