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Innominate artery grafts are often utilized in pediatric cardiac surgery and very rarely lead to complications, including infection. Here, we present a unique case of an infant who underwent repair of coarctation of the aorta and hypoplastic arch using a Gore-Tex graft for antegrade cerebral perfusion. The graft subsequently became infected with Pseudomonas and formed a pseudoaneurysm with resultant tracheal compression. The presentation, diagnosis, and management of this mycotic pseudoaneurysm are described.

Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality.This was a retrospective review of 938 consecutive patients undergoing congenital heart surgery at a single institution over a 2-year timeframe. The number of postoperative surgical and unplanned diagnostic procedures were counted and the impact on hospital length of stay and mortality was assessed.581 of the 938 (62%) patients had zero postoperative diagnostic or surgical procedures. These patients had a median length of stay of 6 days with a single operative mortality (0.2%). 357 of the 938 (38%) patients had one or more postoperative diagnostic or surgical procedures. These patients had a total of 1586 postoperative procedures. There was a significant correlation between the number of postoperative procedures and both hospital length of stay and mortality (p .001). Patients who required 10 or more postoperative procedures had a median hospital length of stay of 89 days and had a 50% mortality. There were no survivors in patients who had 15 or more postoperative procedures.The data demonstrate that the number of postoperative procedures was highly correlated with both hospital length of stay and mortality.

Jacobsen syndrome (JS) is a rare genetic condition characterized by intellectual disability, hematologic abnormalities, and congenital heart defects. A male infant presented at birth with phenotypic findings of JS and echocardiographic findings of hypoplastic left heart syndrome (HLHS). Array comparative genomic hybridization was performed at age three days and revealed an 8.1 Mb terminal deletion on the long arm of chromosome 11, consistent with JS. At five days of age, a hybrid stage 1 procedure was performed. At age 46 days, he underwent a Norwood operation followed by bidirectional Glenn at age six months. He is presently 23 months old and doing well. With careful consideration of the individual patient and comorbidities associated with JS, we propose that at least a subset of patients with JS and HLHS can do well with staged surgical palliation.

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.

Background: Unifocalization and pulmonary artery reconstructions have been developed to treat complex disorders of pulmonary artery development. These procedures require extremely long periods of cardiopulmonary bypass (CPB) to facilitate surgical repair. The objective of this study was to document the prevalence of complications in patients undergoing unifocalization or pulmonary artery reconstructions associated with prolonged periods of CPB. Methods: This was a retrospective review of 100 consecutive patients who underwent unifocalization (n = 66) or pulmonary artery reconstructions (n = 34) with CPB times in excess of five hours. Thirty-eight of these operations were primary procedures, whereas 62 were reoperations. Results: The median age at surgery was 15 months, median duration of CPB was 473 minutes, median number of postoperative complications was 5, and the median length of hospital stay was 24 days. The most frequently encountered complications were low cardiac output (43%), open sternum (40%), reintubation (24%), arrhythmia (17%), and bronchoscopy (17%). There was a correlation between the total number of complications and overall length of hospital stay ( R 2 = 0.64). Major adverse cardiac events (MACE) occurred in 11 patients with one hospital mortality. Patients who experienced MACE had a median length of stay that was 35 days longer (56 vs 21 days) than patients who did not experience MACE. Conclusions: The data demonstrate that complications were relatively frequent in this cohort of patients and had a linear association with hospital length of stay. Major adverse cardiac events were encountered at a modest prevalence but had a profound impact on measures of outcome.

Background This study reports the long-term outcomes using glutaraldehyde-treated cryopreserved homograft pericardium (CPH) in neonates, infants, children, and young adults undergoing congenital cardiac surgery. Methods A retrospective review was performed of all patients at a single institution (Rady Children’s Hospital, San Diego, CA) who had undergone surgical implantation with CPH between 2006 and 2016. The study identified 134 consecutive patients who underwent implantation of a total of 276 patches. The baseline demographic characteristics, primary cardiac diagnosis, surgical characteristics, operative reports, and postoperative catheterization and reoperation reports were analyzed. The use of CPH was categorized by specific anatomic insertion site. Results The median age at patch implantation was 1.47 years (range, 1 day to 31.6 years). The numbers and locations of patch use were 124 for pulmonary arterial repair, 57 for repair of the aorta, 49 for septal repair, and 43 at other sites. At a median follow-up of 5.29 years, 9 patients had died (6.7%), but none of those deaths were related to CPH. Twelve patients (8.96%) underwent reoperations, and 18 patients (13.4%) underwent catheter interventions at sites of CPH implantation. The 10-year freedom from patch-induced reoperation and catheter intervention rates were 88.5% and 86.9%, respectively. Overall patch failure–free survival was 85.8% and 79.0% at 5 and 10 years, respectively. Conclusions The use of CPH patch in the surgical correction of congenital heart disease is effective and durable, as evidenced by the low reintervention rates. These results are comparable to the early and midterm outcomes of other similarly used surgical patches..

