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Objectives. Preconditioning of cells or organs by transient sublethal ischemia-reperfusion (IR), termed ischemic preconditioning (IPC), protects the cell or organ from a subsequent prolonged ischemic insult. The mechanisms of this effect remain to be fully elucidated. We have recently reported that IPC of a forearm results in alterations in gene expression profiles of circulating polymorphonuclear leukocytes. The goal of the current study was to determine if the observed changes in gene expression lead to functional changes in neutrophils. Methods. We examined the effect of repetitive transient human forearm ischemia (three cycles of 5 min ischemia, followed by 5 min of reperfusion) on the function of circulating neutrophils. Neutrophil functions were examined before, after 1 d, and after 10 d of daily transient forearm ischemia. To modulate IR-induced inflammation the neutrophils were stimulated with N-formyl-methionyl-leucyl phenylalanine (FMLP) and lipopolysaccharide (LPS). Results. Neutrophil adhesion was significantly decreased on day 1 and remained low on day 10 (P [ 0.0149) without significant change in CD11b expression. Phagocytosis was significantly suppressed on day 10 compared with day 0 (P < 0.0001). Extracellular cytokine levels were low in the absence of an exogenous stimulus but stimulation with LPS induced significant changes on day 10. We observed a trend in reduction of apoptosis on day 1 and day 10 that did not reach statistical significance (P < 0.08). Conclusion. This study indicates that repetitive IPC of the forearm results in substantial alterations in neutrophil function, including reduced adhesion, exocytosis, phagocytosis, and modified cytokine secre

Aim: Intermittent limb ischaemia prior to cardiac ischaemia is a cardioprotective stimulus. This study was to investigate whether this peripheral stimulus had any effects on basal coronary blood flow and resistance, and to explore its potential mechanisms by studying the effect of femoral nerve transection and Katp blockade by glibenclamide. Methods: Remote ischaemic preconditioning (rIPC) was induced by four 5-min cycles of lower limb ischaemia. Coronary resistance was measured using standard formulae and coronary blood flow in the left anterior descending artery (LAD) by a flow probe. In experiment 1, coronary ischaemia was induced by inflation of a cuff placed around the mid-LAD, and inflated until cessation of flow. Left ventricular (LV) function was assessed using dp/dt and Tau at 1 and 30 min of ischaemia. Experiment 1: 20 pigs were randomized to control (n = 6), rIPC (n = 7) or femoral nerve transection + rIPC (n = 7) groups. The femoral nerve was transected before the rIPC protocol. All data were collected at fixed heart rates of 120 bpm. Coronary resistance was decreased and flow was increased significantly by the rIPC stimulus (P = 0.003, P = 0.016, paired t-test), and these changes were preserved after femoral nerve transection. Experiment 2: 19 pigs were randomized to control (n = 5), rIPC (n = 8) or glibenclamide-treated rIPC (n = 6) groups. Data were collected at baseline, and during incremental pacing between 120 and 180 bpm. Results: Experiment 1: Coronary resistance was decreased and flow was increased significantly by rIPC stimulus (P = 0.003, P = 0.016, paired t-test), and these changes were preserved after femoral nerve transaction. rIPC was associated with superior LV function (dp/dtmax) at 30 min, compared with controls and the rIPC + femoral nerve transaction group. Experiment 2: Coronary resistance was significantly lower, and LAD flow was significantly higher in rIPC group (P

Remote ischemic preconditioning reduces myocardial infarction (MI) in animal models. We tested the hypothesis that the systemic protection thus induced is effective when ischemic preconditioning is administered during ischemia (PerC) and before reperfusion and examined the role of the K+-dependent ATP (KATP) channel. Twenty 20-kg pigs were randomized (10 in each group) to 40 min of left anterior descending coronary artery occlusion with 120 min of reperfusion. PerC consisted of four 5-min cycles of lower limb ischemia by tourniquet during left anterior descending coronary artery occlusion. Left ventricular (LV) function was assessed by a conductance catheter and extent of infarction by tetrazolium staining. The extent of MI was significantly reduced by PerC (60.4 ± 14.3 vs. 38.3 ± 15.4%, P = 0.004) and associated with improved functional indexes. The increase in the time constant of diastolic relaxation was significantly attenuated by PerC compared with control in ischemia and reperfusion ( P = 0.01 and 0.04, respectively). At 120 min of reperfusion, preload-recruitable stroke work declined 38 ± 6% and 3 ± 5% in control and PerC, respectively ( P = 0.001). The force-frequency relation was significantly depressed at 120 min of reperfusion in both groups, but optimal heart rate was significantly lower in the control group ( P = 0.04). There were fewer malignant arrhythmias with PerC during reperfusion ( P = 0.02). These protective effects of PerC were abolished by glibenclamide. Intermittent limb ischemia during myocardial ischemia reduces MI, preserves global systolic and diastolic function, and protects against arrhythmia during the reperfusion phase through a KATPchannel-dependent mechanism. Understanding this process may have important therapeutic implications for a range of ischemia-reperfusion syndromes.

