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Background: The neonatal repair of interrupted aortic arch and ventricular septal defect (IAA/VSD) presents a surgical challenge. Although one-stage repair has become well established, left ventricular outflow tract obstruction (LVOTO) continues to be an important factor affecting survival and reintervention rates after IAA/VSD repair. We investigated the relationship between the preoperative aortic annulus and the rates of reoperation for LVOTO., Methods: Between July 1994 and July 2006, 38 patients with IAA/VSD have undergone complete single-stage repair. Patients with single ventricle physiology, transposition of the great arteries, or truncus arteriosus were excluded. Surgical technique involved the aortic arch reconstruction and VSD closure. Three patients underwent subaortic resection at the time of first operation and one patient underwent Yasui operation. The patients were divided into two groups according to whether the aortic annulus is greater than the patient's weight (kg) + 1.5 mm or less., Results: The average follow-up was 7.9 +/- 4.2 years. Among the patients with small aortic annulus (n = 12), there was one hospital death and 6 reoperations for LVOTO, and one late death. There was only one reoperation for LVOTO among the patients with larger aortic annulus (n = 26, p < 0.001). The patients whose aortic annulus is less than patient's weight (kg) + 1.0 mm had poor outcomes if the LVOTO is not addressed at the time of the first operation., Conclusions: Neonatal single-stage repair for IAA/VSD achieves excellent survival. For the patients whose aortic annulus is greater than patient's weight (kg) + 1.5 mm, low reoperation rate for LVOTO is expected. For the patient whose aortic annulus is less than patient's weight + 1.5 mm, almost half of them needed reoperation. An LVOT bypass procedure (Yasui or Norwood) is recommended if the aortic annulus is less than the patient's weight + 1.0 mm., (Copyright 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Substantial controversy persists regarding the need and efficacy of a routine myectomy in the treatment of discrete subaortic stenosis. Although some believe myectomy more effectively relieves subaortic narrowing, this is uncertain, and complications, including heart block and aortic valve injury, are concerns. The aims of the study were as follows: (1) to analyze the role of enucleation for relief of subaortic stenosis and the risk factors associated with recurrence and reoperation and (2) to delineate the characteristics of the patients who might benefit from enucleation alone., Methods: From January 1990 through May 2007, 221 patients with subaortic stenosis underwent biventricular repair. Of those, 106 patients had discrete subaortic stenosis. The preoperative peak left ventricular outflow tract gradient, as determined by means of transthoracic echocardiographic analysis, was 67.3 +/- 29 mm Hg. Forty patients had previous operations for other intracardiac anomalies. Mean age at repair was 7 years. Sixty-one patients underwent isolated enucleation, and 45 patients underwent concomitant myectomy. Patients with recurrent subaortic stenosis whose first operation was performed elsewhere were excluded from analysis., Results: There was 1 early death and 1 late death. The postoperative peak left ventricular outflow gradient decreased to 12.5 +/- 12.9 mm Hg (P < .001). No patient had development of heart block or required a pacemaker. A recurrent gradient of greater than 30 mm Hg was found in 26 (27%) patients, and 8 (7.5%) patients had reoperations. Actuarial freedom from reoperation rates at 5, 10, and 15 years were 94.7% +/- 1.8%, 89.6% +/- 3.5%, and 84.8% +/- 4.9%, respectively.Of those patients who had not undergone a previous cardiac operation, there were no significant differences in the rates of recurrence (28% vs 27%) or reoperation (4.7% vs 4.4%) between the enucleation group and the concomitant myectomy group. For the patients who had a previous cardiac operation, the concomitant myectomy group had a significantly lower rate of recurrence (44% for enucleation vs 13% for enucleation plus myectomy, P = .031)., Conclusions: For those patients undergoing primary operations for discrete subaortic stenosis, routine myectomy does not offer superior relief of left ventricular outflow tract obstruction; enucleation alone provides good results in this selected population. However, in those patients with associated cardiac anomalies, concomitant additional myectomy is recommended.

Background: The right ventricle (RV) has a lower ability than the left ventricle (LV) to adapt to systemic load. The molecular basis of these differences is not known. We compared hypertrophy-signaling pathways between the RV and the LV in patients with congenital heart disease (CHD)., Methods: Gene expression was measured using DNA microarrays in myocardium from children with CHD with LV or RV obstructive lesions undergoing surgery. The expression of 175 hypertrophy-signaling genes was compared between the LV (n=7) and the RV (n=11). Hierarchic clustering was performed., Results: Seventeen genes (10%) were differentially expressed between the LV and the RV. Expression of genes for angiotensin, adrenergic, G-proteins, cytoskeletal, and contractile components was lower (P < .05) and expression of maladaptive factors (fibroblast growth factors, transforming growth factor-beta, caspases, ubiquitin) was higher in the RV compared with the LV (P < .05). Five of 7 LV samples clustered together. Only 4 of 11 RV samples clustered with the LV. Genes critical to adaptive remodeling correlated with the degree of LV hypertrophy but not RV hypertrophy., Conclusion: The transcription of pathways of adaptive remodeling was lower in the RV compared with the LV. This may explain the lower ability of the RV to adapt to hemodynamic load in CHD.

