P1.022: A rare case of Reye’s Syndrome in a patent with congenital heart disease

Introduction: Reye’s syndrome is a serious fatal condition in children where a child experiences acute encephalopathy with fatty liver failure. In the 1980s, cases of Reyes syndrome peaked in prevalence leading to a discovery of its association with aspirin use, particularly following acute infections. Since that time, the recommendation against aspirin use in children has significantly decreased the amount of Reye’s syndrome cases. However, there remains a subset of children that require aspirin use particularly for its anti-coagulant properties. In the following case, we describe a child with a congenital heart condition requiring aspirin prophylaxis who developed Reye’s syndrome after an influenza infection.

Case Description: The patient is a 5 year old with history of a complete AV canal status post two patch repair with subsequent mitral valve stenosis requiring mitral valve replacement with a prosthetic valve and balloon dilations. Due to her prosthetic valve, she had been on aspirin prophylaxis. Patient presented with symptoms of vomiting, fevers and hypoxia. The initial concern was for increased left atrial pressure due to stenosed mitral valve leading to pulmonary hypertension. She was taken to the catheterization lab for a transseptal Brockenbrough through intact atrial septum, static balloon atrial septostomy and mitral valvuloplasty. Shortly after arrival back in CICU, she become profoundly irritable, agitated and confused. She experienced hypotension, hypoglycemia as well as metabolic acidosis with a rising lactate. She was sedated and intubated with continued hemodynamic instability through the night. The following morning, her liver enzymes had tripled and overall hepatic function had declined. On review of her chart, she had been diagnosed with influenza A eight days prior to admission. Due to concern for Reye’s syndrome gastroenterology and hepatology were consulted. Due to coagulation risk, liver biopsy and CSF were unable to be obtained. However, her clinical picture was strongly consistent with Reye’s syndrome including the acute change in mental status and three fold increase in liver enzymes, along with other noted lab abnormalities including coagulopathy, hypoglycemia and lactic acidosis. Supportive care was initiated, head and liver imaging obtained and ammonia levels were closely monitored. aspirin was already being held due to recent catheterization. Improvement of mental status and liver enzymes were noted over the next several days. On discharge, she was transitioned to clopidogrel for prophylactic therapy.

Discussion: Luckily, this patient was identified in stage 1 of Reye’s syndrome and therefore was able to be discharged home with normal liver function and baseline mental status. This case importantly highlights a population that frequently uses aspirin prophylaxis and remains at risk for Reye’s syndrome: congenital heart patients.

Conclusion: Keeping the differential of Reye’s syndrome is crucial for identifying it early, stopping the offending agent and switching to a different therapy.