Table 1.

Updated classification of pulmonary hypertension (PH)

Updated complications of PH
PAH
Idiopathic PAH
Heritable PAH
BMPR2
ALK-1, ENG, SMAD9, CAV1, KCNK3
Unknown
Drug and toxin induced
Associated with
Connective tissue disease
HIV infection
Portal hypertension
Congenital heart disease
Schistosomiasis
Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
PPHN
PH due to left heart disease
Left ventricular systolic dysfunction
Left ventricular diastolic dysfunction
Valvular disease
Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
PH due 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 lung diseases
CTEPH
PH with unclear multifactorial mechanisms
Haematologic disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH

ALK-1, activin-like receptor kinase 1; BMPR2, bone morphogenic protein receptor type 2; CAV1, caveolin 1; CTEPH, chronic thromboembolic pulmonary hypertension; ENG, endoglin; HIV, human immunodeficiency virus; KCNK3, a gene-encoding potassium channel super family K member 3; PAH, pulmonary arterial hypertension; PPHN, persistent pulmonary hypertension of the newborn; SMAD9, decapentaplegic 9.

The Fifth World Symposium on Pulmonary Hypertension, 2013, Nice, France.