Ann Thorac Surg 1998;65:280
© 1998 The Society of Thoracic Surgeons
How to Do It
Repair of Complex Unroofed Coronary Sinus by Anastomosis of Left to Right Superior Vena Cava
Jacques A. M. van Son, MD, PhD,
Jörg Hambsch, MD,
Friedrich W. Mohr, MD, PhD
Herzzentrum, University of Leipzig, Leipzig, Germany
Accepted for publication August 23, 1997.
Dr van Son, Herzzentrum, University of Leipzig, Russenstrasse 19, D-04289 Leipzig, Germany.
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Abstract
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An extracardiac repair technique is described for the anomalously connecting left superior vena cava in complex unroofed coronary sinus syndrome. In this technique, the left superior vena cava is divided distally and is anastomosed to the right superior vena cava in an end-to-side fashion; in addition, the intracardiac anomalies are corrected. The main advantage of this technique consists of avoidance of the various disadvantages of construction of a complex intraatrial baffle.
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Introduction
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The unroofed coronary sinus syndrome is a spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and the left atrium is absent. The anomaly is strongly associated with a persistent left superior vena cava (SVC) with a connecting vein between both SVCs being absent in 80% to 90% of the cases [1][2]. Unroofed coronary sinus syndrome has as its most common major associated intracardiac anomaly a partial or complete atrioventricular canal defect, often with a common atrium. This combination of anomalies usually is associated with atrial isomerism, most frequently with bilaterally morphologically right atrial appendages [3][4][5]. In this clinical setting, biventricular repair consisting of construction of a complex intraatrial baffle from the pulmonary veins to the mitral valve, closure of the ventricular component of the atrioventricular septal defect, and repair of other associated anomalies has a reported mortality rate of as high as 50% [4][5]. Based on this experience, we recently have strived for an extracardiac repair of the anomalously connecting systemic venous component in complex unroofed coronary sinus syndrome.
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Technique
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Through a median sternotomy, a large pericardial patch is harvested for later use in the repair of the atrioventricular canal. Both SVCs including the right brachiocephalic vein, the azygos vein, and the bilateral proximal internal jugular veins and subclavian veins are dissected and mobilized. Utmost care is taken to avoid damage to the bilateral phrenic nerves and the thoracic duct. After cannulation of the aorta, the inferior vena cava, and both SVCs (with the cannulas placed as cephalad as possible), cardiopulmonary bypass is instituted and the atrioventricular canal and other intracardiac anomalies, if present, are repaired. After completion of the intracardiac repair, during the warming phase, the left SVC is divided at its entrance into the roof of the left-sided atrium (Fig 1A).
The left atrium is oversewn with a running suture. The left SVC is carried up over the aortic arch and anastomosed to the medial aspect of the right SVC with a running 7-0 polyglyconate suture (Maxon;Davis & Geck, Inc, Danbury, CT) (Fig 1B). The patient is weaned from cardiopulmonary bypass, the arterial and venous cannulas are removed, and pressures are measured in the right brachiocephalic vein and the left SVC to rule out obstruction at the left SVC-to-right SVC anastomosis.
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Technique of extracardiac repair of anomalous systemic venous connection in complex unroofed coronary sinus syndrome with bilateral superior venae cavae. (A) After completion of intracardiac repair, the left superior vena cava is divided at its entrance into the roof of the left-sided atrium. The hemiazygos vein is also divided. A trapdoor incision is made in the medial aspect of the right superior vena cava in such a fashion that an anteriorly hinged flap is created. (B) The left superior vena cava is anastomosed end-to-side to the right superior vena cava using a continuous resorbable suture.
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Patients and Results
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The reported technique was applied in 2 young infants (aged 7 and 10 weeks) with unroofed coronary sinus syndrome, right isomerism with bilateral SVCs and absent left innominate vein, and complete atrioventricular canal; 1 also had mild infundibular stenosis. Both patients had symptoms of moderate congestive heart failure. In both patients, repair of the atrioventricular canal (with resection of the infundibular stenosis in 1) and anastomosis of the left SVC to the right SVC resulted in an excellent hemodynamic result. Postoperatively, the left atrial pressures were 11 and 12 mm Hg. Notably, there was absence of facial edema, venous engorgement, or chylothorax. At follow-up of 14 and 6 months, both patients are clinically well without clinical and echocardiographic evidence of obstruction at the site of the left SVC-to-right SVC anastomosis.
