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Objectives: Interventricular septal hematoma (IVSH) is a rare complication, which may result from ventricular septal defect (VSD) repair. IVSH can result in conduction and/or hemodynamic abnormalities related to impaired ventricular filling or outflow tract obstruction. We report the novel use of extracorporeal membrane oxygenation (ECMO) for management., Methods: Echocardiography reports (January 1980 to December 2016) were searched for the term "hematoma" in our institutional database and reviewed to determine appropriate cases. Charts and imaging (reports) data were abstracted. All intraoperative and select postoperative echocardiograms were reread by a pediatric cardiologist., Results: N = 12 patients with IVSH. Mean age and weight at surgery were 59 ± 41 days and 3.4 (2.9-5.1) kg, respectively, while the most frequent diagnosis was tetralogy of Fallot. Although all patients had intraoperative transesophageal echocardiography (TEE), only 55% (6 of 11, missing [m] = 1) of IVSH were discovered intraoperatively. Of the 5 patients not discovered intraoperatively (m = 1), IVSH was postoperatively detected secondary to arrhythmia/decompensation by echocardiogram 10.1 ± 7.9 hours postoperatively. Five patients (42%) were managed with ECMO (1 unable to separate from bypass). Overall mortality was 33%. For patients in whom ECMO was used, 2 of 5 (40%) survived. Mean time to IVSH resolution in all survivors was 20 ± 185 days., Conclusions: IVSH from VSD repair can result in clinical decompensation and mortality. This may relate to the high proportion missed intraoperatively. ECMO should be considered an important modality, which can allow for IVSH resolution. However, considerations must be made to allow for appropriate anticoagulation to avoid hematoma expansion and repeat imaging during ECMO to continually assess the interventricular septum., (Copyright © 2019. Published by Elsevier Inc.)

Objectives: To investigate the prevalence of hearing loss after cardiac surgery in infancy, patient and operative factors associated with hearing loss, and the relationship of hearing loss to neurodevelopmental outcomes., Study Design: Audiologic and neurodevelopmental evaluations were conducted on 348 children who underwent repair of congenital heart disease at the Children's Hospital of Philadelphia as part of a prospective study evaluating neurodevelopmental outcomes at 4 years of age. A prevalence estimate was calculated based on presence and type of hearing loss. Potential risk factors and the impact of hearing loss on neurodevelopmental outcomes were evaluated., Results: The prevalence of hearing loss was 21.6% (95% CI, 17.2-25.9). The prevalence of conductive hearing loss, sensorineural hearing loss, and indeterminate hearing loss were 12.4% (95% CI, 8.8-16.0), 6.9% (95% CI, 4.1-9.7), and 2.3% (95% CI, 0.6-4.0), respectively. Only 18 of 348 subjects (5.2%) had screened positive for hearing loss before this study and 10 used a hearing aid. After adjusting for patient and operative covariates, younger gestational age, longer postoperative duration of stay, and a confirmed genetic anomaly were associated with hearing loss (all P < .01). The presence of hearing loss was associated with worse language, cognition and attention (P <.01)., Conclusions: These findings suggest that the prevalence of hearing loss in preschool children after heart surgery in infancy may be 20-fold higher than in the 1% prevalence seen in the general population. Younger gestational age, presence of a genetic anomaly, and longer postoperative duration of stay were associated with hearing loss. Hearing loss was associated with worse neurodevelopmental outcomes., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation., Methods and Results: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P =0.006) and lateral tunnel Fontan (HR, 4.3; P =0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P =0.007), Norwood operation (HR, 2.3; P =0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P =0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P <0.001) and hypoplastic left heart syndrome (HR, 2.0; P =0.01)., Conclusions: In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation., (© 2017 American Heart Association, Inc.)

Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (interquartile range 1.4 to 11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% confidence interval [CI] 38 to 55). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (hazard ratio [HR] 1.7, 95% CI 1.2 to 2.4, p = 0.001), pleural drainage >14 days (HR 1.5, 95% CI 1.01 to 2.2, p = 0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06 to 1.3, p = 0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17 to 76, p <0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96 to 99). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course., Methods and Results: Children who underwent biventricular repair of a conotruncal anomaly from January 1, 2006, to June 30, 2013, and had a postoperative transthoracic echocardiogram were included. Images were reviewed for residual intramural or nonintramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was postoperative hospital length of stay. A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2 mm in size. Forty-nine patients (11%) had intramural VSDs, and 207 (47%) had nonintramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared with those with nonintramural VSDs or no residual VSD (14 of 49 [29%] versus 15 of 207 [7%] versus 6 of 186 [3%]; P<0.0001). In addition, those with intramural VSDs had longer postoperative hospital length of stay compared with those with nonintramural VSDs or no residual VSD (20 days [interquartile range, 11-42 days] versus 7 days [interquartile range, 5-14 days] versus 6 days [interquartile range, 4-11 days]; P=0.0001). These associations remained significant after adjustment for known risk factors for poor outcomes, including residual VSD size and operative complexity., Conclusions: Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer postoperative hospital length of stay. It is important to recognize intramural VSDs in the postoperative period., (© 2015 American Heart Association, Inc.)

