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Eur J Cardiothorac Surg 2000;17:643-647
© 2000 Elsevier Science NL

Primary repair of aortic arch obstruction with ventricular septal defect in preterm and low birth weight infants

^a Division of Pediatric Cardiovascular Surgery, Section of Cardiac Surgery, F7830 C.S. Mott Children's Hospital, University of Michigan School of Medicine, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA
^b Division of Pediatric Cardiology, Department of Pediatrics, C.S. Mott Children's Hospital, University of Michigan School of Medicine, Ann Arbor, MI, USA

Corresponding author. Tel.: +1-734-936-4980; fax: +1-734-763-7353
e-mail: elbove{at}umich.edu

	Abstract

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion References

 
Objective: Previous reports have suggested that prematurity and low birth weight are risk factors for definitive surgical intervention in congenital cardiac malformations. The following data review our experience with primary repair of the complex malformation of aortic arch obstruction with ventricular septal defect (VSD) in this patient population. Methods: Since 1988, 21 consecutive preterm (36 weeks) and/or low birth weight (<3000 g) infants with interrupted aortic arch (IAA; n=10), or aortic coarctation (n=11) with VSD, underwent primary arch repair and VSD closure. The mean weight at operation was 2310 g (range, 1200–2900 g), including 12 patients at 2500 g. The gestational age ranged from 30 to 41 weeks (mean, 36.4 weeks). Five patients with interrupted arch and two patients with coarctation also had severe subaortic stenosis, which was relieved by transatrial incision of the infundibular septum. Results: The overall hospital mortality was 14% (3/21). Death was related to low cardiac output in association with severe subaortic stenosis (n=2) and sepsis (n=1). Late mortality occurred in three patients, two of which were non-cardiac. The mean follow-up was 33 months. Two patients had significant recurrent arch obstruction, which was successfully relieved by balloon angioplasty and surgical correction in one each. The survival at 30 days, and at 1 and 3 years was 86, 76 and 70%, respectively. Conclusions: Complete primary repair of aortic arch obstruction with VSD can be achieved with good results, even in the preterm and low birth weight infant. Therefore, early surgical repair of this congenital malformation is recommended.

Key Words: Congenital cardiac surgery • Preterm infants • Aortic arch obstruction • Ventricular septal defect closure

	1. Introduction

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion References

 
An aortic arch obstruction associated with a ventricular septal defect (VSD) is an uncommon congenital malformation, which often requires immediate surgical repair in the neonatal period. Although the perioperative morbidity and mortality have significantly improved during recent years [1–5], surgical treatment, either by a staged approach or by primary repair, remains a challenge. This is particularly true for patients presenting with low birth weight or prematurity, which are risk factors for early and late mortality after neonatal cardiac surgery [6,7]. The technical challenge of primary repair and concerns regarding the use of cardiopulmonary bypass (CPB) in premature infants have led many centers to favor a staged approach, with initial reconstruction of the aortic arch and subsequent VSD closure, with or without pulmonary artery banding.

Relatively little data evaluating the results of complete repair of congenital heart defects in low birth weight and premature infants are available [7–9]. The present study summarizes our experience with the primary repair of aortic arch obstruction and VSD in the premature and/or low birth weight infant to assess early and intermediate outcome.

	2. Materials and methods

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion References

 
Between August 1988 and August 1998, 11 consecutive preterm (36 weeks) and 10 consecutive fullterm, but low birth weight (<3000 g) infants with interrupted aortic arch (IAA; n=10) or aortic coarctation (n=11) and VSD underwent primary repair at C.S. Mott Children's Hospital, the University of Michigan Health System. Patients with complex intracardiac lesions, such as atrioventricular septal defects and conotruncal abnormalities, were excluded from this review. The gestational age ranged from 30 to 41 weeks (median, 36 weeks), and median age at operation was 9 days (range, 3–25 days). The operative weight ranged from 1200 to 2900 g (mean, 2310 g); with twelve patients below 2500 g. All patients were in congestive heart failure prior to surgery, and received an infusion of prostaglandin E₁ to maintain ductal patency. Inotropic support and mechanical ventilation was required in 14 infants to further improve their preoperative condition.

Associated cardiac lesions and important non-cardiac abnormalities for the entire patient group are presented in Table 1.

