©Janssen-Cilag NV - EM-38436 - 21-jul-2020 - vu/er Luc Van Oevelen, Antwerpseweg 15-17, 2340 Beerse, ©Janssen-Cilag B.V. - EM-38436 - 21-jul-2020
The 2015 European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines classify pulmonary hypertension (PH) into five main groups, according to their similar clinical presentation, pathological findings, haemodynamic characteristics and treatment strategy.
Adapted from Galiè et al. 2016
These 5 PH groups are further subcategorised.
1.2.1 Bone morphogenetic protein receptor type 2 (BMPR2) mutation
1.2.2 Other mutations
1.3 Drug- and toxin-induced
1.4 Associated with:
1.4.1 Connective tissue disease
1.4.2 Human immunodeficiency virus (HIV) infection
1.4.3 Portal hypertension
1.4.4 Congenital heart disease
1’.2.1 Eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) mutation
1’.2.2 Other mutations
1’.3 Drug-, toxin- and radiation-induced
1’.4 Associated with:
1’.4.1 Connective tissue disease
1’.4.2 HIV infection
2.1 Left ventricular systolic dysfunction
2.2 Left ventricular diastolic dysfunction
2.3 Valvular disease
2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
2.5 Congenital/acquired pulmonary veins stenosis
3.1 Chronic obstructive pulmonary disease
3.2 Interstitial lung disease
3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
3.4 Sleep-disordered breathing
3.5 Alveolar hypoventilation disorders
3.6 Chronic exposure to high altitude
3.7 Developmental lung diseases
4.1 Chronic thromboembolic pulmonary hypertension
4.2 Other pulmonary artery obstructions
4.2.2 Other intravascular tumours
4.2.4 Congenital pulmonary arteries stenoses
4.2.5 Parasites (hydatidosis)
5.1 Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
5.2 Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis, neurofibromatosis
5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
5.4 Others: pulmonary tumoural thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without dialysis), segmental pulmonary hypertension
Clinical guidelines set out the diagnostic pathway so patients can be referred and treated as quickly as possible.
Modern treatments can significantly improve patients’ symptoms and slow the rate of clinical deterioration.
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On this page you will find interactive 3D animations of the human anatomy and various syndromes. This allows you to zoom in on the anatomy, tissue structures, disease mechanisms and the course of the disease.