BACKGROUND: Brain death is associated with profound disturbances of systemic and myocardial oxygen transport, but little is known regarding the acute response of systemic oxygen consumption (VO(2)). METHODS: Brain death was induced in 6 pigs (30.6 +/- 3.0 kg) by balloon inflation into the cranial cavity. VO(2) was continuously measured by respiratory mass spectrometry. Blood pressures and gases were measured from the aorta, superior vena cava, and coronary sinus, with arterial epinephrine and norepinephrine, prior to brain death, at 1, 10, and 90 minutes after brain death. Cardiac output (CO), systemic vascular resistance (SVR), oxygen delivery (DO(2)), oxygen extraction (EO(2)), and myocardial oxygen (mEO(2)) and lactate extractions (mE(1ac)) were calculated. Left ventricular contractility was assessed by micromanometer tipped catheters. RESULTS: VO(2) increased from 4.8 +/- 0.9 to 6.3 +/- 0.9 mL/min/kg 1 minute after brain death (P < .001), and subsequently decreased to below baseline at 90 minutes (P < .001). Left ventricular contractility, CO, and DO(2) increased 1 minute after brain death (P < .001), followed by a rapid decrease to baseline within 10 minutes (P < .001). SVR and EO(2) decreased after brain death (P < .01) and remained low. Lactate remained unchanged. mE(1ac) decreased after brain death despite a decrease in mEO(2) (P < .01), and returned to baseline at 90 minutes. CONCLUSIONS: The initial surge in VO(2) after brain death is offset by the greater increase in DO(2), thus tissue perfusion remains adequate. The lower than baseline VO(2) and SVR at the end of the study period may indicate general metabolic and hemodynamic compromise. The information regarding the profound metabolic alterations imposed by brain death may have implications for management of brain death donors.

Patients with Fontan physiology may eventually require heart transplantation (HT). We determined the rates and outcomes of HT in a national, population-based multicentre study.From 1990 to 2015, 1369 patients underwent the Fontan procedure as recorded in the Australia and New Zealand Fontan Registry. We identified those who underwent HT and analysed their outcomes. We compared rates of HT between two catchment areas. In area 1 (n=721), patients were referred to the national paediatric HT programme or its associated adult programme. In area 2 (n=648), patients were referred to the national paediatric HT programme or one of the other adult HT programmes.Mean follow-up time post-Fontan was 11±8 years. Freedom from Fontan failure was 74%±3.9% at 20 years. HT was performed in 34 patients. Patients living in area 1 were more likely to have HT (4.0%, 29/721 vs 0.8%, 5/648, p0.001) with a cumulative proportion of 3.4% vs 0.7% at 10 years and 6.8% vs 1.2% at 20 years (p=0.002). Area 1 patients were more likely to undergo HT (hazard ratio 4.7, 95% CI 1.7 to 13.5, p=0.003) on multivariable regression. Post-HT survival at 1, 5 and 10 years was 91%, 78% and 71%, respectively. Compared with other patients with congenital heart disease (n=87), Fontan patients had similar in-hospital outcomes and long-term survival.Although HT after the Fontan procedure can be achieved with excellent outcomes, most patients with Fontan failure do not undergo HT. Significant regional differences in rates of HT in Fontan patients exist.

Objectives Partial atrioventricular septal defect (pAVSD) is repaired with excellent long-term survival. However, up to 25% of patients require reoperations. This study reviews results of reoperation following pAVSD repair at a single institution. Methods From 1975 to 2012, 40 patients (16%, 40/246) underwent reoperation following pAVSD repair at the study institution. The data were retrospectively reviewed. Results The mean time to reoperation was 5.4 ± 5.8 years. The most common reoperations were left atrioventricular valve (LAVV) surgery (78%, 31/40) and resection of left ventricular outflow tract obstruction (20%, 8/40). The most common cause for LAVV surgery was regurgitation through the cleft (58%, 18/31), followed by central regurgitation (29%, 9/31). Most cases of LAVV regurgitation were treated by repair (77%, 24/31), rather than replacement (23%, 7/31). Since the introduction of a patch augmentation technique for LAVV repair in 1998, the rate of repair has increased from 54 to 94% (P = 0.012). The early mortality rate was 2.5% (1/40). The survival rate was 90% (95% CI: 76-96) at 10 years and 83% (95% CI: 60-94) at 20 years. The rate of freedom from further reoperation was 66% (95% CI: 46-80) at 10- and 20-year follow-up. Conclusions The most common cause for reoperation following pAVSD repair was LAVV regurgitation through the LAVV cleft. Reoperation is performed with survival comparable to that of primary pAVSD repair, yet the rate of further reoperations remains high. The patch augmentation technique for LAVVR has significantly increased the rate of successful LAVV repair.

Currently, the extracardiac Fontan operation is a procedure of choice in patients undergoing a staged palliation for univentricular hearts. However, it is not always easy to prevent the right pulmonary artery twisting after implantation of the extracardiac conduit. Herein, we described a simple modification, which we referred to as T-Fontan procedure, to prevent right pulmonary artery stenosis after extracardiac Fontan operation.

Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention. Methods We conducted a retrospective review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne. Results Twenty-nine (69% [29 of 42]) patients underwent coronary reimplantation, 12 (29% [12 of 42]) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% [1 of 42]) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months–31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months–25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% [11 of 42]) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% [5 of 11]) had concomitant MV repair at the time of ALCAPA repair, 3 (27% [3 of 11]) had late MV repair or replacement, and the remaining 3 (27% [3 of 11]) patients had mild MR at last follow-up. Thirty-six (90% [36 of 41]) patients had normal left ventricular function and 4 (10% [4 of 41]) patients had mildly reduced left ventricular (LV) function at last follow-up. Conclusions ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up.

Objectives The risk of mortality while awaiting heart transplantation (HT) may be greater in the setting of a smaller population and lower donor organ rates. Optimizing allocation policy and identifying the most vulnerable patients will help reduce mortality. As such, we aimed to evaluate the predictors of outcomes in patients awaiting HT as part of a National Paediatric HT Programme. Methods Between 1988 and 2015, 200 children were listed for HT as part of a National Paediatric HT Programme. We categorized patients as experiencing one of four competing events: (i) transplant, (ii) death, (iii) delisting for clinical deterioration and (iv) delisting for clinical improvement. Comparison was made across three study eras: Era 1: 1988-95; Era 2: 1996-2005; Era 3: 2006-15. A multivariable competing risk regression analysis was performed to determine the independent predictors of transplantation and the composite event of death or delisting for clinical deterioration. Results Among the 200 patients listed, 60% (119/200) underwent transplantation, 22% (44/200) died on the waiting list, 7% (13/200) were delisted for clinical deterioration and 11% (21/200) were delisted due to clinical improvement, with 2% (3/200) being still active on the waiting list. The mortality-rate for patients who remained on the waiting list decreased from 27% in Era 1 to 18% in Era 3. The survival from wait-listing was 57, 47 and 39% at 5, 10 and 15 years, respectively. On multivariable competing risk regression, older age (HR 1.09, 95% CI 1.01-1.18, P = 0.029) and the absence of inotropic support (HR 2.22, 1.23-4.00, P = 0.0073) were predictors of reaching transplantation. Higher creatinine (per 20 µmol/l, HR 1.42, 1.03-1.95, P = 0.03) was a predictor of the composite endpoint of death or delisting for deterioration. Conclusions In this analysis of a National Paediatric HT Programme, waiting-list mortality has decreased over time though it remains higher than countries with higher donor rates. The requirement for inotropic support and worsening renal function were predictors of failure to reach transplantation. These patients are likely to benefit the most from ventricular-assist device therapy and higher priority listing.

