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Ann Thorac Surg 2010;89:544-551. doi:10.1016/j.athoracsur.2009.10.057
© 2010 The Society of Thoracic Surgeons

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Original Articles: Pediatric Cardiac

Current Expectations for Surgical Repair of Isolated Ventricular Septal Defects

Brandi Braud Scully, BAa,b, David L.S. Morales, MDa,b, Farhan Zafar, MDa,b, E. Dean McKenzie, MDa,b, Charles D. Fraser, Jr, MDa,b,*, Jeffrey S. Heinle, MDa,b

a Michael E. DeBakey Department of Surgery, Division of Congenital Heart Surgery, Baylor College of Medicine, Houston, Texas
b Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Texas

Accepted for publication October 23, 2009.

* Address correspondence to Dr Fraser, Congenital Heart Surgery Service, Baylor College of Medicine, 6221 Fannin St, Mail Code: WT 19345-H, Houston, TX 77030-2399 (Email: charlesf{at}bcm.tmc.edu).

Presented at the Fifty-fifth Annual Meeting of the Southern Thoracic Surgery Association, Austin, TX, Nov 5–8, 2008.


    Abstract
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Background: Ventricular septal defect (VSD) is the most commonly recognized congenital heart defect. With the development of device closure for intracardiac defects, we sought to evaluate current expectations for surgical closure of isolated VSD.

Methods: Between January 1, 2000, and December 31, 2006, 215 patients underwent isolated VSD repair at a median age of 10 months (range, 20 days to 18 years) and a median weight of 7 kg (range, 2 to 66 kg). The following VSD types were found: 172 perimembranous (80%), 28 supracristal (13%), 6 inlet (3%), and 9 muscular (4%). One hundred eight patients (50%) had evidence of congestive heart failure or failure to thrive preoperatively. Thirty-one patients (14%) had aortic valve cusp prolapse, and 63 (29%) had genetic abnormalities.

Results: Incidence of significant postoperative complications was extremely low. No patient underwent reoperation for a residual VSD. None had complete heart block. One operative mortality (0.5%) and 2 late deaths (0.9%) occurred. Median postoperative hospital length of stay was 5 days (range, 2 to 187 days). In the immediate postoperative period, 6 patients (2.8%) required reoperation. No patients were discharged on antiarrhythmic agents, had complete heart block, or required permanent pacing. At mean follow-up of 2.1 ± 2.0 years, 99.5% (211 of 212) of patients were asymptomatic from a cardiac standpoint. None exhibited greater than mild new-onset tricuspid valve regurgitation. No aortic valve injuries occurred.

Conclusions: Surgical closure of isolated VSD is a safe, effective therapy. Risk of death, complete heart block, and reoperation is minimal. As new technologies for VSD closure evolve, results such as these should be considered when evaluating patients, choosing therapeutic options, and counseling families.


    Introduction
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Ventricular septal defect (VSD) is the most commonly recognized congenital heart defect [1], occurring in approximately 2 of 1,000 live births [2], and one of the most common defects requiring surgical intervention. Surgical closure of VSD was first performed in 1955 by Lillehei and associates [3], and since then improvements in surgical repair, including improved techniques of cardiopulmonary bypass, myocardial preservation, anesthesia, and postoperative care, have greatly reduced operative mortality [4]. Complications are rare, but can include residual VSD, heart block, emergent reoperation, neurologic injury, and death [1, 4].

Recent reports have indicated a very low incidence of postoperative complications, with several studies from the past decade reporting no incidence of complete heart block [5–7]. Owing in part to such consistently excellent results, there have been few recent reports describing outcomes for surgical management of an isolated VSD [8]. Although acknowledging the safety and effectiveness of surgical closure, proponents for device closure of VSD frequently associate surgical closure of VSD with morbidity, mortality, postoperative patient discomfort, and sternotomy and its subsequent scar [9]. With the advent of device closure of VSD, the most current results of surgical repair warrant further consideration. This study aimed to evaluate the current expectations for surgical closure of an isolated VSD.


    Patients and Methods
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Patient Population
The Texas Children's Hospital Congenital Heart Surgery Database was queried for patients 18 years of age or younger undergoing VSD repair between January 1, 2000, and December 31, 2006. The medical records of these patients were retrospectively reviewed after approval by the Baylor College of Medicine Institutional Review Board on February 2, 2007. Given the retrospective nature of the study, permission was given for waiver of consent.

