TABLE 1

World Health Organization (WHO) groups of pulmonary hypertension (PH) and cardiovascular magnetic resonance (CMR)

Group 1Group 2Group 3Group 4Group 5
PH categoryPAHPulmonary venous hypertension due to left-heart diseasePH due to lung diseasePH due to chronic thromboembolismPH due to multifactorial or miscellaneous cause
Major causesIdiopathic
Heritable
CTD
HIV
Drug/toxin induced
Shunts (CHD)
Left heart disease (left-ventricular systolic and diastolic dysfunction)
Valvular heart disease (mitral, aortic)
ILD
COPD
Sleep apnoea
Chronic thromboembolismMyeloproliferative disorders
Sarcoidosis
Glycogen storage disease
Chronic haemolytic anaemia
Thyroid disease
Chronic kidney disease
Diagnostic criteria by RHCMean PAP >20 mmHg
PAWP ≤15 mmHg
PVR ≥3 Wood units
Mean PAP >20 mmHg
PAWP >15 mmHg
PVR <3 or ≥3 Wood units
Mean PAP >20 mmHg
PAWP ≤15 mmHg
PVR ≥3 Wood units
Mean PAP >20 mmHg
PAWP ≤15 mmHg
PVR ≥3 Wood units
Presence of both precapillary and/or postcapillary PH
Mean PAP >20 mmHg; PAWP ≤15 mmHg; PVR ≥3 Wood units
or
Mean PAP >20 mmHg; PAWP >15 mmHg; PVR <3 or ≥3 Wood units
Major diagnostic findings by CMRAberrant pulmonary veins
Shunts
Left ventricular hypertrophy
Left ventricular dysfunction
Dilated left atrium
Valvular disease
Pulmonary vein stenosis
Gross fibrosis of the lung parenchymaThrombus in the pulmonary vesselLymphadenopathy
Absence of spleen
Focal LGE (sarcoidosis)
Essential diagnostic CMR modalityDedicated cardiac views
Flow mapping (phase contrast flow velocity and/or 4-D flow analysis)
MRA
Short and/or long axis views cine SSFP
LGE
Valve lesion quantification
Cine SSFP
Flow (phase contrast flow velocity or flow analysis)
MRA
MRALGE
CMR prognostic focusShort and/or long axis views cine SSFP
Right ventricular function
Right ventricle–pulmonary artery coupling

PAH: pulmonary arterial hypertension; CTD: connective tissue disorder; CHD: congenital heart disease; ILD: interstitial lung disease; COPD: chronic obstructive pulmonary disease; RHC: right-heart catheterisation; PAP: pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; LGE: late gadolinium enhancement; MRA: magnetic resonance angiography; SSFP: steady-state free procession.