Pulmonary Hypertension in Adult Congenital Heart Disease
Pulmonary hypertension (PH) is a haemodynamic and pathophysiological disorder associated with a variety of cardiovascular and respiratory conditions, including congenital heart disease (CHD) . PH is defned by international guidelines as an increase in mean pulmonary arterial pressure (meanPAP) ≥25 mmHg at rest, assessed by cardiac catheterization. The normal values of resting mean PAP are 14 ± 3 mmHg, with an upper limit of 20 mmHg. The signifcance of values in the “grey zone” of 21–24 mmHg is unclear, and currently, due to lack of data, there is no reliable defnition of PH occurring with exercise. A comprehensive international clinical classifcation for PH categorizes the multiple conditions in which PH may be present according to similarities in clinical presentation, pathology, haemodynamics and treatment (Table 1.1). Haemodynamically, PH can be distinguished into precapillary and post-capillary based on the absence or presence of a rise in mean left atrial/mean pulmonary wedge/left ventricular end-diastolic pressure (≤15 mmHg). Patients in group 1, pulmonary arterial hypertension (PAH), have precapillary PH with a pulmonary vascular resistance (PVR) >3 Wood units (WU) in the absence of other causes of precapillary PH such as lung disease (belonging to group 3), chronic thromboembolic PH (CTEPH group 4) or other rare diseases (group 5).