2.1. Surgical technique
Surgery was performed through a median sternotomy with deep hypothermic circulatory arrest. In cases of IAA, the main pulmonary trunk and the ascending aorta were both cannulated. Patients with coarctation of the aorta underwent single arterial cannulation of the ascending aorta. A single venous cannula was inserted in the right atrial appendage in all patients. CPB was initiated with cooling to between 17 and 20°C, during which time, the head vessels and the proximal descending thoracic aorta were widely mobilized. In patients with an anomalous right subclavian artery, the artery was divided to improve mobilization of the upper descending thoracic aorta. After the circulation was arrested and the head vessels were occluded, a single dose of dilute blood cardioplegia solution was administered (20 ml/kg). In each patient with IAA, aortic arch reconstruction was accomplished by direct anastomosis utilizing a continuous absorbable suture. Nine patients with coarctation underwent aortic arch reconstruction by an extended end-to-end anastomosis [12]. The remaining two patients with coarctation had severe transverse arch hypoplasia. One patient required an end-to-side anastomosis of the descending aorta to the ascending aorta. In the second patient, a glutaraldehyde fixed autologous pericardial patch was utilized to reconstruct the undersurface of the aortic arch. In all but two patients, VSD closure was accomplished by a transatrial approach using a prosthetic patch and a continuous suture technique. Right ventriculotomy was employed in one patient with a subarterial VSD. Another patient presented with an overriding pulmonary artery, through which a supracristal VSD was closed. In patients with severe subaortic narrowing, a stay suture was placed in the infundibular septum to facilitate exposure and visualization of the aortic valve [11]. Excision of the infundibular septum was frequently not feasible, but simple incision was effective in widening the outflow tract, thereby relieving subaortic stenosis. The mean CPB time was 64 min (range, 45–85 min), the mean cross clamp time was 46 min (range, 33–68 min) and the mean circulatory arrest time was 45 min (range, 33–68 min). Seven infants underwent delayed sternal closure.

2.2. Statistical analysis
The distribution of each variable was evaluated with univariate analysis prior to bivariate analysis. Univariate data are presented as mean values (95% CI). Comparison between categorical data was performed with the chi-square test. The Student's t-test was used to compare continuous data, and the Wilcoxon rank sum test was used to analyze non-parametric ordinal variables. Kaplan–Meier survival analysis was performed. The data was stored in Microsoft Excel and analyzed with SAS statistical software.

	3. Results

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion References

 
Early mortality occurred in three patients (14%). In two patients, death was related to low cardiac output, in association with severe subaortic stenosis and a hypoplastic aortic valve in both. One of these patients suffered acute myocardial failure and could not be weaned from CPB, and the other died on the first postoperative day. The third patient died 41 days after successful repair from gram-negative sepsis associated with DiGeorge syndrome. There were three additional late deaths, only one of which was cardiac related. This premature infant with IAA type B underwent reoperation for recurrent arch obstruction 4 months after the initial operation, as well as resection of a subaortic membrane 12 months later, which had not been present during the initial repair. He died 4 months after the last operation of cardiac failure of unknown cause in another hospital. The remaining two non-cardiac related deaths occurred in a premature infant 3 months after repair from aspiration, and from acute bilateral bronchopneumonia 18 weeks after successful operation in the other. The overall mortality was associated with: longer CPB time, 78 min (95%CI, 67–89) vs. 61 min (95%CI, 56–66), P=0.05; longer X-clamp time, 59 min (95%CI, 50–68) vs. 43 min (95%CI, 40–46), P=0.01; and longer circulatory arrest time, 58 min (95%CI, 48–67) vs. 42 min (95%CI, 39–45), P=0.02. The age at the time of operation was 14 days (95%CI, 7–21) vs. 9 days (95%CI, 7–11), P=0.23, and the weight at the time of operation was 1.98 kg (95%CI, 1.48–2.44) vs. 2.45 kg (95%CI, 2.21–2.69), P=0.06.

As shown in Fig. 1, the actuarial survival for the entire group at 30 days, and at 1 and 3 years was 86, 76 and 70%, respectively.

View larger version (8K): [in this window] [in a new window]   Fig. 1. Actuarial survival curve with the hospital mortality included.

	4. Discussion

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion References

 
This retrospective analysis of our experience with the primary repair of aortic arch obstruction associated with VSD confirms that this approach can be accomplished with good results, even in the preterm and low birth weight infant. The early mortality of 14% and the single cardiac related death during follow-up are consistent with previous reports [2,4,5]. Several risk factors for early death after repair of IAA/CoA have been reported, including age and weight, subaortic narrowing, and single stage repair with myotomy or myomectomy for LVOTO [1,3]. Because this study only includes critically ill patients operated on soon after birth and weighing less than 3000 g, it is not surprising that age and weight were not found to be risk factors for death. However, the time of CPB, circulatory arrest and aortic cross clamping were significantly longer for patients who died, suggesting the importance of a rapid and accurate repair in these tiny and fragile infants.

