Classifications (Dana Point 2008)

1. Pulmonary arterial hypertension

1.1. Idiopathic (iPAH)

Cause unknown

1.2. Heritable pulmonary arterial hypertension

Family or genetic cause

1.3. PAH associated with drugs and toxins

E.g. triggered by appetite suppressants

1.4. Pulmonary arterial hypertension secondary to diseases, such as:

  • Connective tissue diseases
  • HIV infection
  • Portal hypertension (= portopulmonary hypertension = PPHT)
  • Congenital heart diseases
  • Schistosomiasis
  • Chronic haemolytic anaemia

1.5. Persistent pulmonary hypertension of the newborn

(Sudden cardiac death)

1.6. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary haemangiomatosis (PCH)

2. Pulmonary hypertension secondary to left heart diseases

Systolic left ventricular dysfunction
Diastolic left ventricular dysfunction
Valvular disease of the left heart

3. Pulmonary hypertension secondary to lung diseases and/or hypoxaemia

COPD (chronic obstructive pulmonary disease)
Interstitial lung disease
Other pulmonary disease with mixed restrictive and obstructive pattern
Sleep apnoea syndrome
Disease with alveolar hypoventilation
Chronic exposure to high altitudes
Pulmonary developmental disorder

4. Chronic thromboembolic pulmonary hypertension (CTEPH)

5. Pulmonary hypertension with other diseases

This category includes diseases that may cause pulmonary hypertension as a result of inflammatory processes or mechanical obstruction (haematological diseases, sarcoidosis, storage diseases, tumours, etc.). Treatment is restricted to treatment of the underlying disease.

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