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BackgroundSurgery for CHD has been slow to develop in parts of the former Soviet Union. The impact of an 8-year surgical assistance programme between an emerging centre and a multi-disciplinary international team that comprised healthcare professionals from developed cardiac programmes is analysed and presented.Material and methodsThe international paediatric assistance programme included five main components – intermittent clinical visits to the site annually, medical education, biomedical engineering support, nurse empowerment, and team-based practice development. Data were analysed from visiting teams and local databases before and since commencement of assistance in 2007 (era A: 2000–2007; era B: 2008–2015). The following variables were compared between periods: annual case volume, operative mortality, case complexity based on Risk Adjustment for Congenital Heart Surgery (RACHS-1), and RACHS-adjusted standardised mortality ratio.ResultsA total of 154 RACHS-classifiable operations were performed during era A, with a mean annual case volume by local surgeons of 19.3 at 95% confidence interval 14.3–24.2, with an operative mortality of 4.6% and a standardised mortality ratio of 2.1. In era B, surgical volume increased to a mean of 103.1 annual cases (95% confidence interval 69.1–137.2, pConclusionsThe model of assistance described in this report led to improved adjusted mortality, increased case volume, complexity, and independent operating skills.

BackgroundBalloon atrial septostomy is performed in infants with dextro-transposition of the great arteries to improve oxygenation before surgery. It is performed in the catheterisation laboratory with fluoroscopy or at the bedside using echocardiography. It is unclear whether procedural safety and efficacy is superior in one location versus the other, although the bedside procedure may improve resource utilisation and present an opportunity for reducing cost. This study compares safety and efficacy of atrial septostomy performed at the patient’s bedside versus the catheterisation laboratory.MethodsNeonates with dextro-transposition of the great arteries who underwent balloon atrial septostomy from October, 2000 to January, 2014 were included. Medical and procedural records, echocardiograms, and catheterisation data were reviewed. Comparisons between the two procedural locations included patient demographics, pre- and post-procedure oxygen saturations, and outcomes. Complications reviewed included bleeding, arrhythmia, cardiac trauma, stroke, and death. Coronary artery evaluations were recorded. T-tests were used for continuous variables, and Fisher’s exact tests were used for all categorical variables. Wilcoxon rank sum and analysis of covariance modelling were used for time variables and oxygen saturation, respectively.ResultsA total of 88 infants met the inclusion criteria. Among them, 53 underwent septostomy at the bedside and 35 underwent septostomy in the catheterisation laboratory. No safety or outcome benefit was identified between the two procedural locations.ConclusionSeptostomy performed at the bedside and in the catheterisation laboratory had similar outcomes and efficacy. Further, bedside septostomy has the advantage of no radiation exposure, and obviating risks with patient transfer from the ICU to the catheterisation laboratory.

BackgroundPulmonary balloon valvuloplasty is a safe and effective treatment for children with pulmonary valve stenosis. A few studies evaluate the long-term outcomes of the procedure, particularly the degree of pulmonary regurgitation. We evaluated the outcomes of children >1 year following valvuloplasty for pulmonary valve stenosis.MethodsA retrospective analysis of children with pulmonary valve stenosis following pulmonary balloon valvuloplasty at a single institution was performed. Clinic summaries, catheterisation data, and echocardiographic data were reviewed. Inclusion criteria were isolated pulmonary valve stenosis, age ResultsA total of 53 patients met inclusion criteria. The median age at valvuloplasty was 0.4 years (0.01–10.6 years). The last follow-up was 4.8±2.3 years following valvuloplasty. The pre-valvuloplasty peak instantaneous gradient by echocardiography was 60.6±14.6 mmHg. The peak gradient at the first postoperative echocardiography was reduced to 25.5±12 mmHg (pConclusionsPulmonary balloon valvuloplasty remains a safe and effective treatment for children with isolated pulmonary valve stenosis, with excellent long-term outcomes and no mortality. A few patients require further intervention. Long-term follow-up demonstrates decreased, residual stenosis. Patients have a small, acute increase in pulmonary regurgitation following valvuloplasty, but no long-term progression.

