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There are no reports on the performance of the arterial switch operation (ASO) in a normal heart with normally related great vessels. The objective of this study was to determine whether the ASO could be performed in a healthy animal model. Cardiopulmonary bypass (CPB) and coronary translocation techniques were used to perform ASO in neonatal piglets or a staged ASO with prior main pulmonary artery (PA) banding. Primary ASO was performed in four neonatal piglets. Coronary translocation was effective with angiograms confirming patency. Piglets could not be weaned from CPB due to right ventricle (RV) dysfunction. To improve RV function for the ASO, nine piglets had PA banding. All survived the procedure. Post-banding RV pressure increased from a mean of 20.3 ± 2.2 mmHg to 36.5 ± 7.3 mmHg (p = 0.007). At 58 ± 1 days post-banding, piglets underwent cardiac MRIs revealing RV hypertrophy, and RV pressure overload with mildly reduced RV function. Catheterization confirmed RV systolic pressures of 84.0 ± 6.7 mmHg with LV systolic pressure 83.3 ± 6.7 mmHg (p = 0.43). The remaining five PA banded piglets underwent ASO at 51 ± 0 days post-banding. Three of five were weaned from bypass with patent coronary arteries and adequate RV function. We were able to successfully perform an arterial switch with documented patent coronary arteries on standard anatomy great vessels in a healthy animal model. To our knowledge this is the first time this procedure has been successfully performed. The model may have implications for studying the failing systemic RV, and may support a novel approach for management of borderline, pulsatile left ventricles.

Insertion of conduits from the right ventricle (RV) to the pulmonary artery (PA) is a commonly used technique for repair of congenital heart defects. The vast majority of infants and children will require reoperation and/or re-intervention to replace the conduit. Some children may require multiple reoperations, with the risk of death and morbidity increasing significantly with each subsequent operation. We evaluated the feasibility and performance of a relatively novel anisotropic conduit for cardiovascular repair in the growing lamb model.Lambs were allocated into a control (Control conduits did not expand, all 3 animals developed one or more adverse events including tachypnea, ascites, inappetence, lethargy, and mortality due to severe right heart failure and significantly higher peak trans-conduit gradients (48.5 ± 5.1 p = 0.02). The test conduits spontaneously expanded up to 14.8 ± 0.8 mm in diameter, no adverse events were observed in any animals and trans-conduit gradients were significantly lower (27.0 ± 8.3, p = 0.02).Anisotropic ePTFE conduits can be safely implanted in growing lambs with stable hemodynamics. This spontaneously expanding anisotropic conduit may represent a novel approach to congenital heart repairs that would avoid the need for reoperation or multiple operations.

Cardiovascular repair in children often requires implant of conduits which do not have growth potential and will require reoperation. In the current study we sought to determine the feasibility of catheter-based interventions of anisotropic conduits inserted as interposition grafts in the main pulmonary artery (MPA) of growing lambs.Lambs underwent interpositional implant of either an anisotropic expanded polytetrafluoroethylene (ePTFE) (Test) conduit or conventional PTFE (Control) conduit. In the postoperative period, lambs were anesthetized and underwent catheter-based interventions consisting of hemodynamic and angiographic data collection, balloon dilation and/or stenting of the conduit at 3, 6 or 9 month postoperative time point.At 3 months, control lambs showed significant increases in right ventricular pressures and trans-conduit gradients in comparison to test lambs. Test conduit diameters were significantly larger compared to controls due to spontaneous radial expansion of the anisotropic conduit. Balloon dilation of test conduits at 3 and 6 months showed a reduction in RV pressure and statistically significant improvement in the RV outflow tract gradient as well as significant increase in graft diameter, compared to both control and pre-dilation conditions. Furthermore, the test conduit diameter increased significantly compared to the pre-balloon and control conditions at each time point. Necropsy of test conduits showed no evidence of tears, perforations, or clot and smooth interiors with well-healed anastomoses.Anisotropic conduits implanted as interposition grafts in the MPA show spontaneous expansion, and can safely and effectively undergo catheter-based interventions, with significant increases in graft diameter occurring after balloon dilation.

BACKGROUND Four similar transfusion reactions involving infants were reported in less than 1 year. After transfusion of red blood cells (RBCs) via syringe in the operating room, each patient experienced discolored urine, laboratory evidence of hemolysis, and acute kidney injury. Clerical and serologic investigations were unremarkable. Mechanical hemolysis was considered. STUDY DESIGN AND METHODS Simulated syringe transfusions were performed. Measurements included hematocrit (Hct), free hemoglobin, and visual hemolysis index. Washed and unwashed RBCs were tested with or without a recently introduced one-way valve, using a 24- or 16-gauge intravenous catheter. Constant manual pressure (1.43 ± 0.49 mL/sec) or syringe pump (2 mL/min) was used and a subset was timed. RESULTS The valve increased hemolysis during manual transfusion using both catheters with washed and unwashed RBCs. With the 24-gauge catheter, the change in Hct was -3.53 ± 0.69% with the valve and 0.22 ± 0.13% without (p

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital cardiac lesion, is an important cause of myocardial ischemia and infarction in children, and carries a high mortality in the first year of life. In the current era, repair of ALCAPA is most commonly performed by aortic implantation. Coronary elongation techniques are available for cases where the ectopic coronary artery originates at point in the pulmonary artery that is distant from the aortic root. In cases where clinically significant mitral regurgitation (MR) is present mitral valve repair is usually not performed. The early outcomes for aortic implantation of ALCAPA are excellent in the current era, with survival rates exceeding 90%. A small number of children, those who present with cardiogenic shock and very poor ventricular function, may require post-repair mechanical circulatory support using ECMO or LVAD and still have high survival rates and expect good long-term recovery. Following repair of ALCAPA by aortic implantation, ventricular functional parameters such as depressed ejection fraction, ventricular dilation and MR should recover within 8 months of repair.

