Outcomes of the arterial switch for transposition during infancy using a standardized approach over 30 years.

OBJECTIVES: The aim of this study was to describe the early and late outcomes of the arterial switch for transposition. METHODS: A single-centre retrospective cohort study was conducted to assess the early and late outcomes of arterial switch performed during infancy using a standardized institutional approach between 1988 and 2018, compared by morphological groups. RESULTS: A total of 749 consecutive patients undergoing arterial switch during infancy were included, 464 (61.9%) with intact septum, 163 (21.8%) with isolated ventricular septal defect and 122 (16.3%) with complex transposition with associated lesions, including 67 (8.9%) with Taussig-Bing anomaly. There were 34 early deaths [4.5%, 95% confidence interval (CI) 3.1-6.1] with only 10 (2.6%) early deaths since 2000. Complex morphology (odds ratio 11.44, 95% CI 4.76-27.43) and intramural coronary artery (odds ratio 5.17, 95% CI 1.61-15.91) were identified as the most important risk factors for 90-day mortality. Overall survival was 92.7% (95% CI 90.8-94.6) at 5 years and 91.9% (95% CI 89.9-94.1) at 20 years; in hospital survivors, there were 15 (2.1%) late deaths during a median follow-up of 13.7 years. Cumulative incidence of surgical or catheter reintervention was 16.0% (95% CI 14.5-17.5) at 5 years and 22.7% (95% CI 21.0-24.0) at 20 years; early and late reinterventions were more common in the complex group, with no difference between the other groups. CONCLUSIONS: Using a standardized approach, the arterial switch can be performed with low early mortality, moderate rates of reintervention and excellent long-term survival. Concomitant lesions were the most important risk factor for early death and were associated with increased risk of late reintervention.

Four right ventricle to pulmonary artery conduit types.

OBJECTIVE: The most durable valved right ventricle to pulmonary artery conduit for the repair of congenital heart defects in patients of different ages, sizes, and anatomic substrate remains uncertain. METHODS: We performed a retrospective analysis of 4 common right ventricle to pulmonary artery conduits used in a single institution over 30 years, using univariable and multivariable models of time-to-failure to analyze freedom from conduit dysfunction, reintervention, and replacement. RESULTS: Between 1988 and 2018, 959 right ventricle to pulmonary artery conduits were implanted: 333 aortic homografts, 227 pulmonary homografts, 227 composite porcine valve conduits, and 172 bovine jugular vein conduits. Patients weighed 1.6 to 98.3 kg (median 15.3 kg), and median duration of follow-up was 11.4 years, with 505 (52.2%) conduits developing dysfunction, 165 (17.2%) requiring catheter intervention, and 415 (43.2%) being replaced. Greater patient weight, conduit z-score, type and position, as well as catheter intervention were predictors of freedom from replacement. Multivariable analysis demonstrated inferior durability for smaller composite porcine valve conduits, with excellent durability for larger diameter conduits of the same type. Bovine jugular vein conduit longevity was inferior to that of homografts in all but the smallest patients. Freedom from dysfunction at 8 years was 60.7% for aortic homografts, 72% for pulmonary homografts, 51.2% for composite porcine valve conduits, and 41.3% for bovine jugular vein conduits. Judicious oversizing of the conduit improved conduit durability in all patients, but to the greatest extent in patients weighing 5 to 20 kg. CONCLUSIONS: Pulmonary and aortic homografts had greater durability than xenograft conduits, particularly in patients weighing 5 to 20 kg. Judicious oversizing was the most significant surgeon-modifiable factor affecting conduit longevity.

Anatomical Studies of Transposition - An Argument for a Unifying Morphological Classification.

In the setting of transposition, recognition of the variability in the morphology of the outflow tracts and the arterial trunks is essential for surgical repair. Presence of a unifying system for classification would minimize the risk of misunderstanding. We examined an archive of 142 unrepaired hearts with transposition, defined as the combination of concordant atrioventricular and discordant ventriculoarterial connections, combined with the clinical records of 727 patients undergoing the arterial switch procedures. In the setting of usual atrial arrangement, we found the aortic root in leftward or posterior location in up to 5% of our cohorts, making the common term "d-transposition" ambiguous. Variability in the relationship of the trunks was commoner in the setting of deficient ventricular septation, especially when the trunks were side-by-side (14% vs 5.5% when the ventricular septum was intact). Bilateral infundibulums were present in 14% of cases, and bilaterally deficient infundibulums in 3.5%. Both of these findings were more common with deficient ventricular septation. Fibrous continuity between the tricuspid and pulmonary valves was not always seen with perimembranous defects, particularly when there were bilateral infundibulums. Fibrous continuity between the leaflets of the tricuspid and mitral valves, in contrast, proved a unifying characteristic of the perimembranous defect. The combination of concordant atrioventricular and discordant ventriculoarterial connections is best described simply as "transposition," with precision provided when details are given of atrial arrangement and the relationships of the arterial trunks.

