Hostname: page-component-cd9895bd7-8ctnn Total loading time: 0 Render date: 2024-12-18T13:19:48.341Z Has data issue: false hasContentIssue false

Anatomical considerations for the management of a left-sided superior caval vein draining to the roof of the left atrium

Published online by Cambridge University Press:  29 September 2016

Jelena Saundankar
Affiliation:
Department of Congenital Cardiology, Southampton General Hospital, Southampton, United Kingdom
Andrew B. Ho*
Affiliation:
Department of Congenital Cardiology, Southampton General Hospital, Southampton, United Kingdom
Anthony P. Salmon
Affiliation:
Department of Congenital Cardiology, Southampton General Hospital, Southampton, United Kingdom
Robert H. Anderson
Affiliation:
Department of Congenital Cardiology, Southampton General Hospital, Southampton, United Kingdom
Alan G. Magee
Affiliation:
Department of Congenital Cardiology, Southampton General Hospital, Southampton, United Kingdom
*
Correspondence to: Dr A. Ho, Department of Congenital Cardiology, Southampton General Hospital, Southampton SO16 6YD, United Kingdom. Tel: 023 8077 7222; Fax: 023 8120 4526; E-mail: [email protected]

Abstract

Aims

The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management.

Methods and Results

Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug.

Conclusions

Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Type
Original Articles
Copyright
© Cambridge University Press 2016 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Biffi, M, Boriani, G, Frabetti, L, Bronzetti, G, Branzi, A. Left superior vena cava persistence in patients undergoing pacemaker or cardioverter-defibrillator implantation: a 10-year experience. Chest 2001; 120: 139144.CrossRefGoogle ScholarPubMed
2. Parikh, SR, Prasad, K, Iyer, RN, Desai, N, Mohankrishna, L. Prospective angiographic study of the abnormalities of systemic venous connections in congenital and acquired heart disease. Cathet Cardiovasc Diagn 1996; 38: 379386.3.0.CO;2-A>CrossRefGoogle ScholarPubMed
3. Tuchman, H, Brown, JF, Huston, JH, Weinstein, AB, Rowe, GG, Crumpton, CW. Superior vena cava draining into left atrium; another cause for left ventricular hypertrophy with cyanotic congenital heart disease. Am J Med 1956; 21: 481484.CrossRefGoogle Scholar
4. Raghib, G, Ruttenberg, HD, Andersen, RC, Amplatz, K, Adams, 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: 906918.CrossRefGoogle ScholarPubMed
5. Davis, WH, Jordaan, FR, Snyman, HW. Persistent left superior vena cava draining into the left atrium, as an isolated anomaly. Am Heart J 1959; 57: 616622.Google Scholar
6. 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: 948953.CrossRefGoogle ScholarPubMed
7. de Leval, MR, Ritter, DG, McGoon, DC, Danielson, GK. Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc 1975; 50: 599610.Google ScholarPubMed
8. Zimand, S, Benjamin, P, Frand, M, Mishaly, D, Smolinsky, AK, Hegesh, J. Left superior vena cava to the left atrium: do we have to change the traditional approach? Ann Thorac Surg Elsevier 1999; 68: 18691871.Google Scholar
9. Mutialu, N, Fajardo, D, Sullivan, ID, Tsang, V. Repair of persistent left superior vena cava to unroofed coronary sinus defect by retro-aortic implantation (Modified Warden Type procedure). J Card Surg 2016; 32: 103105.CrossRefGoogle Scholar
10. Troost, E, Gewillig, M, Budts, W. Percutaneous closure of a persistent left superior vena cava connected to the left atrium. Int J Cardiol 2006; 106: 365366.CrossRefGoogle Scholar
11. Geggel, RL, Perry, SB, Blume, ED, Baker, CM. Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device. Catheter Cardiovasc Interv 1999; 48: 369373.3.0.CO;2-1>CrossRefGoogle ScholarPubMed
12. Thal, S, Boyella, R, Arsanjani, R, et al. Unusual combination of holt-oram syndrome and persistent left superior vena cava. Congenit Heart Dis 2012; 7: E46E49.CrossRefGoogle ScholarPubMed
13. Kirklin, JW, Barratt-Boyes, BG. Cardiac Surgery. Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications . Wiley, New York, USA, 1986.Google Scholar