Analysis of single umbilical artery with concurrent congenital anomaly: Is it a risk factor for poor prognosis? A cross-sectional study


Background: A single umbilical artery (SUA) may coexist with a single anomaly or multiple congenital anomalies. Although anomalies associated with SUA can primarily cause high perinatal mortality, their clinical significance has not been evaluated.

Objective: We investigated the relationship between the clinical features and the type or number of concurrent anomalies in neonates with SUA.

Materials and Methods: In this cross-sectional study, 104 neonates with SUA were enrolled from January 2000- to December 2020 at Dongsan hospital, Daegu, South Korea. Data on the maternal history and the neonates demographic characteristics, clinical course, chromosomal analysis, and congenital anomalies, were collected.

Results: Among the neonates with SUA included, 77 (74.0%) had one or more congenital anomalies; 66 (63.5%) were cardiac, 20 (19.2%) were genitourinary, 12 (11.5%) were gastrointestinal, 5 (4.8%) were central nervous system, 12 (11.5%) were skeletal, and 5 (4.8%) were facial anomalies. The number of concurrent anomalies ranged from 0–4. Neonates with SUA and concurrent gastrointestinal anomaly had a high incidence of initial positive ventilation, intubation, and inotropic drug use and lower Apgar score at 1 min and 5 min. 7 (6.7%) neonates with SUA died. Low birth weight (odds ratio = 6.16, p = 0.05), maternal multiparity (2.41, p = 0.13), gastrointestinal anomaly (5.06, p = 0.11), and initial cardiac resuscitation (7.77, p = 0.11) were risk factors for mortality in neonates with SUA.

Conclusion: Neonates with SUA and concurrent gastrointestinal anomaly, low birth weight, maternal multiparity, and initial cardiac resuscitation had poor outcomes.

Key words: Single umbilical artery, Congenital abnormalities, Perinatal mortality.

[1] Nayak SS, Shukla A, Girisha KM. Anomalies associated with single umbilical artery at perinatal autopsy. Indian Pediatr 2015; 52: 73–74.

[2] Vafaei H, Rafeei Kh, Dalili M, Asadi N, Seirfar N, Akbarzadeh-Jahromi M. Prevalence of single umbilical artery, clinical outcomes and its risk factors: A cross-sectional study. Int J Reprod BioMed 2021; 19: 441–448.

[3] Mailath-Pokorny M, Worda K, Schmid M, Polterauer S, Bettelheim D. Isolated single umbilical artery: Evaluating the risk of adverse pregnancy outcome. Eur J Obstet Gynecol Reprod Biol 2015; 184: 80–83.

[4] Xu Y, Ren L, Zhai S, Luo X, Hong T, Liu R, et al. Association between isolated single umbilical artery and perinatal outcomes: A meta-analysis. Med Sci Monit 2016; 22: 1451– 1459.

[5] Gutvirtz G, Walfisch A, Beharier O, Sheiner E. Isolated single umbilical artery is an independent risk factor for perinatal mortality and adverse outcomes in term neonates. Arch Gynecol Obstet 2016; 294: 931–935.

[6] Li T-G, Wang G, Xie F, Yao J-M, Yang L, Wang M-L, et al. Prenatal diagnosis of single umbilical artery and postpartum outcome. Eur J Obstet Gynecol Reprod Biol 2020; 254: 6–10.

[7] Dagklis T, Siargkas A, Apostolopoulou A, Tsakiridis I, Mamopoulos A, Athanasiadis A, et al. Adverse perinatal outcomes following the prenatal diagnosis of isolated single umbilical artery in singleton pregnancies: A systematic review and meta-analysis. J Perinat Med 2021; 50: 244–252.

[8] Friebe-Hoffmann U, Hiltmann A, Friedl TWP, Lato K, Hammer R, Janni W, et al. Prenatally diagnosed single umbilical artery (SUA)-retrospective analysis of 1169 fetuses. Ultraschall Med 2019; 40: 221–229.

[9] Thummala MR, Raju TN, Langenberg P. Isolated single umbilical artery anomaly and the risk for congenital malformations: A meta-analysis. J Pediatr Surg 1998; 33: 580–585.

