The purpose of this study was to assess the incidence of congenital heart disease (CHD) in fetuses with premature atrial contractions (PAC) and to check whether these fetuses should be referred for a special fetal echocardiogram. Out of 120 fetuses with PACs and 75 fetuses with CHD both conditions were present in only 9 fetuses (= 7.5%). Two pregnancies were terminated because of associated severe fetal anomalies. After birth PACs disappeared in all other cases spontaneously. Despite higher numbers, the incidence of fetal CHD was not statistically significant higher in fetuses with PACs. Thus, recognition of fetal PACs should lead to a referral for a special fetal echocardiographic examination.