Introduction: Cardiopulmonary bypass (CPB) can be associated with deleterious clinical effects. However, the impact of CPB on inflammatory, immunological and other homeostatic pathways remains poorly understood. We investigated the impact of CPB on the plasma proteome in children undergoing tetralogy of Fallot repair. Methods: Blood samples were taken from 20 children prior to and at the end of CPB and 6h, 12h and 24h after CPB. Plasma was analysed by liquid chromatography-mass spectrometry (LC-MS) in a label-free, untargeted approach. Data were analysed using Genedata software to identify peptides that were differentially expressed (pResults: The proteins that were found to be differentially expressed were haptoglobin isoform 1 preproprotein, isoform 2 of semaphorin-6C, vitamin D-binding protein, inter-alpha-trypsin inhibitor, ceruloplasmin, apolipoprotein B100 and fibrinogen alpha. Conclusion: CPB alters the plasma proteome with differences most apparent at 6h and 12h post CPB. There was a return to baseline with no proteins differentially regulated by 24h.

The bovine jugular vein (Contegra) conduit has been described as an alternative to the homograft for right ventricle (RV) to pulmonary artery (PA) connection. We assessed the outcomes of Contegra conduits and homografts at a single institution.We conducted a retrospective review of children (n = 249) who underwent RV-to-PA conduit (Contegra or homograft) implantation from 2001 to 2011.Median operation age was 4.8 years (2 days-18 years). Indications for surgery were as follows: primary conduit insertion (n = 131; 53%), previous conduit failure (n = 57; 23%) or Ross procedure (n = 61; 25%). There were 113 (45%) Contegra conduits and 136 (55%) homografts (92 pulmonary, 44 aortic) inserted. Early mortality was 5% (n = 12). Overall survival was 89% (95% confidence interval (CI): 84-92%) at 5 years and 87% (95% CI: 81-92%) at 10 years. Mortality was associated with smaller conduit size (P = 0.044) and syndrome diagnosis (P = 0.012). Freedom from reoperation was 85% (95% CI: 77-91%) and 75% (95% CI: 59-86%) at 5 years for homografts and Contegra conduits, respectively. Patients required conduit replacement (15%) for endocarditis (n = 4; 11%) or graft failure (n = 34; 89%). Eleven patients developed distal conduit stenosis with the majority occurring in Contegra conduits (n = 7; 64%) (P = 0.004). A larger conduit (P = 0.007) was protective against reoperation. There was no difference in reoperation between conduits (P = 0.41). Mean follow-up was 5 ± 3.2 years (96% complete). Majority of survivors (99%) were in New York Heart Association Class II/I.The Contegra conduit and homograft demonstrate similar mid-term outcomes in children. Smaller conduit size is associated with higher graft failure and mortality.

Background Remote ischemic preconditioning (RIPC) has been applied in paediatric cardiac surgery. We have demonstrated that RIPC induces a proteomic response in plasma of healthy volunteers. We tested the hypothesis that RIPC modifies the proteomic response in children undergoing Tetralogy of Fallot (TOF) repair. Methods and Results Children (n=40) were randomized to RIPC and control groups. Blood was sampled at baseline, after cardiopulmonary bypass (CPB) and 6, 12 and 24h post-CPB. Plasma was analysed by liquid chromatography mass spectrometry (LC-MS) in an untargeted approach. Peptides demonstrating differential expression (p

OBJECTIVES: We conducted a randomized controlled trial of the effects of remote ischemic preconditioning (RIPC) in children undergoing repair of congenital heart defects. BACKGROUND: Remote ischemic preconditioning reduces injury caused by ischemia-reperfusion in distant organs. Cardiopulmonary bypass (CPB) is associated with multi-system injury. We hypothesized that RIPC would modulate injury induced by CPB. METHODS: Children undergoing repair of congenital heart defects were randomized to RIPC or control treatment. Remote ischemic preconditioning was induced by four 5-min cycles of lower limb ischemia and reperfusion using a blood pressure cuff. Measurements of lung mechanics, cytokines, and troponin I were made pre- and postoperatively. RESULTS: Thirty-seven patients were studied. There were 20 control patients and 17 patients in the RIPC group. The mean age and weight of the RIPC and control patients were not different (0.9 +/- 0.9 years vs. 2.2 +/- 3.4 years, p = 0.4; and 6.9 +/- 2.9 kg vs. 11.5 +/- 10 kg, p = 0.06). Bypass and cross-clamp times were not different (80 +/- 24 min vs. 88 +/- 25 min, p = 0.3; and 55 +/- 13 min vs. 59 +/- 13 min, p = 0.4). Levels of troponin I postoperatively were greater in the control patients compared with the RIPC group (p = 0.04), indicating greater myocardial injury in control patients. Postoperative inotropic requirement was greater in the control patients compared with RIPC patients at both 3 and 6 h (7.9 +/- 4.7 vs. 10.9 +/- 3.2, p = 0.04; and 7.3 +/- 4.9 vs. 10.8 +/- 3.9, p = 0.03, respectively). The RIPC group had significantly lower airway resistance at 6 h postoperatively (p = 0.009). CONCLUSIONS: This study demonstrates the myocardial protective effects of RIPC using a simple noninvasive technique of four 5-min cycles of lower limb ischemia and reperfusion. These novel data support the need for a larger study of RIPC in patients undergoing cardiac surgery.