Strategies to Minimise Need for Prosthetic Aortic Valve Replacement in Congenital Aortic Stenosis-Value of the Ross Procedure.

To examine the role and outcomes of all interventions for aortic stenosis in children, with focus on freedom from reintervention and the aim to minimise prosthetic aortic valve replacement (pAVR) during childhood. Retrospective analysis of 194 consecutive children who underwent any aortic valve intervention for a biventricular repair strategy at a single institution between 1995 and 2017. Data were obtained from hospital records and follow-up was 100% complete. Over a 22-year period, 194 children underwent total 313 aortic valve procedures: Primary interventions were surgical valvotomy (SV)/surgical repair (SR) in 94 (48.5%), balloon valvuloplasty (BV) in 60 (30.9%), pAVR in 8 (4.1%) and Ross/Ross-Konno procedure in 32 (16.5%). Median age at first intervention was 1.1 years (interquartile range [IQR] 0.1-9.4) and varied with type of intervention: SV/SR were most common in neonates (33, 75%) and infants (35, 68%), whilst BV was most frequent in older children (42, 42%). Operative survival was 99% (2 early deaths, both in neonates with critical aortic stenosis and poor left ventricular function) and 15-year survival was 95%. A Ross procedure was performed in 79 (40.7%) patients over the 15-year study period, 1 of whom required late pAVR for autograft failure. Freedom from any reintervention after SV/SR and BV was 41% and 40% at 10 years, compared to 90% at 10 years with the Ross procedure (P < 0.001). Among neonatal SV/SR and BV, 98% required reintervention during childhood with no difference between groups. Valve morphology did not influence freedom from ultimate valve replacement. In patients who went on to have a Ross procedure, median time from initial intervention to Ross was 2.8 years (IQR 0.1-11.9) in neonates and 6.0 years (IQR 3.1-7.5) in all other age groups. Overall freedom from pAVR was 97% at 10 years and was similar in the SV/SR and BV groups. A strategy of simple valve repair and primary Ross procedure provides excellent survival and good freedom from pAVR. However, reintervention rates after simple interventions for congenital AS are high, especially in younger age groups. The Ross procedure offers the best freedom from reintervention of any technique and wider use of primary Ross in younger age groups should be considered.

Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†.

OBJECTIVES: We sought to define the early and late outcomes of unifocalization based on a classification of the native pulmonary artery (nPA) system and major aortopulmonary collateral arteries (MAPCAs) with a policy of combined recruitment and rehabilitation and to analyse the role of unifocalization by leaving the ventricular septal defect (VSD) open with a limiting right ventricle-pulmonary artery (RV-PA) conduit in borderline cases. METHODS: An analysis of 271 consecutive patients assessed for unifocalization at a single institution between 1988 and 2016 was performed. Patients were classified according to the pulmonary blood supply: group A, unifocalization based on nPA only; group B, based on nPA and MAPCAs; group C, MAPCAs only (absent nPAs). RESULTS: Unifocalization was achieved in 249 (91.9%) cases with an early mortality of 2.8%. Group A included 72 (28.9%) patients, group B 119 (47.8%) patients and group C 58 (23.3%) patients with no difference in early survival between groups. Survival at 5, 10 and 15 years was 90.0% (85.9-94.3), 87.2% (83.5-91.2) and 82.3% (75.2-89.9), respectively. Late survival in groups A and B was similar but 10- and 15-year survival in group C decreased to 79.2% (68.2-92.1) and 74.3% (61.1-90.4) (P = 0.02), respectively. A mean of 1.9 (±0.6) MAPCAs were recruited per patient (range 0-6). The VSD was left open with a limiting RV-PA conduit in 97 (39.0%) cases, but subsequently closed in 48 patients, giving a total of 200 (80.3%) patients achieving VSD closure (full repair). Delaying VSD closure was not associated with increased risk for early or late survival. A central shunt to rehabilitate the nPAs was used in 56 (22.5%) cases. This was associated with a reduction in the number of MAPCAs recruited, but still required a mean of 1.8 (±0.5) MAPCAs recruited per patient to achieve unifocalization. In multivariate risk analysis, those suitable for single-stage full repair had the best long-term outcomes. Group C anatomy was associated with poor late survival compared to groups A and B (hazard ratio 2.7). CONCLUSIONS: Survival is maximized by a combined approach of rehabilitation and recruitment. MAPCAs should always be recruited if they supply areas with absent nPA supply. A strategy of leaving the VSD open with a limiting RV-PA conduit is a safe and effective way of managing borderline cases.

