Isolated Coarctation of the Aorta: Current Concepts and Perspectives.

Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.

Atrial septostomy in patients supported with venoarterial extracorporeal membrane oxygenation: Analysis of the IMPACT registry data.

BACKGROUND: Patients with severe cardiac dysfunction, supported on extra-corporeal membrane oxygenation (ECMO), develop left atrial hypertension and often warrant atrial decompression. The procedural risks and outcomes, however, are not well established. Objective of this study is to understand the utilization, safety, and outcomes of atrial septostomy in children supported on venoarterial ECMO utilizing the IMPACT registry database. METHODS: Data from 55 independent sites reporting to the registry was used for this descriptive study. Patients supported with cardiac ECMO that underwent percutaneous atrial septostomy (PAS) were included and data analyzed. RESULTS: Between 2011 and 2018, 223 patients underwent atrial septostomy and were reported to the registry. Mean age was 4.65 years with 52% being males. Diagnoses of heart failure, sepsis, arrhythmia, and renal insufficiency were commonly noted prior to ECMO. The procedure was performed urgently or emergently in most cases (80%). PAS was associated with significant procedural complications such as arrhythmia (6.8%), tamponade (5.4%), and unplanned surgery (3.6%) but no procedural mortality. Overall hospital mortality was 46% (103/223) with 46 of these deaths occurring within 7 days of procedure. Regression analysis showed that African American race (vs Caucasian), race other than Caucasian, chronic lung disease and emergent procedure need were significantly associated with early post-procedural mortality (all p < 0.05). CONCLUSION: Using a large multicenter cardiac catheterization registry, we found that left atrial decompression can be performed without procedural mortality but is associated with significant morbidity. Impact of race, chronic lung disease and procedural urgency on early mortality warrant further studies.

IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease.

The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.

Premounted stents for branch pulmonary artery stenosis in children: A short term solution.

OBJECTIVE: Define outcomes of premounted stent implantation (PMS) for branch pulmonary artery stenosis (BPAS). BACKGROUND: PMS for BPAS in children raises concern of long term viability, with limited maximal expansion. METHODS: We reviewed our cardiac database over an 11-year period ending in 2013. Primary endpoint was need for surgical stent intervention (SSI). Other endpoints included acute results and repeat interventions (RI). RESULTS: 82 PMS were implanted in 60 children for BPAS. Median weight was 6.3 (25th -75th 4.6-9.8) kg. Median stent diameter was 6 (range 4-9) mm. Acutely, vessel diameter improved from 2.0 (25th -75th 1.6-3.4) to 5.0 (25th -75th 4.2-5.9) mm (p < 0.001), pressure gradient decreased from 41 (25th -75th 29-50) to 11 (25th -75th 7-18) mmHg (n = 47, p < 0.001), RV:Ao pressure ratio decreased from 100% (25th -75th 85-110%) to 59% (25th -75th 49-74%) (n = 40, p < 0.001). Freedom from SSI was 81% at 1 year and 35% at 5 years. Freedom from RI was 50% at 1 year and 14% at 5 years. 86% of PMS underwent SSI during a concomitant planned cardiac surgery. 45% patients had stent redilation, improving stent diameter from 4.6 (25th -75th 4.1-5.4) to 5.7 (25th -75th 4.9-7) mm (p < 0.001). 1 stent (3%) was able to be fractured longitudinally. CONCLUSIONS: PMS is an effective short term solution for BPAS in children. PMS is associated with expected early need for transcatheter reintervention to accommodate for growth, but also has high rates of SSI.

Direct physician reporting is associated with reductions in radiation exposure in pediatric cardiac catheterizations.

OBJECTIVE: The objective is to quantify radiation dose in children undergoing cardiac catheterization and determine the impact of increased reporting transparency on total radiation exposure. BACKGROUND: Cardiac catheterization (cath) can result in significant radiation exposure in children. There has been growing interest in quantifying and reducing radiation exposure in pediatric cath procedures. Our center underwent a slight change in practice recently that resulted in direct physician reporting of radiation dose following every case. METHODS: We reviewed cath procedures across three different eras in four cath categories: post-heart transplant annual cath, unilateral pulmonary artery (PA) stent placement, pre-Fontan cath, and pre-Glenn cath. The eras were defined as: Era 1, 1/2009-1/2011; Era 2, 1/2011-9/2013; and Era 3, 9/2013-5/2014. In Era 3, the physician performing the cath was responsible for reporting the radiation data. RESULTS: Across the three eras, there were significant decreases in cumulative air KERMA (mGy) among all four cath categories. From Era 2 to Era 3, the greatest decreases in radiation were noted, particularly in dose area product (cGy·cm2) in the transplant annual evaluation and pre-Glenn cases. In Era 1, 2 cases (1.2%) had a frame rate reduction, while in Era 2, 22 cases (12.0%), and in Era 3, 83 cases (21.6%) had frame rate changes (P<0.01). CONCLUSIONS: Increased physician awareness of radiation exposure is associated with a significant reduction in radiation dose across a variety of cath procedures. This is seen not only by the overall reduction in radiation across case types, but also as the frame rate was more frequently changed during individual cases, indicating an important change in physician behavior and practice.

