Summary
A full-term baby girl was diagnosed at birth using echocardiography to have a single
ventricle with common atrioventricular valve and subaortic ridge. On the 22nd day after birth she was admitted to hospital for intensive care after sudden onset
of cyanosis. Three days later she died after repeated attacks of bradycardia. At necropsy
the heart was examined by sequential segmental approach. Situs solitus with double-inlet
right ventricle and ventriculoarterial discordance was revealed. The left atrial floor
was separated from the ventricle by a small hole leading to a translucent blind pouch,
which bulged toward the subaortic outflow tract with its hypoplastic tensor apparatus
connected to the subaortic infundibulum. The aortic valve was normal. The pulmonary
trunk with a competent bicuspid valve arose from the left-sided rudimentary left ventricle.
The pulmonary blood flow was limited by a slit-like interventricular communication.
Obstruction of the pulmonary venous return due to the natural closing of the patent
foramen ovale and the mitral atresia led to her premature death.
Zusammenfassung
Es wird über ein 24 Tage alt gewordenes Mädchen berichtet, bei dem nach der Geburt
echokardiographisch ein Single-Ventrikel mit subaortaler Leiste diagnostiziert worden
war und bei dem die Autopsie ein double inlet right ventricle mit ventrikuloarterieller
Diskordanz und einer linksatrialen, subaortal verlaufenden blinden Aussackung ergab.
Key words
Blind mitral pouch - Subaortic mitral pouch - Double-inlet right ventricle
