Flail Tricuspid and Mitral Valve in Neonatal Lupus

Neonatal lupus erythematosus (NLE) typically presents as congenital heart block; flail atrioventricular valve leaflets is especially rare.

A neonate with flail tricuspid and mitral valve resulted in severe atrioventricular valve insufficiency. There were high anti-Ro60 antibody titers consistent with NLE. Steroids and immunoglobulins were administered. Mitral and tricuspid valve repairs were undertaken. An inability to wean from extracorporeal membrane oxygenation led to the mitral valve being replaced with a modified Melody valve and then mechanical mitral valve.

This is the first reported case of NLE presenting with bilateral atrioventricular valve chordal rupture, necessitating early valve replacement. This highlights a severe manifestation of NLE and has implications for diagnosis, surveillance, and novel management strategies.

NLE can present with atypical, life-threatening valvular pathology of both the tricuspid and mitral valve. The Melody valve offers a promising bridge to a definitive valve replacement in the mitral position.