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Ann Thorac Surg 1997;64:1782-1785
© 1997 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Mid- to Long-Term Results of the Two-Stage Approach for Type B Interrupted Aortic Arch and Ventricular Septal Defect

Cardiac Institute, Children's Hospital and Health Center, San Diego, California

Accepted for publication May 9, 1997.

	Abstract

Top Abstract Introduction Material and Methods Results Comment References

 
Background. Type B interrupted aortic arch with ventricular septal defect is a complex congenital heart defect that may have associated left ventricular outflow tract obstruction. Surgical management has evolved from a two-stage approach to the currently favored single-stage approach. The following data summarize our experience with the two-stage approach over a 15-year period.

Methods. Between 1980 and 1995, 27 consecutive patients with type B interrupted aortic arch and ventricular septal defect underwent surgical management using the two-stage approach. There were 15 girls and 12 boys; 21 patients had the DiGeorge syndrome.

Results. Stage I was performed at a median age of 4 days. Twenty-six (96%) of 27 patients survived first-stage palliation. One patient survived stage I palliation but died before undergoing stage II. Twenty-five patients underwent second-stage repair at a median age of 6 weeks (range, 1 to 46 weeks). There were 2 early deaths and 1 late death. Actuarial analysis demonstrates 1- and 5-year survival rates of 85% and 81%, respectively. Twenty-two survivors have been followed up for an average of 8 ± 2 years. Freedom from reoperation for arch graft enlargement has been 86% at 3 years and 55% at 5 years. Freedom from reoperation for left ventricular outflow tract obstruction has been 82% at both 3 and 5 years.

Conclusions. The two-stage approach can achieve good mid- to long-term palliation of patients with type B interrupted aortic arch and ventricular septal defect. These results should provide a reference from which to gauge the long-term success of the single-stage approach.

	Introduction

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See also page 1785.

Interrupted aortic arch (IAA) in association with ventricular septal defect (VSD) is a relatively uncommon form of congenital heart defect. Interruption between the left carotid and left subclavian arteries is the most common form of IAA, and it is classified as type B. Many of these patients have an aberrant right subclavian artery (termed B-II) and many also have variable degrees of subaortic stenosis [1, 2]. In addition, IAA and VSD often is seen in conjunction with the DiGeorge syndrome [3].

The surgical approach to IAA and VSD has evolved considerably over the past two decades [4, 5]. Until recently, management of the newborn with IAA consisted of the insertion of a synthetic tube from the ascending to the descending aorta along with the placement of a pulmonary artery band. This approach has been referred to as the two-stage approach [6]. The VSD subsequently was closed at a second operation. Unfortunately, the arch grafts invariably are outgrown, necessitating additional operations.

Because of the shortcomings of the two-stage approach, there has been increasing enthusiasm during the past few years for the one-stage approach, in which the aortic arch is reconstructed and the VSD is closed at a single operation [7, 8]. A number of institutions have demonstrated the feasibility of this approach, which now largely has supplanted the two-stage method [9].

The purpose of this article was to review our experience with the two-stage approach for type B IAA and VSD. This experience extends over a 15-year period, providing lengthy follow-up for most patients in the series. These data should provide a valuable reference point from which to judge the success of the single-stage approach.

	Material and Methods

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This study was a retrospective review of our experience with type B IAA and VSD. The series began in 1980 and concluded in 1995 when we made the institutional decision to begin using the single-stage approach. During the period from 1980 to 1995, 27 patients underwent operation for type B IAA and VSD. There were 15 girls and 12 boys. The average birth weight was 3,120 g (range, 1,800 to 4,200 g).

Data are expressed as means ± SD. Actuarial analysis was performed using the DecVax 11/750 computer (Digital Equipment Corp, Waltham, MA) and the Clinfo Program (Bolt, Beranek, and Newman, Cambridge, MA).

	Results

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Twenty-seven patients underwent operation for type B IAA and VSD. The VSD was characterized as supracristal in 9 patients, posterior malalignment in 16 patients, and other in 2 patients. Arch vessel anatomy was found to be normal (B-I) in 10 patients, whereas 17 patients had an aberrant right subclavian artery (B-II). The aortic valve was characterized as tricuspid in 3 patients, bicuspid in 23 patients, and a variant of unicuspid in 1 patient.

Twenty-one of 27 patients subsequently have proven to have the DiGeorge syndrome. This currently is determined by cytogenetic tests demonstrating the 22q deletion. Seventy-one percent of the patients with the DiGeorge syndrome had no thymus, whereas 29% did have a thymus. Six patients did not have the DiGeorge syndrome, and all 6 of these patients had thymic tissue present.

All 27 patients underwent first-stage repair. This operation was performed through a left thoracotomy. The tube graft was placed end-to-side onto both the ascending and descending aortas without the use of cardiopulmonary bypass. Pulmonary artery bands were placed in 26 of 27 cases. The median age at operation was 4 days (range, 1 to 17 days). The sizes of the ascending-to-descending arch grafts were as follows: 10 mm (n = 2), 8 mm (n = 15), 6 mm (n = 7), 2 x 6 mm (n = 1), and 5 mm (n = 2).

