Abstract Background Sickle cell disease (SCD) involves chronic hemolytic anemia, vaso-occlusion, pain crisis, and end-organ damage. This case depicts the complications arising from SCD, including acute chest syndrome, non-ischemic dilated cardiomyopathy, refractory vasoplegic shock, and a catastrophic stroke. Case Description A 44-year-old male with a medical history of well controlled SCD and hypertension was admitted for community acquired pneumonia. He complained of mild chest pressure and was admitted to the intensive care unit for monitoring, but the concern for acute crisis was low. Further evaluation incidentally revealed new-onset non-ischemic type cardiomyopathy (NICM) with an ejection fraction of 19% and global hypokinesis. On day 2, the patient had excruciating back pain and was urgently planned for an exchange transfusion (ET). He had pulseless-electrical activity and a cardiac arrest before the ET could begin. He was successfully resuscitated and ET was completed at a slow rate. Patient remained on triple pressors and had labile blood pressure. On day 3, a fourth vasopressor was added but the patient developed refractory vasoplegic shock and severe acidosis. He also developed a dilated non-reactive left pupil but was too unstable for transport to imaging to confirm a stroke. Eventually, the patient had another cardiac arrest and passed away. Discussion Vasoplegic shock in SCD is a life-threatening scenario, driven by endothelial dysfunction, severe nitric oxide (NO) depletion, ischemia-reperfusion injury, and the production of reactive oxygen species (ROS) and pro-inflammatory cytokines. The pathophysiology for cardiomyopathy includes anemia-driven high-output status, recurrent microvascular occlusion, endothelial dysfunction, ischemia-reperfusion injury, and myocardial fibrosis. Heart failure in crisis is driven by intravascular release of heme, which scavenges NO, resulting in vasoconstriction, severe pulmonary hypertension and eventual biventricular failure. Management involves exchange transfusions to reduce HbS concentration and hemodynamic support. However, the multifaceted nature of these complications necessitates a comprehensive approach to treatment. This abstract is funded by: -
Saleem et al. (Fri,) studied this question.