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There has been a rapid increase in the practice of interventional catheter treatment of congenital heart disease. Catheter retrieval of embolized cardiac devices and other foreign bodies is essential, yet no large studies have been reported in the paediatric population. Retrospective 15-year review of all children who underwent transcatheter foreign body retrieval in a tertiary cardiac centre from January 1997 to September 2012. Transcatheter retrieval of foreign bodies from the cardiovascular system was attempted in 78 patients [median age 4 (0.02-16) years and median weight 15 (1.7-74) kg] including 46 embolized devices. Transcatheter retrieval was successful in 70/78 (90%), surgical retrieval was required in 6. In two patients, small embolized coils were left in situ. Gooseneck snare was the most commonly used retrieval device. Median procedure and screening times were 90 (15-316) and 31 (2-161) min, respectively. There were no procedural deaths. Transient loss of foot pulses occurred in 5 and 2 patients required blood transfusion. Transcatheter retrieval of cardiovascular foreign bodies can be performed safely in the majority of children thus obviating the need for surgery. It is essential to have a comprehensive inventory of retrieval equipment and interventional staff conversant with its use.

Aims In patients with an extra-cardiac Fontan circulation, there is no direct access to the heart. The insertion of a permanent pacemaker requires surgery to insert epicardial pacing wires. We present the implantation of a permanent endocardial pacing lead from the superior vena cava (SVC) into the atrium via direct passage from the right pulmonary artery (RPA). Methods and results A permanent pacing lead was passed directly from the SVC to the RPA and then into the atrial mass. Direct passage from the RPA (attached directly to the right SVC) into the atrial mass was achieved using a trans-septal puncture needle. Conclusion This novel technique is an alternative to epicardial pacing in patients with an extra-cardiac Fontan circulation, thus avoiding the need for surgical intervention. It may also be applied to gain access to the atrial mass for arrhythmia ablation therapy.

Objectives This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing

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 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.

To assess the indication, technical aspects, and outcome of stenting of the right ventricular outflow tract (RVOT) in the management of symptomatic patients with severely limited pulmonary blood flow.Retrospective case note and procedure review of patients undergoing stenting of the RVOT over an 8 year period.Between 2005 and 2012, 52 selected patients underwent percutaneous stent implantation into a very narrow RVOT to improve pulmonary blood flow. Median age at stent implantation was 63 (range 4-406) days and median weight was 3.8 (1.7-12.2) kg.52 patients underwent stent implantation. Median procedure time was 57 (24-260) min and fluoroscopy time 16 (5.5-73) min. There was one procedural death (1.9%) and one emergency surgery (1.9%). Saturations increased from 71% (52-83%) to 92% (81-100%) (p0.001). Two patients required early shunts due to inadequate palliation and two died from non-cardiac causes. Sixteen further catheter interventions were undertaken (balloon in 7, further stent in 9). Twenty-nine patients underwent delayed surgery (complete repair in 26, palliative in 3) at a median of 172 (52-758) days post-stenting. Left pulmonary artery Z score increased from a pre-interventional value of -1.75 (-4.96 to 0.67) to a pre-surgical value of -0.55 (-4.12 to 1.97), (p0.01). Median right pulmonary artery Z score increased from -2.63 (-7.70 to 0.89) to -0.75 (-6.69 to 1.18) (p0.01) . Seventeen patients remain well palliated after a median of 122 (40-286) days.Stenting of the RVOT is an effective treatment option in the initial management of selected patients with very reduced pulmonary blood flow.

ObjectivesThe aim of this study was to assess the efficacy of radiofrequency catheter ablation (RFCA) in the treatment of hypertrophic obstructive cardiomyopathy in children.BackgroundHypertrophic obstructive cardiomyopathy is an uncommon cause of left ventricular outflow tract obstruction in children. In symptomatic patients, open heart surgical myectomy has hitherto been the only therapeutic option.MethodsIn 32 children, at a median age of 11.1 (range 2.9 to 17.5) years and weight of 31 (15 to 68) kg, ablation of the hypertrophied septum was performed using a cool-tip ablation catheter via a femoral arterial approach. The median number of lesions was 27 (10 to 63) and fluoroscopic time was 24 (12 to 60) min.ResultsThe majority of patients demonstrated an immediate decrease in the catheter pullback gradient (mean 78.5 ± 26.2 mm Hg pre-RFCA versus mean 36.1 ± 16.5 mm Hg post-RFCA, p < 0.01) and a further reduction in the Doppler echocardiographic gradient (mean 96.9 ± 27.0 mm Hg pre-RFCA versus 32.7 ± 27.1 mm Hg post-RFCA, p < 0.01) at follow-up. One patient died due to a paradoxical increase in left ventricular outflow tract obstruction, and another had persistent atrioventricular block that required permanent pacing. Six patients required further procedures (surgery, pacing, or further RFCA) during a median follow-up of 48 (3 to 144) months.ConclusionsThe preliminary results of RFCA for septal reduction in children with hypertrophic cardiomyopathy are promising and merit further evaluation.

Objective: To analyze the safety and clinical impact of interventional cardiac catheter procedures in the management of early postoperative problems after completion of an extracardiac Fontan procedure. Background: The mortality after Fontan procedure has consistently decreased over the last decade. The role of interventional catheterization to address early postoperative problems in this setting has not been studied systematically. Methods: Over a 9.7-year period, 289 patients underwent an extracardiac fenestrated Fontan procedure with two early deaths (0.7%) and takedown in four (1.4%). Twenty-seven patients (9.3%) underwent 32 interventional cardiac catheter procedures at a median interval of 12.2 (1–30) days. The median weight was 14.5 (13.5–25) kg. The case notes and procedure records were reviewed retrospectively. Results: Fontan pathway obstructions were treated in 11 patients with stent implantation with good results and no complications. Stent fenestration of the Fontan circulation was performed in 16 patients with one episode of transient hemiparesis and one episode of pericardial effusion. Three patients underwent initial balloon dilatation of branch pulmonary arteries or fenestration with little effect and underwent stent treatment 6 (5–9) days later. One patient had device closure of a large atrial fenestration. In one patient, residual anterograde pulmonary blood flow was occluded using a device. There were no deaths and in-hospital course was improved in all. Conclusion: Interventional cardiac catheter procedures can be performed safely and effectively in the early postoperative period after Fontan completion to address hemodynamic problems. These techniques contribute significantly to achieve a very low mortality and address morbidity after Fontan completion. © 2010 Wiley-Liss, Inc.