Trends in surgical and catheter interventions for isolated congenital shunt lesions in the UK and Ireland.

OBJECTIVE: To evaluate time trends in the use of catheter and surgical procedures, and associated survival in isolated congenital shunt lesions. METHODS: Nationwide, retrospective observational study of the UK National Congenital Heart Disease Audit database from 2000 to 2016. Patients undergoing surgical or catheter procedures for atrial septal defect (including sinus venosus defect), patent foramen ovale, ventricular septal defect and patent arterial duct were included. Temporal changes in the frequency of procedures, and survival at 30 days and 1 year were determined. RESULTS: 40 911 procedures were performed, 16 604 surgical operations and 24 307 catheter-based interventions. Transcatheter procedures increased over time, overtaking surgical repair in 2003-2004, while the number of operations remained stable. Trends in interventions differed according to defect type and patient age. Catheter closure of atrial septal defects is now more common in children and adults, although surgical interventions have also increased. Patent foramen ovale closure in adults peaked in 2009-2010 before falling significantly since. Surgery remains the mainstay for ventricular septal defect in infants and children. Duct ligation is most common in neonates and infants, while transcatheter intervention is predominant in older children. Excluding duct ligation, survival following surgery was 99.4% and ≈98.7%, and following catheter interventions was 99.7% and ≈99.2%, at 30 days and 1 year, respectively. CONCLUSIONS: Trends in catheter and surgical techniques for isolated congenital shunt lesions plot the evolution of the specialty over the last 16 years, reflecting changes in clinical guidelines, technology, expertise and reimbursement, with distinct patterns according to lesion and patient age.

Tetralogy of Fallot: morphological variations and implications for surgical repair.

OBJECTIVES: Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management. METHODS: The Birmingham Children's Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process. RESULTS: Anomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants. CONCLUSIONS: Anatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.

Fate of the Left Ventricular Outflow Tract After Rastelli With Selective Infundibular Muscle Resection.

BACKGROUND: The Rastelli procedure has been criticized for a high rate of left ventricular outflow tract obstruction (LVOTO) and translocation procedures have been proposed as an alternative. Infundibular resection can be performed during Rastelli to optimize the outflow tract. This study examines whether a selective policy to enlarge the outflow tract improves Rastelli outcomes across all morphological variants. METHODS: Single institution 29-year experience in 105 patients. Patients were classified into transposition of the great arteries with ventricular septal defect, congenitally corrected transposition of the great arteries with ventricular septal defect, and double outlet right ventricle morphology groups. The infundibular muscle was routinely resected if prominent, in 28 cases. RESULTS: Early mortality was 1.9% (2 of 105) and actuarial survival was 95.4% (95% confidence interval [CI], 89% to 99%) at 1 year, 92.9% (95% CI, 85% to 97%) at 5 years, and 84.5% (95% CI, 74% to 92%) at 10 years. The cumulative freedom from LVOTO was 99% (95% CI, 96% to 100%), 97% (95% CI, 92% to 99%), and 90% (95% CI, 88% to 96%) at 1, 5, and 10 years, respectively. The incidence was similar in all morphological groups and those undergoing infundibular resection were not at higher risk of late LVOTO. Eleven patients required surgical reoperation on the left ventricular outflow tract over a median follow-up period of 8.5 years, with no mortality, although 2 of these patients developed complete heart block. Left ventricular function was well preserved in 98.1% of all cases, including all of those requiring left ventricular outflow tract reoperation. CONCLUSIONS: The Rastelli is a safe procedure that can be applied in a variety of morphological variants. LVOTO remains a late complication of Rastelli, but can be minimized by concomitant infundibular muscle resection. Late reoperation is safe and ventricular function is well preserved in greater than 95% of cases at late follow-up. The operation has stood the test of time and avoids many of the risks of translocation procedures.

