Post-capillary Pulmonary Hypertension in ACHD

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure at rest ⩾ 25 mmHg as assessed by right heart catheterization. Haemodynamically PH can be further distinguished into pre-capillary PH depending on the presence of a raised left atrial or pulmonary wedge pressure. Post-capillary PH is common in patients with congenital heart disease, in whom systemic ventricular dysfunction, valve disease, outflow tract obstruction or pulmonary venous stenosis can lead to a rise in post-capillary pressures. In this chapter, we discuss the pathogenesis, impact and management of postcapillary PH in congenital heart disease.