Introduction: Infants and children with congenital aortic stenosis and coarctation of the aorta can be treated by catheter intervention. There are several pharmacological and mechanical techniques described to overcome the balloon movement; none, however, have proved entirely satisfactory. An alternative method to achieve balloon stability is the use of rapid ventricular pacing. We describe our experience with titrating the pacing rate and the use of this technique. Methods: A retrospective review of database was performed, to identify patients who underwent transcatheter intervention with rapid ventricular pacing. Invasive systemic pressures were documented with a catheter in the aorta. Rapid ventricular pacing was initiated at the rate of 180 per minute and increased by increments of 20 per minute to a rate required to achieve a drop in systemic pressure by 50% and a drop in pulse pressure by 25%. The balloon was inflated only after the desired pacing rate was reached. Pacing was continued until the balloon was completely deflated. Results: Thirty patients were identified, 29 of whom had interventions with rapid ventricular pacing. Balloon valvuloplasty of aortic valve was performed on 25 patients while 4 patients had stenting for coarctation by this technique. The rate of ventricular pacing required ranged from 200 to 260 per minute with a median rate of 240. Balloon stability at the time of intervention was achieved in 27 patients. Conclusion: Rapid ventricular pacing is a safe and effective method to provide transient decrease in cardiac output at the time of transcatheter interventions to achieve balloon stability. (J Interven Cardiol 2010;23:7–13)

Percutaneous techniques are being increasingly used in adult congenital heart disease but there is limited experience in the treatment of native nonvalvar right ventricular outflow tract obstruction. We describe two cases of percutaneous stenting of the subpulmonary region where surgery was not an option.

Background— Patent foramen ovale (PFO) is prevalent in patients with migraine with aura. Observational studies show that PFO closure resulted in migraine cessation or improvement in ≈80% of such patients. We investigated the effects of PFO closure for migraine in a randomized, double-blind, sham-controlled trial. Methods and Results— Patients who suffered from migraine with aura, experienced frequent migraine attacks, had previously failed ≥2 classes of prophylactic treatments, and had moderate or large right-to-left shunts consistent with the presence of a PFO were randomized to transcatheter PFO closure with the STARFlex implant or to a sham procedure. Patients were followed up for 6 months. The primary efficacy end point was cessation of migraine headache 91 to 180 days after the procedure. In total, 163 of 432 patients (38%) had right-to-left shunts consistent with a moderate or large PFO. One hundred forty-seven patients were randomized. No significant difference was observed in the primary end point of migraine headache cessation between implant and sham groups (3 of 74 versus 3 of 73, respectively; P =0.51). Secondary end points also were not achieved. On exploratory analysis, excluding 2 outliers, the implant group demonstrated a greater reduction in total migraine headache days ( P =0.027). As expected, the implant arm experienced more procedural serious adverse events. All events were transient. Conclusions— This trial confirmed the high prevalence of right-to-left shunts in patients with migraine with aura. Although no significant effect was found for primary or secondary end points, the exploratory analysis supports further investigation. The robust design of this study has served as the model for larger trials that are currently underway in the United States and Europe.

Objective: This study was undertaken to identify the factors affecting early and late outcome following the Fontan procedure in the current era. We have examined whether conventional selection criteria, the ‘Ten Commandments’, are still applicable in the current era. Materials and methods: Between January 1988 and July 2004, 406 patients underwent a modified Fontan procedure at a median age of 4.7 years (IQR, 3.8—7.1 years). The single functional ventricle was of left (n = 241, 59%) or right ventricular morphology (n = 163, 40%). The modified Fontan procedure was performed using an atriopulmonary connection (n = 162, 40%) or total cavopulmonary connection (TCPC) involving a lateral atrial tunnel (n = 50, 12%) or extracardiac conduit (n = 194, 48%). They were fenestrated in 216 patients (53%). Results: The early mortality was 4.4% (n = 18) and four other patients required takedown of the Fontan circulation. On multivariable analysis, early outcome was adversely influenced by two factors (p < 0.05): preoperative impaired ventricular function and elevated pulmonary artery pressures. Two risk models were constructed for early outcome based on preoperative and predictable operative variables (Model 1) and all preoperative and operative data (Model 2). Both models were calibrated across all deciles (p = 0.83, p = 0.25) and discriminated well. The area under the ROC curve was 0.85 and 0.89, respectively. There were 21 late deaths, 1 patient required late takedown of the Fontan circulation and 3 required orthotopic cardiac transplantation. Actuarial survival was 90 � 2%, 86 � 2% and 82 � 3% at 5, 10 and 15 years, respectively. Multivariable analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. The freedom from reintervention was 83 � 4%, 76 � 4% and 74 � 8% at 5, 10 and 15 years, respectively. Additional risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. Conclusions: Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricularfunction and elevated pulmonaryartery pressures have an adverse influenceon both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions. Methods: Between 1993 and 2004, 226 patients underwent Stage II following NP. Ninety-eight patients (43%) had completion of Fontan circulation (Stage III) and a further 107 (47%) are on course for Fontan completion with 21 (9%) inter-stage deaths. During NP, the aortic arch was reconstructed without additional material (n = 91, 40%) or with a pulmonary homograft patch (n = 135, 60%). Pulmonary blood flow was supplied by modified Blalock-Taussig shunt (n = 177, 78%) or right ventricle to pulmonary artery conduit (RV-PA; n = 49, 22%). The CPAs defect was closed directly (n = 69, 31%) or with a patch (n = 157, 69%). Complete resection of coarctation was performed in 126 patients (56%). Results: Ninety-seven patients (43%) required surgical reoperation to CPAs during Stage II. Actuarial freedom from reoperation was 60 ± 3%, 52 ± 4% and 50 ± 4% at 1, 5 and 10 years, respectively. On multivariable analysis, NP with RV-PA increased risk of reoperation (LR8.3, 5.3-13.2; p < 0.001). Forty-one patients (18%) required catheter-based reintervention on CPAs. Actuarial freedom from reintervention was 98 ± 1%, 72 ± 4% and 58 ± 6% at 1, 5 and 10 years, respectively. CPA problems were almost exclusively limited to the proximal Left pulmonary artery. On multivariable analysis, catheter-based reintervention became more common with time. Complete resection of coarctation increased risk of reintervention (LR 3.9, 1.6-9.6; p < 0.005). Arch reconstruction and CPAs repair techniques did not affect risk of reoperation or reintervention on CPAs. Conclusions: CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP. The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage II procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP.

