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Ann Thorac Surg 1999;68:983-988
© 1999 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Management of ventricular septal defect: a survey of practice in the United Kingdom

^a Departments of Pediatrics and Surgery, National Heart and Lung Institute, Imperial College School of Medicine, and Royal Brompton and Harefield NHS Trust Hospitals, London, England, United Kingdom

Address reprint requests to Prof Anderson, Section of Paediatrics, Royal Brompton Campus, National Heart and Lung Institute, Imperial College School of Medicine, Dovehouse St, London SW3 6LY, England
e-mail: r.anderson{at}ic.ac/uk

	Abstract

Top Abstract Introduction Material and methods Results Comment Appendix References

 
Background. A survey of pediatric cardiac surgeons was performed to establish current opinions in the United Kingdom concerning closure of ventricular septal defect.

Methods. Questionnaires were sent to 14 pediatric cardiac centers in 1995 (16 surgeons, 100% response), and again in 1997 (20 surgeons, 100% response).

Results. Results are presented for 1997, with findings from 1995 shown in parentheses. Eleven (6) surgeons used bypass exclusively, 9 (10) sometimes used circulatory arrest. Operative techniques were similar, although the material used for the patch varied. Multiple defects were approached via the transatrial route by 18 (15), right ventriculotomy by 11 (7) and left ventriculotomy by 7 (6). The juxta-arterial defect was approached via the transpulmonary route by 16 (13), a combination by 9 (11), transatrial by 10 (6), and transventricular by 9 (5). The most common indications for pulmonary arterial banding were "Swiss cheese" defect for 13 (13), and functionally single ventricle for 5 (6). Ventricular septal defect associated with coarctation was repaired in two stages by 13 (10), a single stage by 5 (3), or either by 1 (3).

Conclusions. Pediatric cardiac surgeons in the United Kingdom demonstrate a uniform, evidence-based approach to the management of ventricular septal defect.

	Introduction

Top Abstract Introduction Material and methods Results Comment Appendix References

 
Closure of a ventricular septal defect is the most common pediatric cardiac operation. Such defects occur both in isolation and as part of most complex forms of congenital heart disease. The first successful procedure was reported in 1955 [1], but there remain several areas of controversy in management. Because of this, we conducted a survey of pediatric cardiac surgeons to establish current opinions in the United Kingdom.

	Material and methods

Top Abstract Introduction Material and methods Results Comment Appendix References

 
In 1995, questionnaires were sent to 14 pediatric cardiac centers to ascertain uniformity of techniques for closure. In 1997, the same questionnaire was sent to all pediatric cardiac surgeons in the United Kingdom to update the results, to see whether there had been any changes of opinion, and to ask permission to publish the results. The questionnaire is reproduced in the Appendix. Part of the questionnaire included illustrations of the technique used by the organizer of the survey (DFS) to close the various types of ventricular septal defect (Fig 1). Each of the surgeons were asked to comment if, and how, their own technique varied from that used by Mr Shore. It could have been advantageous to ask each surgeon to illustrate the techniques used independently, but we judged we would receive a more structured response if we circulated the same figures for all to study. The answers were tabulated using an Excel spreadsheet (Microsoft Corporation, Redmond, WA). Results for the 2 years were evaluated and compared. Numbers were too small for formal statistical analysis, but trends and areas of controversy were highlighted by comments made by respondents.

View larger version (66K): [in this window] [in a new window]   Fig 1. These drawings show the surgical technique used in our institution by the senior author of this paper (D.F.S.) to close some anatomic variants of ventricular septal defect. We illustrated (A) closure of a perimembranous defect opening to the right ventricular outlet via the right ventricle, (B) closure of a comparable perimembranous defect transatrially, (C) closure of a muscular inlet defect, and (D) closure of the muscular inlet defect when associated with a perimembranous defect. The surgeons were asked (question #12) to indicate how their own techniques differed from those illustrated. See text for discussion.

	Results

Top Abstract Introduction Material and methods Results Comment Appendix References

Support technique
The most common support technique was bypass, with 11 (6) surgeons using bypass exclusively. The remaining 9 (10) used both bypass and circulatory arrest. Six (5) of the surgeons using circulatory arrest specified a weight for the patient of less than 2.5–5 kg. (In 1995, 2 surgeons used circulatory arrest for patients below the ages of 3 months and 12 months, respectively.)

