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Longitudinal follow-up, resource utilization, and health disparities are top congenital heart research and care priorities. Medicaid claims include longitudinal data on inpatient, outpatient, emergency, pharmacy, rehabilitation, home health utilization, and social determinants of health-including mother-infant pairs.The New York Congenital Heart Surgeons Collaborative for Longitudinal Outcomes and Utilization of Resources linked robust clinical details from locally held state and national registries from 10 of 11 New York congenital heart centers to Medicaid claims, building a novel, statewide mechanism for longitudinal assessment of outcomes, expenditures, and health inequities.The authors included all children 18 years of age undergoing cardiac surgery in The Society of Thoracic Surgeons Congenital Heart Surgery Database or the New York State Pediatric Congenital Cardiac Surgery Registry from 10 of 11 New York centers, 2006 to 2019. Data were linked via iterative, ranked deterministic matching on direct identifiers. Match rates were calculated and compared. Proportions of the linked cohort trackable over 3, 5, and 10 years were described.Of 14,097 registry cases, 59% (n = 8,322) reported Medicaid use. Of these, 7,414 were linked to New York claims, at an 89% match rate. Of matched cases, the authors tracked 79%, 74%, and 65% of children over 3, 5, and 10 years when requiring near-continuous Medicaid enrollment. Allowing more lenient enrollment criteria, the authors tracked 86%, 82%, and 76%, respectively. Mortality over this time was 7.7%, 8.4%, and 10.0%, respectively. Manual validation revealed ∼100% true matches.This establishes a novel statewide data resource for assessment of longitudinal outcome, health expenditure, and disparities for children with congenital heart disease.

Objective Although the right ventricle (RV) to pulmonary artery conduit in stage 1 Norwood operation results in improved interstage survival, the long-term effects of the ventriculotomy used in the traditional technique remain a concern. The periscopic technique (PT) of RV to pulmonary artery conduit placement has been described as an alternative technique to minimize RV injury. A retrospective study was performed to compare the effects of traditional technique and PT on ventricular function following Norwood operation. Methods A retrospective study of all patients who underwent Norwood operation from 2012 to 2019 was performed. Patients with baseline RV dysfunction and significant tricuspid valve regurgitation were excluded. Prestage 2 echocardiograms were reviewed by a blinded experienced imager for quantification of RV function (sinus and infundibular RV fractional area change) as well as for regional conduit site wall dysfunction (normal or abnormal, including hypokinesia, akinesia, or dyskinesia). Wilcoxon rank-sum tests were used to assess differences in RV infundibular and RV sinus ejection fraction and the Fisher exact test was used to assess differences in regional wall dysfunction. Results Twenty-two patients met inclusion criteria. Eight underwent traditional technique and 14 underwent PT. Median infundibular RV fractional area change was 49% and 37% (P = .02) and sinus RV fractional area change was 50% and 41% for PT and traditional technique (P = .007) respectively. Similarly qualitative regional RV wall function was better preserved in PT (P = .002). Conclusions The PT for RV to pulmonary artery conduit in Norwood operation results in better preservation of early RV global and regional systolic function. Whether or not this benefit translates to improved clinical outcome still needs to be studied.

Introduction: A paucity of data exists of the anatomic-physiological mechanisms for sub-pulmonic systolic anterior motion (SAM) of the mitral valve (MV) in d-looped transposition of the great arteries post atrial switch (d-TGA/AtS). Hypothesis: The development of SAM may be inherent to the anatomy of the MV or affected by external factors such as a dilated systemic right ventricle (RV) or pectus deformity. Methods: Clinical and cardio-imaging analysis of 18 adult patients with d-TGA/AtS (Age 42±6 years old, 56% Male) between 2015-2019 was performed. Echocardiography data included mitral apparatus anatomy (Figure) and CT/MRI data included biventricular dimensions, function, and Haller index (HI). Results: Patients with leaflet SAM (n=5) compared to patients without SAM (n=13) had significantly higher MV protrusion height (2.1 ± 0.4 vs 1.5 ± 0.4 cm p ≤0.01, respectively, Table) and longer anterior MV leaflet length (3.0 ± 0.24 vs 2.6 ± 0.34 cm p ≤0.05, respectively, Table) when compared to those without. CT/MRI showed higher sub-pulmonic left ventricular ejection fraction (LVEF) in the SAM group relative to No SAM (68 ± 5 vs 54 ± 7 % p ≤0.005, respectively, Table). RV size and function, chest deformity (HI>3.5), presence of a ventricular lead pacemaker, and septal thickness did not play a role in development of SAM (Table). Conclusions: An elongated mitral apparatus is associated with the development of SAM in d-TGA/AtS and the development of LVOT obstruction. Increased sub-pulmonic LVEF contributes to SAM, whereas the systemic RV dimensions, septal thickness, and degree of chest deformity do not correlate with SAM.

Surgically corrected congenital heart defects may result in unique long-term complications. An 18 year old man with tricuspid atresia, normally related great arteries, and ventricular septal defect (VSD), status post a staged surgical palliation culminating in an extra-cardiac Fontan, presented

Given the purported hemodynamic advantages of the right ventricle (RV) to pulmonary artery (PA) conduit, many surgeons have adopted it as their preferred source of pulmonary blood flow during stage I palliation for hypoplastic left heart syndrome. Potential disadvantages of the RV-PA shunt include ventricular dysfunction, pseudoaneurysm formation, arrhythmia, and conduit obstruction, which can lead to a higher rate of unplanned reinterventions. The "dunk" technique was described to reduce the RV incision and proximal conduit obstruction; however, insertion of the ringed graft from the epicardium can be cumbersome and risk RV injury. We introduce a simplified, alternative method of placing the conduit, which we call the periscope technique, whereby the graft is withdrawn from within the RV cavity.

