Skip Navigation

European Journal of Echocardiography 2007 8(5):398-401; doi:10.1016/j.euje.2006.06.004
This Article
Right arrow Abstract Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Kong, P.K.
Right arrow Articles by Ahmad, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Kong, P.K.
Right arrow Articles by Ahmad, F.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Copyright © 2006, The European Society of Cardiology

Unroofed coronary sinus and persistent left superior vena cava

P.K. Kong* and F. Ahmad

Department of Cardiology, Russells Hall Hospital, Dudley Group of Hospitals NHS Trust, Pensnett Road, Dudley DY1 2HQ, United Kingdom

Received 13 February 2006; received in revised form 26 May 2006; accepted after revision 2 June 2006.

pkdd210{at}yahoo.co.uk

* Corresponding author. Tel.: +44 121 4261462.


   Abstract
Top
 Abstract
Introduction
 Case study
 Discussion
 Conclusion
 References
 
This report describes a case of unroofed coronary sinus and persistent left superior vena cava discovered during an echocardiographic investigation for dilated pulmonary artery. An unroofed coronary sinus is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava. The latter is a usual cause of a dilated coronary sinus. The detection of a dilated coronary sinus should therefore prompt the search for abnormal coronary sinus drainage and other cardiac abnormalities. The treatment of unroofed coronary sinus and persistent left superior vena cava is undertaken only after assessing the pre- and post-treatment haemodynamics of all co-existing abnormalities.

Keywords: Unroofed coronary sinus; Persistent left superior vena cava; Congenital heart disease; Echocardiography


   Introduction
Top
 Abstract
 Introduction
Case study
 Discussion
 Conclusion
 References
 
The complex of an unroofed coronary sinus (UCS) and a persistent left superior vena cava (PLSVC) is a rare congenital heart disease first described by Raghib et al. in 1965.1 A normal coronary sinus drains the cardiac veins into the right atrium. A UCS, in addition to draining the cardiac veins, also communicates abnormally with the left atrium. This abnormal communication is thought to be due to impaired development of the partition between the left atrium and the coronary sinus – an alternative explanation is subsequent dissolution of this partition.2 A PLSVC, abnormally draining the left internal jugular and subclavian veins into the coronary sinus, is due to impaired degeneration of the embryonic left counterpart of the normal right superior vena cava.3 A UCS or a PLSVC may be further associated with other cardiac abnormalities.


   Case study
Top
 Abstract
 Introduction
 Case study
Discussion
 Conclusion
 References
 
A 65-year-old woman presented with haemoptysis attributed to chest infection. She did not have breathlessness or cyanosis and the haemoptysis resolved. Her chest X-ray was suggestive of a dilated right pulmonary artery subsequently confirmed with a thoracic computed tomography. A bronchoscopy ruled out bronchial malignancy. Her electrocardiograph showed right bundle branch block with normal axis.

She was referred for an echocardiographic evaluation of her right heart. A transthoracic echocardiography showed a left-to-right shunt from the left atrium into a dilated UCS. The atria, right ventricle and main pulmonary artery were also dilated (Figs. 1–3GoGo). The right ventricle showed volume overload with diastolic flattening of the interventricular septum and the pulmonary artery systolic pressure was mildly raised. The biventricular systolic function was good.


Figure 1
View larger version (26K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 1 Parasternal long axis (PLAX) view showing a dilated coronary sinus, left atrium and right ventricle.

 


Figure 2
View larger version (20K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 2 Apical 2-chamber (A2C) view showing a dilated unroofed coronary sinus (UCS) and a dilated left atrium.

 


Figure 3
View larger version (23K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 3 Posteriorly angulated apical 4-chamber (A4C) view showing a dilated coronary sinus (CS), right atrium and right ventricle.

 
Normal saline agitated with patient's blood was injected into patient's left antecubital vein. Bubble contrast appeared in the coronary sinus before appearing in the right heart thus confirming a PLSVC (Fig. 4). Some bubble contrast also appeared in the left atrium consistent with some right-to-left shunt but the shunt is mainly left-to-right. Not all the pulmonary veins were visualised and associated thoracic venous abnormalities such as anomalous pulmonary venous drainage could not be ruled out.


Figure 4
View larger version (26K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 4 Posteriorly angulated apical 4-chamber (A4C) view showing saline bubble contrast in the coronary sinus confirming the presence of a persistent left superior vena cava.

 
She therefore had a transoesophageal echocardiography that showed partial anomalous pulmonary venous drainage (PAPVD) with the right pulmonary veins emptying into the superior vena cava. The left pulmonary veins were drained normally. A repeat saline bubble contrast study confirmed the PLSVC (Fig. 5). No atrial or ventricular septal defect was seen. The echocardiographer detected colour flow from the left atrium into the coronary sinus but could not detect the UCS per se.


Figure 5
View larger version (30K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 5 Transoesophageal echocardiography confirming the presence of a persistent left superior vena cava with saline bubble contrast. Note the diastolic flattening of the interventricular septum due to right ventricular overload causing a D-shaped left ventricle.

 
The patient proceeded to having a right cardiac catheter that crossed aberrantly into the left atrium and left ventricle signifying an interatrial shunt not detected on the transoesophageal echocardiography. A repeat analysis of the transoesophageal echocardiography revealed crossing of some bubbles from the coronary sinus into the left heart. The UCS was therefore detected on the transthoracic echocardiography and right cardiac catheterisation but not fully on the transoesophageal echocardiography. The right cardiac catheterisation also revealed a pulmonary artery systolic pressure at upper limit of normal, shunt ratio of 2.2:1, normal pulmonary vascular resistance and step-up in the oxygen saturation at the level of high right atrium.