Dextro-transposition of the great arteries (d-TGA) is a lethal congenital heart defect in which the great arteries-the pulmonary artery and aorta-are transposed to create ventriculoarterial discordance. Corrective surgical interventions have resulted in significant improvements in morbidity and mortality for this once-fatal congenital heart defect. The initial palliative surgery for d-TGA was the atrial switch operation, which provided physiological correction. The Mustard and Senning "atrial switch" procedures, in which an atrial baffle is created to produce a discordant atrioventricular connection on the existing discordant ventriculoarterial connection, showed preliminary success for the correction of d-TGA. However, follow-up evaluations demonstrated increasing complications from the right ventricle utilized as a systemic ventricle, resulting in progressive right ventricular dysfunction. Thus, the search continued for an anatomical correction of d-TGA to return the great arteries to their normal ventricular connections. The arterial switch operation (ASO), though attempted and theorized by many, was first successfully performed by Dr. Jatene and colleagues in 1975. For ASO, the distal main pulmonary artery and the distal ascending aorta are transected and then anastomosed to their respective ventricles with relocation of the coronary arteries to the neoaorta. The ASO has replaced the atrial switch operation since the 1980s and is now the standard surgical correction for d-TGA. As more patients who have undergone ASO are living into adulthood, late complications of this procedure have become more evident. The most common late postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency, and neopulmonic stenosis. Adults who have undergone ASO in childhood will need follow-up with surveillance imaging and evaluation of new symptoms or declining function to prevent and manage late postoperative complications. This review describes the management strategies for common late complications in patients who have undergone ASO.

A 42-year-old man sustained a stroke secondary to malignant hypertension and was found to have complete aortic interruption. We report a case of real-time image-guided endovascular repair to highlight the value of preprocedural planning and intraprocedural cone beam computed tomography. Two-dimensional fluoroscopy enhanced with three-dimensional landmarks from cone beam computed tomography was used to direct a Nykanen radiofrequency wire (Baylis Medical, Montreal, Quebec, Canada) through the interruption, avoiding critical adjacent structures. A covered Cheatham-Platinum stent (NuMED, Inc, Hopkinton, NY) was deployed successfully, recanalizing the thoracic aorta. The pressure gradient normalized, and the patient was discharged on postoperative day 1. At 10-month follow-up, the patient was on an antihypertensive regimen of two minimum-dose drugs.

To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs).Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach.This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery).Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction.Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

We report the case of a 27-year-old Jehovah's Witness patient with severe pulmonary insufficiency and right ventricular dilatation 16 years after primary repair, who sought transcatheter therapy as a means to avoid surgery and the risk of blood product administration. A hybrid procedure involving pulmonary artery banding to a diameter amenable to fixation of a Melody valve (Medtronic Inc.) via trans-ventricular puncture was performed.

Background Balloon angioplasty (BA) is effective in relieving neoaorta recoarctation (reCoa) after the Norwood procedure. However, recurrence is not uncommon and risk factors for success and recurrence require further elucidation. We report the results of BA for reCoa following the Norwood procedure. We examine acute results and risk factors associated with success and recurrence after BA. Methods Patients who underwent BA between November 2000 and June 2010 were studied. Factors for immediate success and recurrence after BA were determined using logistic regression. Recurrence-free survival was evaluated using the Kaplan–Meier curve. Results Forty-seven angioplasties were performed in 39 patients. Mean age at catheterization was 5.0 ± 4.1 months. BA was successful in 40 angioplasties (85.1%). A higher preprocedure gradient across the coarctation (P = 0.04) and a higher ratio of balloon to descending aorta (P = 0.01) were associated with success. Six patients required redilation. Risk factors for recurrence included older age at Norwood (P = 0.02), younger age (P = 0.03), lower weight (P = 0.04) and smaller body surface area at balloon angioplasty (BA) (P = 0.04), and shorter duration between surgery and angioplasty (P = 0.03). Freedom from recurrence from the first catheterization was 82% after 6 months and 78% after 1 year. There were no neurologic sequelae or deaths. Conclusions BA is effective acutely and long term with limited morbidity and mortality. Recurrence occurs usually within the first year. Delay in performing the initial angioplasty may be beneficial in reducing the risk of recurrence, but further study is needed. © 2012 Wiley Periodicals, Inc.

