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Ann Thorac Surg 2004;78:1989-1993
© 2004 The Society of Thoracic Surgeons
a Klinik für Herzchirurgie, Universität Leipzig, Herzzentrum, Leipzig, Germany
b Kinderkardiologie, Universität Leipzig, Herzzentrum, Leipzig, Germany
c Anästhesie, Universität Leipzig, Herzzentrum, Leipzig, Germany
Accepted for publication May 6, 2004.
* Address reprint requests to Dr Kostelka, Universität Leipzig, Herzzentrum, Klinik für Herzchirurgie, Strümpellstr 39, 04289 Leipzig, Germany
kostelm{at}medizin.uni-leipzig.de
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Abstract |
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 Top Abstract IntroductionPatients and MethodsResultsCommentReferences |
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METHODS: Twenty-four consecutive patients with IAA (n = 12) or CoA-HyAA (n = 12) with VSD underwent early one-stage correction. Patients' mean age was 12 days (range, 2 to 188); mean weight was 3.6 kg (range, 2.1 to 7.3), 6 patients were less than 2.5 kg. Three IAA were type A, 5 type B1, 3 type B2, and 1 type C. Associated anomalies included a large VSD in all, left ventricular outlet tract obstruction in 5, transposition of the great arteries, aortopulmonary window, persistent truncus arteriosus, and double-outlet right ventricle in 1 patient. Selective brain perfusion through innominate artery and selective coronary perfusion through aortic root during aortic arch reconstruction was used in all patients. Mean follow-up was 2.2 ± 0.9 years.
RESULTS: There was no early, no late mortality, and no postoperative neurologic complications. Mean crossclamp duration was 72 ± 32 minutes, lowest temperature 22.8 ± 4°C and selective brain and coronary perfusion duration was 34 ± 13 minutes. Eighteen patients required delayed sternal closure at 1.7 days postoperatively. New perioperative management reduced the overall morbidity. Four patients after IAA plus VSD repair developed aortic arch restenosis and were successfully treated by balloon dilatation. One patient with d-TGA underwent right ventricular outflow tract reconstruction of right ventricular outlet tract obstruction 7 months after the initial repair. Pressure gradients across the anastomosis at most recent follow up were less than 10 mm Hg. All patients are asymptomatic and are developing normally.
CONCLUSIONS: One-stage complete correction is feasible in newborns with aortic arch obstruction with VSD. Complex cardiac anatomy presents no additional risk for the procedure. The early one-stage correction yields excellent surgical results and good functional outcome.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Despite the absence of randomized studies comparing both approaches for aortic arch obstruction plus VSD, primary one-stage correction has been adopted in several centers in recent years [1, 3, 7]. That adoption was probably due to improvements and refinements in the operative technique including improvements in the techniques of perfusion. However, the morbidity remains as high as 10% to 15% [1, 8, 9]. Furthermore, there is a reduced but persistent risk of perioperative as well as postoperative mortality in the range of 10% to 14% [1–3]. The aim of this study was to analyze our current results of one-stage correction for aortic arch obstruction associated with VSD.
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Patients and Methods |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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The IAA was type A in 3 patients, type B in 8, and type C in 1. In all patients with CoA, the median diameter of the aortic arch was 2.5 mm [2–4], the diameter of the ascending aorta 7 mm [5–9]. All patients underwent echocardiographic examinations before discharge from the hospital and at follow-up visits. A total of 13 patients received postoperative evaluation using cardiac catheterization.
Surgery was performed through median sternotomy in all patients. For small priming volume extracorporeal circulation (ECC), superior and inferior vena cava were cannulated with metal-tip 10F to 12F cannulas (Stöckert, Germany) and the ascending aorta with angulated 2- to 2.6-mm cannula (Stöckert). In all patients with IAA, additional temporary cannulation of the patent ductus arteriosus (2.0-mm arterial cannula) was used for systemic perfusion during the cooling. All patients had a left atrial vent inserted through the right upper pulmonary vein and a small cardioplegic cannula placed in the aortic root for selective coronary perfusion. Patients were cooled to 22°C (range, 18 to 28°C), during which time further dissection of the aortic arch, head vessels, and the descending aorta was performed. Topical cooling of the head was used in all patients.