Background: The combination of transposition of the great arteries and coarctation of the aorta (TGA/CoA) presents a surgical challenge. We have adopted a concurrent aortic arch repair and arterial switch operation with excellent results. These patients tend to have a small aortic (ie, neopulmonary) annulus. This study evaluates the significance of right-sided obstruction after single-stage repair of TGA/CoA., Methods: Between May 1991 and May 2006, 53 patients with TGA/CoA or Taussig-Bing/CoA (n = 20; 38%) underwent a complete single-stage repair. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 51 patients (96%). The aortic arch was enlarged in 36 patients (75%) with patch augmentation. Two patients (4%) underwent the augmentation of the right ventricular outflow tract (RVOT) at the first operation., Results: There was one hospital death (operative mortality, 1.9%) and one late death. The difference between the preoperative aortic and pulmonary annulus was significant (6.7 vs 10.4 mm, p < 0.001). The peak pressure gradient across the proximal RVOT at discharge was 16 +/- 16 mm Hg (range, 0 to 62 mm Hg). There have been six reoperations (11%) and four catheter interventions (7.5%) for right-sided obstruction. Freedom from reintervention/reoperation for right-sided obstruction at 1, 5, and 10 years is 95%, 87%, and 80%, respectively., Conclusions: Neonatal single-stage repair for TGA/CoA achieves excellent survival without transannular patch repair at the first operation. Although some of the patients have pressure gradient across the RVOT, these lesions were amenable to reintervention with minimal morbidity.

Objectives: The use of mechanical circulatory support to bridge pediatric patients to cardiac transplantation presents unique challenges because of the difficult anatomy and physiology in these patients., Methods: The United Network for Organ Sharing provided deidentifed patient-level data. The study population included 2532 transplantations performed on patients less than 19 years old in status 1/1A/1B between 1995 and 2005. Mechanical circulatory support was used in 431 patients: 241 (9.5%) received ventricular assist devices, 171 (6.8%) underwent extracorporeal membrane oxygenation, and 19 (0.8%) received intra-aortic balloon pumps., Results: Patients supported on ventricular assist devices had similar levels of hospitalization and intensive care use and less need for inotropic support (P < .0002) than had those not needing support. Five- and 10-year posttransplantation survival was better in patients receiving ventricular assist devices and patients not receiving mechanical circulatory support than in patients receiving extracorporeal membrane oxygenation or intra-aortic balloon pumping (P < .0001). Among mechanically supported patients, patients with a body surface area of less than 0.30 (odds ratio, 1.70; 95% confidence interval, 1.18-2.43) and those requiring extracorporeal membrane oxygenation (odds ratio, 1.65; 95% confidence interval, 1.15-2.35) or intra-aortic balloon pumping (odds ratio, 1.91; 95% confidence interval, 1.02-3.56) had higher long-term mortality. The use of a ventricular assist device at transplantation did not predict higher long-term, posttransplantation mortality., Conclusions: Pediatric patients requiring a pretransplantation ventricular assist device have long-term survival similar to that of patients not receiving mechanical circulatory support. Early survival among patients undergoing extracorporeal membrane oxygenation and infants is poor, reinforcing the need for improvements in device design and physiologic management of infants and neonates.

Objective: Studies of high-risk pediatric cardiac transplant recipients are lacking. The purpose of this study is to evaluate early posttransplant survival in high-risk pediatric patients., Methods: The United Network for Organ Sharing (UNOS) provided de-identified patient-level data. The study population included 3502 recipients aged less than 21 years who underwent transplantation from January 1, 1995, through December 31, 2005. Recipients were stratified on the basis of the presence or absence of high-risk criteria: pulmonary vascular resistance index greater than 6 Wood units/m2 (n = 285, 8.1%), creatinine clearance less than 40 mL/min (308, 8.8%), hepatitis C positivity (33, 0.9%), donor/recipient weight ratio less than 0.7 (80, 2.3%), panel reactive antibody greater than 40% (235, 6.7%), retransplantation (235, 6.7%), and age less than 1 year old (840, 24.0%)., Results: Overall, 1575 (45.0%) patients met at least one high-risk criterion. Higher numbers of high-risk criteria in a patient were correlated with increased 30-day mortality (0 high-risk criteria: 5.2%; 1 criterion: 7.9%; 2 criteria: 12.9%; and 3 or more criteria: 25.0%; P < .0001) and poor long-term survival (P < .0001). Among patients with high-risk criteria, a simplified scoring scale accurately predicts both 30-day and contingent 1-year mortality (P < .0001)., Conclusions: Individually, the effect of high-risk criteria on posttransplant survival varied; however, increasing numbers of criteria in a patient resulted in a cumulative increase in mortality. A scoring scale allows for the prediction of approximate mortality rates after transplantation. These findings suggest that recipient criteria for transplantation should focus on the number of high-risk criteria as well as clinical status, rather than the presence or absence of a single risk factor.