Purpose Heart failure is a significant cause of morbidity and mortality in congenital heart disease patients especially for the subset whose right ventricle functions as the systemic ventricle. The physiological and molecular differences in the right ventricle (RV) and left ventricle (LV) lead to different abilities to adapt to adverse conditions and respond to pharmacological therapeutics. Currently data describing the molecular changes that occur in the systemic RV are lacking. Available animal models create an RV with volume and/or pressure overload however the LV remains at high pressure. Ventricular-ventricular interactions have a critical impact on cardiac function and are likely to alter gene expression independent of RV function. Methods To explore the distinct genetic regulatory networks of the RV and LV, we sequenced RNA from the RV and LV of late fetal, 1-week old, and 1-month old piglets. We identified gene expression differences in both chambers over this time course. Further, we have established a novel large animal model which creates a high pressure systemic RV with a low pressure subpulmonic LV by performing a reverse arterial switch followed by an atrial septectomy to allow sufficient oxygenation via atrial mixing. Using samples isolated from the RV following the reverse arterial switch procedure, we will determine the transcriptomic changes associated with the systemic RV. Gene expression profiles will be compared to age matched normal RV to identify molecular pathways that are perturbed in the systemic RV and compared to our LV and RV postnatal remodeling data to identify beneficial and pathogenic pathways for the RV under increased loading conditions. Results Analysis of postnatal cardiac chambers shows differentially expressed RV genes cluster into distinct patterns. In preliminary studies to generate a systemic RV, our team has successfully executed acute surgery in four week old piglets. Biventricular function was maintained with adequate systemic oxygenation saturation and angiography established that both the right and left reimplanted coronary arteries were patent. Conclusion As predicted by the lack of RV response to LV based therapies, our data confirmed that the genetic signature of the RV and LV during postnatal cardiac remodeling is distinct. Creation of a novel systemic RV model provides a platform to develop new therapeutics for congenial heart disease patients.

Background Hemidiaphragm paralysis from phrenic nerve injury is a known complication of congenital cardiac surgery. Return of diaphragm function has been reported; however, prior studies on this subject have been limited by small numbers, static assessment methods, or observation of plicated or non-plicated patients alone. To describe return of function, we reviewed fluoroscopy and ultrasonography in all diagnosed cases of diaphragmatic paralysis. Methods Surgical cases at our institution between 1991 and 2010 were identified for patients with postoperative hemidiaphragm paralysis diagnosed by chest X-ray, ultrasound, or fluoroscopy. Follow-up ultrasound and fluoroscopic studies were reviewed for return of diaphragm function. Results Seventy-two cases of postoperative hemidiaphragm paralysis were identified. Forty cases were plicated prior to discharge. Plicated patients were younger at time of diagnosis (median 46 days average 3.6 months; p = 0.025) and had a larger proportion of single ventricle diagnoses (48% vs 16%) compared with non-plicated patients. Twenty-six patients with paralysis were excluded in follow-up due to lack of studies documenting diaphragm function after the diagnostic study. Of the remaining 46 cases, median follow-up was 353 days (range: 6 days to 17 years). Plicated and non-plicated patients regained function at similar frequency (60% and 54.8%, respectively). Plication status, Risk Adjustment for Congenital Heart Surgery (RACHS) 1 score, age at diagnosis, and side of paralysis did not predict failure of recovery. Conclusions In the current era, return of diaphragm function after phrenic nerve injury sustained during congenital cardiac surgery is a known occurrence; however, predicting recovery continues to be difficult.

Background Pulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN). Objective We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN. Results Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen. Conclusions We report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.

Objective To compare the course of hypoplastic left heart syndrome (HLHS) patients diagnosed prenatally with any degree of atrial restriction with those without evidence of atrial restriction. Design Retrospective, cohort. Methods Prenatally diagnosed HLHS patients from August 1999 to January 2009 were categorized as nonrestrictive versus restrictive, defined by left atrial hypertension on pulmonary venous Doppler and/or an intact interatrial septum. Results Of 73 total fetal patients identified, 49 were live-born. Survival at 2 years was 29/35 [83% confidence interval (CI): 59.5%–88.9%] for the nonrestrictive group and 6/14 (43% CI: 17.7%–6.0%) for the restrictive group (p

Background The purpose of this study is to determine the outcome and performance of bovine pericardial valves in the pulmonary position. Methods This is a retrospective review of all patients with congenital heart disease who had pulmonary valve replacement using a bovine pericardial valve from 2002 to 2009 at a single institution. Results There were 73 consecutive patients, with a median age of 17.3 years (range, 2.1 to 64.4). Their diagnosis was tetralogy of Fallot (n = 47), pulmonary stenosis (n = 11), or other (n = 15). Sixty-nine patients had 91 previous surgical procedures. The mean time from last surgery was 19.9 ± 11.6 years. Forty-three patients had concomitant surgical procedures. There were no perioperative deaths. Clinical follow-up was available in 68 patients (93%). There were no late deaths, and all patients were in New York Heart Association functional class I during a median follow-up period of 2.6 years (range, 0.2 to 8.0). One patient had endocarditis necessitating valve removal 2 years after surgery. Freedom from pulmonary valve reoperation was 100%, 97.7%, and 97.7% at 1, 3, and 5 years, respectively (95% confidence interval: 93.2% to 100%). Mean pulmonary valve gradient at follow-up was 19 ± 14 mm Hg. Degree of pulmonary insufficiency was less than moderate in 62 patients, moderate in 4, and more than moderate in 2. Freedom from moderate-severe or severe pulmonary insufficiency was 97.7%, 89.1%, and 89.1% at 1, 3, and 5 years, respectively (5-year 95% confidence interval: 77.0% to 100%). Conclusions Pulmonary valve replacement using a bovine pericardial valve can be accomplished with low perioperative morbidity and favorable midterm outcomes. Further follow-up is necessary to evaluate the long-term performance of bovine pericardial valves in the pulmonary position.

Objective Preoperative brain injury, particularly stroke and white matter injury, is common in neonates with congenital heart disease. The objective of this study was to determine the risk of hemorrhage or extension of preoperative brain injury with cardiac surgery. Methods This dual-center prospective cohort study recruited 92 term neonates, 62 with transposition of the great arteries and 30 with single ventricle physiology, from 2 tertiary referral centers. Neonates underwent brain magnetic resonance imaging scans before and after cardiac surgery. Results Brain injury was identified in 40 (43%) neonates on the preoperative magnetic resonance imaging scan (median 5 days after birth): stroke in 23, white matter injury in 21, and intraventricular hemorrhage in 7. None of the brain lesions presented clinically with overt signs or seizures. Preoperative brain injury was associated with balloon atrial septostomy ( P = .003) and lowest arterial oxygen saturation ( P = .007); in a multivariable model, only the effect of balloon atrial septostomy remained significant when adjusting for lowest arterial oxygen saturation. On postoperative magnetic resonance imaging in 78 neonates (median 21 days after birth), none of the preoperative lesions showed evidence of extension or hemorrhagic transformation (0/40 [95% confidence interval: 0%–7%]). The presence of preoperative brain injury was not a significant risk factor for acquiring new injury on postoperative magnetic resonance imaging ( P = .8). Conclusions Clinically silent brain injuries identified preoperatively in neonates with congenital heart disease, including stroke, have a low risk of progression with surgery and cardiopulmonary bypass and should therefore not delay clinically indicated cardiac surgery. In this multicenter cohort, balloon atrial septostomy remains an important risk factor for preoperative brain injury, particularly stroke.