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

Background— An increasing number of patients with congenital heart disease (CHD) are reaching adulthood and may require heart transplantation. The survival of these patients after listing and transplantation has not been evaluated. Methods and Results— A total of 41 849 patients (aged >18 years) were listed for primary transplantation during 1995–2009. Patients with a history of CHD (n=1035; 2.5%) were compared with those with other causes (non-CHD group) (n=40 814; 97.5%); 26 055 (62.3%) reached transplantation and were subdivided into those with (reoperation group; n=10 484; 40.2%) and without (nonreoperation group; n=15 571; 59.8%) a previous sternotomy. Survival on the waiting list was similar between groups, but mechanical ventricular assistance was not associated with superior survival to transplantation among CHD patients. CHD patients were more likely to have body mass index P P P Conclusions— Adults with CHD have high 30-day mortality but better late survival after heart transplantation. Mechanical circulatory assistance does not improve waiting list survival in these patients. This may be due to a combination of highly complex reoperative surgery and often poor preoperative systemic health.

Management of atrial septal defects (ASD) in patients with abnormal RV anatomy or function is challenging, as simple ASD closure may not be hemodynamically tolerated. A 34-year-old woman presented with progressive dyspnea. Physical exam revealed hypoxia (SpO2 72%), fixed split S2, and digital

Background— Tetralogy of Fallot (TOF) typically results in clinical cyanosis or volume overload of the left ventricle (LV), depending on the direction and magnitude of shunting across the ventricular septal defect (VSD). The present study examines the effects of surgical TOF repair on LV mechanics and compares these changes between patients with VSD shunts that are predominantly right-to-left (R-L; “blue TOF”) and those with VSD shunts that are predominantly left-to-right (L-R; “pink TOF”). Methods and Results— Eleven patients (6 R-L and 5 L-R) 4.3 to 18.4 months old (median 7.1 months old) were studied. LV end-diastolic area (EDA) was calculated from transesophageal echocardiograms obtained during initiation and weaning of cardiopulmonary bypass. LV end-diastolic pressure was measured by micromanometer. Compliance was assessed by end-diastolic pressure-area curves. Contractility was assessed from preload recruitable stroke work by the stroke work–versus–LV EDA relation. VSD shunt direction was determined by preoperative Doppler echocardiography. Changes in LV function at the conclusion of cardiopulmonary bypass included decreased stroke area (from 6.6±0.9 to 4.1±0.4 cm 2 /m 2 , P =0.012) and ejection fraction (from 55±2% to 41±3%, P 2 /m 2 , P =0.003), which suggests a decrease in ventricular compliance. Additionally, the end-diastolic pressure-area curves shifted to the left in all patients. Preload recruitable stroke work decreased (from 34.8±2.4 to 21.8±2.6 mm Hg, P =0.007), which demonstrates a decrease in ventricular contractility. When separated by preoperative shunt direction, LV EDA increased in R-L patients by 0.9±0.5 cm 2 /m 2 postoperatively but decreased in L-R patients by 4.3±0.8 cm 2 /m 2 ( P Conclusions— LV diastolic and systolic function are depressed after TOF repair. Mechanical effects of the VSD patch and myocardial depressant effects of ischemia and reperfusion during surgery probably contribute to the observed changes in LV mechanics. Different effects of surgical repair on LV preload in pink and blue TOF also contribute to the spectrum of clinical results observed after surgery.

Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI)6 W.U. x m(2) have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 +/- 5.6 years and a mean time from listing to HTX of 43 +/- 60 days. Preoperative and postoperative (114 +/- 40 days) PVRI were 5.9 +/- 4.4 and 2.9 +/- 1.5 W.U. x m(2), respectively. At time of most recent follow-up (mean = 5.7 +/- 4.6 years), the mean PVRI was 2.0 +/- 1.0 W.U. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI6 W.U. x m(2) (chi(2)= 7.4, p0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI6 W.U. x m(2) survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.

Background The bidirectional Glenn cavopulmonary anastomosis (BDG) represents the standard interim procedure in treatment of patients with single-ventricle physiology. Anterograde pulmonary blood flow (APBF) maintained after BDG has been shown both to improve and to complicate postoperative clinical course. We studied its effects on outcome after BDG and eventual Fontan completion. Methods From November 1995 to November 2005, 60 patients underwent BDG and Fontan. All patients had APBF from the ventricle to the pulmonary artery at time of BDG. In group 1 (n = 39) APBF was maintained after BDG, whereas APBF was interrupted at BDG in group 2 (n = 21). Cardiac catheterization data, interstage morbidity, and postoperative outcome variables were recorded. Results Pre-BDG hemodynamics differed only in that the mean pulmonary artery pressure was higher in group 2 (17.0 ± 4.4 mm Hg) than in group 1 (13.8 ± 4.5 mm Hg; p = 0.03). There were no differences between groups 1 and 2 in BDG outcome variables. At pre-Fontan catheterization, group 1 had higher mean pulmonary artery pressure (13.3 versus 10.9 mm Hg, p = 0.01), arterial oxygen saturation (85.8 versus 80.9%, p = 0.0001), and fewer collateral vessels were coil embolized than in group 2 (0.9 versus 1.6, p = 0.02). Mean ventricular end-diastolic pressure was similar between groups. The Nakata index in group 1 remained stable from pre-BDG to pre-Fontan (348 versus 391, p = 0.24), but it decreased in group 2 (375 versus 227, p = 0.046). Conclusions Patients with anterograde pulmonary blood flow after BDG had a modest increase in pulmonary artery growth and arterial oxygen saturations, and decreased collateral vessel formation. This did not, however, confer additional benefit on outcome after BDG or on eventual Fontan completion.

