Figures
- FIGURE 1
Schematic of the pathophysiological concept of chronic thromboembolic pulmonary hypertension following acute pulmonary embolism (PE). VTE: venous thromboembolism. Reproduced from [5] with permission from the publisher.
Tables
- TABLE 1
Comparison of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH)
CTEPH PAH Common pathophysiological link Microvascular arteriopathy resulting in endothelial dysfunction and vascular remodelling Common symptoms Dyspnoea, fatigue, weakness and syncope Aetiology Unresolved emboli after PE Abnormal proliferation of endothelium and smooth muscle in vessel walls, small vessel thrombi are possible Unknown aetiology in the absence of previous PE Risk factors Previous PE/VTE and recurrent VTE, infected pacemaker, ventriculoatrial shunt, splenectomy, antiphospholipid antibodies, and lupus anticoagulant Genetic mutation, certain drugs/toxins, HIV, connective tissue disorders, congenital heart disease Disease phenotype Primarily affects the elderly of both sexes, episodic course with “honeymoon” periods Typically affects young women, progressive course Diagnosis Segmental perfusion defects in V′/Q′ scan, pulmonary angiography, CT No segmental perfusion defects in V′/Q′ scan, right heart catheterisation Treatment PEA surgery for suitable patients, balloon pulmonary angioplasty for segmental/subsegmental disease, medical treatment targeting dysfunctional pathways in endothelial cells of inoperable patients Medical treatment targeting dysfunctional pathways in endothelial cells -
PE: pulmonary embolism; VTE: venous thromboembolism; V′/Q′: ventilation/perfusion; CT: computed tomography; PEA: pulmonary endarterectomy. Information from [8].
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Vol 24 Issue 136
Table of Contents
Chronic thromboembolic pulmonary hypertension: a distinct disease entity
