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Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Bioprosthetic aortic valve replacement (bAVR) in patients with congenital heart disease is challenging due to age, size and complexity. Our objective was to assess survival and identify predictors of re-operation. Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014. Kaplan-Meier estimation of time to re-operation and Cox regression were utilized. Average age was 45.2 years (IQR 17.8-71.1) and 30% were

The use of ventricular assist device (VAD) in children has increased, but the decision of left VAD (LVAD) vs biventricular support remains a challenge. Children who undergo LVAD placement are at risk for right ventricular failure (RHF), but the incidence has not been described.Analysis was performed for patients18 years old who underwent durable LVAD placement within the Pedimacs registry (September 19, 2012-February 28, 2017), excluding single ventricle morphology and temporary devices. RHF was defined as the need for right ventriculalr assist device (RVAD) or prolonged inotrope use between 1 week to 1 month and 1 to 3 months. End-points included death, heart transplant (HT), and recovery.A total of 272 durable LVAD were placed of which 37 died on device over 24 month follow-up, primarily from multiorgan failure and neurologic dysfunction. RVAD occurred in 12 children at median 8.5 days, with 9 undergoing HT and 3 dying on device. In patients with only LVAD, RHF was present in 111/207 (55%) between 1 week to 1 month and 28/116 (25%) between 1 and 3 months. Younger age, smaller weight, Intermacs profile 1, chemical paralysis, and pulsatile flow VAD were associated with RHF. RHF was associated with increased risk of death on device at both1 month (hazard ratio 3.2, 95% CI 1.4-7.7, p = 0.007) and3 month (hazard ratio 6.9, 95% CI 2-23.1, p = 0.002).In children, RHF is common after durable LVAD implantation, but subsequent RVAD is relatively rare. RHF in children, as indicated by prolonged inotrope support, was associated with an increased risk of death on the device. Whether early RVAD support and higher waitlist status may improve the outcome remains unknown.

Data are limited on outcomes associated with mitral valve surgery in infants. Prior studies report high mortality and increased risk for late cardiac failure particularly for those with mitral stenosis. We sought to evaluate outcomes in patients with mitral stenosis (MS) or regurgitation (MR) who had mitral valvuloplasty or replacement in the first year of life. A retrospective analysis of all patients in a single institution who underwent mitral valvuloplasty or replacement in their first year of life from 2004 to 2016 (n = 25), excluding patients with single ventricle pathology or those undergoing surgery for atrioventricular canal defect, was carried out. Median age and weight at surgery were 76.5 days (range 2-329) and 4.5 kg (range 3.0-10.1), respectively. The primary mitral pathology was MR in 16 and MS in 9 patients. Median follow-up among living patients was 4 years (range 106 days-12.3 years). Overall survival was 96% at 30 days and 87.8% at 1, 5, and 10 years. There were three early deaths (12%), all within 6 weeks of surgery. There were no late deaths. Three patients required valve replacement, 1 of which had a primary mitral valve replacement and died within 30 days of surgery. Re-intervention-free survival (surgical and catheter based) was 83.8%, 73.3%, and 48.9% at 1, 5, and 10 years per Kaplan-Meier estimates. There was no difference in re-intervention-free survival between patients with MR versus MS. No risk factors for death or re-intervention were identified. Mitral valvuloplasty and replacement can be performed in infants under 1 year of age with acceptable survival and need for re-intervention.

Recently, transcatheter closure of sinus venosus defects (SVDs) with covered stents has been reported. Because of wide anatomic variability of SVDs, it is unclear which patients are candidates for transcatheter closure. We used cardiac magnetic resonance and computed tomographic imaging to

We describe a seven-year-old female with acute pericarditis presenting with pericardial tamponade, who screened positive for coronavirus disease 2019 (COVID-19 [SARS-CoV-2]) in the setting of cough, chest pain, and orthopnea. She required emergent pericardiocentesis. Due to continued chest pain and orthopnea, rising inflammatory markers, and worsening pericardial inflammation, she underwent surgical pericardial decortication and pericardiectomy. Her symptoms and pericardial effusion resolved, and she was discharged to home 3 days later on ibuprofen and colchicine with instruction to quarantine at home for 14 days from the date of her positive testing for COVID-19.

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees’ experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.

