Paediatric pulmonary hypertension: aetiology, pathophysiology and treatment

Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art

“…Pulmonary arterial hypertension (PAH) is a progressively deteriorative disease characterized by an increase of pulmonary vascular resistance (PVR) caused by the vascular structural remodeling of pulmonary arteries, likely causing right ventricular failure (1). Nowadays, the 6th WSPH Task Forces proposes (2) to include PVR equal to or over three Wood units in the definition of all forms of pre-capillary PH associated with mean pulmonary artery pressure (mPAP) > 20 mmHg (3). Idiopathic pulmonary arterial hypertension (IPAH) is one of the most common PAH categories in children (4).…”