Ann Thorac Surg 2005;79:1428-1430
© 2005 The Society of Thoracic Surgeons
How to do it
Transposition of the Great Arteries and Quadricuspid Pulmonary Valve: An Unusual Combination
Marco Ricci, MD*,a,
Gordon A. Cohen, MD, PhDa,
Ergin Kocyildirim, MDa,
Sachin Khambadkone, MDa,
Martin J. Elliott, MDa
a Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom
Accepted for publication December 2, 2003.
* Address reprint requests to Dr Ricci, Division of Cardiothoracic Surgery, University of Miami, 1611 NW 12th Ave, Holtz Center 3072, Miami, FL 33136, USA
mricci{at}med.miami.edu
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Abstract
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Quadricuspid semilunar valves are very rare, especially in association with other congenital cardiac anomalies. A quadricuspid pulmonary valve has never been described in the setting of transposition of the great arteries. In this brief article we describe one such case, and we discuss the operative strategy during the arterial switch operation with particular reference to the technique of coronary translocation.
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Introduction
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Congenital anomalies of the semilunar valves are rare and most often involve the presence of a bicuspid or unicuspid valve [1]. Apart from quadricuspid valves found in the setting of the common arterial trunk, quadricuspid semilunar valves are exceedingly uncommon, with the pulmonary valve being affected more frequently than the aortic valve [2]. When the pulmonary valve is involved, its abnormal architecture rarely alters the function of the valve and the anomaly often remains silent [1, 2]. In contrast, when the aortic valve is involved there appears to be a distinct risk for the development of aortic insufficiency as demonstrated by several reports [3, 4].
Although the occurrence of a quadricuspid semilunar valve as an isolated anomaly is rare, its prevalence is well documented [1–5]. In contrast, the coexistence of this anomaly with other cardiac malformations seems to be even more unusual, and data from the literature are sparse [1, 2]. Among the cardiac defects, quadricuspid semilunar valves have been found in combination with coronary artery anomalies, atrial septal defects, ventricular septal defects, atrioventricular septal defects, and coarctation of the aorta [1, 2].
According to the literature, there has been only one report of a quadricuspid semilunar valve found in association with transposition of the great arteries [6]. The quadricuspid semilunar valve with transposition of the great arteries and ventricular septal defects was found in a postmortem patient who did not undergo surgical correction, and it entailed the presence of a quadricuspid aortic valve along with a unicuspid pulmonary valve, which had caused left ventricular outflow tract obstruction [6].
We believe that the coexistence of transposition of the great arteries with a quadricuspid pulmonary valve has not yet been reported. The aim of this article is to describe one such case and to discuss the surgical implications of this anomaly with particular reference to the management of the coronary arteries during the arterial switch operation.
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Technique
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A newborn female was admitted to our institution with severe cyanosis and a cardiac anomaly that entailed complete transposition of the great arteries, atrial septal defects, and patent ductus arteriosus. Two-dimensional echocardiography also revealed that the aorta and the pulmonary trunk were in an anterior-posterior relationship. The coronary arrangement was the most common, as the left anterior descending and circumflex coronary arteries originated as a single orifice from sinus 1, and the right coronary artery originated from sinus 2 (one left anterior descending artery and circumflex, 2 R; Leiden classification). However, this investigation also showed that the architecture of the posterior semilunar valve (pulmonary) was abnormal. The valve appeared to have four separate cusps of unequal size (Fig 1). In contrast, the anterior aortic valve was tricuspid with the two coronary arteries originating from each of the facing sinuses.
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Fig 1. Two-dimensional transthoracic echocardiography showing the aorta and the pulmonary trunk in an anterior-posterior relationship. The posterior semilunar valve (pulmonary valve) appears to be quadricuspid.
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The initial management of this patient included continuous prostaglandin infusion and a balloon atrial septostomy. The surgical management entailed an arterial switch, along with closure of the atrial septal defects and patent ductus arteriosus. At surgery, after the pulmonary arteries were widely dissected, cardiopulmonary bypass at moderate hypothermia was established by aortic and single venous atrial cannulation. The coronary anatomy was confirmed, and the epicardial course of the coronary arteries was noted. There were no abnormal branches between the aorta and the pulmonary trunk. The arrangement of the great arteries, semilunar valves, and coronary arteries is shown in Figure 2A.
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Fig 2. Arterial switch operation in transposition of the great arteries and quadricuspid pulmonary valve. (A) Arrangement of the great arteries, semilunar valves, and coronary arteries. The anterior aortic valve is tricuspid and the coronary pattern is the normal one. The posterior pulmonary valve is quadricuspid. (B) A button of neo-aortic wall is excised from the facing sinus between the two anterior commissural posts at and below the sino-tubular junction. (C) The two coronary buttons are implanted on the neo-aorta side-by-side using a continuous 7-0 polypropylene suture. (D) A second 7-0 running suture is used to join the two buttons. (E) Reimplantation of the coronary buttons has been completed. Note that a neo-sinus is created by partially approximating the upper edge of the two coronary buttons.