Objective The objective of the study was to determine perioperative B-type natriuretic peptide levels in infants and children undergoing bidirectional cavopulmonary anastomosis or total cavopulmonary connection, and the predictive value of B-type natriuretic peptide levels for outcome. Methods Plasma B-type natriuretic peptide levels were measured before and 2, 12, and 24 hours after surgery in 36 consecutive patients undergoing bidirectional cavopulmonary anastomosis (n = 25) or total cavopulmonary connection (n = 11). B-type natriuretic peptide levels were evaluated as predictors of outcome. Results B-type natriuretic peptide levels increased after surgery, peaking at 12 hours in most patients. In the bidirectional cavopulmonary anastomosis group, patients with 12-hour B-type natriuretic peptide ≥ 500 pg/mL had a longer duration of mechanical ventilation (165 ± 149 hours vs 20 ± 9 hours, P = .004), longer intensive care unit stay (11 ± 7 days vs 4 ± 2 days, P = .001), and longer hospital stay (20 days ± 12 vs 9 days ± 5, P = .003). A 12-hour B-type natriuretic peptide ≥ 500 pg/mL had a sensitivity of 80% and a specificity of 80% for predicting an unplanned surgical or transcatheter cardiac intervention, including transplantation ( P = .03). In the total cavopulmonary connection group, preoperative B-type natriuretic peptide levels were highest in patients with total cavopulmonary connection failure compared with patients with a good outcome (88 ± 46 pg/mL vs 15 ± 6 pg/mL, P = .03). Conclusion Postoperative B-type natriuretic peptide levels predict outcome after bidirectional cavopulmonary anastomosis, and preoperative levels are greater in patients with both early and late total cavopulmonary connection failure compared with patients with a good outcome.

ObjectivesNeonates undergoing cardiac surgery are at high risk for adverse outcomes. B-type natriuretic peptide is used as a biomarker in patients with cardiac disease, but the predictive value of B-type natriuretic peptide after cardiac surgery in neonates has not been evaluated. Therefore, the objective of this study was to determine the predictive value of perioperative B-type natriuretic peptide levels for postoperative outcomes in neonates undergoing cardiac surgery.MethodsPlasma B-type natriuretic peptide determinations were made before and 2, 12, and 24 hours after surgery in 36 consecutive neonates. B-type natriuretic peptide levels and changes in perioperative B-type natriuretic peptide were evaluated as predictors of postoperative outcome.ResultsB-type natriuretic peptide levels at 24 hours were lower than preoperative levels (24-h/pre B-type natriuretic peptide ratio < 1) in 29 patients (81%) and higher (24-h/pre B-type natriuretic peptide ratio ≥ 1) in 7 patients (19%). A 24-hour/pre B-type natriuretic peptide level of 1 or greater was associated with an increased incidence of low cardiac output syndrome (100% vs 34%, P = .002) and fewer ventilator-free days (17 ± 13 days vs 26 ± 3 days, P = .002), and predicted the 6-month composite end point of death, an unplanned cardiac operation, or cardiac transplant (57% vs 3%, P = .003). A 24-hour/pre B-type natriuretic peptide level of 1 or greater had a sensitivity of 80% and a specificity of 90% for predicting a poor postoperative outcome (P = .003).ConclusionIn neonates undergoing cardiac surgery, an increase in B-type natriuretic peptide 24 hours after surgery predicts poor postoperative outcome.

Background Papillary muscle rupture in the fetus and neonate is a rare event that leads to severe mitral or tricuspid insufficiency and is associated with high perinatal mortality. We undertook surgical repair of this lesion in the neonatal period and report on our midterm results. Methods Three neonates with tricuspid insufficiency and 1 infant with mitral insufficiency, all due to papillary muscle or chordal rupture, underwent surgical repair with artificial chordal replacement and a modification of the de Vega annuloplasty technique that allowed external adjustment of the annulus size under transesophageal echocardiographic guidance after separation from cardiopulmonary bypass. Results All patients recovered well from the operation. There have been no late deaths and no valve-related complications. On discharge, all 3 patients had evidence of trace to mild atrioventricular valve regurgitation. At a median follow-up of 33 months (range, 7 to 50; 123 patient-months), all 4 patients are growing normally. Three patients have had no change in the degree of tricuspid or mitral regurgitation. One patient required reoperation at 54 months postoperatively for acute mitral insufficiency secondary to separation of an artificial chorda from the ventricular wall. Conclusions Surgical repair of critical neonatal tricuspid and mitral insufficiency associated with papillary muscle or chordal rupture is feasible and can result in good early and midterm results. Our modification of the De Vega annuloplasty technique with the ability to externally adjust the size of the annulus under echocardiographic guidance may improve the accuracy of the repair in the neonate.

Background A coronary artery arising from the wrong aortic sinus, with or without a proximal course between the aorta and the pulmonary artery, may predispose to myocardial ischemia, myocardial infarction, or sudden death. Methods We repaired this lesion in 5 symptomatic patients, all of whom had experienced exercise-related chest pain, dyspnea, or syncopal episodes. All patients underwent patch angioplasty of the proximal anomalous coronary artery with glutaraldehyde-treated autologous pericardium. When the coronary artery coursed between the aorta and the main pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary artery to create additional space and prevent compression. Results All patients recovered well and to date have demonstrated no evidence of recurrent myocardial ischemia. Conclusions The combination of coronary artery angioplasty and translocation of the pulmonary artery seems to effectively address all the mechanisms that can generate ischemia.

