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. 2023 Sep;23(5):449–454. doi: 10.7861/clinmed.2023-23.5.Cardio4

Table 1.

Clinical classification of PH3

Group 1: Pulmonary arterial hypertension (PAH)
1.1 Idiopathic

  • 1.1.1 Non-responders at vasoreactivity testing

  • 1.1.2 Acute responders at vasoreactivity testing

1.2 Heritable
1.3 Associated with drugs and toxins
1.4 Associated with:

  • 1.4.1 Connective tissue disease

  • 1.4.2 HIV infection

  • 1.4.3 Portal hypertension

  • 1.4.4 Congenital heart disease

  • 1.4.5 Schistosomiasis

1.5 PAH with features of venous/capillary (PVOD/PCH) involvement
1.6 Persistent PH of the newborn
Group 2: PH associated with left heart disease
2.1 Heart failure:

  • 2.1.1 with preserved ejection fraction

  • 2.1.2 with reduced (≤40%) or mildly reduced (41–49%) left ventricular ejection fraction

2.2 Valvular heart disease
2.3 Congenital/acquired cardiovascular conditions leading to post-capillary PH
Group 3: PH associated with lung diseases and/or hypoxia
3.1 Obstructive lung disease or emphysema
3.2 Restrictive lung disease
3.3 Lung disease with mixed restrictive/obstructive pattern
3.4 Hypoventilation syndromes
3.5 Hypoxia without lung disease (eg high altitude)
3.6 Developmental lung disorders
Group 4: PH associated with pulmonary artery obstructions
4.1 Chronic thrombo-embolic PH
4.2 Other pulmonary artery obstructions (including sarcomas, other malignant or non-malignant tumours, arteritis without connective tissue disease, congenital pulmonary arterial stenoses, and hydatidosis)
Group 5: PH with unclear and/or multifactorial mechanisms (discussion with +/− referral to PH centres should be considered)
5.1 Haematological disorders (including inherited and acquired chronic haemolytic anaemia and chronic myeloproliferative disorders)
5.2 Systemic disorders (including sarcoidosis, pulmonary Langerhans's cell histiocytosis, and neurofibromatosis type 1)
5.3 Metabolic disorders (including glycogen storage diseases and Gaucher's disease)
5.4 Chronic renal failure with or without haemodialysis
5.5 Pulmonary tumour thrombotic microangiopathy
5.6 Fibrosing mediastinitis

HIV = human immunodeficiency virus; PAH = pulmonary arterial hypertension; PCH = pulmonary capillary haemangiomatosis; PH = pulmonary hypertension; PVOD = pulmonary veno-occlusive disease.