ObjectiveThe study objective was to identify how the evolution of surgical strategies influenced the outcome after the Norwood procedure.MethodsFrom 1992 to 2004, 367 patients underwent the Norwood procedure (median age, 4 days). Three surgical strategies were identified on the basis of arch reconstruction and source of pulmonary blood flow. The arch was refashioned without extra material in group A (n = 148). The arch was reconstructed with a pulmonary artery homograft patch in groups B (n = 145) and C (n = 74). Pulmonary blood flow was supplied by a modified Blalock-Taussig shunt in groups A and B. Pulmonary blood flow was supplied by a right ventricle to pulmonary artery conduit in group C. Early mortality, actuarial survival, and freedom from arch reintervention or pulmonary artery patch augmentation were analyzed.ResultsEarly mortality was 28% (n = 102). Actuarial survival was 62% ± 3% at 6 months. Early mortality was lower in group C (15%) than group A (31%) or group B (31%; P

Objective: This retrospective study compared the size of the central pulmonary arteries in patients with hypoplastic left heart syndrome (HLHS) following either a classical Norwood or Norwood procedure with a right ventricle to pulmonary artery (RV–PA) conduit. Methods: Between May 2001 and May 2003, 30 patients with HLHS underwent cardiac catheterization prior to stage II palliation. Patients were initially palliated with a classical Norwood (Classical group, nZ18) or Norwood procedure with RV–PA conduit (RV–PA group; nZ12). Indexed maximum and minimum diameters of the LPA and RPA were measured using the McGoon ratio. Cardiac catheterisation was performed at a median age of 4.0 months. There was no difference in the time interval to catheterisation (PZ0.13), Qp:Qs (PZ0.41) or median haemoglobin (PZ0.42) between the groups. Results: The combined PA diameter was larger in the RV–PA group (B) than the classical group (A) (1.99G0.38 versus 1.63G 0.29, P!0.05). There were marked differences in the relative size of the pulmonary arteries between the two groups. In RV–PA patients, the LPA and RPA sizes were comparable (0.99G0.22 versus 1.00G0.31, PZ1.00) whereas, in the classical group, the LPA was smaller than the RPA (0.75G0.15 versus 0.88G0.17, P!0.05). Both techniques were also associated with discrete PA stenoses at the site of shunt insertion. Stenoses were more severe in RV–PA group (RV–PA), causing a 42G16% reduction in the combined PA diameter compared with a 28G18% reduction in Classical group (classical) (P!0.05). Conclusions: The Norwood procedure with RV–PA conduit is associated with better and more evenly distributed central pulmonary artery growth. Nevertheless, it is also associated with central PA stenoses, which may require subsequent reconstruction.

Background— Some patients with a morphological right ventricle (mRV) in the systemic circulation require early intervention because of progressive systemic ventricular dysfunction or atrioventricular valve regurgitation. They may be eligible for anatomic repair (correction of atrioventricular and ventriculoarterial discordance) but require prior training of the morphological left ventricle (mLV). Methods and Results— Forty-one patients with congenitally corrected transposition of the great arteries or a previous atrial switch procedure embarked on a protocol of pulmonary artery (PA) banding with a view to anatomic repair. All had an mRV in the systemic circulation and a subpulmonary mLV that was not conditioned by either volume or pressure load. Two patients were not banded, and 39 were followed up for a median of 4.3 years (range, 25 days to 12.6 years). Sixteen patients achieved anatomic repair, with 3 in the early stages of the training protocol. After 2 years, 12 patients were not suitable for anatomic repair and persisted with palliative banding; 8 were functionally improved; and 4 died, underwent transplantation, or required debanding. PA banding improved functional class but did not improve tricuspid regurgitation in the long term for patients not achieving anatomic repair. mLV function was a critical determinant of survival with a PA band as well as survival after anatomic repair. Patients >16 years were unlikely to achieve anatomic repair. Conclusion— PA banding is a safe and effective method of training the mLV before anatomic repair. It is also an effective palliative procedure for those who do not attain this goal.

Objective Congenital heart defects with major aortopulmonary collateral arteries show marked variability in the size and distribution of native pulmonary arteries. We sought to classify the size and distribution of native pulmonary arteries and to determine their influence on surgical outcome. Methods Between 1989 and 2002, 164 patients underwent surgical intervention for congenital heart defects with major aortopulmonary collateral arteries (median age, 10 months). Three patterns of native pulmonary arteries were identified: intrapericardial native pulmonary arteries present (group I); confluent intrapulmonary native pulmonary arteries without intrapericardial native pulmonary arteries (group II); and nonconfluent intrapulmonary native pulmonary arteries (group III). Thirty-seven (23%) patients had single-stage and 76 (47%) patients had multistage complete repair. Thirty (18%) patients await septation, and 8 (5.0%) patients are not septatable. Follow-up is 98% complete (median follow-up, 5.8 years). Results In the 164 patients there were 15 (9.1%) early and 12 (7.3%) late deaths. Early mortality after complete repair was 4.4% (n = 5). Actuarial survival was 90% ± 3% and 85% ± 4% at 1 and 10 years, respectively. Actuarial freedom from surgical or catheter reintervention in septated patients was 77% ± 4% and 45% ± 8% at 1 and 10 years, respectively. On multivariate analysis, the morphology of the native pulmonary arteries was the only factor that influenced actuarial survival after complete repair ( P = .04). Group III had the highest risk of death after septation ( P = .008). Group II fared better than group III after the initial operation ( P Conclusions Current classifications of congenital heart defects with major aortopulmonary collateral arteries are based on the presence or absence of intrapericardial pulmonary arteries. We have identified a subgroup without intrapericardial native pulmonary arteries but with confluent intrapulmonary native pulmonary arteries. This group has a better outcome than those with nonconfluent intrapulmonary native pulmonary arteries.