ObjectivesThe transannular patch used to relieve right ventricular outflow tract obstruction in children with tetralogy of Fallot may result in pulmonary insufficiency. We hypothesized that pulmonary valve cusp augmentation with pericardium would decrease pulmonary insufficiency and improve the early outcome for transatrial–transpulmonary tetralogy of Fallot repair requiring transannular patch.MethodsSince November 2001, 41 patients with tetralogy of Fallot and 2 patients with isolated pulmonary valve stenosis had relief of right ventricular outflow tract obstruction with either a transannular patch plus pulmonary valve cusp augmentation (n = 18) or a transannular patch alone (n = 25). Data were retrospectively collected.ResultsThe median age (5.3 vs 3.2 months; P = .09) and weight (6.4 vs 5.2 kg; P = .3) were similar for the cusp augmentation and transannular patch groups, respectively. The diameter of the pulmonary valve annulus (6.4 vs 6.0 mm; P = .57) and the McGoon index (1.47 vs 1.69, P = .75) were also similar. The mean aortic clamp time (48 ± 18 minutes vs 52 ± 19 minutes; P = .46) and median cardiopulmonary bypass time (89 vs 91 minutes; P = .9) did not differ. One patient with a transannular patch died of multiorgan system failure. Patients with a pulmonary valve cusp augmentation had a shorter duration of intubation (1 vs 3 days; P < .001) and intensive care unit stay (2 vs 8 days; P < .001). Thirteen patients with a transannular patch and 1 patient with a pulmonary valve cusp augmentation required inotropic support for more than 72 hours (P = .001). Discharge echocardiograms demonstrated moderate or severe pulmonary insufficiency in 5 patients with a pulmonary valve cusp augmentation and in 21 patients with a transannular patch (P < .001). At 7.5 months, 3 patients (17%) with a pulmonary valve cusp augmentation had progression of pulmonary insufficiency.ConclusionsAugmentation of a pulmonary valve cusp reduces the incidence of clinically significant postoperative pulmonary insufficiency. This technique may improve the early outcome for children with tetralogy of Fallot requiring a transannular patch.

Background Congenital absence of the pulmonary valve syndrome (APVS) is a rare cardiac defect characterized by aneurysmal pulmonary arteries, hypoplastic pulmonary valve cusps, and tracheobronchial abnormalities. Absence of the pulmonary valve syndrome usually occurs in conjunction with ventricular septal defect (VSD) and right ventricular outflow tract obstruction (RVOTO). Surgical mortality rates as high as 16% to 56% have been reported. Here, we describe the surgical results using the Lecompte maneuver, reduction pulmonary arterioplasty, and a valved right ventricle to pulmonary artery (RV-PA) conduit. Methods Medical records and operative and echocardiography reports for all surgical APVS cases were retrospectively examined for pertinent clinical variables. A patient with left bronchial compression due to enlarged pulmonary arteries associated with totally anomalous pulmonary venous drainage (TAPVD) is included to illustrate the value of the Lecompte maneuver. Results From January 2002 to December 2004, 4 children with APVS had surgery at a median age of 5 months (range, 3 months to 3.5 years). Three had malalignment VSD and RVOTO. Four had respiratory signs (cough, wheeze, tachypnea, oxygen dependence, ventilator dependence), and all 5 had evidence of tracheobronchial compression by computed tomography or magnetic resonance imaging. Repair included a Lecompte maneuver, a valved conduit with reduction pulmonary arterioplasty, and VSD closure as necessary. The TAPVD patient had repair of the anomalous veins and a Lecompte maneuver. There have been no deaths, and all patients were discharged in good condition. Follow-up is complete at a median of 24 months (range, 9 to 35). Echocardiography has shown no progressive enlargement of the pulmonary arteries. Conclusions Use of the Lecompte maneuver, reduction arterioplasty, and a valved conduit for repair of APVS provides favorable early and midterm results.

Several congenital heart defects require surgery that acutely increases pulmonary blood flow (PBF). This can lead to dynamic alterations in postoperative pulmonary vascular resistance (PVR) and can contribute to morbidity and mortality. Thus the objective of this study was to determine the role of nitric oxide (NO), endothelin (ET)-1, and their interactions in the alterations of PVR after surgically induced increases in PBF. Twenty lambs underwent placement of an aortopulmonary vascular graft. Lambs were instrumented to measure vascular pressures and PBF and studied for 4 h. Before and after shunt opening, lambs received an infusion of saline ( n = 9), tezosentan, an ETA- and ETB-receptor antagonist ( n = 6), or Nω-nitro-l-arginine (l-NNA), a NO synthase (NOS) inhibitor ( n = 5). In control lambs, shunt opening increased PBF by 117.8% and decreased PVR by 40.7% ( P < 0.05) by 15 min, without further changes thereafter. Plasma ET-1 levels increased 17.6% ( P < 0.05), and total NOS activity decreased 61.1% ( P < 0.05) at 4 h. ET-receptor blockade (tezosentan) prevented the plateau of PBF and PVR, such that PBF was increased and PVR was decreased compared with controls at 3 and 4 h ( P < 0.05). These changes were associated with an increase in total NOS activity (+61.4%; P < 0.05) at 4 h. NOS inhibition (l-NNA) after shunt placement prevented the sustained decrease in PVR seen in control lambs. In these lambs, PVR decreased by 15 min ( P < 0.05) but returned to baseline by 2 h. Together, these data suggest that surgically induced increases in PBF are limited by vasoconstriction, at least in part by an ET-receptor-mediated decrease in lung NOS activity. Thus NO appears to be important in maintaining a reduction in PVR after acutely increased PBF.

Objective During repair of aortic coarctation through a left thoracotomy without cardiopulmonary bypass, clamping the proximal transverse aortic arch occludes antegrade flow to the left carotid and vertebral arteries. It is assumed that flow through the right carotid and vertebral arteries is adequate for cerebral perfusion. The study objective is to determine whether aortic occlusion impairs left hemispheric cerebral oxygen balance measured by near-infrared spectroscopy. Methods In 18 children having repair of aortic coarctation, we measured the maximum change and integral for hemoglobin D (difference of oxyhemoglobin and deoxyhemoglobin), total oxygenation index, and the redox state of cytochrome aa3. Thirteen subjects had recordings from the left hemisphere to test the hypothesis that aortic occlusion impairs left hemispheric oxygen balance. Five subjects had recordings from the right hemisphere for comparison. Results After aortic clamping, a significant decrease in hemoglobin D was observed in recordings from the left hemisphere compared with those from the right hemisphere ( P = .03, maximum change in hemoglobin D). Total oxygenation index and cytochrome aa3 were generally preserved. There was an inverse linear relationship for the change in hemoglobin D during clamp application and after removal (Spearman rho=−0.74), with increased hemoglobin D after clamp removal in those subjects with the greatest decrease of hemoglobin D during arch occlusion. Linear regression analysis identified nitroprusside administration as significantly associated with a decrease in hemoglobin D ( P Conclusions Significant impairment in left hemispheric cerebral oxygen balance was identified during arch clamping. The neurodevelopmental significance of impaired cerebral oxygen balance detected by near-infrared spectroscopy during aortic coarctation repair remains to be elucidated.