Transcatheter interventions across fresh suture lines in infants and children: an 8-year experience.

OBJECTIVES: The purpose of this study was to examine the outcomes of catheter dilation interventions in general upon surgical anastomotic sites in the immediate postoperative period. BACKGROUND: Early postoperative cardiac catheter intervention is regarded as high-risk, particularly when a dilation intervention across a fresh suture line is performed. METHODS: All catheter dilation interventions performed upon a fresh suture line within 30 days of congenital heart surgery between August 2005 and December 2013 were reviewed. Values are reported as median and interquartile range (IQR). Primary endpoint was procedural success, which was defined as an increase in vessel diameter of >75% of the adjacent normal vessel or 50% increase over pre-dilation diameter. Secondary endpoints included procedural complications and survival to hospital discharge. RESULTS: Forty-five patients, median age 0.15 years (0.03-0.51 years), weight 4.1 kg (3.1-6.4 kg), underwent 62 interventional procedures on median postoperative day 7 (3-13 days). Among the 62 dilation interventions at surgical anastomotic areas, were 30 stent and 32 balloon angioplasty procedures. There were two major complications including: arterial dissection during stent placement and a procedural mortality due to pulmonary artery avulsion during angioplasty. There were 12 deaths (27%) prior to hospital discharge. The median ratio of maximum stent diameter to stenosis diameter was 2.62 (2.27-3.73). The median ratio of maximum balloon diameter to stenosis diameter was 2.27 (1.84-2.94). Stent placement was more likely to result in procedural success (OR 2.1; 95% confidence interval 1.6-2.8). CONCLUSION: Though caution is paramount, early postoperative catheter dilation intervention across fresh suture lines can be performed safely in small, critically ill children.

Cardiac catheterization in the early post-operative period after congenital cardiac surgery.

OBJECTIVES: This study sought to demonstrate that early cardiac catheterization, whether used solely as a diagnostic modality or for the use of transcatheter interventional techniques, can be used effectively and with an acceptable risk in the post-operative period. BACKGROUND: Cardiac catheterization offers important treatment for patients with congenital heart disease. Early post-operative cardiac catheterization is often necessary to diagnose and treat residual anatomic defects. Experience with interventional catheterization to address post-operative concerns is limited. METHODS: This was a retrospective cohort study. The medical and catheterization data of pediatric patients who underwent a cardiac catheterization ≤30 days after congenital heart surgery between November 2004 and July 2013 were reviewed. Patients who underwent right heart catheterization and endomyocardial biopsy after heart transplantation were excluded. RESULTS: A total of 219 catheterizations (91 interventional procedures, 128 noninterventional catheterizations) were performed on 193 patients. Sixty-five interventions (71.43%) were dilations, either balloon angioplasty or stent implantation. There was no difference in survival to hospital discharge between those who underwent an interventional versus noninterventional catheterization (p = 0.93). One-year post-operative survival was comparable between those who underwent an intervention (66%) versus diagnostic (71%) catheterization (p = 0.58). There was no difference in the incidence of major or minor complications between the interventional and diagnostic catheterization cohorts (p = 0.21). CONCLUSIONS: Cardiac catheterization, including transcatheter interventions, can be performed safely in the immediate post-operative period after congenital heart surgery.

Hot topics in tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.

Aortic complications after pediatric cardiac transplantation in patients with a previous Norwood reconstruction.