Objectives: The aim of this study was to evaluate Fontan peri-operative outcomes for 771 consecutive patients., Background: Since the initial description by Fontan, mortality associated with the Fontan operation has declined substantially. However, postoperative effusions remain a significant challenge. Effusions are a key determinant of postoperative length-of-stay and have been shown to be associated with the development of protein-losing enteropathy and with decreased survival., Methods: This study was a single-center, retrospective review of 771 patients who underwent Fontan palliation from 1992 to 2009., Results: Patients were divided into 3 eras dictated by shift in clinical practice. Overall mortality was 3.5%, 1% since 1996. Importantly, age at Stage II palliation decreased from Era 1 to Era 3 (7.1 vs. 5.9 months; p = 0.0001), whereas age at Fontan increased (1.7 vs. 2.8 years; p = 0.0001). The proportion of patients with prolonged hospital stay (46.7% vs. 8.2% vs. 19.5%, p < 0.001) decreased substantially after Era 1. A diagnosis of hypoplastic left heart syndrome and longer operative support times were associated with prolonged pleural drainage (odds ratio [OR]: 2.17, p < 0.001; OR: 1.2, p = 0.001) and hospital stay (OR: 1.48, p = 0.05; OR: 1.34, p < 0.001). In patients who underwent invasive assessment, higher pulmonary artery pressure was associated with death (OR: 1.37, p < 0.001) and prolonged hospital stay (OR: 1.09, p = 0.019). Pulmonary arterial pressure ≥15 mm Hg was 90% specific for discriminating unfavorable outcomes., Conclusions: Mortality in the modern era is rare, whereas postoperative pleural drainage remains the dominant morbidity. Elevated pulmonary artery pressure seems to be a marker of unfavorable outcome. Continued investigation is warranted to determine whether medical interventions or alterations to operative strategy can alter peri-operative results and improve long-term outcomes., (Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: Low weight at birth is a risk factor for increased mortality in infants undergoing surgery for congenitally malformed hearts. There has been a trend towards performing surgery in patients early, and for amenable lesions, in a single stage rather than following initial palliative procedures. Our goal was to report on the current incidences of morbidities and mortality in infants born with low weight and undergoing surgery for congenital cardiac disease., Methods: We made a retrospective review of the data from patients meeting our criterions for entry from July, 2000, through July, 2004. The criterions for inclusion were weight at birth less than or equal to 2500 grams, and congenital cardiac malformations requiring surgery during the initial hospitalization. A criterion for exclusion was isolated persistent patency of the arterial duct. We assessed preoperative, intraoperative, and postoperative variables., Results: We found a total of 105 patients meeting the criterions for inclusion. The median weight at birth was 2130 grams, and median gestational age was 36 weeks. The most common morbidity identified was infections of the blood stream. Infections, and chronic lung disease, were associated with increased length of stay. Survival overall was 76%. Patients with hypoplastic left heart syndrome, or a variant thereof, had the lowest survival, of 62%. The needs for cardiopulmonary resuscitation, or extracorporeal membrane oxygenation, post-operatively were the only factors identified as independent risk factors for mortality., Conclusion: Patients undergoing surgery during infancy for congenital cardiac disease who are born with low weight have a higher mortality and morbidity than those born with normal weight.

Background: The prevalence of perioperative stroke in infants undergoing operations for congenital heart disease has not been well described. The objectives of this study were to determine the prevalence of stroke as assessed by postoperative brain magnetic resonance imaging (MRI), characterize the neuroanatomic features of focal ischemic injury, and identify risk factors for its development., Methods: Brain MRI was performed in 122 infants 3 to 14 days after cardiac operation with cardiopulmonary bypass, with or without deep hypothermic circulatory arrest. Preoperative, intraoperative, and postoperative data were collected. Risk factors were tested by logistic regression for univariate and multivariate associations with stroke., Results: Stroke was identified in 12 of 122 patients (10%). Strokes were preoperative in 6 patients and possibly intraoperative or postoperative in the other 6 patients, and were clinically silent except in 1 patient who had clinical seizures. Arterial-occlusive and watershed infarcts were identified with equal distribution in both hemispheres. Multivariate analysis identified lower birth weight, preoperative intubation, lower intraoperative hematocrit, and higher blood pressure at admission to the cardiac intensive care unit postoperatively as significant factors associated with stroke. Prematurity, younger age at operation, duration of cardiopulmonary bypass, and use of deep hypothermic circulatory arrest were not significantly associated with stroke., Conclusions: The prevalence of stroke in infants undergoing operations for congenital heart disease was 10%, half of which occurred preoperatively. Most were clinically silent and undetected without neuroimaging. Mechanisms included thromboembolism and hypoperfusion, with patient-specific, procedure-specific, and postoperative contributions to increased risk.