Background: Surgical and transcatheter management of muscular ventricular septal defects (MVSD) have independent drawbacks. Hybrid procedures are becoming increasingly utilized to manage congenital cardiac lesions including MVSDs. This report describes the mid-term results of perventricular device closure of MVSDs at a single institution. Methods: The cardiology database of patients who underwent attempted perventricular MVSD closure from a single institution was reviewed. Results: Between January 2004 and December 2009, six patients underwent attempted perventricular MVSD closure in the operating room. Mean age was 9.8 ± 9.1 months; mean weight was 7.2 ± 3.7 kg. In five patients, closure was successful without use of bypass. In one patient, the device embolized to the left ventricle after release and patch closure of the MVSD was performed on cardiopulmonary bypass. The mean interval from the procedure to the most recent echocardiogram for the patients with successful perventricular closure was 39.8 ± 25.2 months. Three patients demonstrated no residual shunt at the last echocardiogram. Two patients had mild, hemodynamically insignificant shunting; one had a left ventricular pseudoaneurysm that was embolized during repeat catheterization. Conclusions: Perventricular closure of MVSDs is attractive because it overcomes the limitations of surgery and catheterization. Additionally, it spares the need for cardiopulmonary bypass and its comorbidities. In some instances, however, successful deployment of the device is not possible. Our mid-term results demonstrate overall success but identify possible complications that are not immediately identified in the short term. © 2011 Wiley-Liss, Inc.

Background: Pulmonary artery (PA) stents are utilized to treat branch pulmonary stenosis (BPS). Often patients with PA stents undergo subsequent cardiac surgery for other indications, and the stents can be manipulated during the procedure. Objective: The purpose of this study was to evaluate the outcome of branch PA stenoses following surgical manipulation of previously implanted PA stents and to determine factors associated with future reintervention. Methods: Catheterization data, operative reports, and clinical summaries were reviewed on patients with PA stents placed between September 1989 and December 2006 undergoing subsequent cardiac surgery. Surgical manipulation was recorded as removed, trimmed, or longitudinally transected, and patched. Those that were not manipulated were defined as untouched. Results: 459 patients had branch PA stents placed. About 54 patients, with 70 stents in branch PA's. subsequently had further cardiac surgery. The median age of stent placement was 7.5 (0.5–32.4) years with a median age of surgery of 12.7 (5.1–39.6) years. Surgical manipulation was performed in 23 (33%) PA's and 47 (66%) stents were untouched. Stent removal occurred in 11 (16%), with transecting longitudinally and patching in 5 (7%), and trimming in 7 (10%). Comparing the surgical manipulation and the untouched groups, there was no difference in median age of stent placement [7.2 (0.5–30.2) versus 7.6 (1.8–32.4) years, p = 0.40], wt [21.0 (5.3–86.5) versus 24.7 (9.0–96.0) kg, p = 0.42], or residual catheterization gradient across the stent [3 (0–59) versus 4 (0–50) mmHg, p = 0.81]. Catheter reintervention (stent n = 6 or balloon dilation n = 14) on the previously stented PAs was similar between the surgically manipulated (median 7.5 years) and untouched groups (median 11.5 years) (p = 0.31). In multivariate analysis, the factors associated with future catheter reintervention were having the stent transected longitudinally and patched (p = 0.003) and a lower weight (p = 0.006) at the time of stent placement. Conclusions: Surgical stent manipulation is often performed in patients who have PA stents. Surgical manipulation does not alter the need for future reintervention and catheter re-intervention may be more likely when the stents are transected longitudinally and patched. © 2010 Wiley-Liss, Inc.

Detection of viral genome in rejecting cardiac transplant patients has been reported, with coxsackievirus and adenovirus causing premature graft failure. Recently, parvovirus B19 (PVB19) genome in myocardial samples has been increasingly reported, but its role in cardiac pathology and effect on transplant graft survival are unknown. The objectives of this study were to determine if changes in the viruses identified in the myocardium represent an epidemiologic shift in viral myocardial disease and whether PVB19 adversely affects transplant graft survival.From September 2002 to December 2005, nested polymerase chain reaction was used to evaluate endomyocardial biopsy specimens for 99 children (aged 3 weeks-18 years) with heart transplants for the presence of viral genome. Cellular rejection was assessed by histology of specimens. Transplant coronary artery disease (TCAD) was diagnosed by coronary angiography or histopathology.Specimens from 700 biopsies were evaluated from 99 patients; 121 specimens had viral genome, with 100 (82.6%) positive for PVB19, 24 for Epstein-Barr virus (EBV; 7 positive for PVB19 and EBV), 3 for CMV, and 1 for adenovirus. Presence of PVB19 genome did not correlate with rejection score, nor did a higher viral copy number. Early development of advanced TCAD (p0.001) occurred in 20 children with persistent PVB19 infection (6 months).PVB19 is currently the predominant virus detected in heart transplant surveillance biopsy specimens, possibly representing an epidemiologic shift. Cellular rejection does not correlate with the presence or quantity of PVB19 genome in the myocardium, but children with chronic PVB19 infection have increased risk for earlier TCAD, supporting the hypothesis that PVB19 negatively affects graft survival.