Despite increasing surgical success with staged palliation of hypoplastic left heart syndrome and its variants, some of these children eventually may require cardiac transplantation. Sixteen (7.8%) of 206 children ≤18 years old undergoing primary heart transplantation had a previous Norwood palliation. Two (12.5%) developed significant aortic problems after transplantation related to the initial homograft reconstruction of the aorta. Patient 1 developed acute graft failure requiring extracorporeal membrane oxygenator support post-transplant. During acute retransplantation 2 days later, the new donor aorta was sewn to a remnant of the initial donor aorta rather than to the heavily calcified reconstructed native aorta. Two months later, the patient required reoperation for acute airway compression from an aortic pseudoaneurysm caused by necrosis of the bridge of aortic tissue from the first transplant. Patient 2 had multiple balloon dilatations of recurrent coarctation after transplantation. Eighteen years post-transplant, during work-up for chronic fever and weight loss, computerized tomography showed a mycotic aneurysm of the reconstructed transverse aorta with contained rupture necessitating removal and replacement of the entire reconstructed aorta. Although uncommon, aortic complications in pediatric heart transplant patients with previous Norwood arch reconstruction can present with unusual manifestations requiring heightened vigilance.

Heart transplantation in children with a Fontan procedure.

BACKGROUND: Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 190 primary pediatric heart transplants. METHODS: Since 1988, 27 (14.2%) of 190 children less than 18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 163 (85.8%) non-Fontan primary transplants, the Fontan patients were similar in age (8.2 ± 5.0 vs 6.5 ± 6.0 years), presensitization, and pretransplant clinical status. More Fontan patients had prior operations (100% vs 50%; p < 0.0001) and needed pulmonary artery reconstruction (100% vs 23.5%; p < 0.0001). Twelve (44%) had protein-losing enteropathy. RESULTS: Donor ischemic times (211 ± 72 vs 170 ± 61 minutes; p = 0.0018) and cardiopulmonary bypass times (197 ± 91 vs 121 ± 53 minutes; p < 0.0001) were greater in the Fontan group as were durations of ventilator support (4.9 ± 6.6 vs 2.6 ± 3.9 days; p = 0.018) and hospital stay (20.2 ± 17.5 vs 14.3 ± 12.4 days; p = 0.0435). The Fontan group had one 30-day mortality. One-year actuarial survival (81.5% vs 84.6%, Fontan vs non-Fontan) and five-year actuarial survival (65.5% vs 66.2%, Fontan vs non-Fontan) were similar, as was rejection incidence at one year (2.0 ± 2.0 vs 1.7 ± 1.9 episodes per patient; p = 0.3972). Five Fontan patients (18.5%) required retransplantation 4.9 ± 3.6 years posttransplant compared with 18 non-Fontan patients (11.0%) retransplanted 5.2 ± 3.4 years posttransplant (p = 0.3346). CONCLUSIONS: Contrary to prior reports, we did not identify any early or midterm disadvantage for children undergoing heart transplantation after a previous Fontan procedure despite more complex transplant operations. We contend that carefully selected children with a failing Fontan circulation can do as well as other children with heart transplantation.

Fibrofatty changes in failed pediatric cardiac allografts.

The pathogenesis of right ventricular fibrofatty changes can be broadly divided into genetic or acquired. The genetic cause is termed arrhythmogenic right ventricular dysplasia, an inherited cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium, and represents an underdiagnosed cardiac entity leading to syncope, recurrent ventricular tachycardias, heart failure, and sudden death. Our study demonstrates that fibrofatty changes can also be seen in pediatric cardiac allografts. Conversely, fat replacement without fibrosis may be seen secondary to infectious myocarditis, chronic inflammation, and ischemia and as part of the aging process. We examined 29 failed cardiac allografts to identify the etiology of graft failure. In this study, 4 patients (13%) had severe right ventricular fibrofatty changes, and when compared with control patients, those with fibrofatty changes had a shorter interval from transplant to graft failure, 2.75 years vs 5.45 years (P  =  0.029). Neither body mass index nor other physiologic parameters found on electrocardiography, echocardiography, or cardiac catherization were different between groups. Furthermore, arrhythmias indicative of arrhythmogenic right ventricular dysplasia were not observed in the study group. This study suggests the fibrofatty infiltration in cardiac allografts is a clinically different entity from arrhythmogenic right ventricular dysplasia and has an unknown etiology. Our study findings suggest that identifying fibrofatty infiltrates in cardiac transplant patients during routine right ventricular biopsy can be a predictive factor for shortened life of the pediatric cardiac allograft.

The IMPACT registry: IMproving Pediatric and Adult Congenital Treatments.