Objective: The risk of death during the interstage period remains high after stage 1 reconstruction for single ventricle lesions, despite improved surgical results. The purpose of this study is to identify risk factors for interstage death and to describe the events leading to interstage death., Methods: A nested case-control study was conducted of 368 patients who underwent stage 1 reconstruction at a single center between January 1998 and April 2005., Results: Among the 313 (85%) hospital survivors, there were 33 (10.5%) interstage deaths. Cases more frequently presented with intact or restrictive atrial septum (9 [27%] vs 4 [4%]; P < .001), were older at the time of surgery (5 [2-40] vs 3 [1-42] days; P = .005), had more postoperative arrhythmias (12 [36%] vs 15 [15%]; P = .01), and a higher incidence of airway or respiratory complications (12 [36%] vs 19 [19%]; P = .04). By multivariate analysis, only intact atrial septum (odds ratio 7.6; 95% confidence intervals 1.9-29.6; P = .003) and age at operation greater than 7 days (odds ratio 3.8; 95% confidence intervals 1.3-11.2; P = .017) were predictors of interstage death., Conclusions: The presence of intact atrial septum and older age at the time of surgery are associated with a higher risk of interstage death. In addition, postoperative arrhythmia and airway complications are associated with a higher risk of interstage death in univariate analysis. The results of this study provide a focus for interstage monitoring and risk stratification of these high-risk infants, which may improve overall survival.

Background: To our knowledge, late electrophysiologic outcomes after the Ross procedure have not been described. The purpose of this study was to assess rhythm and conduction disturbances at midterm follow-up after the Ross procedure., Methods: A cross-sectional analysis of Ross procedure survivors (January 1, 1995 to December 31, 2005) followed at our institution was performed, including resting and 24-hour ambulatory electrocardiography (Holter monitoring). Rhythm and conduction disturbances were described, and predictors of arrhythmia were identified., Results: Of 64 eligible patients, 47 (71%) participated. Median age at surgery was 8.7 years (age range, 34 days to 34 years). Twenty-five patients (53%) had isolated aortic valve disease and 22 (47%) had complex left-sided heart disease. At median follow-up of 8.9 years (range, 2.6-11.1 years), 46 patients (98%) exhibited sinus rhythm. Sinus node dysfunction (SND), defined as a pause of 2 seconds or longer or bradycardia for age, was present in 7 patients (15%). Complete heart block requiring a pacemaker occurred in 2 patients (4%). Ventricular tachycardia (VT) was present in 7 patients (15%), including nonsustained VT in 5 patients on Holter monitoring, and sustained VT in 2 patients requiring defibrillator placement. In multivariate analysis, concurrent arch repair at the time of the Ross operation (p = 0.04), longer cross-clamp time at the time of Ross operation (p = 0.04), and right ventricular outflow tract obstruction on follow-up echocardiogram (p = 0.03) were associated with SND. Longer cross-clamp time (p = 0.03) was also associated with VT, along with older age at surgery (p = 0.06 for trend)., Conclusions: At midterm follow-up after the Ross procedure, rhythm and conduction disturbances occur in one third of patients, including SND in 15%, atrioventricular block in 4%, and VT in 15%. Routine surveillance for late arrhythmias after the Ross procedure is warranted.

Background: Hyperglycemia has been associated with worse outcome after traumatic brain injury and cardiac surgery in adults. It is not known whether postoperative hyperglycemia results in worse neurodevelopmental outcome after infant cardiac surgery., Methods: Secondary analysis of postoperative glucose levels was performed in infants younger than 6 months of age enrolled in a prospective study of genetic polymorphisms and neurodevelopmental outcomes who were undergoing repair of two-ventricle cardiac defects. Neurodevelopmental outcomes at 1 year of age were assessed with the Bayley Scales of Infant Development-II, yielding two indices: Mental Developmental Index and Psychomotor Developmental Index., Results: Surgical repair was performed in 247 infants with 1 in-hospital and 3 late deaths. Neurodevelopmental evaluation was performed in 188 of 243 (77%) survivors. Glucose levels at cardiac intensive care unit admission and during the first 48 postoperative hours were available for 180 of 188 patients. Mean admission glucose was 328 +/- 106 mg/dL; maximum glucose was 340 +/- 109 mg/dL. At least one glucose was greater than 200 mg/dL in 160 of 180 patients, and 49 of 180 patients (27%) had a glucose greater than 400 mg/dL. Only 1 patient had a glucose less than 50 mg/dL. Female sex (p = 0.02), but no other patient or operative variable, was associated with higher glucose levels. Mean Mental Developmental Index and Psychomotor Developmental Index were 90.6 +/- 14.9 and 81.6 +/- 17.2, respectively. Hyperglycemia was not associated with lower Mental Developmental Index and Psychomotor Developmental Index scores for the entire cohort or for neonates alone., Conclusions: Hyperglycemia is common early after infant cardiac surgery, but is not associated with worse neurodevelopmental outcome at 1 year of age.

Objective We sought to define the neurodevelopmental status of school-aged survivors of total anomalous pulmonary venous connection repaired during infancy. Methods All school-aged survivors of total anomalous pulmonary venous connection repair performed at a single institution were eligible. Thirty children returned for neurologic examination and neurodevelopmental testing. Results The median age at total anomalous pulmonary venous connection repair was 16 days (range, 1-141 days), and age at testing was 11 years (range, 6-19 years). Pulmonary venous return was supracardiac in 14 patients, infracardiac in 12 patients, cardiac in 3 patients, and mixed in 1 patient. Preoperative obstructed total anomalous pulmonary venous connection was present in 6 patients. Circulatory arrest was used in all repairs, with a median duration of 35 minutes (range, 17-55 minutes). At follow-up, microcephaly (head circumference <5%) was present in 28%. Neuromuscular examination was suspect or abnormal in 27%. Mean Full-scale IQ (95.3 +/- 18.5) and Verbal IQ (98.6 +/- 20.2) were not different from population norms, but Performance IQ (92.3 +/- 16.9) was significantly lower than population norms ( P = .02). Fine motor skills and visual-motor coordination were significantly impaired ( P < .01 for Grooved Pegboard and Test of Visual-Motor Integration). Patients with total anomalous pulmonary venous connection also had difficulty with tests of attention (Test of Everyday Attention for Children, P < .01), but results of tests of memory function were not significantly different from population norms. Conclusions School-aged survivors of infant total anomalous pulmonary venous connection repair exhibit a significant incidence of neurodevelopmental difficulties. Fine motor function, visual-motor integration, and attention are the most commonly affected domains. Evaluation of these children is indicated to identify those who are at risk for learning disabilities and who could benefit from early intervention.

