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Ann Thorac Surg 2003;75:1012-1014
© 2003 The Society of Thoracic Surgeons

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Case report

Anatomic correction for corrected transposition after pulmonary unifocalization

^a Department of Pediatric and Congenital Heart Surgery, The Children’s Hospital at The Cleveland Clinic, Cleveland, Ohio, USA

Accepted for publication August 29, 2002.

^* Address reprint requests to Dr Mee, Department of Pediatric and Congenital Heart Surgery, M-41 The Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH 44195, USA
e-mail: meer{at}ccf.org

	Abstract

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A Senning plus Rastelli operation was performed in a patient who had a rare combination of congenitally corrected transposition of the great arteries (S,L,L) with dextrocardia, major aortopulmonary collaterals, and diminutive central pulmonary arteries with arborization defects. The patient required four preparatory operations including bilateral unifocalizations of the aortopulmonary artery collaterals. Pulmonary artery to systemic pressure ratio after the double switch operation was 0.6. The patient demonstrates good biventricular function on echocardiogram at 3 months after the operation.

	Introduction

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The use of the double switch operation (DSO) for the management of congenitally corrected transposition of the great arteries (ccTGA) is becoming increasingly widespread [1]. Surgical management is especially difficult in the presence of diminutive central pulmonary arteries with arborization defects and major aortopulmonary collateral arteries (MAPCAs). We encountered this very rare disease entity, which required staged unifocalization of the MAPCAs before the DSO.

An 8-month-old male patient was referred with ccTGA (S,L,L) with a large ventricular septal defect, atrial septal defect, dextrocardia, pulmonary atresia with very small confluent pulmonary arteries and arborization defects. A total of five MAPCAs supplied nine pulmonary segments in the following pattern: two segments of the right upper lobe, three segments of the right lower lobe, two segments of the left lower lobe, and two segments of the left upper lobe. The central pulmonary arteries measured 1.5 to 2 mm on angiogram. Echocardiography demonstrated normal biventricular function without tricuspid or mitral regurgitation.

Four preparatory operations were performed: operation 1, end-to-side anastomosis of the main PA to the ascending aorta at 8 months old (Melbourne shunt); operation 2, unifocalization of the right lower lobe MAPCA, modified Blalock-Taussig (B-T) shunt using a 5-mm polytetrafluoroethylene (PTFE) graft at 15 months old; operation 3, unifocalization of two MAPCAs to the left lung, 5-mm left B-T shunt at 16 months old; and operation 4, extensive patch angioplasty of the branch pulmonary arteries, division of the three previous shunts, and insertion of a 6-mm PTFE shunt from right innominate artery to the reconstructed pulmonary artery at 23 months old. The systemic oxygen saturation (SaO₂) was maintained at 75% to 80% throughout these operations.

A cardiac catheterization immediately after the last operation revealed a pulmonary vascular resistance of 10 Wood units and systemic vascular resistance of 12.8 Wood units. The patient then underwent repeat cardiac catheterization at 4 years and 4 months. The final study revealed a Qp/Qs of 0.8, mean pulmonary artery pressure of 26 mm Hg, and a pulmonary vascular resistance that had fallen to 4 Wood units.

At 4 years and 10 months of age the patient underwent complete repair at a weight of 15.4 kg. Before the operation a "classic" repair was still an option with ventricular septal defect (VSD) closure and creation of a left ventricle to pulmonary artery connection. However, at operation, there was very limited space on the left ventricular free wall between multiple coronary arteries for conduit placement. Therefore, a Senning-Rastelli operation was performed using an 18-mm Hancock conduit for the right ventricle to pulmonary connection as described [2]. A Shumacker modification [3] of the Senning procedure was used for reconstruction of the pulmonary venous chamber. Patch angioplasties of the left and right upper pulmonary artery branches were also performed (Fig 1). The patient developed complete atrioventricular block after the procedure and a dual-chamber epicardial pacemaker system was implanted on postoperative day 5. The pulmonary artery to systemic pressure ratio was 0.6 after the repair as determined in the operating room.

