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Ann Thorac Surg 2001;71:1260-1264
© 2001 The Society of Thoracic Surgeons

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Original article: cardiovascular

Surgery for coarctation of the aorta in infants weighing less than 2 kg

^a Department of Cardiac Surgery, The Children’s Hospital, Boston, Massachusetts, USA
^b Department of Cardiology, The Children’s Hospital, Boston, Massachusetts, USA
^c Department of Biostatistics, The Children’s Hospital, Boston, Massachusetts, USA

Accepted for publication November 6, 2000.

Address reprint requests to Dr Bacha, Pediatric Cardiac Surgery, Section of Cardiac and Thoracic Surgery, MC 5040, The University of Chicago Children’s Hospital, 5841 Maryland Ave, Chicago, IL 60637
e-mail: ebacha{at}surgery.bsd.uchicago.edu

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
Background. Low- and very low-birth weight infants are now candidates for reparative cardiac surgery. Outcomes after coarctation repair have not been characterized in this patient population.

Methods. We performed a retrospective review of 18 consecutive neonates less than 2 kg who underwent repair of aortic coarctation between August 1990 and December 1999.

Results. Median weight was 1,330 g, and median gestational age was 31 weeks. A ventricular septal defect was present in 5 patients, and Shone’s complex in 4. Sixteen patients had resection and end-to-end anastomosis, and 2 had resection and subclavian flap. Median clamp time was 15.5 minutes. One patient died during hospitalization. Two patients died late postoperatively (5-year estimated survival 80%). Mean follow-up was 28.5 months. Eight patients (44%) had a residual or recurrent coarctation, 5 underwent balloon dilation, and 3 underwent reoperation. Freedom from reintervention for recoarctation was 60% at 5 years. Shone’s complex or a hypoplastic arch was an independent risk factor for decreased survival (p < 0.001). Very low birth weight was a multivariate predictor for increased risk of recoarctation (p = 0.01).

Conclusions. Coarctation repair in less than 2-kg premature non-Shone’s infants can be performed with a low mortality. The rate of recoarctation is higher in the very low-birth weight infants, but can be managed with low risk.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
The benefits of early correction of congenital heart disease in low-birth weight neonates have been documented previously [1–4]. However, repair of coarctation at a very young age or at a low birth weight could be anticipated to carry an increased risk of recurrent coarctation [3–5]. Balloon dilation of native coarctation in the very young has been reported to result in unacceptable rates for recoarctation: 83% for neonates and 39% for infants, respectively [6]. In addition, previously reported mortality rates after coarctation repair in less than 2-kg infants have been high, ranging from 10% to 25% [2, 3, 7]. The aim of this study was to review the surgical results after repair of aortic coarctation in low- and very low-birth weight infants (defined as less than 2 kg and less than 1.5 kg, respectively), and to identify specific predictors of outcome.

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
Patient characteristics
Between August 1990 and December 1999, 18 consecutive neonates with a birth weight of less than 2 kg (median weight at surgery 1,330 g, range 800 to 1,950 g) underwent surgery for critical aortic coarctation at The Children’s Hospital (Boston, MA). This study was begun in 1990 because a previous study on low-birth weight neonates from our institution [2] spanned up to that date. All charts were retrospectively reviewed, including follow-up clinic notes and latest echocardiograms. All data procurement were carried out after institutional approval according to guidelines established by the committee on clinical investigation.

Thirteen patients (72%) were male. The median gestational age (GA) was 31 weeks (26 to 35 weeks) and the median age at surgery was 12 days (4 to 46 days). Fifteen patients had a ductal-dependent lesion. Four patients were being treated for possible necrotizing enterocolitis. Fourteen patients had pre- and postoperative assessment of an intracranial hemorrhage either with head ultrasound or head computed tomogram as part of our routine management of premature infants: 6 patients had a grade I intraventricular hemorrhage (IVH) and 1 had a grade II IVH. All patients were intubated: 10 had mild hyaline membrane disease, 3 had moderate hyaline membrane disease, and 2 were on a high-frequency oscillator. Before coarctation repair, 1 patient had a catheterization with attempted balloon dilation of the coarctation and aortic valve, and 1 patient underwent a congenital diaphragmatic hernia repair. Associated cardiac malformations were present in 11 patients: ventricular septal defect (VSD) in 5 and Shone’s complex or multilevel obstruction of the left side of the heart in 4, with a combination of hypoplastic arch in 4, valvar aortic stenosis in 3, and subaortic stenosis in 2.

