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Ann Thorac Surg 2000;69:568-571
© 2000 The Society of Thoracic Surgeons

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Original Articles

Single coronary artery and complete transposition of the great arteries: a technical challenge resolved?

^a Division of Cardiovascular Surgery, The Hospital for Sick Children, The University of Toronto, Toronto, Canada
^b Division of Cardiology, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario, Canada

Address reprint requests to Dr Black, Department of Cardiac Surgery, The Lucile Packard Children’s Hospital, Stanford University School of Medicine, Stanford, CA 94305-5407
e-mail: michael.black{at}leland.stanford.edu

	Abstract

Top Abstract Introduction Material and methods Results Comment References

 
Background. The origin of the coronary arteries from a single aortic sinus remains a rare congenital anomaly, once regarded as having little clinical significance. Contemporary surgical practice, however, frequently demands precise coronary reimplantation. In this article we emphasize a prophylactic surgical technique found especially helpful in the repair of D-transposition of the great arteries (D-TGA)/single coronary artery.

Methods. We reviewed the institutional cardiac registry.

Results. Since 1985, 398 neonates with D-TGA were repaired with the arterial switch procedure. A mortality rate of 38% was encountered in "simple" D-TGA (n = 174)/single coronary (2.9% left facing sinus (IRLCx), 7.5% right facing sinus (IIRLCx)) and 41% in neonates with D-TGA (n = 224)/single coronary (3.6% IRLCx, 12% IIRLCx). During the past 3.5 years the surgical mortality rate of neonates (n = 6) treated with origin of the coronary arteries from a single aortic sinus has dropped to 0%.

Conclusions. The surgical repair of D-TGA/single coronary artery continues to trouble surgeons. The implantation of a well-mobilized coronary "button" into a previously anastomosed neoaorta remains a key prophylactic technique in the achievement of good technical results.

	Introduction

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A prophylactic surgical technique is described that should allow for improved acute and intermediate survival following the relocation of high-risk coronary patterns in those afflicted with D-transposition of the great arteries (D-TGA). Several patterns of coronary arteries remain predisposed to late occlusion, particularly those with the origin of all coronary arteries from a single aortic sinus [1–3]. The ultimate fate of coronary patency in such abnormal patterns remains unknown, partly because of a possible persistent aberrant course or an intramural origin.

	Material and methods

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Surgical technique
After the induction of anesthesia and placement of appropriate monitoring lines, the neonate is placed on cardiopulmonary bypass utilizing a standard technique of bicaval cannulation and moderate hypothermia (25°C). A left ventricular vent is placed through the right superior pulmonary vein.

The external features of the coronary arteries are inspected. Once the nadir temperature is achieved, the aorta is cross clamped and blood cardioplegia solution is administered anterogradely through the aortic root in an initial dose of 30 cc/kg followed by approximately 20 cc/kg selectively every 20–30 minutes as long as the aortic cross-clamp remains in place. The caval snares are tightened and the aorta is transected approximately 1 cm above the aortic valve.

With absence of an intramural coronary, a single coronary button is developed using sharp dissection. The coronary ostium is examined and sometimes found to be multiple and eccentric. When an intramural course of the coronary arteries is present, the surgical strategy must be modified (see Comment). When two coronary ostia arise from a single aortic sinus, separation of the ostia into two distinct buttons is required followed by their individual reimplantation.

The right or left single coronary artery is typically large and frequently draped over the aortic root (depending on the origin form, sinus I or II, respectively) (Figs 1A, 1B). The coronary artery found draped over the proximal aorta is well mobilized including separation from the underlying conal musculature. Low energy settings (electrocautery) have been found to be ideal in combination with gentle probing of the coronary artery to further elucidate its course. It is not uncommon for small conal branches to be sacrificed to prevent future tethering upon posterior relocation. The great arteries are frequently malpositioned and not found in a direct anterior-posterior location (similar to the finding with an intramural course, ie, side by side).

View larger version (129K): [in this window] [in a new window]   Fig 1. (A–E) The five types of single coronary origins encountered in our patient population. Note the inevitable "draping" of the coronary artery anterior to the neopulmonary artery (A, B), a possible long-term nidus for coronary occlusion due to dynamic changes that may occur in the right ventricular outflow tract. (Cx = circumflex artery; LAD = left anterior descending artery; LM = left main; RCA = right coronary artery.)

A "trapdoor" of aortic tissue and pericardial flaps have not been found useful with the coronary transfer. The reimplantation site is usually quite proximal in the neoaortic root. With careful planning (dividing both the aortic and pulmonary arteries) avoidance of reimplantation in the neoaortic suture line can be achieved. The coronary button is fixed into position with 8-0 monofilament sutures. Inspection through the aortic root commonly provides ample opportunity for the concomitant closure of outlet ventricular septal defects (VSDs). A right atriotomy provides exposure for repair of the iatrogenic atrial septostomy or perimembranous VSD.

After careful removal of air, reperfusion and rewarming are initiated. The heart should "pink up" immediately with the resumption of electrical-mechanical activity. The pulmonary arterioplasty is performed in a routine fashion except with a single coronary artery explanted only one neopulmonary sinus requires reconstruction. Weaning from cardiopulmonary bypass is performed, followed by a brief period of modified ultrafiltration.

Methods
We retrospectively reviewed the institutional registry of neonates afflicted with isolated D-TGA or in association with VSD with the origin of the coronary arteries from a single aortic valve sinus. Eight neonates were identified as having origin of the coronary arteries from a single aortic sinus.

