HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract 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): Thierry Carrel Alain Serraf François Lacour-Gayet Claude Planché Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Carrel, T. Articles by Planché, C. Search for Related Content PubMed PubMed Citation Articles by Carrel, T. Articles by Planché, C.

Ann Thorac Surg 1996;61:940-944
© 1996 The Society of Thoracic Surgeons

---

Original Articles: Cardiovascular

Transposition of the Great Arteries Complicated by Tricuspid Valve Incompetence

Department of Pediatric Cardiac Surgery, Marie-Lannelongue Surgical Center, University Paris Sud, Le Plessis Robinson, France

Accepted for publication November 30, 1995.

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Background. Tricuspid valve insufficiency secondary to structural anomalies of the valve itself or to an iatrogenic complication of the Rashkind procedure is very rarely associated with transposition of the great arteries. This condition represents an interesting perioperative challenge. Rapid restoration of the tricuspid valve to a low-pressure system by arterial switch operation associated with tricuspid repair should theoretically improve the outcome in terms of myocardial and valve function.

Methods. Thirteen of 839 patients who underwent an arterial switch operation for various forms of transposition of the great arteries presented with moderate to severe tricuspid insufficiency. Three of them had a ventricular septum defect. Nine experienced severe cardiac failure with profound hypoxemia. Ventilatory support was necessary in 7, 6 had renal or hepatic dysfunction, and 5 had coagulation disorders. Inotropic support was started preoperatively in 8 patients.

Results. Tricuspid lesions were as follows: primary annular dilatation and lack of coaptation at the commissural level (n = 1), straddling tricuspid valve (n = 1), redundant tricuspid valve tissue leading to left ventricular outflow tract obstruction (n = 1), small cleft of the septal leaflet (n = 1), and dysplastic valve tissue with juxtacommissural regurgitation (n = 1). In 8 patients, the cause of the tricuspid valve insufficiency was most probably an iatrogenic lesion, with rupture of the papillary muscle (n = 2), rupture of the chordae (n = 1), or tear of the anterior leaflet (n = 5), whereas no clear cause could be found in 1 patient. Repair consisted of the arterial switch operation associated with tricuspid valve repair in 10 patients. In 2 patients with only discrete anomaly and in 1 without a clear cause of tricuspid regurgitation, no valve repair was performed. Three patients had their ventricular septal defect closed. There were one early and one late death, both not related to the tricuspid lesions. Late postoperative (mean, 6.5 years) evaluation revealed normal left ventricular function in 10, with no tricuspid incompetence in 7 and trivial tricuspid insufficiency in 3.

Conclusions. Restoration of an incompetent tricuspid valve in a low-pressure system by the arterial switch operation combined with valve repair provides good ventricular and valvar results. Preoperative management and appropriate timing of operation seem to be of utmost importance.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Transposition of the great arteries (TGA) can be associated with a wide spectrum of intracardiac and extracardiac lesions. One of these is tricuspid valve incompetence (TVI); the etiology includes structural abnormalities of the valve itself and iatrogenic lesions during the Rashkind procedure [1–5]. The preoperative management and the optimal timing of operation sometimes represent a true challenge in this particular situation.

Nowadays, the arterial switch operation is probably the best option to achieve definitive surgical repair [6, 7], but its long-term advantages compared with those of atrial repair have not been definitely established. The main advantages of the anatomical repair are obvious: use of the left ventricle to provide systemic stroke work without creation of an additional intracardiac anomaly. Moreover, it appears that the risk of sudden death is less with the arterial switch operation, whereas the atrial inversion repair shows a constant hazard phase that extends indefinitely [8, 9]. In late presentation, the rapid two-staged procedure represents the most attractive approach, which may achieve excellent results; a delayed venous inflow inversion remains a low-risk procedure [10].

In case of moderate to severe tricuspid valve incompetence, the arterial switch operation constitutes a ``mandatory'' indication because this procedure alone restores low pressure across the tricuspid valve. This seems to be particularly important to achieve good or excellent early and long-term valve function, whereas every intraatrial repair might worsen the fate of the tricuspid valve.