Follow-up was carried out by direct communication with the patient’s family or local physician. One patient was lost to follow-up after a period of 36 months.

Statistical analysis
Estimated rates of survival and freedom from recoarctation were determined by the Kaplan-Meier product-limit method with 70% confidence intervals (CI) based on Greenwood’s formula. Univariate analysis was evaluated by the log-rank test. The Cox proportional-hazards regression model using a stepwise forward selection procedure was utilized to identify the variables independently predictive of each outcome, with risk measured by the hazard ratio and corresponding 70% CI. Variables included age at surgery, gender, gestational age, birth weight, weight at surgery, presence of VSD, presence of Shone’s disease or hypoplastic arch, clamp time, surgical technique (extended end-to-end, use of a subclavian flap), and the presence of mild (< 15 mm Hg) or moderate (> 15 mm Hg) arm-leg gradient immediately after surgery. Recoarctation was treated as a time-dependent covariate. A two-tailed p value less than 0.05 was considered statistically significant throughout. Analysis of the data was conducted using the SPSS software package (version 9.0; SPSS Inc, Chicago, IL).

	Results

Top Abstract Introduction Patients and methods Results Comment References

 
Operative results
All patients were operated on via a left posterolateral thoracotomy. The coarctation was resected and a primary end-to-end anastomosis was performed in 16 (89%) patients (with extension into the arch in 9), and 2 patients underwent resection of the coarctation and reverse subclavian flap augmentation of the distal arch with end-to-end anastomosis. In all cases, a running suture of 6-0 or 7-0 polypropylene was used. The median clamp time was 15.5 minutes (12 to 41 minutes). Arm-leg gradients were measured in all patients either in the operating room or in the intensive care unit; 10 patients had no arm-leg gradient, 5 had a gradient of less than 15 mm Hg, and 3 had a gradient of more than 15 mm Hg (17, 20, and 25, respectively). One patient died during the same hospitalization (hospital mortality 5.5%). She was a 1.7-kg 33-week GA baby girl with ductal-dependent coarctation, severe arch hypoplasia, and VSD who underwent excision of the coarctation and reverse subclavian flap augmentation of the distal arch at 7 days of age. Because of evidence of left-to-right shunting and the presence of a moderate residual coarctation (gradient of 25 mm Hg), she underwent VSD closure and aortic arch patch augmentation utilizing deep hypothermic circulatory arrest 10 days after the initial procedure. Although she initially did well with no residual coarctation or VSD seen on a postoperative catheterization, she could not be extubated and eventually died of sepsis at age 2 months.

Postoperative complications
Median intubation time and intensive care length of stay were 9 (1 to 60) and 13 days (4 to 60 days), respectively. The patient with a preoperative grade II IVH progressed to grade II-III postoperatively. No other neurologic complications occurred. All other preoperative head imaging studies remained unchanged postoperatively. No phrenic or recurrent laryngeal nerve damage was detected. One patient developed a chylothorax that required thoracic duct ligation.

Long-term results
Survival
The mean follow-up was 28.5 months (range 4 to 91 months). Two patients with Shone’s syndrome died late. One died 4 years after coarctation repair after 5 additional procedures, which included mitral valve plasty with supramitral ring resection, balloon dilation of the aortic valve, subaortic stenosis resection, mitral valve replacement, and heart transplantation. Another patient died 3 months after his initial surgery. He represented with mitral stenosis (MS). Balloon dilation of the mitral valve resulted in severe mitral regurgitation (MR). A stage I Norwood procedure was performed for severe MS/MR (weight at the time: 2.1 kg). After a reasonable early postoperative course, the patient developed multiple intracardiac thrombi. Despite operative removal, the patient died several days later.

The Kaplan-Meier actuarial 5-year survival is 80% (70% CI = 70% to 90%) (Fig 1). Univariate analysis revealed that the presence of Shone’s disease or hypoplastic arch was associated with a higher probability of death (P = 0.004, log-rank test). The multivariate Cox regression model confirmed that these factors were independently predictive of survival, with the risk of death being eight times higher each month for patients with Shone’s disease or hypoplastic arch (hazard ratio = 8.8, 70% CI = 3.3 to 23.9, p < 0.001). Survival was not associated with age at surgery, gender, gestational age, birth weight, presence of VSD, recoarctation, clamp time, or the presence of mild or moderate gradient immediately postsurgery (all p > 0.10, Table 1).

