Diagnosis and Assessment of Disease Severity
Table 2. Updated Clinical Classification of Pulmonary Hypertension (Dana Point, 2008)
Main modifications to the previous Venice classification are in green. 1 - Pulmonary arterial hypertension (PAH) ÎIdiopathic PAH
ÎHeritable > BMPR2 > ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia) > Unknown
ÎDrug- and toxin-induced (See Table 1)
ÎAssociated with > Connective tissue diseases > HIV infection > Portal hypertension > Congenital heart diseases > Schistosomiasis > Chronic hemolytic anemia
ÎPersistent 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
> Systolic dysfunction > Diastolic dysfunction > Valvular disease
3 - Pulmonary hypertension owing to lung diseases and/or hypoxia
> Chronic obstructive pulmonary disease > Interstitial lung disease > Other pulmonary diseases with mixed restrictive and obstructive pattern > Sleep-disordered breathing > Alveolar hypoventilation disorders > Chronic exposure to high altitude > Developmental abnormalities
4 - Chronic thromboembolic pulmonary hypertension (CTEPH) 5 - Pulmonary hypertension with unclear multifactorial mechanisms
> Hematologic disorders: myeloproliferative disorders, splenectomy > Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis
> Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders > Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
ALK1, activin receptor-like kinase type 1; BMPR2, bone morphogenetic protein receptor type 2; HIV, human immunodeficiency virus