Long-Term Outcome of Interrupted Arch Repair With Direct Anastomosis and Homograft Augmentation Patch.

BACKGROUND: This study analyzed outcomes of interrupted aortic arch (IAA) repair using a standardized technique to interpret the role of the arch repair on late outcomes in a complex and heterogeneous group of patients. METHODS: This single institution study covered the period from 1988 to 2015. A total of 120 cases of IAA were divided into four groups: IAA with ventricular septal defect (VSD) (n = 38), IAA with a Norwood or Damus-Kaye-Stansel procedure (n = 41), IAA with truncus arteriosus (n = 24), and a miscellaneous group (n = 17). Arch repair was performed using a standard technique of direct anastomosis with homograft patch augmentation. RESULTS: IAAs were predominantly type B (n = 81, 68%), and type A (n = 34, 28%), with a significant association of type B with truncus arteriosus and of type A with an aortopulmonary window (p < 0.01). Survival was similar in all groups. The incidence of catheter or surgical reintervention was 18% (confidence interval [CI], 10% to 25%) at 5 years and 18% (CI, 10% to 25%) at 10 years, with catheter reintervention more common and occurring before 18 months. Surgical reintervention occurred in 7% (CI, 2% to 13%) at 5 and 10 years and at 10 years the reintervention rate was lower in the group with truncus arteriosus (0%) and in the group with a Norwood or Damus-Kaye-Stansel procedure (5%). There was no bronchial obstruction or aortic aneurysm. The Cox proportional hazard model showed that weight at surgery <2.5 kg and era of surgery were predictive of outcome, with surgical mortality rates in all variants dropping to 8.3% in the last 15 years of the study. CONCLUSIONS: Repair of IAA using direct anastomosis and patch augmentation is applicable to all variants and provides good long-term arch patency. Survival is strongly associated with weight at surgery.

The importance of age and weight on cavopulmonary shunt (stage II) outcomes after the Norwood procedure: Planned versus unplanned surgery.

OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit. Unplanned surgery was any situation in which surgery was expedited because of refractory desaturation, angiographic findings, or failure to discharge. RESULTS: There were 222 planned and 75 unplanned procedures. Planned surgery was performed at median of 5.4 (4.6-6.3) months compared with 3.9 (3.3-5.0) months for the unplanned group (P < .001). Early mortality was 1.3% in the planned group compared with 5.3% in the unplanned group (P = .07). At 5 and 10 years, survival was significantly worse in the unplanned group (73% vs 85%, P = .03). On multivariable analysis, atrioventricular valve regurgitation, the need for atrioventricular valve repair, and impaired ventricular function (graded composite variable) were the predominant risk factors (hazard ratio [HR], 7.49), with an incremental risk within these 3 variables. The planned versus unplanned nature of the surgery was less significant (HR, 1.75) because it was eclipsed by ventricular function and tricuspid intervention. Additional factors were aortic atresia/mitral atresia subgroup (HR, 2.07) and somatic growth (z-score weight) at the time of surgery (HR, 0.60 for z-score change from -2.54 to -1.16). Age and weight at surgery were not significant risk factors for survival. Interstage interventions on the aortic arch or right ventricle to pulmonary artery conduit did not adversely influence outcomes. CONCLUSIONS: The ventricular function, atrioventricular valve regurgitation, and need for tricuspid intervention were the primary risk factors for survival after the cavopulmonary shunt operation. Although unplanned surgery is associated with poor outcomes, this is related to the primary risk factors and not the timing or age of the patient. Somatic growth also has a significant influence on survival. Thus, an earlier cavopulmonary shunt operation is safe in infants who are thriving, but those with poor weight gain are at higher risk.

Atresia valvular aórtica o hipoplasia grave y comunicación interventricular: estrategias de corrección biventricular y resultados a medio plazo / Aortic valve atresia or severe hypoplasia and ventricular septal defect: surgical strategies for biventricular repair and mid-term results

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Holes and channels between the ventricles revisited.