Approach
Transatrial closure was always attempted by 15 (9) surgeons, and nearly always by 3 (3). Both transatrial and transventricular routes were used by 2 (0). Two (1) surgeons mentioned transpulmonary closure, 1 was conducting a randomized trial of transatrial versus transventricular approach, and 0 (3) did not specify their approach.

Most surgeons, 18 (10), stated that their initial approach was influenced by echocardiographic appearances, 2 (4) stated that it was not. None indicated that they used intraoperative echocardiography. Of those who altered their approach, 4 (5) specified that they opened the pulmonary trunk for doubly committed juxta-arterial defects, and 1 (1) that he used the transventricular approach for apical muscular defects. Most of the surgeons examined the defect transatrially.

Operative technique
All reported that the operative techniques employed were similar to those used by Mr Shore (Fig 1). Differences appeared in the exact position and proportion of interrupted versus continuous sutures, the number and type of sutures through the septal leaflet of the tricuspid valve, and the position and type (Teflon or pericardium) of pledgets or buttresses. When asked to describe the way in which they avoided the conduction system, 16 (15) stated that they stay away from the posteroinferior margin (in a perimembranous VSD) by a distance, specified by 1 (4), of 0.5 to more than 5 mm, and/or by placing sutures through the base of the septal leaflet of the tricuspid valve. Two (3) surgeons mentioned using a larger patch in this region, and 1 (3) placing sutures superficially on the right side of the septum. One (0) surgeon gave no reply. The most commonly used material for patching was Dacron (C.R. Bard, Haverhill, PA) by 12 (12), then bovine pericardium by 7 (4), Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) by 7 (3) and autologous pericardium by 1 (0). Some surgeons used two materials.

The surgeons were asked if they ever detached the leaflets of the tricuspid valve, and 12 (9) answered yes, of which 10 (4) stated rarely, and 8 answered (7) no.

Multiple ventricular septal defects
When asked for recently used techniques, the most common approach was given as transatrial by 18 (15) surgeons, followed by right ventriculotomy by 11 (7), and left ventriculotomy by 7 (6). One surgeon used right ventriculotomy for a specific type of apical defect [2]. Glue was used by 4 (4), umbrella closure by 4 (6), and the anterior buttressed technique by 2 (0).

Juxta-arterial ventricular septal defects
Recently employed techniques were, in order of popularity, transpulmonary closure by 16 (13), transatrial by 10 (6), a combination by 9 (11), and transventricular by 9 (5). Indications for closure of a juxta-arterial defect were: aortic incompetence or aortic valve prolapse for 9 (9), shunt size for 8 (6), failure to thrive or congestive cardiac failure for 3 (2), the diagnosis alone for 4 (3), associated lesions for 2 (1), the same indications as other ventricular septal defects for 1 (3), raised pulmonary arterial pressure or pulmonary blood flow for 1 (1), and endocarditis for 0 (1). No answer was given by 3 (0).

Indications for banding of the pulmonary trunk
The most common indication by far was multiple ventricular septal defects ("Swiss cheese" defect) 13 (13). Other indications were complex congenital heart disease, including functionally single ventricle in 5 (6), major concurrent disease or very systemically unwell infant in 3 (1), large ventricular septal defect associated with coarctation in 1 (3), large muscular defect in 1 (0), significant residual shunt following primary repair of multiple ventricular septal defects in 1 neonate (0), and preparation for the arterial switch, 0 (1). No answer was given by 3 (0).

Ventricular septal defect associated with coarctation
The majority of surgeons, 13 (10), used a two-stage repair, 5 (3) a single-stage, and 1 (3) either. In the presence of a hypoplastic arch, an additional 1 (3) performed a single-stage repair. Of those who performed single-stage repair, 1 (3) specified a midline approach, 0 (2) a thoracotomy plus sternotomy, and 1 (1) used either. Pulmonary arterial banding was sometimes used by 5 (4) surgeons. No answer was given by 1 (0).

	Comment

Top Abstract Introduction Material and methods Results Comment Appendix References

 
The initial stimulus for the survey was to provide data for a discussion at the Cardiac Surgical Course held in 1995 at the Royal Brompton Hospital. Many respondents expanded their responses. These comments were particularly informative, and the answers considered sufficiently interesting that a follow-up survey was conducted.

