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. 2023 Feb 23;61(2):2201471. doi: 10.1183/13993003.01471-2022

FIGURE 2.

FIGURE 2

Genetic counselling path for pulmonary arterial hypertension (PAH) patients and their relatives. Patients should receive genetic counselling before and after genetic testing. If a (likely) pathogenic variant is identified, family testing should be encouraged. If asymptomatic carriers are identified they should undergo regular clinical follow-up. IPAH: idiopathic PAH; FPAH: familial PAH; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; CHD APAH: congenital heart disease-associated PAH; CPET: cardiopulmonary exercise testing; TTE: transthoracic echocardiography.