To date, relative little attention has been directed toward the challenging problems of patients presenting with simple or complex TGA complicated by tricuspid valve dysfunction. The aim of this report is to present our concepts of management and to analyze the perioperative course of 13 patients who presented with this rare association.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Between April 1984 and July 1995, a total of 839 arterial switch operations were performed in 568 patients with TGA and intact ventricular septum and in 271 patients with more complex forms, including mainly TGA with ventricular septal defect (VSD) and aortic coarctation. Of this large series of neonates and infants, we identified 13 patients (10 males, 3 females; mean weight at the first operation, 4.1 kg; range, 3.1 to 10.6 kg) who presented with moderate to severe TVI. The median age of the patients was 23 days, extending from 4 to 216 days. Eight patients presented with TGA and intact ventricular septum, 3 with TGA and VSD, and 1 with combined TGA, VSD, and CoA, whereas the last patient was demonstrated to have a Taussig-Bing anomaly with subpulmonary stenosis. Eleven patients had undergone a Rashkind atrioseptostomy before either palliative or reconstructive operation. During this period, no patient with TVI was operated on for TGA using intraatrial inflow inversion.

Preoperative examinations consisted of echocardiography in all patients and cardiac catheterization in 9 of them. The following structural anomalies of the tricuspid valve were found: primary annular dilatation and lack of coaptation at the commissural level (n = 1), straddling tricuspid valve (n = 1), redundant tricuspid valve tissue leading to left ventricular outflow tract obstruction (n = 1), small cleft of the septal leaflet (n = 1), and dysplastic valve tissue with juxtacommissural regurgitation (n = 1). In 7 patients, the cause of the TVI was most probably an iatrogenic lesion during the Rashkind maneuver, with rupture of the papillary muscle (n = 2), rupture of the chordae (n = 1), and tear of the anterior valve leaflet (n = 5).

Figures 1 to 3 demonstrate the principal findings in 3 different patients with severe tricuspid regurgitation. In the most extreme form depicted in Figure 1B, there was an absence of antegrade filling from the right ventricle into the ascending aorta, and the coronary arteries were filled in a retrograde way through the patent ductus. Although not consistent with the hemodynamic situation due to an iatrogenic lesion, the central venous pressure decreased significantly after the Rashkind procedure in an majority of the patients.

View larger version (68K): [in this window] [in a new window]   Fig 1. . (A) Right ventricular injection in a patient with transposition of the great arteries and intact ventricular septum shows absent antegrade filling of the ascending aorta but severe tricuspid regurgitation due to rupture of the anterior leaflet. (B) Filling of the left ventricle demonstrates antegrade flow to the pulmonary artery and through the ductus, which contributes to the perfusion of the descending aorta and retrograde filling of the ascending aorta and the coronary arteries.

View larger version (61K): [in this window] [in a new window]   Fig 2. . Preoperative echocardiogram of a neonate after balloon atrial septostomy. The septal leaflet is prolapsing into the right atrium (RA) due to a rupture of the papillary muscle (white arrow). (LA = left atrium; LV = left ventricle; RV = right ventricle.)

View larger version (126K): [in this window] [in a new window]   Fig 3. . Preoperative angiogram of a neonate presenting with transposition of the great arteries, ventricular septal defect, and tricuspid valve dysplasia, complicated by severe tricuspid valve incompetence. Intraoperative inspection revealed annular dilatation, lack of coaptation at the commissural level, and redundant leaflet tissue. Repair consisted of resection of the dystrophic tissue and Wooler annuloplasty.

Cardiopulmonary bypass was instituted after aorto-bicaval cannulation and conducted in deep hypothermia (20° to 24°C). After we ensured that the left ventricle would be conditioned enough to support systemic circulation, 10/13 patients underwent a primary arterial switch operation using similar surgical technique and myocardial protection [11]. The VSD was closed with a small prosthetic patch in 3 patients. Two patients underwent prior palliative operation, consisting of repair of aortic coarctation, pulmonary banding, and systemic--pulmonary shunt in 1 and isolated pulmonary banding in a patient who presented late with the Taussig-Bing anomaly. The arterial switch repair was performed 10 days later in the first and 5 years later in the second patient.

Tricuspid valve repair consisted of reinsertion of the papillary muscle (n = 2), reinsertion of the ruptured chordae combined with quadrangular resection (n = 1), direct suture of the leaflet tear (n = 4), annuloplasty at the commissural level (n = 2), closure of a small cleft (n = 1), and resection of redundant valvular tissue (n = 1). In 2 patients with only discrete anomaly of the tricuspid valve and in the patient without a clear cause of tricuspid incompetence, no valve repair was performed.