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

Background Dacron tube grafts have been used in the surgical management of cardiovascular disease since the 1970s. Complications at the site of the anastomosis have been well described. Non-anastomotic failure is far less common. We present a series of four patients who presented with complications of non-anastomotic failure of woven Dacron tube grafts. Methods A retrospective chart review of four patients who presented to our institution between March 2014 and March 2017 with clinical complications of a Dacron tube graft was conducted. Results All four patients underwent a staged surgical repair for an interrupted aortic arch between the years of 1988 and 2001. All four patients underwent revision of their original interposition graft (Gore-Tex, W.L. Gore & Associates, Flagstaff, AZ) with implantation of a Hemashield woven Dacron tube graft (Maquet, Rastatt, Germany). From 13 to 22 years postimplant of the Dacron tube graft, all patients presented with symptoms or clinical evidence of primary graft failure. Two patients underwent urgent surgical intervention and did not survive. One patient underwent attempted surgical intervention, which was aborted in the setting of profuse bleeding, and ultimately had an endovascular rescue of the tube graft with a Zenith Alpha endograft (Cook Medical, Bloomington, IN). One patient underwent elective endovascular intervention prior to onset of symptoms. Conclusion Non-anastomotic failure of woven Dacron tube grafts can occur in the thoracic aorta in young adults and may be managed with endovascular techniques.

Percutaneous therapies for congenital heart disease have been evolving rapidly despite limited investment from industry. The Melody transcatheter pulmonary valve (Medtronic, Inc, Minneapolis, MN USA) replacement therapy represents an important advancement in this arena. It has been approved in the United States for use in the pulmonary position, on a Humanitarian Device Exemption status. Off-label use of the Melody transcatheter pulmonary valve has extended to the mitral, pulmonary, and aortic valves, especially in previously implanted valves with prosthetic valve degeneration. The single-ventricle patient poses additional challenges. However, there exists one report in the English literature of a patient undergoing Melody transcatheter neoaortic valve replacement after the patient developed severe neoaortic regurgitation after Fontan palliation. Here, we describe a patient with hypoplastic left heart syndrome, palliated with a Norwood modified Blalock-Taussig shunt, with a progressively regurgitant quadricusp neoaortic valve who underwent bioprosthetic valve replacement. There was early prosthetic valve degeneration after a year of bioprosthesis implantation. As he was declined for transplantation, he underwent successful perventricular Melody valve-in-valve replacement.

Preoperative tachycardia is uncommon before an initial Fontan procedure. Catheter intervention can risk complications in small patients. A patient with fetal tachycardia and double inlet left ventricle underwent Norwood and bidirectional Glenn procedures. She had persistent tachycardia. A three-dimensional electroanatomic mapping system was modified for the operative procedure; it was used to identify the arrhythmia substrate and cryoablate an atrioventricular nodal tachycardia. Mapping added 20 minutes to the operation. She is arrhythmia-free and off medication after 4 months. This three-dimensional system was successfully adapted for an intraoperative SVT ablation in this small patient, and it may be useful in other settings.

Catheter ablation of arrhythmias in small patients has inherent significant risk because of the patient, catheter, and lesion size. Heart block, perforation, and coronary damage may occur. This report shows adaptation of a three-dimensional electroanatomic mapping system for intraoperative use in a small patient with Ebstein's anomaly who was undergoing a cone procedure and a bidirectional Glenn procedure. Use of this technique gave clear identification of the epicardial insertion points of two accessory pathways causing Wolff-Parkinson-White syndrome and resistant episodes of supraventricular tachycardia. Successful three-dimensional mapping and cryoablation were performed with a short procedure time and no complications. The patient remains without preexcitation or arrhythmia 6 months postoperatively.