Purpose: Neonatal seizures are relatively common and an important early sign of acute encephalopathy in those who survive infant heart surgery. The contemporary occurrence of seizures in this setting is not fully known, and their electrographic characteristics are incompletely described. This study describes the characteristics of electrographic neonatal seizures (ENSs) in contemporary infants with congenital heart disease (CHD) surgically repaired by using cardiopulmonary bypass, with or without deep hypothermic circulatory arrest., Methods: Consecutive infants undergoing heart surgery were monitored by video-EEG for 48 h postoperatively to establish the time of first seizure, total number of ENSs, site(s) of ENS(s) origin and other characteristics., Results: ENSs occurred in 21 (11.5%) of 183 infants. None had clinically visible seizures. The mean time to the first ENS was 21 h (range, 10-36 h). The total number of ENSs among the entire cohort was 1,429. Mean total number of ENSs per patient over a 48-h period was 72 (range, 1-217). Phenobarbital administration was associated with a > or =50% reduction in seizure counts in five (41.7%) of 12 subjects., Conclusions: ENSs were relatively common in a large, contemporary cohort of infants after infant heart surgery. A wide variation was noted in seizure burden, but many experienced numerous seizures. Electrographic neonatal seizures are a candidate outcome end point in future neuroprotection trials in this patient population.

Background: Epicardial pacemakers are often required in children and young adults who cannot undergo a transvenous system because of patient size, vascular barriers, or significant residual intracardiac shunts. Prophylactic epicardial pacing leads, placed at the time of concomitant congenital heart surgery, may reduce a late thoracotomy or sternotomy. The efficacy of prophylactic epicardial leads in the pediatric population is unknown., Methods: A retrospective review of the cardiovascular surgery and pacemaker databases at The Children's Hospital of Philadelphia identified all patients less than or equal to 21 years of age, who underwent placement of an epicardial pacing lead between January 1, 1990 and December 31, 2002. Prophylactic epicardial pacing leads placed at the time of a concomitant congenital heart procedure were compared to standard epicardial leads that were connected to a simultaneous programable generator. Pacing and sensing threshold data were obtained in prophylactic epicardial leads at the time of lead retrieval and 6 month follow-up and compared to standard epicardial pacing leads., Results: Twenty-two (13 ventricular, 9 atrial) prophylactic epicardial pacing leads were retrieved in 13 patients at a median of 252 days (7 days to 3.98 years) from the time of initial implant and compared to 256 (164 ventricular, 92 atrial) standard epicardial leads placed in 142 patients. Nineteen (86%) prophylactic epicardial leads had acceptable pacing and sensing thresholds at lead retrieval. Only 1 patient with atrial and ventricular leads had poor pacing and sensing at retrieval and required a redo-sternotomy for placement of new atrial and ventricular epicardial pacing leads. For the remaining atrial (n = 7) and ventricular (n = 12) prophylactic epicardial leads, there was no significant difference in pacing (atrial, 1.59 +/- 1.1 microJ; ventricular, 1.98 +/- 1.9 microJ) or sensing (atrial, 3.6 +/- 1.8 mV; ventricular, 13.8 +/- 4.4 mV) compared to standard pacing (atrial, 2.1 +/- 1.8 microJ; ventricular, 1.9 +/- 3.4 microJ) and sensing (atrial, 3.3 +/- 1.7 mV; ventricular, 11.3 +/- 5.3 mV) epicardial leads. Six-month follow-up pacing and sensing thresholds were not significantly different between the prophylactic and standard epicardial pacing leads., Conclusions: Prophylactic epicardial pacing leads can be successfully placed and retrieved in a subset of children and young adults who will likely require pacing at a later date. Prophylactic leads have comparable pacing and sensing qualities at lead retrieval and short-term follow-up compared to standard epicardial leads. Consideration for prophylactic epicardial pacing leads will likely reduce the need for a late thoracotomy or sternotomy.