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After the duct was divided, the aorta was cross-clamped, and cold blood cardioplegia was administered. The anterior aorta was transected above the sino-tubular junction, and the coronary buttons were developed. As the aortic valve was tricuspid, preparation of the buttons proceeded in the usual fashion, scalloping widely the sinuses of Valsalva. Part of the reconstruction of the neo-pulmonary artery with a single patch of native pericardium was performed at this stage. The pulmonary trunk was then transected near its bifurcation, and the aorta was brought behind the pulmonary bifurcation (Lecompte maneuver). The posterior semilunar valve (neo-aortic) was confirmed to have four cusps of unequal size. One of them was anterior and directly faced the coronary buttons (Fig 2B). As a result, it was decided to transfer both coronary ostia jointly into the same sinus, using a technique similar to that described by Vouhé and collegues [7].
At first, a small segment of aortic wall was excised from the anterior neo-aortic sinus (Fig 2B). Coronary buttons and proximal coronary trunks were carefully dissected from the epicardium, sufficiently enough to allow a tension-free anastomosis. The two coronary buttons were then implanted side-by-side into the same neo-aortic sinus (Figs 2C, 2D). In the end, the suture line joining the buttons was brought over and around their edges (Fig 2E) in an attempt to recreate a sinus and restore the size of the neo-aorta at the sino-tubular junction. Finally, the neo-aortic anastomosis was constructed and the atrial septal defects were closed under a brief period of circulatory arrest. The reconstruction of the neo-pulmonary artery was then completed with a single patch of native pericardium after the aortic cross clamp had been removed and rewarming was in progress. Great care was taken to avoid an oversized pericardial patch, as this could have resulted in compression of the coronary buttons.
The patient made an uneventful recovery. Two-dimensional echocardiography performed 8 months after the operation revealed a satisfactory repair, good bi-ventricular function, unobstructed flow into both coronary ostia, and a functionally normal quadricuspid neo-aortic valve.
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Comment
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A quadricuspid pulmonary valve is unlikely to be observed in association with transposition of the great arteries. However, if encountered, it may alter the intraoperative strategy and the technique of coronary translocation. In the vast majority of patients undergoing the arterial switch operation, we routinely use a technique of coronary reimplantation that entails the construction of two medially-based trap doors in each of the facing neo-aortic sinuses. However, in the patient described herein, this was prevented by the configuration of the pulmonary (neo-aortic) valve. The valve was found to have four cusps, and there was one large anterior sinus that directly faced the coronary buttons, lending itself to accept both coronaries side-by-side.
Some surgeons would argue that in certain situations in which coronary translocation is anticipated to be problematic, an alternative strategy such as an atrial switch procedure should be considered. In our patient we favored the use of a technique previously described by others [7] and adapted to this anatomy. The technique of transferring both coronary ostia jointly into the same sinus after excising a single button of neo-aortic wall has been reported previously by Vouhé and colleagues [7]. In their experience this strategy was initially found to be widely applicable to nearly all coronary patterns and to effectively reduce the perioperative risk of coronary complications [7]. However, its routine application has been hindered by an alarming rate of early coronary artery obstruction at follow-up noted by the same authors, perhaps as a result of compression of the coronary ostia by the neo-pulmonary trunk [8].
Although we used a similar strategy with our patient, a few technical considerations may be worth discussing. Based on the report by the French group [8], we made a special effort to avoid an excessively large native pericardial patch to reconstruct the neo-pulmonary trunk. This was done to avoid compression of the coronary buttons, a concern when using this technique. Also, as in the description by Vouhé and collegaues, the neo-aortic valve was tricuspid, the area chosen for reimplantation was generally directly above the nadir of the anterior commissural post, thus above the sino-tubular junction [7]. In contrast, our patient had a quadricuspid neo-aortic valve with a large anterior sinus (Fig 2A). Thus the area chosen for reimplantation corresponded to the anterior neo-aortic sinus between the two anterior commissural posts at and below the sino-tubular junction (Fig 2B). As this could have interfered with valve function, a particular effort was made to recreate a neo-aortic sinus as shown in Figure 2E. This was an attempt to restore the geometry of the neo-aortic root and preserve the diameter of the neo-aorta at the sino-tubular junction. This may have been especially important in the presence of a quadricuspid neo-aortic valve, which could by itself expose the patient to a greater risk of having aortic regurgitation develop in the future.
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Acknowledgments
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We are very grateful to Faith Hanstater, Surgical Research Administrator, for her assistance. Research at Great Ormond Street Hospital NHS Trust benefits from Research and Development funding received from the NHS Executive.
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References
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- Hedayat KM, Sharp E, Weinhouse E, Riggs TW. A quadricuspid pulmonic valve diagnosed in a live newborn by two-dimensional echocardiography. Pediatr Cardiol. 2000;21:279–281[Medline]
- Portela FA, Hazekamp MG, Bartelings MM, Gittemberger-de Groot AC. Quadricuspid aortic valve in transposition of the great arteries. J Thorac Cardiovasc Surg. 2002;123:348–349[Free Full Text]
- Vouhé PR, Haydar A, Ouaknine R, et al. Arterial switch operation: a new technique of coronary transfer. Eur J Cardiothorac Surg. 1994;8:74–78[Abstract]
- Bonnet D, Bonhoeffer P, Piechaud JF, et al. Coronary obstructions after reimplantation of the two coronary ostia in a single orifice during arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg. 1996;10:482[Medline]
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Abstract
Introduction