ObjectivesA critical shortage of donor organs has caused many centers to use less restrictive donor criteria, including the use of adult-age donors for pediatric recipients. The purpose of this study is (1) to describe the supply of pediatric (0-18 years) heart donors, (2) to explore the relationship between donor age and long-term survival, and (3) to define threshold age ranges associated with decreased long-term survival.MethodsThe United Network of Organ Sharing provided deidentified patient-level data. Primary analysis focused on 1887 heart transplant recipients aged 9 to 18 years undergoing transplantation from October 1, 1987, to September 25, 2005. Kaplan-Meier analysis and log-rank tests were used in time-to-event analysis. Receiver operating characteristic curves and stratum-specific likelihood ratios were generated to compare survival at various donor age thresholds.ResultsThe number of pediatric donors decreased (P < .001) over the study period, particularly from 1993 (n = 640) through 2004 (n = 432). Among recipients aged 9 to 18 years, univariate analysis demonstrated a statistically significant (P < .001) inverse relationship between donor age and survival. Stratum-specific likelihood ratio analysis generated 3 strata for donor age: the low-risk, intermediate-risk, and high-risk groups consisted of donors aged 13 years or younger (n = 611, 32.41%), 14 to 51 years (n = 1258, 66.7%), and 52 years and older (n = 16, 0.85%), respectively. In the low-risk, intermediate-risk, and high-risk groups median survival was 4069 days (11.1 years), 3495 days (9.57 years), and 1197 days (3.28 years), respectively.ConclusionsAlthough donors aged 13 years or less offer pediatric recipients the best chance for achieving long-term survival, donors aged 14 to 51 years offer good outcomes to pediatric recipients. Consideration should be given to expanded use of well-selected adult-age donors for pediatric recipients.

Objective Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle–pulmonary artery continuity. Methods Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality). Results Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 ± 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function. Conclusions We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle–pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.

Background. Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group. Methods. From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA. Site of aortic arch interruption was distal to the left subclavian artery in 16% and between the left common carotid and subclavian artery in 84%. From the common arterial trunk, the pulmonary arteries arose from a main pulmonary trunk in 46%, common orifice in 22%, and separate orifices in 32%. At presentation, truncal valve stenosis was present in 12% and regurgitation in 22%. Results. There were 34 deaths, with a single early hazard phase. Overall survival from admission was 44%, 39%, and 31% at 6 months, 1 year, and 10 years, respectively. One patient had primary cardiac transplantation and 4 died without any intervention. The IAA repair alone was performed in 7 patients, with single stage repair of both IAA and TA in 38 patients. Associated factors for overall time-related death include female gender (p < 0.001), type III TA (p < 0.001) and one institution (low-risk; p < 0.001). Results improved somewhat over time (p < 0.001). At 5 years after IAA repair only 28% were alive without arch repair intervention, and at 5 years after TA repair only 18% were alive without conduit reoperation. Conclusions. The combination of IAA and TA carries high early mortality, with high risk of reinterventions in survivors. One stage repair of both TA and IAA is the optimal management.

Background Repair of complete atrioventricular canal early in infancy has traditionally carried greater morbidity and mortality than repair performed later. However, an individualized anatomy-based repair may give young infants outcomes that are equivalent to older patients. Methods We retrospectively reviewed 139 patients who underwent complete atrioventricular canal repair from January 2005 to December 2012. An individualized approach was used: 2-patch repair was performed in 98 patients for large ventricular septal defects and a modified single-patch ("Australian technique") was used in 41 for "shallow" ventricular septal defects. Results The average age was 25.5 ± 3.9 weeks, 50% were boys, and 78% had trisomy 21. Mean follow-up was 5.1 ± 0.2 years, with 100% completeness of data. There were 3 in-hospital deaths (2.1%) and 1 late death (0.7%). A permanent pacemaker was required in 2 patients (1.4%). The rate for left atrioventricular valve reoperation was 8% at a mean of 211 ± 238 days after the original repair (range, 6 to 682 days). Compared with patients aged older than 3 months, the 39 patients (28%) who were younger than 3 months had similar perioperative courses and rate of reoperation. Compared with patients with an Australian repair, the 98 patients (71%) with a 2-patch repair were more likely to have trisomy 21 and had slightly increased cardiopulmonary bypass and cross-clamp times but similar outcomes. Multivariate analysis showed postoperative left atrioventricular valve regurgitation greater than 2 and left ventricular outflow tract obstruction were significant risk factors for reoperation on the left atrioventricular valve (both p Conclusions Repair of complete atrioventricular canal using an individualized surgical approach yields reoperation and early mortality rates similar for younger infants compared with older infants, obviating the need to delay operation in symptomatic patients.

