Background: Over the past two years, the utilization of venovenous extracorporeal membrane oxygenation (VV-ECMO) for the treatment of coronavirus disease 2019 (Covid-19) acute respiratory distress syndrome (ARDS) has increased. While supporting respiratory function, VV-ECMO requires large-bore indwelling venous cannulas which risk bleeding and infections, including endocarditis. Case Summary: We describe the cases of a 48-year-old female and 39-year-old male patient hospitalized for Covid-19 pneumonia who developed ARDS and right ventricular failure, requiring VV-ECMO and Protek Duo cannulation. The female patient was decannulated from VV-ECMO after 123 days, but subsequently developed a segmental pulmonary embolism and tricuspid mass measuring 6.4 x 5.5 x 0.5 cm (fig A). After 116 days of VV-ECMO support, the male patient developed a tricuspid valve vegetation measuring 1.4 x 1.1 cm (fig B) with an adjacent right atrial component measuring 2.1 x 1.3 cm. The Inari FlowTriever system was used to percutaneously remove both the pulmonary embolism from the female patient and the tricuspid masses from both patients. Pathological examination of the mass from the female patient demonstrated Candida albicans endocarditis in the setting of Candida fungemia (fig C). Pathologic examination of the specimens from the male patient demonstrated endocarditis consistent with Pseudomonas aeruginosa in the setting of Pseudomonas bacteremia (fig D). Both patients experienced resolution of fungemia and bacteremia after percutaneous vegetation removal, and after prolonged hospital courses were discharged with supplemental oxygen. Discussion: VV-ECMO and right ventricular support devices are invasive and associated with bloodstream infection and infective endocarditis. Percutaneous debulking of valvular vegetations, associated with these right-sided indwelling catheters, may be an effective method of source control to prevent progression to valve surgery.

Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by a defect in the branched-chain alpha-ketoacid dehydrogenase complex (BCKDC). This leads to the accumulation of the branched-chain amino acids (BCAAs) leucine, isoleucine, and valine, which can cause neurotoxicity. Patients with MSUD are carefully managed from birth with dietary restrictions and can acutely decompensate in the setting of infections or injury. We present the case of a 29-year-old female with a history of MSUD and rheumatoid arthritis on methotrexate and adalimumab who presented to our emergency department with symptoms suggestive of a metabolic crisis including nausea, vomiting, and presyncope. She was diagnosed with coronavirus disease 2019 (COVID-19) and admitted. An initial leucine level was mildly elevated at 253 μmol/L, consistent with her underlying metabolic condition. She was placed on an infusion of normal saline and 10% Dextrose (D10) in addition to a protein-restricted sick-day diet. Remdesivir therapy was initiated due to her immunocompromised status and high risk for decompensation but had to be discontinued due to nausea and vomiting that negatively impacted the patient’s oral intake. Her leucine level peaked at 647 μmol/L; however, her neurologic examination remained benign without signs of cerebral edema. With prompt involvement of our metabolic genetics team and initiation of intravenous fluids and the sick-day diet protocol, we avoided a metabolic crisis. The patient was discharged on day 5 of hospitalization with no complications from COVID-19 infection. This case highlights the individualized approach to the treatment of COVID-19 infection in a patient with a metabolic disorder. COVID-19 infection in the setting of MSUD has only been reported in two prior publications, one being a severe metabolic crisis with neurologic involvement. Fortunately, our patient experienced a mild case of COVID-19 without significant respiratory symptoms, and we were able to prevent a metabolic crisis during admission.