Faculty Bibliography

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.

BACKGROUND: An increasing number of patients with congenital heart disease (CHD) are reaching adulthood and may require heart transplantation. The survival of these patients after listing and transplantation has not been evaluated. METHODS AND RESULTS: A total of 41 849 patients (aged >18 years) were listed for primary transplantation during 1995-2009. Patients with a history of CHD (n=1035; 2.5%) were compared with those with other causes (non-CHD group) (n=40 814; 97.5%); 26 055 (62.3%) reached transplantation and were subdivided into those with (reoperation group; n=10 484; 40.2%) and without (nonreoperation group; n=15 571; 59.8%) a previous sternotomy. Survival on the waiting list was similar between groups, but mechanical ventricular assistance was not associated with superior survival to transplantation among CHD patients. CHD patients were more likely to have body mass index <18.5 at transplantation (P<0.0001), were younger, and had fewer comorbidities. Early mortality among patients with CHD was high (reoperation, 18.9% versus 9.6%; P<0.0001; nonreoperation, 16.6% versus 6.3%; P<0.0001), but by 10 years, overall survival was equivalent (53.8% versus 53.6%). Analysis was limited by the lack of specific information regarding the CHD diagnosis in most patients. CONCLUSIONS: Adults with CHD have high 30-day mortality but better late survival after heart transplantation. Mechanical circulatory assistance does not improve waiting list survival in these patients. This may be due to a combination of highly complex reoperative surgery and often poor preoperative systemic health.

OBJECTIVE: The relationship between volume and outcome in many complex surgical procedures is well established. BACKGROUND: No published data has examined this relationship in pediatric cardiac transplantation, but low-volume adult heart transplant programs seem to have higher early mortality. METHODS: The United Network for Organ Sharing (UNOS) provided center-specific data for the 4647 transplants performed on patients younger than 19 years old, 1992 to 2007. Patients were stratified into 3 groups based on the volume of transplants performed in the previous 5 years at that center: low [<19 transplants, n = 1135 (24.4%)], medium [19-62 transplants, n = 2321(50.0%)], and high [>/=63 transplants, n= 1191 (25.6%)]. A logistic regression model for postoperative mortality was developed and observed-to-expected (O:E) mortality rates calculated for each group. RESULTS: Unadjusted long-term survival decreased with decreasing center volume (P<0.0001). Observed postoperative mortality was higher than expected at low-volume centers [O:E ratio 1.39, 95% confidence interval (CI) 1.05-1.83]. At low volume centers, high-risk patients (1.34, 0.85-2.12)--especially patients 1 year old or younger (1.60, 1.07-2.40) or those with congenital heart disease (1.36, 0.94-1.96)--did poorly, but those at high-volume centers did well (congenital heart disease: 0.90, 0.36-1.26; age<1 year: 0.75, 0.51-1.09). Similar results were obtained in the subset of patients transplanted after 1996. In multivariate logistic regression modeling, transplantation at a low-volume center was associated with an odds ratio for postoperative mortality of 1.60 (95% CI, 1.14-2.24); transplantation at a medium volume center had an odds ratio of 1.24 (95% CI, 0.92-1.66). CONCLUSION: The volume of transplants performed at any one center has a significant impact on outcomes. Regionalization of care is one option for improving outcomes in pediatric cardiac transplantation

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.

Application of the Fontan procedure has allowed the survival of many patients with univentricular cardiac lesions into mid adulthood. Despite its ingenious design, implementation, and thoughtful modifications, its attendant hemodynamic perturbations persist; central venous hypertension and reduced cardiac output. These aberrations in physiology have led to pernicious changes in organ function. A more thorough understanding of these derangements and attempts at Fontan revision have temporized, yet the morbidity and patient attrition persists. Mechanical assistance to normalize the circulation is being investigated and holds some promise. At present, cardiac transplantation remains the last stage of palliation for many.

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.

Although cardiac tumours are rare, such tumours are increasingly being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. Cardiac haemangiomas account for less than one-twentieth of all primary cardiac tumours, with most being diagnosed in the neonatal period. We report 3 instances of successful neonatal resection of cardiac haemangioma subsequent to prenatal diagnosis. Such diagnosis is important in perinatal management, since early surgical intervention provides a good prognosis