	Results

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Operative mortality was 7.6% (1/13). This patient, operated on at the beginning of the experience with the arterial switch operation (1985), presented with a single coronary artery. Initially, he could be weaned from the cardiopulmonary bypass without difficulty and with a normal electrocardiogram. Unfortunately, hemodynamic deterioration developed several hours postoperatively and cardiac resuscitation was unsuccessful. In 5/13 patients, the sternal wound was left open at the end of the operation and could be closed after a mean interval of 3.5 days. Significant complications were observed in 6 patients, including transient global or left ventricular failure requiring extensive inotropic and vasodilator support in 5 patients.

Preoperative renal failure persisted 3 weeks postoperatively in 2 patients, 1 of whom was treated with peritoneal dialysis. Hepatic dysfunction manifested by hyperbilirubinemia and elevated transaminase levels but with normal coagulation parameters occurred in another patient.

A late postoperative death was observed in a child after 3 months, due to septic multiorgan failure, which most probably originated from pulmonary infection. This child had left our hospital 1 month after the operation and was admitted to the referring hospital with septicemia 2 months later. After a mean follow-up of 6.5 years, all survivors are asymptomatic; 2 of them are on a mild diuretic therapy. Two patients are in the lowest group of body weight according to the percentile distribution. Late postoperative echocardiographic examination was available in every patient and demonstrated absence of tricuspid regurgitation in 8 patients and slight to moderate regurgitation in 3 patients, 1 of them presenting with an enlarged right atrium. Interestingly, only 1 patient who had tricuspid valve repair had a moderate regurgitation, whereas 2 of the 3 patients without valve repair demonstrated slight TVI.

The left ventricular contractility was demonstrated to be normal in 9 patients, whereas 1 patient demonstrated late postoperative dyskinesia of the septal and posterior walls. One patient suffered from postintubation laryngeal stenosis and needed parenteral nutrition during several months.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Transposition of the great arteries complicated by TVI is a very rare association in the broad spectrum of intracardiac and extracardiac anomalies encountered in TGA. This critical situation requires a well-defined preoperative management as well as optimal timing of operation. It may be associated with a substantial neonatal morbidity and mortality. Our experience demonstrates that early restoration of the tricuspid valve in the low-pressure circuit during the arterial switch procedure achieves very satisfactory early and late results. Therefore, we recommend a ``mandatory'' arterial switch operation soon after birth, eg, after initial cardiopulmonary stabilization and treatment of hepatic or renal failure. In late presentation, conditioning the left ventricle with subsequent anatomic repair probably represents a better option than the atrial inversion repair.

The adequacy of cardiac output and the level of tissular oxygenation is directly related to the magnitude of the TVI. Also, the right ventricular overloading associated with minimal shunting through the ductus arteriosus tends to decrease the suitability of the left ventricle to sustain systemic loading conditions. Therefore, the notions of effective pulmonary and systemic blood flow represent the most important determinants of the systemic oxygen saturation [4, 8, 12]. In neonates with TGA and severe TVI, the majority of the right ventricular volume regurgitates into the right atrium. In these cases, there may be a significant drop in systemic pressure and the perfusion of the coronary arteries becomes ductus-dependent. This reduction in transaortic blood flow and the associated pulmonary steal phenomenon lead to further desaturation and multiorgan failure.

The preoperative left ventricular function represents the most important determinant of a successful arterial repair. In our experience with TGA and severe TVI, the left ventricle returns sooner to low pressure and might not be suitable to sustain systemic circulation. Furthermore, the right ventricle may dilate rapidly after development of TVI and compress the smaller posterior left ventricle. For this reason, it is extremely important to assess left ventricular function at short intervals by echocardiography [10, 12, 13].

Preoperative intensive care treatment is directed to cardiopulmonary stabilization and restoration of normal renal and hepatic function. The coagulation should also be optimized. We consider these patients suitable for an arterial switch operation when there is a trend toward recovery of vital organs. When this is not the case, the rapid two-stage procedure might be an another option, providing that the global cardiac status is not worsening [10].