Patients undergoing isolated VSD repair were extracted from this group. We excluded all patients with concomitant procedures except those who underwent repair of atrial septal defect, patent foramen ovale closure or patent ductus arteriosus ligation. Patients with multiple VSDs or who had undergone previous pulmonary artery banding also were not included in this study. Two hundred fifteen patients were identified as satisfying these criteria. This represented 6.4% of the cardiopulmonary bypass cases performed during this period. Follow-up was at the discretion of the primary cardiologist. Mean follow-up was 2.1 ± 2.0 years.

Surgical Technique
All patients underwent median sternotomy. Cardiopulmonary bypass with moderate hypothermia was used in all patients except 1 patient who underwent deep hypothermic circulatory arrest for repair of his defect. Intermittent cold crystalloid cardioplegia was used for myocardial preservation. Four surgeons operated during this period. The use of interrupted or continuous sutures and use of primary versus patch material were dictated by surgeon preference. Transesophageal echocardiography was routinely used except in patients with a contraindication, such as previous fundoplication or patient size.

Statistical Analysis
Statistical analyses were performed as follows: means, medians, standard deviations, and ranges were used to describe continuous variables. Frequencies and percentages were used for categorical data. Fisher's exact text and the {chi}2 test were used to analyze binary variables. Student's t test was used to analyze the continuous variables. Statistical significance was a probability value of less than 0.05. All analyses were conducted with SPSS 15.0 (SPSS, Chicago, IL).

Definitions
Operative mortality was defined per the guidelines in The Society of Thoracic Surgeons Congenital Database Taskforce and the joint European Association for Cardiothoracic Surgery–Society of Thoracic Surgeons congenital database committee report [10]. Characterizations of defect size, degree of valvular insufficiency, and degree of residual VSD, demonstrated on postoperative echocardiograms, were based on the judgment of the interpreting staff cardiologists. In general, a VSD was considered large if it was measured to be larger than the aortic annulus, and was considered small if it measured less than one half the size of the aortic annulus. Degree of tricuspid insufficiency was a qualitative assessment based on the breadth of the jet across the valve and depth of the regurgitation into the right atrium. The degree of aortic insufficiency was determined by a combination of qualitative assessment, vena contracta, and pressure half-time. A clinical diagnosis of failure to thrive was made by the referring cardiologist, and in general was made for children whose weight fell below the fifth percentile for their age or who weighed less than 80% of their ideal weight based on the National Center for Health Statistics' standard growth charts, or if the patient crossed multiple percentiles on his or her growth curve. Estimates of right ventricular and pulmonary artery pressures were made using the tricuspid regurgitation jet velocity at the time of echocardiography or on direct measurement at the time of cardiac catheterization. Residual VSDs were defined as small if they measured less than 2 to 3 mm and were pressure restrictive. Outpatients were defined as patients admitted to the hospital either the morning of or the night before surgery.


    Results
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Patient Demographics
The median age at repair was 10 months (range, 20 days to 18 years) and weight was 7 kg (range, 2 to 66 kg). For the subgroup of patients with genetic abnormalities, the median age was 6.1 months (range, 37 days to 7.5 years) and the median weight was 5.4 kg (range, 2.4 to 25.7 kg), compared with nonsyndromic patients whose median age was 14.5 months (range, 20 days to 17.8 years) and median weight was 9.1 kg (range, 3.0 to 65.8 kg). The probability values for both age and weight of syndromic versus nonsyndromic patients were less than 0.001. Sixty-three patients (29%) had genetic or syndromic abnormalities (47 [22%] with trisomy 21). One hundred seventy-one patients (80%) were outpatients before their surgery. One hundred seventy-two patients (80%) had a perimembranous VSD, with the remainder of the cohort distributed as follows: 28 supracristal (13%), 6 inlet (3%), and 9 muscular (4%) defects. One hundred thirty-eight patients (64%) underwent concomitant repair of an atrial septal defect or patent foramen ovale closure, and 76 patients (36%) had a patent ductus arteriosus ligation.

Indications for Surgery
Indications for operative intervention were taken from the referring cardiologist's note or the surgeon's preoperative note and are summarized in Table 1.


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Table 1 Indications for Surgery Based on Referring Cardiologist's Note and Surgeon's Preoperative Note
 
Echocardiographic findings on preoperative studies are summarized in Table 2. Of the patients with left atrial enlargement, 53 were characterized as mild, 45 as moderate, and 3 as severe. Of the patients with left ventricular enlargement, 45 were characterized as mild, 33 as moderate, and 4 as severe. Of patients with aortic insufficiency, 2 had moderate and none had severe insufficiency.


