The changing landscape of chronic thromboembolic pulmonary hypertension management

Figures

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  FIGURE 1. Treatment algorithm for managing chronic thromboembolic pulmonary hypertension (CTEPH), from the European Society of Cardiology/European Respiratory Society guidelines [1, 2]. Pulmonary endarterectomy is the standard of care for patients with technically operable CTEPH and an acceptable risk/benefit ratio for surgery. #: technically operable patients with a non-acceptable risk/benefit ratio can be considered for balloon pulmonary angioplasty (BPA). ¶: in some centres, medical therapy and BPA are initiated concurrently. PH: pulmonary hypertension. Reproduced and modified from [1, 2] with permission.

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  FIGURE 2. The management options for chronic thromboembolic pulmonary hypertension (CTEPH) target different pathogenic manifestations in different parts of the pulmonary vascular bed. A schematic representation of a pulmonary artery is shown (note that vessel diameter is not to scale). Pulmonary endarterectomy (PEA) is used to remove thromboembolic lesions primarily in the proximal main artery (diameter of ∼3 cm), and lobar and segmental arteries [13, 14]; in expert surgical centres, lesions in distally located mid-segmental and sub-segmental branches can be targeted by PEA [14], down to vessels of 2 mm in diameter. Balloon pulmonary angioplasty (BPA) mainly targets distal lesions in the segmental and sub-segmental vasculature, down to small pulmonary arteries of 2–5 mm in diameter. Medical therapy targets microvasculopathy, including intimal thickening and fibromuscular proliferation, in vessels of 0.1–0.5 mm in diameter [10]. a) Computed tomography scan of a pulmonary artery. b) Organised fibrotic material removed during PEA. c) Selective pulmonary angiogram of segmental and sub-segmental pulmonary arteries, showing irregular vessel contour and occlusion, typical of CTEPH. d) Microscopic…