Objective: To describe incidence, characteristics, predictive factors, and sequelae of stent fractures in congenital heart disease. Background: Stent fracture in congenital heart disease patients is rarely reported. Methods: Patients with stents implanted from 1990 to 2006, with subsequent fluoroscopy were enrolled. Information obtained included: stent type, location, balloon diameter, and residual narrowing. Fracture characteristics, radiographic appearance, and clinical sequelae were also obtained in those with fractures identified. Results: Two hundred and sixty-five patients (583 stents) had fluoroscopy 4.2 ± 3.3 years after stent implantation. The majority of stents (395, 67.7%) were placed in a pulmonary artery (PA) or its branches. Sixteen stents (2.7%) were placed in a right ventricle to pulmonary artery (RV-PA) conduit. Fourteen fractures (2.4%) were identified 5.0 ± 3.2 years after implantation: RV-PA conduit 4/16, proximal right PA 4/135, proximal left PA 6/184 (P = 0.003). Identification was primarily made on lateral fluoroscopy. Review of chest radiographs demonstrated fractures in only 71%. In fractured PA stents, five were redilated and three had additional stents placed. Of the four conduit stent fractures: one underwent redilation, one had embolized segments, and one was reinforced with a second stent that also fractured and embolized a segment during redilation at a later catheterization. All fractures were asymptomatic. Conclusions: With the exception of RV-PA conduit stents, fracture is rare and asymptomatic. PA stent fractures occurred in 2.5% and did not embolize, whereas RV-PA conduit stent fractures were significantly more common and may embolize. © 2008 Wiley-Liss, Inc.

Heparin-induced thrombocytopenia (HIT) is a potentially life-threatening, adverse effect of heparin therapy. Patients with this complication require an alternative approach to anticoagulation. Bivalirudin is a direct thrombin inhibitor with an efficacy comparable to that of heparin, a short half-life, and reduced bleeding complications in adults. We present the case of a 2-year-old boy with HIT Type II who underwent recanalization of an occluded superior vena cava and stent placement, utilizing bivalirudin as anticoagulant.

Severe mitral regurgitation predicts poor outcomes in adults with left ventricular dysfunction. Frequently, adult patients now undergo initial mitral valve surgery instead of heart transplant. Pediatric data are limited. This study evaluates the efficacy of mitral valve surgery for severe mitral regurgitation in children with dilated cardiomyopathy. This is a single-institution experience in seven children (range, 0.5-10.9 years) with severe mitral regurgitation and dilated cardiomyopathy who underwent mitral valve surgery between January 1988 and February 2005, with follow-up to January 2006. Children with dilated cardiomyopathy had a depressed fractional shortening preoperatively (24.4% +/- 6.1%) that remained depressed (22.9% +/- 7.6%) 1.3 +/- 1.2 years after surgery (p = 0.50). Left ventricular end-diastolic (6.5 +/- 1.5 to 4.8 +/- 1.8 z-scores, p0.01) and end-systolic (6.8 +/- 1.5 to 5.5 +/- 2.1 z-scores, p0.05) dimensions improved. Hospitalization frequency had a median decrease of 6.0 hospitalizations per year (p0.02). Three patients were transplanted 0.2, 2.4, and 3.5 years after surgery. There was no perioperative mortality. Mitral valve surgery in children with dilated cardiomyopathy was performed safely and improved symptoms, stabilizing ventricular dysfunction in most patients. Mitral valve surgery should be considered prior to heart transplant in children with dilated cardiomyopathy and severe mitral regurgitation.

Obesity and cachexia are risk factors for adverse outcomes in adult transplant patients. However, little is known about the effects of body mass index (BMI) on outcomes in pediatric heart transplant patients.Patients2 years of age undergoing heart transplantation from 1985 to 2004 at our institution were included in this study. BMI was assessed at the time of transplant and at 1 year post-transplant. Long-term outcomes were assessed by weight group.The cohort included 105 patients with a mean age at transplant of 9.6 +/- 5.3 years. The mean BMI percentile at the time of transplant was 39 +/- 34, with 22 (21%) patients underweight (5th percentile) and 8 (8%) patients overweight (or = 95th percentile). Among patients surviving to 1 year (n = 92), the mean BMI percentile increased to 57 +/- 33 (p0.05). Overall graft survival was decreased in patients underweight at transplant, mean 6.7 years (95% confidence interval [CI] 3.6 to 9.9), vs normal weight patients, mean 10.6 years (95% CI 8.8 to 12.4) (p0.05). Patients overweight at transplant did not have decreased graft survival. Neither low nor high BMI at 1 year post-transplant was associated with adverse outcomes. On multivariate analysis, being underweight at transplant was an independent predictor of decreased graft survival (p = 0.03).Weight gain was nearly universal post-transplant with only 4% of patients underweight at 1 year. In the small number of patients overweight at transplant, graft survival was similar to normal-weight patients. Conversely, being underweight at transplant was an independent predictor of decreased graft survival.