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Table 2 Findings on Preoperative Echocardiography
 
Twelve patients underwent preoperative cardiac catheterization. Ten patients had a pulmonary to systemic blood flow ratio greater than 1.5:1. Eleven patients had increased right ventricular or pulmonary arterial pressures, and 1 patient had increased pulmonary arterial resistance. No patients had fixed pulmonary hypertension. No patient was suspected of having advanced pulmonary vascular disease or irreversible pulmonary vascular changes.

By chest radiograph, 174 patients (81%) were found to have cardiomegaly; 156 patients (73%) had evidence of increased pulmonary blood flow.

Surgical Approach
Of the perimembranous VSDs, 170 (99%) were closed from a transatrial approach, 1 from a transventricular approach, and 1 from a transaortic approach. Of the supracristal VSDs, 23 of 28 patients (82%) were closed from a transpulmonary approach, 4 from a transatrial approach, and 1 from a combined transatrial and transpulmonary approach. All inlet and muscular VSDs, including apical VSDs, were closed transatrially through the tricuspid valve. In 52 patients (24%), the tricuspid valve was incised to aid in exposure of the defect. One hundred ninety-three patients underwent patch repair of their VSD. Twenty-two patients (10%) had VSDs closed primarily using interrupted sutures. Ventricular septal defect patch material used included autologous pericardium (n = 120, 56%) Dacron (n = 43, 20%), and bovine pericardium (n = 30, 14%). Two hundred six patients (96%) had intraoperative transesophageal echocardiography.

Operative Data
Median operative time was 224 minutes (range, 100 to 456 minutes). Median cardiopulmonary bypass time was 104 minutes (range, 35 to 301 minutes). Median cross-clamp time was 67 minutes (range, 5 to 207 minutes). Moderate hypothermia was used during cardiopulmonary bypass (mean temperature, 28° ± 1.7°C). One patient with dextrocardia required 44 minutes of deep hypothermic circulatory arrest owing to the difficulty of visualizing the defect. One hundred sixty-four patients (76%) received blood products, either packed red blood cells, platelets, or fresh-frozen plasma, intraoperatively. The mean weight for patients receiving blood products (7 kg) was significantly lower than that for those patients who did not receive blood products (26 kg; p < 0.001).

Hospital Course
Median intensive care unit length of stay was 2 days (range, 1 to 14 days). Median postoperative hospital length of stay was 5.0 days (range, 2 to 187 days). For outpatients, median hospital length of stay was 4.0 days (range, 2 to 13 days). Six reoperations (2.5%) were performed for seven events. Two patients (1%) had postoperative bleeding requiring reoperation. One patient required mediastinal exploration and evacuation of a pericardial effusion and left femoral artery embolectomy on the same day as his intracardiac repair. One patient returned to the operating room 2 days postoperatively as a result of a superficial wound hematoma, and 1 patient required exploration and drainage of a superficial wound infection 20 days postoperatively. One patient underwent repair of an incisional hernia 13 days postoperatively. There were no mediastinal or deep sternal wound infections. There were no neurologic complications or seizures by clinical examination.

Mortality
One operative mortality (0.5%) and 2 late deaths (0.9%) occurred. The operative mortality occurred in a former 32-week premature infant with a partial trisomy 2p mosaicism who had been hospitalized since birth. He underwent surgical closure of a perimembranous VSD at 6 months of age. He died on postoperative day 49 of respiratory failure with acute decompensation. One 5-month-old patient with VACTERL syndrome (sacral agenesis, anal atresia, rib and vertebral anomalies, and malrotation) underwent VSD closure for a perimembranous VSD. He was discharged on postoperative day 27 and returned with a bowel perforation 32 days postoperatively, which caused his death. One patient with Rubinstein-Taybi syndrome died more than 2 years postoperatively, of a viral syndrome and abdominal catastrophe. There were no deaths in the outpatient group.

Follow-Up
No patients underwent reoperation for a residual VSD. At last echocardiogram follow-up (mean, 1.1 ± 1.6 years), 180 patients (84%) had no residual VSD. Of the remaining 35 patients, the residual VSDs were characterized as small by the echocardiographer.