Graphical abstract, Highlights • ARCAPA is a rare congenital anomaly. • Anomalous coronary artery origins are often isolated and diagnosed incidentally. • Echocardiography is the initial modality for diagnosing coronary artery anomalies. • Diagnosis by echocardiography requires a high index of suspicion. • Patients with suspected coronary anomalies should undergo advanced imaging.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases. We report a rare case of an infant with APW, interrupted aortic arch, and pulmonary atresia with intact interventricular septum and right ventricle–dependent coronary circulation. This report describes the anatomy of this lesion set, the complex surgical palliation that was required, and the management of postoperative complications.

In 2007 we began a hybrid program for hypoplastic left heart syndrome (HLHS) variants to potentially improve outcome in high-risk patients. During implementation we offered both hybrid and Norwood approaches to all risk categories. The purpose of this study was to perform a comparative analysis of intermediate survival.Newborns were evaluated jointly for high-risk characteristics, including birth weight less than 2.5 kg, prematurity (especially35 weeks), central nervous system abnormalities, multiorgan failure, intact or severely restrictive atrial septum, severe ventricular dysfunction, and severe atrioventricular valve regurgitation. We prefer Norwood for standard risk and hybrid for high risk, but all groups crossed over into all treatment pathways resulting in the following 5 treatment groups: standard risk Norwood; high-risk Norwood; standard risk hybrid ductal stent (HDS); high-risk hybrid DS; and high-risk hybrid prostaglandin E1 (HPGE). We reviewed all consecutive patients from 2007 to 2012, obtained follow-up, and analyzed the results.Sixty-eight newborns presented (median 2.96 kg, 8 days); 29 (43%) were high and 39 (57%) were standard risk. There were 14 stage I hospital deaths strongly associated with risk: 3 of 39 standard (7.7%) and 11 of 29 high (38%, p = 0.002). Stage I discharge mortality was highest for high-risk Norwood and high-risk HPGE groups (p0.001). Actuarial survival up to 5 years demonstrated superior survival for Norwood versus hybrid (78.1% vs 56.4%, p = 0.0182). With risk stratification there was suboptimal survival for all 3 high-risk groups (p = 0.003); HDS fared better than HPGE but had higher birth weight (p0.001).While a risk-stratified approach for HLHS variant patients with selective use of hybrid palliation resulted in acceptable stage I mortality, the longer term mortality for high-risk patients remains higher than for standard risk regardless of treatment modality. Intrinsic patient risk factors (rather than treatment modality) likely determine long-term outcome in experienced centers. Our current high-risk approach has evolved to HPGE application with Norwood conversion whenever deemed medically possible.

Background Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS. Methods Retrospective review of all patients who underwent transcatheter SPS stenting from 1/2006 to 12/2013. Results Of 229 surgically implanted SPS, 25 transcatheter stent interventions were performed in 22 patients. The majority had pulmonary atresia (n = 9) or HLHS (n = 10). Their median age was 4 ms (range 10 days to 4 years) and median weight 4.9 kg (range 3–14). Nine had a central and 15 had a BT shunt with a median shunt size of 3.75 mm (range 3–6). The interval from shunt placement to intervention was 1.9 ms (range 4 days–3.8 years). The indication for intervention was increasing cyanosis in10 patients and delaying final repair in 9. Two patients were on ECMO at the time of intervention. The median shunt diameter increased from 2.3 to 4.1 mm and oxygen saturation from 72 to 85% (P

Objectives We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS). Background Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection. Methods A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed. Results Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P

Cardiac structural disorders can be detected noninvasively with echocardiography or cardiac MRI. Neither of these techniques, however, visualizes emboli, although thrombi can often be seen on valves or walls. Transcranial Doppler ultrasound (TCD) has been shown to be exquisitely sensitive at detecting microemboli, which may be a harbinger of impending macroembolization resulting in stroke.1,2 This report demonstrates the presence of a major valvular thrombus not visualized on echocardiography, but detected by TCD microemboli detection.

Communicating bronchopulmonary foregut malformations are uncommon anomalies. This report describes a case of "esophageal bronchus" managed successfully in a newborn with a nearly total pneumonectomy.

