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OBJECTIVES The hands-on surgical training course utilizes 3-dimensional (3D)-printed heart models to simulate complex congenital heart operations. This study aimed to validate a model and assessment tool in the simulation of 2 techniques of the Norwood operation and investigate whether technical performance improves following rehearsal with or without proctor presence. METHODS Five ‘experienced’ and 5 ‘junior’ surgeons performed 2 techniques of the Norwood operation on 3D-printed models of hypoplastic left heart syndrome. Performances were retrospectively assessed by 10 raters with varying experience in congenital heart surgery (CHS) (Medical Doctorate versus non-Medical Doctorate). Assessments were made with the procedure-specific Hands-On Surgical Training-CHS tool. Results were analysed for technical performance and rater consistency. Following validation, 30 surgeons (24 with proctor guidance and 6 with training videos and objective feedback only) simulated the Norwood operation twice. RESULTS Performance scores were consistently higher for experienced surgeons and raters discriminated clearly between the experienced and junior surgeons (P ≤ 0.001). The hands-on surgical training-CHS tool showed high inter-rater (0.86) and intra-rater (0.80) reliability among all raters. Scores for both experienced and junior surgeons were highly consistent across all raters, with no statistically significant difference (P = 0.50). All surgeons successfully performed the Norwood operation. Sixty attempts were scored in total. Eighty-seven percentage (26/30) of surgeons’ scores (mean: attempt 1 = 92, attempt 2 = 104) and times [mean: attempt 1 = 1:22:00, attempt 2 = 1:08:00 (h:mm:ss)] improved between the 2 attempts by 9% and 15% respectively (P ≤ 0.001). Total scores of all surgeons in the non-proctored subgroup (6) improved by 15% on average (mean: attempt 1 = 86, attempt 2 = 105, P = 0.002). CONCLUSIONS Procedure-specific assessment tools can be developed to evaluate technical performance for complex CHS simulation and be performed reliably by non-expert raters. Rehearsal both under supervision and independently leads to technical skill improvement further supporting its value in CHS training.

Intramural coronary arteries remain a major risk factor and technical challenge of the arterial switch operation for transposition of the great arteries. We report a 27-year-old woman who presented with acute coronary syndrome late after arterial switch with a complex coronary pattern (intramural left and right coronary from sinus 2). Computed tomography demonstrated that the pericardial 'saddle bag' used to reconstruct the intramural coronary was compressed between the neoaortic root and the neopulmonary trunk. During surgery, the coronary system was laid open into the neoaorta with patch enlargement, and the patient made an uneventful recovery.

OBJECTIVES: Controversy exists concerning outcomes of patients with different morphologic subtypes of hypoplastic left heart syndrome undergoing the Norwood procedure, in particular, aortic atresia-mitral stenosis (AA-MS) patients receiving a systemic–pulmonary modified Blalock-Taussig (mBT) shunt. We sought to determine the influence of known risk factors and morphology on early survival in our cohort of Sano–Norwood patients with right ventricle–pulmonary artery (RV–PA) conduits as the source of pulmonary blood flow. METHODS: We studied early survival in patients with Sano-modified Norwood procedures from 2002 to 2010 and included both typical and atypical (including unbalanced atrioventricular canal defect) morphologic variants. We included a comparison group composed of classical Norwood patients with mBT shunts. RESULTS: Of 264 Sano–Norwood patients, 49 had AA-MS and 30 had atypical morphologies. Weight ≤2.5 kg was associated with a poorer 1-year survival (P = 0.0007), though ascending aorta (AscAo) size