At mean electrocardiogram follow-up of 1.2 ± 1.7 years, 99% (207 of 209) of patients were in a normal sinus rhythm, and 1% (2 of 209) were in an atrial rhythm. Fifty-five patients (26%) had a right bundle-branch block. No patients had a left bundle-branch block. No patients exhibited complete heart block or required permanent pacing. No patient left the operating room with complete heart block or in a junctional arrhythmia. No patient was discharged on antiarrhythmic agents.

Ninety-seven percent of patients (n = 209) had normal left ventricular function at a mean echocardiogram follow-up of 1.1 ± 1.6 years. Five patients (2%) had mildly depressed postoperative left ventricular function, and 1 patient (0.5%) had moderately depressed function on echocardiogram 4 days postoperatively (no late follow-up echocardiogram was available for this patient). No patient had severely depressed left ventricular function.

No patient had new onset aortic insufficiency greater than trivial and no patient with preoperative aortic insufficiency had greater than mild aortic insufficiency postoperatively. No patient exhibited greater than mild new-onset tricuspid valve regurgitation. Two patients with preoperatively mild tricuspid valve regurgitation had moderate tricuspid valve regurgitation at last follow-up. No statistical significance was found between surgical techniques for the development of tricuspid valve regurgitation, including primary closure versus patch closure, running versus interrupted sutures, or incision versus nonincision of the tricuspid valve.


    Comment
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Results for surgical repair of ventricular septal defects have evolved significantly since the first series of direct vision closure reported by Lillehei and colleagues in 1955 [3]. Advances in surgical technique and intraoperative and perioperative management have led to marked improvement in outcomes for these patients. Long-term survival and clinical outcomes for these patients are excellent, with one recent study citing 92% of patients in New York Heart Association class I more than 20 years after repair [11]. For infants who undergo surgical closure of VSD, long-term results are similarly positive, with Kuribayashi and associates [7] reporting all survivors growing normally with a good quality of life after more than 10 years. Other recent studies report similar outcomes, with the risk of mortality, major morbidity, emergent reoperation, and significant residual VSD approximately 1% [4].

Historically, complete heart block has been a serious complication of VSD closure, and one that is associated with an increased risk of late death [12, 13]. The conduction system is particularly at risk during closure of perimembranous VSDs because of the close anatomic relationship of the atrioventricular node and the bundle of His to the inferoposterior margin of the VSD [14–18]. Increasing knowledge of the anatomy of the conduction system has greatly decreased the incidence of intraoperative injury [19–21], and surgeons can now avoid the incidence of complete heart block in the vast majority of patients. In fact there have been numerous studies from the past decade that report zero incidence of complete heart block after VSD closure [5–7]. Anderson and associates [20] recently reviewed the results of a large series of patients undergoing VSD repair at the Great Ormond Street Hospital for Children during a 26-year period. Based on the results of their study, they suggest that in light of existing knowledge of the atrioventricular conduction axis, the risk of iatrogenic complete heart block with VSD closure should be less than 1% and the expected mortality rates for these patients should be approaching 0%.

The results of our series of patients undergoing isolated VSD repair in this decade support the findings of Anderson and coworkers [20]; there was no incidence of heart block and an operative mortality of less than 1%. Furthermore, overall morbidity was minimal, with no patient requiring reoperation for residual VSD, no observed valve-related complications despite the intimate location of the tricuspid and aortic valves to the margins of a perimembranous defect, and preservation of left ventricular function postoperatively. This should be the expectation of families and clinicians for patients undergoing VSD repair in the current era.

Right bundle-branch block is a recognized finding after VSD closure, and this was also seen in our study, with 55 patients (26%) in our cohort having right bundle-branch block. The significance of long-standing right bundle-branch block has been debated, and a recent report by Pederson and colleagues [22] studied the long-term significance of right bundle-branch block on left ventricular function after surgical closure of VSD. They noted that right bundle-branch block continues to be a common finding after surgical repair, which in long-term follow-up does not appear to affect systolic ventricular function but may be associated with diastolic dysfunction. Right bundle-branch block after surgical closure of VSD remains a common finding that warrants long-term evaluation.

This study was not specifically designed to study the indications for operative intervention but rather to review the results of those patients with isolated defects undergoing surgical repair. The results of this study reinforce the fact that surgical closure of an isolated VSD is a safe and effective therapy, with current expected mortality, incidence of heart block, or need for reoperation for residual VSD less than 1%. With this low operative risk taken into account, indications for, and timing of, surgery may need to be reexamined. Based on our current clinical experience and expectations we consider surgical intervention for patients with isolated VSDs in the following circumstances.