The New York State Department of Health developed a Pediatric Cardiac Surgery Reporting System in 1991 that contains detailed information on demographics, diagnoses, procedures, comorbidities, complications, and discharge information for every pediatric cardiac surgery patient in the state. The Department and the Congenital Cardiac Services Subcommittee of the Department's Cardiac Advisory Committee have used data from this system to assess, assure, and improve quality of care and to generate public reports on an ongoing basis. Two reports (one covering 3years and the other covering 4years) have been published, and a third report, comprising the years 2006–2009, will be released shortly. These reports contain information on patient diagnoses, patient severity groups, and risk-adjusted in-hospital mortality rates for all hospitals that perform pediatric cardiac surgery. The goal of the DOH and the CAC is to improve the quality of cardiac care in NYS. Providing the hospitals and cardiac surgeons in NYS with data about their own outcomes for these procedures allows them to examine the quality of the care they provide and to identify areas that need improvement. These data are fed back to providers on an ongoing basis in addition to in the published reports. The overall results of this program of ongoing review show that significant progress is being made. The volume of surgeries and the number of hospitals where they are performed have both decreased dramatically in the years since NYS first began reporting risk-adjusted outcomes for pediatric cardiac surgery. In 1997, 16 hospitals performed 1749 pediatric cardiac surgeries. By 2009, there were only 10 hospitals performing 1304 surgeries. The average number of surgeries per hospital in 1997 was 109 compared to 139 in 2010. Many factors, including increased use of catheter-based therapies, may have contributed to the overall decline in the number of surgeries. The overall mortality rate for pediatric cardiac surgery dropped from 4.08% in 2002 to 2005 period to a mortality rate of 3.35% in 2006 to 2009 period with no major changes in overall patient risk, although part of this decrease may also be due to improvements in the field in general. A multivariable analysis demonstrated that a dedicated pediatric cardiac ICU proved to be a significant independent predictor of mortality. The methods for assessing patients' risk of in-hospital mortality have changed with each successive report, and this study describes the current method and the risk factors used in the method. The Department's other initiatives for improving quality, including consolidation of services, linking processes and structures of care to outcomes, and other regulatory actions, are also described.

Objective. The purpose of this study was to establish an objective criterion to help assess adequacy of the transverse arch in newborns with coarctation and thereby optimize preoperative surgical decision making. Methods. Echocardiograms of 47 patients

Permanent cardiac pacing in pediatric patients presents challenges related to small patient size, complex anatomy, electrophysiologic abnormalities, and limited access to cardiac chambers. Epicardial pacing currently remains the conventional technique for infants and patients with complex congenital heart disease. Pacemaker lead failure is the major source of failure for such epicardial systems. The authors hypothesized that a retrocostal surgical approach would reduce the rate of lead failure due to fracture compared with the more traditional subrectus and subxiphoid approaches. To evaluate this hypothesis, a retrospective chart review analyzed patients with epicardial pacemaker systems implanted or followed at Rady Children’s Hospital San Diego between January 1980 and May 2007. The study cohort consisted of 219 patients and a total of 620 leads with epicardial pacemakers. Among these patients, 84% had structural congenital heart disease, and 45% were younger than 3 years at time of the first implantation. The estimated lead survival was 93% at 2 years and 83% at 5 years. The majority of leads failed due to pacing problems (54%), followed by lead fracture (31%) and sensing problems (14%). When lead failure was adjusted for length of follow-up period, no significant differences in the rates of failure by pocket location were found.

Background Late left pulmonary artery (LPA) stenosis occurs commonly after the Norwood procedure, and complicates subsequent stages. Compression by the neoaorta and ductal stump may favor flow into the right pulmonary artery, resulting in LPA hypoplasia. We hypothesize that an early compromise of LPA flow contributes to late LPA stenosis, and have modified our shunt to compensate. Methods We reviewed 34 consecutive neonates undergoing the Norwood procedure between 1999 and 2002, and morphometric data from angiograms obtained before the bidirectional cavopulmonary anastomosis (BDCPA). The Norwood technique included an autologous arch reconstruction with or without augmentation, and a polytetrafluoroethylene Blalock-Taussig shunt (BTS). Starting February 2001, the distal shunt was modified from an end-to-side construction to an oblique anastomosis directed into the retroaortic LPA. Results Norwood survival was 82%. LPA stenosis required plasty in 10 of 13 (77%) premodification survivors, and in 2 of 9 (22%) postmodification ( p = 0.027). Bypass time was 151 ± 65 minutes with LPA plasty versus 95 ± 50 minutes without. Mortality (15% vs 0%), hospital stay (25 ± 35 vs 9 ± 6 days), and incidence of subsequent interventions were correspondingly higher with LPA stenosis. Ten of 13 patients (77%) with a BTS insertion point outside the central region of the pulmonary artery required LPA plasty, versus 2 of 9 (22%) with an insertion nearer to the center ( p = 0.027). Conclusions An oblique distal BTS anastomosis directed leftward onto the retroaortic pulmonary artery at the time of the Norwood procedure may prevent late LPA stenosis and its attendant morbidity.

William I. Norwood, working at the Boston Children's Hospital, developed and refined a surgical procedure that permitted babies born with hypoplastic left-heart syndrome (HLHS) to survive and enjoy life ([1][1]). The high mortality rates reported during the 1980s have gradually declined as various

We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS).Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection.A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed.Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P 0.05). Post discharge, there were seven reinterventions in the DS group and two reinterventions in the SPS group.DS may provide a safe and effective alternative to SPS in selected patients with PAIVS.

Background— Many patients with congenital heart disease require permanent pacing for rhythm management but cannot undergo transvenous lead placement. In others, epicardial scarring prohibits adequate sensing and pacing thresholds using epicardial leads. This study describes long-term lead performance using a transmural atrial (epicardial to endocardial) pacing approach in patients with congenital heart disease. Methods and Results— For transmural atrial (TMA) lead access, a bipolar, steroid-eluting transvenous lead was placed from the epicardium via purse-string incision or atriotomy and affixed to atrial endocardium. Records were reviewed for patient data and acute and long-term lead performance for TMA leads placed 1998 to 2004. Forty-two of 48 TMA leads remain active at last follow-up. Two leads fractured, 4 were functional at >5-year follow-up but no longer active. Freedom from lead failure 98% (95% confidence interval, 86%–100%) at mean follow-up 7.8 years. TMA leads gave excellent sensing and pacing characteristics at implant and chronically. Median acute and chronic sensing thresholds were 3 and 2.8 mV, respectively; median acute and chronic pacing thresholds at 0.5 ms were 0.9 and 0.7 V, respectively. TMA leads performed similarly in Fontan patients. Overdrive pacing for intra-atrial re-entrant tachycardia was successful in 7 of 8 patients. One patient with high baseline risk died of stroke 7 years after implant. No lead-associated thrombi were observed. Conclusions— TMA pacing leads had excellent longevity, initial, and chronic functional properties and provide an alternative to epicardial leads in patients with congenital heart disease. Patients who cannot receive transvenous leads, have epicardial scarring or have intra-atrial re-entrant tachycardia could benefit most from routine use of this technique.

The extant nomenclature for mitral valve disease is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Mitral valve disease has been subdivided into stenotic and regurgitant lesions. Lesions have been characterized further by etiology and by anatomic location: supravalvar, valvar, and subvalvar. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Background . The modified Fontan procedure separates the systemic and pulmonary circulations in patients born with a functional single ventricle. Delayed recovery is frequently observed after this procedure. It was our hypothesis that complement activation or cytokine generation may contribute to the pathophysiology of this problem. Methods . We measured activated complement C3, thromboxane B 2 , interleukin-6, and tumor necrosis factor-α levels by immunoassay in 16 patients undergoing Fontan procedure. Patient plasma samples were obtained preoperatively, on initiation of cardiopulmonary bypass, after administration of protamine, and 1, 4, 8, and 24 hours postoperatively. Results . There was no early or late mortality in this cohort of patients. Low cardiac output developed in 3 of 16 patients, and pleural effusions developed in 5. The median length of hospital stay was 9 days. Activated complement C3 levels increased from a baseline of 1,486 ± 564 to 4,600 ± 454 ng/mL after cardiopulmonary bypass and administration of protamine, and returned to baseline by 24 hours. The level of interleukin-6 increased from 42 ± 32 to 176 ± 22 pg/mL and at 24 hours remained elevated at 71 ± 15 pg/mL. Neither thromboxane B 2 nor tumor necrosis factor-α levels increased significantly. Conclusions . The data demonstrate threefold to fourfold increases in activated complement C3 and interleukin-6, indicating that both humoral and cellular systems are affected. It is our conclusion that complement and cytokine activation may contribute to the delayed recovery observed after Fontan procedure.

