Anatomic reconstruction of recurrent aortic arch obstruction in children.

OBJECTIVE: Anatomical reconstruction of the aortic arch following aortic arch surgery is challenging. The placement of an extra-anatomical aortic bypass has been proposed for these difficult cases. This approach is not ideal in children due to possible long-term complications. This study presents the results of our policy to reconstruct the aortic arch in recurrent obstruction in children, which are not amenable to balloon dilatation. METHODS: Seven children with a median age of 8 years (range 1 month-15 years) were operated for aortic arch obstruction following correction of an aortic coarctation. Six children presented another intra-cardial lesion (2 subaortic membranes; 2 VSDs, 1 ostium stenosis of the left main coronary artery, and 1 mitral valve insufficiency). The surgical approach involved a sternotomy, cardiopulmonary support using two arterial inflow cannulas (one above and one below the aortic arch), and moderate hypothermia. Enlargement of the aortic arch was performed by a sliding plasty in four patients and by a patch plasty in three patients. Associated cardiac defects were corrected as well. RESULTS: It was technically possible to perform the planned operation in all patients. All patients survived and none presented significant postoperative complications. There were no residual gradients in six patients and a gradient of 10 mmHg in one patient postoperatively. One patient showed transient recurrent nerve palsy which recovered within 6 weeks. Follow-up echocardiographic and MRI studies revealed a normal appearing aortic arch with laminar flow. CONCLUSIONS: Although more demanding, an anatomical reconstruction of the aortic arch can be performed in infants and children with recurrent obstruction of the aortic arch with excellent initial results. This approach may prove superior to an extra-anatomic bypass in the long-term.     

Results with the freestyle porcine aortic root for right ventricular outflow tract reconstruction in children

BACKGROUND: The ideal choice for valved reconstruction of the right ventricular outflow tract (RVOT) in children is undetermined. This study explores the Freestyle porcine aortic root for these patients. METHODS: From January 1998 to December 2002, 56 patients ages 1.6 to 29.9 years old (mean 11.8 years old) underwent RVOT reconstruction using a Freestyle porcine aortic root. The patients averaged 1.9 prior operations (range 0 to 5) for tetralogy of Fallot +/- pulmonary atresia (28 patients), critical pulmonary stenosis (10 patients), Ross procedure (5 patients), pulmonary atresia/intact ventricular septum (4 patients), complete atrioventricular septal defect +/- tetralogy of Fallot (4 patients), and others (5 patients). At time of RVOT reconstruction, 42 patients (75%) had additional procedures including the following: tricuspid or mitral repair (24 patients), pulmonary arterioplasty +/- Glenn (12 patients), ventricular septal defect closure (5 patients), aortic valve replacement (3 patients), placement of a cardioverter/defibrillator or pacemaker (3 patients), and others (8 patients). RESULTS: One patient developed mediastinitis; another was treated for Candida endocarditis (his excised homograft unexpectedly grew Candida). All patients are well on follow-up from 2 to 60 months (mean 30 +/- 20 months) with no deaths. The patient with endocarditis underwent conduit replacement for recurrent pulmonary stenosis 3.5 years postoperatively. Echocardiography revealed mild or no pulmonary insufficiency in 93%. The calculated mean peak systolic RVOT gradient by echocardiography was 19.7 +/- 15.4 mm Hg. CONCLUSIONS: These data demonstrate excellent results with the Freestyle bioprosthesis for RVOT reconstruction in children. This valve may serve as a readily available alternative to homograft valves in RVOT reconstruction, particularly since early insufficiency seems to be less problematic. Questions of long-term durability and significance of echocardiographic stenosis remain unanswered.     

Anatomic and functional aortic valvuloplasty for correction of aortic valve prolapse in ventricular septal defect with aortic insufficiency

We describe a new procedure of aortic valvuloplasty for aortic valve prolapse in ventricular septal defect with aortic insufficiency syndrome. This technique allows an anatomic and functional aortic valve reconstruction that prevents late failure of aortic valve repair and reoperation. Midterm results demonstrate the feasibility and durability of this new procedure.     

Primary repair for aortic arch obstruction associated with ventricular septal defect

Background

The aim of the present study was to evaluate the current outcome and reoperation rate after applying a one-stage correction strategy for interrupted aortic arch (IAA) with ventricular septal defect (VSD) and also for aortic coarctation and hypoplastic aortic arch (CoA-HyAA) with VSD beginning September 1999.

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.     

Mild supravalvular aortic stenosis with left coronary obstruction in a neonate

A premature infant with a large patent ductus arteriosus and mild supravalvular aortic stenosis had evidence of severe myocardial ischemia after ductus ligation. Urgent reoperation was required to relieve severe obstruction of the left coronary orifice and the supravalvular stenosis was repaired. This unusual presentation early in infancy emphasizes that left coronary artery obstruction should be suspected even in the presence of mild supravalvular stenosis.     

