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Characteristics and postoperational outcomes in paediatric patients with coronary sinus septal defect: a retrospective cohort study

Published online by Cambridge University Press:  26 March 2025

Chengcheng Pang
Affiliation:
Department of Maternal-Fetal Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China Department of Pediatric Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Huimin Wang
Affiliation:
Department of Radiology, Guangdong Provincial Key Laboratory of Artificial Intelligence in Medical Image Analysis and Application, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Junjun Shen
Affiliation:
Department of Maternal-Fetal Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China Department of Pediatric Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Liuqing Yang
Affiliation:
Department of Pediatric Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Jianglin Li
Affiliation:
Department of Pediatric Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Hui Liu
Affiliation:
Department of Radiology, Guangdong Provincial Key Laboratory of Artificial Intelligence in Medical Image Analysis and Application, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Wei Pan
Affiliation:
Department of Maternal-Fetal Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China Department of Pediatric Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
Zhiwei Zhang*
Affiliation:
Department of Maternal-Fetal Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China Department of Pediatric Cardiology, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China
*
Corresponding author: Zhiwei Zhang; Email: [email protected]

Abstract

Objective:

To summarise the characteristics and postoperative outcomes in paediatric patients with coronary sinus septal defect.

Method:

This retrospective study recruited paediatric patients diagnosed with coronary sinus septal defect from the Guangdong Cardiovascular Institute between 2011 and 2023. Clinical characteristics, echocardiographic parameters, surgical procedures, and postoperative outcomes were collected from electronic health records.

Results:

Among the 68 patients, 50% were male, with a median age of 1.0 years. Four cases (5.9%) were diagnosed during the prenatal period. The proportions of patients with type I, II, III, and IV coronary sinus septal defect were 51.5%, 5.9%, 16.1%, and 26.5%, respectively. The most common coexisting cardiac anomalies were persistent left superior caval vein. Twenty-seven cases were either missed or misdiagnosed by echocardiogram, accounting for 39.7% of the overall cases, with type I being the most frequently missed diagnosis. Fifty-four patients underwent surgery, two patients received transcutaneous intervention, while the remaining patients did not undergo any surgery or intervention. At follow-up, two patients with type I coronary sinus septal defect died from multiorgan dysfunction, and one patient underwent reoperation due to narrowing of the extracardiac tunnel. The remaining patients did not experience any major events and recovered well.

Conclusion:

Paediatric patients with coronary sinus septal defect often do not exhibit specific clinical manifestations. Enhancing our understanding of the anatomic and haemodynamic characteristics of coronary sinus septal defect can improve the diagnostic accuracy of echocardiography. If diagnosis is suspected, confirmation can be obtained by cardiac CT and cardiac magnetic resonance. Accurate preoperative and intraoperative diagnosis of coronary sinus septal defect contributes to high surgical success rates and favourable treatment outcomes.

Type
Original Article
Copyright
© The Author(s), 2025. Published by Cambridge University Press

