(1663) 42-Year-Old Female Survivor With Acute Chest Syndrome Requiring VA ECMO

Introduction: Acute chest syndrome carries a high mortality rate in patients with sickle cell disease. It is the most common cause of death in patients with sickle cell disease, and it accounts for approximately 25% of all death. The associated mortality rates are 4.3% and 1.1% in adults and children respectively. Acute chest can cause hemodynamic instability. ECMO may be required to support the patient for hemodynamic compromise.

Description: 42-year-old female with sickle cell disease, hemoglobin SS genotype with homozygous alpha thalassemia was presented to the ED with chest pain. Her initial CTA chest did not show evidence of pulmonary embolism, but it did show early signs of acute chest syndrome. Her chest pain worsened after admission, and she began exchange transfusions for acute chest syndrome. She developed a new oxygen requirement and was ultimately found to have large pulmonary emboli on repeat imaging. The patient’s hypoxia and hemodynamics continued to worsen requiring transfer to the MICU. She was found to have severe right heart strain on echocardiogram for which she was taken to the Cath lab for further evaluation. She was initiated on ECMO for hemodynamic instability and worsening hypoxia. After establishing ECMO for her pulmonary embolisms she was immediately administered tPA. Her ECMO course lasted 2 days during which her right sided heart failure significantly improved. She was decannulated and continued to do well and was ultimately discharged with full neurologic function.

Discussion: ECMO is commonly a bridging therapy until definitive therapy can be provided. This includes treatments such as invasive cardiac surgery, transplant, or support for treatable metabolic derangements. We provided circulatory support through ECMO while she received plasma exchanges for her acute chest syndrome. In the context of acute chest syndrome when exchange transfusion has been initiated, ECMO provides time for definitive therapy to be effective. More data will be needed to elucidate the true mortality and morbidity benefits. This case supports more research for ECMO in the context of acute chest syndrome with hemodynamic instability.