Surgery for aortic stenosis in children: a 40-year experience

BACKGROUND: Aortic stenosis (AS) is encountered in approximately 5% of children with heart disease. The indications for surgery and the surgical techniques for AS are well established. This report focuses on the early and long-term outcomes in children with AS over a 40-year period. METHODS: Included in this study were 508 patients ranging in ages from 1 day to 19 years, who were operated on for AS between 1960 and 2002. Eighty-one percent (414 of 508) of the patients had left ventricular outflow tract obstruction (LVOTO) at a single level: 40 supravalvar, 242 valvar (critical AS in 85 neonates and young infants and in 157 older children), and 132 subvalvar. Nineteen percent (94 of 508) of the patients had LVOTO at more than one level. Associated congenital cardiac defects were found in 32% of the patients. RESULTS: The overall hospital mortality rate was 8% (40/508) with neonates with critical AS having the highest mortality (33%). The late mortality was 4% for the entire group. Follow-up was 95% complete. The mean follow-up was 8.5 +/- 7.1 years. In the subgroup with multilevel LVOTO (n = 94), the average intraoperative peak systolic left ventricular-aortic gradient decreased from 80 to 22 mm Hg after repair but increased progressively to 74 +/- 36 mm Hg (p < 0.05) before reintervention was required. One hundred twenty-one patients (24%) underwent 151 reoperations for recurrent or residual LVOTO or aortic regurgitation. Actuarial curves predict a 20-year survival of 88% and 62% freedom from reoperation for all patients with AS. Symptomatic improvement in survivors was excellent (90% New York Heart Association class I). CONCLUSIONS: Surgical relief of LVOTO in infants and children can be accomplished with low mortality and morbidity. Neonates with critical AS have significantly higher mortality and morbidity due to their complex anatomy and their critical presentation that affects outcome. Aortic valvotomy delays valve replacement in a significant percentage of children. The Ross procedure and mechanical aortic valve replacements have had a low mortality and morbidity in our series. Valve replacement will eventually be required in most children presenting with valvar AS and multilevel LVOTO while repair of discrete subaortic stenosis and supravalvar AS may not require reoperation in most patients. Children with LVOTO should have lifetime follow-up.     

Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and children

Background/Purpose: Duodenal obstruction, such as that resulting from atresia or web, routinely has been corrected by laparotomy and duodenoduodenostomy. Until recently, no one has reported on the use of minimally invasive techniques to correct this congenital anomaly. Over the last 6 months we have approached 4 patients, 3 with atresia and one with a web, laparoscopically. Three were newborns, and one was 8 months old. Methods: All procedures were performed with 3-mm instruments and scopes. Results: Operating time in all cases was less then 90 minutes. Visualization was excellent, and there were no intraoperative complications. Feedings were started on postoperative day 5 in all 3 neonates and day 3 in the infant. All 4 were on full feedings after 3 days. Follow-up upper gastrointestinal tests show no evidence of stricture or obstruction. Conclusion: Laparoscopy provides an excellent way to evaluate and treat congenital duodenal obstruction. J Pediatr Surg 37:1088-1089.     

Outcome of aortic valve repair in children with congenital aortic valve insufficiency

OBJECTIVE: Surgical aortic valvotomy has a long history of providing excellent palliation for aortic stenosis in infancy and childhood. The fate of aortic valve repairs for dominant aortic regurgitation in this same age group is considerably less clear. METHODS: From 1990 to 2000, a total of 21 patients underwent aortic valve repair for aortic regurgitation at our institution. Seventeen patients were younger than 17 years at the time of repair (3-17 years, mean 8.1 +/- 3.7 years). Of these 17 children, 6 (35%) had bicuspid valves and 11 (65%) had tricuspid valves. Type of repair varied with valve type, but repair generally consisted of commissure resuspension, partial commissure closure, triangular resection of redundant leaflets, or some combination. RESULTS: There were no deaths. Follow-up ranged from 1 to 11 years (mean 5.3 +/- 2.4 years). At present 3 of 17 (17.6%) have mild aortic regurgitation according to echocardiography and 6 (35.2%) have moderate aortic regurgitation. In 8 of 17 cases (47.1%) the repair clearly failed, requiring reoperation from 0.5 to 73 months after the original operation (mean 18.9 months). Reoperation consisted of 6 Ross procedures and 2 mechanical aortic valve replacements. There were no deaths at the secondary operation. CONCLUSION: Aortic valve repair in children with a dominant feature of aortic insufficiency tended to fail progressively and at a high rate. Leaflet thickening was associated with higher risk of repair failure in this series. The threshold for aortic valve replacement should remain low.     

Use of the aberrant right subclavian artery in complex aortic arch reconstruction

An aberrant right subclavian artery can be used in a variety of ways in complex aortic arch reconstructions. Four patients (3 with interrupted aortic arch and 1 with coarctation) in whom this technique was used are presented.     

Apico-aortic conduits in children with severe left ventricular outflow tract obstruction

BACKGROUND: Infrequently, congenital heart defects are complicated by left ventricular outflow tract obstruction (LVOTO) not amenable to conventional reconstruction. Apico-aortic conduits provide a means of palliating such patients until definitive repair is possible. The purpose of this study was to review a single institution's current experience with apico-aortic conduits. METHODS: The medical records of pediatric patients receiving apico-aortic conduits were reviewed. Demographics, operative techniques, preoperative and postoperative physiologic variables, morbidity, mortality, and functional class were recorded. Off-pump and on-pump procedures were categorized for comparison. RESULTS: Ten cases of apico-aortic conduits for left ventricular outflow tract obstruction were identified. Indications included congenital aortic stenosis, aortic atresia, and subaortic stenosis. Six procedures were performed off-pump and four required median sternotomy with cardiopulmonary bypass as necessitated by concomitant procedures. There was one operative death. The remaining patients demonstrated hemodynamic improvements and are all alive to date. One patient required conduit valve replacement. All patients are in New York Heart Association classification I or II at the time of last follow-up. CONCLUSIONS: Apico-aortic conduits provide a safe and effective treatment alternative for select cases of left ventricular outflow tract obstruction. Off-pump techniques are feasible in the majority of cases. This valuable adjunct should be considered whenever conventional repair of left ventricular outflow tract obstruction is considered prohibitive.     

Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children

BACKGROUND: We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. METHODS: Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 +/- 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 +/- 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. RESULTS: Mean pressure gradient decreased significantly more in the surgical group (from 64.8 +/- 30.8 mm Hg to 12.8 +/- 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 +/- 21.4 mm Hg to 21.5 +/- 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV (p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty (p < 0.04). CONCLUSIONS: Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.