Background Surgical valvotomy for critical aortic stenosis in children enables relatively accurate commissurotomies to be fashioned, resulting in the formation of two or three leaflets. We hypothesized that outcomes after surgery may be best in patients in whom three leaflets are produced. Methods A retrospective review of infants undergoing primary surgical valvotomy at our institution during a 12-year period was carried out. Patients who had additional intracardiac defects were excluded. Clinical and echocardiographic follow-up data were analyzed. Results Fifty-four patients fulfilled the study criteria. Median age at surgery was 3 weeks (range, 0 to 51 weeks). Commissurotomy resulted in bileaflet anatomy in 41 patients (group A) and trileaflet anatomy in 13 patients (group B). Operative mortality was 5% in group A and 0% in group B ( p = 1.0). In group A, 18 patients required one or more aortic valve reinterventions, including valve replacement in 8 patients. In group B, there was only one reintervention (repeat valvotomy). Kaplan-Meier analysis showed that at 10 years, comparisons of group A versus group B were as follows: actuarial survival, 85% versus 100% ( p = 0.15); freedom from reintervention, 33% versus 92% ( p = 0.01); freedom from aortic reoperation, 45% versus 92% ( p = 0.04); and freedom from aortic valve replacement, 57% versus 100% ( p = 0.07). Conclusions Long-term outcomes after aortic valvotomy are significantly better in infants in whom surgery results in trileaflet rather than bileaflet anatomy. Preoperative evaluation of valve morphology may enable selection of a group of patients in whom results of surgery are excellent.

Objective: This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology. Methods: Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n ¼ 44, 42%) or right ventricular morphology (n ¼ 59, 58%). The TCPC was performed using an extracardiac conduit (n ¼ 84, 82%) or a lateral atrial tunnel (n ¼ 19, 18%), and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation; early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days – 5.2 years). Results: Early mortality was 1.9% (n ¼ 2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (^ 1 SEM) was 95.6 ^ 2.5%. Forty-two patients (41%) had prolonged pleural drainage ($ 14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (^ 1 SEM) were 92.2 ^ 5.0 and 73.4 ^ 6.0%, respectively. The Fontan procedure was associated with an improved functional class (P , 0:005) and all current survivors (n ¼ 99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P , 0:05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P , 0:05), increased postoperative pulmonary artery pressures (P , 0:005) and an unfenestrated Fontan procedure (P , 0:01). Conclusions: In this contemporary series, the modified Fontan procedure was characterised by low early mortality, excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival. q 2003 Elsevier Science B.V. All rights reserved.

Background The incidence of coarctation after stage I Norwood procedure varies between 11% and 37%, and it contributes to late death after this operation. We describe the incidence and report the results of percutaneous balloon angioplasty of neoaortic arch obstruction in patients after the modified Norwood procedure for hypoplastic left heart syndrome (HLHS). Methods During a period of 5 years, 136 patients (94 male, 42 female) underwent a first stage modified Norwood procedure for HLHS. All 95 survivors (69.8%) underwent cardiac catheterization before the second stage. Neoaortic arch obstruction was diagnosed on documentation of a peak systolic gradient of >10 mm Hg across the arch associated with angiographic evidence of localized narrowing of the aortic lumen. Results Twenty-one (22.1%) of the 95 survivors were documented to have neoaortic arch obstruction. Seventeen patients underwent percutaneous balloon angioplasty for the relief of the neoaortic arch obstruction. The predilatation peak gradient across the arch was reduced significantly by angioplasty from 28.6 ± 16.9 mm Hg (range 10 to 73 mm Hg) to 5.3 ± 5.2 mm Hg (range 0 to 19 mm Hg) ( P Conclusion The absence of serious sequelae after the procedure or need for reintervention, as shown by our study, makes balloon angioplasty an effective technique and the treatment of choice for the relief of recoarctation of the neoaortic arch in patients with staged palliation of HLHS. (Am Heart J 2000;140:227-31.)

BackgroundOur aims were to evaluate left ventricular uptake of radionuclide in patients with Mustard's or Senning's procedure, comparing them with patients who had undergone banding of the pulmonary trunk and conversion to the arterial switch.MethodsTechnetium perfusion scans were performed on 27 patients (25 male), aged from 10 to 28 years with a mean of 17.8 years and a standard deviation of 5.8 years, who had undergone Mustard's or Senning's procedure for correction of complete transposition. Of the 27 patients, six had been accepted for staged conversion to an arterial switch. At the time of the study, two of the six patients had undergone completion to the switch and four had undergone banding of the pulmonary trunk with two then proceeding to the arterial switch. Cardiac catheterisation to measure left ventricular pressure was performed in all six patients and scores for left ventricular uptake of isotope were compared with echocardiographic index of the thickness of the left ventricular posterior wall and measure ments of left ventricular pressure.ResultsUptake of isotope by the left ventricle was generally poor, but was higher in patients following banding and conversion to the arterial switch, as well as in two patients with native obstruction of the left ventricular outflow tract, and one other who subsequently was found to have pulmonary venous obstruction. There was a positive correlation between the thickness of the left posterior wall in diastole and left ventricular uptake of isotope (r = 0.74, p< 0.05). There also a positive correlation between left ventricular pressure and uptake of the isotope (r = 0.68, pConclusionsUptake of radionuclide by the left ventricle after Mustard's or Senning's procedure for complete transposition appears to reflect ventricular pressure and myocardial mass. A prospective study would be required to determine the predictive ability of such scans regarding the ultimate outcome of conversion to arterial switch, but our initial findings suggest that the technique provides an additional non-invasive method of monitoring left ventricular response to pulmonary arterial banding.