OBJECTIVE Cardiac output is a useful measure of myocardial performance. Standard methods of determining cardiac output are not without risk and can be problematic in children. Arterial pulse wave analysis (PulseCO), a novel, minimally invasive cardiac output determination technique, offers the advantage of continuous monitoring, convenience, and low risk. This technique has not been validated in children. The purpose of this study was to validate PulseCO as an accurate means of noninvasively determining real-time cardiac output in children. DESIGN Prospective, single-center evaluation. SETTING Children's hospital. PATIENTS Any child with a structurally normal heart, undergoing hemodynamic evaluation in the cardiac catheterization laboratory, was included. INTERVENTIONS A prograde right heart catheterization was performed, and cardiac output was determined using the thermodilution technique, via placement of a pulmonary arterial catheter. MEASUREMENTS AND MAIN RESULTS Thermodilution results were compared with continuous real-time cardiac output measurements obtained with the PulseCO system, and they were then analyzed by standard correlation techniques and Bland-Altman analysis. Twenty patients were evaluated with a median age of 10.5 yrs and a median weight of 25 kg. The mean thermodilution cardiac index was 3.3 +/- 0.9 L/min/m, whereas the mean PulseCO cardiac index was 3.1 +/- 0.9 L/min/m. Standard Pearson correlation tests revealed a correlation coefficient of .94 (p < .001). Bland-Altman analysis revealed excellent clinical agreement with a mean difference of 0.19 L/min/m and a precision of 0.28 L/min/m at 2 sd. CONCLUSIONS Arterial pulse wave analysis by the PulseCO system provides a novel, minimally invasive method of determining real-time cardiac output in children.

Background— Some patients with hypertrophic cardiomyopathy (HCM) or left ventricular hypertrophy also present with skeletal myopathy and Wolff-Parkinson-White (WPW) syndrome; mutations in the gene encoding the lysosome-associated protein-2 (LAMP-2) have been identified in these patients, suggesting that some of these patients have Danon disease. In this study we investigated the frequency of LAMP2 mutations in an unselected pediatric HCM population. Methods and Results— LAMP2 was amplified from genomic DNA isolated from peripheral lymphocytes of 50 patients diagnosed with HCM and analyzed by direct DNA sequencing. In 2 of the 50 probands (4%), nonsense mutations were identified. In 1 family the proband initially presented with HCM as a teenager, which progressed to dilated cardiomyopathy (DCM) and heart failure. Skeletal myopathy and WPW were also noted. The teenage sister of the proband is a carrier of the same LAMP2 mutation and has HCM without skeletal myopathy or WPW. The other proband presented with HCM, WPW, and skeletal myopathy as a teenager, whereas his carrier mother developed DCM during her 40s. Skeletal and cardiac muscle sections revealed the absence of LAMP-2 on immunohistochemical staining. Conclusions— LAMP2 mutations may account for a significant proportion of cases of HCM in children, especially when skeletal myopathy and/or WPW is present, suggesting that Danon disease is an underrecognized entity in the pediatric cardiology community.

A 12 year-old girl with corrected transposition (L-transposition of the great arteries with ventricular inversion) and congenital complete atrioventricular block underwent epicardial pacemaker implantation with a single ventricular lead at 1 week of life. Subsequently, she presented for routine follow-up care in the electrophysiology clinic with no symptomatic concerns. She denied chest pain. Her physical examination was unremarkable, with the pacemaker generator palpable in the left subcostal region. ECG at that time demonstrated ventricular pacing with 100% capture and no evidence of atrioventricular conduction. Her chest x-ray revealed that the pacemaker lead completely encircled the cardiac silhouette and prompted concern for cardiac strangulation (Figure 1). A subsequent computed tomography scan showed a likely restrictive effect on both ventricles caused by the pacemaker lead encircling the heart with concern …