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Comment
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In the presence of an unrestrictive or mildly restrictive connecting vein, the standard repair for unroofed coronary sinus syndrome with left SVC consists of ligation of the left SVC and patch closure of the coronary sinus atrial septal defect. In the presence of a restrictive connecting vein in this setting, we have performed the same operation in addition to augmentation of the diminutive connecting vein with an autologous pericardial patch [6]. In the much more common situation of a left SVC with absent connecting vein, the usual repair consists of resection of the atrial septum followed by placement of a pericardial patch in such a fashion that all of the pulmonary veins drain under the baffle to the mitral valve orifice [4]. The proximity of pulmonary veins to the orifice of the left SVC makes the placement of such a baffle cumbersome, with the inherent potential for pulmonary venous obstruction. Another major concern with regard to the latter technique relates to the creation of a small and low-compliance left atrial compartment with its adverse effect on left ventricular filling. These concerns apply especially when the intraatrial baffle repair is performed in complex forms of unroofed coronary sinus syndrome, often in association with partial or complete atrioventricular canal. The cumulative effects of creation of a small and low-compliance left atrial compartment in the setting of increased volume loading (as a result of abolition of the left-to-right shunt) of the (often relatively small) left ventricle may be incompatible with postrepair survival. This is evidenced by reported high mortality rates for biventricular repair of complex coronary sinus [4][5]. The advantage of the extracardiac repair technique that we propose consists of avoidance of construction of an intraatrial baffle, thus neutralizing the various disadvantages that are inherent to the latter technique. In addition, the proposed extracardiac repair technique is more straightforward technically and allows for a reduction in cardiopulmonary bypass time.
In our experience, adequate mobilization of the left SVC and its connecting veins allows for a course of the left SVC anterior to the aortic arch and a tension-free anastomosis of the left SVC to the right SVC. If a course of the left SVC anterior to the aortic arch is technically not feasible, a course under it may be a good alternative [7]. In the absence of a right SVC or in the presence of a diminutive right SVC, consideration may be given to anastomosis of the left SVC to the right-sided atrial appendage [7][8][9]. Alternatively, in patients beyond the neonatal stage, bidirectional or bilateral left superior cavopulmonary anastomoses may be performed [10].
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References
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- Helseth HK, Peterson CR Atrial septal defect with termination of left superior vena cava in the left atrium and absence of the coronary sinus: recognition and treatment. Ann Thorac Surg 1974;17:186-192.[Medline]
- Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM, Jr Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979;27:418-425.[Abstract]
- Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg 1995;110:641-650.[Abstract/Free Full Text]
- Kirklin JW, Barratt-Boyes BG Unroofed coronary sinus syndrome. In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery, 2nd ed. New York: Churchill-Livingstone, 1993:683-692.
- Hirooka K, Yagihara T, Kishimoto H, et al. Biventricular repair in cardiac isomerism. Report of seventeen cases. J Thorac Cardiovasc Surg 1995;109:530-535.[Abstract/Free Full Text]
- Van Son JAM, Falk V, Mohr FW Pericardial patch augmentation of restrictive innominate vein and division of left superior vena cava in unroofed coronary sinus syndrome. J Thorac Cardiovasc Surg 1997;114:132-134.[Free Full Text]
- Reddy VM, McElhinney DB, Hanley FL Correction of left superior vena cava draining to the left atrium using extracardiac techniques. Ann Thorac Surg 1997;63:1800-1802.[Abstract/Free Full Text]
- Taybi H, Kurlander GJ, Lurie PR, Campbell JA Anomalous systemic venous connection to the left atrium or to a pulmonary vein. AJR 1965;94:62-77.
- Shumacker HB, 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]
- Foster ED, Baeza OR, Farina MF, Shaher RM Atrial septal defect associated with drainage of the left superior vena cava to left atrium and absence of the coronary sinus. J Thorac Cardiovasc Surg 1978;76:718-720.[Abstract]
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Abstract
Introduction
