Chronic Thromboembolic Pulmonary Disease and Chronic Thromboembolic Pulmonary Hypertension

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary disease (CTEPD) are two terms characterizing symptomatic patients with chronic thromboembolic occlusions of pulmonary arteries with or without pulmonary hypertension at rest. Their diagnosis follows evolving schemas that integrate technological advances of pivotal imaging modalities among which computed tomography angiography plays a major role. This review article summarizes the current knowledge on the natural history of acute pulmonary embolism and its evolution toward chronic pulmonary embolism, as well as the imaging clues, for the identification of chronically obstructed pulmonary arteries. The requirements for imaging at the time of therapeutic decisions are also described in the light of recent updates in the literature from multidisciplinary groups of experts. Because an early diagnosis of CTEPH remains a challenge for the medical community, several practical issues are included in this article with the objective of improving the knowledge and collaboration between radiologists and clinicians in service to the patient.

Recognition, diagnosis, and operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross‐sectional scientific survey (CLARITY)

“…Imaging (primarily V/Q scan and CTPA) plays a central role in the diagnosis of CTEPH to document the perfusion defects and the chronic occlusions of proximal pulmonary arteries by organized fibrotic clots 17 . It is important to note that a negative CTPA does not exclude CTEPH 3 .…”

“…As shown by our findings, key barriers remain, necessitating a variety of approaches, ideally tailored to medical specialists. Education of healthcare professionals, especially those involved in the care of patients with acute PE and radiologists, remains imperative to improve disease awareness and timely diagnosis 17 . Early diagnosis and referral of patients with CTEPH can furthermore be facilitated through the organization of PE clinics, through structured follow‐up of acute PE patients 22 as well as the development and validation of screening algorithms to support clinical decision‐making 3 …”

“…Therefore, there is an inherent risk among non‐CTEPH specialized physicians, who rely on CTPA to screen patients with PH, to miss the disease. Furthermore, early recognition of CTEPH is challenged by knowledge gaps within the imaging community 17 . In a separate subanalysis among 160 cardiologists and 156 pulmonologists, the sequence of diagnostic procedures performed to diagnose CTEPH was shown to differ 18 .…”

“…Imaging (primarily V/Q scan and CTPA) plays a central role in the diagnosis of CTEPH to document the perfusion defects and the chronic occlusions of proximal pulmonary arteries by organized fibrotic clots. 17 It is important to note that a negative CTPA does not exclude CTEPH. 3 Therefore, there is an inherent risk among non‐CTEPH specialized physicians, who rely on CTPA to screen patients with PH, to miss the disease.…”

“…Furthermore, early recognition of CTEPH is challenged by knowledge gaps within the imaging community. 17 In a separate subanalysis among 160 cardiologists and 156 pulmonologists, the sequence of diagnostic procedures performed to diagnose CTEPH was shown to differ. 18 Whether procedures were planned simultaneously was not captured by our survey.…”