Objectives: (1) To examine the interaction of donor age with ischemic time and their effect on survival and (2) to define ranges of ischemic time associated with differences in survival., Methods: The United Network for Organ Sharing provided de-identified patient-level data. The study population included 33,640 recipients undergoing heart transplantation between October 1, 1987, and December 31, 2004. Recipients were divided by donor age into terciles: 0 to 19 years (n = 10,814; 32.1%), 20 to 33 years (11,410, 33.9%), and 34 years or more (11,416, 33.9%). Kaplan-Meier survival functions and Cox regression were used for time-to-event analysis. Receiver operating characteristic curves and stratum-specific likelihood ratios were generated to compare 5-year survival at various thresholds for ischemic time., Results: In univariate Cox proportional hazards regression, the effect of ischemic time on survival varied by donor age tercile: 0 to 19 years (P = .141), 20 to 33 years (P < .001), and 34 years or more (P < .001). These relationships persisted in multivariable regression. Threshold analysis generated a single stratum (0.37-12.00 hours) in the 0- to 19-year-old group with a median survival of 11.4 years. However, in the 20- to 33-year-old-group, 3 strata were generated: 0.00 to 3.49 hours (limited), 3.50 to 6.24 hours (prolonged), and 6.25 hours or more (extended), with median survivals of 10.6, 9.9, and 7.3 years, respectively. Likewise, 3 strata were generated in the group aged 34 years or more: 0.00 to 3.49 (limited), 3.50 to 5.49 (prolonged), and 5.50 or more (extended), with median survivals of 9.1, 8.5, and 6.3 years, respectively., Conclusions: The effect of ischemic time on survival after heart transplantation is dependent on donor age, with greater tolerance for prolonged ischemic times among grafts from younger donors. Both donor age and anticipated ischemic time must be considered when assessing a potential donor.

Objective: Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle-pulmonary artery continuity., Methods: Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality)., Results: Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 +/- 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function., Conclusions: We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle-pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.

Objectives: A critical shortage of donor organs has caused many centers to use less restrictive donor criteria, including the use of adult-age donors for pediatric recipients. The purpose of this study is (1) to describe the supply of pediatric (0-18 years) heart donors, (2) to explore the relationship between donor age and long-term survival, and (3) to define threshold age ranges associated with decreased long-term survival., Methods: The United Network of Organ Sharing provided deidentified patient-level data. Primary analysis focused on 1887 heart transplant recipients aged 9 to 18 years undergoing transplantation from October 1, 1987, to September 25, 2005. Kaplan-Meier analysis and log-rank tests were used in time-to-event analysis. Receiver operating characteristic curves and stratum-specific likelihood ratios were generated to compare survival at various donor age thresholds., Results: The number of pediatric donors decreased (P < .001) over the study period, particularly from 1993 (n = 640) through 2004 (n = 432). Among recipients aged 9 to 18 years, univariate analysis demonstrated a statistically significant (P < .001) inverse relationship between donor age and survival. Stratum-specific likelihood ratio analysis generated 3 strata for donor age: the low-risk, intermediate-risk, and high-risk groups consisted of donors aged 13 years or younger (n = 611, 32.41%), 14 to 51 years (n = 1258, 66.7%), and 52 years and older (n = 16, 0.85%), respectively. In the low-risk, intermediate-risk, and high-risk groups median survival was 4069 days (11.1 years), 3495 days (9.57 years), and 1197 days (3.28 years), respectively., Conclusions: Although donors aged 13 years or less offer pediatric recipients the best chance for achieving long-term survival, donors aged 14 to 51 years offer good outcomes to pediatric recipients. Consideration should be given to expanded use of well-selected adult-age donors for pediatric recipients.

