Tricuspid Valve Endocarditis in Four Patients with Unrepaired Restrictive Perimembranous Ventricular Septal Defects

Congenital heart disease (CHD) is the most common predisposing factor for pediatric infective endocarditis (IE). Although patients with unrepaired ventricular septal defects (VSDs) are at greater risk of IE than those without CHD, the American Heart Association (AHA) considers VSDs to be relatively low risk and therefore does not recommend antibiotic prophylaxis against IE. Even among patients with VSDs who develop IE, current AHA and European Society for Cardiology (ESC) guidelines do not recommend surgical VSD closure, despite the potential for a second IE event. We present a case series of four children with small, restrictive, perimembranous VSDs who developed tricuspid valve (TV) IE. All four experienced delayed diagnosis and secondary complications, including three with septic pulmonary emboli. All four patients ultimately underwent surgical VSD closure. These cases highlight the importance of recognizing IE as a possible cause of prolonged fever in children, even among those with even 'low-risk' CHD. The cases also draw attention to the potential benefits of VSD closure in patients who develop IE.