Support technique
It is well established that, for periods of more than 35 minutes, the incidence of measurable neurological deficit is greater with circulatory arrest than bypass [3]. However, it is not known whether the short period of circulatory arrest required for closure of a ventricular septal defect closure is significantly more harmful than bypass. The advantages of circulatory arrest are a bloodless field and improved access in small hearts, because the cannulas can be removed. As techniques and equipment improve, indications for circulatory arrest have become more stringent, and more surgeons use exclusively bypass for closure. Our surveys reflect the increasing use of bypass in preference to circulatory arrest. Some surgeons feel that closure of a ventricular septal defect is no longer an indication for circulatory arrest. Others feel that, for short procedures, there is no significant increase in risk.

Approach
Transventricular closure was initially more popular, although both transventricular and transatrial approaches were described in the 1950s [4]. In an American survey of pediatric cardiac surgeons in 1983 [5], practice was equally split between transventricular and transatrial approaches. There is some evidence of impairment of right ventricular function [6], and of an increased incidence of bundle branch block [7], following ventriculotomy. But there are no long-term studies comparing transatrial with transventricular approach, and there is no evidence of clinically relevant sequels in the long-term after simple closure via the right ventricle. Experience with other approaches has increased, and most surgeons (as evidenced by our survey) now use the transatrial (and transpulmonary) approach either exclusively or preferentially. It is easier to avoid the conduction tissue, which is more superficial on the left ventricular aspect of the septum, via a transatrial approach, from which the position of the bundle of His at the apex of the triangle of Koch can easily be identified [8]. Some defects, however, are difficult to reach from the atrium (particularly apical and juxta-arterial defects) [8], and excessive traction on the tricuspid valve risks causing tricuspid regurgitation.

Echocardiography has now replaced angiography as the main modality for imaging ventricular septal defects. The precise location of the defect, its exact relationship to valvar components, and hence its muscular or perimembranous nature, can be visualized in exquisite detail in the vast majority of cases. Cardiac catheterization is now reserved for measurement of pulmonary vascular resistance when this is indicated. Echocardiography allows more precise planning of the operative procedure. The majority of surgeons said the echocardiographic appearances influenced their approach, although some said they would still open the atrium first. This allows assessment, visualization of the site of the conduction tissue, and precise placement of other incisions [8]. None of the surgeons in the United Kingdom, at the time of the survey, reported or commented on the use of intraoperative echocardiography.

Operative technique
Operative techniques were remarkably uniform. Within the techniques of suturing there were only minor differences. All surgeons were aware of, and avoided, the position of the conduction tissue [9], although the distance from the edge of the defect which was considered to be at risk varied from half a millimeter to more than half a centimeter. The incidence of complete heart block following ventricular septal defect closure in modern series is very low, reflecting the improved understanding of the precise location of the conduction tissue and the care taken by surgeons to avoid injury to it.

The material used for the patch varied widely, suggesting either lack of a "perfect" material, or that differences between those available are small. Pericardium can calcify and can become aneurysmal [10]. Prosthetic materials carry a greater risk of endocarditis and Gore-Tex (W.L. Gore) takes longer to endothelialize. Since only a small patch is required, and it endothelializes rapidly, any differences are probably not of any practical significance.

Take-down (and re-attachment) of the septal leaflet of the tricuspid valve was described in 1957 [4] as a method of improving exposure when approaching a ventricular septal defect from the right atrium. In some cases, this is used as an alternative to right ventriculotomy [11]. Mid-to-long term follow-up has shown healing of the incision, and normal valvar function. However, the technique puts at risk a structure not otherwise involved in the procedure. In our survey the majority of surgeons sometimes detached the tricuspid valve.

Multiple ventricular septal defects
Multiple holes in the septum pose a challenge in management as evidenced by the variety of techniques recently used by those surveyed. Most surgeons had used the right atrial approach for at least some cases. The apical part of the septum is difficult to visualize from the atrium. In addition, it can be difficult to locate defects from the right ventricle because, although from the left ventricular aspect there may be two or three clearly identifiable defects, these often have multiple openings beneath and between the coarse trabeculations overlying the right ventricular aspect of the septum. The left ventricular approach is a "last resort," but successful closure is more likely as the limits of the defects can more clearly be seen. Although the definition and closure is easier from the left ventricle, this approach is not commonly used because of the long-term consequences of left ventriculotomy on left ventricular function [12]. Glue has been used successfully to close associated small ventricular septal defects after a large defect has been closed with a patch [13]. Intraoperative closure with umbrellas has also been reported for the closure of some of the defects in patients with multiple ventricular septal defects, but with a high operative mortality [14]. A significant proportion of the surgeons had used either right or left ventriculotomy, and several had used glue and/or intraoperative insertion of umbrellas. The anterior buttressed technique [15] had been used by some surgeons in the recent survey, but by none in 1995. Banding was still a common option for management (see below).