If the defect is nonrestrictive we consider closure in early infancy, particularly if the patient is symptomatic or if there is no evidence of spontaneous closure. For restrictive defects, if the patient remains symptomatic despite medical therapy or if there is evidence of a significant left to right shunt by either clinical examination, chest radiograph (eg, cardiomegaly, increased pulmonary vascular markings, interstitial edema), or echocardiogram (eg, estimated pulmonary to systemic blood flow ratio of more than 1.5:1, left atrial or left ventricular enlargement), these patients would be considered for surgical intervention. Similarly, if at cardiac catheterization the pulmonary to systemic blood flow ratio is greater than 1.5:1 or pulmonary arterial pressures are elevated, we would consider intervention. If there is evidence of involvement of the aortic valve (ie, prolapse of the aortic valve leaflet into the defect with or without aortic insufficiency), we believe that surgical intervention is warranted to avoid the potential for progressive aortic valve injury. If aortic insufficiency is present in association with a VSD, we also would recommend closure of the defect. By similar reasoning, we would recommend closure for patients with a supracristal type VSD because of the involvement of the aortic valve with the defect.

Asymptomatic patients with small, restrictive VSDs in the absence of indications for closure such as aortic insufficiency or endocarditis represent a controversial group for surgical closure [6] and continue to be discussed at our institution. The results of this study would support the assertion that these defects could be closed with low operative risk.

Of interest in this study, the patients with genetic syndromes in our cohort were repaired at an earlier age than their nonsyndromic counterparts (median age of 6.1 months versus 10 months in nonsyndromic patients; p < 0.001). This likely reflects the symptomatic nature of the patients and the difficulty in managing their multiple medical and surgical issues. In general we do consider these patients for earlier surgical closure.

With the advent of catheter-based therapies for device closure of intracardiac defects, there has been interest in avoiding the potential complications associated with surgical repair of these defects. Given the potential risks of surgical repair and the need for cardiopulmonary bypass, interventional transcatheter techniques have been developed for device closure of VSDs [23, 24]. Percutaneous VSD closure may be an appealing alternative to surgery for many patients and families because the hospital length of stay is shorter, there is less pain and discomfort, there is no midline scar, and it is associated with high procedural success and closure rates with reportedly low mortality and morbidity [25–30]. These studies have reported similar rates of VSD closure and conduction abnormalities when compared with historic surgical control series [25].

With the recent US Food and Drug Administration approval of the muscular Amplatzer VSD occluder (AGA Medical, Plymouth, MN), percutaneous closure of muscular VSDs has become a viable option in those patients who pose a difficult surgical repair [23, 24], particularly those patients with apical muscular and multiple muscular defects that are challenging for the surgeon to achieve complete closure. Success with the muscular device has led to the development of an asymmetric device designed for closure of perimembranous defects [31, 32]. Although not yet approved for use in the United States, several international studies and a Phase I US trial have explored the potential for device closure of these more common perimembranous defects [28]. Of concern with device closure of perimembranous defects is the potential for the development of conduction abnormalities and valve injury given the intimate relationship of the defect to the conduction system and the aortic and atrioventricular valves. Complete heart block has been reported in 2.9% to 5.7% of the patients in these studies, and the development of heart block can occur at 1 year or more after device placement [9, 25, 26, 28, 33].

In a prospective, nonrandomized study comparing device closure with surgical repair of perimembranous defects, Xunmin and colleagues [30] reported equivalent results between the two strategies. Patients in this study, however, were older than 2 years of age, and the median age at device closure was 7.5 years. A major limitation of device closure remains the young age and low weight of children who require early VSD closure owing to heart failure, failure to thrive, and pulmonary hypertension. Given the current technology, transcatheter closure of intracardiac defects is limited to those patients weighing more than 8 to 10 kg. Based on the current study's median patient weight at time of operation of 7 kg, the majority of patients fall below this weight limit and would not be eligible for transcatheter closure.

Proponents of device closure of VSDs, while acknowledging the safety and effectiveness of surgical closure, continue to point to the "significant morbidity and mortality" [34] associated with surgical repair of VSD. Largely, these comments are supported by historic or outdated surgical data and do not reflect the current results with surgical repair of isolated VSDs. The current surgical data [6, 30, 35, 36], including this study, support the expectation that heart block should be less than 1% and operative mortality should approach zero percent. As the technology for transcatheter closure of VSD continues to evolve, results such as these must be the benchmark by which results are compared.

Limitations
This study is limited by its retrospective nature and the inherent limitations of all retrospective studies. Patients were not randomly assigned to operative versus nonoperative management. Only those patients who were referred for surgery and underwent surgical repair were reviewed. Therefore, no direct conclusions can be made regarding operative versus nonoperative management in this cohort. Indications for surgery are based on the retrospective review of the referring cardiologist's clinical notes and the surgical preoperative note.

Although there were no adverse neurologic events recognized in this patient cohort, there was no systematic assessment done of preoperative and postoperative neurodevelopmental status or sequelae. Neurologic outcomes continue to be an important consideration in patients undergoing both surgical and nonsurgical interventions.

In addition, follow-up of these patients is short. Thirty-five patients (16%) were not seen after their first postoperative clinic visit. Ventricular septal defect patients are often referred from cardiologists outside of Texas Children's Hospital and thus are difficult to monitor. Although this study did not address long-term follow-up for these patients, long-term survival and clinical outcomes for patients after surgical closure of isolated VSD are consistently excellent [7, 11], and we would anticipate the same for this study population.

In addition, we intentionally excluded patients with multiple VSDs. We recognize that patients with multiple VSDs can be a challenging group for surgical repair and may be an important subset of VSD patients to be put forward for consideration for device closure. However, the focus of this study was patients with isolated, single VSDs.

Conclusions
In the current era, results for surgical repair of isolated VSD are excellent. Families and clinicians should expect an extremely low incidence of adverse events, including reoperation for residual VSD, extended hospital stay, arrhythmias, valve injury, depressed ventricular function, or heart block. This information should be reassuring to clinicians and to the families of patients with VSDs undergoing surgical repair. Although recognizing the value of transcatheter device closure for surgically challenging VSDs, concerns remain for the use of the current devices for the management of defects that are readily accessible to the surgeon. As new technologies continue to evolve, current surgical results such as these should be considered when evaluating patients, choosing therapeutic options, and counseling families.


    Discussion
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
DR ANDREW C. FIORE (St. Louis, MO): Jeff, I congratulate you and your associates at Texas Children's Hospital for once again presenting truly outstanding results in a large series, 215 patients, having surgical closure of isolated VSD (ventricular septal defect). This report along with others in the literature will serve as a contemporary benchmark that our interventional cardiology colleagues will have to reach to as their technology improves with respect to VSD closure.

I have three questions for you. Two are technical and one is a management problem. I would like you to answer the technical questions, if you would, in the 2- to 4-kg neonate, which is a particularly challenging subgroup. How do you manage important large chordae tendineae from the tricuspid valve that cross the VSD and make it difficult to place a patch over the full circumference of the defect? Was your management strategy for this problem related to the postoperative tricuspid regurgitation?

I am aware that the residual shunting postoperatively was small, but as you look back on the techniques you used to close the defect, could you correlate the degree of postoperative residual shunt with the technique used to close the VSD? Was there any advantage of continuous suture or interrupted pledgeted mattress sutures?

As I read your manuscript and thank you for providing it, one of the mortalities was in a baby with Down's syndrome. As I looked at the numbers, nearly 50 patients had trisomy 21 with an isolated VSD. This is a challenging subgroup. The one mortality noted was operated on at 6 months of age. Looking back on your data, how would you manage that subgroup now? What age would you recommend operating on a newborn baby with trisomy 21, a clinically large VSD, and heart failure?

Thank you.

DR HEINLE: Thank you for those comments and also for reviewing our manuscript. Those thoughts are very helpful.

Your first question was about technically how we deal with large prominent chordae crossing the VSD and does this relate to TR (tricuspid regurgitation), and again, I think this is for perimembranous and the inlet defects, particularly. For the majority of the patients we were able to mobilize those enough so that we can see the rims of the defect, but if we can't, we are willing to incise the tricuspid valve, septal and/or anterior leaflets, either with an incision paralleling the annulus or a radial incision to expose the margins of the defect, and I think that is a helpful technique. Recently Dr Gaynor and his colleagues at CHOP (Children's Hospital of Philadelphia) have published their results with exposure through a leaflet incision and have shown that you do get good exposure and you probably don't injure the tricuspid valve doing that if you take care. So I think that is a useful technique where exposure is difficult.

