Definition and classification
1. Pulmonary arterial hypertension (PAH)
| 1.1 Idiopathic |
1.2 Heritable | |
1.3 Drugs and toxins induced | |
1.4 Associated with (APAH): | |
1.4.1 Connective tissue disease | |
1.4.2 Infection with human immunodeficiency virus | |
1.4.3 Portal hypertension | |
1.4.4 Congenital heart disease | |
1.4.5 Schistosomiasis | |
1.4.6 Chronic haemolytic anaemia | |
1.5 Persistent pulmonary hypertension of the newborn | |
2. Pulmonary hypertension with left heart disease
| 2.1 Systolic dysfunction |
2.2 Diastolic dysfunction | |
2.3 Valvular disease | |
3. Pulmonary hypertension due to lung diseases and/or hypoxia
| 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 abnormalities | |
4. Chronic thromboembolic pulmonary hypertension
| |
5. PH with unclear and/or multifactorial mechanisms
| 5.1 Haematological disorders: myeloproliferative disorders, splenectomy. |
5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis | |
5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders | |
5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis |
Group 1: Pulmonary arterial hypertension
Group 1.1/1.2 Idiopathic and heritable PAH
Group 1.3 Drug- and toxin-induced PAH
1. Definite | |
Aminorex | |
Fenfluramine | |
Dexfenfluramine | |
Toxic rapeseed oil | |
Benfluorex | |
2. Likely | |
Amphetamines | |
L-tryptophan | |
Metamphetamines | |
Dasatinib | |
3. Possible | |
Cocaine | |
Phenylpropanolamine | |
St John’s Wort | |
Chemotherapeutic agents | |
Selective serotonin reuptake inhibitors | |
Pergolide | |
4. Unlikely | |
Oral contraceptives | |
Estrogen therapy | |
Cigarette smoking |
Group 1.4.1 PAH associated with connective tissue diseases
Group 1.4.2 HIV infection
Group 1.4.3 Porto-pulmonary hypertension
Group 1.4.4 Congenital heart diseases
Group 1.4.5 Schistosomiasis
Group 1.4.6 Chronic hemolytic anemia
Group 1’: Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
Group 2: Pulmonary hypertension due to left heart disease
Group 3: Pulmonary hypertension due to lung diseases and/or hypoxia
Group 4: Chronic thromboembolic pulmonary hypertension
Group 5: PH with unclear or multifactorial etiologies
Group 5.1 Hematologic disorders
Group 5.2 Systemic disorders
Group 5.3 Metabolic disorders
Group 5.4 Miscellaneous conditions
Epidemiology
Genetics
Pathophysiology
Cellular factors
Autoimmunity and PAH
Molecular factors
Histopathology: vascular changes
Arterial lesions
Isolated medial hypertrophy
Concentric and eccentric non-laminar intimal fibrosis
Concentric laminar intimal fibrosis
Complex lesions
Venous and venular lesions (Pulmonary veno-occlusive disease and pulmonary hemangiomatosis)
Clinical description
Symptoms and clinical signs of PH
CLASS I | Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain, or near syncope. |
CLASS II | Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain, or near syncope. |
CLASS III | Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain, or near syncope. |
CLASS IV | Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. |