Juxta-arterial ventricular septal defect
A subgroup of ventricular septal defects which lie immediately below the arterial valves are at risk of aortic valvar prolapse and incompetence and, in the longer term, aneurysm of the sinus of Valsalva [16]. Unfortunately, the terminology used to describe such holes is not uniform, although the anatomy and pathophysiology are well recognized [9]. Aortic incompetence secondary to prolapse of the valve into the defect is a well-recognized complication. Once established, the aortic regurgitation is difficult to treat [17], and many patients require aortic valvar replacement. In addition, these defects rarely close spontaneously except by prolapse of an aortic valvar leaflet into the defect. The natural history of this lesion was studied retrospectively by Momma and colleagues [16] in 395 patients with juxta-arterial defects documented by surgery or angiography. They found that all patients developed aortic incompetence or aneurysm of a sinus of Valsalva, or else had a large shunt. As patients not undergoing cardiac catheterization or surgery were excluded from the analysis, the overall incidence of sequels for juxta-arterial defects is unknown. This is reflected in the variation in indications for closure in our survey, with 4 (3) surgeons considering the diagnosis a reason to operate in itself. Aortic valvar prolapse or incompetence was the most common indication, followed by the size of the shunt and other criteria common to all ventricular septal defects.

Because this defect lies immediately below the conjoined hinges of the pulmonary and aortic valvar leaflets, it can be difficult to reach from the right atrium. The transaortic approach may be used if the aortic valve is being repaired at the same time, but then the sutures are placed on the left ventricular side, where the conduction tissue is more superficial and is thus more at risk [18]. All approaches were used in our survey, the most common being transpulmonary closure or a combination of transatrial and transpulmonary routes. The transventricular route was used more frequently for this defect in the recent survey than in 1995.

Indications for banding of the pulmonary trunk
Banding was developed as a treatment for large ventricular septal defects in infants at a time when the operative risks of banding and subsequent debanding were less than that of primary repair in infancy [19]. Banding is a technically simple procedure, performed without bypass; banding reduces pulmonary blood flow, thus preventing pulmonary vascular disease while the defect gets smaller or spontaneously closes and the child grows. Debanding requires cardiopulmonary bypass (to excise the band and the narrowed segment of the pulmonary trunk). Both banding and debanding do carry a significant operative risk (Kirklin and Barratt-Boyes [20] quote 16% and 10% respectively). This risk has not changed significantly over time, whereas the risk of primary closure in neonates and infants is now much lower than in previous eras (less than 1% in some series) [21]. Thus, primary closure has become the procedure of choice. The indications for banding have changed as surgery in small babies has become safer, and most "simple" defects are now closed as a primary procedure. Banding still has a place in the management of multiple or "Swiss cheese" defects, allowing spontaneous closure of some of the defects, and thus simplifying closure of the remainder. This indication was given by most of the surgeons in our survey, the next most common indication being complex congenital heart disease, particularly in patients with a functionally single ventricle as part of staging to a modified Fontan procedure. Perhaps surprisingly, 1 (0) surgeon still listed a large muscular defect as an indication for banding, and 1 (3) considered ventricular septal defect associated with coarctation an indication. Three (1) surgeons stated that they would consider banding in a child who was so systemically unwell that repair was felt to be too risky. Thus, the indications for banding are changing, but it remains an important procedure in the armamentarium of the pediatric cardiac surgeon.

Ventricular septal defect associated with coarctation
When a ventricular septal defect is associated with coarctation of the aorta, there are several options for surgical management. Complete repair may be effected in a single stage, either repairing both lesions via a median sternotomy, or repairing the coarctation through a left thoracotomy and then turning the patient and performing a medium sternotomy for repair of the septal defect. Alternatively, a multistage approach may be used, repairing the coarctation via a left thoracotomy (with or without banding), and closing the septal defect weeks or months later, or days later if the infant cannot be weaned from the ventilator.