The world as we once knew it has been drastically altered secondary to coronavirus disease 2019 (COVID-19). The impact of these changes, particularly for those practicing in the medical profession, extends beyond the physical to the psychological, emotional, and spiritual. We discuss the factors that contribute to these stresses, way to manage them, and how we as leaders of our teams can inspire resilience and help our colleagues endure these most difficult times.

Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database.Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model.Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P .0001), major morbidity (adjusted odds ratio, 3.7; P .0001), and tracheostomy (adjusted odds ratio, 16.7; P .0001).Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.

The World Society for Pediatric and Congenital Heart Surgery has endorsed the establishment of an international platform for the exchange of knowledge and experience for those that treat patients with a congenital heart defect. On January 1, 2017, the release of the World Database for Pediatric and Congenital Heart Surgery opened a new era in evaluation of treatment with congenital heart defects. The contribution of data from countries with established congenital surgical databases will greatly enhance the efforts to provide the most accurate measure of overall surgical outcomes across the globe.

Background The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival. Methods The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P. Results A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months. Conclusions Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival.

Introduction: A association between partial anomalous pulmonary venous connection (PAPVC) and systemic venous anomalies has been described in the literature, but the true incidence is yet to be elucidated. At our institution, we sought to find the incidence of a persistent left superior vena cava (PLSVC) in patients undergoing an operation for PAPVC. Methods: A retrospective review of all pediatric and adult patients with PAPVC who underwent surgical repair from February 2006 to February 2016. All clinical, radiographic, and operative data were reviewed. Results: Eighty-five patients underwent surgical repair. A PLSVC was identified in 15 (17.6%) patients. Every PLSVC drained/connected to the coronary sinus. A bridging vein was present in only 26.7% (4 of 15). Intraoperative management of the PLSVC consisted of direct cannulation in nine (60%) patients, temporary occlusion in one (6.7%) patient, and ligation in one (6.7%) patient. In the 15 PLSVC patients, 6 (40%) had a secundum atrial septal defect (ASD), 2 (13.3%) had a patent foramen ovale (PFO), 10 (66.6%) had a sinus venosus type defect, and 3 (20%) had both an ASD and sinus venosus–type defect. Of the 15 patients with PAPVC and PLSVC, 14 (93%) had anomalous drainage of pulmonary vein(s) on the right side, whereas 1 (7%) had veins on both sides with anomalous drainage. Conclusion: Persistent left superior vena cava is present in 17.6% of patients undergoing an operation for PAPVC. Awareness of this association as well as the intraoperative management of PLSVC is advised for those that perform operations for PAPVC. As improper myocardial protection and cardiopulmonary bypass strategies in the presence of a PLSVC can result in deleterious outcomes.

Coronary ostial atresia seen with pulmonary atresia and coronary-cameral fistulae or, more rarely, in isolation manifested as left main coronary artery atresia, is well described. We describe the clinical course and post-mortem findings in a neonate who suffered a fatal cardiac arrest and was found to have congenital absence of both coronary ostia in a single/common coronary system.

This report describes a unique case involving an obese 16-year-old boy with a mosaic form of trisomy 13 and no previous cardiac history who presented with a new murmur, hypertension, pleural effusions, and congestive heart failure in the context of sore throat and fever. Evaluation revealed a diagnosis of ruptured noncoronary sinus of Valsalva (SOV) aneurysm. The diagnosis and surgical management of a ruptured noncoronary SOV aneurysm in a pediatric patient are briefly outlined. An SOV aneurysm is an anatomic dilation of one of the sinuses of the aortic root. Aneurysmal dilation occurs more commonly in the right aortic sinus (70%-80%), compared to the noncoronary sinus (23%-25%), and more rarely the left coronary sinus (5%). Rupture of these aneurysms has been reported to be both spontaneous and secondary to physical exertion, hypertension, or trauma. Signs of rupture include a continuous murmur, patients may present with chest pain or with symptoms of acute congestive heart failure. Diagnosis, in this case, was made by transthoracic echocardiography with careful interpretation of color Doppler images.

