Ann Thorac Surg 1997;63:832-833
© 1997 The Society of Thoracic Surgeons
Case Reports
Early Coronary Sinus Reroofing Using the Left Atrial Baffle
Thierry Beyens, MD,
Hélène Demanet, MD,
Frank E. Deuvaert, MD
Cardiac Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium
Accepted for publication October 8, 1996.
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Abstract
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Surgical correction of unroofed coronary sinus, left superior vena cava, dextrocardia, and situs solitus in a 4-month-old infant consisted of reroofing the coronary sinus by means of a left atrial flap while redirecting the left superior vena cava to the right atrium. Excellent access to the left side of the left atrium was afforded by the associated dextrocardia.
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Introduction
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Unroofed coronary sinus (CS) is a defect in the wall separating the left atrium (LA) from the CS. When this defect is associated with a left superior vena cava (LSVC), it allows mixed shunting from the LSVC to the LA and, through the "CS" type defect, from the LA to the right atrium (RA). Definitive correction in a small infant with dextrocardia, situs solitus, LSVC, and unroofed CS was possible by using an LA flap, similar to the RA flap used in a Senning operation.
A 4-month-old infant had had aortic coarctation repair at the age of 10 days. He remained in heart failure with a predominantly left-to-right interatrial shunt (pulmonary-to-systemic flow ratio = 2.6). The anatomic diagnosis was situs solitus, dextrocardia, two hemodynamically insignificant ventricular septal defects, no right superior vena cava, and a large LSVC draining into an unroofed CS. There was no residual gradient at the site of coarctation repair.
Surgical correction was begun using median sternotomy with hypothermic cardiopulmonary bypass. During a short period of total circulatory arrest, a posteriorly hinged LA flap was created by incising the LA parallel to the left atrioventricular groove from the anterior border of the LSVC ostium. The free anterior border of the flap was brought inside the heart and used to reroof the CS, by baffling the LSVC to the CS ostium in the RA (Fig 1
). An autologous pericardial patch was used to augment the residual LA cavity.
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Fig 1. . Left atrial flap reroofing the coronary sinus, connecting the left superior vena cava to the coronary sinus ostium. ( CS = coronary sinus; LAA = left atrial appendage; LAF = left atrial flap; LSVC = left superior vena cava; PA = pulmonary artery.)
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The patient is asymptomatic at 2 years of age with unrestricted systemic and pulmonary venous drainage shown on echocardiography.
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Comment
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The unroofed CS syndrome is a spectrum of cardiac anomalies in which part or all of the common wall between the CS and the LA is absent. In most cases, there is a persistent LSVC, which connects to the CS in the left upper corner of the LA [1], and a CS atrial septal defect in the posteroinferior region of the atrial septum, in the usual position of the ostium of the CS. Absence of the right superior vena cava and the association with dextrocardia (with situs solitus) have not been described yet.
Surgical intervention is advisable because of the presence of mild or severe cyanosis and because of the risk of cerebral paradoxic emboli (10% to 25%) [2]. Abnormal drainage of an LSVC (in the absence of a right superior vena cava) in the LA, with unroofed CS and intact atrial septum, can be treated by reroofing the CS through the RA. This is done by excising the atrial septum and replacing it with a pericardial patch as a repositioned atrial septum, placing all the systemic venous orifices to its right [3]. Alternatively, the procedure requires rerouting the CS to the roof of the LA using a triangular flap of atrial septum, followed by reconstruction of the atrial septum using a pericardial patch, diverting blood from the LSVC to the RA [4].
None of these techniques, however, is applicable to a small infant without a high risk for early or late obstruction of the systemic or pulmonary venous channels. In our case, dextrocardia and situs solitus allowed us to perform a definitive operation in infancy for unroofed CS with LA drainage of an LSVC, by creating an LA flap to reconstitute the roof of the CS and by redirecting flow from the LSVC to the CS ostium. An autologous pericardial patch was used to reconstruct the LA wall. Echocardiographic studies performed 2 years after intervention showed unrestricted systemic and pulmonary venous drainage and spontaneous closure of the muscular ventricular septal defects.
The association of dextrocardia with an LSVC draining in an unroofed CS in the absence of an RSVC in a heart with situs solitus offered us the possibility to perform a definitive repair with growth potential using an LA flap to reroof the CS while reorienting LSVC flow to the RA in a 4-month-old infant.
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Footnotes
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Address reprint requests to Dr Beyens, Department of Cardiac Surgery, Hôpital Brugmann, place A. Van Gehuchten, 4, B-1020 Brussels, Belgium.
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References
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- Shumacker HB Jr, King H, Waldhausen JA. The persistent left superior vena cava: surgical implications with special reference to caval drainage into the left atrium. Ann Surg 1967;165:797–805.[Medline]
- Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr. Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979;27:418–25.[Abstract/Free Full Text]
- Miller GAH, Ongley P, Rastelli GC, Kirklin JW. Surgical correction of total anomalous systemic venous connection: report of a case. Mayo Clin Proc 1965;40:532–8.[Medline]
- Sand ME, McGrath LB, Pacifico AD, Mandke NV. Repair of left superior vena cava entering the left atrium. Ann Thorac Surg 1986;42:560–4.[Abstract/Free Full Text]
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Abstract
Introduction
