Editorial
Copyright ©The Author(s) 2015.
World J Cardiol. May 26, 2015; 7(5): 230-237
Published online May 26, 2015. doi: 10.4330/wjc.v7.i5.230
Table 1 Updated classification of pulmonary hypertension
Pulmonary arterial hypertension
Idiopathic PAH
Hereditable PAH
BMPR2
ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia), SMAD9, CAV1, KCNK3
Unknown
Drug and toxin induced
Associated with:
Connective tissue diseases
HIV infection
Portal hypertension
Congenital heart diseases
Schistosomiasis
Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
Persistent pulmonary hypertension of the newborn
Pulmonary hypertension 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
Pulmonary hypertension 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 abnormalities
Chronic thromboembolic pulmonary hypertension
PH with unclear and/or multifactorial mechanisms
Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH