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Ann Thorac Surg 1995;59:573-578
© 1995 The Society of Thoracic Surgeons

Associated Atrial Septal Defects Increase Perioperative Morbidity After Ventricular Septal Defect Repair in Infancy

Sections of Thoracic Surgery and Pediatric Cardiology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Although closure of ventricular septal defects (VSDs) is currently associated with a relatively low risk, infants with associated atrial septal defects (ASDs) seem to have a higher perioperative morbidity. To clarify this impression, we reviewed our entire experience (since 1977) with closure of simple VSDs in 163 infants (age, 12 months). Of these, 57 had significant ASDs (ASD-VSD subgroup). Hospital mortality was 3.7% (6/163) overall and 1.4% (2/145) since 1980. Actuarial survival at 10 years was 92% ± 5%. Significant morbidity occurred in 15.5% (16/103) of the VSD subgroup versus 48.1% (26/54) of the ASD-VSD subgroup (p 0.001). Multivariate analysis identified the presence of multiple VSDs and early date of operation as risk factors for hospital death, and younger age, an associated ASD, the size of the VSD, and use of hypothermic circulatory arrest as risk factors for significant perioperative morbidity. Compared with the VSD subgroup, the ASD-VSD subgroup had a higher hospital mortality (5.3% [3/57] versus 2.8% [3/106]), were younger (5.1 ± 2.9 versus 7.2 ± 2.9 months; p = 0.001), had a higher preoperative pulmonary artery pressure (70.2 ± 19.0 versus 62.7 ± 21.8 mm Hg; p = 0.08), needed more inotropic support (12.3% versus 3.7%; p = 0.07), needed more prolonged ventilation (3.3 versus 1.8 days; p = 0.02), and had longer postoperative hospital stays (11 versus 8 days; p = 0.005). The increased postoperative morbidity associated with infants who have a significant ASD in addition to a VSD is generally unappreciated, and may relate to the different hemodynamics associated with left-to-right shunting at both the atrial and ventricular levels.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
See also page 578.

An isolated ventricular septal defect (VSD) is the most common congenital cardiac lesion, representing 20% to 30% of all congenital heart malformations, and has an incidence of about 1 per 1,000 live births [1–3]. The hospital mortality associated with surgical closure of a simple VSD in many series is currently less than 5% [4–9], and the associated morbidity is generally low, though less well defined in the literature.

It has been our impression, however, that infants with VSDs who also have significant shunting at the atrial level, and who need surgical intervention within the first year of life, behave differently from those with isolated VSDs: They seem to suffer increased morbidity after repair, often require more inotropic support postoperatively, and take longer to recover from their surgical procedure. Because this observation is not well documented, we undertook this review to better define the relationship between associated atrial septal defects (ASDs) and the outcome from the repair of VSDs in infancy.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Patient Population
During March 1994, the hospital records and cardiothoracic surgery database were reviewed to identify all patients less than 12 months of age at the time of surgical closure of an isolated VSD at the Children's Hospital of Oklahoma. Included were patients (1) with a concomitant atrial septal defect (ASD) or patent ductus arteriosus, or both, (2) who had undergone repair of coarctation or pulmonary artery banding, or both, and (3) with other simple cardiac lesions such as mild pulmonary stenosis and vascular rings. Patients with more complex associated cardiac defects were excluded.

During the 17-year period between 1977 and March 1994, 163 infants fulfilling these criteria were identified, and they constitute the study group. One hundred forty-five patients have undergone surgical treatment since January 1980. There were 92 female and 71 male infants. The mean age at operation was 6.5 ± 3.0 months (range, 0.6 to 12 months), and the mean weight was 5.4 ± 1.6 kg (range, 2.2 to 9.8 kg) (Table 1).

The VSD was perimembranous (conoventricular) in 116 patients, an inlet septal defect (atrioventricular canal type) in 19, an outlet septal defect (conal septal defect) in 5, and a muscular (trabecular) defect in 10; it constituted multiple VSDs in 13 [10, 11]. Of the 33 patients with Down's syndrome, 23 had perimembranous defects and 10 had inlet-type defects. The VSDs were arbitrarily classified as large if they were closed with a patch and small if they were suture obliterated.

Forty patients underwent 45 surgical procedures before their VSD repair. These included aortic coarctation repair in 28, tracheoesophageal fistula repair in 2, a Ladd procedure for duodenal atresia in 2, and placement of a ventriculoperitoneal shunt for hydrocephalus in 1 (Table 2).

The median postoperative hospital stay was 7 days (range, 5 to 68 days) and the median total hospital stay was 9 days (range, 6 to 93 days). A mean follow-up of 50 ± 47 months (range, 0 to 170 months) was achieved in 147 patients (90.1%).

Outcome
Hospital mortality is defined as death occurring during the initial period of hospitalization or within 30 days of repair.

Significant perioperative morbidity was defined as (1) the need for more than 48 hours of ventilation postoperatively, or for reintubation after extubation; (2) the need for more than one intravenous inotropic agent for longer than 48 hours; (3) development of the postpericardiotomy syndrome; (4) the occurrence of wound or mediastinal infection; (5) the development of permanent or temporary arrhythmia that prolonged the hospital stay beyond 7 days; (6) the development of any infection, atelectasis, or congestive heart failure, or the need for oxygen resulting in a prolonged postoperative hospital stay beyond 7 days; and (7) the development of any seizure activity or neurologic deficit in the postoperative period.

Technique of Operation
All VSD repairs were performed using cardiopulmonary bypass and at least moderate hypothermia. Hypothermic circulatory arrest was used in 72 patients (37 of the 57 in the ASD-VSD subgroup and 35 of the 106 in the VSD subgroup), usually in the smaller ones. Cold crystalloid or blood cardioplegia was used to arrest the heart. The VSDs were repaired through the right atrium (n = 136; 83.4%), a right ventriculotomy (n = 21; 12.9%), a left ventriculotomy (n = 4; 2.5%), or the pulmonary artery (n = 2; 1.2%).

Statistical Analysis
The operation reports, discharge summaries, cardiac catheterization data, follow-up records, correspondence, and full hospital records were reviewed, and the collected data entered into a single database. Complete catheterization data were only available in 29 patients. Within this database, the subgroup of 57 infants with ASDs closed at operation was created and their data compared with those in the 106 patients with isolated VSD (VSD subgroup) (Table 3). Differences between these two subgroups were analyzed using the two outcome events of interest: hospital death and perioperative morbidity. Dichotomous variables were compared using ² tests, and continuous variables were compared with Wilcoxon rank-sum analysis.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Mortality
Hospital mortality was 3.7% (6/163). This was 2.8% for the VSD subgroup (3/107) and 5.3% for the ASD-VSD subgroup (3/57) (p = not significant). Three of the 6 patients who suffered hospital deaths had multiple VSDs. There were no deaths among the 33 infants with Down's syndrome. Since 1980, the hospital mortality was 1.4% (2/145), which is significantly less than that for the earlier era (p = 0.001). Multivariate analysis identified the presence of multiple VSDs and earlier date of operation as risk factors for hospital mortality (both p = 0.001).

Four additional patients died within 6 months of their operation, for a 6-month survival of 93.9% (153/163). Three of the four late deaths were related to noncardiac abnormalities. The actuarial survival at the 10-year follow-up was 92% ± 5% (Fig 1).

View larger version (10K): [in this window] [in a new window]   Fig 1. . Actuarial survival with 70% confidence limits for 163 infants undergoing repair of a ventricular septal defect.

Comparison between the ASD-VSD subgroup and the VSD subgroup revealed that the ASD-VSD patients were younger and smaller at operation, more often had circulatory arrest used in the repair, and tended to have higher preoperative pulmonary artery pressures. In addition, they more often needed substantial inotropic support postoperatively, had more pulmonary complications, and were ventilated longer postoperatively. Both their total hospital stay and postoperative hospital stays were also longer (see Table 3).

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Hospital Mortality
Since the first repairs of a VSD performed by Lillehei and colleagues in 1954 using controlled cross-circulation [12] and by Kirklin in 1956 using the early mechanical pump-oxygenator [13], the early mortality related to the surgical repair of VSDs in infancy has steadily decreased, from around 20% in the 1970s [14–16] to less than 5% in many recent series [4–6, 8, 9, 17, 18].

Improvements in preoperative diagnosis, surgical skills, operating room technology, and perioperative care made during the 1970s and early 1980s led to the widespread adoption of primary repair rather than preliminary pulmonary artery banding as the treatment of choice for symptomatic infants with isolated VSDs, except for those with multiple VSDs of the swiss cheese variety [4, 7, 19–21]. Our hospital mortality of 3.7% since 1977 and 1.4% since 1980 is further evidence that such a policy is well-founded. We, too, have found that the presence of multiple VSDs is an incremental risk factor for death (three of the six early deaths in our series occurred in patients with multiple VSDs; p = 0.001), and that this subset of patients is best managed by preliminary pulmonary artery banding and repair at about 2 years of age. This is particularly applicable to infants who are very small or in whom the defects are predominantly in the apical muscular septum. Many of these muscular defects may undergo spontaneous closure in the interim after pulmonary artery banding, facilitating the definitive repair [22, 23].

Prior repair of coarctation was not found to be a significant risk factor for subsequent VSD repair in this analysis; we continue to recommend initial coarctation repair only for the overwhelming majority of these patients, and reserve concomitant pulmonary artery banding for those with either large outlet-type VSDs (conal septal defects) or multiple large muscular VSDs. If spontaneous closure does not occur, or the infants continue to have congestive cardiac failure after their coarctation repair, we repair the VSD during the same hospitalization or soon thereafter. This is consistent with the practice described in other recent reports [24, 25].

The actuarial survival in our patients at the 10-year follow-up was 92% ± 5%, with no appreciable mortality after 1 year following repair, indicating a good long-term prognosis for this patient cohort (see Fig 1).

Perioperative Morbidity
We defined perioperative morbidity rather liberally in this analysis. By these standards, 26.7% (42/157) of our patients experienced significant morbidity. Although the incidence of complete heart block, mediastinitis, reoperation, and neurologic deficits was low, pulmonary complications such as infections, considerable atelectasis, and serous effusions were a common source of morbidity and prolonged hospital stay (see Table 5). This may reflect the degree of congestive heart failure and malnutrition commonly present preoperatively in these chronically ill small infants.

Despite the limitations of a study such as this in which it is difficult to differentiate cardiac-related from non–cardiac-related morbidity, multivariate analysis identified younger age at operation, the use of hypothermic circulatory arrest, the presence of an associated ASD, and the size of the VSD as independent risk factors for perioperative morbidity (see Table 4). When weight at operation was substituted for age at operation, the results of the analysis were essentially the same. This is not surprising, as the technical aspects of repairing a VSD in a 2.5-kg infant are little different from those used in a 6.0-kg infant; however, the effects of cardiopulmonary bypass and hypothermic circulatory arrest, and the ``third-spacing'' of fluid often occurring postoperatively in this subgroup of patients are associated with increased morbidity and a longer time to extubation. These, as well as other perioperative factors peculiar to major surgical procedures in very small infants (eg, airway management, fluid and electrolyte control, and feeding), result in their slower initial recovery compared with that in their older, larger infant counterparts. Hence, the finding of younger age at operation, use of circulatory arrest, and size of VSD as risk factors for morbidity is not unexpected.

Our analysis confirmed our clinical impression that the presence of a concomitant ASD per se increases the risk of perioperative morbidity. This is not difficult to conceptualize: additional left-to-right shunting at the atrial level increases the total left-to-right shunt; this correspondingly diminishes the cardiac output and aggravates the cardiac failure and failure to thrive. In addition, the hemodynamic characteristics of both ventricles in the setting of a large ASD in terms of preload and compliances may differ from that encountered in the setting of a simple VSD, a situation analogous to that occurring in the small subset of infants with a large isolated ASD who present with severe congestive heart failure. Unfortunately, in a historical analysis such as this, there are not sufficient appropriate data (eg, ventricular mass and volume indices) to permit these issues to be addressed. However, these infants are usually in severe congestive cardiac failure preoperatively, and have often been hospitalized numerous times for tube feeding, and for the control of cardiac failure and respiratory infections by the time they are referred for surgical treatment. Earlier operation, performed within the first 1 to 2 months of life, may lead to a lower perioperative morbidity by minimizing both the pulmonary effects of chronic congestive failure and the negative impact of poor nutritional status at the time of operation.

In our series, the ASD-VSD patients were smaller and younger at operation than were the VSD patients, and both characteristics have been associated with a poorer prognosis in terms of recovering and ``catching up'' after repair [5, 8, 18]. Our ASD-VSD population also tended to have a higher preoperative pulmonary artery pressure, suggesting higher shunt ratios or higher pulmonary vascular resistance, or both. Because increasingly fewer patients presenting for VSD repair in infancy undergo preoperative cardiac catheterization studies, we do not have sufficient data to analyze these variables in a meaningful manner.

The ASD-VSD subgroup had a higher percentage of Down's patients than did the VSD group (29% versus 15%; p = 0.02); however, the presence of Down's syndrome did not increase the risk of either morbidity or mortality after operation. The findings from our analysis corroborate a recent observation that the muscular septum is usually free of defects in Down's syndrome patients [26]: of 33 such patients in our analysis, 23 had perimembranous defects and 10 had inlet-type defects.

The ASD-VSD subgroup in this analysis tended to have more postoperative arrhythmias (most of them temporary), more pulmonary complications, a higher incidence of low cardiac output, and needed a longer period of postoperative ventilation than did the VSD group; they also had a longer postoperative hospital stay (see Table 3). Although they more often required hypothermic circulatory arrest for their repair, the period of arrest was within the safe range and was no different from that of the other patients (34 ± 13 versus 35 ± 14 minutes; p = not significant).

From the findings yielded by this study, it is apparent that currently most VSDs can be repaired in infancy with a low risk of death; those infants who are very small or who have multiple muscular VSDs would probably still benefit from a two-stage approach to their repair. However, those with concomitant large intraatrial shunts are at increased risk for perioperative morbidity due largely to their more serious preoperative status and more severe congestive heart failure. Rather than attempting to buy time for the patient to grow, this risk may be negated by an earlier operation.

Although these observations are intuitively known to most pediatric cardiologists and surgeons, this study was conducted to document them objectively. With the accent in medicine changing to cost containment and shortening of hospital and intensive care unit stays, it is important to recognize that this subgroup of patients does experience increased perioperative morbidity. The fact that they often need to stay in the hospital longer than would otherwise be anticipated becomes more relevant in terms of planning and the counsel given to parents, health care providers, and health care insurers.

	Acknowledgments

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
We thank Karen Dale for her assistance in preparation of the manuscript.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Presented at the Forty-first Annual Meeting of the Southern Thoracic Surgical Association, Marco Island, FL, Nov 10–12, 1994.

Address reprint requests to Dr Knott-Craig, Section of Thoracic and Cardiovascular Surgery, University of Oklahoma Health Sciences Center, PO Box 26901, Oklahoma City, OK 73190.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments 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): Christopher J. Knott-Craig Ronald C. Elkins Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Knott-Craig, C. J. Articles by Razook, J. R. Search for Related Content PubMed PubMed Citation Articles by Knott-Craig, C. J. Articles by Razook, J. R. Related Collections Related Article