Objective The purpose of this study was to evaluate the outcome and risk factors for implant failure in pediatric patients who underwent pulmonary position homograft placement for right ventricular outflow tract obstruction compared with conduit placement as a component of the Ross operation. Actuarial 5-year survivals for cryopreserved right ventricle-to-pulmonary artery homografts range from 55% to 94% at all ages. It is not known whether there is a difference in homograft durability when utilized for right ventricular outflow tract obstruction or as part of the Ross operation. Methods The records of all pediatric patients receiving a right ventricle-to-pulmonary artery homograft from July 1989 through October 2003 were reviewed. Ninety-eight consecutive patients were studied (26 Ross, 72 non-Ross). In addition to Ross versus non-Ross comparisons, other potential risk factors for homograft failure analyzed included age at operation, follow-up time, type of surgery, and homograft type and size. Results Ross and non-Ross patients were comparable in age at the time of the operation and follow-up time. Homograft failure rates were 12% and 51% for Ross and non-Ross patients, respectively. Freedom from reintervention was 93% in the Ross and 66% in the non-Ross group at 5 years (P = .019). On multivariate analysis, non-Ross operation and age less than 2 years were significant predictors of homograft failure. Conclusions 1. Pediatric patients undergoing the Ross operation have longer homograft survival than pediatric patients treated for right ventricular outflow tract obstruction, independent of age. 2. Homografts placed in patients less than 2 years of age have shorter homograft survival.

Objectives We reviewed our experience with repair of truncus arteriosus to assess the effect of type of right ventricular outflow tract reconstruction on perioperative morbidity, survival, and freedom from catheter-based interventions and reoperation. Methods Patients undergoing repair of truncus arteriosus from June 1990 through February 2004 were evaluated on the basis of operative procedure regarding preoperative and postoperative variables, the need for postoperative catheter-based intervention or reoperation, and survival on the basis of univariate, multivariable, and actuarial analyses. Results Of 54 study patients, 15 (28%) received a valved homograft, and 39 (72%) received a direct connection with a variety of hood materials. Five (9.1%) patients died. Valved homograft recipients were more likely to require reoperation than patients receiving direct connections (40% vs 15%, P = .046); however, valved homograft and direct connection recipients had a similar incidence of the combined end point of reoperation or catheter-based intervention (40.0% vs 37.5%, P = .865). Univariate and multivariable modeling demonstrated use of valved homografts or direct connections with an autologous pericardial hood to be predictive of the need for later catheter-based intervention or reoperation. Actuarial analysis demonstrated a trend toward improved outcomes in the direct connection group and within the direct connection cohort, a statistically significant difference on the basis of hood type. Conclusions Although the direct connection technique might not prevent later catheter-based intervention, it does reduce the need for reoperation. Outcomes among direct connection recipients were associated with hood type: polytetrafluoroethylene hoods (W. L. Gore & Associates, Inc, Tempe, Ariz) had the lowest rate of reintervention, and untreated autologous pericardial hoods had the highest rate of reintervention. We report excellent outcomes with primary repair of truncus arteriosus. Where anatomically appropriate, we advocate the direct connection technique.

Objectives We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children. Methods The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation. Results A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 ± 15.9 years, and the average follow-up was 4.1 ± 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. Discussion Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.

This study investigated changes in left ventricular (LV) geometry and systolic function after corrective surgery for atrial (ASD) and ventricular septal defects (VSD).Transesophageal LV short-axis echocardiograms were recorded before and after operative repair of ASD (n = 11) and VSD (n = 7). Preload was measured using LV end-diastolic area indexed for body surface area. Measurements of septal-freewall (D1) and anterior-posterior (D2) endocardial diameters were used to assess LV symmetry from D1/D2. Systolic indices included stroke area, area ejection fraction, and fractional shortening.Preload, stroke area, area ejection fraction, and fractional shortening of D1 increased after ASD repair but decreased after VSD repair (p0.05). End-diastolic symmetry increased after ASD closure and decreased after VSD closure (p0.05). Increases in stroke area and ejection fraction after ASD correction primarily reflected increased shortening of D1. A positive correlation was found overall between percent change in end-diastolic area (EDA) and percent change in area ejection fraction (r(2) = 0.80, p0.0001, n = 18).Preload was the primary determinant of changes in LV function in this series of ASD and VSD repairs. Intraoperative changes in position of the interventricular septum affected systolic and diastolic LV symmetry and septal free wall shortening. Additional studies are needed to define changes in afterload and contractility as well as diastolic compliance and systolic mechanics.

Optimal antimicrobial prophylaxis for the pediatric cardiac surgical patient is unknown. We have reviewed our experience with more than 4,000 pediatric cardiac surgical patients at the University of Michigan to evaluate antibiotic prophylaxis regimens.Three antibiotic prophylaxis protocols were serially used during a 6-year period: Protocol 1 (n = 786): cefazolin was administered before operation and continued as long as thoracostomy tubes or central venous catheters were present; Protocol 2 (n = 1095): cefazolin was discontinued 48 hours postoperatively, regardless of the presence of tubes or catheters; Protocol 3 (n = 2039): cefazolin was continued as long as thoracostomy tubes were present, but not for central venous catheters. Patients with an open chest postoperatively received vancomycin and gentamicin until chest closure. This was identical during all three protocols. We retrospectively determined the rate of surgical site infections and unrelated bloodstream infections (the latter for both cardiac medical and surgical patients) for the three protocols.Surgical site infections per 100 operations for protocols 1, 2, and 3 was 2.04, 6.58, and 1.67, respectively (p0.05 for protocol 2 versus protocols 1 and 3). The mean age of patients with a surgical site infection ranged from 12 to 15.4 months. Patients with an open chest had a higher rate of surgical site infection (18.8% for protocol 2 and 9.3% for protocol 3). Bloodstream infections per 1,000 patient days for protocols 1, 2, and 3 were 2.18, 6.51, and 5.02, respectively (p0.05 protocol 1 versus protocols 2 and 3).These data suggest that pediatric cardiac surgical patients may benefit from prophylactic antibiotics as long as thoracostomy tubes are in place.