Although the trend to earlier repair has generally been widely accepted, the Rashkind maneuver remains part of the standard therapy in neonates presenting with cyanotic D-transposition of the great arteries, despite the effectiveness of prostaglandin infusions in alleviating systemic hypoxemia [4, 5, 8]. The complication rate of the Rashkind procedure has been reported to range between 0.5% and 2%, including mainly injuries of the atrioventricular valves and rhythm disturbances in addition to the common risks of neonatal cardiac catheterization. Dealing with the necessity of balloon atrioseptostomy, Waldhausen's group demonstrated that selected infants with a nonrestrictive patent foramen ovale may undergo successful early arterial switch repair of TGA without prior balloon atrial septostomy. In this report, their decision to perform the Rashkind procedure was based on assessment of clinical cardiopulmonary condition, arterial blood gas, and pH values before and after prostaglandin infusion. The absence of prior balloon septostomy had no significant negative impact on the survival of infants who underwent the arterial switch repair [14]. The somewhat higher incidence of iatrogenic lesions consecutive to the balloon septostomy described here can probably be explained by the fact that several children came from developing countries with less experience in invasive cardiology.

The etiology of tricuspid valve dysfunction in TGA includes congenital malformations and iatrogenic lesions during the Rashkind procedure [1, 2, 3, 15]. In the most complete overview of tricuspid valve abnormalities, Huhta and associates [1] described 38 autopsy specimens from patients with TGA and VSD. Abnormalities (n = 40) encountered included severe straddling of the stretch apparatus, valvular dysplasia, left ventricular outflow tract obstruction by accessory tissue, double orifice, and abnormal chordal insertion. In only 14% was the abnormality judged to have a significant impact on surgical management. Malformations of the tricuspid valve represent a very rare cardiac anomaly and can be divided into two main groups: those in which the primary lesion is downward displacement of the basal attachment of one or two leaflets (known as Ebstein's malformation) and those without downward displacement but with deformation of the leaflets or the tension apparatus, usually described as dysplasia [15].

Within the anomalies that may be associated with TGA, the TVI may dictate the surgical option. Independent of the quality of the valve repair, keeping this valve in the systemic circulation increases the risk and the rapidity of development of postoperative valvular and myocardial dysfunction. For this reason, we believe that atrial inflow inversion is contraindicated in this particular pathology. The obvious disadvantage of redirecting venous inflow is that the left ventricle continues to work as a pulmonary ventricle, while the anatomic right ventricle and the tricuspid valve must continue to function in the systemic circulation. Furthermore, intraatrial operation alone can compromise the valve repair. Some functional deterioration does occur late after total correction [9]: the incidence of systemic ventricular failure was as high as 7.7% and systemic atrioventricular valve incompetence necessitated operation in 3 patients. Even in infants presenting late with TGA, atrial inflow inversion should be considered as last surgical option. In these patients, a two-staged approach-prior pulmonary banding with systemic--pulmonary shunt to prevent severe desaturation followed by arterial switch-seems to give the best results [10]. We performed this type of operation in 2 patients with very good results. Both children recovered perfectly after the arterial switch operation, and the tricuspid valve function is very satisfactory 7 and 9 years postoperatively.

The postoperative management of such patients corresponds closely to that applied after arterial switch in simple TGA. Delayed closure of the sternum is routine when unstable postoperative hemodynamics are encountered. Current medical treatment includes moderate inotropic support in association with vasodilators and diuretics. The coagulation disorders are corrected in the operating room. The need for ventilatory support and the requirement for inotropic support were longer than after arterial switch for simple TGA.

The perioperative death in our series was most probably not related to the presence of tricuspid incompetence but occurred secondarily to a coronary relocation problem. Although preoperative renal or hepatic dysfunction persists, usually during the first postoperative week, peritoneal dialysis is routinely used in these patients.

In conclusion, the ideal surgical procedure in TGA should have a low perioperative morbidity and mortality, a reasonable incidence of late complications, and ability to restore complete anatomic and physiological function [16]. In patients presenting with TGA, with or without VSD, complicated by moderate to severe TVI, we believe that a ``mandatory'' arterial switch operation is indicated in every case. Rapid restoration of the tricuspid valve into the low-pressure system during the arterial switch combined with tricuspid repair improves the myocardial and valvular outcomes. In late presentation, conditioning of the left ventricle by banding of the pulmonary artery followed by the anatomic repair may be a better therapeutic option than intraatrial inflow inversion.

