TABLE 1 .

] World Health Organization Dana Point Classification of Pulmonary Hypertension (2008)

Classification
1. Pulmonary arterial hypertension
1.1. Idiopathic
1.2. Heritable
1.2.1. BMPR2
1.2.2. ALK1 endoglin (with or without hereditary hemorrhagic telangiectasia
1.2.3. Unknown
1.3. Drug and toxin induced
1.4. Associated with
1.4.1. Connective tissue diseases
1.4.2. HIV infection
1.4.3. Portal hypertension
1.4.4. Congenital heart diseases
1.4.5. Shistosomiasis
1.4.6. Chronic hemolytic anemia
1.5. Persistent pulmonary hypertension of the newborn
1.6. Pulmonary venoocclusive disease
2. Pulmonary hypertension owing to left-sided heart disease
2.2. Systolic dysfunction
2.2. Diastolic dysfunction
2.3. Valvular disease
3. Pulmonary hypertension owing to lung diseases and/or hypoxia
3.1. COPD
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. Development abnormalities
4. Chronic thromboembolic pulmonary hypertension
5. Pulmonary hypertension with unclear multifactorial mechanisms
5.1. Hematologic disorders: myeloproliferative disorders, splenectomy
5.2. Systemic disorders: sarcoidosis, pulmonary Langerhans’ cell histiocytosis
5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
5.4. Others: tumoral obstruction fibrosing mediastinitis, chronic renal failure or dialysis

ALK1 = activin receptor-like kinase type 1; BMPR2 = bone morphogenetic protein receptor type 2. (Adapted with permission from Simonneau et al.⁶)