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Identification of heart transplant recipients at highest risk for a poor outcome could lead to improved posttransplantation survival. A chart review of primary heart transplantations from 1993 to 2006 was performed. Analysis was performed to evaluate the risk of graft loss for those with a transplantation age less than 1 year, congenital heart disease (CHD), elevated pulmonary vascular resistance (index > 6), positive panel reactive antibody or crossmatch, liver or renal dysfunction, mechanical ventilation, or mechanical circulatory support (MCS). Primary transplantation was performed for 189 patients. Among these patients, 37% had CHD, 23% had mechanical ventilation, and 6% had renal dysfunction. Overall graft survival was 82% at 1 year and 68% at 5 years. The univariate risk factors for graft loss included mechanical ventilation (hazard ratio [HR], 1.9; 95% confidence interval [CI], 1.15-3.18), CHD (HR, 1.68; 95% CI, 1.04-2.70), and renal dysfunction (HR, 3.05; 95% CI, 1.34-6.70). The multivariate predictors of graft loss were CHD (HR, 1.8; 95% CI, 1.02-2.64), mechanical ventilation (HR, 1.9; 95% CI, 1.13-3.10), and the presence of two or more statistically significant univariate risk factors (SRF) (HR, 3.8; 95% CI, 2.00-7.32). Mechanical ventilation, CHD, and the presence of two or more SRFs identify pediatric patients at higher risk for graft loss and should be considered in the management of children with end-stage heart failure. [ABSTRACT FROM AUTHOR]

Background: Anomalous aortic origin of the coronary artery (AAOCA) from the opposite sinus of Valsalva with an interarterial course has received much attention due to its association with sudden death in otherwise healthy individuals. AAOCA is relatively common and may have significant public health implications. While our knowledge of its pathophysiology and natural history remains incomplete, an emphasis has been placed on surgical correction. Discussion: In 2005 we published a review examining the rates of sudden death with AAOCA, as well as complications of surgical management. Evidence now points even more strongly to lower rates of sudden death, while surgical outcomes data now better documents associated risks. Summary: Armed with this updated information, we agree with the need for a national registry to better track patients with AAOCA. We submit that the risks of surgical management outweigh any benefits in the asymptomatic patient with anomalous right coronary artery, and expectant management should also be strongly considered even in asymptomatic patients with anomalous left coronary artery. [ABSTRACT FROM AUTHOR]

Objectives: We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes. Methods: The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006. Results: The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect closure (10%). The incidence of residual septal defects and the degree of left atrioventricular valve regurgitation (LAVVR) did not differ by repair type. The median interval of intensive care stay were 4 days, ventilation use 2 days, and total hospitalization 8 days. All were independent of the presence of trisomy 21 (80% of the cohort). The in-hospital mortality rate was 2.5% (3/120). The overall 6-month mortality rate was 4% (5/120). The presence of associated anomalies and younger age at surgery were independently associated with a longer hospital stay. The age at repair was not associated with residual ventricular septal defect or moderate or greater LAVVR at 6 months. Moderate or greater LAVVR occurred in 22% at 6 months, and the strongest predictor for this was moderate or greater LAVVR at 1 month (odds ratio, 6.9; 95% confidence interval, 2.2–21.7; P < .001). Conclusions: The outcomes after repair of complete atrioventricular septal defect did not differ by repair type or the presence of trisomy 21. An earlier age at surgery was associated with increased resource use but had no association with the incidence of residual ventricular septal defect or significant LAVVR. [ABSTRACT FROM AUTHOR]

Objectives: Temporary epicardial pacing wires are commonly placed during pediatric cardiac surgery. Data are sparse on postoperative pacing in this population. The objective of this study was to determine the frequency of use and identify predictors for the use of temporary epicardial pacing wires. Methods: Perioperative data were prospectively collected on all patients who underwent cardiac surgery at our institution (n = 162). Results: A total of 117 (72%) patients had temporary epicardial pacing wires placed. Postoperatively, 23 (20%) of 117 patients had hemodynamic improvement with the use of temporary epicardial pacing wires. Indications for pacing were slow junctional rhythm (11/23 [48%]), junctional ectopic tachycardia (7/23 [31%]), pace termination of supraventricular tachycardia (3/23 [13%]) and atrial flutter (1/23 [4%]), and complete heart block (1/23 [4%]). By using univariate analysis, single-ventricle anatomy, heterotaxy, the Fontan procedure, use of circulatory arrest, intraoperative arrhythmia, pacing in the operating room, and use of vasoactive medications were predictors for hemodynamic improvement with the use of temporary epicardial pacing wires (P < .05). On multivariate analysis, the Fontan procedure, circulatory arrest, and intraoperative arrhythmias were independent predictors (P < .01). When excluding all patients with any of these 3 risk factors, only 2% were paced. Patients with clinically significant pacing had longer chest tube drainage (P < .01) and intensive care unit length of stay (P < .01). There were no complications associated with temporary epicardial pacing wires. Conclusions: The Fontan procedure, use of circulatory arrest, and intraoperative arrhythmias were associated with hemodynamic improvement with postoperative pacing and might represent indications for empiric intraoperative placement of temporary epicardial pacing wires. Patients without these risk factors were less likely to require pacing. Temporary epicardial pacing wires were safe and useful in the management of arrhythmias after pediatric cardiac surgery. [Copyright &y& Elsevier]

The article discusses the repair of truncus arteriosus (TA) through an operation using an interrupted aortic arch. This defect has been observed with a large subarterial ventricular septal defect (VSD). The seriousness of the sickness is revealed by tachypnea which signifies decrease in PVR. An operation is needed to close septal defects, separate the circulation of the pulmonary and systemic systems and repair aortic arch. An echocardiography is recommended before the operation.

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 &y& Elsevier]

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. [Copyright &y& Elsevier]

Abstract: 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-β, 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. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

Cardiac retransplantation is often the only therapy to treat GV or other causes of allograft failure. Previous reports of retransplantation have conflicting results. In this series of 18 re-transplants in 16 patients from 1984–2005, indications for retransplantation were: GV (67%); GV with cellular rejection (28%); acute graft failure (2.5%); and chronic graft failure (2.5%). Mean age at retransplantation was 12.3 (range: 0.7–22) years with a mean primary graft survival of 5.3 years (range: 8 days–10.5 years). There was no short-term mortality with only three deaths at 4, 10, and 16 years post-retransplantation. Fourteen of 18 patients had risk factors for adverse outcomes following retransplantation: ECMO support in one patient prior to retransplantation; impaired renal function in three patients; elevated panel reactive antibody screen in seven patients; a history of PTLD in five patients; and a recent episode of rejection (13–36 days) in four patients. One-, five- and ten-year survival after retransplantation was 100%, 83% and 66%, respectively, comparable to survival after primary transplantation. Freedom from rejection was not significantly different between primary and retransplantations. All patients who underwent treatment for PTLD had excellent results after retransplantation with one recurrence 16 months after retransplant. Overall, patients had excellent survival after retransplantation even in those with risk factors for poor outcome. [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]