Ann Thorac Surg 1999;68:571-573
© 1999 The Society of Thoracic Surgeons
Case Reports
Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, Ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries
Pietro A. Abbruzzese, MDa,
Renzo Bianco, MDa,
Marco Cavaglià, MDc,
Gianni Ciriotti, MDb,
Alberta Rizzo, MDc
a Divisioni di Cardiochirurgia, Ospedale Infantile Regina Margherita, Torino, Italy e
b Divisioni di Cardiologia, Ospedale Infantile Regina Margherita, Torino, Italy e
c Divisioni di Servizio di Anestesia e Rianimazione Cardiaca, Ospedale Infantile Regina Margherita, Torino, Italy
Address reprint requests to Dr Abbruzzese, Divisione di Cardiochirurgia, Ospedale Infantile Regina Margherita, Piazza Polonia 94, 10126 Torino, Italy
e-mail: cardio{at}domino.it
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Abstract
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The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An Ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.
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Introduction
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Reconnection of the left ventricle with the aorta, and of the right ventricle with the pulmonary artery, seems nowadays to be the best corrective option in selected cases of corrected transposition of the great arteries [1]. When a ventricular septal defect and pulmonary stenosis or atresia are present, a Rastelli operation is performed together with one of the atrial switches. Under these circumstances, one or more shunting procedures are often performed before repair, sometimes distorting the pulmonary arteries. Furthermore, additional complicating features are frequent in corrected transposition [2], making the final surgical procedure long and cumbersome.
A cyanotic 6-year-old boy was admitted to our unit with a diagnosis of S,L,L, ventricular septal defect, pulmonary atresia, and two previous systemic-to-pulmonary shunts. Additionally, the child had severe Ebstein anomaly of the tricuspid valve, significantly decreasing the size of the right ventricle, and dextrocardia. A new cardiac catheterization better defined the anatomy: the pulmonary arteries were confluent but severely distorted with distal bilateral stenoses, especially on the right side. The entire proximal right pulmonary artery was hypoplastic (Fig 1). Both shunts were functioning with anastomotic strictures. The ventricular septal defect was perimembranous and nonrestrictive. The tricuspid valve was downward displaced but it was not insufficient. A rapid two-stage repair was planned and performed.
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Fig 1. Frontal view of pulmonary angiogram, showing distortion and bilateral stenoses of the pulmonary branches.
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Initially, at 24°C, a bidirectional cavopulmonary anastomosis was performed without aortic cross-clamping; pulmonary branches were enlarged from hilum-to-hilum, using the cavopulmonary anastomosis itself to enlarge the distal right pulmonary artery, while the remaining right and the whole left pulmonary arteries were patched with bovine pericardium. Short periods of low flow were necessary to obtain a bloodless field. Bypass was easily discontinued after 242 minutes. Good hemodynamics were observed with an O2 saturation of 70%–75%. The patient was transferred to the intensive care unit in good condition.
Three days later, through the same incision and cannulation sites, cardiopulmonary bypass was reestablished and the temperature was lowered to 24°C. The aorta was cross-clamped and cold crystalloid cardioplegia was administered every 20 minutes. The right atrium, whose free wall was poorly represented, because of the overlapping of the rightward oriented ventricular mass, was opened with a transverse incision from the base of the right atrial appendage to the interatrial septum. The interatrial septum was removed, the coronary sinus was cut back, and a bovine pericardial baffle was prepared for the hemi-Mustard procedure. A longitudinal right ventricular incision was next performed, starting approximately 5 mm below the aortic valve. The ventricular septal defect was tunneled to the aorta through the ventriculotomy, using a baffle obtained from a 20-mm corrugated Dacron tube. Next, the atrial switching procedure was performed. The previously prepared bovine pericardial baffle was sutured as for an ordinary Mustard procedure, except that the superior suture line was carried directly to the superior remnants of the atrial septum. The atrial incision was directly closed. Finally, a 20-mm porcine-valved conduit was tailored, distally anastomosed to the pulmonary bifurcation, and then proximally to the edges of the ventriculotomy. The aorta was unclamped after 135 minutes. With a normal sinus rhythm, cardiopulmonary bypass was discontinued after 296 minutes. The patient had an uneventful recovery and remains well 3 months after the operation. A postoperative echocardiogram shows an adequate repair with no residual shunts nor significant gradients at any level.
This case summarizes the possible complexity of surgical treatment of congenitally corrected transposition of the great arteries. In addition to pulmonary atresia and ventricular septal defect, this child had two other typical malformations: an Ebstein anomaly of the tricuspid valve, significantly decreasing the size of the right ventricle, and a dextrocardia in S,L,L that obscured the right atrium and decreased the extent of its free wall. Moreover, the two previously performed systemic-to-pulmonary shunts had severely distorted and stenosed both pulmonary arteries. A rapid two-stage approach was a convenient method of solving all problems with the least operative risk.
The small size of the right ventricle made its capability of handling the entire cardiac output doubtful. Therefore, it was decided to adopt a one and a half ventricle type of repair [4], in which the systemic venous return was partially diverted to the pulmonary arteries by a bidirectional cavopulmonary anastomosis, which decreased the volume load of the right ventricle. Moreover, the presence of a dextrocardia would have made a Senning or a Mustard procedure difficult to perform and, therefore, exclusion of the superior vena cava from the atrial baffle made the procedure simpler.
The other major problem was the need of hilum-to-hilum enlargement of the pulmonary artery branches. This was solved by a very distally performed cavopulmonary anastomosis and by patching the remaining length of the pulmonary arteries with bovine pericardium.
A rapid two-stage approach avoided extremely long cardiopulmonary bypass and cross-clamping times. A meticulous enlargement of the pulmonary arteries and the bidirectional cavopulmonary anastomosis were the only procedures performed during the first operation, well preparing the second stage.
In conclusion, a very complex corrective procedure could be staged, thus allowing more time for accurate completion of repair. The very high incidence of tricuspid anomalies and right ventricular malformations observed in corrected transposition of the great arteries [3] makes the so-called one and one half ventricle repair an attractive option in most of these patients, therefore widening the possible applications of this rapid two-stage approach.
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References
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Imai Y. Double switch operation for congenitally corrected transposition. Adv Card Surg 1997;9:65-86.[Medline]
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Ilbawi M.N., DeLeon S.Y., Backer C.L., et al. An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia. J Thorac Cardiovasc Surg 1990;100:410-415.[Abstract]
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Van Praagh R., Papagiannis J., Grunenfelder J., Bartram U., Martanovic P. Pathologic anatomy of corrected transposition of the great arteries. Am Heart J 1998;135:5:772-5:785.
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Miyaji K., Shimada M., Sekiguchi A., Ishizawa A., Isoda T., Zunemoto M. Pulmonary atresia with intact ventricular septum. " Ann Thorac Surg 1995;60:1762-1764.[Abstract/Free Full Text]
Accepted for publication January 13, 1999.
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Abstract
Introduction