Ten cases of elective late pulmonary valve implantation after repair of tetralogy of Fallot were reviewed. The interval after initial repair ranged from 1.5 to 43 years (mean, 20.0 +/- 12.3 years). There was no hospital mortality or late death during a mean follow-up of 12.5 months. Preoperatively, 9 patients were in New York Heart Association functional class III-IV; after pulmonary valve implantation, all 10 patients were in class I-II (average improvement, 1.7 classes). Left ventricular ejection fraction improved significantly (from 62.1% +/- 4.7% to 70.2% +/- 4.9%), as did fractional shortening (from 34.0% +/- 5.0% to 40.0% +/- 4.2%). Right ventricular diameter decreased significantly (from 32.3 +/- 7.5 to 24.4 +/- 5.4 mm). QRS duration decreased significantly (155.2 +/- 27.1 vs. 140.0 +/- 21.2 msec), but there was no significant difference in QT interval (460.9 +/- 29.6 vs. 451.9 +/- 50.6 msec). Hospital stay was 4-7 days. One patient had preoperative ventricular fibrillation requiring resuscitation and an implantable cardiac defibrillator; another needed a defibrillator at the time of pulmonary valve implantation, because of ventricular arrhythmias. It was concluded that late pulmonary valve implantation after tetralogy of Fallot repair had significant benefits and carried low operative risk.

The right atrium has a significantly higher capacitance than the left atrium, and this may affect the Doppler flow pattern across an atrial septal defect (ASD) in unilateral atrioventricular (AV) valve atresia. This Doppler flow pattern is often used to assess ASD adequacy in this setting. We studied the effect of atrial capacitance and ASD size on the trans-ASD Doppler flow pattern in an in vivo flow model of alternate left or right AV valve atresia (LAVVA and RAVVA). We assessed trans-ASD Doppler flow patterns using the max/min velocity ratio and mean interatrial pressure gradients (PGs). In both models, ASD flow rate correlated with mean trans-ASD PG, but for similar flow rates the slope was higher in the LAVVA model. In LAVVA, a persistent PG was consistently observed, with low max/min ratio (median, 1.46; range, 1.03-3.13), whereas in RAVVA, phasic flow was common (median, 8.0; range, 2.8-20). Because atrial capacitance affects mean PG and Doppler flow pattern across the ASD, we propose that the assessment of ASD adequacy in RAVVA should not rely on Doppler findings.

To determine and compare outcome of the modified Norwood procedure using either a systemic to pulmonary artery (SPA) shunt or right ventricle to pulmonary artery (RV-PA) conduit in a consecutive series of neonates at a single institution.The medical records were retrospectively examined for preoperative demographic and echocardiographic data, operative variables, and postoperative clinical and hemodynamic data. From November 2001 to March 2003, 21 neonates had a modified Norwood procedure (SPA shunt, n = 8; RV-PA conduit, n = 13) at a median age of 5 days (range 1 to 18 days) and a median weight of 2.9 kg (range 1.7 to 4.1 kg). Of the 21 infants, 12 were considered high risk due to presence of low birth weight (n = 4), extracardiac or genetic anomalies (n = 5) or obstruction to pulmonary venous return (n = 5). Nine "high risk" infants were in the RV-PA conduit group.Overall Norwood operation survival was 90% (19/21) and did not differ between groups. There were 2/19 interstage deaths and Kaplan-Meier survival at 1 year is 79%. Neonates in the RV-PA conduit group had significantly higher diastolic blood pressures at 1, 6, and 24 hours postoperatively (p0.05). Neonates in the SPA shunt group had significantly higher heart rates at 1 hour postoperatively (p0.05) than those in the RV-PA group. There was a trend to higher number of ventilatory interventions to balance Qp:Qs in the SPA shunt group (p = 0.06).In a relatively high-risk group, neonates having an RV-PA conduit as part of the Norwood procedure have favorable postoperative hemodynamics and a good likelihood of stage I survival.

Background Whereas indications expand and results improve with increasing refinements to the Fontan procedure the overall impact on outcomes related to tricuspid atresia remains suboptimally defined. Methods We reviewed 225 consecutive patients presenting between 1971 and 1999. All patients had classic tricuspid atresia with absent right atrioventricular connection and with D-transposition of the great arteries in 21%, pulmonary outflow obstruction in 75%, and aortic outflow obstruction in 11%. Results Ten patients died before any intervention and 3 patients were lost to follow-up. Palliative procedures (includes 151 with systemic shunt, 27 pulmonary artery banding, 60 venous shunt) were performed in 203 patients, with 44 deaths, 8 patients awaiting Fontan, 12 patients Fontan contraindicated, and 11 patients lost-to-follow-up. A total of 137 patients had the Fontan procedure (9 patients without previous procedures) with 7 early deaths, 11 late deaths, and 3 patients progressing to heart transplantation. Total survival for the cohort was 90% at the age of 1 month, 81% at 1 year, 70% at 10 years, and 60% at 20 years with no significant change over the time period. Independent factors associated with ineligibility or death without Fontan (n = 68, 30%) included earlier birth date, lower birth weight, presence of aortic arch anomaly and greater right ventricular hypoplasia, and no palliative procedure. There were no significant changes in mortality with Fontan over the study time period with survival of 95% at 1 month, 93% at 1year, and 82% at 10 years. Conclusions Improvements in outcomes with tricuspid atresia will require attention to management and risk factors before Fontan.

Truncus arteriosus (common arterial trunk) is a devastating congenital anomaly which left untreated in some cases can cause death within hours. It has remained a challenge even in recent years. Children may present with heart failure, myocardial ischemia, cardiovascular collapse, or may be only mildly cyanotic and nearly asymptomatic. Repair consists of closure of the ventricular septal defect, separation of the pulmonary arteries from the arterial trunk, and insertion of a valved homograft between the right ventricle and pulmonary artery. The truncal valve is repaired if there is significant regurgitation. Concomitant arch reconstruction is performed in those with an interrupted aortic arch. Repair early in infancy has led to 95% survival and nearly eliminated the perioperative risk of pulmonary hypertensive crisis. Reintervention for right ventricular outflow obstruction is usually necessary and occurs between 4 and 7 years of age. Up to 40% of the truncal valves are at risk for late deterioration. Clinical outcomes are favorable in the present era; however, reintervention is a mandatory part of patient management.