Objective This study's objective was to evaluate insertion techniques and device fit of the pediatric version of the Impella ventricular assist device in swine which had similar sized carotids and left ventricles (LVs) as children weighing 10–20 kg. Background Options for minimally invasive circulatory support in children are limited. A modified device based on the current Impella 2.5 platform was created in an effort to provide minimally invasive circulatory support for children. Methods Animal studies (n = 10) were performed to determine technical feasibility of device implant via the right common carotid artery (RCCA) in swine with a carotid and LV size similar to children with a BSA

Continuous-flow (CF) ventricular assist devices (VADs) have largely replaced pulsatile-flow VADs in adult patients. However, there are few data on CF VADs among pediatric patients. In this study we aimed to describe the overall use, patients' characteristics and outcomes of CF VADs in this population.The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a national registry for U.S. Food and Drug Adminstration (FDA)-approved VADs in patients19 years of age. Patients undergoing placement of durable CF VADs between September 2012 and June 2015 were included and outcomes were compared with those of adults from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS).CF VADs were implanted in 109 patients at 35 hospitals. The median age at implantation was 15 years (2.8 to 18.9 years) and median weight was 62 kg (range 16 to 141 kg). The underlying disease was cardiomyopathy in 89 (82%) patients. The INTERMACS level at time of implant was Level 1 in 20 (19%), Level 2 in 64 (61%) and Levels 3 to 7 in 21 (20%) patients. Most were implanted as LVADs (n = 102, 94%). Median duration of support was 2.3 months (range1 day to 28 months). Serious adverse event rates were low, including neurologic dysfunction (early event rate 4.1 per 100 patient-months with 2 late events). Competing outcomes analysis at 6 months post-implant indicated 61% transplanted, 31% alive with device in place and 8% death before transplant. These outcomes compared favorably with the 3,894 adults supported with CF VADs as a bridge to transplant.CF VADs are commonly utilized in older children and adolescents, with excellent survival rates. Further study is needed to understand impact of patient and device characteristics on outcomes in pediatric patients.

Background: The single ventricle reconstruction trial showed better one-year transplant-free survival for the right ventricle-to-pulmonary artery (RV-to-PA) conduit over the modified Blalock-Taussig shunt (mBTS) at Norwood operation. However, concerns remain about the long-term effects of a neonatal ventriculotomy. In our institution, we have used specific selection criteria for the use of mBTS in the Norwood operation. Methods: We reviewed 122 consecutive neonates undergoing the Norwood procedure from December 2006 to December 2013. We used the following criteria to select our source of pulmonary blood flow: (1) presence of a dominant morphologic left ventricle; (2) presence of antegrade blood in an ascending aorta that is greater than 3 mm; and (3) presence of significant large “crossing coronaries” on ventricle. All patients who met any of the above 3 criteria underwent an mBTS while the remaining patients underwent an RV-to-PA conduit. Results: Seventy-five (61.5%) patients had the RV-to-PA conduit and 47 (38.5%) patients had an mBTS. The overall surgical mortality was 9%. Mean follow-up interval was 23.5 months. Actuarial transplant-free survival was similar at 12, 24, 36, and 48 months in both the mBTS group and the RV-to-PA conduit group. In the RV-to-PA conduit group, actuarial transplant-free survival was 73% at 12 months, 71% at 24 months, 71% at 36 months, and 67% at 48 months, while in the mBTS group, actuarial transplant-free survival was 82% at 12 months, 75% at 24 months, 75% at 36 months, and 75% at 48 months. Conclusion: Our selection criteria for mBTS have allowed us to obtain equivalent transplant-free survival at 12, 24, 36, and 48 months when compared to the RV-to-PA conduit group.

To assess the difference in time to and predictors of reintervention according to valve type in surgical bioprosthetic pulmonary valve replacement (PVR) in patients younger than 30 years of age from multiple centers.Data were retrospectively collected for 1278 patients30 years of age undergoing PVR at 8 centers between 1996 and 2015.Mean age at PVR was 19.3 ± 12.8 years, with 719 (56.3%) patients ≤18 years of age. Diagnosis was tetralogy of Fallot in 626 patients (50.5%) and 165 (12.9%) had previous PVR. Median follow-up was 3.9 years (interquartile range, 1.2, 6.4). Multiple valve types were used, most commonly CE PERIMOUNT, 488 (38.2%), CE Magna/Magna Ease, 361 (28.2%), and Sorin Mitroflow 322 (25.2). Reintervention occurred in 12.7% and was most commonly due to pulmonary stenosis (68.8%), with most reinterventions occurring in children (85.2%) and with smaller valve sizes (P .001) Among adults aged 18 to 30 years, younger age was not a significant risk factor for reintervention. Surgical indication of isolated pulmonary regurgitation was associated with a lower risk of reintervention (P .001). Overall, 1-, 3-, 5-, and 10-year freedom from reintervention rates were 99%, 97%, 92%, and 65%. The only independent risk factors for reintervention after controlling for age and valve size were lack of a concomitant tricuspid valve procedure (P = .02) and valve type (P .001); Sorin and St Jude valves were associated with similar time to reintervention, and deteriorated more rapidly than other valve types.In this large multicenter study, 8% of patients have undergone reintervention by 5 years. Importantly, independent of age and valve size, reintervention rates vary by valve type.

BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center. RESULTS: Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5–171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06–4.93 years). CONCLUSIONS: IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

Objective Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality. Methods From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed. Results Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg). Conclusions Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes.

The HeartWare HVAD has been used as a bridge to cardiac transplantation in the pediatric population. We describe outcomes following HeartWare HVAD implantation at a single center. A retrospective chart review was performed of all HeartWare HVAD implants performed at our institution between May 2013 and March 2015. Eight children between the ages of 9 and 17 years underwent HVAD implantation as a bridge to transplant (N = 7 cardiomyopathy, N = 1 complex single ventricle). There was one operative death in the complex single ventricle patient. Seven patients (87%) were successfully bridged to transplant. Median time of support was 24.5 days (range, 6-91 days). All transplanted patients are alive and well at a median follow-up of 448 days. Our results demonstrated that mechanical support with HeartWare HVAD is feasible in patients of varying sizes (from older children to adolescents).

Cardiac rhabdomyomas in patients with tuberous sclerosis complex are usually detected antenatally or during infancy, with subsequent stabilisation or spontaneous regression. Development of a new cardiac rhabdomyoma beyond infancy is very rare. We report a male child who needed resection of a large rhabdomyoma in neonatal life, and then developed a new-onset rhabdomyoma at 2 years of age in a different location, needing another resection. Routine surveillance for cardiac rhabdomyomas in asymptomatic patients with tuberous sclerosis is essential.

Incidence of thrombosis after initial stage 1 single-ventricle palliation is high. Most centers use aspirin as an antiplatelet agent to prevent thrombosis in surgically placed shunts. We hypothesize there is a significant incidence of aspirin resistance in infants after stage 1 palliation and this resistance can be overcome by an increased aspirin dose.This is a prospective observational study of 20 patients with single-ventricle physiology who required single-ventricle palliation with a controlled source of pulmonary blood flow (Norwood/Sano, Norwood/Blalock-Taussig [BT] shunt or BT shunt alone). Aspirin resistance was determined using thromboelastography with platelet mapping (TEG) and urine thromboxane (UTX). The UTX level of less than 1,500 pg/mL and TEG value of more than 50% were used to define as adequate platelet inhibition. The UTX was measured prior to starting aspirin (20 mg/day) and TEG and UTX were obtained after 5 days of aspirin therapy A repeat UTX was measured for patients who were determined to be aspirin resistant by TEG (50% arachidonic acid inhibition) after doubling the dose (40 mg/day). Clinical variables including patient diagnosis, age of surgery, and cardiopulmonary bypass requirement, weight, hemoglobin, and platelet count were assessed to determine their association with aspirin resistance.Eighty percent of patients were aspirin resistant using TEG (95% CI, 56% to 94%) and none of the patients achieved a UTX level of less than 1,500 pg/mL. Aspirin resistant patients did not respond to an increased dose of aspirin between the fifth and tenth days of therapy (p = 0.820). Patients did, however, respond to aspirin treatment when comparing the baseline UTX measurement with those recorded on the fifth day (p = 0.008) and the tenth day (p = 0.0361) of aspirin therapy. The UTX levels did not differ between those who were and those who were not aspirin resistant by TEG at any of the measurement times. The clinical variables were not associated with aspirin resistance status.There is a high incidence of aspirin resistance in the immediate postoperative period after single-ventricle shunt palliation. Aspirin might not be an adequate agent for shunt prophylaxis in this patient population. Further studies are needed to identify at-risk patients who might benefit from additional testing and specific anticoagulation.

Explantation of LVAD followed by heart transplantation is probably one of the most difficult procedures in modern day cardiac surgery. This is especially true in the pediatric population. Dense adhesions, obliterated planes of dissection, and the proximity of the heart to the sternum all increase the difficulty and risks associated with the recipient heart and LVAD explantation. We present a simple technique using Gore-Tex to help facilitate sternal re-entry and aid in the safe and efficacious explant of LVADs.