he events that give rise to the c1evelol)iiieiit of the T constellation of lesions that we refer to as tetralogj of Fallot are still micertain. Regardless of the true teleological cause, it is hel1)ful for the surgeon to approach this defect as resulting from anterocephalad deviation of the ventricular outlet septum. "monology with sequellae." The resulting malalignment ventricular septal defect (VSD), aortic override, ancl right ventricular outflow tract ol~struction are thus easily understood. The right ventricular hypertrophy can he appreciated as secondary to the VSD and right ventricular outflow tract ohstructioii. Right ventricular outflow obstruction is increased by hypertrophy of the anterior limb of the septal ]land, anomalous anterior muscular bands, and outlet septum as well as pulmonary valve and arterial anomalies (Fig I). Classically the VSD is circular, juxtaaortic, and nonrestrictive and results from the malalignment of the parietal extension of the infundibular and trabecular septae. As such, the defect lies between the infundibular septum and is cradled by the anterior and posterior limbs of the septal band (Fig 11).

In this case report, we describe the use of the Aquadex™ system for ultrafiltration therapy in the pediatric cardiac intensive care setting in a patient with fluid overload and acute kidney injury after congenital heart surgery. The patient is an 11-year-old, 25 kg male with complex single ventricle anatomy who underwent a one and a half ventricle repair. The patient experienced multiple organ dysfunction syndrome including acute kidney injury in the early post-operative period secondary to low cardiac output syndrome and tachyarrhythmia. Ultrafiltration using the Aquadex™ system was utilized to treat fluid overload in the setting of acute kidney injury and hemodynamic instability. Negative fluid balance was safely achieved. It was subsequently possible to wean ventilatory and inotropic support. We conclude that the use of ultrafiltration therapy is feasible in hemodynamically unstable pediatric patients with significant fluid overload in the setting of acute kidney injury following congenital heart surgery.

Objective To review more than a decade of experience with complete repair of tetralogy of Fallot (TOF) in neonates at the University of Michigan; to assess early and late survival, perioperative complications, and the incidence of reoperation; and to analyze patient, procedural, and morphologic risk factors to determine their effects on outcome. Background Data Palliation of TOF with systemic-to-pulmonary artery shunts has been the accepted standard for symptomatic neonates and infants. Complete repair has traditionally been reserved for infants older than 6 months of age because of the perception that younger and smaller infants face an unacceptably high surgical risk. Results A retrospective review from August 1988 to November 1999 consisted of 61 consecutive symptomatic neonates with TOF who underwent complete repair. Thirty-one patients had TOF with pulmonary stenosis, 24 had TOF with pulmonary atresia, and 6 had TOF with nonconfluent pulmonary arteries. The mean age at repair was 16 ± 13 days, and the mean weight was 3.2 ± 0.7 kg. Before surgery, 36 patients were receiving an infusion of prostaglandin, 26 were mechanically ventilated, and 11 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 49 patients and a right ventricle-to-pulmonary artery conduit in 12. Cardiopulmonary bypass time averaged 71 ± 26 minutes. Hypothermic circulatory arrest was used in 52 patients (mean 38 ± 12 minutes). After cardiopulmonary bypass, the average intraoperative right/left ventricular pressure ratio was 55% ± 13%. There were no new clinically apparent neurologic sequelae after repair. The postoperative intensive care unit stay was 9.1 ± 8 days, with 6.8 ± 7 days of mechanical ventilation. There was one hospital death from postoperative necrotizing enterocolitis on postoperative day 71 and four late deaths, only one of which was cardiac-related. Actuarial survival was 93% at 5 years. Follow-up was available for all 60 hospital survivors and averaged 62 months (range 1-141 months). Twenty-two patients required a total of 24 reoperations at an average interval of 26 months after repair. Indications for reoperation included right ventricular outflow tract obstruction (19), branch pulmonary artery stenosis (11), severe pulmonary insufficiency (4), and residual ventricular septal defect (1). The 1-month, 1-year, and 5-year freedom from reoperation rates were 100%, 89%, and 58%, respectively. Conclusions Complete repair of TOF in the neonate is associated with excellent intermediate-term survival. Although the reoperation rate is significant, this is to be expected with the complex right ventricular outflow tract and pulmonary artery anatomy seen in symptomatic neonates and the need for conduit replacement in patients with TOF with pulmonary atresia.

High intensity focused ultrasound (HIFU) is an evolving technology with potential therapeutic applications. Utilizing frequencies of 500 kHz to 10 MHz, HIFU causes localized hyperthermia at predictable depths without injuring intervening tissue. Applications in neurosurgery, urology, oncology and, more recently, cardiology for selective cardiac conduction tissue ablation have been promising. A 'noninvasive' technique for causing localized tissue damage to relieve hemodynamic and life-threatening obstruction in patients with congenital cardiac anomalies could replace more invasive procedures. We, therefore, investigated the ability of HIFU to create lesions in mammalian cardiac tissues ex vivo. Porcine valve leaflet, canine pericardium, human newborn atrial septum, and right atrial appendage were studied. Specimens were mounted and immersed in a water bath at room temperature. Using a 1-MHz phased array transducer, ultrasound energy was applied with an acoustic intensity of 1630 W/cm(2) or 2547 W/cm(2) until a visible defect was created (duration 3 to 25 sec). Macroscopic and microscopic examination demonstrated precise defects ranging from 3 to 4 mm in diameter. No damage was identified to the surrounding tissues. Our study concluded that HIFU can create precise defects in different cardiac tissue without damage to the surrounding tissue. Further investigation is needed to assess potential clinical uses of this technology.