Your second question was about residual VSDs and was there anything in our technique that we recognized as a risk for a residual defect. Again, this is a retrospective study. The 4 surgeons used what they felt was the appropriate approach. I don't have the data of interrupted versus continuous suture material, but looking back through the operative notes, it didn't strike me that there was one that was more of a causative factor. And again, we didn't really look at it, but residual VSD, whether you took the leaflet down or not, I don't think matters. Again, in the Gaynor paper they might argue with that, but we didn't see that in ours. The only thing that really came out with residual VSD, it wasn't statistically significant but getting close, was the size of the patient; the smaller the patient, the more likely to have a residual defect. Again, I would just like to emphasize, our echocardiographers characterized the residual defects as trivial, tiny, or small. There was only one defect that was 3 mm and all the rest were 2 mm or less, so presumably not significant.

Your third question, which also struck me the most in this study, was the frequency of genetic syndromes in children with VSDs. We did see a significant difference in the weight and age of those patients going to operation. The children with genetic syndromes came earlier to operation than did their counterparts. We did not perform multivariate analysis, but it is interesting that this comes out. Others have shown that there are differences in these kids, even with the conduction tissue. Doctor Gaynor was sharing with me yesterday the differences they have seen in their patients with genetic syndromes in terms of neurologic development. I think these kids are different.

As far as when will we operate on this group of children, I would be more likely to operate on a Down's child with a big VSD earlier, regardless of symptoms. If you have atrial enlargement, even if you have minor symptoms or potentially if you don't have symptoms but you have a significant shunt, I think when you take that away from the equation for these babies that do better and it is easier to manage the other issues related to their genetic syndromes. So we would approach these patients more aggressively.

DR ROBERT D. B. JAQUISS (Little Rock, AR): That was a great presentation, Jeff, and I think the cardiologists have got a very high bar to shoot for here, but when they talk about shooting at the bar, one of the things that they mention is the fact that we are having to put these children on cardiopulmonary bypass to do this, and there is this specter of subtle neurologic injury that ought to be stamped out if it can be, and clearly, if 30 of your patients have syndromes, it is unrealistic to look at the mean IQ in your whole group. But late testing of these kids to prove or disprove that their neurologic status is reasonable and the same as age-matched controls I think would be a valuable contribution. You certainly have numbers to power that sort of comparison.

The other one is just a simple technical question having to do with transfusion, which is another advantage since it is not nominally touted for percutaneous closure of these. Do you have data about that?

DR HEINLE: In our patients, 76% of the patients had a blood transfusion. Those who received transfusion were significantly different in weight and age. So that the median weight of those that received transfusion was 7 kg and the median weight of those that didn't was 26 kg. So I do believe it is related to size of these patients.

As far as the cardiologists and the use of cardiopulmonary bypass, in some respects it is almost a moot point since the majority of our patients are smaller in size than the lower limit acceptable for device closure. So if you have a symptomatic kid, at least at this point, device closure would not be an option. If you get a different device or if it can be placed periventricular, then I think you have more of an argument. There may be a little more discussion in the older kids where there is an option of avoiding bypass. I don't have data, but we believe there is a very low incidence of neurologic injury, and I would trade that risk for a competent aortic valve, I think. I see Bill Gaynor standing to address the neurologic comments; it will be interesting to hear what he says.

DR J. WILLIAM GAYNOR (Philadelphia, PA): I did want to address the neurodevelopmental outcomes. We have looked at 1-year and 4-year outcomes in a group of kids who had VSDs repaired in the first 6 months of life. They were usually referred for surgery because of severe failure to thrive. The strongest predictor of an adverse neurodevelopmental outcome was a genetic syndrome. The scores are within the normal range for the kids without genetic syndromes, even though these are kids who underwent surgery because of poor growth at a time of maximal brain growth. So I think in that situation, even in the very small sick kids, the developmental outcomes are good.

And I can't discuss the full results, but next week at the AHA (American Heart Association) meeting we are presenting the results of a prospective study of school-age children who had surgery with CPB (cardiopulmonary bypass) for a variety of defects, including VSDs. We included a matched group of kids having pectus repair to control for anesthesia, pain, and length of stay. There was a third group of kids who had small defects which didn't require surgery. We performed preoperative and postoperative testing on the two operative groups, and bypass was not a risk factor for worse outcomes.


    References
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 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 

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