	Results

Top Abstract Introduction Material and methods Results Comment References

 
Since 1985, 398 neonates with D-TGA were addressed surgically at our institution with an arterial switch procedure. Of the 174 neonates with isolated D-TGA (intact septum), 18 were diagnosed as having a single coronary arterial pattern, 2.9% (n = 5) left facing sinus (IRLCx) and 7.5% (n = 13) right facing sinus (IIRLCx) (sinus I refers to the left-facing sinus and sinus II to the right-facing sinus). The combined surgical mortality rate in this cohort was 38% (n = 7). Of the 224 neonates with D-TGA/VSD, 35 were diagnosed as having a single coronary arterial pattern, 3.6% (n = 8) IRLCx and 12% (n = 27) IIRLCx. The combined surgical mortality rate was 41% (n = 14). In both cohorts a greater proportion of deaths occurred in those afflicted with IIRLCx. During the past 3.5 years the surgical mortality rate of neonates treated with origin of the coronary arteries from a single aortic sinus has dropped to 0% (n = 6) (see Table 1). No child has required subsequent coronary intervention since the original surgical intervention. Coronary patency has been documented by transthoracic postoperative echocardiography in all patients. Dobutamine stress echocardiography is planned for follow-up in all patients.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
The primary technical limitation of the arterial switch most commonly relates to the precise transfer of the coronary arteries without undue tension, torsion, or kinking. Particularly susceptible to the latter is the origin of the coronary arteries from a single aortic sinus. In most series, the origin of all coronaries from a single aortic sinus continues to be associated with a greater degree of mortality [1, 2, 4–9].

The complexity and variety of coronary arterial anatomy in hearts with TGA have been well documented [9, 10]. All three main coronary arteries arise from a single aortic sinus in 7% to 9% of those afflicted with D-TGA. Rarely the coronary arteries arise from two or more separate ostia within the same aortic sinus. Concomitant abnormalities of the coronary ostia are not infrequent, ie, eccentric, "slitlike," or juxtaposed to the valve commissures. The identification of a great vessel arrangement other than in an anterior-posterior position as well as commissural malalignment (preoperative studies, ie, echocardiography and angiography) should arouse concerns with regards to an abnormal course or origin of the coronary arteries. An intramural passage of the coronary arteries has been identified as being independently associated with an increased risk of mortality [11].

The performance of the arterial switch without coronary transfer has been advocated by some to deal with either single origin or intramural course of the coronaries [12–14]. Early mortality may be decreased, however, late right ventricular outflow tract obstruction by the commonly oversized intraarterial baffle and late ostial stenosis remain theoretical hazards that may require future revision. Of greater significance, the continued abnormal course of the coronary persists as a potential nidus for mortality among children as they mature. Sudden death remains frequent among young adults with an abnormal course of their coronary arteries (usually found between the great arteries). Dilation of the pulmonary artery during high output states such as exercise may recreate in essence the lethal situation described above (neopulmonary root) by maintaining an abnormal course of the left or right coronary anterior to the pulmonary artery [3, 15–17].

Modifications that unroof the intramural portion of coronary with subsequent single button division and reimplantation (two coronary buttons) should address both the abnormal origin and course and have been utilized successfully in both patient 3 and patient 5 [7]. Patient 3 (Fig 1C) was found to have origin of both coronary arteries from sinus II, both of which were intramural in origin. Unlike patient 5 in the series by Asou and colleagues [7], after takedown of the posterior aortic commissure the single coronary button was divided successfully into two separate buttons. Reimplantation proceeded in the usual manner after completion of the aortic anastomosis.

The technique detailed in this article (see Surgical Technique) has been found useful in all cases of D-TGA in which the arterial switch is the planned procedure. The technical misfortune of incorrect positioning of the coronary button can likely be avoided as well as prolonged myocardial ischemia and recurrent reperfusion injuries. Complex coronary arterial patterns can be repaired predictably and the completed repair visualized within the neoaortic root before release of the aortic cross-clamp. Additional benefits include the ability to inspect the suture lines (coronary button) for untoward bleeding before completion of the neopulmonary artery. It is not uncommon to be somewhat surprised at the location chosen for coronary implantation once the aortic cross-clamp is reapplied.

The abnormal course of a coronary artery (single coronary) even after effective transfer may, however, persist, being draped over the neopulmonary artery (in an anterior position) or in a posterior course, behind the neoaorta (Figs 1D, E). The latter pattern is likely to cause less long-term morbidity/mortality perhaps because the aorta is less likely to undergo conformational changes with increased exercise, ie, coronary insufficiency should be evident immediately in the operating room if present. The abnormal course of the coronary artery (either anterior or posterior) is not a surgically correctable feature. Even with successful coronary relocation, the ultimate fate/patency of the "draped" coronary artery remains unknown. Circumferential suture line (button) fibrosis, mechanical kinking, and reactive injury secondary to probing or the selective instillation of cardioplegia have been proposed as more common mechanisms of late coronary occlusion [3]. Will future coronary bypass surgery become necessary in this subset of children? Regardless of the mechanisms, an underestimation of the actual frequency of coronary obstruction is likely present due to the acquisition of gradual collateral formation. Many children remain asymptomatic even with significant coronary arterial disease [3]. Thus longer periods of follow-up with selective coronary arterial visualization will be required to determine the true incidence of coronary occlusive disease.

Frequently associated with side-by-side great arteries, and commissural malalignment, the origin of all coronary arteries from a single aortic sinus continues to trouble surgeons. The modifications detailed in this article provide a safe and predictable method of coronary transfer even when associated with complex coronary patterns or an intramural course. Long-term follow-up will be required to demonstrate sufficient ostial and coronary patency to affirm this method’s superiority.

	Acknowledgments

 
We acknowledge Mr Phil Dakin for his artistic contributions.

	References

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