Table 2

Classification of pulmonary hypertension (5th World Symposium on Pulmonary Hypertension, Nice 2013)5

1. Pulmonary arterial hypertension (PAH)
1.1. Idiopathic PAH
1.2. Heritable PAH1.2.1. BMPR2
1.2.2. ALK-1, ENG, SMAD9, CAV1, KCNK3
1.2.3. Unknown
1.3. Drug and toxin induced
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 diseases
1.4.5. Schistosomiasis
1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
1″ Persistent pulmonary hypertension of the newborn
2. Pulmonary hypertension due to left heart disease2.1. LV systolic dysfunction
2.2. LV diastolic dysfunction
2.3. Valvular disease
2.4. Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
3. Pulmonary hypertension due to lung diseases and/or hypoxia3.1. 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. Chronic thromboembolic pulmonary hypertension
5. Pulmonary hypertension with unclear multifactorial mechanisms5.1. Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
5.2. Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertensive