Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 16, 2022; 10(5): 1592-1597
Published online Feb 16, 2022. doi: 10.12998/wjcc.v10.i5.1592
Misdiagnosis of unroofed coronary sinus syndrome as an ostium primum atrial septal defect by echocardiography: A case report
Jin-Ling Chen, Cai-Gui Yu, Dai-Jiao Wang, Hong-Bin Chen
Jin-Ling Chen, Cai-Gui Yu, Dai-Jiao Wang, Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
Hong-Bin Chen, Department of Pulmonary and Critical Care Medicine, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
Author contributions: Chen JL designed the study and wrote the paper; Chen HB collected the patient’s clinical data; Yu CG and Wang DJ analyzed the data.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the 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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Hong-Bin Chen, PhD, Chief Physician, Department of Pulmonary and Critical Care Medicine, Renmin Hospital of Wuhan University, No. 238 Jiefang Road, Wuchang District, Wuhan 430060, Hubei Province, China. rainman1974@yeah.net
Received: August 10, 2021
Peer-review started: August 10, 2021
First decision: October 16, 2021
Revised: October 24, 2021
Accepted: January 11, 2022
Article in press: January 11, 2022
Published online: February 16, 2022
Processing time: 184 Days and 17.2 Hours
Abstract
BACKGROUND

Unroofed coronary sinus syndrome (UCSS) is a rare congenital heart disease, which has variable morphologic features and is strongly associated with persistent left superior vena cava (PLSVC). However, it is often difficult to visualize the left-to-right shunt pathway through the CS by transthoracic echocardiography (TTE).

CASE SUMMARY

A 37-year-old female was admitted to the hepatological surgery department of a hospital with complaint of subxiphoid pain that had started 1 wk prior. Physical examination revealed a grade 3/6 systolic murmur at the left margin of the sternum, between the 2nd and 3rd intercostal cartilage. The patient underwent echocardiography and was diagnosed with ostium primum atrial septal defect (ASD); thus, she was subsequently transferred to the cardiovascular surgery department. A second TTE evaluation before surgery showed type IV UCSS with secundum ASD. Right-heart contrast echocardiography (RHCE) showed that the right atrium and right ventricle were immediately filled with microbubbles, but no microbubble was observed in the CS. Meanwhile, negative filling was observed at the right atrium orifice of the CS and right atrium side of the secundum atrial septal. RHCE identified UCSS combined with secundum ASD but without PLSVC in this patient.

CONCLUSION

This rare case of UCSS highlights the value of TTE combined with RHCE in confirming UCSS with ASD or PLSVC.

Keywords: Congenital heart disease; Coronary sinus; Atrial septal defect; Persistent left superior vena cava; Echocardiography; Right heart contrast echocardiography; Case report

Core Tip: Unroofed coronary sinus syndrome (UCSS) is difficult to diagnose. Transthoracic echocardiography (TTE) of a 37-year-old female revealed ostium primum atrial septal defect (ASD). A second TTE showed type IV UCSS with secundum ASD. Right-heart contrast echocardiography (RHCE) confirmed UCSS and ASD with no persistent left superior vena cava (PLSVC). The patient was misdiagnosed because the defect location was near the endocardial cushions, which was mistaken for a defect of the ostium primum atrial septum. This case highlights the special value of TTE and RHCE for a rare case of type IV UCSS combined with ASD but without PLSVC.