Management of chronic thromboembolic pulmonary hypertension: current status and emerging options

Aravinda Page, Jason M Ali, Jane Maraka, Robert Mackenzie-Ross, David P Jenkins

Chronic thromboembolic pulmonary hypertension (CTEPH) is the only cause

of pulmonary hypertension for which there is a potential cure, in the form of surgical pulmonary

endarterectomy. There is a strong link between pulmonary embolism and the development

of CTEPH. Although initially felt to be a rare complication, this position has been reviewed,

following several studies suggesting that up to 8.8% of patients develop CTEPH within the

2 years following pulmonary embolism. However, there is a significant discrepancy in the

number of patients being diagnosed, referred and treated for CTEPH. In this review, we

discuss the challenges of diagnosing CTEPH and present the current and emerging medical

and surgical management options available for patients suffering with CTEPH.

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