In 2013, the 5th World Symposium on pulmonary hypertension took place in Nice, France and modified the classification system for pulmonary hypertension.
The modified system divides pulmonary hypertension into five groups:
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group 1: pulmonary arterial hypertension (disorders of the pulmonary arteries themselves)
- 1.1: idiopathic pulmonary arterial hypertension
- 1.2: heritable pulmonary hypertension
- 1.3: drug- and toxin-induced pulmonary hypertension
- 1.4: pulmonary hypertension associated with
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group 2: pulmonary hypertension due to left-sided heart disease
- 2.1: systolic dysfunction
- 2.2: diastolic dysfunction
- 2.3: valvular disease
- 2.4: congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
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group 3: 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 lung disease
- group 4: pulmonary hypertension due to chronic pulmonary thromboembolism
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group 5: pulmonary hypertension due to unclear multifactorial mechanisms
- haematologic disorders
- systemic disorders
- metabolic disorders
- other conditions
- fibrosing mediastinitis
- tumoural obstruction
- chronic renal failure
- segmental pulmonary hypertension