Table 1—

Updated clinical classification of pulmonary arterial hypertension(Dana Point, 2008) 2

1. Pulmonary arterial hypertension
 1.1 Idiopathic pulmonary arterial hypertension
 1.2 Heritable
  1.21 BMPR2
  1.2.2 ALK1, endoglin (with or without hereditary haemorrhagic 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 Schistosomiasis
  1.4.6 Chronic haemolytic anaemia
 1.5 Persistent pulmonary hypertension of the newborn
 1’. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
2. Pulmonary hypertension owing to left heart disease
 2.1 Systolic dysfunction
 2.2 Diastolic dysfunction
 2.3 Valvular disease
3. Pulmonary hypertension owing 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. Pulmonary hypertension with unclear multifactorial mechanisms
 5.1 Haematological disorders: myeloprolifearative disorders, splenectomy
 5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis
 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
 5.4 Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
  • Alk1: activin receptor-like kinase type 1; BMPR2: bone morphogenetic protein receptor protein type 2.