Reimbursement for limited echocardiograms focusing on known pathology rather than complete studies has recently received widespread attention. Few data are available to determine if these limited examinations provide enough information to adequately evaluate many forms of congenital heart disease. Stenosis of normally connecting pulmonary veins is a congenital or acquired cardiac anomaly that is difficult to diagnose clinically and may be detectable only by echocardiography. To evaluate the yield of complete versus limited echocardiograms for detecting the presence and development of stenosis in pulmonary veins with anatomically normal connections, the cardiology database was searched for all patients with this diagnosis presenting between June 1990 and January 2000. Charts were reviewed for demographic data, associated defects, surgeries, and outcomes. Angiograms and echocardiograms were reviewed for location and severity of pulmonary vein stenosis. A pulsed-wave Doppler signal of > 1.6 mm/sec with loss of phasic flow was used to define stenosis. Eighteen patients were identified and ranged in age at first evaluation from 1 day to 17 years (median 15 days). All 18 patients had associated cardiac anomalies, and 4 (22%) of 18 had Trisomy 21. Pulmonary vein stenosis was detected on the initial evaluation in 5 patients, detected 8 +/- 5 months after the initial echocardiogram in 11 patients, and missed by echocardiography in 2 patients who were diagnosed by cardiac catheterization. The initial echocardiograms were complete, with pulsed-Doppler sampling of all four pulmonary veins in 17 of 18 patients. Of the 12 patients who had echocardiographic evidence of late stenosis, 10 had 17 limited interim studies prior to eventually having a complete diagnostic follow-up study. Of the two patients in whom the diagnosis was missed by echocardiography, one initial study was technically inadequate (17-year-old) and one had only limited interim studies after the initial echocardiogram. Of the 9 patients in whom repair of the pulmonary vein stenosis was attempted, 3 had no residual obstruction and 6 had progressive stenosis (three deaths). Of the remaining nine patients who had no intervention for their pulmonary venous stenosis, four have died from progressive pulmonary hypertension. Stenosis of normally connecting pulmonary veins is an uncommon lesion that has a significant impact on clinical outcome. The stenoses might be undetectable on limited echocardiograms that focus on evaluating only specified pathology. Complete follow-up examinations might be warranted to diagnose this lesion. This may be an important consideration when formulating reimbursement policies.

Objectives: Very little is known regarding the immune response to cryopreserved allograft valves and patch material used in the surgical repair of congenital heart defects. Methods: We prospectively measured the frequency of panel reactive antibodies directed against HLA class I (HLA-A, B, and C) and class II (HLA-DR/DQ) alloantigens in 24 children receiving cryopreserved allografts. We compared them with results in 11 previously reported control patients. Sixteen of the study patients underwent placement of a valved conduit (11 pulmonic, 5 aortic) between the right ventricle and pulmonary arteries, 6 underwent patch angioplasty of stenotic vessels with cryopreserved pulmonary artery, and 2 underwent placement of a pulmonary monocusp patch. Study patients had panel reactive antibodies measured before, 1 month, 3 months, and 1 year after the operation. Results: With allograft implantation, panel reactive antibodies increased from 1.9% ± 5% before the operation to 62% ± 33% at 31 ± 8 days after the operation, 92% ± 15% at 3.3 ± 0.6 months after the operation, and 85% ± 18% at 1.1 ± 0.2 years after the operation. The control group showed no change in panel reactive antibodies, with a level of 1.6% ± 1% before the operation, 3.2% ± 1% 28 ± 5 days after the operation, and 1.7% ± 1% 2.7 ± 0.3 months after the operation. Class II antibodies (anti-HLA-DR/DQ) rose to 49% ± 35% at 30 ± 8 days and 70% ± 26% at 3.3 ± 0.6 months after the operation. Conclusions: Cryopreserved allograft material induces a marked response that involves both class I and class II anti-HLA antibodies within 3 months after operation in children. This alloantibody response may represent a form of “rejection,” may have implications for those who require subsequent cardiac transplantation, and may play a role in early allograft failure. (J Thorac Cardiovasc Surg 2000;119:324-30)

Background . Pulmonary vein (PV) stenosis with anatomically normal connection is considered rare, unresponsive to treatment, progressive, and usually fatal. Methods . We reviewed the records of 13 children with this diagnosis at our center since 1990. Results . The number of stenosed PVs ranged from all PVs (n = 5); three PVs (n = 1); two PVs (n = 5); and one PV (n = 2). All patients had associated congenital cardiac abnormalities. Operation on PV stenosis was attempted in 7 patients (54%), 2 of whom have done well and 5 of whom have not. Two patients underwent heart transplantation for inoperable associated cardiac lesions. Significantly more patients with three or four stenosed PVs died (83%) compared with patients with one or two stenosed PVs (0%). Conclusions . (1) Pulmonary vein stenosis with anatomically normal connection is associated with other congenital cardiac abnormalities, (2) presentation and outcome are contingent on the number of stenosed PVs, (3) surgical palliation may be helpful in some patients, and (4) heart transplantation for inoperable associated cardiac abnormalities may be an option in patients with only one or two stenosed PVs.

