Unroofed Coronary Sinus, Left-sided Superior Vena Cava and Mitral Insufficiency: A Case Report and Review of the Literature

Overview

Journal World J Cardiol

Publisher Baishideng Publishing Group

Specialty Cardiology & Vascular Diseases

Date 2024 Nov 4

PMID 39492968

Authors

Fouad Bitar

Ziad Bulbul

Yehya Jassar

Rana Zareef

Jennifer Abboud

Mariam Arabi

Fadi Fouad Bitar

Affiliations

Soon will be listed here.

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.

References

Brown J, CRUMPTON C, HUSTON J, ROWE G, Tuchman H, WEINSTEIN A . Superior vena cava draining into left atrium; another cause for left ventricular hypertrophy with cyanotic congenital heart disease. Am J Med. 1956; 21(3):481-4. DOI: 10.1016/0002-9343(56)90047-x. View

Bertram H, Paul T, Kaulitz R, Luhmer I, Kallfelz H . [Coronary sinus defects: rare form of interatrial communication]. Z Kardiol. 1996; 85(12):899-905. View

Yamada K, Miyazaki S, Oboshi M, Suezawa C, Sakakibara Y, Nishimura K . Surgical repair of unroofed coronary sinus with severe mitral regurgitation in an elderly patient. J Cardiol Cases. 2019; 19(1):29-32. PMC: 6342724. DOI: 10.1016/j.jccase.2018.09.002. View

Martinez-Villar M, Gran F, Ferrer Q, Giralt G, Sabate-Rotes A, Albert D . Persistent Left Superior Vena Cava With Absent Right Superior Vena Cava. Rev Esp Cardiol (Engl Ed). 2015; 69(2):220-1. DOI: 10.1016/j.rec.2015.10.002. View

Xie M, Yang Y, Cheng T, Wang X, Li K, Ren P . Coronary sinus septal defect (unroofed coronary sinus): echocardiographic diagnosis and surgical treatment. Int J Cardiol. 2012; 168(2):1258-63. DOI: 10.1016/j.ijcard.2012.11.113. View

Wang C, Ma L, Li Y, Pan X . Percutaneous repair of unroofed coronary sinus with an atrial septal occluder. Eur Heart J Case Rep. 2023; 7(2):ytad032. PMC: 9897234. DOI: 10.1093/ehjcr/ytad032. View

Choi J, Anderson R, Macartney F . Absent right superior caval vein (vena cava) with normal atrial arrangement. Br Heart J. 1987; 57(5):474-8. PMC: 1277203. DOI: 10.1136/hrt.57.5.474. View

Doksoz O, Guven B, Yozgat Y, Ozdemir R, Mese T, Tavli V . Absent right superior vena cava with persistent left superior vena cava which drains to unroofed coronary sinus in a child with atrioventricular septal defect and cor triatriatum sinister: preop correct diagnosis and successful surgery in a single.... Congenit Heart Dis. 2013; 9(2):E37-40. DOI: 10.1111/chd.12065. View

Sarwar G, Ahmed B, Suleman N, Khan G . Complicated unroofed coronary sinus syndrome. J Coll Physicians Surg Pak. 2005; 15(3):171-3. DOI: 03.2005/JCPSP.171173. View

10.

Chemtob R, Mick S, Gillinov M, Hodges K, Wierup P . Repair of bileaflet prolapse in Barlow syndrome: The 4-chord technique. J Card Surg. 2019; 34(7):605-609. DOI: 10.1111/jocs.14078. View

11.

RAGHIB G, Ruttenberg H, Anderson R, AMPLATZ K, ADAMS Jr P, Edwards J . TERMINATION OF LEFT SUPERIOR VENA CAVA IN LEFT ATRIUM, ATRIAL SEPTAL DEFECT, AND ABSENCE OF CORONARY SINUS; A DEVELOPMENTAL COMPLEX. Circulation. 1965; 31:906-18. DOI: 10.1161/01.cir.31.6.906. View

12.

Konstantinides S, van Tournout F, Bennett J . Unroofed coronary sinus associated with atrial septal defect and significant mitral regurgitation. J Thorac Cardiovasc Surg. 1990; 99(2):377-8. View

13.

Bartram U, Van Praagh S, Levine J, Hines M, Bensky A, van Praagh R . Absent right superior vena cava in visceroatrial situs solitus. Am J Cardiol. 1997; 80(2):175-83. DOI: 10.1016/s0002-9149(97)00314-7. View

14.

Egoh Y, Isomatsu Y, Kurosawa H, Kawana M, Iwasaki T, Takahashi S . [A case of unroofed coronary sinus with mitral and tricuspid valve regurgitation]. Nihon Kyobu Geka Gakkai Zasshi. 1989; 37(4):755-9. View

15.

Sheikh A, Mazhar S . Persistent left superior vena cava with absent right superior vena cava: review of the literature and clinical implications. Echocardiography. 2014; 31(5):674-9. DOI: 10.1111/echo.12514. View

16.

Kumar V, Thingnam S, Soni R, Walia S, Kumar B . Absent right superior vena cava with left superior vena cava draining into unroofed coronary sinus with common atrium in a child with an atrioventricular septal defect. J Card Surg. 2020; 35(7):1743-1745. DOI: 10.1111/jocs.14686. View

17.

Gajjar T, Rao J, Desai N . Echocardiographic diagnosis and surgical closure of coronary sinus type of atrial septal defect. J Card Surg. 2011; 26(6):653-6. DOI: 10.1111/j.1540-8191.2011.01340.x. View

18.

Chaturvedi A, Dubinsky T, Maki J . MR findings of a rare defect, coronary sinus ASD. Int J Cardiovasc Imaging. 2010; 28(2):429-30. DOI: 10.1007/s10554-010-9670-8. View

19.

Sun T, Fei H, Huang H, Chen O, Zheng Z, Zhang C . Transesophageal echocardiography for coronary sinus imaging in partially unroofed coronary sinus. Echocardiography. 2013; 31(1):74-82. PMC: 4232913. DOI: 10.1111/echo.12302. View

20.

Shi H, Yan J, Wang Q, Hua Z, Li S, Zhang J . Surgical management of unroofed coronary sinus syndrome: A 20-year-single-center experience. J Card Surg. 2020; 36(2):589-595. DOI: 10.1111/jocs.15269. View