Objectives: Neonates with congenital heart disease may appear hemodynamically stable after operation and then suddenly experience catastrophic decompensation. An improved means of predicting which infants will suddenly die in the early postoperative period may lead to lifesaving interventions. Studies indicate that blood lactate level is proportional to tissue oxygen debt, but information linking lactate levels with outcome in infants after operation is limited. We sought to determine whether a change in lactate level over time was predictive of a poor outcome defined as death within the first 72 hours or the need for extracorporeal membrane oxygenation. Methods: To test this hypothesis, we studied prospectively 46 infants who were less than 1 month old and were undergoing complex cardiac surgical palliation or repair. Postoperative arterial oxygen saturation, bicarbonate, and lactate levels were recorded on admission to the intensive care unit and every 3 to 12 hours for the first 3 days. Results: Thirty-seven patients had a good outcome, and 9 patients had a poor outcome. Mean initial lactate level was significantly greater in patients with a poor outcome (9.4 ± 3.8 mmol/L) than in patients with a good outcome (5.6 ± 2.1 mmol/L; P =.03). However, an elevated initial lactate level of more than 6 mmol/L had a low positive predictive value (38%) for poor outcome. In contrast, a change in lactate level of 0.75 mmol/L per hour or more was associated with a poor outcome (P

FISCHBACH, P.S., ET AL.: Use of a Single Coil Transvenous Electrode with an Abdominally Placed Im-plantable Cardioverter Defibrillator in Children. While transvenous defibrillaior electrode placementavoiding a thoracotomy is preferable, electrode size, a large intercoil spacing, and the need for subclavic-ular device placement preclude this approach in mo.'it children. We investigated a single RV coil to an ab-dominally placed active can ICD device. Five children ages 8-16 years (weight 21-50 kg. mean 35 kg) un-derwent ICD placement. Placement of a single coil Medtronic model 6932 or 6943 electrode was performedvia the left subclavian vein approach and the electrode positioned in the HV apex with the coil lying alongthe RV diaphragmatic surface. The ICD (Medtronic Micro Jewel II model 7223Cx) was implanted in a leftabdominal pocket with the lead tunneled from the infraclavicular region to the pocket. Implant DFTs were^ 15 f using a biphasic waveform. DFTs rechecked within 3-month postimplant were unchanged. Leadimpedance at implant ranged from 38 to 56 fl, mean 51 fl. Follow-up was 3-21 months (total 82 months)with no electrode dislodgment. lead fractures, or inappropriate discharges. Two of the five patients havehad successful appropriate ICD discharges. Transvenous ICD electrode placement can be performed inchildren as small as 20 kg with the device implanted in a cosmetically acceptable abdominal pocket thatis well tolerated. Fxcellent DFTs can be achieved. This approach avoids a thoracotomy in all hut thesmallest child, does not require subclavicular placement of the device, and avoids use of a second in-travascular coil. (PACE 2000; 23:884-887)defibrillator, children, arrhythmia, abdominal implantIntroductionThe value of implantable cardioverter defibril-lators [ICDs) as a lifesaving intervention has beonpreviously demonstrated in the pediatric popula-tion/ The experience with ICDs in this age group islimited, accounting for less than 1% of all im-planted devices.^ Similar to the indications for de-vice implantation in adult patients as outlined re-cently by the American College of Cardiology andthe American Heart Association,^ the need for anICD in the pediatric population arises from one ofthree causes: (1) primary electrical disease [con-genital long QT syndrome, idiopathic ventricularfibrillation fVF]); (2) cardiomyopathy [arrhythmo-genic right ventricular dysplasia, hypertrophic, di-lated); and [3) following repair of congenital heartdisease (tetralogy of Fallot, transposition of thegreat arteries). Previously, the size of the transve

minal obstruction may occur. Recurrent suppurative pneumonia can occur due to obstruction. Our patient aspirated a chicken bone 4 years previously and underwent several rigid bronchoscopy attempts to extract the foreign body. She was admitted to the Istanbul Medical School after an episode of pneumonia and had dyspnea and audible wheezing because of near total airway obstruction diagnosed via bronchoscopy. Ibrahim Sersar and colleagues reported a series of 3300 patients, 3 of whom required bronchotomy because extraction of the inhaled foreign body failed. In another study, 164 patients underwent bronchoscopy for foreign body aspiration, and 6% of patients required surgical extraction. Early bronchoscopy has high successful rates in the extraction of foreign bodies. Bronchotomy is usually enough to extract the foreign body, which was performed after early bronchoscopy. Because of the delay in extraction, our patient required an extended operation, a segmental main bronchial resection with supra-aortic anastomosis. After unsuccessful attempts, a bronchotomy would have been a less-invasive procedure for this patient. Thus, we advise an early operation for organic foreign bodies.

Objective: Coronary physiology in infants with congenital heart disease remains unclear. Our objective was to better understand coronary physiology in infants with congenital heart disease. Methods: We used positron emission tomography with nitrogen 13–labeled ammonia to measure myocardial perfusion at rest and with adenosine (142 μg/kg/min × 6 minutes) in five infants after anatomic repair of a congenital heart lesion (group I), and in five infants after Norwood palliation for hypoplastic left heart syndrome (group II). The groups were matched for age, weight, and time from the operation. Results: Resting coronary flow in the left ventricle in group I was 1.8 ± 0.2 ml/min/gm; resting flow in the right ventricle in group II was 1.0 ± 0.3 ml/min/gm ( p = 0.003). Coronary flow with adenosine was 2.6 ± 0.5 ml/min/gm in group I and 1.5 ± 0.7 ml/min/gm in group II ( p = 0.02). Absolute coronary flow reserve was the same in both groups (1.5 ± 0.2 in group I vs 1.6 ± 0.3 in group II, p = 0.45). Oxygen delivery was reduced in group II compared with group I at rest (16.1 ± 4.2 ml/min/100 gm vs 28.9 ± 4.42 ml/min/100 gm, p = 0.02) and with adenosine (25.5 ± 8.1 ml/min/100 gm vs 42.3 ± 5.8 ml/min/100 gm, p = 0.02). Conclusions: Infants with repaired heart disease have higher resting flow and less coronary flow reserve than previously reported for adults. After Norwood palliation, infants have less perfusion and oxygen delivery to the systemic ventricle than do infants with a repaired lesion. This may in part explain why the outcome for patients with Norwood palliation is less favorable than for others. (J Thorac Cardiovasc Surg 1998;115:103-10)

