Journal article

Publication year: 2023

Journal: The Journal of Maternal-Fetal & Neonatal Medicine

Volume number: 36

Issue number: 1

ISSN: 1476-7058

eISSN: 1476-4954

DOI Link: https://doi.org/10.1080/14767058.2023.2203791

Publisher: Taylor and Francis Group

Abstract: 

Objective
This study aimed to analyze prenatal diagnosis, perinatal outcomes, and postnatal follow-up in fetuses with ectopia cordis (EC).

Methods
This retrospective analysis accessed 31 patients with EC who were either diagnosed or referred to a tertiary Fetal Medicine centers for EC diagnosis in Brazil, Germany, Italy, and Poland. We analyzed prenatal diagnosis, perinatal outcomes, and follow-up in these patients.

Results
Our study included a cohort of 31 fetuses with EC, 4 and 27 of whom had partial and complete protrusion of the heart through a ventral defect in the thoracoabdominal wall, respectively. EC was diagnosed by fetal echocardiography at a mean gestational age of 20.3 +/- 8.6 weeks (range, 8-35 weeks). Of the four cases, in which the karyotype was performed, all of them had a normal result (1 - 46,XX and 3 - 46,XY). Five patients showed conotruncal abnormalities and six ventricular septal defects. Termination of pregnancy (TOP) was performed in 15 cases (48%) and seven pregnant women had spontaneous fetal demise (22.5%). Of the seven fetuses that were born alive, four of them died, and three infants underwent surgery. Among these three infants, all of them survived, one was 5 months, 13 years old and 29 years old at the time of study completion.

Conclusions
Ectopia cordis is associated with high mortality rates and intracardiac/extra-cardiac defects. Ventricular septal defects and conotruncal anomalies were the more common intracardiac defects associated with EC. However, in this cohort of fetuses with EC the incidence of PC was lower than reported in the literature.

Harvard Citation style: Araujo Jr, E., Coutinho, L., Bravo-Valenzuela, N., Aquino, P., da Rocha, L., Rizzo, G., et al. (2023) Ectopia cordis: prenatal diagnosis, perinatal outcomes, and postnatal follow-up of an international multicenter cohort case series, The Journal of Maternal-Fetal & Neonatal Medicine, 36(1), Article 2203791. https://doi.org/10.1080/14767058.2023.2203791

APA Citation style: Araujo Jr, E., Coutinho, L., Bravo-Valenzuela, N., Aquino, P., da Rocha, L., Rizzo, G., Tonni, G., Respondek-Liberska, M., Slodki, M., Wolter, A., & Axt-Fliedner, R. (2023). Ectopia cordis: prenatal diagnosis, perinatal outcomes, and postnatal follow-up of an international multicenter cohort case series. The Journal of Maternal-Fetal & Neonatal Medicine. 36(1), Article 2203791. https://doi.org/10.1080/14767058.2023.2203791