Twenty-six (96%) of 27 patients survived first-stage palliation. There was 1 death in a patient who presented in extremis and who died of multisystem organ failure 2 days after operation. A second patient survived stage I but died of intractable heart failure at 3 months of age before undergoing the second stage.

Twenty-five patients underwent second-stage repair. This operation consisted of synthetic patch repair of the VSD, removal of the pulmonary artery band, and patch reconstruction of the band site. The median age at operation was 6 weeks (range, 6 days to 46 weeks). Five of 25 patients had the second stage performed within 2 weeks of the first stage because of heart failure or pulmonary artery distortion. Six patients underwent second-stage repair between 3 and 6 weeks of age.

Repair of the VSD was performed using continuous cardiopulmonary bypass in 6 patients, whereas a period of circulatory arrest was used in 19 patients. When used, the mean duration of circulatory arrest was 35 ± 8 minutes (range, 13 to 53 minutes). The mean cross-clamp time was 43 ± 10 minutes (range, 26 to 64 minutes) and the mean bypass time was 83 ± 24 minutes (range, 45 to 127 minutes). The VSD was repaired through the atrium in 13 patients and through the ventricle in 12 patients. Two patients required pacemaker insertion after repair of the VSD.

Of the 25 patients who underwent stage II repair, there were 2 (8%) early deaths. One patient sustained a cardiac arrest on the evening of operation and could not be resuscitated. A second patient died of sepsis and multisystem organ failure 7 weeks after operation. There were 23 survivors of second-stage repair, with 1 late death. The latter patient had been doing well from a clinical standpoint, but died suddenly at home. This was 1 of 2 patients who had required pacemaker insertion after stage II repair.

The 22 survivors have been followed up for an average of 8 ± 2 years. Follow-up is available in 100% of these patients. Actuarial survival for all 27 patients in this series is shown in Figure 1. The 1-year and 5-year survival rates are 85% and 81%, respectively.

View larger version (16K): [in this window] [in a new window]   Fig 1. . Actuarial survival analysis of 27 patients presenting with type B interrupted aortic arch and ventricular septal defect.

View larger version (17K): [in this window] [in a new window]   Fig 2. . Freedom from reoperation for enlargement of ascending-to-descending arch graft.

View larger version (15K): [in this window] [in a new window]   Fig 3. . Freedom from reoperation for management of left ventricular outflow tract obstruction.

	Comment

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Reoperations for enlargement of the aortic arch grafts have been performed in 12 of 22 survivors of the two-stage approach. It is evident that 100% of the patients eventually will need this operation. Thus, this is a three-stage approach at best. The need for arch graft enlargement usually is determined in the cardiac catheterization laboratory. We recommend reoperation when the ascending-to-descending aortic pressure gradient exceeds 35 to 40 mm Hg. This gradient cannot be assessed by clinical examination in patients who have an aberrant right subclavian artery (63% incidence of B-II anatomy in our series), because all four extremity vessels arise from the descending aorta with this anatomy. It also is evident that the size of the tube graft placed at stage I will influence the timing of the subsequent reoperation [4]. Unfortunately, many patients with type B IAA and VSD have a small ascending aorta, making it difficult to place a large graft at the first operation.

Reoperations directed at relief of left ventricular outflow tract obstruction have been performed in 4 of 22 survivors. All four patients who required relief of left ventricular outflow tract obstruction had B-II arch anatomy, confirming the previous reports suggesting this association [10, 11]. The average age at operation was 13 months (range, 10 to 19 months). Conversely, 18 patients have not required left ventricular outflow tract procedures; these patients have an average left ventricular gradient of 10 mm Hg (measured by catheterization or estimated by echocardiography; range, 0 to 30 mm Hg). There has been no progression of gradient in these patients as they have been followed up longitudinally. Thus, it is our impression that the need for operation directed at left ventricular outflow tract obstruction is limited to a relatively small percentage of patients and will declare itself early in the clinical course [12].

The single-stage approach currently is the preferred therapy for IAA and VSD. There are many advantages of this approach over the two-stage approach, including fewer operations, the possibility of arch growth [13], and the avoidance of pulmonary artery banding, which could exacerbate subaortic stenosis [14]. Excellent results have been reported in a number of single-institution series [15]. The Congenital Heart Surgeon's Society multi-institutional study reported a 35% operative mortality rate [16], suggesting that there is a learning curve with this procedure [17]. Mid- to long-term follow-up of the single-stage approach has not yet been reported.

In summary, the two-stage approach historically has provided a means of managing type B IAA and VSD. Our single-institution experience with this approach has yielded good mid- to long-term results. The principal disadvantage of this approach is the need for multiple reoperations.

	References

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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): Richard D. Mainwaring John J. Lamberti Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mainwaring, R. D. Articles by Lamberti, J. J. Search for Related Content PubMed PubMed Citation Articles by Mainwaring, R. D. Articles by Lamberti, J. J. Related Collections Related Article