This study reports the mid-term survival for neonates undergoing extracorporeal membrane oxygenation (ECMO) after Norwood palliation at a single center. Limited data exist on the mid-term survival of patients undergoing ECMO after Norwood palliation. We reviewed our ECMO experience from July 1994 to October 2008 and compared two groups: patients who required ECMO after Norwood palliation and patients who underwent Norwood palliation without ECMO. We analyzed 30-day survival, survival to hospital discharge, and survival to most recent follow-up. One hundred sixty patients underwent Norwood palliation for hypoplastic left heart syndrome (HLHS) and its variants. A total of 32 patients (20 %) required postoperative ECMO. Using Kaplan–Meier analysis, the predicted survival rates for Norwood/non-ECMO patients to 30 days, 1 year, and 3 years after the procedure are 87.6 % (CI 79.5–91.5 %), 62.5 % (CI 54.3–71.0 %), and 59.9 % (CI 50.8–67.8 %), respectively. Survival to 30 days, 1 year, and 3 years after Norwood was significantly decreased in Norwood/ ECMO patients, with predicted survival rates of 50.0 % (CI 31.9–65.7 %), 24.6 % (CI 11.4–40.4), and 13.2 % (CI 3.9–28.3 %), respectively (p

A 15-year-old girl presented with persistent fevers, night sweats, leukocytosis, an elevated erythrocyte sedimentation rate, and a 13-pound weight loss over 2 months. Duplex Doppler scans, computed tomographic scan, and magnetic resonance imaging studies were suggestive of Takayasu's arteritis. Left ventricular dysfunction occurred during the episode of active disease, and an endomyocardial biopsy demonstrated increased HLA-DR (human leukocyte antigen-DR) on the endothelium and evidence of immune complex deposition in the walls of small vessels. One year later, after treatment with corticosteroids and resolution of clinical symptoms, repeat endomyocardial biopsy revealed focal interstitial fibrosis and persistent immune complex deposition. These results indicate that the inflammatory, vasculitic process affecting the large vessels in Takayasu's arteritis may also involve the endomyocardium and its small vessels resulting in ventricular dysfunction.

Objectives: Determine the long-term outcomes of branch pulmonary artery (PA) stents. Background: PA stents in congenital heart disease effectively relieve stenoses in the short-term. Published long-term data are limited. Methods: Patients enrolled in an FDA IDE protocol from 1989–92 were included. Clinical follow-up and catheterization data were evaluated. Patients were included if >5 year follow-up data was available or if mortality occurred following the initial procedure. Results: There were five deaths: four due to progression of their underlying heart disease, and one from a complication during a follow-up catheterization. Clinical data for 43 surviving patients demonstrated 39 patients (91%) are in NYHA class I or II. Seven patients underwent surgical intervention during the follow-up period (five RV-PA conduit, two Fontan revisions), but none addressed PA stenosis. Final repeat catheterizations were performed in 36 patients (55 stents) 7.2 ± 4.3 years post stent insertion with 1.2 ± 0.9 further procedures with stent dilations. In this subgroup, the minimum vessel diameter increased from 4.7 ± 1.8 to 13.4 ± 2.4 mm (P < 0.001), and the pressure gradient improved from 41 ± 25 to 9 ± 11 mm Hg (P < 0.001). Higher initial gradient and smaller balloons were associated with a final stent diameter of

Perventricular device closure has become an important management adjunct for ventricular septal defects deemed inoperable and difficult to address in the catheterization laboratory. Complications directly related to the procedure are rarely reported, and all have been manifest in the periprocedural period. We present the case of a 6-week-old boy who underwent perventricular closure of a muscular ventricular septal defect and had a left ventricle pseudoaneurysm identified 10 months later during catheterization to close residual septal defects. We describe the subsequent percutaneous coil embolization of the pseudoaneurysm and clinical follow-up.