BACKGROUND: Although holes, or channels, between the ventricles are the commonest congenital cardiac malformations, there is still no consensus as to how they can best be described and categorised. So as to assess whether it is possible to produce a potentially universally acceptable system, we have analysed the hearts categorised as having ventricular septal defects in a large archive held at Birmingham Children's Hospital. Materials and methods We analysed all the hearts categorised as having isolated ventricular septal defects, or those associated with aortic coarctation or interruption in the setting of concordant ventriculo-arterial connections, in the archive of autopsied hearts held at Birmingham Children's Hospital, United Kingdom. RESULTS: We found 147 hearts within the archive fulfilling our criterions for inclusion. All could be classified within one of three groups depending on their borders as seen from the right ventricle. To provide full description, however, it was also necessary to take account of the way the defects opened to the right ventricle, and the presence or absence of alignment between the septal components. CONCLUSIONS: By combining information on the phenotypic specificity defined on the basis of their borders, the direction of opening into the right ventricle, and the presence or absence of septal malalignment, it proved possible to categorise all hearts examined within the archive of Birmingham Children's Hospital. Our findings have necessitated creation of new numbers within the European Paediatric Cardiac Code.

Long-term fate of the aortic valve after an arterial switch operation.

OBJECTIVE: To evaluate long-term performance of the aortic valve after an arterial switch operation (ASO), in terms of regurgitation and reoperation. METHODS: A longitudinal analysis of patients who underwent an ASO between 1988 and 1998 at the Birmingham Children's Hospital. Any type of aortic valve surgery after ASO was censored. To determine progression of aortic valve regurgitation, cardiology follow-up and echocardiograms from the operation until 2012 were reviewed. RESULTS: A total of 362 patients underwent ASO; median age was 8 days. Ventricular septal defect was present in 151, aortic coarctation in 39, and interrupted aortic arch in 6 patients. The trap door technique was used for coronary translocation when possible; 4.1% had intramural coronaries. There were 331 alive patients; follow-up was complete at 93%; median duration was 16 years (interquartile range, 12-18.2 years). Overall survival at 10 and 20 years was 97.6% and 95.2%, respectively. Aortic valve reoperation occurred in 5 patients, giving freedom from aortic surgery of 99.3% and 97.7% at 10 and 20 years, respectively. Reoperations were aortic valve replacement (3), Bentall operation (1), and aortic valve repair (1). Freedom from ≥moderate regurgitation at 10 with 20 years was 97% to 80%, respectively. Degree of regurgitation immediately after ASO was strongly predictive of late aortic valve function: patients discharged with mild regurgitation had a significantly increased risk of progression compared with those with no regurgitation (20 years of freedom from ≥moderate regurgitation, 50% vs 96%, P = .0000). CONCLUSIONS: Reoperation on the aortic valve is rarely necessary, even late after an ASO, but a significant number of patients do have late aortic regurgitation and continue to need observation.

Twenty-year outcome of anomalous origin of left coronary artery from pulmonary artery: management of mitral regurgitation.

BACKGROUND: This study is a single-center experience with surgical repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with focus on the management of associated mitral regurgitation (MR). METHODS: We performed a retrospective analysis of cases presenting to a quaternary referral center between November 1990 and October 2011. RESULTS: In all, 25 patients (18 female) presented with a diagnosis of ALCAPA at a median age of 5 months (range, 1.5 to 102). Twenty-one patients (84%) had moderate to severe impairment of left ventricular function with median fractional shortening of 14% (range, 2% to 33%), and 19 patients (76%) had moderate to severe MR. Surgery was performed with direct coronary reimplantation in 16 patients (64%) and intrapulmonary tunnel (Takeuchi repair) in 9 (36%). Four patients had mitral valve repair at time of surgery, all for structural anomalies. Functional MR with a structurally normal mitral valve was not repaired. The median duration of postoperative follow-up was 93 months (range, 9 to 240). There were no early or late deaths, and no patient required mechanical support. Four patients (16%) required surgical or catheter reintervention. At last follow-up, 24 of 25 patients were asymptomatic; the left ventricular function was normal in 22 patients. Moderate MR was present in 4 patients. There was significant improvement in left ventricular function and MR (p < 0.01) during follow-up. CONCLUSIONS: Surgical repair of ALCAPA has good long-term results with low mortality and reintervention rates. The majority of MR is functional and will improve with reperfusion, but structural mitral valve abnormalities should be repaired at the time of surgery.

Comparison of the Ross/Ross-Konno aortic root in children before and after the age of 18 months.