After clamping the ascending and descending aorta, the left carotid and left subclavian arteries were snared, and low-flow antegrade perfusion of the innominate artery (the arterial cannula was turned into the innominate artery, which was then snared proximally) and low-flow coronary perfusion through the cardioplegic cannula connected to the parallel tubing of the aortic cannula were maintained. The ECC perfusion was kept at 20% of the normal flow. The patent ductus arteriosus was ligated and resected carefully. The aortic arch was the reconstructed using a direct anastomosis of the descending to the ascending aorta in all 12 patients with IAA, and an extended end-to-side anastomosis of the descending aorta to the proximal aortic arch following the blind closure of the distal aortic arch behind the left subclavian artery in all 12 patients presenting with CoA-HyAA. Aortic arch reconstruction was performed on the beating heart during the cooling. After the arch reconstruction, the head vessels and the descending aorta were reopened and the full flow ECC reinstituted; a single-shot 30 mL/kg St. Thomas crystalloid solution was infused into the aortic root. The VSD was closed using an autologous pericardial patch and interrupted 5-0 polypropylene sutures, buttressed with Teflon pledgets. The transatrial approach was used in 22 patients and the transventricular approach in 2 patients. Three of 5 patients with IAA (type B2) had additional subaortic stenosis resection due to posterior malalignment of the infundibular septum and received transatrial subaortic stenosis resection.
A small prime ECC circuit (total priming volume 210 mL) was used. The reservoir as well as the pump were placed directly beside the surgeon. The prime consisted of 120 mL packed red cells, 80 mL 5% human albumin, 3 mL/kg 15% mannitol, 5 mL sodium bicarbonate, 100 IU/kg heparin, and 20 mg/kg steroids (Urbason; Aventis Pharma Deutschland GmbH, Bad Soden, Germany). During the operation the hematocrit was maintained above 30%. Management of Trasylol (Aprotinin; Bayer, Leverkusen, Germany) was as follows: 30,000 U/kg before ECC, 30,000 U/kg in ECC before commencing, and finally 30,000 U/kg after ECC discontinuation. Hemofiltration was used in all patients during the perfusion. Perioperative therapy as well as the details on extracorporeal circulation are shown in Table 1. Results are given as median and range or as mean ± SD.
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Results |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Bypass time was 154 minutes (range, 122 to 285), aortic crossclamp duration 70 minutes (39 to 165), and median selective brain and coronary perfusion duration was 39 minutes (14 to 50). Weaning from bypass was uneventful in all patients using moderate dopamine (3 µg · kg–1 · min–1) and dobutamine support (4 to 8 µg · kg–1 · min–1), Corotrop 50 µ/kg in CPB before discontinuation of ECC (Sanofi, Paris, France). All patients received volume-controlled ventilation with 20 ppm nitric oxide. Delayed chest closure was performed in 18 patients after 1.7 ± 1.2 days.
Postoperatively, all patients were transferred to the pediatric intensive therapy unit in a hemodynamically stable condition. Patients were intubated for 4.9 days (range, 0.4 to 27), inotropic therapy was required for 6 days (1.1 to 27). Intensive therapy unit stay was 9.2 days (range, 2.8 to 28) and in-hospital stay was 13.5 days (6 to 36). Postoperative capillary leak syndrome presenting with severe edema occurred in 2 patients. Renal failure requiring peritoneal dialysis was present in 1 patient.
Postoperatively, 21 patients were in sinus rhythm, 2 in atrioventricular rhythm, and 1 required temporary external pacemaker stimulation. All patients were in sinus rhythm at discharge.
During the postoperative course there were no neurologic deficits and no seizures in any patient. On ultrasonography examinations there was no bleeding, edema, or hydrocephalus in comparison with routine preoperative cerebral ultrasonography examinations. The echo-Doppler showed normal cerebral flow pattern in all surgical patients.
Echocardiography revealed good left and right ventricular function in all patients postoperatively. A small pinhole residual VSD was present in 1 patient and a small apical muscular VSD in 1 patient. Moderate tricuspid valve incompetence was seen in 4, moderate pulmonary valve incompetence in 2, moderate mitral valve incompetence in 1, and moderate aortic valve incompetence in 1 patient.
