TABLE 1.

Revised European Society of Cardiology and European Respiratory Society guidelines for PAH with signs of venous/capillary involvement

Recommendation in 2015 [ 40 ]	Class	Recommendations in 2022 [ 4 ]	Class
A combination of clinical findings, physical examination, bronchoscopy and radiological findings is recommended to diagnose PVOD/PCH	I	A combination of clinical and radiological findings is recommended to diagnose PAH with signs of venous and/or capillary involvement (PVOD/PCH)	I
		In patients with PVOD/PCH, the use of drugs approved for PAH may be considered with careful monitoring of clinical symptoms and gas exchange	IIb
		Lung biopsy is not recommended to confirm a diagnosis of PVOD/PCH	III

Table adapted from [4]. PAH: pulmonary arterial hypertension; PCH: pulmonary capillary haemangiomatosis; PVOD: pulmonary veno-occlusive disease.