Background: Primary graft failure, or circulatory insufficiency immediately after transplantation, frequently occurs after pediatric cardiac transplantation and is the most common cause of death after infant transplantation. Risk factors for pediatric primary graft failure are poorly defined., Methods: We retrospectively reviewed donor, procedural and recipient characteristics for primary graft failure in 165 pediatric cardiac transplant recipients (median age at transplant 1.1 years) by multivariatle logistic regression. Primary graft failure was defined as the need for mechanical circulatory support or use of multiple intravenous inotrope/pressors, including epinephrine, for circulatory support within the first 24 hours after transplantation., Results: Primary graft failure occurred in 54 patients (33%); 24 patients (15%) required mechanical support for their graft failure; and primary graft failure was the cause of death or graft loss in 10 patients. Recipient risk factors associated with an increased risk of primary graft failure included diagnosis of congenital heart disease and a need for mechanical support before transplantation. Ventilator support before transplantation and maximal pulmonary vascular resistance index were risk factors for the development of isolated right ventricular graft failure. Donor risk factors associated with an increased risk for primary graft failure included increasing donor recipient weight and body surface area ratios; increasing donor ischemic time; anoxia as a cause of death; and increasing cardiopulmonary resuscitation time. Donor blood type O+ and hyperdynamic donor systolic function were associated with a decreased risk of primary graft failure., Conclusions: Multiple donor, recipient and procedural risk factors, including the type and severity of heart disease in the recipient before transplantation, are associated with primary graft failure after pediatric cardiac transplantation. Avoidance of matching high-risk donors to high-risk recipients may improve morbidity and mortality after transplantation.

Objective: To identify factors associated with mortality in children with heart disease managed with extracorporeal membrane oxygenation (ECMO)., Design: Retrospective chart review., Setting: Tertiary care university-affiliated children's hospital., Patients: All pediatric cardiac intensive care unit patients managed with ECMO between January 1, 1995, and June 30, 2001., Interventions: None., Results: During the study period, 137 patients were managed with ECMO in the pediatric cardiac intensive care unit. Of the 137 patients, 80 (58%) survived > or =24 hrs after decannulation, and 53 (39%) survived to hospital discharge. Patients managed with ECMO following cardiac surgery were analyzed separately from patients not in the postoperative period. Factors associated with an increased probability of mortality in the postoperative patients were age <1 month, male gender, longer duration of mechanical ventilation before ECMO, and development of renal or hepatic dysfunction while on ECMO. Single ventricle physiology and failure to separate from cardiopulmonary bypass were not associated with an increased risk of mortality. Cardiac physiology and indication for ECMO were not associated with mortality rate. Although longer duration of ECMO was not associated with increased mortality risk, patients with longer duration of ECMO were less likely to survive without heart transplantation., Conclusions: In a series of 137 patients managed with ECMO in a pediatric cardiac intensive care unit, survival to hospital discharge was 39%. In postoperative patients only, mortality risk was increased in males, patients <1 month old, patients with a longer duration of mechanical ventilation before initiation of ECMO, and patients who developed renal or hepatic failure while on ECMO.

Objective: To identify pre- and intraoperative variables associated with postoperative acute neurologic events (ANEs), including seizures and coma, in newborn survivors of congenital heart surgery undergoing deep hypothermic circulatory arrest (DHCA), and to risk-stratify this population on the basis of preoperative risk variables for the purpose of designing future neuroprotection trials., Methods: Survivors of newborn heart surgery who were enrolled in a neuroprotection trial provided a comprehensive database for the evaluation of pre- and intraoperative variables that influence the postoperative occurrence of ANEs (seizures or coma). Patients with hypoplastic heart syndrome were excluded. After characterization of the study population, stepwise logistic regression, combined with clinical judgment, was used to identify variables that were most likely to be associated with an increased risk of seizures in the study sample and that were most likely to be generalized to other populations., Results: Data were available on 164 nonhypoplastic left heart syndrome survivors who underwent newborn heart surgery using DHCA. ANEs occurred in 31 (18.9%) including "seizures alone" (n = 28), "coma alone" (n = 2) or "seizures and coma" (n = 1). A preoperative risk model was constructed demonstrating that infants with a genetic condition and aortic arch obstruction had a 47.8% risk of ANEs compared with all other remaining infants, who had a 9.9% risk. It was also found that prolonged DHCA time (>or=60 minutes) can be a significant risk for infants who have a preexisting genetic condition; however, infants who have genetic conditions and do not undergo prolonged DHCA time or have an aortic arch obstruction are not at increased risk of ANEs., Conclusions: This study provides new information about the occurrence of ANEs after newborn heart surgery. Seizures or coma, which appeared in approximately 19% of all non-hypoplastic left heart syndrome survivors, were not random events but were significantly associated with specific types of congenital heart disease, the presence of genetic conditions, and prolonged DHCA time. The 3 identified variables permitted individual cases to be assigned to low-, intermediate-, or high-risk categories. Because neonatal seizures are a good surrogate marker of long-term neurologic outcome, these models provide useful information to stratify individual patients for risk of seizures in future neuroprotection trials.

Background: Junctional ectopic tachycardia (JET) occurs commonly after pediatric cardiac operation. The cause of JET is thought to be the result of an injury to the conduction system during the procedure and may be perpetuated by hemodynamic disturbances or postoperative electrolyte disturbances, namely hypomagnesemia. The purpose of this study was to determine perioperative risk factors for the development of JET., Methods: Telemetry for each patient admitted to the cardiac intensive care unit from December 1997 through November 1998 for postoperative cardiac surgical care was examined daily for postoperative JET. A nested case-cohort analysis of 33 patients who experienced JET from 594 consecutively monitored patients who underwent cardiac operation was performed. Univariate and multivariate analyses were conducted to determine factors associated with the occurrence of JET., Results: The age range of patients with JET was 1 day to 10.5 years (median, 1.8 months). Univariate analysis revealed that dopamine or milrinone use postoperatively, longer cardiopulmonary bypass times, and younger age were associated with JET. Multivariate modeling elicited that dopamine use postoperatively (odds ratio, 6.2; p = 0.01) and age less than 6 months (odds ratio, 4.0; p = 0.02) were associated with JET. Only 13 (39%) of the patients with JET received therapeutic interventions., Conclusions: Junctional ectopic tachycardia occurred in 33 (5.6%) of 594 patients who underwent cardiac operation during the study period. Postoperative dopamine use and younger age were associated with JET. It may be speculated that dopamine should be discontinued in the presence of postoperative JET.