Objective: To assess the surgical results of the Norwood procedure and subsequent clinical outcome in the setting of transposition of the great arteries (TGA) with a dominant morphologic left ventricle. Methods: Among 486 patients who underwent the Norwood procedure from 1988 to 2007 at our institution, there were 37 patients with TGA and left ventricular dependant circulation with the following associated lesions: double inlet left ventricle (DILV) (n = 24), tricuspid atresia (n = 9), ventricular septal defect (VSD) with hypoplastic right ventricle (RV) (n = 4). Outcomes for all three-staged procedure were compared with the overall Norwood group. Results: Early mortality was 21.6% (8/37) compared to 26.7% (120/449) in the overall Norwood group (p = ns). There was only one subsequent death giving a 5- and 10-year actuarial survival of 72.8 7.4% compared to 55.3 2.6% and 52 2.9% at 5 and 10 years for the overall series (p = 0.06). Median follow-up was 4.7 (0.7—10.2) years. Eighteen patients underwent stage III completion at 3.9 1.5 years from the second stage with no mortality. Preoperative mean pulmonary artery (PA) pressureand transpulmonarygradientwere respectively 11.6 3.4 and5.2 3.3 mmHg.All patientshad goodleft ventricle(LV) functionat time of stage III. All patients except one are currently in NYHA I. One patient (with DILV) had congenital heart block and required a pacemaker. There was no postoperative heart block. The systemic outflow was unobstructed in all patients and no patient required any additional intracardiac procedure. Conclusions: The Norwood procedure provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining unobstructed systemic outflow tract and avoiding the risk of postoperative heart block. # 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: A small sub-group of patients with hypoplastic left heart syndrome (HLHS) have normal-sized ascending aorta and arch. An alternative to the Norwood I procedure in these patients is the creation of an aorto-pulmonary (AP) window with a distal pulmonary artery band (PAB). We reviewed our experience with this technique and compared outcomes to the Norwood procedure for HLHS. Methods: All patients treated for HLHS in a single institution between 1992 and 2005 were analysed. This identified 13 patients treated with AP window and PAB compared to 333 patients undergoing stage I Norwood procedure. An unrestrictive AP window was created and the main PA was banded. Patient records and echocardiograms were analysed. Median follow-up was 10 (IQR 0—655) days and 100% complete. Results: There were seven early deaths (54%) in the AP window group and two conversions to Norwood circulation. This was a significantly worse outcome than for the Norwood procedure over the same period, which had an early mortality of 29% (p = 0.03). Kaplan—Meier actuarial analysis demonstrated a continued survival benefit of the Norwood group at 6 months (p = 0.0005). Deaths were due to either low cardiac output syndrome (n = 4) or sudden unheralded arrest (n = 3). This occurred despite aortic cross-clamp and circulatory arrest times being significantly lower in the AP window group compared to the Norwood group (35 27 vs 55 16 min, p < 0.01 and 16 29 vs 55 20 min, p < 0.01, respectively). No differences in arterial saturations or systolic blood pressure existed between the groups, but diastolic blood pressure was significantly lower in the AP window group at 27 10 mmHg compared to 42 8 mmHg in the Norwood group (p = 0.01) with evidence of flow reversal in the descending aorta. Differences in diastolic blood pressure between groups were abolished after conversion to stage II. Conclusions: Despite favourable anatomy and shorter ischaemic times, the AP window/PAB technique has a poor outcome compared to the Norwood procedure for HLHS. Low diastolic blood pressure with reversal of descending aortic flow in diastole was a feature of the AP window/PAB circulation. We recommend the Norwood procedure for these sub-types. This may have implications for newer ‘hybrid’ procedures for HLHS which create a similar palliative circulation. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

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 results of arterial switch operation and closure of ventricular defects (ASO + VSDc) for double outlet right ventricle with sub-pulmonary ventricular septal defect (Taussig-Bing anomaly). Methods: Between 1988 and 2003, 33 patients (25 mate, 76%) with Taussig-Bing anomaly underwent ASO + VSDc (median age 39 days, 1 day-2.1 years). The relationship of the great arteries was antero-posterior (Group I, n = 19) or side-by-side (Group II, n = 14). Coronary anatomy (Yacoub's classification) was exclusively type A or D in Group I and predominantly type D or E in Group II (64%). Incidence of sub-aortic obstruction and aortic arch obstruction was similar in Group I and II (37% vs 57%, p = 0.25 and 84% vs 79%, p = 0.98, respectively). Twenty-five patients (76%) had one-stage total correction. Risk factors were analysed using multivariable analysis. Follow-up was complete (median interval of 6.2 years; range, 0.6-15.2 years). Results: There were three early (9%) and one late death. Actuarial survival was 88 ± 6% at 1 and 10 years. There were two early and four late re-operations. Freedom from re-operation was 90 ± 5% and 75 ± 9% at 1 and 10 years. Four patients required cardiological re-interventions. Freedom from re-intervention at 5 and 10 years was 79 ± 9%. On multivariable analysis, complex coronary anatomy (type B and C) was a risk for early mortality (p < 0.001) but all other anatomical variables and staged strategy did not influence early or actuarial survival. Conclusions: The ASO + VSDc approach can be applied to Taussig-Bing anomaly with acceptable mortality and morbidity and it is the procedure of choice at our institution. Anatomical variables did not influence outcomes with this strategy. A staged strategy is still appropriate in complex cases.

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.

