Anomalies of the brain and congenital heart disease: a study of 52 necropsy cases.

During routine autopsy examinations several macroscopic anomalies of brains were noted in children with congenital heart disease (CHD). To define these observations 52 autopsies of cases with CHD as well as 52 controls were compiled. Cases were divided into two groups according to the absence (group I) or presence (group II) of a multimalformative syndrome (MMS). Fifty percent of the sample was associated with MMS. Brain anomalies were classified into grades I, mild and focal; II, intermediate, focal or diffuse; and III, severe and diffuse. Group I was associated with brain anomalies (mainly of grades I and II with altered gyri) in 19 of 28, whereas in Group II the brains frequently had compromise of lobes, gyri, and fissures, 18 of 24. The relationship between the types of CHD, grade of brain anomalies, and MMS is delineated. The high frequency of brain anomalies associated with CHD but without other malformations (67.8%) suggests a clinical counterpart of these lesions.