Clinical Classification of Pulmonary Hypertension

 

Processes associated with pulmonary hypertension

(1) pulmonary arterial hypertension (PAH)

(2) pulmonary venous hypertension (PVH) associated with left-sided heart disease

(3) pulmonary hypertension associated with pulmonary disease and/or hypoxemia

(4) pulmonary hypertension associated with chronic thromboemoblic disease

(5) pulmonary hypertension associated with compression of pulmonary blood vessels

(6) pulmonary hypertension associated with pulmonary intravascular tumors

(7) pulmonary hypertension due to miscellaneous causes

 

Conditions associated with PAH:

(1) idiopathic PAH

(2) familial

(3) collagen vascular disease

(4) congenital systemic-to-pulmonary shunts

(5) portal hypertension

(6) HIV infection

(7) drugs

(8) toxins

(9) hyperthyroidism

(10) hemolytic anemias and hemoglobinopathies

(11) myeloproliferative disorders

(12) glycogen storage disease or Gaucher's disease

(13) hereditary hemorrhagic telangiectasia

(14) pulmonary veno-occlusive disease (PVOD)

(15) pulmonary capillary hemangiomatosis

(16) persistent pulmonary hypertension of the newborn (PPHN)

(17) miscellaneous

 

Conditions associated with PVH:

(1) left atrial disease

(2) left ventricular systolic dysfunction

(3) left ventricular diastolic dysfunction

 

Conditions associated with chronic pulmonary disease and/or hypoxemia

(1) chronic obstructive pulmonary disease (COPD)

(2) interstitial lung disease

(3) sleep-disorderd breathing

(4) alveolar hypoventilation disorders

(5) chronic altitude sickness

(6) developmental abnormalities

 

Conditions associated with chronic thromboembolic disease:

(1) obstruction of the proximal pulmonary arteries

(2) obstruction of the distal pulmonary arteries

(3) massive tumor emboli

(4) intravascular parasites

(5) embolic foreign material

 

Conditions associated with compression of pulmonary blood vessels:

(1) lymphadenopathy

(2) fibrosing mediastinitis

(3) mediastinal tumor

 

Pulmonary intravascular tumors:

(1) intravascular large B-cell malignant lymphoma

 

Miscellaneous causes of pulmonary hypertension:

(1) sarcoidosis

(2) histiocytosis X

(3) lymphangiomatosis