Repair of ruptured tendinous chords in the newborn with flail tricuspid valve and functional pulmonary atresia

The Blue Baby Blues: A Rare Case of Cyanosis in the Newborn

“…The infant presents immediately or shortly after birth with profound cyanosis and refractory hypotension, respiratory distress, and a grade 2-3/6 murmur with an active precordium (Lim, Huh & Jun, 2004). The infant typically will not respond to a hyperoxia test, will have diminished PVMs on CXR, and an ECHO will reveal tricuspid insufficiency with functional pulmonary atresia, meaning absence of flow through the pulmonary valve as a result of complete tricuspid insufficiency (Boon, Hruda & Schoof, 2007). In most cases, the infant will not respond, or will only respond minimally, to maximal respiratory and inotropic support and the only life saving intervention is surgical repair of the valve.…”