Background. Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group. Methods. From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA. Site of aortic arch interruption was distal to the left subclavian artery in 16% and between the left common carotid and subclavian artery in 84%. From the common arterial trunk, the pulmonary arteries arose from a main pulmonary trunk in 46%, common orifice in 22%, and separate orifices in 32%. At presentation, truncal valve stenosis was present in 12% and regurgitation in 22%. Results. There were 34 deaths, with a single early hazard phase. Overall survival from admission was 44%, 39%, and 31% at 6 months, 1 year, and 10 years, respectively. One patient had primary cardiac transplantation and 4 died without any intervention. The IAA repair alone was performed in 7 patients, with single stage repair of both IAA and TA in 38 patients. Associated factors for overall time-related death include female gender (p < 0.001), type III TA (p < 0.001) and one institution (low-risk; p < 0.001). Results improved somewhat over time (p < 0.001). At 5 years after IAA repair only 28% were alive without arch repair intervention, and at 5 years after TA repair only 18% were alive without conduit reoperation. Conclusions. The combination of IAA and TA carries high early mortality, with high risk of reinterventions in survivors. One stage repair of both TA and IAA is the optimal management.

Background Congenital coronary artery fistula (CAF) is a rare anomaly that may lead to the development of early heart failure or late complications. We reviewed our experience and outcomes of surgically corrected CAF. Methods From June 1982 to October 2012, 13 children aged between 3 days and 5.2 years (median 13.9 months) underwent repair of CAF. Four patients (30.8%) presented with congestive heart failure. Both coronary arteries were affected with equal incidence. All patients underwent preoperative cardiac catheterization. Repair was undertaken with cardiopulmonary bypass in 10 patients (76.9%), and 5 patients (38.5%) had concomitant repair of associated cardiac lesions. Seven patients (53.8%) underwent epicardial closure, and in 6 patients (46.2%), transcoronary/endocardial closure was used. Results There were no early or late deaths. Transient myocardial ischemia occurred in 2 patients (14.4%). One patient (7.7%) underwent reoperation (aortic valve repair) for a bicuspid aortic valve, 22 years after initial surgery. Follow-up data were complete for 12 patients (92.3%), with a median follow-up of 6 years (range, 1 month to 31 years). All patients were in New York Heart Association class I with no symptoms. There were no long-term cardiovascular sequelae, and no patient required coronary reintervention. Conclusions Early surgical repair of CAF in children can be performed safely and carries an excellent long-term prognosis.

Anomalous aortic origin of the left coronary artery from the right coronary sinus is a rare congenital anomaly that may cause sudden death. Direct translocation of the left coronary artery could be difficult due to its intramural course and proximity to the aortic valve. We described the surgical management of a child after sudden hemodynamic collapse and the coronary translocation technique that prevented distortion of the aortic root and allowed successful translocation of the abnormal coronary artery despite its intramural course immediately behind the aortic valve commissure.

Isovolumic acceleration (IVA) obtained by tissue Doppler echocardiography (TDE) is a sensitive and relatively load-independent index for assessing systolic ventricular function. IVA also has the ability to describe the force-frequency relationship during incremental atrial pacing in vivo.We sought to assess the ability of IVA to detect global left ventricular (LV) dysfunction induced by coronary constriction.In 6 open-chest anesthetized pigs we examined right ventricular and LV long-axis function by TDE (4-chamber view) with simultaneous invasive measurements of intraventricular pressure, maximum dP/dt, minimum dP/dt, and tau by microtip catheter. A pneumatic cuff was placed around the proximal portion of left anterior descending coronary artery (LAD) and distal flow was monitored by transonic flow probe. Mean arterial pressures were monitored by indwelling cannula. Baseline studies assessed force-frequency relationships with TDE and invasive measurements during incremental pacing from 100 to 200/min (20/min increments every 10 minutes). The protocol was repeated 10 minutes after balloon inflation to reduce LAD blood flow by 50%.Compared with baseline, LV pressure decreased significantly (P = .03, 2-way analysis of variance) as did maximum dP/dt (P.004) with LAD constriction. At the same time IVA and isovolumic velocity at the LV free wall were significantly reduced (P.002 and P = .04, respectively) and both IVA and isovolumic velocity were correlated with dP/dt (r = 0.45, P.002, and r = 0.35, P.02, respectively). TDE systolic indices were unchanged in the right ventricle.IVA detects changes in global LV systolic function during LAD constriction and may be a useful clinical tool to diagnose ischemia.

In the dawn of cardiac surgery in the 1940s to 1950s, Alexander A. Vishnevsky assembled together a fine team of surgeons and conducted intense surgical research that resulted in clinical application of modified Blalock-Taussig shunt, cavo-pulmonary shunt, and paved the way to clinical orthotopic heart and lung transplantation and coronary bypass surgery in Russia. The team of surgeons lead by Vishnevsky also performed the world's first cavo-pulmonary shunts and experimental orthotopic heart and lung transplantation. Herein, we describe the pioneering contributions of Vishnevsky and his team to cardiac surgery.

