Asian Cardiovasc Thorac Ann 1998;6:52-53
© 1998 Asia Publishing EXchange Pte Ltd
Extracardiac Repair of Complex Unroofed Coronary Sinus
Jacques AM van Son, MD, PhD,
Jörg Hambsch, MD,
Friedrich W Mohr, MD
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Herzzentrum University of Leipzig Leipzig, Germany
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For reprint information contact: Jacques AM van Son, MD Herzzentrum University of Leipzig Russenstrasse 19 D-04289 Leipzig, Germany Tel:49 341 865 1445 Fax:49 341 865 1452
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ABSTRACT
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A technique for conversion of an intra-atrial baffle repair to an extracardiac repair is described for complex unroofed coronary sinus syndrome (including a complete atrioventricular septal defect) with connection of the left superior caval vein to the roof of the left-sided atrium. The left superior caval vein was divided distally and anastomosed to the right superior caval vein in an end-to-side fashion. In addition, the baffle in the left-sided atrium connecting the pulmonary veins with the left atrioventricular valve orifice was removed and the atria were septated. This extracardiac repair technique avoids the various disadvantages of the intra-atrial baffle technique such as creation of a small left atrium with low compliance.
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INTRODUCTION
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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 missing. The anomaly is strongly associated with a persistent left superior caval vein and a bridging vein is absent in 80% to 90% of these cases.1,2 A partial or complete atrioventricular septal defect, often with a common atrium, is the most frequent intracardiac anomaly associated with unroofed coronary sinus syndrome. This combination of anomalies is usually associated with atrial isomerism, most frequently with bilateral morphologically right atrial appendages.3–5 In this clinical setting, a biventricular repair consisting of repair of the ventricular component of the atrioventricular septal defect, repair of associated anomalies, and construction of a left atrial baffle from the pulmonary veins to the mitral valve, has a reported mortality rate as high as 50%.4,5 We report a patient with complex unroofed coronary sinus syndrome in whom such an operation was recently converted into an extracardiac repair of the anomalously connecting systemic venous component.
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CASE REPORT
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A 3-month-old infant with unroofed coronary sinus syndrome, right isomerism, bilateral superior caval veins without a bridging vein, complete atrioventricular septal defect, and moderate infundibular stenosis was referred with symptoms of congestive heart failure. The initial repair consisted of patch closure of the ventricular component of the atrioventricular septal defect, closure of the cleft in the left atrioventricular valve, resection of the infundibular stenosis, and construction of a baffle (using autologous pericardium) in the left atrium connecting the pulmonary veins with the orifice of the left atrioventricular valve. The early postoperative stage was characterized by bouts of tachycardia (exceeding 170 beats per minute) and systemic hypotension. Although there was a gradual improvement in the hemodynamic status of the patient during the subsequent weeks, the tachycardia remained. In addition, the patient developed a mild-to-moderate degree of pulmonary venous congestion. Cardiac catheterization demonstrated an intact left atrial baffle with a relatively small and shallow left atrial compartment underneath it. The left atrial pressure was 15 mm Hg. Based on the clinical symptoms and the observations made at cardiac catheterization, it was decided to convert the baffle repair to an extracardiac modification.
Through a secondary median sternotomy, a pericardial patch was harvested. Both superior caval veins including the right brachiocephalic vein, the azygos vein, the bilateral proximal internal jugular veins, and the subclavian veins were dissected and mobilized. Utmost care was taken to avoid damage to the bilateral phrenic nerves and the thoracic duct. After cannulation of the aorta, the inferior caval vein, and both superior caval veins (with the cannulae placed as cephalad as possible), cardiopulmonary bypass was instituted. The baffle in the left-sided atrium was resected through a right atriotomy and the atrial component of the atrioventricular septal defect was closed with a pericardial patch. After completion of the intracardiac repair, the left superior caval vein was transected at its entrance into the roof of the left-sided atrium and the atrium was over-sewn. Subsequently, a trapdoor incision was made in the medial aspect of the right superior caval vein in such a fashion that an anteriorly hinged flap was created (Figure 1
). This flap facilitated the anastomosis with the left superior caval vein, which was made with a continuous 7/0 polyglyconate suture (Davis & Geck, Inc., Danbury, CT, USA). The further operative course was uncomplicated. The intraoperative left atrial pressure was 11 mm Hg. Pressure measurements in the right brachiocephalic vein and the left internal jugular vein were equal, thus ruling out an obstruction across the left-to-right superior caval vein anastomosis. The postoperative course was uncomplicated. The left atrial pressure on the first postoperative day was 9 mm Hg. At 6-month follow-up there was no tachycardia or pulmonary venous congestion.
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Figure 1. Conversion of an intra-atrial baffle repair to a left-to-right superior caval vein anastomosis for complex unroofed coronary sinus syndrome with bilateral superior caval veins. After completion of the intracardiac repair including septation of the atria, the left superior caval vein 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 caval vein in such a fashion that an anteriorly hinged flap is created. The left superior caval vein is anastomosed end-to-side to the right superior caval vein using a continuous resorbable suture.
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DISCUSSION
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In the common situation of a left superior caval vein with an absent bridging vein, the usual repair consists of resection of the entire atrial septum, followed by placement of a pericardial patch in such a fashion that all of the pulmonary veins drain under the patch to the mitral valve orifice.4 Concerns with regard to the latter technique relate to the creation of a small left atrial compartment with low compliance that may negatively affect left ventricular filling and that has the potential for the development of pulmonary venous obstruction. These concerns apply especially when the intra-atrial baffle repair is performed in complex versions of unroofed coronary sinus syndrome, often in association with partial or complete atrioventricular septal defect. The cumulative effects of the creation of a low-compliant left atrial compartment with increased volume loading of the left heart (after abolition of the left-to-right shunt) in the presence of a variable degree of underdevelopment of the left ventricle, as often present in this anatomic setting, may be incompatible with post-repair survival as evidenced by the reported high mortality rates.4,5 Conversion of the intracardiac repair to an extracardiac modification as presented here, neutralizes the various disadvantages that are inherent to the intra-atrial baffle technique. In the absence of a right superior caval vein or the presence of a diminutive right superior caval vein in the setting of complex unroofed coronary sinus, consideration may be given to anastomosis of the left superior caval vein to the right-sided atrial appendage.
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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
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Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr. Surgical experience with unroofed coronary sinus. Ann Thorac Surg
1979;27:418–25.[Abstract]
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Rubino M, Van Praagh S, Kadoba K, et al. 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;110:641–50.[Abstract/Free Full Text]
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Kirklin JW, Barratt-Boyes BG. Unroofed coronary sinus syndrome. In: Kirklin JW, Barratt-Boyes BG, editors. Cardiac surgery. 2nd ed. New York: Churchill-Livingstone, 1993:683–92.
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Hirooka K, Yagihara T, Kishimoto H, et al. Biventricular repair in cardiac isomerism. Report of seventeen cases. J Thorac Cardiovasc Surg
1995;109:530–5.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