OBJECTIVES: Evaluation of long-term results after the Ross/Ross-Konno operation in patients <18 months of age, focusing on pulmonary autograft performance. METHODS: Retrospective analysis of patients who underwent the Ross/Ross-Konno operation (1991-2011). Data were obtained from hospital records and follow-up was 100% complete. RESULTS: Between January 1991 and December 2011, 140 patients underwent the Ross/Ross-Konno operation and 22 patients were <18 months of age (male/female: 15/7). The median age was 166 days. 14 patients had a Ross operation and 8 patients a Ross-Konno operation. Presentation at surgery was aortic valve stenosis in 13, regurgitation in 7 and mixed disease in 2. Only 4 patients (18%) had no surgery prior to Ross/Ross-Konno, and among the others the previous most frequent operation was aortic valvotomy (55%). There were 3 early deaths, all in high-risk cases with poor preoperative left ventricular function. At discharge there was no neoaortic regurgitation in 10, trivial in 4 and mild in 5. The median follow-up is 10.8 years (range 0.96-21). There was 1 late death due to progressive ventricular dysfunction 2.4 years after Ross-Konno and mitral valve replacement. Survival for patients <18 months was 81% at 5-10 years (18 patients) and for older patients was 98.2% at 5-10 years. There was no neoaortic regurgitation in 8 patients, mild in another 8 and moderate in 2 with a freedom from moderate regurgitation significantly lower in comparison with older patients (100-80% vs 83.5-73.4% at 5-10 years). Freedom from aortic reoperation for patients <18 months was significantly higher compared with older (100 vs 95.4-84.4% at 5-10 years, P < 0.04). Reoperation in the RVOT was higher in younger patients compared with the other (85-64.6% vs 97.2 and 84.7% at 5-10 years P = 0.02). Z-score aortic root diameter remained constant in the <18-months group compared with significant dilatation in the >18-months patients (P < 0.01). CONCLUSIONS: Ross/Ross-Konno can be invaluable in the younger age group but not without risk in the setting of ventricular dysfunction. Long-term performance of the neoaortic valve is significantly better than in older children, which is related to maintenance of normal root dimensions compared with progressive dilatation in patients undergoing Ross/Ross-Konno at older ages.

Early double switch with Lecompte maneuver for life-threatening airway obstruction.

A 6-month-old boy with congenitally corrected transposition of the great arteries, ventricular septal defect, and mild pulmonary valve stenosis presented with acute obstructive respiratory life-threatening events. Airways obstruction was caused by aneurysmal dilation of the branch pulmonary arteries compressing the carina and bronchi. The child was ventilator dependent. A double switch operation with Lecompte maneuver was performed, bringing the pulmonary arteries forward and relieving the airway compression. The child made a complete recovery and remains well 9 months later with no clinical or radiologic signs of airway compression.

Surgery following primary right ventricular outflow tract stenting for Fallot's tetralogy and variants: rehabilitation of small pulmonary arteries.

OBJECTIVES: Primary surgical repair of Tetralogy of Fallot (ToF) in small infants with small pulmonary arteries (PAs) or complex anatomies can be hazardous. We assessed the effect of right ventricular outflow tract (RVOT) stenting on subsequent surgical intervention with attention to growth of the PAs. METHODS: Primary RVOT stenting was performed in 32 symptomatic patients with ToF physiology. Twenty patients had surgical intervention, with 15 undergoing complete repair to date. Median age at stenting was 61 (range 8-406) days, and median weight, 3.9 (range 1.8-12.2) kg. RESULTS: Stenting improved saturations from 72 ± 8 to 92 ± 2% (P < 0.001). Four patients required early surgical palliation for persistent desaturation (within 4 weeks). Twenty patients went on to have surgical intervention at a median time of 220 days after stenting. There was no operative mortality after complete repair. Removing the stent lengthened the procedure time and 86% required transannular patch (TAP; bypass time 109 ± 42 min, cross clamp 68 ± 29 min). Median intensive therapy unit stay was 2 days. There was 1 late death at 3 months due to chronic lung disease. The median left PA Z-score increased from a preinterventional value of -1.27 (-0.19 to -2.87) to a presurgical value of +0.11 (-4.12 to +1.97). The median right PA Z-score increased from -2.02 (-1.77 to -4.68) to -0.65 (-0.29 to -2.04) over the preinterventional and presurgical time intervals. Growth was greatest in the right PA. CONCLUSIONS: Primary RVOT stenting facilitates staged palliation for ToF in small infants and complex anatomies. Improved PA blood flow generated by the stent leads to growth of the branch PAs and may improve the substrate for subsequent surgical repair. Surgery is safe; however, the majority will require a TAP.