Background . Patients with univentricular hearts and ventriculoarterial discordance with potentially obstructed systemic blood flow continue to pose difficult management problems. The goals of neonatal palliative operations are to control pulmonary blood flow while avoiding pulmonary artery distortion, to relieve systemic outflow tract obstruction, and to avoid heart block. Methods . Between January 1987 and December 1996, 38 patients with either tricuspid atresia or a double-inlet left ventricle and ventriculoarterial discordance underwent a modified Norwood procedure. Their mean age was 15 days, and their mean weight was 3.4 kg. Aortic arch anomalies were present in 92% of the patients. Morbidity and mortality statistics, intraoperative data, and postoperative echocardiograms were reviewed. Results . There were 3 early deaths (7.8%) and 5 late deaths (13.1%). The actuarial survival rates at 1 month, 1 year, and 5 years were 89%, 82%, and 71%, respectively. Follow-up was complete in all children at a mean interval of 30 ± 9 months. None of the patients had significant neoaortic valve insufficiency, and 1 patient required therapy for residual aortic arch obstruction. Nine patients (30% of the survivors) have undergone the hemi-Fontan procedure, and 18 patients (60%) successfully have undergone the Fontan procedure. Conclusions . In this patient population, we recommend the modified Norwood procedure as the neonatal palliative treatment of choice. It can be performed with acceptable early morbidity and mortality, and it improves suitability for the Fontan procedure. It reliably relieves all levels of systemic outflow tract obstruction, controls pulmonary blood flow, and avoids heart block.

Background. Aortic valve replacement with a pulmonary autograft (Ross procedure) is being applied more commonly in children. Although indications for this procedure have been expanded, the presence of a dilated aortic annulus has remained a relative contraindication. In this condition, the use of an undersized autograft in an enlarged aortic annulus may result in significant aortic regurgitation. Methods. Among 68 children and young adults undergoing the Ross procedure, 15 (age range, 8 to 24 years) with severe aortic regurgitation or stenosis and an aortic annulus diameter that was at least 2 mm larger than the pulmonary annulus had aortic root tailoring. In this group, the diameter of the aortic annulus measured 26.6 ± 1.3 mm (mean ± standard error of the mean), whereas that of the pulmonary annulus was 22 ± 0.9 mm. The mean annular difference was 4.6 ± 0.7 mm (range, 2 to 12 mm). The aortic annulus was reduced by excising a triangular wedge of tissue posteriorly from the aortic valve annulus at the level of the commissure between the left and noncoronary cusps extending into the anterior leaflet of the mitral valve. The edges were reapproximated over a calibrated dilator to adjust the final size of the aortic annulus to 2 mm smaller than that of the pulmonary autograft. Circumferential felt strips were not used in any patient. Results. All patients survived and morbidity was limited to one reoperation for bleeding. Doppler echocardiographic examination performed at discharge demonstrated that no patient had more than trace to 1+ aortic regurgitation and none had evidence of aortic stenosis. Over a mean follow-up period of 6.3 ± 1.5 months (range, 1 to 16 months) there has been no late morbidity or mortality and no progression of aortic regurgitation. Conclusions. Aortic root tailoring further extends the use of the Ross procedure to patients with excessive aortic annular dilation while maintaining the potential for growth, which is particularly important in the pediatric population.

We performed a prospective, randomized, placebo-controlled, double-blind trial to assess the efficacy of aprotinin in 61 children (median age 3.7 yr) undergoing reoperative open heart surgery (OHS). Three demographically similar groups were studied : large-dose aprotinin (ALD), small-dose aprotinin (ASD), and placebo (P). Over the first 24 postoperative hours fewer patients in the aprotinin groups received packed red cells (ALD, 53% ; ASD, 89% ; and P, 95% ; P = 0.001), platelets (ALD, 32% ; ASD, 50% ; and P, 65% ; P = 0.04), and fresh frozen plasma (ALD, 16% ; ASD, 17% ; and P, 60% ; P = 0.003) than placebo patients. Most importantly, aprotinin patients had fewer exposures to banked blood components (ALD, median 1 U ; and ASD, median 2 U) than P (median 6 U ; P = 0.001), with no difference in overall complication rate. Use of aprotinin was associated with a savings in the patient charges for blood components, operating room time, and duration of hospitalization. In conclusion, aprotinin decreased the number of units of banked blood components used during the first 24 postoperative hours in reoperative pediatric OHS. Aprotinin thus decreases the risks associated with exposure to banked blood components and reduces hospital charges.

