E5064. Insight Into Sickle Cell Disorder: A Radiologic Journey Through Cardiothoracic Manifestations?
Authors
Aws Kamona;
Johns Hopkins University School of Medicine
Cheng Ting Lin;
Johns Hopkins University School of Medicine
Background
Sickle cell disorder (SCD), is the most prevalent inherited disorder of hemoglobin synthesis, resulting in formation of abnormally shaped (sickled) red blood cells that results in microvascular occlusion with recurrent episode of tissue and organ ischemia and infarction leading to progressive multisystem organ damage, which can manifest in variable acute and chronic cardiothoracic manifestations. Vasoocclusion, thromboembolic disease, and recurrent infections as a result of functional asplenia are the predominant factors resulting in the different clinical manifestations.
Educational Goals / Teaching Points
Provide pathophysiological context on how SCD affects various cardiothoracic structures. Review the role of imaging in diagnosing cardiothoracic disorders attributable to SCD. Imaging features of the common and uncommon sequelae of SCD in cardiothoracic imaging studies.
Conclusion
Cardiothoracic manifestation are mainly secondary to vasoocclusion, ischemia, and infection. Understanding the cardiothoracic manifestations of SCD on imaging is essential for appropriate diagnoses and management, given the nonspecific clinical symptomatology, ultimately improving clinical outcome and quality of life of individuals with this disease.