P0471 - Investigating the use of ECMO in children with ANCA+ Pulmonary Hemorrhage: A Case Report

Introduction: Extracorporeal membrane oxygenation (ECMO) is a life-saving tool utilized in the pediatric intensive care unit (PICU) for a variety of conditions. ECMO generally requires anti-coagulation to prevent the formation of clots within the circuit, limiting its use in patients with active bleeding. This case, however, demonstrates the benefit of ECMO in pulmonary hemorrhage despite the risks.

Case Description: A previously healthy 13-year-old female presented with fever, shortness of breath, and hypoxemia. She required high flow nasal cannula and chest x-ray showed bilateral infiltrates. Labs demonstrated mild leukocytosis (11.51 x10³ uL) and anemia with a hemoglobin (Hgb) and hematocrit (Hct) of 8.7 g/dL and 26 g/dL, respectively. She was admitted for presumed pneumonia and started on ceftriaxone, vancomycin, and azithromycin. Over the next 48 hours, her respiratory status worsened, and she required bilevel positive airway pressure in the PICU. She also had worsening anemia (Hgb 6.3 g/dL, Hct 19.7 g/dL). CT of the chest revealed multifocal patchy and confluent consolidations in the bilateral lungs with superimposed ground glass opacities. Her anemia and new imaging findings were suspicious for pulmonary hemorrhage. She had also developed a vasculitic rash, hemoptysis, and elevated urine protein to creatine ratio concerning for glomerulonephritis, all of which suggested rheumatologic disease. Rheumatologic labs initially revealed hypocomplementemia (C3 of 61 mg/dL, C4 of 7mg/dL). She worsened, requiring mechanical ventilation, but remained persistently hypoxemic. Veno-venous (VV) ECMO was started to provide lung rest and time for treatment with plasmapheresis and steroids. She was bolused with 50 units/kg of heparin at cannulation followed by a continuous heparin infusion 4 hours post cannulation at 10 units/hour for 12 hours before titration up of the infusion. She was maintained on lower, tight anticoagulation goals of an anti-Xa level of 0.1-0.2. ECMO flows were higher than typical VV ECMO flows, ranging from 80-90cc/kg/min with a sweep gas as high as 4L/min, titrating down as she improved. No increased bleeding or thrombotic events occurred. While she was on ECMO, her labs showed antineutrophil cytoplasmic antibodies (ANCA, 1:2560, normal < 1:20) and elevated serine protease 3 antibodies (954 AU/mL), consistent with granulomatosis with polyangitis (GPA). ECMO continued for six days during which she received five rounds of therapeutic plasma exchange, two rounds of intravenous immunoglobulin, and three methylprednisolone pulses. She ultimately recovered and biopsy of her left kidney confirmed GPA.

Discussion: Pulmonary hemorrhage creates impaired gas exchange in the lungs that can be refractory to mechanical ventilation. This case highlights the utility of ECMO in pulmonary hemorrhage and shows that with proper management of anti-coagulation, the risk of increased bleeding is low.


Conclusion: This patient’s recovery demonstrates the need for a larger scale study investigating the benefit of ECMO in ANCA+ pulmonary hemorrhage.