Background: Early outcomes after repair of total anomalous pulmonary venous connection continue to improve; however, little information is available concerning long-term functional status and quality of life., Methods: One hundred patients underwent isolated total anomalous pulmonary venous connection repair at The Children's Hospital of Philadelphia between 1983 and 2001. Medical records were reviewed and a standardized questionnaire was administered to guardians of survivors., Results: Median age at repair was 15.5 days (range, 1 to 563 days). Overall hospital mortality was 14%, decreasing from 19% before 1995 to 5% after 1995. At 15 years after repair, actuarial survival was 84% and freedom from late death or reintervention for hospital survivors was 85%. At a median follow-up of 5.9 years (range, 0 to 17.7 years) 64% of guardians described their child's overall health as excellent, 27% good, 9% fair, and 0% poor. With regard to school performance, 40% of children were characterized as above average, 29% average, 4% below average, and 27% were in special education classes or had repeated grades. By multivariable logistic regression, the presence of associated chromosomal or noncardiac syndromes and pulmonary venous obstruction were found to be significant factors with regard to parental assessment of both overall health and school performance., Conclusions: The majority of children who undergo isolated total anomalous pulmonary venous connection repair can expect an excellent long-term functional outcome. Factors present before operation, such as pulmonary venous obstruction and associated anomalies, can influence overall health and school performance in the long term.

Objective: To review and describe pneumatosis intestinalis (PI) in children who have undergone thoracic organ transplantation and evaluate potential risk factors., Methods: We retrospectively reviewed abdominal radiographs obtained from June 1992 through September 2000 in all pediatric (age <21 years) thoracic organ recipients who survived at least 1 week after transplantation. In this group, a case was defined as an episode of radiographically confirmed PI; those without PI were assigned as controls. Variables analyzed included demographic data, gastroenteritis history (stool cultures or symptoms of gastroenteritis), and transplant-related factors (ie, graft type, rejection history, immunosuppression regimen). Significance was defined as P <.05., Results: Over this 8-year period, PI occurred in 8 (7%) of 116 patients (0.86% annual risk). No child had >1 diagnosed episode of PI. Of these 8 cases, 7 presented with 1 or more abdominal symptoms. Three of these children had rotavirus antigen isolated in their stool, 2 others were noted to have stool positive for Clostridium difficile toxin, and in the other 3, no pathogen was identified. All cases were treated with a regimen of intravenous antibiotics and total parenteral nutrition. There were no deaths; however, 1 patient developed an Aspergillus pulmonary infection during his course of antibiotic therapy, and another underwent an exploratory laparotomy without bowel resection. Significant risk factors included black race (unadjusted odds ratio: 16), younger age at presentation (age <5 years; unadjusted odds ratio: 9), higher steroid dose (steroid dose >0.5 mg/kg/d; unadjusted odds ratio: 7), and a higher tacrolimus level at presentation (tacrolimus level >1; unadjusted odds ratio: 6). PI did not occur with a steroid dose <0.4 mg/kg/d. Variables not associated with increased risk for developing PI included gender, graft type, total white blood cell count, recent antibiotic use, concurrent use of an antimetabolite, cytomegaloviral infection, past use of extracorporeal membrane oxygenation, and graft rejection history., Conclusions: Significant risk factors for the development of PI in our pediatric thoracic organ transplantation population included black race, younger age, higher daily steroid dosing, and a high tacrolimus level at presentation. In the children diagnosed with PI, there were no related deaths, significant gastrointestinal sequelae, or complications. These findings suggest that in this population, PI will often have a benign course when treated aggressively, and that steroid dosing should be reduced to <0.5 mg/kg/d whenever possible.

Background: Thrombus formation is common after a Fontan operation. We investigated the frequency and location of thrombus in our population of children based on the type of Fontan operation performed., Methods and Results: Between January 1987 and January 1999, 592 patients underwent echocardiography after Fontan operation and 52 (8.8%) had intracardiac thrombus. Median age at Fontan operation was 1.9 years (range 0.8 to 35.1). Freedom from thrombus was 92%, 90%, 84% and 82% at 1, 3, 8, and 10 years after Fontan operation, respectively. There was no difference in freedom from thrombus, based on type of operation (atriopulmonary vs. lateral tunnel) or presence of fenestration. Thrombus was detected in the systemic venous atrium in 26 (48%), in the pulmonary venous atrium in 22 (44%), in both atria in 1 (2%), in the hypoplastic left ventricular cavity in 2 (8%), and in the ligated pulmonary artery stump in 1 (2%)., Conclusions: Thrombus formation occurs with equal frequency in all types of modifications and is seen in the pulmonary, as well as the systemic venous atria. Our study suggests that thrombus formation is inherent to the physiology after Fontan operation and is not related to the type of modification performed.