Objectives: We reviewed our experience with repair of truncus arteriosus to assess the effect of type of right ventricular outflow tract reconstruction on perioperative morbidity, survival, and freedom from catheter-based interventions and reoperation., Methods: Patients undergoing repair of truncus arteriosus from June 1990 through February 2004 were evaluated on the basis of operative procedure regarding preoperative and postoperative variables, the need for postoperative catheter-based intervention or reoperation, and survival on the basis of univariate, multivariable, and actuarial analyses., Results: Of 54 study patients, 15 (28%) received a valved homograft, and 39 (72%) received a direct connection with a variety of hood materials. Five (9.1%) patients died. Valved homograft recipients were more likely to require reoperation than patients receiving direct connections (40% vs 15%, P = .046); however, valved homograft and direct connection recipients had a similar incidence of the combined end point of reoperation or catheter-based intervention (40.0% vs 37.5%, P = .865). Univariate and multivariable modeling demonstrated use of valved homografts or direct connections with an autologous pericardial hood to be predictive of the need for later catheter-based intervention or reoperation. Actuarial analysis demonstrated a trend toward improved outcomes in the direct connection group and within the direct connection cohort, a statistically significant difference on the basis of hood type., Conclusions: Although the direct connection technique might not prevent later catheter-based intervention, it does reduce the need for reoperation. Outcomes among direct connection recipients were associated with hood type: polytetrafluoroethylene hoods (W. L. Gore & Associates, Inc, Tempe, Ariz) had the lowest rate of reintervention, and untreated autologous pericardial hoods had the highest rate of reintervention. We report excellent outcomes with primary repair of truncus arteriosus. Where anatomically appropriate, we advocate the direct connection technique.

Left ventricular ejection is depressed immediately after repair of ventricular septal defect (VSD). Postrepair functional depression seen after VSD closure could result from a reduction in preload. However, other mechanisms could be at work. Functional depression could also be caused by closure of a low-impedance path for left ventricular ejection, the introduction of a stiff akinetic patch, or the operation itself. We reasoned that functional depression mediated by changes in preload or afterload should symmetrically affect end-diastole and end-systole, whereas depression resulting from changes in septal mechanics should be localized. We, therefore, performed segmental wall-motion analysis on intraoperative echocardiograms from patients undergoing VSD and atrial septal defect repair. After VSD closure, there was an asymmetric change in left ventricular end-systolic segment length and a decrease in fractional segment shortening localized to the septal and lateral walls, whereas patients with atrial septal defect had a symmetric increase in fractional shortening. These results suggest that acute functional depression after VSD repair is a result of localized impairment of septal function.

Objectives: We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children., Methods: The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation., Results: A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair., Discussion: Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.

Background: We tested the hypothesis that sodium-hydrogen exchange inhibition attenuates ventricular dysfunction after ischemia-reperfusion injury in the intact porcine heart., Methods: Twelve pigs (weight, 30-45 kg) were evenly divided into 2 groups. Baseline ventricular function studies were based on echocardiography, conductance, aortic flow, and left ventricular pressure. Animals were given vehicle (control) or benzamide-N-(aminoiminomethl)-4-(4-[2-furanylcarbonyl]-1-piperazinyl)-3-(methylsulfonyl)methanesulfonate (BIIB 513; 3 mg/kg administered intravenously). Ten minutes later, hearts were subjected to 75 seconds of ventricular fibrillation. After reperfusion for 40 minutes, function studies were repeated. Hearts were arrested and excised. Postmortem data included passive pressure-volume curves and myocardial water content., Results: Preload recruitable stroke work was significantly decreased from baseline after ischemia and reperfusion in the control group (27.7 +/- 2.5 vs 48.0 +/- 5.6 mm Hg [+/- SEM], P =.001) but not in the BIIB 513 group (43.0 +/- 5.8 vs 45.5 +/- 4.1 mm Hg, P = not significant). In vivo diastolic and postmortem passive left ventricular compliance were reduced after ischemia and reperfusion for control animals but remained unchanged for animals receiving BIIB 513. Time required to recover baseline blood pressure after ventricular fibrillation was significantly longer for control animals (159 +/- 15 vs 88 +/- 14 seconds [+/- SEM], P =.008). Myocardial water content (78.97% +/- 0.94% vs 77.86% +/- 0.46% [+/- SEM]) and normalized left ventricular mass (137.24 +/- 6.17 vs 128.41 +/- 1.96 g [+/- SEM]) were insignificantly increased in control animals., Conclusions: Sodium-hydrogen exchange inhibition attenuates ventricular dysfunction after 75 seconds of ventricular fibrillation and 40 minutes of reperfusion. This family of agents might prove useful in patients with severe left ventricular dysfunction undergoing ventricular fibrillation for implantable cardioverter defibrillator testing.