Acute Intraoperative STEMI in a Toddler

Coronary artery disease and acute coronary ischemia are very rare in children. A 22-month-old toddler with history of developmental delay, chromosome 1q21.1 microduplication, and severe hypospadias undergoing routine urologic procedure under general anesthesia presented with refractory hypotension. This was found to be caused by acute diffuse coronary ischemia with electrocardiogram changes and rising cardiac troponin. He experienced acute decompensation requiring mechanical ventilation and vasoactive infusion. Multimodal diagnostics were obtained including echocardiogram, computed tomography, and cardiac catheterization showing impaired cardiac function and diffuse, likely chronic, coronary vasculopathy in both coronary systems. Extensive workup found no clear evidence of infectious, rheumatologic, genetic, or thromboembolic etiology to explain the presentation or chronic cardiac dysfunction. The patient was eventually transitioned to an oral heart failure medication regimen. This presentation is exceptionally rare in children. Further evaluation of similar patient presentations will be needed to understand and guide management.