BackgroundWe have recently demonstrated that remote ischemic preconditioning reduces ischemia-reperfusion injury in animal models. The mechanisms by which the remote ischemic preconditioning stimulus exerts its effect remain to be fully defined, and its effect on myocardial gene expression is unknown. We tested the hypothesis that remote ischemic preconditioning modifies myocardial gene expression immediately after the remote ischemic preconditioning stimulus (early phase) and 24 hours later (late phase).MethodsTwenty male (C57BL/6) 10- to 12-week-old mice were randomized into 4 groups: group 1 (control, early phase; n = 5), group 2 (remote ischemic preconditioning, early phase; n = 5), group 3 (control, late phase; n = 5), and group 4 (remote ischemic preconditioning, late phase; n = 5). Groups 2 and 4 underwent remote ischemic preconditioning induced by 6 cycles of 4 minutes of occlusion and 4 minutes of reperfusion of the femoral artery. Groups 1 and 2 were killed 15 minutes after completion of sham procedure or remote ischemic preconditioning, and the hearts were removed and frozen in liquid nitrogen. Groups 3 and 4 were killed 24 hours after remote ischemic preconditioning, and the hearts were harvested in the same fashion. Gene expression was assessed by using the Affymetrix MG-430A chip (Affymetrix, Santa Clara, Calif).ResultsData filtering (P < .05, analysis of variance) and hierarchic 2-way clustering identified significant differences in gene expression among the 4 groups. Genes involved in protection against oxidative stress (eg, Hadhsc, Prdx4, and Fabp4) and cytoprotection (Hsp73) were upregulated, whereas many proinflammatory genes (eg, Egr-1 and Dusp 1 and 6) were suppressed.ConclusionA simple remote ischemic preconditioning stimulus modifies myocardial gene expression by upregulating cardioprotective genes and suppressing genes potentially involved in the pathogenesis of ischemia-reperfusion injury.

The widespread use of rodent models of cardiovascular disease has demanded improved methods of assessing myocardial function. Recently, invasive measurement of cardiac function using conductance catheterization has become available for assessing ventricular performance in small animals. 1-3 However, although pressure-volume loop analysis has become the gold standard, it is impractical for longitudinal assessment of disease or interventions. The ideal method under these circumstances would be noninvasive, an accurate index of contractile function, resistant to the influence of load, and reproducibly measurable in small animals. Doppler tissue echocardiography (DTE) is a new modality that has been widely used to assess cardiac function noninvasively. 4,5 Many indices of systolic function have been described but, as might be expected, ejection-phase indices have been shown to be load dependent. This limits their applicability to the assessment of intrinsic myocardial contractility. We have recently demonstrated that myocardial isovolumic acceleration (IVA), derived by DTE, is a relatively load-independent index of systolic ventricular function that is also capable of describing the force-frequency relationships in both the right ventricle and left ventricle (LV) in porcine models and human beings. 6,7 DTE has previously been used to assess cardiac function in rodent models. For example, myocardial systolic and diastolic velocity gradients were shown to be abnormal in a rat model of myocardial hypertrophy. 8 However, interpretation of such data obtained with conventional probes, which are limited in terms of both spatial and temporal resolution, is difficult. Furthermore, as in studies of larger animals and human beings, interpretation of noninvasively acquired data can only be complete when the load dependency of the index is understood. To our knowledge, there are no systematically obtained data regarding the load dependency of systolic indices of function measured by DTE. This study assesses the use of DTE indices in small rodents. Using a high-resolution probe, we investigated IVA, isovolumic velocity (IVV), and systolic wall velocity (SW) of the LV myocardium together with simultaneous conductance catheter recording of pressure and volume. We examined contractile function to determine the influence of alterations in preload and afterload, and measured the response of these indices to inotropic change.

Objective We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch. Methods From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions. Results Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure. Conclusion Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.

Objective We have previously reported a limited but favorable experience with a novel sutureless technique for surgical management of postoperative pulmonary vein stenosis occurring after repair of total anomalous pulmonary venous drainage. Because this technique requires integrity of the retrocardiac space for hemostasis, extension of the technique to the primary repair of pulmonary vein anomalies requires evaluation. This analysis reviews our experience with the sutureless technique in patients with postrepair pulmonary vein stenosis, as well as our extension of the technique into primary repair of pulmonary vein anomalies. Methods Retrospective univariable-multivariable analysis of all pulmonary vein stenosis procedures and sutureless pulmonary vein procedures over a 20-year period was performed. Cox proportional hazards modeling was used to identify variables associated with freedom from reoperation or death. Results Sixty patients underwent 73 procedures, with pulmonary vein stenosis present in 65 procedures. The sutureless technique was used in 40 procedures. Freedom from reoperation or death at 5 years after the initial procedure was 49%. Unadjusted freedom from reoperation or death was greater with the sutureless technique for patients with postrepair pulmonary vein stenosis ( P = .04). By using multivariable analysis, a higher pulmonary vein stenosis score was associated with greater risk of reoperation or death. After adjustment, the sutureless repair was associated with a nonsignificant trend toward greater freedom from reoperation or death ( P = .12). Despite the absence of retrocardiac adhesions, operative mortality was not increased with the sutureless technique ( P = .64). Techniques to control bleeding (intrapleural hilar reapproximation) and improve exposure (inferior vena cava division) were identified. Conclusion The sutureless technique for postrepair pulmonary vein stenosis is associated with encouraging midterm results. Extension of the indications for the technique to primary repair appears safe with the development of simple intraoperative maneuvers.

Congenital coronary arterial fistula is a rare cause of congestive heart failure in neonates. We describe a neonate who developed early symptoms because of an unusually large left coronary artery to right ventricular fistula. Computed tomography and coronary angiography delineated anatomy and facilitated successful surgical closure.

Stapling devices have been clinically applied to connect blood vessels. The early application of these staplers in cardiovascular surgery, including coronary bypass surgery, was encouraging. With further refinement of suture material, however, vascular staplers became impractical and soon fell into oblivion. With advancement in robotic surgery, these devices might become valuable tools once again. Herein early experience with vascular circular stapling devices is reviewed.

