(1689) From Support to Salvage: VA ECMO as Bridge in Acute Chest Syndrome With Acute Right Heart Failure

Introduction: Acute chest syndrome (ACS) holds a high mortality in adults with sickle cell disease (SCD). Often, ACS leads to acute cor pulmonale and chronic pulmonary hypertension, further increasing mortality. Despite this, the treatment for ACS is mostly supportive. Case reports noting VenoVenous Extracorporeal Membrane Oxygenation (ECMO) as an advanced therapy for ACS appear in the literature, but little regarding the utility of VenoArterial ECMO in ACS exists.

Description: A 30 year old female presented with a history of SCD complicated by untreated pulmonary hypertension. Her initial workup revealed concerns for hypoxia secondary to ACS and pneumonia. Nasal high flow therapy, broad spectrum antibiotics and exchange transfusion started upon admission to the intensive care unit. Despite supportive management, she continued to decline.

An echocardiogram showed acute right heart failure. Initial pulmonary artery pressures were noted to be markedly elevated from baseline. Despite attempts at medical optimization with inhaled nitric oxide and initiation of epoprostenol the patient continued to show worsening signs of end-organ dysfunction. VA ECMO was initiated to stabilize the right heart allowing for initiation and titration of more aggressive therapies for the patient’s acute on chronic pulmonary hypertension.

After ECMO cannulation the patient’s epoprostenol was slowly titrated while carefully monitoring pulmonary artery pressures. She was titrated to 20ng/kg/min of epoprostenol with stabilization of the pulmonary pressures. The inhaled nitric oxide was subsequently weaned without episodes of instability. Repeat echocardiogram showed improvement in biventricular function and size. Once stable on pulmonary hypertensive therapies, patient decannulated from VA ECMO after approximately one week. She successfully transitioned to selexipag and was discharged to an inpatient rehabilitation facility.

Discussion: Acute chest syndrome is one of the most common causes of mortality in patients who have sickle cell disease. Despite the high mortality rate, the standard of care remains supportive. In this case we expanded the traditional indications for emergent VA ECMO and successfully treated a highly morbid disease.