We describe a simple technique for the implantation of left atrial epicardial pacing leads in children with congenital heart disease who have undergone multiple operations. The pulmonary veins are exposed to reveal the pulmonary venous to atrial confluence using a left thoracotomy. A pacemaker lead is secured to the posterior left atrium inferior to the lower pulmonary vein. This approach provides a reliable site for atrial lead placement without the need for extensive dissection.

Objective: To determine whether operations that theoretically jeopardize the sinus node (hemi-Fontan and/or lateral tunnel Fontan procedures) are associated with a greater risk of sinus node dysfunction than those that theoretically spare the sinus node (bidirectional Glenn and/or extracardiac conduit)., Methods: Between January 1, 1996, and December 31, 1999, a prospective cohort study was conducted evaluating the incidence of sinus node dysfunction in patients undergoing a bidirectional Glenn or hemi-Fontan procedure and those in whom the Fontan repair was completed with either an extracardiac conduit or a lateral tunnel. Sinus node dysfunction was defined (1) as a heart rate more than 2 SD below age-adjusted norms or (2) as a predominant junctional rhythm and/or a sinus pause of more than 3 seconds as determined by the resting electrocardiogram and/or ambulatory monitoring at hospital discharge., Results: Fifty-one patients had a bidirectional Glenn shunt (mean age 7.8 +/- 5.1 months) and 79 a hemi-Fontan procedure (mean age 6.9 +/- 2.8 months). The incidence of sinus node dysfunction on postoperative day 1 was significantly higher after the hemi-Fontan (36%) than after the bidirectional Glenn shunt (9.8%); however, by hospital discharge this difference was no longer apparent (hemi-Fontan [8%]; bidirectional Glenn [6%]; P = not significant). No difference in early sinus node dysfunction was discernible after the extracardiac conduit (4/30 [13%]) compared with the lateral tunnel Fontan procedure (6/46 [13%]) (P = not significant). No diagnostic or perioperative variables were predictive of sinus node dysfunction., Conclusions: Avoidance of surgery near the sinus node has no discernible effect on the development of early sinus node dysfunction. Thus, concerns about early sinus node dysfunction should not override patient anatomy or surgeon preference as determinants of which cavopulmonary anastomosis to perform.

The objectives of this study were to determine the validity of the grade of mitral regurgitation (MR) as imaged by intraoperative transesophageal echocardiography (TEE) in predicting the grade of MR at follow-up. Intraoperative TEE and corresponding follow-up transthoracic studies were retrospectively reviewed and the regurgitant jet area to left atrial area ratio was used to quantify the MR. Patient records were reviewed to identify factors contributing to the development of a certain grade of MR. Intraoperative TEE was useful in detecting severe MR that required further repair at the same time. However, discrepancy in the grade of MR at follow-up was noted in 47% of patients (21 of 47) and unchanged grade of MR was found only in 53% of patients (26 of 47). Blood pressures were significantly lower and heart rates higher intraoperatively. Initial preoperative grade of MR and type of atrioventricular canal defect did not predispose for a particular grade of MR at follow-up. The grade of MR by intraoperative TEE does not predict the grade of MR at follow-up as imaged by transthoracic echocardiography.

Lung transplantation is an important treatment option in children with acquired and congenital pulmonary disease. Indications for lung transplantation include pulmonary hypertension, bronchopulmonary dysplasia, pulmonary vein stenosis, and cystic fibrosis. The early outcome following lung transplantation has improved considerably. Longterm complications, however, including infections, bronchiolitis obliterans, and complications of immunosuppression remain significant problems. Donor availability, especially for neonates, continues to limit the utilization of lung transplantation.

Background: Modified ultrafiltration reduces the deleterious effects of cardiopulmonary bypass in children. Patients undergoing repair of single-ventricle cardiac anomalies may be particularly sensitive to these adverse effects, and benefit from the use of modified ultrafiltration., Methods: From January 1995 to June 1996, 120 consecutive cavopulmonary operations were performed at The Children's Hospital of Philadelphia. Procedures included lateral tunnel fenestrated Fontan (n = 50), extracardiac Fontan (n = 5), hemi-Fontan (n = 60), and bidirectional Glenn shunt (n = 5). Modified ultrafiltration was performed after cardiopulmonary bypass in 41 patients, and results were compared by t test with a control group of 79 patients in whom modified ultrafiltration was not used., Results: There was one death for an operative (30-day) mortality of 0.8%. Age, weight, diagnosis, ischemic arrest time, and cardiopulmonary bypass time were similar between the modified ultrafiltration and control groups. Postoperative blood use, chest tube output, the incidence of pleural and pericardial effusions, and hospital stay were all significantly decreased when modified ultrafiltration was used., Conclusions: By lowering the perioperative morbidity of staged cavopulmonary operations, modified ultrafiltration makes an important contribution to improving outcome after the correction of single-ventricle cardiac anomalies.