View larger version (79K): [in this window] [in a new window]   Fig 1. The scheme of the anatomy at the time of final repair.

	Comment

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The combination of pulmonary atresia with MAPCAs and ccTGA is extremely rare; complete repair has not been reported previously. The management strategy for MAPCA was derived from our experience with tetralogy of Fallot with pulmonary atresia. Our operative approach includes creation of a central end-to-side anastomosis of the diminutive main pulmonary artery to the aorta to optimize the chances for growth of the small central pulmonary arteries. Staged operations then are performed to unifocalize the MAPCAs to the central pulmonary arteries. This patient had severe central and first order branch pulmonary artery stenosis after unifocalizaton and required an additional operation for extensive PA reconstruction, take down of the previous shunts, and to insert a single central shunt before final repair.

The theoretical advantages of committing the morphologic left ventricle to the aorta for ccTGA has been extensively discussed [4–6]. In this case, the final decision to proceed with DSO was made intraoperatively because of the coronary distribution on the free wall of the left ventricle and not because of preexisting right ventricular dysfunction or tricuspid regurgitation. Maintaining the lowest possible pulmonary vascular resistance is an important point to ultimately allow right ventricle to pulmonary artery connection at the time of the DSO. Otherwise, the deleterious effects of a right ventriculotomy and future conduit failure can significantly diminish the theoretical benefits of DSO on right ventricular function. Low threshold for conduit replacement will be important for the long-term preservation of right ventricular function in this patient. Despite this patient’s protracted postoperative course with right ventricular dysfunction and the requirement of mechanical ventilatory support, the hemodynamics seem satisfactory at 3 months after surgery with marked improvement of right ventricular function.

	References

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1. Biliciler-Denktas G., Feldt R.H., Connolly H.M., Weaver A.L., Puga F.J., Danielson G.K. Early and late results of operations for defects associated with corrected transposition and other anomalies with atrioventricular discordance in a pediatric population. J Thorac Cardiovasc Surg 2001;122:234-241.[Abstract/Free Full Text]
2. Imamura M., Drummond-Webb J.J., Murphy D., et al. Results of the double switch operation in the current era. Ann Thorac Surg 2000;70:100-105.[Abstract/Free Full Text]
3. Shumacker H.B., Jr A new operation for transposition of the great vessels. Surgery 1961;50:773-777.
4. Imai Y., Sawatari K., Hoshino S., Ishihara K., Nakazawa M., Momma K. Ventricular function after anatomic repair in patients with atrioventricular discordance. J Thorac Cardiovasc Surg 1994;107:1272-1283.[Abstract/Free Full Text]
5. Ohuchi H., Hiraumi Y., Tasato H., et al. Comparison of the right and left ventricle as a systemic ventricle during exercise in patients with congenital heart disease. Am Heart J 1999;137:1185-1194.[Medline]
6. Kirjavainen M., Happonen J.M., Louhimo I. Late results of Senning operation. J Thorac Cardiovasc Surg 1999;117:488-495.[Abstract/Free Full Text]

This article has been cited by other articles:

				F. Brunelli, A. Amaducci, G. Tasca, Z. Mhagna, and B. Amari Staged Correction of Double Discordance, Situs Inversus, and Absence of True Pulmonary Arteries Ann. Thorac. Surg., November 1, 2005; 80(5): e17 - e19. [Abstract] [Full Text] [PDF]

				B. W. Duncan, R. B.B. Mee, C. I. Mesia, A. Qureshi, G. L. Rosenthal, S. G. Seshadri, G. K. Lane, and L. A. Latson Results of the double switch operation for congenitally corrected transposition of the great arteries Eur. J. Cardiothorac. Surg., July 1, 2003; 24(1): 11 - 20. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Brian W. Duncan Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ando, M. Articles by Mee, R. B.B. Search for Related Content PubMed PubMed Citation Articles by Ando, M. Articles by Mee, R. B.B. Related Collections Congenital - cyanotic