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References

Zieliński, P, Konka, M, Hoffman, P, Różański, J, Michałowska, I. Unroofed coronary sinus atrial septal defect. J Cardiac Surg 2019; 34: 4142. doi: 10.1111/jocs.13971.CrossRefGoogle ScholarPubMed
Kobayashi, M, Muneuchi, J, Sugitani, Y, Yamada, A, Ezaki, H, Watanabe, M. Coronary anomalies associated with ventricular septal defect. Heart Vessels 2023; 38: 255264. doi: 10.1007/s00380-022-02144-6.CrossRefGoogle ScholarPubMed
Al-Dairy, A, Ahmad, R, Hasan, R. Misdiagnosis of persistent left superior vena cava with unroofed coronary sinus as a coronary sinus-type atrial septal defect. Clin Case Rep 2023; 11: e7826. doi: 10.1002/ccr3.7826.CrossRefGoogle ScholarPubMed
Selcuk, A, Korun, O, Sasmazel, A. Persistent left superior vena cava draining into the unroofed coronary sinus in a patient with tetralogy of fallot and coronary sinus orifice atresia. Cardiol Young 2023; 33: 11991200. doi: 10.1017/s1047951122003638.CrossRefGoogle Scholar
Yarrabolu, TR, Doshi, UH, Douglas, WI, Aly, AM, Balaguru, D. Unusual presentation of unroofed coronary sinus with cyanosis after ventricular septal defect closure. World J Pediatr Congenit Heart Surg 2015; 6: 8385. doi: 10.1177/2150135114543388.CrossRefGoogle ScholarPubMed
Xie, MX, Yang, YL, Cheng, TO, et al. Coronary sinus septal defect (unroofed coronary sinus): echocardiographic diagnosis and surgical treatment. Int J Cardiol 2013; 168: 12581263. doi: 10.1016/j.ijcard.2012.11.113.CrossRefGoogle ScholarPubMed
Aljizeeri, A, Dennie, CJ, Chan, KL, Beauchesne, LM. Unroofed coronary sinus atrial septal defect: diagnosis by multimodality cardiac imaging. Echocardiography (Mount Kisco, NY) 2014; 31: E228E229. doi: 10.1111/echo.12588.CrossRefGoogle ScholarPubMed
Chen, JL, Yu, CG, Wang, DJ, Chen, HB. Misdiagnosis of unroofed coronary sinus syndrome as an ostium primum atrial septal defect by echocardiography: a case report. World J Clin Cases 2022; 10: 15921597. doi: 10.12998/wjcc.v10.i5.1592.CrossRefGoogle Scholar
Tian, C, Su, W, Zhu, Z, Zhou, S, Fang, Z, Hu, X. Unroofed coronary sinus syndrome: a case report. Zhong nan da xue xue bao Yi xue ban = Journal of Central South University Medical sciences 2020; 45: 9195. doi: 10.11817/j.issn.1672-7347.2020.180767.Google ScholarPubMed
Zieliński, P, Kowalski, M, Kuśmierczyk, M, Hoffman, P, Michałowska, I. The coronary sinus interatrial connection with total unroofing coronary sinus discovered late after correction of secundum atrial septal defect. J Cardiac Surg 2020; 35: 31533154. doi: 10.1111/jocs.14990.CrossRefGoogle ScholarPubMed
Cossor, W, Addetia, K, Balkhy, HH, Spencer, KT, Patel, AR. Unroofed coronary sinus atrial septal defect: a multi-modality imaging approach. Eur Heart J Cardiovasc Imaging 2015; 16: 12631263. doi: 10.1093/ehjci/jev190.CrossRefGoogle ScholarPubMed
Gajjar, TP, Rao, JN, Desai, NB. Echocardiographic diagnosis and surgical closure of coronary sinus type of atrial septal defect. J Cardiac Surg 2011; 26: 653656. doi: 10.1111/j.1540-8191.2011.01340.x.CrossRefGoogle ScholarPubMed
Yeager, SB, Chin, AJ, Sanders, SP. Subxiphoid two-dimensional echocardiographic diagnosis of coronary sinus septal defects. Am J Cardiol 1984; 54: 686687. doi: 10.1016/0002-9149(84)90282-0.CrossRefGoogle ScholarPubMed
Goyal, SK, Punnam, SR, Verma, G, Ruberg, FL. Persistent left superior vena cava: a case report and review of literature. Cardiovasc Ultrasoun 2008; 6: 50. doi: 10.1186/1476-7120-6-50.CrossRefGoogle ScholarPubMed
Rees, S, Tahir, M, Ahmad, SJ, Subkovas, E. Rare case of right ventricular dilatation associated with anomalous pulmonary venous drainage, sinus venosus atrial septal defect and persistent left superior vena cava. BMJ case reports 2021; 14: e239687. doi: 10.1136/bcr-2020-239687.CrossRefGoogle ScholarPubMed
Kutty, S, Biko, DM, Goldberg, AB, Quartermain, MD, Feinstein, SB. Contrast-enhanced ultrasound in pediatric echocardiography. Pediatr Radiol 2021; 51: 24082417. doi: 10.1007/s00247-021-05119-3.CrossRefGoogle ScholarPubMed
Urmeneta Ulloa, J, de Célix MDC, Gómez Rubín, Cabrera, J, Álvarez Vázquez, A, de Vega, VMartínez. Unroofed coronary sinus atrial septal defect: 4D flow cardiovascular magnetic resonance vs. cardiac computed tomography. Eur Heart J Cardiovasc Imaging 2020; 21: 10471047. doi: 10.1093/ehjci/jeaa071.CrossRefGoogle ScholarPubMed
Sugimori, H, Nakao, T, Ikegaya, Y, et al. Coronary sinus atrial septal defects in adults over the past 20 years at new Tokyo hospital: case series. J Cardiothorac Surg 2021; 16: 150. doi: 10.1186/s13019-021-01522-x.CrossRefGoogle ScholarPubMed
Levy, PT, Thomas, AR, Wethall, A, Perez, D, Steurer, M, Ball, MK. Rethinking congenital heart disease in preterm neonates. Neoreviews 2022; 23: e373e387. doi: 10.1542/neo.23-6-e373.CrossRefGoogle ScholarPubMed
Nasr, VG, Markham, LW, Clay, M, et al. Perioperative considerations for pediatric patients with congenital heart disease presenting for noncardiac procedures: a scientific statement from the American heart association. Circ Cardiovasc Qual Outcomes 2023; 16: e000113. doi: 10.1161/hcq.0000000000000113.CrossRefGoogle ScholarPubMed
van der Bom, T, Zomer, AC, Zwinderman, AH, Meijboom, FJ, Bouma, BJ, Mulder, BJ. The changing epidemiology of congenital heart disease. Nat Rev Cardiol 2011; 8: 5060. doi: 10.1038/nrcardio.2010.166.CrossRefGoogle ScholarPubMed