Ripal Patel
University of Maryland School of Medicine
Baltimore, MD
Disclosure information not submitted.
Gregory Ede, MD
Pediatric Resident
University of Maryland Medical Center
Baltimore, Maryland, United States
Disclosure information not submitted.
Josia Schlogl, MD
Pediatric Critical Care Fellow
University of Maryland Medical Center
Baltimore, Maryland, United States
Disclosure information not submitted.
Adrian Holloway, MD
Pediatric Critical Care Attending
University of Maryland Medical Center, United States
Disclosure information not submitted.
Title: Fatal MIS-C in a Child with Palliated Single Ventricle Physiology
Introduction: We report a case of MIS-C, confirmed on autopsy, in a toddler with hypoplastic left heart syndrome (HLHS).
Description: The patient was a 15 month-old male with HLHS (aortic and mitral atresia) with a superior cavo-pulmonary anastomosis, and complete heart block with a pacemaker, admitted for respiratory distress. He had known exposure to multiple COVID+ family members, a positive SARS-COV-2 RT-PCR, fevers, elevated inflammatory markers, skin and mucosal changes, and multi-system organ dysfunction, evolving into severe, irreversible multiple organ failure. He met laboratory and clinical criteria for MIS-C. He was treated with high-dose Methylprednisolone, IVIG, Anakinra, and convalescent plasma, but continued to clinically deteriorate. Upon death, the family elected for autopsy. Significant autopsy findings included: acute ischemia of the papillary muscles; peripheral lung infarcts and hemophagocytosis in the bone marrow. The hemophagocytosis in the bone marrow has been noted on several of the small number of autopsies performed on children with fatal MIS-C.
Discussion: The patient met WHO and CDC criteria for MIS-C during his hospital stay. Both criteria require fever, elevated inflammatory markers without alternative cause, evidence of prior COVID-19 infection/exposure, and multi-organ involvement. While the pathophysiology of MIS-C remains unknown, the syndrome clinically and immunologically overlaps with other hyperferritinemic syndromes, such as hemophagocytic lymphohistiocytosis and macrophage activating syndrome, where immune-mediated multi-organ injury results from a dysregulated innate immune response-related cytokine storm. The finding of marrow hemophagocytosis on this patient’s autopsy adds to the small body of literature on autopsies of children with fatal MIS-C, which also note hemophagocytosis in the marrow and/or spleen. The immune profile and pathology of patients with MIS-C suggests that Macrophages have a significant role in MIS-C. This case thus adds additional credence to this important role of Macrophages, which in turn can further direct studies into its management, to prevent similar deaths.