ObjectiveThe study objective was to determine the association between preoperative B-type natriuretic peptide levels and outcome after total cavopulmonary connection. Surgical palliation of univentricular cardiac defects requires a series of staged operations, ending in a total cavopulmonary connection. Although outcomes have improved, there remains an unpredictable risk of early total cavopulmonary connection takedown. Theprediction of adverse postoperative outcomes is imprecise, despite an extensive preoperative evaluation.MethodsWe prospectively enrolled 50 patients undergoing total cavopulmonary connection. We collected preoperative clinical data, preoperative plasma B-type natriuretic peptide levels, and postoperative outcomes, including the incidence of an adverse outcome within 1 year of surgery (defined as death, total cavopulmonary connection takedown, or the need for cardiac transplantation).ResultsThe mean age of patients was 4.7 years (standard deviation, 2.1 years). The median (interquartile range) preoperative B-type natriuretic peptide levels were higher in patients who required total cavopulmonary connection takedown and early postoperative mechanical cardiac support (n=3; median, 55; interquartile range, 42-121) compared with those with a good outcome (n=47; median, 11; interquartile range, 5-17) (P&nbsp

Objective: To compare outcomes of extracardiac conduit and lateral tunnel Fontan connections in a single institution over a concurrent time period. Methods: Between January 1994 and September 1998, 60 extracardiac conduit and 47 lateral tunnel total cavopulmonary connections were performed. Age, sex, and weight did not differ between the 2 groups. Compared with the lateral tunnel group (LT group), patients undergoing the extracardiac conduit procedure (EC group) had a trend to a higher incidence of morphologically right ventricle (EC group 48% vs LT group 32%; P

In the last 15 years the development of catheter-directed percutaneous therapies have improved the delivery of care to children with congenital heart lesions. Paralleling these advances, enhanced surgical techniques are now applied to complex cardiac lesions previously thought to be inoperable. This chapter outlines several treatment strategies which utilize surgical and catheter-based algorithms to address congenital heart disorders.

We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV).Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6.Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2).Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.

Background . A common brachiocephalic trunk, in which both common carotid arteries and the right subclavian artery arise from a single trunk off the arch, is a normal variant of aortic arch branching that occurs in approximately 10% of the population. Because three of the four primary sources of cerebral blood flow arise from a single aortic branch, stenosis or occlusion of a common trunk can cause severe ischemic consequences. Common trunk revascularization has been described, but there have been no reports focusing on the management options for occlusive disease of this vascular anatomy. Methods . A retrospective review of our experience with innominate artery revascularization identified 6 patients who underwent revascularization of a common brachiocephalic trunk between 1977 and 1997. All patients were symptomatic, with either total occlusion (n = 3) or critical stenosis (n = 3) caused by atherosclerosis (n = 5) or Takayasu's arteritis (n = 1). Revascularization was achieved by a prosthetic bypass graft from the ascending aorta to the innominate or left common carotid arteries or both (n = 5); or transarterial endarterectomy (n = 1). Concomitant endarterectomy of branch vessels was performed in 3 patients. Results . There was one perioperative death from myocardial infarction, and one perioperative stroke, with death occurring 1 month after hospital discharge. One patient developed cerebral hyperperfusion syndrome 1 week after endarterectomy that resolved without sequelae with antihypertensive medications. During a follow-up period ranging from 1 to 20 years, there was one late death from congestive heart failure 5 years after operation. All surviving patients are alive and free from symptomatic recurrence. Conclusions . Revascularization for occlusive disease of a common brachiocephalic trunk can be achieved with effective and durable relief of symptoms using either a prosthetic bypass graft or endarterectomy. However, neurologic complications in 2 patients, which were fatal in 1, attest to the potential cerebral ischemic threat posed by occlusive disease of a common brachiocephalic trunk.

SUMMARY BACKGROUND DATA: Symptomatic atherosclerotic occlusive disease of the innominate artery is a threatening disease pattern that offers a major challenge in achieving definitive surgical repair. To assess the evolution of treatment strategies and their outcomes for this disease, the authors undertook a review of the cumulative experience for more than 3 decades at one institution. METHODS: Between 1960 and 1997, 94 patients (mean age, 62 years) underwent direct innominate artery revascularization for occlusive atherosclerotic disease to relieve neurologic (n = 85) and/or right upper extremity (n = 26) symptoms or asymptomatic critical stenosis (n = 3). The pattern of atherosclerotic involvement revealed by angiography included critical stenosis (n = 77), complete occlusion (n = 10), and moderate stenosis with plaque ulceration (n = 7). A common brachiocephalic trunk was present in five patients. Transsternal (n = 68) or transcervical (n = 4) innominate endarterectomy was performed in 72 patients and bypass grafting in 22. Forty-one patients underwent concomitant endarterectomy or bypass of innominate branches or adjacent arch vessels, and 3 had coronary bypass grafting. RESULTS: There were three perioperative deaths (3%), all due to cardiac causes. Postoperative morbidity included four strokes (three resolved), two myocardial infarctions, two transient ischemic attacks, and one sternal dehiscence. Follow-up ranged from 8 months to 20 years. Postoperative actuarial survival rate was 96% at 1 year, 85% at 5 years, and 67% at 10 years. Freedom from recurrence requiring reoperation was 100% at 1 year, 99% at 5 years, and 97% at 10 years. CONCLUSIONS: Innominate artery reconstruction is safe and durable when either endarterectomy or prosthetic bypass is used. The anatomic variation and disease distribution permit endarterectomy for most patients. The technique of innominate endarterectomy can be extended safely to outflow and adjacent vessels.

A patient with preoperative pulmonary hypertension and a large left to right intracardiac shunt underwent surgical correction of a complex cardiac anomaly and required extracorporeal life support for unexplained ventricular dysfunction following the procedure. Following recovery, a fistulous connection between the right coronary artery and main pulmonary artery was demonstrated. Implications and management strategies of unsuspected coronary fistula in pediatric heart surgery are discussed.