Objective Acute kidney injury (AKI) is a common complication resulting from cardiopulmonary bypass in infants. Urinary neutrophil gelatinase-associated lipocalin (NGAL) is a sensitive and specific marker of such injury. In this study, we compared the performance of serum cystatin C (Cys C) and serum creatinine (Cr) as early markers of renal dysfunction in infants undergoing cardiac surgery under bypass. Study Design, Setting, and Patients The study was designed as a prospective observational study. The study was conducted in the cardiac intensive care unit (ICU) of a tertiary, academic children's hospital in the United States. Infants (age

Left atrial (LA) decompression in infants with thick atrial septum placed on veno-arterial extracorporeal membrane oxygenation (ECMO) can be reliably achieved with transcatheter atrial septal (AS) stent placement. However, with myocardial recovery and ECMO decannulation the stent causes a permanent left to right shunt and surgical removal of the AS stent is usually accomplished. Transcatheter removal of AS stent is an attractive option as the advantages of LA decompression would then be coupled with the advantage of avoiding a sternotomy for surgical removal of the stent. To our knowledge, this is the first reported case of successful transcatheter retrieval of AS stent used for LA decompression in a 13-month-old child.

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many “short list” versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various “short lists”. In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the “short list” for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.

The use of a right ventricle-to-pulmonary artery conduit has re-emerged as a popular alternative to a systemic artery-to-pulmonary artery shunt in the Norwood procedure. Both proximal obstruction secondary to dynamic compression and distal obstruction at the anastomosis site with the pulmonary arteries are well described. In an effort to prevent complications, a technique where in the conduit is placed through the entire full thickness, or dunked, through the RV free wall has been described. We report a case of a patient with HLHS and positive lupus anticoagulant who developed recurrent conduit obstructions. The “Sano” was abandoned in favor of an autologous source of pulmonary blood flow—the modified Blalock-Taussig shunt using the “turn down” of the carotid artery.

The Senning and Mustard baffles remain important techniques for the treatment of congenitally corrected transposition (cc-TGA), isolated ventricular inversion, and D-transposition of the great arteries with delayed presentation. We describe the treatment of an 8-month old infant with atrioventricular discordance, ventriculoarterial concordance, and dextrocardia with atrial situs inversus. A modified Senning procedure was performed through the "left-sided" atrium. Modifications of the Senning and Mustard baffles remain important tools in the treatment of rare conditions like isolated ventricular inversion.

Introduction The frequency and successful use of pediatric ventricular assist devices (VADs) as a bridge to cardiac transplantation have been steadily increasing since 2003, but the experience in patients with complex congenital heart disease has not been well described. Using a large prospectively collected dataset of children supported with the Berlin Heart EXCOR VAD, we have reviewed the experience in children with single ventricular anatomy or physiology (SV), and compared the results with those supported with biventricular circulation (BV) over the same time period. Methods The EXCOR Investigational Device Exemption study database was retrospectively reviewed. VAD implants under the primary cohort and compassionate use cohort between May 2007 and December 2011 were included in this review. Results Twenty-six of 281 patients supported with a VAD were SV. The most common diagnosis was hypoplastic left heart syndrome (15 of 26). Nine patients were supported after neonatal palliative surgery (Blalock-Taussig shunt or Sano), 12 after a superior cavopulmonary connection (SCPC), and 5 after total cavopulmonary connection (TCPC). Two patients received biventricular assist devices, 1 after stage I surgery and 1 after stage II. SV patients were supported for a median time of 10.5 days (range, 1-363 days) versus 39 days (range, 0-435 days) for BV (P = .01). The ability to be bridged to transplant or recovery in SV patients is lower than for BV patients (11 of 26 [42.3%] vs 185 of 255 [72.5%]; P = .001). Three of 5 patients with TCPC were successfully bridged to transplant and were supported with 1 VAD. Seven of 12 patients with SCPC were bridged to transplant, and only 1 of 9 patients supported after a stage I procedure survived. Conclusions The EXCOR Pediatric VAD can provide a bridge to transplant for children with SV anatomy or physiology, albeit less successfully than in children with BV. In this small series, results are better in patients with SCPC and TCPC. VAD support for patients with shunted sources of pulmonary blood flow should be applied with caution.