The atrial switch operation was developed by the efforts of many surgeons, with the most notable contributions made by Blalock, Hanlon, Albert, Baffes, Senning, and Mustard. The atrial switch operation was the first definitive repair for patients with transposition of great arteries and produced good results. Although it is rarely performed today, the atrial switch is not merely of historical interest as there remain a few important indications for this operation. A thorough understanding of the atrial switch is still required for surgeons dealing with complex congenital cardiac malformations. Herein we summarize the history, review long-term results, and discuss the future of the atrial switch operation.

BACKGROUND: Systemic oxygen consumption is not routinely measured during cardiopulmonary bypass, despite its potential benefits. We aimed to develop a noninvasive method to continuously measure oxygen consumption using respiratory mass spectrometry during hypothermic cardiopulmonary bypass in pigs. METHODS: Nine pigs weighing 18.5 (1.6) kg underwent hypothermic (32 degrees C) cardiopulmonary bypass for 180 minutes with 120 minutes of aortic cross clamping. An AMIS 2000 mass spectrometer (Innovision A/S, Odense, Denmark) was adapted for the on-line measurement of oxygen consumption by sampling the inlet and outlet gases of the membrane oxygenator together with measurement of the "expired" gas volume. RESULTS: Active cooling for 60 minutes reduced the venous blood temperature by 2.9 (0.8) degrees C and VO(2) by 0.70 (0.33) mL/kg/min. The 40-minute active rewarming restored the venous blood temperature by 4.4 (0.4) degrees C and oxygen consumption increased by 1.36 (0.33) mL/kg/min. There was wide interanimal variability, however, particularly at higher venous blood temperatures. Immediately after the release of aortic cross clamp, there was a noticeably acute increase in oxygen consumption in all the pigs (0.64 [0.21] mL/kg/min). CONCLUSIONS: A simple and safe adaptation of mass spectrometry allows continuous measurement of oxygen consumption during hypothermic cardiopulmonary bypass. The wide interindividual variations observed in this pilot study underscore the need to more accurately describe changes in oxygen consumption and how they are affected by temperature, oxygen delivery, and other interventions during cardiopulmonary bypass. As such, the technique may have an important role in clinical research and management of oxygen transport in patients undergoing cardiac surgery.

The global myocardial stress response during cardiac surgery has not been systematically studied, nor is it known whether the response of the neonatal myocardium is intrinsically different from that of older children. To determine the age-related molecular basis of this response, we conducted microarray-based differential gene expression profiling on right ventricular tissue samples acquired in patients of varying ages with right ventricular outflow tract obstruction.We studied gene expression profiles in 24 patients during operations for lesions involving right ventricular outflow tract obstruction age stratified into group I (7 patients, aged 5 to 66 days; mean, 30 days) and group II (17 patients, aged 4 months to 12.5 years; mean, 2.8 years). Myocardial samples were taken from the right ventricular outflow tract after aortic occlusion and archived in liquid nitrogen. RNA isolation, fluorescence labeling of complementary DNA, hybridization to spotted arrays containing 19,008 characterized or unknown human complementary DNAs, and quantitative fluorescence scanning of gene-expression intensity were performed at the University of Toronto Health Network Microarray Centre. Data were analyzed with the Significance Analysis for Microarrays program. Minimum Information About Microarray Experiments-compliant, log2-normalized data sets were compared to ascertain potential statistical differences in gene expression between patient groups.There were no hospital deaths or major postoperative morbid events. We identified 50 transcripts differentially expressed in the neonatal group (the predicted false discovery rate was0.8 transcripts). The neonatal pattern of gene expression (group I) was dominated by genes with literature-validated cardioprotective, antihypertrophic, and antiproliferative properties, including increases in atrial natriuretic peptide, protein phosphatase 2A, small GTPase rap1, and protein inhibitor of activated STAT protein, PIASy. Several transcripts have not been previously reported in heart.Neonatal myocardium has a unique pattern of gene expression, which may result from developmental (age-related) differences or reflect a more severe disease phenotype independent of age effects per se. The neonatal transcript profile seems to reflect a stress-induced protective program composed of genes with functions diametrically opposed to those expected to be related to the pathogenesis of critical right ventricular outflow tract obstruction, thus revealing a novel and compensatory antidisease transcriptional response in the neonatal heart.

Background Bridging leaflet division may facilitate repair of atrioventricular septal defects (AVSD). However, the consequences of bridging leaflet division on early valve function and mortality are not well defined. Methods Records of children undergoing AVSD repair between January 1995 and January 2002 were reviewed. Multivariable analysis defined risk factors for moderate or greater atrioventricular valve regurgitation (AVVR) and death/reoperation within 1 year of repair. Results A total of 209 children (median age 5 months, median weight 5 kg) had defects whose repair included the possibility of bridging leaflet division. Bridging leaflets divided were both (n = 119, 58%), one (n = 30, 15%), or none (n = 55, 27%). Freedom from AVVR (moderate or greater) is 84%, 80%, and 78% at 1, 6, and 12 months. Risk factors include technical factors: number of bridging leaflets divided, longer cross-clamp time, and right-sided annuloplasty. Other risk factors include preoperative AVVR (moderate or greater), double-orifice or parachute left AV valve, and younger age. Freedom from death/reoperation for AVVR is 96%, 92%, and 90% at 1, 6, and 12 months. Risk factors are preoperative AVVR (moderate or greater) and parachute left AV valve. Findings at reoperation (n = 15, 7.2%) were cleft dehiscence or tear along cleft closure (n = 10), dehiscence of divided leaflet from septation patch (n = 1), or other (n = 4). Operative mortality (n = 6, 2.9%) included failed reoperations for AVVR (n = 4), dehiscence of divided leaflet from septation patch (n = 1), and sepsis (n = 1). Conclusions Division of bridging leaflets is a risk factor for AVVR (moderate or greater) during the first year after repair. Preservation of bridging leaflet integrity may improve valve competency, decrease the need for future reoperation, and eliminate some causes of operative mortality.