Tremendous advances have occurred in catheter-based interventions for congenital heart disease. Multicenter trials of these advances are either out of date or have been limited in scope. As such little is known on the application of these techniques in the current era. The IMPACT Registry (IMproving Pediatric and Adult Congenital Treatments) will allow us to measure variability in the performance and outcomes of both diagnostic and interventional cardiac catheterization procedures in all children and adults with congenital heart disease. The IMPACT Registry will be harmonized with the Society of Thoracic Surgeons Congenital Heart Disease Database, thereby allowing us to compare catheter-based interventions with surgical interventions when appropriate. The initial release of the registry will only include hospital-based outcomes, but ultimately it will transition to a longitudinal registry. The IMPACT Registry will provide the necessary benchmark tools for quality improvement activities for cardiac catheterization procedures in congenital heart disease.

Donor-recipient race mismatch and graft survival after pediatric heart transplantation.

BACKGROUND: Black recipient race has been shown to predict poorer graft survival after pediatric heart transplantation. We analyzed our single-center experience comparing graft survival by race and the impact of donor-recipient race mismatch. METHODS: One hundred sixty-nine consecutive primary pediatric heart transplant patients were analyzed by donor and recipient race (white recipient, 99; black recipient, 60; other, 10). The groups were similar in preoperative characteristics. There were fewer donor-recipient race matches in blacks compared with whites (10 versus 71; p < 0.0001). RESULTS: Although 30-day and 6-month graft survival was similar for black and white recipients (93.9% and 85.8% versus 93.3% and 83.3%, respectively), overall actuarial graft survival was significantly lower in blacks (p < 0.019). Blacks tended to have a higher incidence of positive retrospective crossmatch (n = 26, 43%) than whites (n = 29, 29%), but this was not statistically significant (p = 0.053). The median graft survival for black recipients was 5.5 years compared with 11.6 years for whites. Donor-recipient race mismatch predicted poorer graft survival (5-year graft survival 48.9% versus 72.3%; p = 0.0032). The median graft survival for donor-recipient race-matched patients was more than twice that for mismatched patients (11.6 years versus 4.4 years). Cox proportional hazard analysis showed that donor-recipient race mismatch neutralized the effect of race on graft survival. CONCLUSIONS: Graft survival after pediatric heart transplantation is inferior for black recipients compared with white recipients. These differences may be explained by a high incidence of donor-recipient race mismatch, which also predicts poorer outcome for all racial groups with pediatric heart transplantation. These data may have implications for future donor allocation schemes.

Costs associated with ventricular assist device use in children.

BACKGROUND: Ventricular assist devices (VADs) allow children with severe heart failure to be bridged to successful heart transplantation. Ventricular assist devices are being used with increasing frequency in the pediatric population, and newer devices allow even young infants to be supported. Ventricular assist device implantation and maintenance, however, is quite expensive, and the cost-effectiveness of VAD use in adults has been questioned. To date, an economic analysis of VAD support in children has not been undertaken. METHODS: We used Pediatric Health Information System, an administrative database of the Child Health Corporation of America (a consortium of Children's Hospitals in North America), to determine the outcomes and costs related to VAD use in children. Data on patients younger than 18 years of age from 2002 to 2007 were reviewed. Hospital charges were converted to costs based on cost-to-charge ratios. Projected survival for subjects who were successfully bridged to heart transplant was derived from published data. The model assumed that if a VAD strategy were not used, the majority of subjects would have required extracorporeal membrane oxygenation support as a bridge to transplantation. Cost-utility was expressed as cost per quality-adjusted life years saved. All future costs and benefits were discounted at 3%. RESULTS: There were 145 children who underwent VAD implantation at 19 centers in North America. The median age at admission was 8.5 years; the range was newborn to 17.7 years. The median duration of VAD support was 43 days (range, 1 to 465 days). Ninety-four patients (65%) survived to heart transplantation. Thirty-nine (27%) patients died during hospitalization. There were 12 patients (8%) who had VAD explantation and survival to hospital discharge. The mean hospital cost was $624,798. When compared with a strategy of extracorporeal membrane oxygenation support, the calculated cost-utility for VAD as a bridge to transplantation was $119,937 per quality-adjusted life year saved. When key assumptions were changed, the cost-utility varied from $88,304 to $282,320 per quality-adjusted life year saved. CONCLUSIONS: Ventricular assist devices allow an effective bridge to heart transplantation in children. Under base-case assumptions, the cost-effectiveness ratios exceed the threshold of $100,000 per quality-adjusted life year saved. The cost-utility of this strategy, however, is comparable to a number of other life-saving technologies.

