FIGURE 1

Diagnostic algorithm for pulmonary hypertension (PH) in children. CHD: congenital heart disease; PFT: pulmonary function test; D_LCO: diffusing capacity of the lung for carbon monoxide; CT: computed tomography; RV: right ventricular; V/Q: ventilation/perfusion; CTEPH: chronic thromboembolic PH; CTA: CT angiography; PA: pulmonary artery; PEA: pulmonary endarterectomy; AVT: acute vasodilator testing; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVRI: pulmonary vascular resistance index; WU: Wood Units; 6MWT: 6-min walk test; CPET: cardiopulmonary exercise test; MRI: magnetic resonance imaging; CTD: connective tissue disease; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; IPAH/FPAH: idiopathic/familial pulmonary arterial hypertension.