Definition of PH
Pulmonary hypertension (PH) is pathophysiologic consequence of vasculopathic changes in the pulmonary vessels that may occur as a result of predisposing factors. Pulmonary arterial hypertension (PAH) is characterised by the presence of precapillary PH in absence of other causes of precapillary PH and of postcapillary (=venous) PH.
PAH is defined by specific hemodynamic criteria obtained via right heart catheterization (RHC), including a mean pulmonary arterial pressure (mPAP) >25 mm Hg, a normal or reduced cardiac output, and a postcapillary pressure <15 mmHg.
The upper limit of normal mPAP is 20 mmHg. The significance of a mPAP between 21 and 24 mmHg is unclear. The definition of PH on exercise as mPAP >30 mmHg as assessed by RHC is not supported by published data, and healthy individuals can reach much higher values. Thus no definition for PH on exercise as assessed by RHC can be provided at the present time.
Important definitions
PH is a haemodynamic and pathophysiological condition defined as an increase in Ppa ≥25 mmHg at rest as assessed by right heart catheterisation. PH can be found in multiple clinical conditions.
The definition of PH on exercise as Ppa >30 mmHg as assessed by right heart catheterisation is not supported by published data.
PAH (group 1) is a clinical condition charecterised by the presence of pre-capillary PH in the absence of other causes of pre-capillary PH such as PH due to lung dieseases, chronic thromboembolic PH, or other rare diseases. PAH includes different forms that share a similar clinical picture and virtually identical pathological changes of the lung microcirculation.
PH: pulmonary hypertension; Ppa: mean pulmonary arterial pressure; PAH: pulmonary arterial hypertension.
Haemodynamic definitions of pulmonary hypertension
| Definition | Characteristics | Clinical groups |
| Pre-capillary PH |
mPAP >20 mmHg |
1,3,4 and 5 |
| Isolated post-capillary PH (IpcPH) | mPAP >20 mmHg PAWP >15mmHg PVR <3WU |
2 and 5 |
| Combined pre- and post-capillary PH (CpcPH) | mPAP >20 mmHg PAWP >15mmHg PVR ≥3WU |
2 and 5 |
mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; WU: Wood Units. #: group 1: PAH; group 2: PH due to left heart disease; group 3: PH due to lung diseases and/or hypoxia; group 4: PH due to pulmonary artery obstructions; group 5: PH with unclear and/or multifactorial mechanisms.