Background It is widely held that the postoperative course of patients with hypoplastic left heart syndrome (HLHS) after stage 1 palliation is characterized by hemodynamic instability, which in part may be due to excessive pulmonary blood flow. Hence, avoidance of alkalosis and the use of minimally oxygen-enriched inspiratory gas are thought by many to be important, although there is little pertinent published data. This study was undertaken to characterize the postoperative course and to determine whether the F io 2 and blood pH are related to indices of hemodynamic stability in these infants. Methods and Results The postoperative course of 25 consecutive infants undergoing first stage palliation for HLHS were retrospectively reviewed and the following data were obtained: arterial pressure, arterial blood gas measurements, the inotropic agents used, and multiple respiratory parameters. There was one operative death, and 2 patients died within 2 days, but 22 were extubated (mean, 5.2±4.1 days after surgery). Hospital mortality was 24%. Mean pH was ≥7.51 for the first 9 hours after surgery and was ≥7.45 for the entire period. The mean F io 2 was ≥50% for the first 18 hours. The Pa o 2 was appropriate (37±6 mm Hg at 1 hour after surgery, increasing to 45±5 mm Hg by hour 73). Only modest inotropic support was needed to maintain appropriate blood pressure. Conclusions These data suggest that neither alkalosis nor relatively high inspired oxygen necessarily cause hemodynamic instability in these patients. To what extent these results are generalizable is unclear, but they suggest that there is nothing inherent with HLHS that mandates postoperative hemodynamic instability or unacceptable mortality.

Since September 1991, 14 consecutive patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries have undergone staged repair. All patients had multiple aortopulmonary collateral arteries and the ductus arteriosus was absent in 11. Mean sizes of the right and left pulmonary arteries were 2.2 ±0.7 mm and 1.9 ±0.8 mm, respectively (range 0.5 to 3.0 mm). Eight patients (57%) have subsequently received complete repair. Age at initial procedure (shunt, right ventricle–pulmonary artery conduit, or direct aorta–pulmonary artery anastomosis) in this group was 5.3 ±6.8 months. The number of operative procedures to achieve complete repair was 2.9 ±0.8 per patient (range 2 to 4). Intraoperative postrepair peak right ventricle–left ventricle pressure ratio was 0.57 ±0.17. Six of 8 patients (75%) required additional interventional procedures (mean 1.5 ±1.2 per patient) for angioplasty of peripheral pulmonary artery stenoses, coil embolization of aortopulmonary collateral arteries, or intraoperative insertion of intravascular pulmonary artery stents. Mean follow-up from complete repair was 8.7 ±8.3 months (range 0.5 to 23.8 months) and is complete. There was one in-hospital death at 45 days, and one late cardiac death at 20.3 months. Six patients had initial palliative operations (unifocalization, right ventricle–pulmonary artery conduit, direct aorta–pulmonary artery anastomosis, or transannular outflow patch) but have not undergone complete repair. Age at initial procedure in this group was 27.9 ± 56.9 months (range 0.27 to 155 months), and mean follow-up from initial procedure was 10.9 ± 11.2 months (range 0 to 31.4 months). The operative mortality rate was 33% (2 of 6 patients). There was one late noncardiac death at 5.3 months. Three patients are awaiting further intervention or repair. This experience suggests that complete repair is feasible even in patients with extremely diminutive pulmonary arteries (≤3.0 mm). Pulmonary artery growth is facilitated by early (3 to 6 month) establishment of central pulmonary artery flow by right ventricle–pulmonary artery conduit (pulmonary arteries >1.5 mm) or by direct ascending aorta–pulmonary artery anastomosis (pulmonary arteries

The optimal treatment of critical aortic stenosis in the neonate and infant remains controversial. We compared transventricular dilation using normothermic cardiopulmonary bypass and percutaneous balloon aortic valvuloplasty with respect to early and late survival, relief of aortic stenosis, degree of aortic insufficiency, left ventricular function, and freedom from reintervention. Between July 1987 and July 1993, 30 neonates and infants underwent transventricular dilation or balloon aortic valvuloplasty for critical aortic stenosis. The patients in the transventricular dilation group (n = 21) ranged in age from 1 to 59 days (mean age 18.0 days +/- 19.1 days) and the balloon aortic valvuloplasty group (n = 9) from 1 to 31 days (mean age 10.0 days +/- 9.0 days). There were no significant differences in weight, body surface area, or aortic anulus diameter between the two groups (p = 1.0). Associated cardiovascular anomalies were more common in the transventricular dilation group (48%) than in the balloon aortic valvuloplasty group (11%). After intervention, the degree of residual aortic stenosis and insufficiency was equivalent in the two groups as assessed by postprocedural Doppler echocardiography. Ejection fraction improved within both groups (transventricular dilation 39% +/- 20.2% versus 47% +/- 22.0%; balloon aortic valvuloplasty 51% +/- 16.1% versus 62% +/- 8.4%), and there was no significant difference between groups. The left ventricular mass/volume ratio increased within both groups (p < 0.05) but with no significant difference between groups (transventricular dilation 1.4 +/- 0.5 gm/ml versus 1.8 +/- 0.6 gm/ml; balloon aortic valvuloplasty 1.1 +/- 0.6 gm/ml versus 1.7 +/- 0.4 gm/ml). Early mortality in the transventricular dilation group was 9.5% and in the balloon aortic valvuloplasty group, 11.1%. There was one late death in the transventricular dilation group. Four patients from the transventricular dilation group (19%) and two patients from the balloon aortic valvuloplasty group (22%) required reintervention for further relief of aortic stenosis. We conclude that both transventricular dilation and balloon aortic valvuloplasty provide adequate and equivalent relief of critical aortic stenosis. The treatment strategy adopted should depend on other factors, including associated cardiovascular anomalies, vascular access, preoperative condition, and the technical expertise available at each institution.