Chronic thromboembolic pulmonary hypertension (CTEPH)

Chronic pulmonary thromboembolic disease is an important cause of severe pulmonary hypertension, and as such is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction, with predictable mortality related to the severity of the underlying pulmonary hypertension.1 In recent years the epidemiology of this condition has been revised considerably. Once considered a rare condition, chronic thromboembolic pulmonary hypertension (CTEPH) was recently documented to complicate 3.8% of acute pulmonary embolic events.2 CTEPH is the only cause of severe pulmonary hypertension which is potentially curable without the need to resort to lung transplantation. Pulmonary endarterectomy (PEA) is the surgical procedure which removes the obstructing thromboembolic material, resulting in significant improvements (and in many cases normalisation) in right ventricular haemodynamics and function. This procedure requires a high degree of anaesthetic and surgical skill, coupled with assiduous preoperative evaluation of potential patients. Surgery is generally considered only in patients with proximal chronic thromboembolic disease as assessed by radiological investigations. Over the past 20 years, the number of these procedures being performed has steadily increased. This reflects increased physician recognition of both pulmonary hypertension in general, and CTEPH in particular. Despite this increasing interest, however, outside of individual case series and reported experience, there are scant data available to support many of the theories and suppositions concerning this condition. Over the past 5–10 years, there have been major advances in the field of pulmonary hypertension. Underpinning this is an increasing understanding of the cellular, molecular and genetic mechanisms underlying pulmonary arterial hypertension (PAH), coupled with an increased range of effective treatment (drug) options for patients with this condition. This has created renewed interest among health professionals in general, and a significant increase in the number of referrals of patients with suspected pulmonary hypertension …

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