Key result
Pulmonary endarterectomy offers a potential cure for chronic thromboembolic pulmonary hypertension, though it is associated with a 2.2% to 5% risk of significant morbidity and mortality.
Why the study?
Chronic thromboembolic pulmonary hypertension is underdiagnosed due to nonspecific symptoms and few clinical signs until late disease stages, necessitating timely diagnosis for potential curative surgery.
Accurate diagnosis and comprehensive assessment of operability using multimodality imaging and hemodynamics are essential for selecting CTEPH patients for potentially curative pulmonary endarterectomy.
Chronic thromboembolic pulmonary hypertension is a rare and relatively poorly understood disease. It remains underdiagnosed and is often not recognized in primary and secondary care, as its symptoms are nonspecific and there are few clinical signs until late in the disease process. However, pulmonary endarterectomy (PEA) offers a potential cure for patients with this type of pulmonary hypertension; therefore, it is important that they are identified and diagnosed in a timely manner. PEA is associated with a 2.2%–5% risk of significant morbidity and mortality, even in experienced PEA centers. Therefore, once chronic thromboembolic pulmonary hypertension is diagnosed, further assessment of operability and patient selection is crucial. Assessment of operability involves determining the distribution and burden of chronic thromboembolic disease, assessing pulmonary hemodynamics, and assessing the functional impairment of the patient. Ventilation perfusion scintigraphy is of value in screening for the presence of chronic thromboembolic disease. However, computer tomography pulmonary angiography and magnetic resonance pulmonary angiography are now increasingly used to image the vascular occlusions directly. This allows assessment of the surgically accessible disease burden. Some centers still advocate conventional selective pulmonary angiography for the latter. Right-heart catheterization remains the gold standard for assessing pulmonary hemodynamics. Higher pulmonary vascular resistances are associated with poorer outcomes as well as increased risks at the time of surgery. This is in part because of the presence of more distal chronic thromboembolic material and distal pulmonary artery remodeling. However, in experienced centers, these patients are being operated on safely and with good outcomes. Patients with chronic thromboembolic disease, who have normal pulmonary hemodynamics, require careful functional assessments to determine the benefits of surgery.
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Jenkins et al. (2014) conducted a review in Chronic thromboembolic pulmonary hypertension. Pulmonary endarterectomy and diagnostic imaging was evaluated. Pulmonary endarterectomy offers a potential cure for chronic thromboembolic pulmonary hypertension, though it is associated with a 2.2% to 5% risk of significant morbidity and mortality.
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