From the Department of Cardiothoracic Surgery, Royal Children’s Hospital, University of Melbourne, and Murdoch Children’s Research Institute, Melbourne, Australia. Disclosures: Authors have nothing to disclose with regard to commercial support. Received for publication April 15, 2016; accepted for publication April 15, 2016; available ahead of print May 17, 2016. Address for reprints: Igor E. Konstantinov, MD, PhD, FRACS, Royal Children’s Hospital, Flemington Rd, Parkville, VIC 3029, Australia (E-mail: igor.konstantinov@rch.org.au). J Thorac Cardiovasc Surg 2016;152:696-7 0022-5223/$36.00 Crown Copyright 2016 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery http://dx.doi.org/10.1016/j.jtcvs.2016.04.064 Intramural ventricular septal defects occur when blood flows underneath the trabeculations.

A persistent fifth aortic arch is exceptionally rare. We report a patient with a double-lumen aortic arch in association with a perimembranous ventricular septal defect and Cornelia de Lange syndrome. We also discuss the morphologic consequences of persistent fifth aortic, arch, which may not be limited to the double-lumen aorta but in fact may be more common.

Settling scores in public is arduous. It is never perfect and someone always gets hurt. Fortunately, this is now a virtually forgotten legacy of the Wild West. However, setting risk scores using public databases could be somewhat similar. Furthermore, the expectations for the all-encompassing risk score, applicable to every patient, are so high that the pearls of wisdom are easily lost in the vast and confronting ocean of

The contemporary surgical management of multiple ventricular septal defects (VSDs) carries a relatively high mortality rate in comparison to that of other biventricular repairs. Several innovative approaches have been recommended for repair of multiple VSDs, but none are uniformly successful in achieving complete elimination of ventricular shunting without causing ventricular dysfunction. This is especially true in cases with highly deficient ventricular septa, the so-called "Swiss cheese" anomaly. The recent introduction of the CardioSEAL (NMT Medical Inc, Boston, MA) double-clamshell device led to an evaluation of intraoperative device-mediated closure of muscular VSDs. Taken in context with our overall experience in the surgical management of multiple VSDs, we suggest that device-mediated closure of muscular VSDs is a useful and potentially ventricular function-sparing option for this challenging problem.

The diagnosis of nonbacterial thrombotic endocarditis (NTBE) is rarely made during life. This report describes a child who had high-grade non-Hodgkin's lymphoma with NTBE and multiple systemic embolism. The transthoracic echocardiographic findings of mitral valve leaflet vegetations and progressive regurgitation led to surgical resection of the vegetations. A high index of suspicion is needed when a clinician is faced with a patient who has malignancy, systemic embolic phenomena, and persistent negative blood cultures.

An interesting and thought-provoking article by DeNino and colleagues 1 appears in the current issue of the Journal. This article describes 2 patients with HeartMate II (Thoratec Corporation, Pleasanton, Calif) left ventricular assist device (LVAD) in whom pump thrombosis developed despite what appeared to be an appropriate initial anticoagulation regimen. Both initially received aspirin at low dose (81 mg daily), heparin, and warfarin. The initial responsiveness to aspirin, defined as less than 550 aspirin reaction units (ARU), was confirmed in both patients. Both became unresponsive to aspirin. Both appeared noncompliant with their anticoagulation regimens. Did both have aspirin resistance, or resistance to taking aspirin? How can one determine the causes of diverse prothrombotic conditions that are pulled together and called ‘‘aspirin resistance’’? What isthis elusiveaspirinresistance anyway? The article by DeNino and colleagues 1 poses more questions than it gives answers. Yet one has to begin somewhere, and this article is an excellent beginning. It does not provide the solution, but rather it brings attention to a complex problem yet to be solved. As we enter the era of personalized medicine, a few comments on aspirin resistance and its relevance to cardiac surgeons appear timely.Aspirin resistance is a real phenomenon and defined as the inability of aspirin to reduce platelet production of thromboxane A2 (TXA2; Figure 1) and thereby platelet activation and aggregation. 2 Aspirin is a nonselective and irreversible inhibitor of both forms of cyclooxygenase (COX), but is weakly more selective for COX-1. Aspirin inhibition of COX-1 is rapid and saturable at low doses. This may leave some COX-2, which is greatly upregulated by proinflammatory conditions, uninhibited. Thus TXA2 can be produced by activated macrophages, monocytes, and endothelial cells through COX-2. Interestingly, higher doses of aspirin cause greater inhibition of COX-2 and dramatically lower concentration of urinary 11-dehydrothromboxane B2. 3,4 The Heart Outcomes Prevention Evaluation study 5 demonstrated that patients

Objective The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne. Methods Review of 32 successive anatomic corrections between 1996 and 2015. Results Twenty-one double-switch (66%), 6 Senning/Bex-Nikaidoh (19%), and 5 Senning/Rastelli (16%) procedures were performed (median age, 1.9 years). Median follow-up was 5.4 years with 4 deaths and 1 heart transplant. Cumulative incidence of late reoperation was 8%, 29%, and 59% at 1, 5, and 10 years, respectively. Twenty-six patients had full follow-up with native hearts. Nineteen had normal left ventricle (LV) function. Late LV dysfunction, mostly mild, was not related to needing a pacemaker ( P = .4) or a pulmonary artery band (PAB) ( P = .08). Previous PAB was linked to the need for aortic valve surgery or neoaortic regurgitation moderate or greater ( P = .03). Six required Senning revision. The introduction of the Shumacker modification of the Senning has generated stable pulmonary venous pathways. Six patients developed postoperative iatrogenic atrioventricular block dependent on a permanent pacemaker. Conclusions Anatomic correction is a surgical challenge. It provides excellent functional outcomes in survivors with a significant need for reoperation and a definite risk of death or transplantation. Normal LV function should be expected in most patients. LV dysfunction was not linked to PAB or pacemaker requirement but surgery without LV training had better long-term LV function. The Shumacker modification provided stable venous pathways. Iatrogenic atrioventricular block remains a challenge.

