Introduction: The incidence of right heart failure in patients with acute chest syndrome (ACS) due to sickle cell disease (SCD) is largely unknown and management is poorly defined. While venoarterial extracorporeal membrane oxygenation (VA ECMO) is increasingly used to manage right heart failure, its use in SCD and ACS has only recently been explored. A growing body of evidence suggests VA ECMO may provide lifesaving support for patients with acute right heart failure secondary to ACS. Description: A 29-year-old female with a history of previously well controlled Hemoglobin S-zero thalassemia on hydroxyurea who presented with complaint of acute chest and back pain. While initially hemodynamically stable, she deteriorated rapidly. Imaging was consistent with ACS but also showed right ventricular (RV) dilation and dysfunction. She received appropriate exchange transfusion and supportive care including intubation for acute hypoxic respiratory failure and aggressive vasopressor support. Despite this, she progressed to multi organ failure and her RV function continued to decline leading to refractory shock. It became clear she required mechanical circulatory support. Unfortunately, her options were limited as an RV thrombus was noted on echocardiogram and she was too unstable for thrombectomy or thrombolysis. As a result, percutaneous right ventricular assistance device (RVAD) and right ventricular impella were contraindicated, and VAECMO was her only viable option. Patient was cannulated & initiated on peripheral VA ECMO, and ECMO course was notable for challenges with ongoing hemolysis and hemoptysis that required multiple transfusions. However, her overall cardiac support and oxygenation needs improved allowing for ECMO decannulation after a week with improving RV function. Following decannulation she continued to experience steady recovery with resolution of her multi organ failure and return to her prior baseline. Discussion: Right heart failure due to ACS is a life-threatening but poorly understood complication. While there is limited data on the use of VA ECMO in this population, the case demonstrates that it can be lifesaving. While VA ECMO with SCD presents unique challenges, it should be considered when a RV failure deteriorates despite aggressive supportive measures and exchange transfusion.
Kolousek et al. (Sun,) studied this question.