We describe successful implantation of a permanent pacemaker via the right subclavian vein in a 28-year-old man with operated transposition of the great arteries where the superior vena cava is completely disconnected from the systemic venous atrium following a previous Glenn procedure.

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.

AimTo analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.MethodsThis study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.5 years.ResultsA staged Norwood/Fontan palliation for hypoplastic left heart syndrome was performed in 527 patients. The 30-day survival rate after individual stages was 76.5% at Stage I, 96.3% at Stage II, and 99.4% at Stage III. A total of 348 interventions were performed in 189 out of 527 patients. Freedom from catheter intervention in survivors was 58.2% before Stage II and 46.7% before Stage III. Kaplan–Meier freedom from intervention post Fontan completion was 55% at 10.8 years of follow-up. Post-stage I interventions were mostly directed to relieve aortic arch obstruction – 84 balloon angioplasties – and augment pulmonary blood flow – 15 right ventricle-to-pulmonary conduit interventions; post-Stage II interventions centred on augmenting size of the left pulmonary artery – 73 procedures and abolishing systemic venous collaterals – 32 procedures. After Stage III, the focus was on manipulating the size of the fenestration – 42 interventions – and the left pulmonary artery −31 procedures.ConclusionInterventional cardiac catheterisation constitutes an integral part in the staged palliative management of patients with hypoplastic left heart syndrome. Over one-third (37%) of patients undergoing staged palliation required catheter intervention over the follow-up period.

In conclusion, our experience suggests that PBA of coarctation of the aorta is a feasible and safe procedure for both native and repaired coarctationin adults. However, some patients may have unsuitable morphology and should not be subjected to PBA. The results are effective in the medium term and may obviate the need for surgery in a large number of patients. The procedure should be routinely considered in adults with this condition, but long-term follow-up remains essential.

Percutaneous device closure of the left atrial appendage (LAA) has been introduced in the last decade as a minimally invasive alternative treatment to long-term anticoagulation to reduce the risk of thrombo-embolism in patients with atrial fibrillation. Echocardiography is an essential tool at all stages of the procedure. Pre-procedural echocardiography is used to screen suitable candidates and to define LAA morphology and dimension; peri-procedural transoesophageal or intracardiac echocardiography has a major role in guiding, delivery, and deployment of the device, for screening of procedural complications and for assessing procedural success; and post-procedural echocardiography is important in the surveillance and monitoring of long-term outcome. This article aims to outline the role of echocardiography at each stage of LAA occlusion.

Jo-hannson et al wonder whether residual migraine burden might berelated to residual right-to-left shunting. The MIST trial was notpowered to assess this and was fully focused through its primary andsecondary end points on the possibility of migraine cessation orreduction in the implanted cohort. The degree of residual shuntingwas a tertiary end point of the study; however, moderate or largeresidual shunts were found in only 6% of patients.The real question arising from the MIST trial is why did PFOclosure for migraine alone prove relatively ineffective if PFO closurefor stroke routinely abolishes incidental migraine? First, manytreatments considered useful in observational registries become lessimpressive when tested in randomized, controlled trials. Second,migraine is a syndrome with no diagnostic test. Patients who havemigraine in the context of stroke normally have migraine of onlymodest severity. By comparison, the patients in the MIST trial werea cohort of severe long-term migraineurs who had failed to improveafter taking at least 2 prophylactic medications. For a sufferer withmoderate migraine, as typically seen in the stroke trials, PFO closuremight reduce the overall risk burden to below the average thresholdto trigger migraine. In the case of an intractable migraineur,however, other factors for migraine headache may be present. If weremove 1 of these factors by PFO closure, it is not surprising thatmigraine resolution after this intervention is rare.The best time to design a clinical trial has always been after thetrial has been completed, and in the case of the MIST trial, the useof a core echocardiographic laboratory would have prevented someof the conjecture surrounding the trial. It must be stressed, however,that the MIST trial was designed and powered to determine whetherPFO closure could effectively eradicate migraine headache, not todetermine whether residual shunts could be matched to residualmigraine. In this primary aspiration, it was ambitious, and ultimatelyunsuccessful, but the trial itself, with its unique sham-controlleddesign, was a success. The MIST trial will be remembered as the firstrandomized trial of PFO closure for migraine and the trial that pavedthe way for others to refine the patient group for whom this treatmentmight be most effective.

Objective. To determine the rate of vascular access complications in patients with adult congenital heart disease (ACHD). Background. Complications of femoral access following coronary angiography or percutaneous coronary intervention have been studied extensively, but the complication rate following catheterization and intervention in ACHD patients is poorly documented. Design, Setting, and Outcome Measures. We present a retrospective audit of vascular access complications in a large tertiary ACHD center over a 12-month period. Complications were defined as any clinically significant hematoma, pseudoaneurysm, arteriovenous fistula, or bleeding resulting in the need for imaging, transfusion, vascular or radiological intervention, or delayed discharge. Results. Of 197 procedures (102 interventions and 95 cardiac catheterizations), a complication rate of 3.6% was identified, comparable to that of coronary angiography and percutaneous coronary intervention. The main complications were femoral artery pseudoaneurysm and hematoma resulting in delayed discharge by a mean of 2⅔ days (range 1–4 days). Predictors of risk for vascular complications include female sex, history of diabetes, and anticoagulation; larger sheath sizes and obesity were not associated with higher complication rate. Conclusions. Adult congenital heart disease patients represent a unique and ever-growing population with a higher incidence of catheterization as children, surgical cut-down scars and anatomical variants. We present a low incidence of femoral access complications in interventional and diagnostic procedures in a large series of ACHD patients over a 12-month period. Patients with risk factors for vascular complications may be considered for device closure of the venous access site.