Objective: To determine the relationship between radiologically identifiable brain injuries and delayed brain development as reflected by brain metabolic and microstructural integrity. Methods: Term newborns with congenital heart disease (CHD) (120 preoperatively and 104 postoperatively) were studied with MRI to determine brain injury severity (BIS), microstructure reflected by fractional anisotropy (FA) and average diffusivity (D av ), and metabolism reflected by N -acetylaspartate (NAA)/choline (Cho) and lactate/Cho. Brain development is characterized by increasing NAA/Cho and white matter FA, and by decreasing D av and lactate/Cho. Results: Newly acquired brain injury was common (41% preoperative, 30% postoperative). Lower white matter FA ( p = 0.005) and lower NAA/Cho ( p = 0.01) were associated with increasing preoperative BIS. Higher neonatal illness severity scores ( p = 0.03), lower preoperative oxygen saturation ( p = 0.002), hypotension ( p p = 0.002) were also predictive of higher preoperative BIS. Preoperative FA, D av , and NAA/Cho did not predict new postoperative BIS. Increasing preoperative BIS predicted higher postoperative D av ( p = 0.002) and lactate/Cho ( p = 0.008). Within the postoperative scan, new brain injuries were associated with lower white matter FA ( p = 0.04). Postoperative BIS (new lesions) was associated with lower postoperative systolic ( p = 0.03) and mean ( p = 0.05) blood pressures. Conclusions: Brain injuries in newborns with CHD are strongly related to abnormalities of brain microstructural and metabolic brain development, especially preoperatively. Both newly acquired preoperative and postoperative brain injuries are related to potentially modifiable clinical risk factors.

Background Term newborns with congenital heart disease (CHD) show delayed brain development as early as the third trimester, especially in single ventricle physiology (SVP). Mechanisms causing delayed brain development in CHD are uncertain, but may include impaired fetal brain blood flow. Our objective was to determine if cardiac anatomy associated with obstruction to antegrade flow in the ascending aorta is predictive of delayed brain development measured by diffusion tensor imaging (DTI) and magnetic resonance spectroscopic imaging (MRSI). Methods Echocardiograms (ECHO) from 36 term newborns with SVP were reviewed for presence of aortic atresia and the diameter of the ascending aorta. Quantitative MR imaging parameters measuring brain microstructural (fractional anisotropy (FA), average diffusivity (Dav)) or metabolic development (N-acetylaspartate (NAA), Lactate/choline (Lac/cho)) were recorded. Results Increasing NAA/cho and white matter FA, and decreasing Dav and lactate/cho characterize normal brain development. Consistent with the hypothesis that delayed brain development is related to impaired brain perfusion, smaller ascending aortic diameter and aortic atresia were associated with higher Dav and lower white matter FA. ECHO variables were not associated with brain metabolic measures. Conclusions These observations support the hypothesis that obstruction to fetal cerebral blood flow impairs brain microstructural development.

ObjectiveThe right ventricle (RV) demonstrates differential adaptations in response to pressure versus volume loading, a phenomenon that may be important in the management of children and adults with congenital heart disease (CHD). The purpose of this study is to elucidate possible transcriptional mechanisms of the RV response to pressure versus volume loading in vivo.MethodsFetal lambs had aortopulmonary shunting or pulmonary artery (PA) banding. Four weeks after spontaneous delivery, ovine hearts were evaluated for hemodynamic changes and changes in expression of sarcomeric gene proteins and transcriptional factors. Western blot densitometry and chromatin immunoprecipitation were applied using standard techniques. Transactivation assays were performed using transient transfections in Schneider's Drosophila line 2 cells in culture.ResultsAfter PA banding, the RV pressure increased from 36 ± 4 mm Hg (n = 4) to 96 ± 8 mm Hg (n = 4, P

Background: Left ventricular outflow tract obstruction (LVOTO) is caused by a spectrum of lesions. This study was performed to determine the outcomes of surgical management of LVOTO. Methods: All patients who had surgery of the LVOT between 2002 and 2010 were retrospectively reviewed. Results: There were 103 consecutive patients with median age 6.8 years (range 8 days to 62 years). Fourteen patients had simple subaortic membrane. Eighty-nine patients had complex LVOTO including fibromuscular obstruction (n = 53), tunnel obstruction (n = 22), hypertrophic cardiomyopathy/muscular obstruction (n = 15), and anomalies of the mitral subvalvar apparatus (n = 13). There were no early deaths. Mean LVOT gradient decreased from 33 mmHg (range 1 to 108 mmHg) to 6 mmHg (range 0 to 45 mmHg) (p < 0.001). Median follow-up was 3.8 years (range 0.9 to 8.5 years). There were three late deaths. Cumulative survival at one, three, and five years was 96% (95% CI 89% to 99%). All patients are in New York Heart Association classes I-II. Ten patients required reoperation (three for recurrent/residual LVOTO). Freedom from reoperation was 94%, 90%, and 78% at one, three, and five years (95% CI 86% to 98%, 80% to 95%, and 59% to 89%, respectively). No patient with complex LVOTO who had release of the fibrous trigones required reoperation [0% (0/26) vs. 16% (10/63) (p = 0.031)]. Factors associated with increased reoperation risk were interrupted aortic arch (OR 6.4, p = 0.22), atrioventricular septal defect (OR 15.4, p = 0.008), and higher mean LVOT gradient at discharge (OR 1.08, p = 0.023). Conclusions: Utilizing a multitude of operative strategies for surgery of the LVOT results in favorable early and midterm outcomes. Residual LVOTO and original cardiac diagnosis are associated with increased reoperation risk. Release of the fibrous trigones decreases reoperation risk in patients with complex LVOTO. (J Card Surg 2012;27:103–111)

More than 60% of newborns with severe congenital cardiac disease develop perioperative brain injuries. Known risk factors include: pre-operative hypoxemia, cardiopulmonary bypass characteristics, and post-operative hypotension. Infection is an established risk factor for white matter injury in premature newborns. In this study, we examined term infants with congenital cardiac disease requiring surgical repair to determine whether infection is associated with white matter injury. Acquired infection was specified by site – bloodstream, pneumonia, or surgical site infection – according to strict definitions. Infection was present in 23 of 127 infants. Pre- and post-operative imaging was evaluated for acquired injury by a paediatric neuroradiologist. Overall, there was no difference in newly acquired post-operative white matter injury in infants with infection (30%), compared to those without (31%). When stratified by anatomy, infants with transposition of the great arteries, and bloodstream infection had an estimated doubling of risk of white matter injury that was not significant, whereas those with single ventricle anatomy had no apparent added risk. When considering only infants without stroke, the estimated association was higher, and became significant after adjusting for duration of inotrope therapy. In this study, nosocomial infection was not associated with white matter injury. Nonetheless, when controlling for risk factors, there was an association between bloodstream infection and white matter injury in selected sub-populations. Infection prevention may have the potential to mitigate long-term neurologic impairment as a consequence of white matter injury, which underscores the importance of attention to infection control for these patients.

