Classification of Pulmonary Hypertension
Group | Type | Specific Disorders |
|---|---|---|
1 | Pulmonary arterial hypertension (PAH) | Disorders associated with PAH: Drug- and toxin-induced PAH Heritable PAH:
Idiopathic PAH PAH with long-term response to calcium channel blockers Persistent pulmonary hypertension of the newborn (PPHN) Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)
|
2 | Pulmonary hypertension with left-heart disease | Congenital or acquired left heart inflow or outflow tract obstruction and congenital cardiomyopathies Left heart diastolic dysfunction, including left heart failure with preserved ejection fraction Left heart systolic dysfunction Valvular heart disorders |
3 | Pulmonary hypertension associated with lung disorders, hypoxemia, or both | Alveolar hypoventilation disorders Chronic exposure to high altitude Chronic obstructive pulmonary disease (COPD) Developmental abnormalities Sleep-disordered breathing Other pulmonary disorders with a mixed restrictive and obstructive pattern |
4 | Pulmonary hypertension due to pulmonary artery obstructions | Nonthrombotic pulmonary embolism (eg, due to tumors, parasites, or foreign materials) Thromboembolic obstruction of distal or proximal pulmonary arteries |
5 | Miscellaneous (unclear or multifactorial mechanisms) | Hematologic disorders:
Systemic disorders: Metabolic disorders:
Other disorders:
|
ALK-1 = activin-like kinase type 1 receptor; BMPR2 = bone morphogenetic protein receptor type 2; CAV1 = caveolin 1; EIF2AK4 = eukaryotic translation initiation factor 2 alpha kinase 4; ENG = endoglin; GDF2 = growth differentiation factor 2; KCNK3 = potassium channel subfamily K member 3; SMAD9 = mothers against decapentaplegic homologue 9; TBX4 = T-box transcription factor 4. | ||
Adapted from the Fifth World Symposium on PAH, Nice, 2013; Simonneau G, Gatzoulis MA, Adatia I, et al: Updated clinical classification of pulmonary hypertension. Journal of the American College of Cardiology 62 (supplement D):D34–D41, 2013. | ||