Pulmonary Embolism and Chronic Superior Vena Cava Occlusion Complicating Central Line-Associated Venous Thromboembolism in a Sickle Cell Disease Patient

Abstract

Sickle cell disease (SCD), the most common genetic disorder globally, is often associated with an increased risk of venous thromboembolic events (VTE). Many of these patients have central lines placed for the purposes of repeated medication administration, blood transfusions, and blood draw, further increasing the risk of VTE. Given the non-specific presentation of VTE and pulmonary embolism, as well as the risk of mortality if interventions are delayed, a high index of suspicion is required for early diagnosis of the condition. We report the case of a 35-year-old woman with SCD and a port-a-cath in place who presented with extensive upper extremity and intrathoracic VTE with associated pulmonary embolism and chronic superior vena cava (SVC) occlusion. We also discuss the peculiarities of the clinical manifestations and management of VTE and pulmonary embolism in the setting of SCD based on the evidence from existing literature.

Keywords: central venous line; sickle cell disease: scd; sob - shortness of breath; superior vena cava (svc) obstruction; venous thromboembolism (vte).