Objectives Bilateral pulmonary artery banding with or without ductal stenting has been performed as a resuscitative intervention for patients considered at too high risk for conventional single ventricle palliation. The purpose of the present study was to determine the outcomes using this strategy. Methods We performed a retrospective review of 24 patients with single ventricle anatomy who were younger than 3 months who had undergone bilateral pulmonary artery banding and ductal stenting or maintenance of prostaglandin E 1 from January 2007 to October 2011 at our institution. The echocardiographic, angiographic, operative, and clinical data were reviewed. Follow-up data were available for 100% of the patients. Results All 24 patients (13 male patients) underwent bilateral pulmonary artery banding at a median age of 8 days (range, 2-44 days). Their gestational age was 38 weeks (range, 27-41 weeks), and their weight was 3.01 kg (range, 1.5-4.4 kg). The cardiac diagnoses included hypoplastic left heart syndrome/variant hypoplastic left heart syndrome in 18, unbalanced atrioventricular canal in 4, and tricuspid atresia in 2. In the hypoplastic left heart syndrome group, 9 (50%) had an intact or a highly restrictive atrial septum requiring open (n = 1) or transcatheter (n = 8) atrial septostomy with or without atrial stent placement (n = 4). Ductal stenting was performed in 14 patients, and 10 patients were continued with prostaglandin E 1 . Fifteen patients (62.5%) survived to undergo a Norwood procedure (n = 7), comprehensive stage 2 (n = 1), or primary cardiac transplantation (n = 7). Of the 9 who died, support was withdrawn in 5 because of a contraindication to transplantation, 1 because of sepsis and/or multiorgan system failure, and 1 for whom palliative care was desired. Two died awaiting transplantation. All 7 patients who underwent a conventional Norwood operation survived to discharge, and 6 of the 7 (85.7%) underwent bidirectional Glenn shunt placement. Of the 7 patients who underwent transplantation, 6 (85.7%) were alive at a median follow-up of 33.6 months. Conclusions Bilateral pulmonary artery banding with or without ductal stenting is an effective method of resuscitation for high-risk neonates and infants with a single ventricle, allowing for reasonable survival to conventional first-stage palliation or primary transplantation.

Despite substantial improvements in survival after pediatric heart transplantation, refractory rejection remains a major cause of morbidity and mortality. We have utilized ALE (Campath-1H) in six consecutive patients with refractory rejection. These rejection episodes persisted despite conventional treatment, which included intravenous methylprednisolone, rituximab, immunoglobulin G, and antithymocyte globulin. In our series, after ALE therapy, LV SF increased from 22%±5% to 33%±5% (P=.01). However, in our series, ALE therapy neither led to persistent LV function recovery nor could it prevent subsequent antibody-mediated rejection.

The thymus, an organ responsible for T cell development, is one of the more stress-sensitive tissues in the body. Stress, in the form of infections, radiation exposure, and steroids, impairs thymic epithelial cell (TEC) functions and induces the programmed cell death of immature thymocytes. MicroRNAs are small noncoding RNAs involved in tissue repair and homeostasis, with several supporting T cell development. We report that miR-205, an epithelial-specific miR, maintains thymopoiesis following inflammatory perturbations. Thus, the activation of diverse pattern recognition receptors in mice causes a more severe thymic hypoplasia and delayed T cell recovery when miR-205 is conditionally ablated in TECs. Gene expression comparisons in the TECs with/without miR-205 revealed a significant differential regulation of chemokine/chemokine receptor pathways, antigen processing components, and changes in the Wnt signaling system. This was partly a consequence of reduced expression of the transcriptional regulator of epithelial cell function, Forkhead Box N1 (Foxn1), and its two regulated targets, stem cell factor and ccl25, following stress. miR-205 mimics supplemented into miR-205-deficient fetal thymic organ cultures restored Foxn1 expression along with ccl25 and stem cell factor. A number of putative targets of miR-205 were up-regulated in TECs lacking miR-205, consistent with an important role for this miR in supporting T cell development in response to stress.

