Introduction Pulmonary alveolar proteinosis (PAP) is a rare condition where surfactant protein progressively accumulates in the lung alveoli. Pharmacological therapies are limited, and the current cornerstone of therapy is serial whole lung lavage (WLL). This procedure is typically performed with single lung-down ventilation, which is associated with the risk of severe hypoxemia. In patients with severe PAP who are already very hypoxemic, WLL can be hazardous and life-threatening. Here, we explore a case of a patient with severe PAP who underwent veno-venous extracorporeal membrane oxygenation (VV-ECMO) to tolerate WLL, exploring the role for mechanical circulatory support in treating patients with this condition. Case A 45-year-old male with a history of PAP that was lost to outpatient follow-up presents to the emergency department with worsening dyspnea. He was found to have acute hypoxemic respiratory failure, was intubated, and required ventilator settings of 100% FiO2 and a PEEP of 15 to maintain saturations. Chest imaging demonstrated findings of worsening PAP with possible superimposed pneumonia. The plan was for the patient to undergo WLL, but due to the severity of his PAP and hypoxemia, there was concern he would not tolerate single lung-down ventilation. The decision was made to proceed with VV-ECMO in order to tolerate the procedure. The patient was cannulated for VV-ECMO, underwent bilateral whole lung lavage, and was decannulated within 3 days. He was treated with antibiotics for superimposed pneumonia. He had rapid clinical improvement, was extubated within 6 days, and discharged from the hospital without supplemental oxygen. Discussion This case examines the role of VV-ECMO in treating patients with severe PAP who are undergoing WLL and at high risk for hemodynamic complications from the procedure. It underscores the utility of VV-ECMO in treating severe, reversible lung injury and reinforces patient characteristics that make up an ideal ECMO candidate. Further investigation is needed to determine if utilizing VV-ECMO in this patient population results in quicker time to recovery and improved outcomes. This abstract is funded by: None
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Twichell et al. (2026) studied this question.
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