Background The "sutureless" repair technique has improved outcomes for post-repair pulmonary vein (PV) stenosis. The purpose of this study is to determine the early outcomes of primary sutureless repair of pulmonary venoocclusive disease in infants with congenital PV stenosis-hypoplasia or PVs at high risk for progressive stenosis. Methods This is a retrospective review of infants who had primary sutureless repair of the PVs from October 2002 to April 2010. Results Twenty-five infants had primary sutureless repair of the PVs. Eighteen infants had total anomalous pulmonary venous return; 14 with obstruction, 10 with heterotaxy syndrome, and 9 with univentricular anatomy. Seven infants had congenital PV stenosis. There were 24 perioperative survivors (96%; 95% confidence interval [CI], 75% to 99%) and 2 late deaths from extracardiac causes. Follow-up was available on 21 out of 22 survivors at a median duration of 34 months (range, 9 to 100 months). Persistence-recurrence of PV stenosis occurred in 3 veins (3%) of 2 infants (8%). On follow-up echocardiography, right ventricular systolic pressure was normal in 13 out of 14 infants with a biventricular heart and 60% of systemic blood pressure in 1 infant. Kaplan-Meier 1-year cumulative survival was 88% (95% CI, 66% to 96%). Kaplan-Meier cumulative disease-free survival was 96% (95% CI, 75% to 99%) at 30 days and 84% (95% CI, 58% to 95%) at 1 year. By Cox proportional hazards, age, univentricular anatomy, and atrial isomerism-heterotaxy syndrome were not associated with an increased risk of death or persistence-recurrence. One-year disease-free survival was lower in infants with prematurity ( p = 0.0055) and low birth weight ( p = 0.0011). Conclusions Primary sutureless repair is a feasible, safe, and relatively effective method of addressing congenital PV stenosis and (or) high-risk PVs, particularly in infants with single ventricle anatomy and (or) heterotaxy syndrome.

We sought to evaluate the relation of a prenatal diagnosis (preDx) with morbidity and mortality during the initial hospitalization in a contemporary cohort of patients with hypoplastic left heart syndrome (HLHS). A retrospective study of patients with HLHS presenting from 1999 to 2010 was performed. Patients with genetic disorders or a gestational age

We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction.

We describe a case of severe aortic stenosis in a 16-year-old male with Hurler's syndrome who had prior bone marrow transplantation. The excised aortic valve leaflets showed characteristic pathologic findings of Hurler's syndrome. This is the first case report of aortic valve replacement in a patient with Hurler's syndrome treated with bone marrow transplantation that demonstrates progression of the aortic valve disease despite treatment.

Low birth weight (LBW) is a risk factor for mortality in neonatal and infant heart surgery. The purpose of this study was to determine the contemporary outcomes and risk factors of cardiac surgery in low weight babies. The records of 75 consecutive infants weighing

Background A modification of the Fontan operation was recently applied, which includes anastomoses of the extracardiac conduit to the right pulmonary artery and inferior vena cava using simple clamping with no additional circulatory support, venous shunting, pulmonary artery preparation, or prior maintenance of azygos vein patency. The objective of this study is to assess the outcomes of this novel off-pump "clamp and sew" Fontan procedure. Methods This is a retrospective review of all patients having a Fontan procedure between January 2009 and October 2010 at a single institution. Results Twelve patients had a Fontan procedure with the use of cardiopulmonary bypass (CPB group), and 12 had an off-pump Fontan procedure (off-pump group). Preoperative demographic and hemodynamic data were similar except for higher mean pulmonary artery pressure in the CPB group (12.2 ± 1.6 mm Hg versus 9.9 ± 2.4 mm Hg; p = 0.02). No patients in the off-pump group required conversion to CPB. The mean inferior vena cava clamp time in the off-pump group patients was 10 ± 3 minutes. There were no early or midterm deaths. No patients exhibited postoperative hepatic or renal dysfunction. Postoperative maximal serum creatinine and aspartate transaminase were significantly lower in the off-pump group compared with the CPB group (0.59 ± 0.12 versus 0.77 ± 0.22 mg/dL; p = 0.03 and 35.5 ± 8.3 versus 53.1 ± 19.0 U/L; p = 0.02, respectively). At median follow-up of 13 months (range, 1 to 20 months), all but 1 patient in the CPB group are in New York Heart Association class I with unobstructed Fontan circulation. Conclusions The clamp and sew technique for completion of an extracardiac conduit Fontan procedure appears safe and feasible for selected patients.

Although the risk of sudden death in children and young adults with anomalous coronary arteries arising from the wrong sinus of Valsalva is well described, the risk for older adults who present with such anatomy is unclear. Age greater than 30 years has been shown to impart a diminished risk of sudden death, although it can occur in older adults. Although simple coronary artery bypass for these patients has been described, the outcome can be hampered by competitive flow in the absence of concomitant coronary disease, and poor long-term patency can be expected. Other techniques include internal unroofing, either across the commissural post or not, with a possible effect on aortic valve function. We report a simple physiologic approach previously described in children and adolescents and now used in 2 cases of symptomatic adult patients with anomalous coronary arteries arising from the wrong sinus of Valsalva with an intra-arterial course between the pulmonary artery and aorta.

Congenital heart disease in newborns is associated with global impairment in development. We characterized brain metabolism and microstructure, as measures of brain maturation, in newborns with congenital heart disease before they underwent heart surgery.We studied 41 term newborns with congenital heart disease--29 who had transposition of the great arteries and 12 who had single-ventricle physiology--with the use of magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) before cardiac surgery. We calculated the ratio of N-acetylaspartate to choline (which increases with brain maturation), the ratio of lactate to choline (which decreases with maturation), average diffusivity (which decreases with maturation), and fractional anisotropy of white-matter tracts (which increases with maturation). We compared these findings with those in 16 control newborns of a similar gestational age.As compared with control newborns, those with congenital heart disease had a decrease of 10% in the ratio of N-acetylaspartate to choline (P=0.003), an increase of 28% in the ratio of lactate to choline (P=0.08), an increase of 4% in average diffusivity (P0.001), and a decrease of 12% in white-matter fractional anisotropy (P0.001). Preoperative brain injury, as seen on MRI, was not significantly associated with findings on MRS or DTI. White-matter injury was observed in 13 newborns with congenital heart disease (32%) and in no control newborns.Term newborns with congenital heart disease have widespread brain abnormalities before they undergo cardiac surgery. The imaging findings in such newborns are similar to those in premature newborns and may reflect abnormal brain development in utero.