ObjectivesThis study sought to evaluate the outcome and prevalence of viral endomyocardial infection after cardiac transplantation.BackgroundViral myocardial infection causes heart failure, but its role after cardiac transplantation is unclear. We hypothesized that viral infection of the cardiac allograft reduces graft survival.MethodsBetween June 1999 and November 2004, 94 pediatric cardiac transplant patients were screened for the presence of viral genome in serial endomyocardial biopsies (EMBs) using polymerase chain reaction (PCR) assays. Graft loss, advanced transplant coronary artery disease (TCAD), and acute rejection (AR) were compared in the PCR-positive (n = 37) and PCR-negative (n = 57) groups, using time-dependent Kaplan-Meier and Cox regression analyses. From November 2002 to November 2004, intravenous immunoglobulin therapy (IVIG) was administered to patients with PCR-positive EMBs. The outcomes of the IVIG-treated, PCR-positive patients (n = 20) were compared with IVIG-untreated, PCR-positive patients (n = 17).ResultsViral genomes were detected in EMBs from 37 (39%) patients; parvovirus B19, adenovirus, and Epstein-Barr virus (EBV) were the most common. The PCR-positive group (n = 37, 25% graft loss at 2.4 years) had decreased graft survival (p < 0.001) compared with the PCR-negative group (n = 57, 25% graft loss at 8.7 years) and developed advanced TCAD prematurely (p = 0.001). The number of AR episodes was similar in both groups. On multivariate analysis, presence of viral genome was an independent risk factor for graft loss (relative risk: 4.2, p = 0.015). The time to advanced TCAD after becoming PCR-positive was longer in the IVIG-treated patients (p = 0.03) with a trend toward improved graft survival (p = 0.06).ConclusionsViral endomyocardial infection is an independent predictor of graft loss in pediatric cardiac transplant recipients. This effect appears to be mediated through premature development of advanced TCAD. IVIG therapy in this subgroup may improve survival and merits further investigation.

Background Accumulating evidence suggests that immune-mediated injury is important in the development of rejection after heart transplantation. We hypothesized that pro-inflammatory cytokine expression would increase in biopsy samples that manifest cellular rejection and that this would correlate with the development and progression of transplant cellular rejection. Methods Children with heart transplants were prospectively enrolled from July 2004 to November 2005. Right ventricular endomyocardial biopsies were obtained during routine catheterization for rejection surveillance. Cellular rejection was graded using criteria established by the International Society for Heart and Lung Transplantation. RNA was extracted from biopsy samples and reverse transcription was used for complementary DNA synthesis. The cDNA product was evaluated by quantitative real-time polymerase chain reaction (PCR) to measure the following cytokines: interleukin (IL)-1β, IL-6 and IL-18; tumor necrosis factor-alpha (TNF-α); and interferon-gamma (IFN-γ). Normalized cytokine mRNA transcripts were correlated with cellular rejection scores and the presence of viral genome. Results Seventy-four children (mean age 9.6 ± 5.5 years, range 0.2 to 20.5 years) were enrolled and 95 biopsies were obtained. None of the cytokines demonstrated a correlation with the cellular rejection score, even within individual patients for whom multiple, serial biopsy samples were studied. Eighteen biopsy samples were found to have parvovirus B19 genome present, but there was no correlation between cytokine levels and the presence of parvovirus. Conclusions Cytokine transcripts in heart transplants do not correlate with cellular rejection. In addition, there is no correlation between cytokine transcripts and the presence of viral genome.

Objective Interrupted aortic arch is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Multiple surgical modalities exist to address this defect. We evaluate the long-term outcome of interrupted aortic arch with the left carotid artery turndown technique from a single institution. Methods Patients with interrupted aortic arch who underwent the carotid turndown procedure were identified between September 1982 and March 2010. Medical and surgical records were reviewed. Mortality data were obtained from state death records. Results Forty-seven patients met inclusion criteria. Median follow-up was 6.2 years (5 days to 23.2 years). Twenty-one patients (45%) had genetic syndromes. There were 4 operative deaths (8.5%) and 9 late deaths (19%). One-year and 5-year survivals were 80.2% and 72.6%, respectively. Seventeen patients (36.2%) required reoperation or other interventions on the aortic arch. Conclusions Left carotid artery turndown offers a favorable surgical outcome. It compares with end-to-end repair, while providing a tension-free anastomosis and avoiding neonatal circulatory arrest and cardiopulmonary bypass. Disadvantages include a 2-stage repair and a significant reintervention rate, particularly when compared with the aortic arch advancement technique. Nevertheless, the reduced exposure to circulatory arrest and bypass and avoidance of left bronchial obstruction are important considerations that may offset these limitations.