Aim To report the experience of 23 tertiary referral European Centres on transcatheter closure of congenital ventricular septal defects (VSD). Methods and results Implantation of transcatheter devices was attempted in 430 patients (pts) with congenital VSDs until July 2005. The following anatomic types were present: 119 muscular, 250 perimembranous, 16 multiple, 45 residual post-surgery. Median VSD size was 7 mm (range 3–22), fluoroscopy time 33 min (range 3–146). Devices implanted were Amplatzer muscular or membranous devices in 364, PDA devices in 12, ASD devices in seven, Starflex in seven, and coils in nine patients. Procedure was successful in 410 cases (95%). Complications: device embolization in five cases (surgery in two, catheter retrieval in three), aortic regurgitation in 14 cases (two of which requiring surgery), tricuspid regurgitation in 27 cases (no surgery was necessary), minor rhythm disturbances in 10 pts, death in one patient, complete heart block (cAVB) in 16 pts [perimembranous 12 of 250 (5%), muscular one of 119 (0.8%), residual post-surgery VSD three of 45 (6.7%)]. CAVB was transient in six patients, requiring permanent pace-makers in 10 cases (3.8%) (six early, four late). In the multivariate analysis, the only variable associated with a risk of the occurrence of complication was age ( P = 0.012) and weight ( P = 0.0035). In the univariate analysis, risk factors for the development of cAVB were, device type ( P = 0.03) and VSD location ( P = 0.05). After the multivariable Cox proportional hazards analysis, no risk factor was found. Conclusion Transcatheter closure of congenital VSDs offers encouraging results. Complications are limited; the most relevant one seems to be the device related to cAVB in perimembranous VSD. More experience and long-term follow-up are mandatory to assess safety and effectiveness of this procedure as an alternative to conventional surgery.

Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10–20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor.

Pulmonary artery stenosis can cause right ventricular strain but intrapulmonary lesions are inaccessible to surgery; moreover, some are also resistant to high pressure balloon angioplasty. An alternative is the use of microsurgical devices mounted on balloons for transvenous delivery, including cutting balloons. The current literature is sparse but seems to indicate a role for cutting balloons in specific situations. The higher cost of these devices does not merit routine use instead of high pressure balloons but a randomised controlled study is underway to address this issue. Until the results of this become available, the role of cutting balloons for pulmonary artery stenosis remains limited to specific situations. The recommendations for the safe use of these devices must be adhered to by all operators.

The long-term survival of children with irreversible intestinal failure is often dependent on adequate central venous access for the administration of parenteral nutrition. In children with occlusion of major central thoracic veins, innovative techniques to establish venous access have been described in the literature. The present report describes an innovative stereotactic technique of catheter insertion in children with occluded internal jugular and brachiocephalic veins. The catheter is inserted percutaneously from the neck into the distal patent stump of the superior vena cava communicating with the right atrium.

Background— The use of permanent synthetic implants to close atrial septal defects (ASD) and patent foramen ovale (PFO) has a number of limitations, including late complications and the limiting of transeptal access to the left heart should it be required for the later treatment of acquired heart disease. BioSTAR is a novel, bioabsorbable, atrial septal repair implant. This phase I pilot study evaluates the feasibility, safety, and effectiveness of BioSTAR for the first time in humans. Methods and Results— We conducted a prospective, open-label, multicenter clinical study in 58 patients aged 28 to 68 years who had a clinically significant ASD or PFO. Percutaneous shunt closure was undertaken with the BioSTAR septal repair implant. Successful device implantation was achieved in 57 (98%) of 58 patients. Closure at 30 days and 6 months, assessed by contrast transthoracic echocardiography, was 48 (92%) of 52 and 54 (96%) of 56, respectively. There was no evidence of a clinically significant response to the device. Transient atrial arrhythmia occurred in 5 patients after implantation. No major safety issues were observed. Conclusions— This study demonstrates the feasibility, safety, and effectiveness of BioSTAR for the closure of ASD and PFO in humans with a high rate of early and complete shunt closure. BioSTAR is a novel septal repair implant designed to provide biological closure of atrial-level defects using the patient’s natural healing response. Because 90% to 95% of the implant is absorbed and replaced with healthy native tissue, future access to the left atrium may be achieved.

Objective: To assess the performance of the bovine Contegra 1 valved conduit used for right ventricular (RV) outflow tract reconstruction, particularly in relation to post-operative RV pressure. Methods: Follow-up study of 64 consecutive right ventricular to pulmonary artery-conduit implants in 62 patients between January 2000 and April 2003. The majority of cases were forms of pulmonary atresia/VSD (n = 24, 39%) or Fallot’s tetralogy (n = 13, 21%). Thirteen cases (21%) had aortic atresia, truncus arteriosus or discordant connections with pulmonary atresia/VSD. Twelve cases (19%) were conduit replacements. Echocardiography was performed for a median follow-up of 14 months (range 0—38 months). Results: Median age at implantation was 13.8 months (range 0.1—244 months) and median weight was 8.9 kg (range 2.1—84.1 kg). Thirty-eight patients (59.4%) were

The condition of pulmonary atresia (PA), ventricular septal defect (VSD), and major aortopulmonary collaterals (MAPCAs) can have an extremely variable pulmonary blood supply and there are essentially three main anatomical subtypes, one with confluent native pulmonary arteries, one with intrapulmonary but nonconfluent pulmonary arteries, and one with MAPCAs and no native pulmonary arteries (1) Surgical repair can be very challenging and the results are further complicated by patchy vascular distribution to the lung parenchyma, multiple stenoses, progressive narrowing of the MAPCAs, and high pulmonary vascular resistance; despite these hurdles, surgical repair is being carried out with increasing frequency and success, but this is also creating a new population of patients who require a specific interventional strategy. These problems, some of which are not easily addressed surgically, can be helped by catheter intervention methods and the two approaches are complementary. Between 1989 and 2002, 164 patients had surgical recruitment of MAPCAs at Birmingham Children's Hospital with repair in 70%. On average, these patients require 2.3 catheter procedures up to the point of repair. The catheter interventions are technically demanding, time consuming, must be repeated, and require specific techniques due to the pathology of the vessels. The management of PA/VSD/MAPCAs involves a multidisciplinary team approach often requiring repeated procedures, especially for those at the worst end of the spectrum. A proactive approach is essential due to known disease progression. Considerable improvement in patients' quality of life and, hopefully, life expectancy makes this approach worthwhile.