BACKGROUND: Pediatric cardiac care is costly and requires extensive resources. We studied the effect of clinical pathways on practice patterns and patient care outcomes in infants and children hospitalized for cardiac surgery. METHODS: In consecutive patients admitted for selected cardiac surgical procedures before (n = 69) and after (n = 173) implementation of clinical pathways, outcomes including hospital length of stay, days in the ICU, time to extubation, ordering of blood studies, costs, and readmissions were compared. Data were analyzed for each of five cardiac surgical procedures: repair of an atrial septal defect, repair of a ventricular septal defect, division of a patent ductus arteriosus, repair of tetralogy of Fallot, and neonatal arterial switch operation to correct transposition of the great arteries. RESULTS: A significant reduction in length of hospital stay, including days in the ICU (decreased 1 to 2 days per admission), was achieved after the clinical pathway was implemented. Reductions in average duration of mechanical ventilation ranged from 28% for repair of a ventricular septal defect to 63% for repair of tetralogy of Fallot. The number of blood studies ordered decreased 20% to 30%. A significant reduction in hospital costs for each procedure, ranging from 16% to 29%, was also achieved with no adverse effects on patients' outcomes. CONCLUSIONS: Use of clinical pathways with children hospitalized for cardiac surgery can shorten length of stay in the hospital, reduce use of resources, and improve cost-effectiveness with beneficial outcomes for patients.

Patients undergoing the modified Fontan procedure may develop low cardiac output postoperatively. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate the influence of triiodothyronine supplementation. Ten consecutive patients under-going the Fontan procedure were administered intravenous triiodothyronine (0.4 mcg per kg) following surgery. Clinical outcome and thyroid hormone profiles were assessed and then compared to a previous series of patients undergoing the Fontan procedure who had not received triiodothyronine supplementation. Both groups initially demonstrated marked decreases in serum free triiodothyronine levels. The group which received triiodothyronine supplementation demonstrated a more rapid return of serum triiodothyronine levels to baseline [259±17 vs 121±15 pg/dl (p

Background: Infants undergoing repair or palliation of congenital heart disease are at risk of renal insufficiency. Development of renal insufficiency increases mortality. This project seeks to determine whether intra- and postoperative renal near-infrared spectroscopy (NIRS) monitoring can reliably predict renal insufficiency after cardiac surgery in infants. Methods: In this prospective, observational cohort study 48 patients undergoing repair or palliation of congenital heart disease in the first 6 months of life were studied intraoperatively and on postoperative day 1 and 2. The NIRS mean and nadir were recorded for the 3 time periods, as were urine output, fluid balance, and serum creatinine. Renal insufficiency was defined as rise in creatinine ≥40% from baseline or oliguria for >4 hours. Near-infrared spectroscopy data were compared to creatinine increase, oliguria, and fluid balance on postoperative day 0, 1, and 2 by regression analysis. Results: Mean renal regional saturation on postoperative day 1 has a strong correlation with increase in creatinine ( P < .001 and R 2 = .6). Mean renal saturation less than 80% predicts renal insufficiency with a sensitivity of 100% and a specificity of 75% ( P < .001). Conclusion: Monitoring of intra- and postoperative renal regional saturation may provide an early, noninvasive marker of renal insufficiency after cardiac surgery in infants. This would be clinically significant if interventions to improve renal regional saturation prevent renal insufficiency.

The Eighth International Conference of the Pediatric Cardiac Intensive Care Society was held in Miami, Florida, December 8 to 11, 2010. The program included a session dedicated to the management of hypoplastic left heart syndrome (HLHS), with particular emphasis on the innovations that have led to contemporary schemes of management and the role of clinical trials in the evolution and acceptance of these strategies. An invited panel of experts reviewed the historical evolution of staged surgical reconstruction, the randomized clinical trials that have been undertaken thus far, and the extent to which these have, or have not, influenced individual and institutional approaches to management of HLHS.

