Bitar F, Bulbul Z, Jassar Y, Zareef R, Abboud J, Arabi M, Bitar FF. Unroofed coronary sinus, left-sided superior vena cava and mitral insufficiency: A case report and review of the literature. World J Cardiol 2024; 16(10): 595-603 [PMID: 39492968 DOI: 10.4330/wjc.v16.i10.595]
Corresponding Author of This Article
Ziad Bulbul, MD, Doctor, Department of Pediatric and Adolescent, American University of Beirut Medical Center, Beirut-Hamra-Cairo Street, Beirut 1103, Lebanon. zb21@aub.edu.lb
Research Domain of This Article
Cardiac & Cardiovascular Systems
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Fouad Bitar, Ziad Bulbul, Rana Zareef, Jennifer Abboud, Mariam Arabi, Fadi Fouad Bitar, Department of Pediatric and Adolescent, American University of Beirut Medical Center, Beirut 1103, Lebanon
Yehya Jassar, Department of Surgery, American University of Beirut Medical Center, Beirut 1103, Lebanon
Author contributions: Bulbul Z conceived the presented idea and the study framework; Bitar F, Jassar Y, Zareef R, Bulbul Z and Abboud J collected the information and wrote the first draft of the manuscript; Bitar FF, Arabi M and Bulbul Z supervised the project and performed the final editing; All authors contributed to corrections and adjustment of subsequent iterations of the manuscript; All authors have read and approved the final manuscript.
Informed consent statement: The patient provided informed oral consent for the publication of the study data.
Conflict-of-interest statement: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
CARE Checklist (2016) statement: The authors have read CARE Checklist (2016), and the manuscript was prepared and revised according to CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Ziad Bulbul, MD, Doctor, Department of Pediatric and Adolescent, American University of Beirut Medical Center, Beirut-Hamra-Cairo Street, Beirut 1103, Lebanon. zb21@aub.edu.lb
Received: February 14, 2024 Revised: August 21, 2024 Accepted: September 11, 2024 Published online: October 26, 2024 Processing time: 246 Days and 1.8 Hours
Abstract
BACKGROUND
Unroofed coronary sinus (UCS) is a rare subtype of atrial septal defect. It is frequently associated with a persistent left superior vena cava and is often part of a more intricate cardiac malformation.
CASE SUMMARY
This report describes a rare case of an adolescent patient with UCS featuring atrial situs solitus, absence of the right superior vena cava and a persistent left superior vena cava draining into the left atrium consistent with total unroofing of the coronary sinus. This was associated with concurrent severe mitral insufficiency secondary to redundant and prolapsing leaflets, and a substantial left-to-right shunt across the coronary sinus orifice. A comprehensive examination of the existing literature is included, shedding light on the diagnostic challenges of UCS and describing the available surgical options within the context of mitral valve surgery.
CONCLUSION
UCS is a complex condition requiring careful consideration of associated anomalies and a tailored surgical approach.
Core Tip: In this report, we present an exceedingly rare case of a teenager with an unroofed coronary sinus and a single persistent left superior vena cava in conjugation with severe mitral regurgitation secondary to redundant and prolapsing leaflets, in the absence of other associated cardiac anomalies. This peculiar condition was detected by echocardiography and corrected by appropriate surgical intervention.