[10] Murphy-Kaulbeck L, Dodds L, Joseph KS, Van den Hof M. Single umbilical artery risk factors and pregnancy outcomes. Obstet Gynecol 2010; 116: 843–850.

[11] Csécsei K, Kovács T, Hinchliffe SA, Papp Z. Incidence and associations of single umbilical artery in prenatally diagnosed malformed, midtrimester fetuses: A review of 62 cases. Am J Med Genet 1992; 43: 524–530.

[12] Arcos-Machancoses JV, Marín-Reina P, Romaguera-Salort E, García-Camuñas Y, Pérez-Aytés A, Vento M. Postnatal development of fetuses with a single umbilical artery: Differences between malformed and non-malformed infants. World J Pediatr 2015; 11: 61–66.

[13] Lubinsky M. The VACTERL association: Mosaic mitotic aneuploidy as a cause and a model. J Assist Reprod Genet 2019; 36: 1549–1554.

[14] Firth D. Bias reduction of maximum likelihood estimates. Biometrika 1993; 80: 27–38.

[15] Benirschke K, Brown WH. A vascular anomaly of the umbilical cord; the absence of one umbilical artery in the umbilical cords of normal and abnormal fetuses. Obstet Gynecol 1955; 6: 399–404.

[16] Kim HJ, Kim JH, Chay DB, Park JH, Kim MA. Association of isolated single umbilical artery with perinatal outcomes: Systemic review and meta-analysis. Obstet Gynecol Sci 2017; 60: 266–273.

[17] Mitchell SE, Reidy K, Da Silva Costa F, Palma-Dias R, Cade TJ, Umstad MP. Congenital malformations associated with a single umbilical artery in twin pregnancies. Twin Res Hum Genet 2015; 18: 595–600.

[18] Lubinsky M. Embryonic hypocellularity, blastogenetic malformations, and fetal growth restriction. Am J Med Genet A 2017; 173: 151–156.

[19] Ahn JH, Choi HJ. Accompanied anomalies in anal atresia or tracheo-esophageal fistula: Comparison with or without VACTERL association. Birth Defects Res 2021; 113: 696– 701.

[20] Ebbing C, Kessler J, Moster D, Rasmussen S. Single umbilical artery and risk of congenital malformation: Population-based study in Norway. Ultrasound Obstet Gynecol 2020; 55: 510–515.

[21] Luo X, Zhai S, Shi N, Li M, Cui S, Xu Y, et al. The risk factors and neonatal outcomes of isolated single umbilical artery in singleton pregnancy. Sci Rep 2017; 7: 7396.

[22] Malova J, Bohmer D, Luha J, Pastorakova A, Cierna Z, Braxatorisova T. Single umbilical artery and reproduction losses in Slovak population: Relation to karyotype and fetal anomalies. Bratisl Lek Listy 2018; 119: 330–334.

[23] Heifetz SA. Single umbilical artery. A statistical analysis of 237 autopsy cases and review of the literature. Perspect Pediatr Pathol 1984; 8: 345–378.

[24] Dagklis T, Defigueiredo D, Staboulidou I, Casagrandi D, Nicolaides KH. Isolated single umbilical artery and fetal karyotype. Ultrasound Obstet Gynecol 2010; 36: 291–295.

[25] Martínez-Payo C, Cabezas E, Nieto Y, Ruiz de Azúa M, García-Benasach F, Iglesias E. Detection of single umbilical artery in the first trimester ultrasound: Its value as a marker of fetal malformation. Biomed Res Int 2014; 2014: 548729.

[26] de Boom ML, Kist-van Holthe JE, Sramek A, Lardenoye SWJ, Walther FJ, Lopriore E. Is screening for renal anomalies warranted in neonates with isolated single umbilical artery? Neonatology 2010; 97: 225–227.

[27] Tai C, Conner T, Pevyandi S, Moon-Grady AJ. Prevalence of congenital heart disease in an isolated single umbilical artery is low at a tertiary referral center. J Ultrasound Med 2021; 40: 1729–1730.

[28] Blum M, Weintraub AY, Baumfeld Y, Rotem R, Pariente G. Perinatal outcomes of small for gestational age neonates born with an isolated single umbilical artery. Front Pediatr 2019; 7: 79.