View larger version (12K): [in this window] [in a new window]   Fig 1. Kaplan-Meier estimates of patient survival after repair of aortic coarctation. Error bars indicate 70% confidence intervals. Numbers of patients in the follow-up on whom the estimates were based are shown in parentheses.

View larger version (14K): [in this window] [in a new window]   Fig 2. Kaplan-Meier estimates of freedom from recoarctation after repair of aortic coarctation. Error bars indicate 70% confidence intervals. Numbers of patients in the follow-up on whom the estimates were based are shown in parentheses.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

Reported mortality rates for coarctation repair in low-birth infants vary from 12.5% to 25% in the recent literature [2, 3]. A study from our own institution spanning up to 1990 had reported a mortality of 25% for coarctation repair in less than 2.5-kg neonates [2]. The marked improvement in surgical mortality reported in this study probably reflects refinements in perioperative care as well as in surgical technique and anesthesia made during the 1990s [1]. Furthermore, keeping in line with our institutional philosophy of stabilizing children before repair and allowing end-organ function to recover, no patient was operated on emergently.

Restenosis after coarctation repair, regardless of technique, has been clearly associated with young age at operation [8–10]. It is also known that the majority of restenoses occur within 1 year of repair [10]. The freedom from recoarctation in the entire series was 60% at 5 years. However, it was also found that very low-birth weight infants (< 1,500 g) had a significantly higher chance of developing a recoarctation or a residual coarctation (6 out of 10 patients) as compared with low-birth weight infants (1 out of 8 patients) (p = 0.01). At the same time, all very low-weight birth infants survived coarctation repair. Neither clamp time nor surgical technique were predictors of recoarctation. This implies that factors related to immature tissues or residual ductal tissue are the cause of recoarctations seen in the very low-birth weight newborns. It is in this high-risk group that technical points such as elimination of all ductal tissue, a tension-free repair, and precise suturing techniques become particularly important. Some degree of aortic arch hypoplasia is almost always associated with neonatal aortic coarctation, and should be addressed at the time of repair. A reverse subclavian flap repair will enlarge the distal arch, whereas an extended end-to-end repair will effectively deal with transverse or distal arch hypoplasia.

The presence of a mild or moderate gradient immediately after surgery did not correlate with recoarctation. This is in contrast to the report by Brouwer and associates [9], which showed that postoperative gradients predict recoarctation. The discrepancy between the two studies may be explained by the difference in weights (median weight of 1,400 g in our study vs 4,000 g in Brouwer’s), underscoring the difficulties with either invasive or noninvasive monitoring of blood pressure in less than 2-kg infants.

Whereas a recurrent coarctation can be dealt with quite effectively with balloon dilation when the child is older [6], it is difficult to deal with a severe symptomatic residual coarctation in this patient population. One has to consider re-repair via left thoracotomy, arch augmentation via sternotomy on cardiopulmonary bypass (2 cases in our experience), or even, in the case of Shone’s complex for example, conversion to a stage I repair. Given the fact that the presence of Shone’s anomaly was also associated with decreased long-term survival in this study, and given recent reports of poor long-term survival in children with multilevel left heart obstruction [8, 11, 12], the indication for a single-ventricle approach should at least be considered in neonates with Shone’s disease. This is further buttressed by recent data showing operative survival rates of up to 96.3% after a Norwood stage I procedure in the presence of a functional left ventricle [13]. While it is beyond the scope of this limited retrospective study to give specific guidelines regarding single- versus two-ventricle repairs in neonates with Shone’s complex and coarctation, it does underscore this potential dilemma.

In conclusion, premature infants weighing less than 2 kg can undergo repair of aortic coarctation with a relatively low mortality. The presence of multilevel left heart obstruction is associated with decreased long-term survival. The recoarctation rate remains important in very low-birth weight patients, but can be effectively dealt with when combined with balloon dilation or reoperation.

	References

Top Abstract Introduction Patients and methods Results Comment 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): Emile A. Bacha John E. Mayer, Jr Richard A. Jonas Pedro J. del Nido Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Bacha, E. A. Articles by del Nido, P. J. Search for Related Content PubMed PubMed Citation Articles by Bacha, E. A. Articles by del Nido, P. J. Related Collections Congenital - acyanotic