Ann Thorac Surg 2001;71:1684-1686
© 2001 The Society of Thoracic Surgeons
Case report
Fenestrated arterial switch operation: surgical approach to an unusual transposition of the great arteries complex
Carmelo Mignosa, MDb,
Vincenzo Duca, MDc,
Innocenzo Bianca, MDa,
Dario Salvo, MDb,
Giuseppe Ferlazzo, MDa,b,c,
Mauro Abbate, MDa,b,c
a Division of Cardiac Surgery, Vittorio Emanuele II, Ferrarotto, S. Bambino Hospital, Catania, Italy
b School of Cardiac Surgery, University of Catania, Catania, USA
c Neonatal Intensive Therapy Unit, V. Cervello Hospital, Palermo, Italy
Accepted for publication June 3, 2000.
Address reprint requests to Dr Mignosa, Divisione di Cardiochirurgia Azienda Ospedali Vittorio Emanuele, Ferrarotto, S. Bambino, Via S. Citelli, 95100 Catania, Italy
e-mail: mignosa{at}ctonline.it
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Abstract
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Transposition of the great arteries with intact ventricular septum and aortopulmonary window is an extremely rare anatomic combination, having been reported just twice previously. Other authors performed a physiologic repair, because the combination was considered unsuitable for an anatomic repair. We describe the case of a 26-day-old baby with such anatomy who was successfully treated with an arterial switch operation. A 4 mm fenestration at atrial level was made for a smoother postoperative course.
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Introduction
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Transposition of the great arteries with intact ventricular septum (TGA-IVS) has been described in combination with various lesions. The association with aorto-pulmonary window (APW) is rare indeed. We know of two previous cases described—Vanini in 1980 [1] and Krishnan and coworkers in 1991 [2]. For treatment, an APW repair plus Senning procedure or arterial switch operation (ASO) without coronary relocation have both been proposed [2].
A 3.2 kg neonate was referred to us after a cardiac murmur was noted on day 22 of life. The echocardiographic data showed situs solitus, concordant atrioventricular (AV) connection, discordant ventriculo-arterial (VA) connection and an antero-posterior relationship of the great vessels. From the posterior aspect of the ascending aorta an APW originated and communicated with the pulmonary artery (PA) just below the bifurcation. The left ventricle (LV) was well functioning and there was a non obstructed patent foramen ovale. On day 26 of life, she underwent corrective surgery.
After cardiopulmonary bypass (CPB) was started with double venous cannulation, the aorta was clamped and cardioplegia given. A small transverse opening on the anterior aspect of the aorta was made to allow evaluation of the coronary anatomy. The coronary pattern was 1LCx, 2R (Leiden classification).
This convinced us that the usual coronary translocation technique was feasable.
The aorta was divided at the level of the inferior aspect of the APW. This left the ascending aorta and the PA bifurcation connected in a single vascular structure superiorly and the aorta and the PA trunk connected inferiorly (Fig 1).
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Fig 1. After the aorta was transected at the inferior level of the aortopulmonary window, there were two different vascular structures left—ascending aorta and pulmonary bifurcation superiorly, and aorta and pulmonary artery trunk inferiorly.
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The vascular structures were divided and the coronary ostia removed with two large cuffs of arterial wall. The coronary relocation was performed utilizing a medially based rectangular flap in the neoaorta [3]. Because of lack of tissue in the neoaorta, the coronary buttons had to be implanted inside the same anterior neoaortic sinus in a lower than usual location. The right atrium was opened and the PFO partially closed, leaving a 4 mm fenestration in its inferior aspect. After the Lecompte manouver was performed, the procedure was completed in the usual fashion with a double patch neopulmonary artery reconstruction.
The patient was easily weaned from CPB. The postoperative course was complicated by a pulmonary hypertensive episode with low HbSO2 and elevated pCO2, and this prolonged the intubation time to 72 hours, after which it was possible to withdraw ventilatory support.
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Comment
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TGA-IVS associated with APW is highly lethal for two reasons. The first is surgical, in terms of technical challenge, and the second is related to the postoperative course, which may be complicated by pulmonary hypertension.
For surgical treatment of this rare combination, the ASO, although theoretically ideal, is considered technically difficult if not impossible because of the APW [2]. Although Kirshnan and coworkers employed a Senning plus APW patch closure, it was suggested that ASO plus coronary flow diversion would have been a better solution. Several techniques of coronary flow diversion have been proposed by different authors in the presence of TGA with complex coronary patterns [4]. Although such a modification to the conventional ASO can be adopted to treat this anomaly, we believe that when a 1LCx, 2R coronary pattern is present, a conventional ASO can be safely performed utilizing coronary relocation technique described herein [3]. This allows direct coronary flow which is always better than any type of coronary flow diversion.
In patients with increased pulmonary blood flow as a result of a large left to right shunt, pulmonary capillaries become rapidly muscularized and can react to various stimuli [5]. Patients with either TGA or APW are at high risk for this complication after neonatal repair. In the combination described, however, pulmonary hypertension develops earlier. It was the cause of death in the case reported by Krishnan and coworkers [2]. In order to minimize the potential adverse effects of this phenomenon in the postoperative period, we left a 4 mm fenestration at atrial level to allow some decompression in the event of a pulmonary hypertensive episode [6]. Our patient did have a pulmonary hypertensive crisis. During this event, the baby had arterial hemoglobin desaturation but maintained a normal cardiac output due to the fenestration [7]. The condition was reversed and after 72 hours she was successfully extubated, subsequently making an uneventful recovery with discharge on the 10th postoperative day.
TGA with APW is a rare and complex anatomic combination. It’s surgical treatment is technically demanding but a conventional ASO can be achieved when a 1LCx 2R coronary pattern is present. Leaving a fenestration at atrial level is an easy and useful tool that may mitigate the adverse effects of postoperative pulmonary hypertensive crisis.
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Acknowledgments
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We thank Dr Valeria Falsaperla for her artistic input in the medical illustration.
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References
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Vanini V. Aortopulmonary window. In: Kirklin J.W., Barrat-Boyes B.G., eds. Cardiac surgery. New York: Churchill Livingstone, 1993:1153-1157.
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Krishnan P., Balram A., Sambamurthy S.S., Meera R., et al. Complete transposition of the great arteries with aortopulmonary window: surgical treatment and embryologic significance. J Thorac Cardiovasc Surg 1991;101:749-751.
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Abstract
Introduction
