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Objective: This study aimed to accurately evaluate the prevalence and outcome of congenital heart disease (CHD) with small for gestational age (SGA)in consecutive newborns. Method: A prospective cohort study was conducted of a series of all livebirth newborns. Two-dimensional and color Doppler echocardiography were performed at 0-4 days of life in 6041 consecutive neonates who were born at Hamamatsu University Hospital, Japan, between May 2005 and April 2015. All newborns with CHD were followed up by echocardiography until a spontaneous resolution of all cardiac lesions occurred or they underwent surgery, received catheter intervention or died. Results: A total of 291 newborns were diagnosed with CHD, while 401 newborns were SGA. Among the newborns with CHD, 46 were SGA (SGA group) and 245 were not (non-SGA group). The prevalence of CHD in the SGA group was significantly higher than that in the non-SGA group (114.7 vs 43.4 per 1000 live birth, p<0.001). Ventricular septal defect was the most frequent cardiac abnormality in both groups. However, the incidence of severe defects in the SGA group was significantly higher than that in the non-SGA group (19.6% vs 7.8%, p=0.025). In addition, the rate of chromosomal or genetic abnormalities in SGA newborns (23.9%) was significantly higher than that observed in newborns without SGA (4.9%, p < 0.0001).Fourteen newborns (30.4%) in the SGA group and 24 newborns (9.8%) in the non-SGA group underwent surgical repair or catheter intervention for cardiac lesions during 6.7±3.3 years of follow-up. Spontaneous resolution of cardiac lesions was observed in 20 (43.5%) newborns in the SGA group and in 145 (59.2%) newborns in the non-SGA group. In the SGA group, the 5-year survival rate and 5-year invasive intervention-free rate were 90.9% and 63.6%, respectively. These rates were significantly lower in comparison to the non-SGA group (99.6% and 87.8%, respectively; log-rank test, p<0.0001). A logistic regression analysis that included clinical characteristics and echocardiographic parameters showed that SGA newborns exhibited a significantly increased risk ofinvasive interventionand death (adjusted OR, 8.22; 95% CI, 2.37-28.52; p=0.001). Conclusion: This study was conducted to prospectively elucidate the association between SGA and CHD in a large cohort of consecutive newborns by echocardiography. The prevalence of CHD in the present study was markedly higher in the SGA newborns. Furthermore, these neonates had a higher risk of mid-term mortality and invasive intervention in comparison to non-SGA newborns. [ABSTRACT FROM AUTHOR]