Right ventricular outflow tract and pulmonary artery stents in children under 18 months of age.

OBJECTIVE: To report our experience on patients less than 18 months of age in whom stents were placed either in the right ventricular outflow tract (RVOT) or pulmonary arteries. BACKGROUND: Although percutaneous placement of intravascular stents in congenital heart disease is common practice, there are few reports regarding placement in young children. METHODS: Retrospective review of our database identified 19 patients less than 18 months of age who underwent placement of 26 stents in either the RVOT or pulmonary arteries. Data evaluated were patient age, gender, weight, site of narrowing, type of stent, gradient across the area of narrowing pre- and post-stent placement, vessel size pre- and post-stent placement, gradient, and vessel size on followup, and ability to redilate the stent if necessary. RESULTS: Mean patient age was 9.7 months (range 0.5-17.5 months). There were 14 males and 5 females with a mean weight of 7.1 kg (range 2.3-10.2 kg). Five patients had stents placed in the RVOT and 14 patients had 21 stents placed in the branch pulmonary arteries. Two Johnson and Johnson P188 stents were used, 23 premounted Genesis biliary stents and 1 other. Gradient across the stenotic area decreased from a mean of 52.9 mm Hg (range 20-80 mm Hg) to 15 mm Hg (range 3-40 mm Hg) (P<.001). CONCLUSIONS: Percutaneous placement of intravascular stents in young children is a viable procedure, can provide excellent relief of stenoses, and increases vessel size in the short term. Although many of these stents cannot be dilated to adult size, their efficacy in small infants and children in whom further surgery will ultimately be required makes them desirable for this group of patients.

OKT3 treatment in refractory pediatric heart transplant rejection.

BACKGROUND: The anti-lymphocyte monoclonal antibody OKT3 has been shown to be effective in the management of steroid-resistant and/or fulminant heart transplant rejection in adults. In addition, some studies suggest that OKT3 may have a role in the management of transplant coronary artery disease (TxCAD). To date, there are limited data regarding the use of OKT3 treatment of refractory rejection or graft failure in children. Our study examines OKT3 treatment in steroid-resistant rejection, rejection with hemodynamic compromise, and TxCAD in children. METHODS: Thirty-eight patients received 53 courses of OKT3 for treatment of rejection and/or graft dysfunction. Primary indications for OKT3 were steroid-resistant rejection (n = 27), rejection with hemodynamic compromise (n = 22), and TxCAD (n = 4). Resolution of rejection was considered absence of biopsy-proven rejection (< grade 2) or resolved TxCAD. RESULTS: OKT3 use in steroid-resistant rejection was associated with a lower incidence of rejection in the 3 months after OKT3 than 3 months before OKT3, median rejection episodes of 2.5 vs 0, p < 0.0001. In rejection with hemodynamic compromise, 20 subjects (91%) demonstrated improved hemodynamics after OKT3 and survived to hospital discharge. The use of OKT3 treatment for TxCAD failed to demonstrate resolution or improvement in angiographic TxCAD in any subject. Only 5 OKT3 treatment courses were stopped secondary to severe adverse side effects. CONCLUSIONS: OKT3 treatment in refractory pediatric heart transplant rejection is efficacious in acute rejection. OKT3 management in pediatric TxCAD is less clear, with no proven benefit identified in this study. OKT3 use in pediatric refractory heart rejection has significant side effects, but is tolerable and safe with close monitoring.

Cardiac retransplantation in childhood: analysis of data from the United Network for Organ Sharing.

OBJECTIVE: For children in whom graft failure develops after cardiac transplantation, retransplantation is often considered. Although some centers have reported equivalent results for retransplantation as for primary transplantation, this strategy remains controversial. We sought to examine outcomes after retransplantation in children and to identify risk factors for mortality. METHODS: United Network for Organ Sharing records of heart transplantation for subjects younger than 18 years from 1987 to 2004 were reviewed. Indications for retransplantation and patient characteristics were evaluated. Analysis was performed with proportional hazards regression, controlling for other potential risk factors. RESULTS: Among the 4227 pediatric heart transplants, there were 219 retransplants. The most common indication for retransplantation was coronary allograft vasculopathy (51%). The mean interval from initial heart transplant to retransplantation was 4.7 +/- 3.8 years. Forty-two retransplants (19%) were performed within 180 days of primary transplantation. Survivals at 1, 5, and 10 years after retransplantation were 79%, 53%, and 44%, respectively. In multivariate analysis, retransplantation was associated with significantly higher mortality than primary transplantation (odds ratio 1.67, 95% confidence interval 1.32-2.12, P < .001). Patients who underwent retransplantation within 180 days of primary transplantation had a significantly lower 1-year survival than did other retransplant recipients (53% vs 86%, respectively, P < .02). Subjects who required mechanical ventilation before retransplantation also had poorer survival (P < .03). CONCLUSION: Survival after cardiac retransplantation in children is inferior to that after primary transplantation. Although results are acceptable, the impact of retransplantation on the availability of donor hearts requires further consideration.

