Viral myocarditis and cardiogenic shock in surgically palliated hypoplastic left heart syndrome

Abstract

In children with physiologically normal hearts, myocarditis is difficult to diagnose and manage due to variable clinical presentation and lack of consensus regarding effective supportive care measures. In those with palliated single ventricle physiology, baseline cardiac function is often altered, and thus, most children receive supportive therapy for congestive heart failure. We theorize that these children are at increased risk of clinically significant impairment in cardiac function in the setting of viral myocarditis.

We present three cases of children diagnosed with hypoplastic left heart syndrome (HLHS) who underwent multi-step surgical palliation. All three children subsequently developed fulminant viral myocarditis later in life, manifesting as symptomatic heart failure and cardiogenic shock. As a result, one child ultimately successfully completed orthotopic heart transplantation, one developed multiple system organ failure, and their care was withdrawn, and one recovered.