Immature mediastinal teratomas refractory to chemotherapy are very rare and require management by an aggressive surgical approach. We describe resection of a large mediastinal teratoma in a 23-year-old man, with a satisfactory early result.

We aimed to determine the effect of elective left heart decompression at the time of initiation of central venoarterial extracorporeal membrane oxygenation (VA ECMO) on VA ECMO duration and clinical outcomes in children in a single tertiary ECMO referral center with a large pediatric population from a national referral center for pediatric cardiac surgery. We studied 51 episodes of VA ECMO in a historical cohort of 49 pediatric patients treated between the years 1990 and 2013 in the Paediatric Intensive Care Unit (PICU) of the Royal Children's Hospital, Melbourne. The cases had a variety of diagnoses including congenital cardiac abnormalities, sepsis, myocarditis, and cardiomyopathy. Left heart decompression as an elective treatment or an emergency intervention for left heart distension was effectively achieved by a number of methods, including left atrial venting, blade atrial septostomy, and left ventricular cannulation. Elective left heart decompression was associated with a reduction in time on ECMO (128 h) when compared with emergency decompression (236 h) (P = 0.013). Subgroup analysis showed that ECMO duration was greatest in noncardiac patients (elective 138 h, emergency 295 h; P = 0.02) and in patients who died despite both emergency decompression and ECMO (elective 133 h, emergency 354 h; P = 0.002). As the emergency cases had a lower pH, a higher PaCO2 , and a lower oxygenation index and were treated with a higher mean airway pressure, positive end-expiratory pressure, and respiratory rate prior to receiving VA ECMO, we undertook multivariate linear regression modeling to show that only PaCO2 and the timing of left heart decompression were associated with ECMO duration. However, elective left heart decompression was not associated with a reduction in length of PICU stay, duration of mechanical ventilation, or duration of oxygen therapy. Elective left heart decompression was not associated with improved ECMO survival or survival to PICU discharge. Elective left heart decompression may reduce ECMO duration and has therefore the potential to reduce ECMO-related complications. A prospective, randomized controlled trial is indicated to study this intervention further.

OBJECTIVES: To evaluate the outcomes associated with surgical repair of scimitar syndrome. METHODS: From 1974 to 2012, 21 patients with scimitar syndrome underwent corrective surgery. Outcomes included mortality, reoperation rate, hospital length of stay and long-term functional status. RESULTS: The mean age of surgery was 5.4 years (2.5 months to 16.7 years). Sixteen (76.2%) patients were symptomatic at presentation. Fifteen (71.4%) patients presented with the infantile form and 6 (28.6%) with the adult form of scimitar syndrome. Operative techniques included baffle repair in 15 (71.4%) patients, reimplantation in 5 (23.8%) patients and pneumonectomy in 1 (4.8%) patient. Six (28.6%) patients were operated in infancy at a mean of 4 months (2.5–9.6 months) of age. There was 1 (4.8%) hospital death and 3 (15%) late deaths. The risk factors for mortality included preoperative pulmonary hypertension (P= 0.006) and surgery during infancy (P= 0.003). The incidence of postoperative pulmonary vein stenosis was 23.8% (n= 5). Three (14.3%) patients underwent reoperation for pulmonary vein stenosis. Neither baffl e( P= 0.6) nor reimplantation (P= 0.55) surgical techniques influenced the rate of stenosis. The follow-up was complete in 15 (88%) survivors at a mean of 13.7 years (1.3–38.5 years). All surviving patients were asymptomatic and had a New York Heart Association functional status Class I. CONCLUSIONS: Mortality was highest in patients who had preoperative pulmonary hypertension and those who were operated on before 1 year of age. Surgical technique did not influence the occurrence of pulmonary vein stenosis. Survivors were asymptomatic at the longterm follow-up.

OBJECTIVES: Recurrent left atrioventricular valve (LAVV) regurgitation after atrioventricular septal defect (AVSD) repair is a difficult tech-nical issue. This study exposes the various techniques successively employed to repair the recurrent LAVV regurgitation and their differentoutcomes. Emphasis however will be put on the new technique used in our unit called cleft patch augmentation, which has been usedcontinuouslysince 1998 in the anatomical context of normal papillary muscles (NPMs).METHODS: This is a retrospective follow-up study using a Cox regression model for risk analyses from November 1991 to July 2008, in-cluding 45 patients who underwent reoperation for LAVVregurgitationafter AVSD repair. Of those, 3 patients were lost tofollow-up; there-fore, 42 patients were included in the study. With regard to the AVSD morphology, therewere partial AVSD in 12, complete AVSD in 30.RESULTS: Age at the primary valve repair was 1.5±2.1 years and the time span to the reoperation was 7.1 years in median (0.41–12.3years). Age at the first reoperation was 10.1±6.8 years. Median follow-up after the reoperation was 7.4 years. Three patients died in thefollow-up period. Freedom from second reoperation at 10 years was 72.8% [59.5–89.0% of 95% confidence interval (CI)]. Of 37 patientswith NPMs, freedom from reoperation at 10 years was 59.4% (37.2–94.7% 95% CI) in cleft closure group whereas, in the cleft patch aug-mentation group,it was 92.3% (78.9–100% 95% CI) (P=0.04). Five patients required valve replacement.CONCLUSIONS: Surgical result for the redo LAVV repair had good outcomes. In the NPM group, the cleft patch augmentation techniquehad better results. Varioustechniques may haveto be performed in combinationaccording to the morphological features.Keywords: Atrioventricular septal defect † Congenital heart disease † Valve repair

Background Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. Methods Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). Results A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. Conclusions Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up.

We report our experience with an adolescent patient who required complex management leading to retransplantation for coronary allograft vasculopathy and also review the role of ventricular assist device support in patients with this clinical entity.