Skip to main content
. Author manuscript; available in PMC: 2011 Dec 1.
Published in final edited form as: J Cardiovasc Pharmacol. 2010 Dec;56(6):696–708. doi: 10.1097/FJC.0b013e3181f9ea8d

Table 1.

Updated Clinical Classification of Pulmonary Hypertension (Dana Point 2008) 2

  • 1. Pulmonary arterial hypertension (PAH)
    • 1.1.Idiopathic PAH
    • 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. Schistosomiasis
      • 1.4.6. Chronic hemolytic anemia
    • 1.5 Persistent pulmonary hypertension of the newborn

  • 1″. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

  • 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 (CTEPH)

  • 5. Pulmonary hypertension with unclear multifactorial mechanisms
    • 5.1. Hematologic 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