She was referred to our regional centre for grown-up congenital heart disease and was treated conservatively with annual echocardiography. The patient remains well two years after her index haemoptysis.


   Discussion
Top
 Abstract
 Introduction
 Case study
 Discussion
Conclusion
 References
 
UCS and PLSVC may cause no symptoms or may cause right ventricular failure,4 paradoxical cerebral embolism and cerebral abscess,5 or cyanosis that may vary with neck position.6 In this case report, the haemoptysis is attributed to chest infection but may also be related to the increased pulmonary blood flow from the shunt.

A PLSVC is typically suspected when aberrant catheterisation of the pulmonary artery is demonstrated on chest X-ray after catheterisation via the left internal jugular or subclavian veins.3 If a UCS is also present, catheterisation of the left heart and aorta is possible7 and can therefore cause spurious ‘pulmonary hypertension’, aortic vascular injuries and embolism.

UCS and PLSVC may be further associated with other cardiac abnormalities such as atrioventricular septal defect, atrial appendage anomalies and coronary sinus ostial atresia.8 This case is associated with a PAPVD. UCS, PLSVC and associated cardiac abnormalities may be investigated with echocardiography,9 cardiac magnetic resonance imaging,10 cardiac computed tomography or cardiac catheterisation.

Treatment of UCS and PLSVC, if needed, is surgical correction of its components and associated abnormalities. For example, the PLSVC may be occluded percutaneously5,6 or re-routed surgically to the right heart circulation such as the left pulmonary artery.11 Treating the PLSVC alone may not be sufficient – the UCS may need to be patched and other associated abnormalities treated accordingly.

These procedures should be performed only after anticipating their haemodynamic effects on the coronary sinus drainage and associated abnormalities. This is to avoid disturbed venous return or residual shunt8 leading to risky re-operations. For example, the normal innominate vein drains the left internal jugular and subclavian veins into the normal right superior vena cava. In its absence, a PLSVC becomes the sole drainage for these left neck and arm veins and should be re-routed and not occluded11 to prevent congestion of the head and upper limb.


   Conclusion
Top
 Abstract
 Introduction
 Case study
 Discussion
 Conclusion
References
 
Dilated coronary sinus is a prompt to look for further cardiac abnormalities such as intracardiac shunts or thoracic venous abnormalities. The complex of UCS and PLSVC is one such abnormality and its treatment requires careful assessment of not only the UCS and PLSVC but also other concomitant cardiac abnormalities to prevent post-treatment haemodynamic complications.


   References
Top
 Abstract
 Introduction
 Case study
 Discussion
 Conclusion
 References
 

  1. Raghib G., Ruttenberg H.D., Anderson R.C., Amplatz K., Adams P. Jr., Edwards J.E. 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.[Abstract/Free Full Text]
  2. Knauth A., McCarthy K.P., Webb S., Ho S.Y., Allwork S.P., Cook A.C., et al. Interatrial communication through the mouth of the coronary sinus. Cardiol Young (2002) 12:364–372.[Web of Science][Medline]
  3. Sarodia B.D., Stoller J.K. Persistent left superior vena cava: case report and literature review. Respir Care (2000) 45:411–416.[Medline]
  4. Kuhn A., Hauser M., Eicken A., Vogt M. Right heart failure due to an unroofed coronary sinus in an adult. Int J Cardiol (2005) [Epub ahead of print].
  5. Troost E., Gewillig M., Budts W. Percutaneous closure of a persistent left superior vena cava connected to the left atrium. Int J Cardiol (2006) 106:365–366.[CrossRef][Web of Science][Medline]
  6. Geggel R.L., Perry S.B., Blume E.D., Baker C.M. 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) 48:369–373.[CrossRef][Web of Science][Medline]
  7. Sweitzer B.J., Hoffman W.J., Allyn J.W., Daggett W.J. Jr. Diagnosis of a left-sided superior vena cava during placement of a pulmonary artery catheter. J Clin Anesth (1993) 5:500–504.[CrossRef][Web of Science][Medline]
  8. Adatia I., Gittenberger-de Groot A.C. Unroofed coronary sinus and coronary sinus orifice atresia. Implications for management of complex congenital heart disease. J Am Coll Cardiol (1995) 25:948–953.[Abstract]
  9. Chen M.C., Hung J.S., Chang K.C., Lo P.H., Chen Y.C., Fu M. Partially unroofed coronary sinus and persistent left superior vena cava: intracardiac echocardiographic observation. J Ultrasound Med (1996) 15:875–879.[Abstract]
  10. Hahm J.K., Park Y.W., Lee J.K., Choi J.Y., Sul J.H., Lee S.K., et al. Magnetic resonance imaging of unroofed coronary sinus: three cases. Pediatr Cardiol (2000) 21:382–387.[CrossRef][Web of Science][Medline]
  11. Takach T.J., Cortelli M., Lonquist J.L., Cooley D.A. Correction of anomalous systemic venous drainage: transposition of left SVC to left PA. Ann Thorac Surg (1997) 63:228–230.[Abstract/Free Full Text]

Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?



This Article
Right arrow Abstract Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Kong, P.K.
Right arrow Articles by Ahmad, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Kong, P.K.
Right arrow Articles by Ahmad, F.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?