The short term effects of cardiopulmonary bypass on neurologic function in children and young adults.

OBJECTIVE: Cognitive deficits are common in adults following surgery utilizing cardiopulmonary bypass (CPB). A previous retrospective study suggested that surgical closure of an ASD in children was associated with neurologic injury, while transcather therapy was not. In a prospective study, we sought to determine whether neurologic deficits occur following repair of non-complex congenital heart lesions in school-age children and young adults. METHODS: Inclusion criteria were: age between 5 and 20 years, cardiac surgery utilizing CPB without deep hypothermic circulatory arrest, and no prior cardiac surgery. Patients underwent psychometric testing 1-3 days prior to surgery and re-evaluation 7-18 days after surgery. In order to determine the test/re-test effect an age-matched cohort of children undergoing transcather closure of ASD under general anesthesia was also evaluated. The primary outcome measures were verbal and picture memory. Additional psychometric tests included: computerized performance test (CPT) and Digit Span (DS). Forty-one patients were enrolled, 29 undergoing surgery with CPB and 12 controls. Surgical procedures included ASD closure (n=13), VSD closure (n=10), resection of sub-aortic stenosis (n=3), mitral valvuloplasty (n=3). Mild hypothermia was used in all cases. The mean duration of CPB was 54+/-22 min. RESULTS: There was no significant difference in any of the psychometric test scores between subjects undergoing surgery with CPB or controls. CONCLUSIONS: There are no marked adverse neurologic effects of CPB in school-age children and young adults undergoing non-complex open-heart surgery. These data are important in counseling patients and families and should be considered in the debate as to the relative merits of transcather versus open-heart repair of various heart lesions.

Cardiac retransplantation in children.

BACKGROUND: Experience with pediatric cardiac retransplantation is limited. Outcomes should be inspected to insure proper use of donor hearts. METHODS: Of 152 pediatric heart transplantations, we performed 20 retransplants in 17 children (3 had a second retransplant). The retransplant children were older than the primary transplant children (11.1 +/- 4.4 years versus 7.1 +/- 6.0 years; p = 0.005). Excluding 1 early retransplant, the interval from primary transplant to retransplant was 5.5 +/- 3.3 years (range, 1.1 to 11.1). The retransplant patients were clinically more ill than the primary transplant patients (United Network for Organ Sharing status I, 75% versus 63%; mechanical circulatory support or dialysis, 20% versus 3.8%). RESULTS: Donor ischemia time (188 versus 165 minutes) and cardiopulmonary bypass time (127 versus 127 minutes) were not significantly different for the retransplant patients. Excluding 1 retransplant patient who required a tracheostomy, days on the ventilator (2.7 versus 2.7), days on inotropic support (3.0 versus 3.2), intensive care unit days (7.2 versus 6.7), and hospital days (15.9 versus 13.8) were similar in the retransplant group. Freedom from rejection at 90 days and 1 year was not different in the retransplant patients. Actuarial patient survival in the patients undergoing first retransplant was similar to the primary transplant patients at 30 days (95% versus 94.7%), 1 year (94.1% versus 80.7%), and 3 years (78.4% versus 73.1%). Two of 3 children receiving a third transplant died within 1 year of redo retransplantation. CONCLUSIONS: Cardiac retransplantation can be performed in children with results comparable with those for primary transplantation despite increased clinical acuity. These early results suggest that cardiac retransplantation in children is a reasonable therapeutic option. Children with repeat retransplantation do not fare as well.

Mechanical thrombectomy using the AngioJect in a child with congenital heart disease.

Acute thrombosis of a vascular channel can be a serious and perhaps fatal complication. We describe a patient who developed complete thrombosis of the left pulmonary artery following catheter placement of a stent in this vessel shortly after open heart surgery. The thrombosis was successfully removed using the Possis AngioJet mechanical thrombectomy catheter.