The congenital heart disease series of the Pathological Museum at La S
apienza University of Rome was revised in order to detect specimens fe
aturing atrioventricular valve dysplasia (AVVD) in fetuses and infants
. Selected for study were 67 specimens from a collection of 667 hearts
: there were 36 cases of isolated tricuspid valve dysplasia (TVD), 11
cases of isolated mitral valve dysplasia (MVD), and 20 cases of combin
ed atrioventricular valve dysplasia. All the valves were graded accord
ing to Becker's criteria, which were morphometrically validated. There
is an increasing degree of dysplasia in terms of differentiation and
detachment of the valve from the ventricular wall. The concept of AVVD
is supported by a substantial morphologic and morphometric analogy be
tween TVD and MVD, in spite of some peculiarities of each grade and of
side-specific anomalies (''mitral arcade'' on the left side and grade
III dysplasia on the right one), probably attributable to differences
in the embryonic development of the two valves. AVVD is sometimes ass
ociated with dysplasia of the semilunar valves, in the setting of a po
lyvalvular disease of possible genetic origin. More often it is combin
ed with other defects that cause ventricular overload. In such cases,
AVVD usually occurs within the overloaded cardiac section, as if it we
re a result of mechanical stress.