Objective: The purpose of the study was to determine the pathological features of the umbilical cord structure associated with live born small for gestational age and premature baby with prenatally diagnosed congenital heart disease (CHD). Materials and methods: A retrospective analysis of the results of macroscopic examination of the umbilical cords was carried out in antenatally formed in the cohort with 115 cases of fetal CHD. A group of cases was divided into subgroups depending on the gestational age at delivery and baby weight at birth. Subroup 1 included 15 cases with premature and/or SGA infants; subgroup 2 consisted of 100 full-term infants with birth weight ≥ 10th percentile. Results: Short (58.2% of cases) and lean (43.4% of cases) umbilical cord, abnormal attachment to the placenta (35.6% of cases), hypo-and hypercoiled cord (4.3% and 19.1% of cases) were most commonly observed, respectively. Pathological variants and their combinations in one umbilical cord were found in 74% and 34% of cases. There were differences between the subgroups in the frequency of occurrence of hypercoiled and lean umbilical cord, and combined pathology, that was 46.6% versus 15%, 73.3% versus 39%, 60% versus 30% in subgroups 1 and 2, respectively, (р<0,05). At the same time, the odds ratio for the birth of premature and/ or small for gestational age baby with CHD was 4.96 (95% CI 1.56–15.7), 4.3 (95% CI 1.28–14.45) and 3.5 (95% CI 1.14–10.8), respectively. Conclusion: Postnatal assessment showed that umbilical cord pathology was in 74% of cases in babies born with congenital heart disease. Hypercoiled, lean umbilical cord and combination of umbilical cord abnormalities were significantly associated with preterm births and small for gestational age newborns. The obtained results indicate the need to expand the protocol for ultrasound assessment of the umbilical cord in cases of fetal cardiac pathology. © A group of authors, 2024.