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Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus

Published online by Cambridge University Press:  24 October 2007

Christine H. Attenhofer Jost ,
Heidi M. Connolly ,
Gordon K. Danielson ,
Joseph A. Dearani ,
Carole A. Warnes  and
A. Jamil Tajik
Christine H. Attenhofer Jost
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
Heidi M. Connolly*
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
Gordon K. Danielson
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
Joseph A. Dearani
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
Carole A. Warnes
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
A. Jamil Tajik
Affiliation:
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
*
Correspondence to: Heidi M. Connolly MD, Division of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester MN 55905, USA. E-mail: [email protected]
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Abstract

Objective

To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with partial fenestrations of the coronary sinus, a rare congenital disorder.

Background

Partial fenestrations of the walls that usually separate the coronary sinus from the left atrium, also known as partial unroofing of the coronary sinus, may result in increased flow of blood to the lungs, cyanosis, or bidirectional shunting. The diagnosis is important, but difficult to confirm.

Methods

We reviewed retrospectively the clinical, echocardiographic, operative, and follow-up data on 25 patients with partial fenestration of the coronary sinus. The patients had a median age of 8 years, and underwent cardiovascular surgery at Mayo Clinic between 1958 and 2003.

Results

The initial diagnosis of a fenestration of the coronary sinus was made by the surgeon at repair of other congenital cardiac anomalies, by cardiac catheterization, or by echocardiography. In 14 patients, fenestration was missed during previous cardiovascular surgery. The most common associated cardiac lesions were atrial septal defects within the oval fossa, persistent left or right superior caval veins, and pulmonary or tricuspid atresia. In 7 patients, the symptoms were at least partially attributable to the fenestration, and included dyspnea, cerebral abscess, transient ischaemic attacks, and cyanosis. The fenestration was addressed surgically in 23 patients, and consisted of closure of the mouth of the coronary sinus, creation of an intra-atrial baffle, or direct repair of the fenestration. Death occurred in 1 patient due to complications unrelated to the repair. In the 24 early survivors, who have been followed up for a median of 85 months, 1 patient has died after a third reoperation for complex congenital cardiac disease.

Conclusions

Fenestrations of the coronary sinus are often missed in the preoperative evaluation of congenitally malformed hearts. When associated with right heart hypoplasia, atrial septal defect, or persistent superior caval vein, fenestrations of the coronary sinus should be considered as a possible additional finding. Once the diagnosis has been made, repair is straightforward.

Keywords

Congenital heart diseasediagnosisechocardiographyheart surgery
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Issue 6 , December 2007 , pp. 592 - 600
Copyright
Copyright © Cambridge University Press 2007