Background: This study was undertaken to identify risk factors for early mortality following the Norwood procedure for hypoplastic left heart syndrome (HLHS) and develop a predictive risk model to monitor clinical performance. Methods and Results: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure at a single institution. The early mortality was 29% (n = 95). Estimated early mortalityimprovedprogressivelyandwas10%attheendoftheseries.Multivariableanalysisidentifiedthatbodysurfaceareaatoperation,sizeof the ascending aorta, preoperative right ventricular function and source of pulmonary blood flow established at operation were risk factors for early mortality (P < 0.05). These variables were included in a preoperative risk model. The duration of cardiopulmonary support was an independent risk factor, which was included in a separate operative risk model. The performance of the risk models was evaluated by goodnessof-fit analyses, using the Hosmer—Lemeshow test and receiver operating characteristic (ROC) curve. Both models were well calibrated across all deciles (P = 0.64, P = 0.77) and discriminated moderately well. The area under the ROC curve was 0.71 for Model 1 and 0.75 for Model 2. Risk adjustment broadly accounted for the variation in early mortality observed during this series. Conclusions: Patient-related and predetermined operative variables have a major influence on the early outcome following the Norwood procedure for HLHS. The identification of these risk factors allows the risk of early mortality to be calculated. This information could be applied as part of a risk-adjusted performance-monitoring system to enable early identification of meaningful changes in practice.

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.

Objective: The purpose of the study was two-fold: (1) to highlight the varied presentation of mediastinal tuberculous lymphadenitis (MTL) in children and (2) to identify parameters, that may help in the early diagnosis of this condition. Methods: Between January 1995 and December 2002, 13 children with histological diagnosis of MTL were retrospectively assessed for age at presentation, history of exposure to TB, presenting symptoms, investigations, initial diagnosis, surgical treatment and outcome. Stepwise multiple linear regression analysis was used to determine potential risk factors for early diagnosis of MTL. Results: Thirteen children presented with: (a) fever, night sweats and weight loss (4); (b) acute respiratory distress (2); (c) cough and shortness of breath (SOB) (5); (d) stridor (1); and (e) chest pain (1). TB was suspected only in 6 children (46%) at presentation. In the other 7 cases (54%) the presumed diagnoses were: neuroblastoma (nZ1), metastatic malignancy (nZ1), bronchial polyp (nZ1), bronchogenic cyst (nZ2), and presumed foreign body (nZ2). Bronchoscopy was diagnostic in identifying cheesy material within the bronchus and organisms on lavage in 4 (30%) and in identifying external compression in 2 (15%). Thoracotomy and excision of the lymph node mass was necessary to treat the mediastinal compression and to ascertain the diagnosis of TB in 3 children (23%). All 13 children had complete resolution of tuberculous lymphadenitis following anti-tuberculous treatment. The diagnostic clues in this cohort of patients were cough and SOB with history of exposure to tuberculosis (PZ0.0001) and bronchoscopy and lavage with positive staining for acid-fast bacilli (PZ0.0001). Conclusions: Tuberculosis was not suspected in 54% of children with MTL, and they posed diagnostic dilemma on admission. Bronchoscopy must be used as a diagnostic tool in children where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision may be necessary to treat the obstructive symptoms. q 2004 Elsevier B.V. All rights reserved.

A 10-year old girl underwent a CT angiogram to investigate right lung hypoplasia. This showed a normal bronchial tree, lung parenchyma and pulmonary venous drainage, but an absent right pulmonary artery. Cardiac catheterization with pulmonary vein wedge injections identified a disconnected hypoplastic right pulmonary artery system supplied by an occluded right-sided ductus arteriosus. Transcatheter recanalization of the ductus re-established right pulmonary artery flow and growth. Ultimately, this allowed for complete surgical repair and restoration of normal perfusion of the right lung, leading to complete functional rehabilitation.

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.

Improved outcomes of the Norwood procedure in hypoplastic left heart syndrome have been achieved by the manipulation of the pulmonary:systemic flow ratio (Qp:Qs) in the early post-operative period, with focus on improving systemic perfusion. As an extension of this Qp:Qs-limiting strategy, we evolved a novel surgical technique to achieve transient flow restriction in the right ventricle–pulmonary artery (RV–PA) conduit for the first 48 h, using haemostatic clips, in a cohort of patients and compared the early outcomes with a non-clipped cohort. Clips were subsequently removed at 48 h at the time of definitive chest closure. We performed RV–PA shunt flow clipping in 37 patients; 75 historical controls had not received clips. Groups were matched for weight, size of ascending aorta, anatomy and circulatory arrest times. Thirty-day mortality was lower in the clipped cohort (2 of 37; 5.4%) versus the unclipped cohort (10 of 75; 13.3%). The minimum blood lactate levels within the first 24 h post-surgery in the unclipped group were significantly higher (P = 0.049), with a significantly lower Qp:Qs in the first 6 h in the clipped patients. These data suggest that limiting Qp:Qs in the early post-operative period following the Norwood procedure may help in the post-operative management of these difficult patients. These results warrant further study.

