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Background: Patients with double inlet left ventricle/l-transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. A Norwood procedure may be performed as the initial palliation. We prefer aortic arch repair and pulmonary artery banding, delaying Damus-Kaye-Stansel (DKS) or BVF resection until the second palliation. The aims of this study were to compare our results with those reported for Norwood strategy and examine the development of systemic outflow obstruction.Methods: Retrospective study of patients with double inlet left ventricle, L-TGA or similar morphology presenting between 1990 and 2000. Follow-up with clinical assessment, echocardiography and catheter studies.Results: Twenty-five patients had initial palliation with pulmonary artery banding with repair of any associated arch obstruction. Twelve patients had DKS performed as part of their second stage procedure, and 3 had DKS performed later for recurrent stenosis after prior enlargement of BVF. Six patients had BVF resection without later restenosis and 4 patients did not develop BVF stenosis. There was one early death (4%) and two late (8%). Fontan completion was achieved in 20 of the 22 survivors. There were no cases of DKS obstruction, no pulmonary valve had more than mild regurgitation.Conclusions: Our approach achieves low operative mortality and morbidity and compares favorably with reported results for Norwood palliation. The significant rate of systemic outflow obstruction in those who did not undergo DKS at the second stage confirms the utility of early DKS in children with this morphology. [Copyright &y& Elsevier]

Background: Aneurysm at previous coarctation repair may be seen more frequently as children operated for this condition survive into adulthood. We use deep hypothermic circulatory arrest to repair these aneurysms.Methods: A case series was conducted using 12-year, single-institution, retrospective chart review.Results: Twenty-one patients underwent left thoracotomy and repair of aneurysm at the site of previous coarctation repair. Three cases presented emergently as aortobronchial fistulas. The age range was 16 to 73 years (median, 26 years). The median circulatory arrest time was 33 minutes (range, 22 to 55 minutes). Repair involved interposition graft replacement. Six patients required additional tube graft replacement of the left subclavian artery. There was 1 operative mortality in a patient having a hypoxic brain injury secondary to an anaphylactic reaction to a plasma expander. There were no embolic strokes or paraplegia. One patient had a recurrent laryngeal nerve paresis. There was 1 case of Horner''s syndrome after subclavian artery replacement.Conclusions: Circulatory arrest allows for the accurate repair of this difficult pathologic process and avoids the risk of clamp-related injuries. Follow-up out to 16 years demonstrates this technique of repair to be durable, with no late deaths or reoperations for recurrent aneurysm. [Copyright &y& Elsevier]

A 15-year-old boy presented with exertional palpitations and chest pain. Investigation revealed anomalous origin of his left anterior descending coronary artery from his pulmonary trunk causing myocardial ischaemia. He previously had aortic coarctation repair with known aortic root dilation and a bicuspid aortic valve. His left anterior descending artery was implanted into the aortic root using a Gortex interposition conduit. This represents an interesting combination of cardiac abnormalities for which repair required consideration of the requirement of further surgery in the future. (Heart, Lung and Circulation 2003; 12: 70-72). [ABSTRACT FROM AUTHOR]

Objective: To describe monitoring of four years' isolated coronary artery bypass surgery outcomes and complications at The Prince Charles Hospital, Brisbane, Australia., Methods: Analysis of Cardiac Surgical Register database using tabulations, funnel plots and random-effects (Bayesian shrinkage) analysis for aggregated data. Combined CUSUM and cumulative observed minus expected (modified VLAD) charts and combined CUSUM and cumulative funnel plots used for individual observation sequential data and binomial control charts and generalised additive models (GAMs) for quarterly sequential data. Risk adjustment employed re-calibrated EuroSCORE., Results: There were 2575 procedures with an unadjusted in-hospital mortality rate of 1.17%. Mean age was 65 years and 21% of patients were female; 43.6% were elective procedures. Median ventilation time was 10 hours and median length of stay in intensive care (ICU) was 23 hours. Return to theatre for bleeding occurred in 3% of cases. Return to theatre for surgical site infection occurred in 0.4% of cases; 4% were re-do procedures. Permanent stroke or neurological deficit occurred in 1%, perioperative myocardial infarction in 0.8%, arrest in 1.2%, renal failure in 1.6% and ICU return in 2.3% of cases., Conclusions: Complication rates and mortality were comparable with similar units. Use of random-effects (Bayesian shrinkage) analysis for aggregated data is encouraged together with generalised additive models (GAMs) and combined CUSUM and cumulative observed minus expected (modified VLAD) charts for sequential data., (Crown Copyright © 2011. 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 ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.

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 male, 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: The study objective was to identify how the evolution of surgical strategies influenced the outcome after the Norwood procedure., Methods: From 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., Results: Early 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 <.05). Actuarial survival at 6 months was better in group C (78% +/- 5%) than group A (59% +/- 5%) or group B (58% +/- 4%; P <.05). Fifty-three patients (14%) had arch reintervention. Freedom from arch reintervention was 76% +/- 3% at 1 year, with univariable analysis showing no difference among groups A, B, and C (P =.71). One hundred patients (27%) required subsequent pulmonary artery patch augmentation. Freedom from patch augmentation was 61% +/- 3% at 1 year, and was lower in group C (3% +/- 3%) than group A (80% +/- 4%) or group B (72% +/- 5%; P <.05)., Conclusions: Survival after the Norwood procedure improved after the introduction of a right ventricle to pulmonary artery conduit, but a greater proportion of patients required subsequent pulmonary artery patch augmentation. The type of arch reconstruction did not affect the incidence of arch reintervention.