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References

1. Raghib, G, Ruttenberg, HD, Anderson, RC, Amplatz, K, JrAdams, P, Edwards, JE. Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus: a developmental complex. Circulation 1965; 31: 906–918.CrossRefGoogle ScholarPubMed
2. Freedom, RM, Culham, JA, Rowe, RD. Left atrial to coronary sinus fenestration (partially unroofed coronary sinus): morphological and angiocardiographic observations. Br Heart J 1981; 46: 63–68.CrossRefGoogle ScholarPubMed
3. Jacobs, JP, Quintessenza, JA, Burke, RP, Mavroudis, C. Congenital Heart Surgery Nomenclature and Database Project: atrial septal defect. Ann Thorac Surg 2000; 69 Suppl: S18–S24.CrossRefGoogle ScholarPubMed
4. Chauvin, M, Shah, DC, Haissaguerre, M, Marcellin, L, Brechenmacher, C. The anatomic basis of connections between the coronary sinus musculature and the left atrium in humans. Circulation 2000; 101: 647–652.CrossRefGoogle ScholarPubMed
5. Knauth, A, McCarthy, KP, Webb, S, et al. . Interatrial communication through the mouth of the coronary sinus. Cardiol Young 2002; 12: 364–372.CrossRefGoogle ScholarPubMed
6. Nakatani, S, Katsuki, K, Miyatake, K. Images in cardiology: unroofed coronary sinus. Heart 2002; 87: 278.CrossRefGoogle ScholarPubMed
7. Adatia, I, Gittenberger-de Groot, AC. Unroofed coronary sinus and coronary sinus orifice atresia: implications for management of complex congenital heart disease. J Am Coll Cardiol 1995; 25: 948–953.CrossRefGoogle ScholarPubMed
8. Quaegebeur, J, Kirklin, JW, Pacifico, AD, JrBargeron, LM. Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979; 27: 418–425.CrossRefGoogle ScholarPubMed
9. Becker, AE, Anderson, RH. Pathology of congenital heart disease. Butterworths, London, Boston, 1981.Google Scholar
10. Schiller, NB, Shah, PM, Crawford, M, et al. . American Society of Echocardiography Committee on Standards, Subcommittee on Quantitation of Two-Dimensional Echocardiograms. Recommendations for quantitation of the left ventricle by two-dimensional echocardiography. J Am Soc Echocardiogr 1989; 2: 358–367.CrossRefGoogle ScholarPubMed
11. Ootaki, Y, Yamaguchi, M, Yoshimura, N, Oka, S, Yoshida, M, Hasegawa, T. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg 2003; 126: 1655–1656.CrossRefGoogle ScholarPubMed
12. Matsuwaka, R, Tomokuni, T, Ishikawa, S, Watanabe, F, Matsushita, T, Matsuda, H. Partially unroofed coronary sinus associated with tricuspid atresia: an important associated lesion in the Fontan operation. Eur J Cardiothorac Surg 1987; 1: 180–182.CrossRefGoogle ScholarPubMed
13. Rose, AG, Beckman, CB, Edwards, JE. Communication between coronary sinus and left atrium. Br Heart J 1974; 36: 182–185.CrossRefGoogle ScholarPubMed
14. Chin, AJ, Murphy, JD. Identification of coronary sinus septal defect (unroofed coronary sinus) by color Doppler echocardiography. Am Heart J 1992; 124: 1655–1657.CrossRefGoogle ScholarPubMed
15. Yeager, SB, Chin, AJ, Sanders, SP. Subxiphoid two-dimensional echocardiographic diagnosis of coronary sinus septal defects. Am J Cardiol 1984; 54: 686–687.CrossRefGoogle ScholarPubMed
16. Sunaga, Y, Hayashi, K, Okubo, N, et al. . Transesophageal echocardiographic diagnosis of coronary sinus type atrial septal defect. Am Heart J 1992; 124: 1657–1659.CrossRefGoogle ScholarPubMed
17. Uemura, H, Ho, SY, Anderson, RH, et al. . The surgical anatomy of coronary venous return in hearts with isomeric atrial appendages. J Thorac Cardiovasc Surg 1995; 110: 436–444.CrossRefGoogle ScholarPubMed
18. Chen, MC, Hung, JS, Chang, KC, Lo, PH, Chen, YC, Fu, M. Partially unroofed coronary sinus and persistent left superior vena cava: intracardiac echocardiographic observation. J Ultrasound Med 1996; 15: 875–879.CrossRefGoogle ScholarPubMed
19. Brancaccio, G, Miraldi, F, Ventriglia, F, Michielon, G, Di Donato, RM, De Santis, M. Multidetector-row helical computed tomography imaging of unroofed coronary sinus. Int J Cardiol 2003; 91: 251–253.CrossRefGoogle ScholarPubMed
20. de Leval, MR, Ritter, DG, McGoon, DC, Danielson, GK. Anomalous systemic venous connection: surgical considerations. Mayo Clin Proc 1975; 50: 599–610.Google ScholarPubMed
21. van Son, JA, Hambsch, J, Mohr, FW. Repair of complex unroofed coronary sinus by anastomosis of left to right superior vena cava. Ann Thorac Surg 1998; 65: 280–281.CrossRefGoogle ScholarPubMed
22. Di Bernardo, S, Fasnacht, M, Berger, F. Transcatheter closure of a coronary sinus defect with an Amplatzer septal occluder. Catheter Cardiovasc Interv 2003; 60: 287–290.CrossRefGoogle ScholarPubMed
23. Ferreira Martins, JD, Anderson, RH. The anatomy of interatrial communications: what does the interventionist need to know? Cardiol Young 2000; 10: 464–473.CrossRefGoogle ScholarPubMed