Background: In the clinical application of transformed skeletal muscle to cardiac assistance there is evidence that the latissimus dorsi muscle (LDM) wrap can undergo atrophy, which would prevent it from providing a sustained functional improvement. Possible causes are ischaemia and degeneration related to the conditioning process. We studied the nutritional and structural changes occurring under different stimulation regimes with the aim of improving the conditioning protocol. Methods: Microelectrodes were used to measure regional perfusion and oxygenation in the rabbit LDM during mobilisation and subsequent repeated contraction. Group A muscles (n = 10) were conditioned for 6 weeks at 10 Hz, Group B muscles (n = 10) for 2 weeks at 2.5 Hz. Each muscle was then mobilised and tested in a hydraulic apparatus which recorded the pressure generated in a closed circuit. Results: Muscles of Group A and Group B demonstrated transformation of fibre type, with a predominance of type I (62 ∠ 4%) fibres in Group A and type IIa (68∠ 9%) fibres in Group B. There was no evidence of muscle degeneration. After 10 min of fatigue testing the pressure produced was 53 ∠ 5% of initial values in Group A and 51 ∠ 8% in Group B, compared to 8 ∠ 1% in the control group (P , 0.001). Maximum rate of relaxation was faster in Group B than in Group A (46 ∠ 3% vs. 36 ∠ 3% of control muscle, P , 0.05). Mobilisation resulted in a decrease in the distal perfusion of the control muscles (P , 0.05) and pO2 decreased by 8.7 ∠ 1.7 mmHg during a fatigue test, which resulted in rapid loss of contractile function to 46 ∠ 1% of the initial value within 1 min. In both Groups A and B the perfusion of all regions of the muscles both before and after mobilisation was greater than that of controls. During the same fatigue test, the pO 2 of the distal regions was maintained and the contractile function fell more slowly to between 70 and 80% of initial values within 1 min. Conclusion: We showed that ischaemia in the distal region of the control LDM could result from mobilisation and repeated contraction. Muscle transformation improved perfusion and prevented a fall in tissue pO2 during a sustained series of contractions. Muscles that were conditioned at 2.5 Hz shared the improved perfusion of the fully transformed muscle, but had faster relaxation characteristics. Short periods of in situ conditioning prior to mobilisation may help to avoid ischaemic changes in distal parts of the LDM while achieving fatigue resistance in the grafted muscle at an earlier postoperative stage. © 1998 Elsevier Science B.V. All rights reserved

OBJECTIVE: Antibiotic-sterilised homograft valves stored at 4 degrees C have been implanted in the subcoronary position in this unit since 1973. This study was undertaken in order to assess the long-term function of these valves. METHODS: All 249 patients undergoing homograft aortic valve replacement (AVR) at the Wessex Cardiothoracic Centre between April 1973 and December 1994 were studied. Homograft valve sizes ranged from 15 mm to 28 mm internal diameter, 202 (81.1%) varying between 18 mm and 22 mm. The mean patient follow-up was 12.4 years with a total follow-up of 3096 patient-years. There were six early deaths (2.4%). RESULTS: On actuarial analysis, survival was 78.5+/-2.7% (1SE) at 10 years, 65.7+/-3.3% at 15 years and 55.0+/-3.9% at 20 years. The freedom from redo AVR was 87.9+/-2.4% at 10 years, 71.7 +/-3.8% at 15 years and 49.7+/-5.6% at 20 years. The freedom from structural degeneration was 85.6+/-2.5% at 10 years, 63.6+/-4.0% at 15 years and 41.9+/-6.4% at 20 years. On multivariate analysis the risk of valve failure was significantly higher in younger patients (P

A case of congenital cardiac rhabdomyoma presenting as left ventricular outflow tract obstruction is reported. Congenital cardiac tumours are rare. Rhabdomyomas are the most common. Fifty-one to 86% of them are associated with tuberous sclerosis. They have a tendency for spontaneous regression. The indications for surgery include haemodynamic compromise and intractable arrhythmias.

We report the unusual presentation of coarctation of the aorta with facial nerve palsy in an infant and a child. The facial nerve palsy and hypertension resolved spontaneously after relief of the aortic coarctation. Our two cases are the first reports of unidentified coarctation of the aorta presenting as facial nerve palsy, with the infant being the youngest to be reported.

We would like to thank Goksel and Tireli for their recent comments [1] on our recently published article [2]. We do not have any experience with off-pump surgery for cavopulmonary shunt or Fontan completion. This technique certainly has attractions of avoiding the potential deleterious effects of cardiopulmonary bypass as highlighted, particularly in terms of pulmonary vascular resistance and inflammatory response. It would be interesting to know the exact impact on the amount and duration of pleural drainage. Our greatest concernwith the off-pump technique is that is does not really allow for any reconstruction or plasty of the pulmonary arteries, which frequently needs addressing at the time of surgery, particularly following the Norwood procedure. We prefer a bypass approach which is close to normothermia on the beating heart and feel this allows for accurate assessment of the PAs and where necessary augmentation and reconstruction. It also guarantees maintenance of cardiac output should there be problems with bleeding or accessing the IVC. We await further evidence to evaluate the benefits and risks of offpump Fontan surgery.

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.