Abstract

OBJECTIVES:Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients.METHODS:A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016.RESULTS:Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified.CONCLUSION:Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.

Highlights

  • Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by organized thromboembolic material [1]

  • From 2013 to 2016, seven CTEPH patients presented lung cavities at diagnosis in the main reference center for pulmonary hypertension in Brazil (Heart Institute, SP), a country with high endemic rates of chronic granulomatous lung diseases. During this 4-year period, we evaluated 140 CTEPH patients at our center and performed pulmonary endarterectomy (PEA) in approximately 110 patients

  • All PH patients at our center followed the diagnostic algorithm suggested by European Society of Cardiology and European Respiratory Society and were reviewed in 2015 [12]

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Summary

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by organized thromboembolic material [1]. The primary therapeutic approach that should be implemented for CTEPH is pulmonary endarterectomy (PEA), a surgery that removes the occluding material from the pulmonary vessels as well as the vascular endothelium [2], and lifelong anticoagulation [3]. Unlike acute pulmonary embolism [4,5,6], CTEPH frequently presents as enlargement of the main pulmonary artery, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns [7]. This study describes seven CTEPH patients with lung cavities and discusses the diagnostic approach and management of these conditions

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