Table. 1—

Diagnostic classification of pulmonary hypertension

Pulmonary arterial hypertension
    Associated with:
        Connective tissue diseases
        Congenital left-to-right shunt
        Portal hypertension
        HIV infection
        Drugs and toxins
        Other conditions#
    Substantial venous or capillary involvement
        Pulmonary veno-occlusive disease
        Pulmonary capillary haemangiomatosis
    Persistent pulmonary hypertension of the newborn
Pulmonary hypertension with left heart disease
    Left-sided atrial or ventricular heart disease
    Left-sided valvular heart disease
Pulmonary hypertension associated with lung disease or hypoxaemia or both
    Chronic obstructive pulmonary disease
    Interstitial lung disease
    Sleep-disordered breathing
    Alveolar hypoventilation disorders
    Chronic exposure to high altitude
    Developmental abnormalities
Pulmonary hypertension due to chronic thrombotic or embolic disease or both
    Thromboembolic obstruction of proximal pulmonary arteries
    Thromboembolic obstruction of distal pulmonary arteries
    Nonthrombotic pulmonary embolism (tumour, parasites, foreign material)
    Sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and compression of pulmonary vessels (adenopathy, tumour, and fibrosing mediastinitis)
  • #: these conditions include thyroid disorders, type-1 glycogen storage disease, Gaucher’s disease, hereditary haemorrhagic telangiectasia, haemoglobinopathies, myeloproliferative disorders and splenectomy. Reproduced from [1] with permission from the publisher.