The echocardiographic findings and clinical courses in 19 fetuses with
marked cardiomegaly [heart length, width, area, cardiothoracic ratio,
heart/thoracic area (CTA) ratio >2 SD] were reviewed. An abnormal hea
rt was the reason for referral in 16 cases of 19 (84.2%). The mean ges
tational age was 31.5 weeks at detection. Intracardiac hemodynamics we
re studied using pulsed, color, and M-Mode color Doppler. Despite the
heterogeneous etiology of marked cardiomegaly (5 Ebstein's anomalies,
5 tricuspid valve dysplasias, 3 dilatative cardiomyopathies and 6 misc
ellaneous) a holosystolic tricuspid regurgitation with right atrial en
largement was found in 17 of the 19 cases (89.4%) as a common pathogen
etic feature. A hydrops was found in 10 cases (52.6%). Besides 1 elect
ive legal abortion, a high mortality rate of 83.3% was observed in the
group followed up (6 intrauterine and 9 neonatal deaths). Among the c
ardiac measurements the CTA ratio seemed to correlate with fetal outco
me, since all three survivors had the lowest values and all fetuses wi
th CTA ratio >0.6 showed a lung hypoplasia as a sequel of compression
by cardiac enlargement. The presence or the development of hydrops, an
obstruction of the right ventricular outflow tract, and the lung hypo
plasia could be considered as signs of poor prognosis. A reliable diff
erentiation between a patent pulmonary valve, pulmonary stenosis and p
ulmonary atresia in severe tricuspid regurgitation using modern Dopple
r means is difficult as seen in our studied group and in the literatur
e. A cardiomegaly could be detected by nonexperienced examiners, but e
ven if its presentation in fetal life could be considered as one of th
e main predictors of poor outcome, such cases have to be referred to c
entres experienced in fetal echocardiography.