Systemic hypoperfusion in a neonate with tricuspid atresia and transposition of the great arteries. Similarity to the hypoplastic left heart syndrome.

Infants with the hypoplastic left heart syndrome present a reproducible appearance that terminates in irreversible shock during the neonatal period. There is disagreement over the exact cause of this appearance. This case involves a newborn infant with tricuspid atresia and transposition of the great arteries, and with the clinical appearance and course usually reserved for neonates with the hypoplastic left heart syndrome. Accurate diagnosis is crucial in all such infants to correctly distinguish those with a hopeless outcome from those amenable to palliative cardiovascular surgery. This case supports the contention that ductal closure alone is not the principal cause of death in infants with hypoplastic left heart syndrome.