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Invited commentary

Mosca, Ralph S
PMID: 22541186
ISSN: 0003-4975
CID: 166513

Outcomes in pediatric cardiac transplantation with a positive HLA cross-match

Richmond, Marc E; Hsu, Daphne T; Mosca, Ralph S; Chen, Jonathan; Quaegebeur, Jan M; Addonizio, Linda J; Lamour, Jacqueline M
Previous studies have shown poor outcomes in pediatric heart transplant recipients with a high PRA or a positive direct donor-recipient cross-match. This study describes outcomes in patients with a positive cross-match at a large pediatric program. Pediatric heart transplant patients at a large single center between January 1993 and July 2009 were reviewed; those with cross-match data were analyzed. Cross-match data were available in 242/262 (92.4%) patients. Indications for transplant were cardiomyopathy (58%), CHD (32%), and retransplant (7%). PRA was >/=10% in 31/213 (14.6%) patients. A retrospective cross-match was positive in 17/31 (55%) patients with PRA >/=10% and 0/182 with PRA <10%. In positive cross-match patients, rejection frequency in the first year post-transplant was higher than negative cross-match patients (1.69 vs. 0.96 episodes/pt year, p = 0.014). There was no difference in rejection frequency after the first year post-transplant (0.18 vs. 0.12 episodes/pt year, p = 0.14). Overall survival was not significantly different between the groups with a median follow-up time of 4.5 yr. Heart transplantation in patients with a positive cross-match may result in good medium-term survival but a higher frequency of early rejection. Further investigation is warranted to define which patients with a positive cross-match will do poorly.
PMID: 22017728
ISSN: 1397-3142
CID: 178197

Multiple risk factors before pediatric cardiac transplantation are associated with increased graft loss

Auerbach, Scott R; Richmond, Marc E; Chen, Jonathan M; Mosca, Ralph S; Quaegebeur, Jan M; Addonizio, Linda J; Hsu, Daphne T; Lamour, Jacqueline M
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.
PMID: 21892650
ISSN: 0172-0643
CID: 178198

Potential Uses of Cord Blood in Cardiac Surgery

Mosca, Ralph S
Despite advances in the fields of prevention, medical intervention and surgical therapy, cardiovascular disease remains a major public healthcare issue. A promising area of research is the potential application of regenerative therapies with pluripotential stem cells to reduce the burden of heart disease and its sequelae. Umbilical cord blood, a rich source of multiple populations of nonembryonic stem cells, will be a valuable resource and has the potential to advance therapeutic options for patients with acquired and congenital heart disease.
PMCID:3771130
PMID: 24089649
ISSN: 2090-9195
CID: 952332

Invited commentary

Srichai, Monvadi G; Mosca, Ralph S
PMID: 21718844
ISSN: 1552-6259
CID: 134919

Surgical management of complete atrioventricular septal defect: associations with surgical technique, age, and trisomy 21

Atz, Andrew M; Hawkins, John A; Lu, Minmin; Cohen, Meryl S; Colan, Steven D; Jaggers, James; Lacro, Ronald V; McCrindle, Brian W; Margossian, Renee; Mosca, Ralph S; Sleeper, Lynn A; Minich, L LuAnn
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.
PMCID:3098918
PMID: 21163497
ISSN: 0022-5223
CID: 178201

A Case of Berry Syndrome Type 2B

Mannelli, Lorenzo; Mosca, Ralph; Henry, Gillian; Srichai-Parsia, Monvadi Barbara
We present a case of a 34-day-old baby boy with congestive heart failure and differential cyanosis. Based on echocardiography and confirmed with computed tomography angiography, the following abnormalities were detected: aortopulmonary window, aortic origin of the right pulmonary artery, patent ductus arteriosus, and hypoplasia/coarctation of the aorta. No other congenital abnormalities were detected, and a diagnosis of Berry Syndrome type 2B was made. No preoperative cardiac catheterization or angiocardiography was needed. The patient underwent complete surgical repair of the aortopulmonary window and reconstruction of the hypoplastic aortic arch. This case highlights the value of cross-sectional imaging with computed tomography angiography in the evaluation of complex congenital heart disease
PMID: 21392264
ISSN: 1747-0803
CID: 135569

Listing and transplanting adults with congenital heart disease

Davies, Ryan R; Russo, Mark J; Yang, Jonathan; Quaegebeur, Jan M; Mosca, Ralph S; Chen, Jonathan M
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.
PMID: 21300954
ISSN: 0009-7322
CID: 178200

Increased short- and long-term mortality at low-volume pediatric heart transplant centers: should minimum standards be set? Retrospective data analysis

Davies, Ryan R; Russo, Mark J; Hong, Kimberly N; Mital, Seema; Mosca, Ralph S; Quaegebeur, Jan M; Chen, Jonathan M
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
PMID: 21183849
ISSN: 1528-1140
CID: 134158

Predictors for hemodynamic improvement with temporary pacing after pediatric cardiac surgery

Ceresnak, Scott R; Pass, Robert H; Starc, Thomas J; Hordof, Allan J; Bonney, William J; Mosca, Ralph S; Liberman, Leonardo
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.
PMID: 20656300
ISSN: 0022-5223
CID: 178203