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Unusual combination of three types of interatrial communications in a child

Published online by Cambridge University Press:  18 November 2020

Tarun R. RammanOpen the ORCID record for Tarun R. Ramman[Opens in a new window],
Sushil Azad and
Krishna S. Iyer
Tarun R. Ramman
Affiliation:
Department of Pediatric and Congenital Heart Surgery, Fortis Escorts Heart Institute, New Delhi, India
Sushil Azad
Affiliation:
Department of Pediatric and Congenital Heart Surgery, Fortis Escorts Heart Institute, New Delhi, India
Krishna S. Iyer*
Affiliation:
Department of Pediatric and Congenital Heart Surgery, Fortis Escorts Heart Institute, New Delhi, India
*
Author for correspondence: Dr K. S. Iyer, M.Ch., Department of Pediatric and Congenital Heart Surgery, Fortis Escorts Heart Institute, New Delhi, India. Tel: +91 11 47134540; Fax: +9111 26825013. E-mail: iyerks@hotmail.com
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Abstract

An unusual combination of three types of interatrial communications – coronary sinus defect, primum defect, and secundum defect – occurring together in a 3-year-old child is presented.

Keywords

coronary sinus defectmultiple ASD’sunroofed coronary sinus

Information

Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 30 , Issue 12 , December 2020, pp. 1933 - 1934
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

Case report

A 3-year-old girl presented with failure to thrive. An echocardiogram revealed a partial atrioventricular septal defect, a persistent left superior caval vein draining to the left atrium with an unroofed coronary sinus, and a small secundum atrial septal defect (Fig 1).

Figure 1.

Transthoracic echocardiogram showing all three inter-atrial defects as labelled.

At surgery, three interatrial communications were identified – a large ostium primum defect, a small secundum defect, and a coronary sinus defect (Fig 2). The left caval vein drained into the left atrium.

Figure 2.

Intra-operative photograph displaying the three inter-atrial defects.

The surgical procedure involved repair of the left atrioventricular valve, closure of the primum defect and coronary sinus defect with a single autologous pericardial patch, and direct closure of the secundum defect. The left caval vein was translocated to the right atrial appendage.

Discussion

Coronary sinus defects are rare interatrial communications that lie outside the confines of the atrial septum. They are formed as a result of defects in the embryonic left arteriovenous fold forming the common wall between coronary sinus and left atrium. They may or may not be associated with a persistent left caval vein. Primum and secundum atrial septal defects are the result of incomplete formation, resolution, and fusion of the septum primum and septum secundum. Reference Naqvi, McCarthy and Ho1

The concurrence of three types of interatrial communications in a single patient is a rarity and is therefore being presented. The surgical management is relatively straightforward.

Acknowledgements

None.

Financial support

This research received no specific grant from any funding agency, commercial or not-for-profit sectors.

Conflict of interest

None.

References

Naqvi, N, McCarthy, KP, Ho, SY. Anatomy of the atrial septum and interatrial communications. J Thorac Dis 2018; 10 (Suppl 24): S2837–S2847.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1.Transthoracic echocardiogram showing all three inter-atrial defects as labelled.

Figure 1

Figure 2.Intra-operative photograph displaying the three inter-atrial defects.