An 8-year-old boy who suffered from Hirschsprung's disease had development of tricuspid valve endocarditis that progressed to aortic root abscess formation, development of a fistulous communication between aorta and right atrium, atrial and ventricular septal defects, and a left ventricle to right atrium defect. Several surgical procedures were required. Operation consisted initially of closure of the septal defects and aortic valve repair. This was followed by homograft replacement of the aortic valve for persistent infection, and further closure of a left ventricle to right atrium fistula.

Coarctation of the aorta can be managed in different ways depending upon the age of presentation, anatomical details of the coarctation, the aortic arch anatomy, and whether the coarctation is native or a recurrence following surgery. In infants, surgery is the preferred treatment of choice, whereas in older children and adults, percutaneous procedures, such as balloon angioplasty or stent implantation, are becoming increasingly popular methods for treating coarctation. Various types of stents have been used and this paper specifically addresses the use of covered or graft Jomed stents in a small group of patients with coarctation. (J Interven Cardiol 2001;14:187–190)

We report two patients with small, muscular ventricular septal defects, both of whom had a potential risk of infective endocarditis and underwent transcatheter closure using a detachable Cook coil. We suggest that in selected patients this technique offers a simple and cost-effective alternative to surgery or double-disc device procedures.

Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.

Body art in the form of tattoos and piercing has become increasingly popular amongst children and teenagers, and is nowadays more socially acceptable despite media reports citing tissue destruction and death. Our study explored the awareness and experience of patients with congenital heart disease, and of cardiologists and professionals responsible for their care.

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modified Damus operation without the use of exogenous material was undertaken in conjunction with creation of an aortopulmonary shunt 3.5 mm in diameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were clinically well without major systemic ventricular dysfunction or atrioventricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava—pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revision of the aortopulmonary shunt. The 4 other survivors are awaiting a cavopulmonary shunt. Univariate analysis yielded the chronologic rank for an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposition of the great arteries as predictors of death. This aggressive surgical approach provides excellent early palliation, and because the operation prevents abnormal ventricular hypertrophy from pressure or volume overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.

A ventricular tunnel is a congenital abnormal paravalvular pathway between the aortic root and the left ventricular cavity bypassing the aortic valve and not penetrating the septal musculature.1 This cardiac anomaly was first described by Levy et al in 1982.2 Surgical closure is usually the treatment of choice. We report the first transcatheter closure of an aorto-left ventricular tunnel using an Amplatzer patent ductus arteriosus (PDA) occluder device (AGA Medical Corporation, Golden Valley, Minnesota). • • • This case concerns a 14-yearold boy whose mother had an uncomplicated pregnancy and an uneventful delivery. The initial neonatal examination did not reveal any heart problems or any other anomalies and the child was discharged after 48 hours. A diastolic murmur was first heard at 7 years of age during a routine check and he was referred to our outpatient clinic where the clinical diagnosis of aortic regurgitation (AR) was made. The echocardiogram showed a bleb in the right coronary sinus of Valsalva just to the left of the origin of the right coronary artery. He was clinically well and asymptomatic. Catheterization was performed in 1993 and confirmed mild AR without evidence of aortic valve prolapse or aneurysm formation. Left ventricular function, coronary arteries, and aortic arch were normal. The child has been under annual review and has remained symptom-free, with the same signs of mild AR. During the last evaluation in March 1999, the echocardiogram showed left ventricular volume overload due to an aorto-left ventricular tunnel and mild AR, although the aortic valve appeared normal. Catheterization was repeated to confirm the diagnosis and consider transcatheter closure. Under general anesthesia and transesophageal echocardiography control, a rightand left-sided cardiac catheterization was performed using a 6Fr sheath in the right femoral vein, a 5Fr sheath in the right femoral artery, and a 5Fr sheath in the left femoral artery. Separate arterial access was used to allow delivery of the device and ensure freedom of coronary flow before deployment. The patient weighed 61.6 kg, and 50 IU/kg of heparin was administered at the beginning of the procedure. The transesophageal echocardiogram showed a normally functioning but volume loaded left ventricle with mild AR. The aorto-left ventricular tunnel was sized at approximately 9 mm at its maximal diameter. Normal right-sided left ventricular and aortic pressures and saturations were recorded. Using a 5Fr pigtail and a 5Fr multitrack catheters (NuMed, Inc., Hopkinton, New York), angiography of the left ventricle, ascending aorta, and coronary arteries was performed to evaluate the anatomy. This showed an aorto-left ventricular tunnel arising from the right coronary sinus of Valsalva, but separate from the right coronary artery, and which drained into the left ventricle. The aorto-left ventricular tunnel measured 6.1 mm at its maximum diameter with a “windsock” shape (Figure 1). A 6Fr Gensini catheter was easily passed through the aorto-left ventricular tunnel into the left ventricle; an exchange guidewire was then introduced and stabilized in the left ventricle. The catheter was then replaced by an 7Fr delivery sheath and dilator which was advanced over the exchange wire into the left ventricle through the aorto-left ventricular tunnel. The dilator was removed and the delivery sheath was deaired. The correct position was confirmed by an injection of radiopaque contrast. A 10/8-mm Amplatzer PDA occluder device (AGA Medical Corporation, Minnesota) was mounted on a delivery wire and introduced into the delivery sheath through a loading device, and then advanced into the left ventricle through the aorto-left ventricular tunnel under fluoroscopic control. The device flange From The Heart Unit, Birmingham Children’s Hospital, Birmingham, United Kingdom. Dr. De Giovanni’s address is: The Heart Unit, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, United Kingdom. E-mail: degiovanni@cableinet.co.uk. Manuscript received November 9, 1999; revised manuscript received and accepted February 4, 2000. FIGURE 1. Anteroposterior projection: aorto-left ventricular tunnel angiography showing the “Windsock” shape.