Fluid retention is a common problem following transannular patch repair of tetralogy of Fallot. The present study was undertaken to evaluate whether humoral substances may contribute to this process. Patients undergoing tetralogy of Fallot repair using a transannular patch technique were compared to patients undergoing simple ventricular septal defect repair. Hormone levels were determined by radioimmunoas say. Fluid retention was defined as pleural effusions persisting beyond 5 days. The data demonstrate that patients undergoing tetralogy of Fallot repair had elevated levels of anti-diuretic hormone, renin, angiotensin II, and aldosterone as compared to a group with ventricular septal defect. The elevation in hormone levels in the tetralogy of Fallot group was principally accounted for by those who developed effusions postoperatively. These results suggest that activation of the renin-angiotensin-aldosterone system may be one of the contributing factors to fluid retention following tetralogy of Fallot repair. (Eur Heart J 1996; 17: 1421-1425)

The Fontan procedure provides a means of separating the systemic and pulmonary circulations in patients with congenital heart defects characterized by a functionally single ventricle. The procedure has undergone numerous changes since its introduction 25 years ago. Technical modifications have now increased its applicability to virtually all forms of univentricular atrioventricular connection, as well as many hearts with biventricular connections. More recently, philosophical changes have been introduced to address increased rates of morbidity and mortality observed in certain subsets of patients. These changes appear to have improved short- and midterm results. The purpose of this review is to offer our strategy for management of such patients. We will discuss the interrelationship between the bidirectional Glenn and Fontan procedures. We will also focus on a number of observations concerning the possible role of accessory pulmonary blood flow, aortopulmonary collateral arteries, and endocrinologic changes.

Pleural effusions after the modified Fontan procedure are unpredictable, increase morbidity, and prolong hospital stay. To assess the relation between preoperative characteristics and postoperative pleural drainage, we performed a retrospective study of 71 patients who underwent Fontan procedures. Analyses revealed no significant relation between duration of effusion and age at Fontan, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, type of Fontan, or prior cavopulmonary anastomosis. Patients with significant aortopulmonary collateral vessels evidenced by angiographic opacification of the pulmonary arteries or veins had more prolonged pleural drainage. The duration of the pleural drainage was significantly less in patients who had aortopulmonary collateral occlusion.

Objective. The purpose of this study was to analyze the efficacy and safety of intravenous amiodarone in young patients with critical, drug-resistant arrhythmias. Background. Intravenous amiodarone has been investigated in adults since the early 1980s. Experience with the drug in young patients is limited. A larger pediatric study group was necessary to provide responsible guidelines for the drug's use before its market release. Methods. Eight centers obtained institutional approval of a standardized protocol. Other centers were approved on a compassionate use basis after contacting the primary investigator (J.C.P.). Results. Forty patients were enrolled. Standard management in all failed. Many patients had early postoperative tachyarrhythmias (25 of 40), with early successful treatment in 21 (84%) of 25. Twelve patients had ventricular tachyarrhythmias: seven had successful therapy, and six died, none related to the drug. Eleven patients had atrial tachyarrhythmias: 10 of 11 had immediate success, but 3 later died. Fourteen patients had junctional ectopic tachycardia, which was treated with success (sinus rhythm or slowing, allowing pacing) in 13 of 14, with no deaths. Three other patients had supraventricular tachycardias, with success in two and no deaths. The average loading dose was 6.3 mg/kg body weight, and 50% of patients required a continuous infusion. Four patients had mild hypotension during the amiodarone bolus. One postoperative patient experienced bradycardia requiring temporary pacing. There were no proarrhythmic effects. Deaths (9 [23%] of 40) were not attributed to amiodarone. Conclusions. Intravenous amiodarone is safe and effective in most young patients with critical tachyarrhythmia. Intravenous amiodarone can be lifesaving, particularly for postoperative functional ectopic tachycardia, when standard therapy is ineffective.

This article summarizes the case histories of six infants who had isolated secundum-type atrial septal defects (ASDs) and presented with failure to thrive in the first year of life. Patients underwent operative closure of their ASD with the expectation that this would improve their feeding and growth pattern. Five of the six children demonstrated little or no improvement in symptoms following operation. Each of these five patients have subsequently shown signs of developmental delay. Our experience with this group suggests that failure to thrive associated with a secundum ASD will frequently have a noncardiac basis.

