Abstract
Congenital heart defects (CHD) represent the most frequent type of the heart disease in childhood, with incidence up to 1 % of all live-born children. Despite the improving echocardiographic diagnostics, part of CHD remains undiagnosed and can manifest in the later age or may be the cause of the early abortion.
On the other hand, some foetuses with prenatally diagnosed severe CHD may be recommended to interruption. Therefore, each pathologist can encounter a malformed heart at the autopsy.
Despite the current quality of the echocardiography, the macroscopic assessment of the heart by the pathologist is still considered the best method for evaluation of the structural heart disease. Knowledge of the basic pathologic anatomy thus remains an important prerequisite for adequately performed paediatric autopsy.