As with banding of the pulmonary trunk, the issue is whether one operation is less risky than two. Given equal overall risks, one operation is socially (and economically) more acceptable. There are favorable reports of single-stage repair [22], but opinion is split. Brouwer and associates [23] reported retrospective data on 80 infants undergoing surgery at less than 3 months of age. Repair of the coarctation was undertaken as an initial procedure in 64 (with banding in 10), and further treatment initiated as necessary. This comprised the "multistage" group. A "single" stage repair of both lesions via a median sternotomy was performed in the other 16. In the "multistage" group, 9% had recoarctation at 5 years but 60% required further treatment for the septal defect (banding, debanding and/or closure). One-quarter of the "multistage" group received only one operation. In the "single" stage group, 40% had recoarctation at 5 years but none required further surgical treatment for the septal defect. Reoperation was performed in 3 of the 13 patients with recoarctation, while the other 10 were treated with angioplasty. There was no statistical difference between the two groups in mortality or average number of operations per patient.

Thus, in the published literature, the various approaches have different risks and benefits, with no strategy being superior overall. In our survey, all strategies were represented. More surgeons favored two-stage repair. Several surgeons would sometimes band the pulmonary trunk in this situation. Those who preferred the single stage approach, and who specified an approach, were equally split between only a midline incision as opposed to thoracotomy followed by sternotomy.

The results of surgical treatment of ventricular septal defect have improved dramatically since the 1950s. The challenges remaining include reducing the mortality and requirements for reoperation in those with multiple defects and defects associated with coarctation, and by timely intervention avoiding valvar replacement in the setting of juxta-arterial defects. This survey shows that pediatric cardiac surgeons in the United Kingdom demonstrate a uniform, evidence-based approach to management, with heterogeneity of approach only where there is lack of evidence of superiority of one technique over another. It also demonstrates that surgeons in the United Kingdom are quick to change their practice in the light of new information, as exemplified by the increase, over a 2-year period, in the use of bypass over circulatory arrest, and of the transatrial over the transventricular approach.

	Acknowledgments

 
We are indebted to all the cardiac surgeons in the United Kingdom who participated in our survey, and who granted us permission to analyze their response, specifically: David R. Anderson, FRCS, William J. Brawn, FRCS, Marc R. de Leval, MD, Martin J. Elliott, FRCS, Roger E. Franks, FRCS, J. R. Leslie Hamilton, FRCS, Marcus P. Haw, FRCS, Morgan P. G. Jamieson, FRCS, Robert K. Lamb, FRCS, Christopher Lincoln, FRCS, Pankaj S. Mankad, FRCS, James L. Monro, FRCS, Francesco Musumeci, MD, Ash Pawade, FRCS, Ravi Pillai, FRCS, James C. S. Pollock, FRCS, Marco Pozzi, MD, Babulal Sethia, FRCS, Victor T. Tsang, FRCS, Kevin Watterson, FRCS, and Sir Magdi H. Yacoub, FRCS. The study could not have been accomplished without their enthusiastic and gracious support. Dr Manisha Lal was a Commonwealth Scholar during her stay in the United Kingdom. Professor Anderson is supported by the British Heart Foundation, together with the Joseph Levy Foundation.

	Appendix

Top Abstract Introduction Material and methods Results Comment Appendix References

 
Ventrical septal defect questionnaire

1. For VSD closure do you use bypass or circulatory arrest?
   
2. Do you always attempt to close the VSD through the atrium or ventricle?
   
3. Is your initial approach influenced by the position of the VSD on echocardiography?
   
4. How would you describe the way that you avoid the conduction tissue in a perimembranous VSD?
   
5. What patch material do you use?
   
6. Do you ever detach the tricuspid valve?
   
7. For multiple muscular VSDs what techniques have you recently employed? a) closure through the left ventricle b) closure through the right ventricle c) closure through the right atrium d) the anterior buttressed technique e) biological glue f) intraoperative umbrella insertion
   
8. For juxta-arterial VSDs what techniques have you recently employed? a) transatrial closure b) transpulmonary closure c) transventricular closure d) a combination
   
9. What are your indications for closure of a juxta-arterial VSD?
   
10. What are your indications for banding of the pulmonary trunk?
    
11. How do you manage VSD and coarctation?
    
12. The enclosed illustrations (Fig 1) are the techniques currently used by Mr Shore for closure of: a) a perimembranous outlet VSD via the transventricular route b) closure of the perimembranous VSD via the transatrial route c) closure of the muscular inlet VSD d) closure of the perimembranous outlet VSD when associated with a muscular inlet VSD
    

	References

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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): Anna F. Merrick Robert H. Anderson Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Merrick, A. F. Articles by Shore, D. F. Search for Related Content PubMed PubMed Citation Articles by Merrick, A. F. Articles by Shore, D. F.