An isolated left common carotid artery (LCA) is an extremely rare condition with only four reported cases. In each case, the isolated carotid artery connects to the right or left pulmonary artery via the ductus arteriosus and the embryologic basis for the abnormalities is believed to reflect an error in the development of the branchial arches. We present a case of an isolated LCA connecting to the main pulmonary artery in association with a right aortic arch and an anomalous origin of the left subclavian artery from the descending aorta. The left ligamentus arteriosus was identified separately. This may represent a disturbance in the septation of the truncoaortic sac secondary to abnormal migration of neural crest cells rather than a pure developmental anomaly of the branchial arches.

Background The modified Fontan procedure has become the treatment of choice for patients born with a univentricular heart. Although the operative mortality has steadily decreased in recent years, the hospital stay is still prolonged in many patients due to fluid retention and pleural effusions. Methods We retrospectively analyzed subsets of patients undergoing the bidirectional cavopulmonary shunt (BDCPS) and modified Fontan procedure in an attempt to define factors influencing operative mortality and morbidity. Results Multivariate analysis of 64 patients undergoing BDCPS revealed that age 6 months or less, concomitant operation, mean pulmonary pressure of 15 mm Hg or less, and mean pulmonary artery ratio of 1.8:1 or less were not statistically significant indicators of risk. Abnormal pulmonary artery architecture was a significant predictor of early and late death (p ≤ 0.01). Retrospective analysis of 71 patients undergoing the modified Fontan procedure revealed no significant relationship between duration of pleural effusions and age at operation, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, or prior BDCPS. Patients with important aortopulmonary collateral vessels defined by angiography had prolonged pleural drainage. Selective use of the total extracardiac conduit and the fenestrated Fontan resulted in low overall mortality with no statistical differences between subsets of patients undergoing different modifications of the Fontan procedure. Conclusions These data indicate that the operative risk for BDCPS or modified Fontan procedure is quite low if a procedure appropriate for the patient is selected. Abnormal pulmonary artery architecture is an important risk factor for death after the BDCPS. Aortopulmonary collateral vessels are associated with fluid retention and pleural effusions after a modified Fontan procedure. Important aortopulmonary collateral vessels should be occluded before or during the modified Fontan procedure.

Mortality is high for children with pulmonary atresia, intact ventricular septum, and important connections between the right ventricle and the coronary arteries because of myocardial ischemia: in systole, suprasystemic right ventricular pressure delivers deoxygenated blood to the coronary artery (or arteries) and in diastole, the right ventricle provides a lower resistance alternative to coronary perfusion of the myocardium. Tricuspid valve closure was performed in 10 such children. None had stenosis of native coronary arteries. A trial of tricuspid valve closure (by balloon) was performed in the cardiac catheterization laboratory in 5 of 10 patients. Seven of 10 children survived surgical closure of the tricuspid valve plus concurrent procedures; none had heart block. Two of the 3 nonsurvivors were probably in inoperable condition due to preoperative myocardial ischemia. Before operation, 4 patients had ischemic changes on electrocardiograms; these changes were abolished after operation. Three of 10 patients have had a Fontan operation with 2 survivors. We conclude that children with pulmonary atresia, intact ventricular septum, important connections between the right ventricle and the coronary arteries, and normal native coronary arteries should have surgical closure of the tricuspid valve within the first year of life and treated thereafter as patients with "tricuspid atresia."

The Damus-Kaye-Stansel operation is useful in the management of complex congenital heart defects. We reviewed our experience with 23 patients who underwent a Damus-Kaye-Stansel procedure. The anastomotic technique was individualized depending on the anatomy. The aortic and pulmonary artery incisions were carried into the sinuses of Valsalva in 9 patients, the aorta was transected in 11 patients, and a patch was used to augment the anastomosis in 13 patients. Concurrent procedures included a Fontan operation (n = 9, mortality = 0), right ventricle-pulmonary artery conduit (n = 5, mortality = 0), bidirectional Glenn procedure (n = 6, mortality = 1), and central aortopulmonary shunt (n = 3, mortality = 2; emergency = 1). Survival is 87% with a median follow-up of 7 years (range, 2 months to 9.2 years). Four patients underwent late revision of the Damus-Kaye-Stansel connection. All survivors are asymptomatic. We conclude that the Damus-Kaye-Stansel connection provides excellent midterm results when the proximal anastomosis is adapted to the anatomy of the patient.