Mean follow-up was 2.3 years (range, 1.5 to 4.4). All patients were developing normally until the most recent outpatient examination. There was normal growth and there were no neurologic symptoms. Echocardiography revealed good ventricular function in all patients. Normal flow without turbulence from the ascending aorta, aortic arch into the descending aorta was seen in 20 patients. Four patients, 2 of them with IAA type B1 and 2 with type B2, presented with recurrent aortic arch stenoses. All had blood flow velocities above 3 m/s and maximum pressure gradients more than 40 mm Hg at the site of the ascending to descending aortic anastomosis. Two of these 4 patients were less than 2.5 kg during the one-stage correction. All 4 were successfully treated with balloon dilation reducing the rest gradient below 15 mm Hg. One other patient was diagnosed having a dynamic, functional stenosis in the right ventricular outflow tract at 6 months follow-up examination. Balloon dilation of the pulmonary valve was unsuccessful. The right ventricular outflow tract was enlarged with a bovine transannular pericardial patch during reoperation. All 5 patients requiring repeat interventions had a normal further development with persisting improvement in hemodynamic function until their most recent follow-up.
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Comment |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Patients present with a variety of stenoses at different levels of the aorta. These range from complete interruptions to severe stenoses at different parts of the proximal or distal arch or the isthmus region. Owing to this variety, we analyzed the results of both patients with IAA and patients presenting with coarctation of the aorta plus hypoplastic aortic arch together. The similar appearance as well as comparable operative repair strategies justify this approach.
Overall, aortic arch obstruction with VSD is a moderately frequent condition. Patients usually present with open persistent ductus arteriosus in stable hemodynamic and metabolic situation. Restrictive patent ductus arteriosus represents a severe condition requiring prostaglandin therapy, inotropic support, and ventilatory support owing to severe hemodynamic deterioration.
All patients with aortic arch obstruction and VSD require surgical therapy. One-stage correction has evolved as the standard approach. Literature results in the 1990s have proven a good outcome when using a one-stage correction strategy even in newborns regardless of birth weight [1–3,10]. However, residual morbidity remains as high as 10% to 15%, and there is a low but persistent risk of mortality, which may be due to underlying patient related factors, especially when presenting in deteriorated conditions. But it has to be kept in mind that one-stage correction of aortic arch obstruction and ventricular septal defect is a challenging operation for any patient. One of our patients required a staged repair owing to acutely evolving aortic dissection as reported previously [11].
Optimal perioperative and postoperative management are required to perform this operation successfully. The perioperative strategy of ECC has been refined during the recent years. New techniques of selective cerebral perfusion have evolved, reducing the theoretical risk of perioperative neurologic damage. The selective coronary perfusion reduced the cardiac arrest duration during the aortic arch reconstruction; that is especially advantageous in patients with very complex intracardiac anatomy in whom a longer cross-clamp duration is expected.
We applied these new techniques in all patients. Selective cerebral perfusion can be achieved quite easily by turning the aortic cannula that is usually directed into the aortic arch directly into the innominate artery. Selective cerebral perfusion should thus be the standard approach for all or at least most intracardiac corrections requiring concomitant aortic arch reconstruction. Similar findings have been published previously [12]. The selective coronary perfusion is delivered through the small plastic cardioplegic cannula through side tubing connected to the aortic cannula. Regarding the technique of extracorporeal perfusion, we applied a small circuit located directly besides the surgeon and thus required a very low amount of priming volume. Routine perioperative hemofiltration allowed us to achieve a negative fluid balance in all patients perioperatively.
As a result of this strategy, we did not observe any postoperative neurologic deficit, and we significantly reduced the postoperative morbidity (only 1 patient with renal failure and need of peritoneal dialysis). The mean hospital stay was 14 days. All children are developing normally at the most recent follow-up examinations.
In conclusion, patients with aortic arch obstruction and VSD should receive one-stage correction, which can be accomplished with an acceptably low risk and good functional outcome even in newborns with very low weight. Complex cardiac anatomy presents no additional risk for the outcome.
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