In the setting of functional single ventricle with pulmonary overcirculation, pulmonary artery banding is frequently used to alleviate symptoms and to prepare for staged repair. At subsequent cavopulmonary anastomosis or Fontan procedure, the pulmonary artery may be ligated at the site of the pulmonary band. This article describes the association of embolic stroke and thrombus in a ligated or divided pulmonary artery stump in three patients with functional single ventricle. These events occurred from 1990 through 1992 among the 1700 inpatient pediatric cardiology admissions at two institutions. The patients, ranging in age from 15 months to 9 years, had cerebral infarctions documented by computed axial tomography scan or magnetic resonance imaging associated with the echocardiographic finding of thrombus in the proximal pulmonary artery stump after the embolic strokes. The strokes occurred 5 days to 5 years after surgery. Two patients had a second infarction within 2 to 5 weeks of the initial stroke. It is concluded that the presence of the ligated pulmonary artery stump may place patients at risk for embolic stroke. Surgical approaches to reduce the risk of thrombus formation should be considered prospectively in this patient group.

Background: Endomyocardial biopsy remains the primary means of rejection surveillance after orthotopic heart transplantation in adults. Perpetual surveillance endomyocardial biopsy has been questioned, however, because of low yield beyond the early posttransplantation period. This issue has not been adequately studied in the pediatric population. The objectives of this study were to define the rate of rejection in infants undergoing orthotopic heart transplantation, correlate rejection with signs and symptoms, and evaluate the utility of surveillance endomyocardial biopsy., Methods: Records of all patients 24 months of age or younger undergoing orthotopic heart transplantation were reviewed; 38 patients underwent 42 transplantation; 256 endomyocardial biopsies were performed for surveillance, cardiac symptoms, noncardiac symptoms, or lowered immunosuppression., Results: There were 22 rejection episodes International Society for Heart and Lung transplantation grade 1B or higher, half of which occurred in neonates 30 days of age or younger. Linearized rejection rates and actuarial freedom from rejection were not different between neonates and older infants. Linearized rejection rates reached a plateau 3 months after orthotopic heart transplantation of 0.07 episodes/100 patient days. No positive surveillance endomyocardial biopsies were obtained beyond 6 months after orthotopic heart transplantation. The probability of a positive biopsy (International Society for Heart and Lung Transplantation grade 1B or higher) was 20% or more for any other indication (odds ratios for rejection were 12.9 for cardiac symptoms, 3.3 for noncardiac symptoms, and 10.8 for lowered immunosuppression as determined by logistic regression more than 6 months after orthotopic heart transplantation)., Conclusions: Rejection rates are not different between neonatal and older infants, and endomyocardial biopsies done solely for surveillance beyond 6 months after orthotopic heart transplantation rarely yield positive results.

Infants with single ventricle and transposition of the great arteries with or without aortic arch obstruction have a poor prognosis due in large part to the development of systemic outflow obstruction, a frequent consequence of pulmonary artery banding. Thus, the initial palliation and long-term treatment options are critical in terms of surgical choices and timing. We report our experience with 9 patients managed by neonatal pulmonary artery banding and early debanding, a Damus-Kaye-Stansel procedure, and either a modified Glenn shunt or a modified Fontan procedure. Some evidence of subaortic stenosis developed in every patient as manifested by a resting gradient across the systemic outflow tract (21.4 +/- 4.2 mm Hg), a small ventricular septal defect relative to the body surface area (1.57 +/- 0.39 cm2/m2), and a small ventricular septal defect relative to the aortic root cross-sectional area (0.70 +/- 0.04 cm2/m2). There were 1 early death and 1 late death after the Damus-Kaye-Stansel procedure. With the exception of 1 patient, the in-hospital course of the survivors was relatively uncomplicated. Two patients with levotransposition of the great arteries have required pacemakers. None of the survivors have residual systemic outflow obstruction. There is trivial or mild pulmonic insufficiency in 5 patients, which is not progressing. One patient had mild to moderate pulmonic insufficiency but died late presumably of an arrhythmia. We conclude that neonatal pulmonary artery banding coupled with planned early debanding, a Damus-Kaye-Stansel procedure, and cavopulmonary anastomosis is a relatively low-risk course for patients with this complex physiology.

Right-sided extracardiac conduits are frequently complicated by obstruction over time. We compared the utility of two-dimensional and Doppler echocardiography and magnetic resonance imaging in the diagnosis of postoperative right-sided obstruction with cardiac catheterization and angiography in 10 patients with xenograft or homograft conduits. Correlation (r = 0.95) between continuous-wave Doppler estimates and catheter pullback pressure gradients across the conduits was excellent. Echocardiography could only visualize five of 10 conduits in their entirety. Magnetic resonance imaging visualized all conduits and showed statistically significant (kappa = 0.58) agreement with angiography in the localization and estimation of severity of a variety of right-sided obstructions in these patients. However, flow voids created by the metallic ring around xenograft valves led to a false negative diagnosis of valvular stenosis in four patients when magnetic resonance imaging was used alone. Doppler studies correctly indicated obstruction in these patients. The combination of magnetic resonance imaging studies and continuous-wave Doppler echocardiography can be useful to noninvasively evaluate right-sided obstruction in postoperative patients with right-sided extracardiac conduits.

Clinical and morphological features are described in a man in whom a pseudoaneurysm arising from the ventricular septum developed after transaortic septotomy for hypertrophic subaortic stenosis. Pseudoaneurysm of the left ventricle or ventricular septum has not been described previously in a patient with hypertrophic cardiomyopathy in whom only the transaortic approach was utilized for septotomy or septectomy or both. In addition, no previous studies have reported pseudoaneurysm arising from the ventricular septum.