	Acknowledgments

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Doctor Carrel was supported by a grant from the Clinic for Thoracic and Cardiovascular Surgery [Prof U. Althaus], University Hospital, CH-3010 Berne, Switzerland.

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Address reprint requests to Dr Serraf, Department of Pediatric Cardiac Surgery, Marie-Lannelongue Surgical Center, 133 av. De la Résistance, F-92350 Le Plessis Robinson, France.

	References

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 

1. Huhta JC, Edwards WD, Danielson GK, Feldt RH. Abnormalities of the tricuspid valve in complete transposition of the great arteries with ventricular septal defect. J Thorac Cardiovasc Surg 1982;83:569–76.[Abstract]
2. Aziz KU, Paul MH, Muster AJ, Idriss FS. Positional abnormalities of atrioventricular valves in transposition of the great arteries. Am J Cardiol 1979;44:1135–9.[Medline]
3. De Vivie R, van Praagh S, Bein G, Eigster G, Vogt J, van Praagh R. Transposition of the great arteries with straddling tricuspid valve. Report of two rare cases with acquired subaortic stenosis after main pulmonary artery banding. J Thorac Cardiovasc Surg 1989;98:205–13.[Abstract]
4. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy: palliative approach to the transposition of the great arteries. JAMA 1966;196:991–4.[Abstract/Free Full Text]
5. Simon G, Camus L, Auriacombe L, Thibert M. L'atrioseptostomie de Rashkind: à propos de 100 observations. Coeur 1973;4:11–5.[Medline]
6. Stark J. Transposition of the great arteries: which operation? Ann Thorac Surg 1984;38:429–31.[Medline]
7. Castañeda AR, Mayer JE, Jonas RA. Transposition of the great arteries: the arterial switch operation. Clin Cardiol 1989;7:369–74.
8. Castañeda AR, Jonas RA, Mayer JE, Hanley FL. D-Transposition of the great arteries. In: Cardiac surgery of the neonate and infant. Philadelphia: Saunders, 1993:409-37.
9. Turina M, Siebenmann R, von Segesser LK, Schönbeck M, Senning A. Late functional deterioration after atrial correction for transposition of the great arteries. Circulation 1989;80(Suppl 1):162–7.
10. Jonas RA, Giglia TM, Sanders S. Rapid, two-stage arterial switch for transposition of the great arteries and intact ventricular septum beyond the neonatal period. Circulation 1989;80(Suppl 1):203–8.
11. Serraf A, Lacour-Gayet F, Bruniaux J, et al. Anatomic correction of transposition of the great arteries in neonates. J Am Coll Cardiol 1993;22:193–200.[Abstract]
12. Sidi D, Heurtematte Y, Kachaner J, et al. Problèmes posés par la préparation du ventricule gauche à la correction anatomique de la transposition simple des grands vaisseaux. Arch Mal Coeur 1983;76:575–9.
13. Wernovsky G, Giglia TM, Jonas RA, et al. Course in the intensive care unit after preparatory pulmonary artery banding and aortopulmonary shunt placement for transposition of the great arteries with low left ventricular pressure. Circulation 1992;86(Suppl 1):133–8.
14. Baylen BG, Grzestczak M, Gleason ME, et al. Role of balloon atrial septostomy before early arterial switch repair of transposition of the great arteries. J Am Coll Cardiol 1992;19: 1025–31.[Abstract]
15. Lang D, Oberhoffer R, Cook A, et al. Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life. J Am Coll Cardiol 1991;17:1161–7.[Abstract]
16. Castañeda AR, Trusler GA, Paul MH, et al. The early results of treatment of simple transposition in the current era. J Thorac Cardiovasc Surg 1988;95:14–9.[Abstract]

This article has been cited by other articles:

				J. L. Myers Transposition of the Great Arteries Ann. Thorac. Surg., March 1, 1997; 63(3): 895 - 898. [Full Text]

This Article Abstract 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): Thierry Carrel Alain Serraf François Lacour-Gayet Claude Planché Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Carrel, T. Articles by Planché, C. Search for Related Content PubMed PubMed Citation Articles by Carrel, T. Articles by Planché, C.