LVOTO is a frequently associated condition in patients with aortic arch obstruction and VSD. In the present study, severe LVOTO was present in seven patients, all of whom underwent transatrial resection/incision of the infundibular septum. In the remaining patient with moderate LVOTO, an attempt at surgical relief of the obstruction was abandoned due to suboptimal exposure of the aortic valve. The relief of LVOTO by transatrial muscle resection appears not only to be safe, but also effective, as evidenced by the lack of any significant recurrence of subaortic stenosis during the follow-up period. Although myomectomy and myotomy have previously been considered as risk factors for death from multiinstitutional trials [1,3], our data do not support this conclusion. In this study, only two of seven patients who had undergone myomectomy died. These two patients had the smallest subaortic diameters (2.5 mm), with a diastolic ratio of 0.4 and 0.5, respectively. In addition, the Z-values of the aortic annulus were -3.2 and -3.7. Therefore, in retrospect, it appears that it would have been better to consider these patients as having aortic annular hypoplasia and treat them with a Norwood or Norwood/Rastelli procedure. The results with this type of operation for aortic atresia or hypoplasia with VSD are encouraging, as previously reported from our institution [13].

In the present study, complications were mainly related to conditions associated with prematurity. Two patients required prolonged ventilatory support due to laryngomalacia and hyaline membrane disease, and one patient, who demonstrated preoperative renal insufficiency, required peritoneal dialysis postoperatively. Major neurological complications were encountered in two patients postoperatively. One 34-week-old preterm infant was diagnosed with grade 2 intraventricular hemorrhage when he experienced seizure activity 4 days after the primary repair, after awakening normally following the operation. Although concern exists regarding the employment of CPB and heparin in premature infants, the interval between the operation and the onset of seizure activity makes it unlikely that this intracranial hemorrhage was primarily related to CPB. The second patient experienced subcortical bihemispheric ischemic brain injury diagnosed by a MRI scan. Because the total circulatory arrest time was 49 min, a time frame which is usually well tolerated under deep hypothermia, the reason for this complication remains uncertain. Overall, eight patients (38%) in this study experienced significant postoperative complications, a rate which compares favorably with previous studies in full term infants [2,14].

Significant recurrent aortic arch obstruction requiring balloon angioplasty or reoperation was infrequent in this series. IAA was addressed by generous primary anastomosis. Resection with extended end-to-end anastomosis was employed for most patients with coarctation, removing the coarctation and the associated shelf of intimal proliferation, as well as enlarging the hypoplastic aortic arch and isthmus.

In conclusion, the primary repair of aortic arch obstruction and VSD closure can be achieved with good results, even in the preterm and low birth weight infant. Mortality and morbidity are primarily related to non-cardiac causes. Severe subaortic obstruction, which is common in this congenital malformation, can be treated safely and effectively by transatrial resection. Recurrent LVOTO or aortic obstruction has been infrequent. In addition, the lack of any significant residual VSD requiring reoperation, and the absence of complete heart block supports our policy of primary repair, even in the smallest infants. Therefore, early surgical repair of this congenital malformation is recommended.

	Footnotes

 
Presented at the 13th Annual Meeting of the European Association for Cardio-thoracic Surgery, Glasgow, Scotland, UK, September 5–8, 1999.

	Appendix A Conference discussion

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion References

 
Dr A. Corno (Lausanne, Switzerland): Since you have a report from the North American Congenital Heart Surgeons Society showing that there is a significantly better survival at 2 years after a staged approach versus primary repair, based on what kind of hypothesis do you start your study with a primary approach?

Are your series of consecutive patients, or, if during the same period, did you offer a staged approach with some patients?

Dr Haas: The series consists of consecutive patients. We did not perform any staged operation in a patient with coarctation or IAA with VSD and subaortic stenosis. We feel more confident that a primary repair is probably better in this specific patient population, because we have the advantage to correct the underlying lesion and to prevent any secondary organ damage associated with prematurity, or associated with persistent congestive heart failure in a case of an unclosed VSD. So, I think for this specific patient population, it is probably the best approach to try to correct the cardiovascular status, and therefore, to improve the overall wellbeing of the infant.

Dr G. Stellin (Padova, Italy): My question is the following. You had two patients who died from severe residual subaortic stenosis. Are you planning in the future to do some more radical operation in these patients, rather than trying to resect the subaortic obstruction, let's say, a Konno–Ross operation?

Dr Haas: I think that's a very interesting point. The two patients who died very early had the smallest subaortic diameters of 2.5 mm. In addition, the Z-values of the aortic annulus, were -3.2 and -3.7. Due to the fact that the aortic annulus was hypoplastic, I would say in retrospect, that it would have probably been better to treat these patients with a Norwood type operation or a Norwood–Rastelli operation, because the aortic annulus was really too small to carry all the cardiac output.

	References

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion Appendix A Conference discussion 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): Richard G. Ohye Ralph S. Mosca Edward L. Bove Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Haas, F. Articles by Bove, E. L. Search for Related Content PubMed PubMed Citation Articles by Haas, F. Articles by Bove, E. L.