Objective The aim of this study was to compare the outcome of the double-switch procedure for congenitally corrected transposition of the great arteries for patients completing morphologic left ventricle training by means of pulmonary artery banding with the outcome of patients whose morphologic left ventricle did not require training. Methods A retrospective study of all patients undergoing the double-switch procedure from 1991 through 2004 was performed. Patients were divided into 2 groups: those not requiring morphologic left ventricle training (n = 33) and those completing morphologic left ventricle training by means of pulmonary artery banding (n = 11). Results The time spent with the morphologic left ventricle conditioned at systemic pressures was longer for the group not requiring morphologic left ventricle training (median, 730 days; interquartile range, 399–1234 vs median, 436 days; interquartile range, 411–646; P = .19). The overall mortality (not requiring morphologic left ventricle training, 12.1%; requiring morphologic left ventricle training, 9.1%; P = 1) and rate of death/transplantation, development of moderate-to-severe morphologic left ventricle dysfunction, or both (not requiring morphologic left ventricle training, 21.2%; requiring morphologic left ventricle training, 45.5%; P = .14) were similar between groups. Actuarial freedom from death/transplantation with good morphologic left ventricular function was superior for patients whose morphologic left ventricle did not require training ( P = .04). The follow-up was not different between groups (not requiring training: median, 1435 days [interquartile range, 285–2570 days]; requiring morphologic left ventricle training: median, 568 days [interquartile range, 399–1465 days]; P = .14). On multivariate analysis, the completion of morphologic left ventricle training predicted death/transplantation, development of moderate-to-severe morphologic left ventricle dysfunction, or both ( P = .02). Conclusions The early results of the double-switch procedure in patients whose morphologic left ventricle required training compare favorably with those of patients whose morphologic left ventricle required no training. There is an increased risk of deterioration of morphologic left ventricle function over time in patients whose morphologic left ventricle requires training, and these patients need to be followed up regularly to detect this.

The publisher regrets that an error has occurred in the reproduction of the above paper. While searching for this article online using medical databases, we found that the Paediatric Cardiology Department in Leicester was accredited for this study. The authors wish to convey that all surgery and analysis was performed at Birmingham Children’s Hospital and this manuscript is reporting work that was done exclusively at this institution.

Objective: This study was undertaken to determine the outcomes of patients with congenitally corrected transposition of the great arteries after restoration of the morphologically left ventricle to the systemic circulation. Methods Between November 1991 and June 2001, a total of 54 patients (median age 3.2 years, range 7 weeks–40 years) with either congenitally corrected transposition of the great arteries (n = 51) or atrioventricular discordance with double-outlet right ventricle (n = 3) underwent anatomic repair. This comprised a Senning procedure in all cases plus arterial switch (double-switch group) in 29 cases (53.7%), plus a Rastelli procedure (Rastelli-Senning group) in 22 cases (40.7%), and plus intraventricular rerouting (Senning-tunnel group) in 3 cases (5.6%). Left ventricular training by PA banding was performed before the double-switch operation in 9 of 29 cases (31%). Follow-up is complete (median 4.4 years). Results Early mortality was 5.6% (n = 3), with 2 late deaths. Kaplan-Meier survivals (± SEM) were 94.4% ± 3.1% at 1 year and 89.7% ± 4.4% at 9 years. Survivals at 7 years were 84.9% ± 7.1% in the double-switch group and 95.5% ± 4.4% in the Rastelli-Senning group ( P = .32). Of the 49 survivors, 46 (94%) were in New York Heart Association functional class I. Six have acquired new left ventricular dilatation or impaired systolic ventricular function. Four patients in the double-switch group had moderate aortic valve regurgitation develop, and 2 of them required valve replacement. Overall freedoms from reoperation at 1 and 9 years were 94.2% ± 3.3% and 77.5% ± 9.0%, with no significant difference between the groups ( P = .60). Conclusions Anatomic repair of congenitally corrected transposition of the great arteries can be carried out with low early mortality. Excellent functional status can be achieved, with good midterm survival. Continued surveillance is necessary for patents with valved conduits and to determine the longer-term function of the aortic valve and the morphologically left ventricle in the systemic circulation.

Background: Paravalvular leak (PVL) occurs in 5% to 17% of patients following surgical valve replacement. Percutaneous device closure represents an alternative to repeat surgery. Methods: All UK and Ireland centers undertaking percutaneous PVL closure submitted data to the UK PVL Registry. Data were analyzed for association with death and major adverse cardiovascular events (MACE) at follow-up. Results: Three hundred eight PVL closure procedures were attempted in 259 patients in 20 centers (2004–2015). Patient age was 67±13 years; 28% were female. The main indications for closure were heart failure (80%) and hemolysis (16%). Devices were successfully implanted in 91% of patients, via radial (7%), femoral arterial (52%), femoral venous (33%), and apical (7%) approaches. Nineteen percent of patients required repeat procedures. The target valve was mitral (44%), aortic (48%), both (2%), pulmonic (0.4%), or transcatheter aortic valve replacement (5%). Preprocedural leak was severe (61%), moderate (34%), or mild (5.7%) and was multiple in 37%. PVL improved postprocedure ( P P P P =0.011). Factors independently associated with death were the degree of persisting leak (HR, 2.87; P =0.037), New York Heart Association class (HR, 2.00; P =0.015) at follow-up and baseline creatinine (HR, 8.19; P =0.001). The only factor independently associated with MACE was the degree of persisting leak at follow-up (HR, 3.01; P =0.002). Conclusion: Percutaneous closure of PVL is an effective procedure that improves PVL severity and symptoms. Severity of persisting leak at follow-up is independently associated with both MACE and death. Percutaneous closure should be considered as an alternative to repeat surgery.