Introduction Congenital malformations of the right atrium are rare heart defects with only a few cases described prenatally. Early diagnosis of these anomalies is becoming increasingly important for proper follow-up and due to the possibility of serious complications such as supraventricular arrhythmia, thromboembolic events, and sudden death.

Objective The atrial appendage aneurysm (AAA) is a dilatation of the atrial appendage. It is considered an extremely rare congenital anomaly. However, this condition is clinically significant because it leads to atrial arrhythmias, recurrent emboli, heart failure, and chest pain. In addition, it is possible to recognize AAA prenatally with fetal echocardiography, even if it rarely happens. However, few fetal AAA cases have been reported in the literature.

Study Design We report a case of a fetal AAA; diagnosed prenatally and with postnatal confirmation. We undertook a systematic review of studies on fetal AAA to synthesize available knowledge on diagnosing and managing this rare condition.

Results A total of eight studies describing 24 patients were identified and analyzed.

Conclusion Despite their rarity, fetal atrial appendage aneurysms necessitate early detect on due to associated severe complications. Our findings emphasize the importance of prenatal diagnosis through fetal echocardiography and highlight the need for further research to optimize management strategies and improve outcomes for affected individuals.

This work has been approved by the Local Ethics Committee of the Women's Clinic, Cartagena, Colombia.

J.M. contributed with ultrasound data, wrote the first draft of the manuscript, reviewed and analyzed the literature, and wrote the final version. N.A. contributed with postnatal images and analysis of the literature. Furthermore, D.V. contributed with ultrasound data, article writing, and data analysis and B.C. contributed with postnatal images and analysis of the literature. All authors reviewed and approved the final version of the manuscript.

Received: 26 January 2024

Accepted: 27 March 2024

Article published online:
04 June 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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