Children with heart failure unresponsive to medical therapy are left with few options for survival. Ventricular assist devices (VADs) are life-saving options for such patients, allowing for bridge to transplantation or cardiac recovery. Retrospective review of cases from May 2006 to October 2010 was undertaken. Fourteen patients underwent implantation of VADs for refractory heart failure. Mean age was 9 years (range 1-17 years), and weight was 41 kg (range 9.7-71 kg). Indications for support: end-stage cardiomyopathy (n = 8), myocarditis (n = 3), univentricular failure (n = 2), and congenital heart disease/postcardiotomy (n = 1). Level of limitation at time of implant included critical cardiogenic shock in six (43%) and progressive decline in eight (57%). Extracorporeal membrane oxygenation was used as a bridge to VAD in five (36%) patients. Preimplant variables: 86% of patients requiring mechanical ventilation (mean 10.3 days), hyperbilirubinemia in 75%, and acute renal insufficiency in 79%. Device selection was systemic VAD in 11 (79%) and biventricular assist device in three (21%). Berlin Heart EXCOR was used in eight patients, while six patients received a Thoratec implantable VAD or paracorporeal VAD. Mean duration of support was 68 days (range 8-363 days). Overall survival was 79%. Ten patients (71%) were successfully bridged to transplantation, three (21%) died while on a device, one remains on support, and no patients were weaned from VAD. Children supported for single ventricle heart failure had a 50% survival with none currently bridged to transplantation. Complications included bleeding requiring reoperation in 21% (n = 3), stroke in 29% (n = 4), and driveline infections in 7% (n = 1). In two patients, a total of six pump exchanges were performed for thrombus formation. Survival for pediatric patients of all ages is excellent using current device technology with a majority of patients being successfully bridged to transplantation. Morbidity is acceptably low considering the severity of illness. Significant challenges exist with long-term extracorporeal support due to lack of donor availability and the high incidence of preformed alloantibodies especially in the failing single ventricle.

There are minimal data regarding chronic management of single-ventricle ventricular assist device (VAD) patients. This study aims to describe our center's multidisciplinary team management of single-ventricle patients supported long term with the Berlin Heart EXCOR Pediatric VAD. Patient #1 was a 4-year-old with double-outlet right ventricle with aortic atresia, L-looped ventricles, and heart block who developed heart failure 1 year after Fontan. She initially required extracorporeal membrane oxygenation support and was transitioned to Berlin Heart systemic VAD. She was supported for 363 days (cardiac intensive care unit [CICU] 335 days, floor 28 days). The postoperative course was complicated by intermittent infection including methicillin-resistant Staphylococcus aureus, intermittent hepatic and renal insufficiencies, and transient antithrombin, protein C, and protein S deficiencies resulting in multiple thrombi. She had a total of five pump changes over 10 months. Long-term medical management included anticoagulation with enoxaparin, platelet inhibition with aspirin and dipyridamole, and antibiotic prophylaxis using trimethoprim/sulfamethoxazole. She developed sepsis of unknown etiology and subsequently died from multiorgan failure. Patient #2 was a 4-year-old with hypoplastic left heart syndrome who developed heart failure 2 years after bidirectional Glenn shunt. At systemic VAD implantation, he was intubated with renal insufficiency. Post-VAD implantation, his renal insufficiency resolved, and he was successfully extubated to daytime nasal cannula and biphasic positive airway pressure at night. He was supported for 270 days (CICU 143 days, floor 127 days). The pump was upsized to a 50-mL pump in May 2011 for increased central venous pressures (29 mm Hg). Long-term medical management included anticoagulation with warfarin and single-agent platelet inhibition using dipyridamole due to aspirin resistance. He developed increased work of breathing requiring intubation, significant anasarca, and bleeding from the endotracheal tube. The family elected to withdraw support. Although both patients died prior to heart transplantation, a consistent specialized multidisciplinary team approach to the medical care of our VAD patients, consisting of cardiothoracic surgeons, heart transplant team, hematologists, pharmacists, infectious disease physicians, psychiatrists, specialty trained bedside nursing, and nurse practitioners, allowed us to manage these patients long term while awaiting heart transplantation.

Although heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined.We used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras.The cardiomyopathy group was significantly (p0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p0.001) for non-HLHS patients (51%, 48%) vs HLHS (71%, 61%), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95% confidence interval, 1.15-2.77, p = 0.009) and CM (80%, 74%; HR, 2.72; 95% confidence interval, 1.59-4.67, p0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups.Use of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.

The Norwood procedure for hypoplastic left heart syndrome (HLHS) aims to provide an unobstructed systemic outflow tract, unrestrictive inter-atrial communication, controlled source of pulmonary blood flow, and reliable source of coronary blood flow. The hybrid palliative strategy of pulmonary artery banding and ductal stenting has emerged as an alternative treatment for neonates with HLHS. Neonates who have undergone a hybrid Norwood but are not candidates for the three-stage single-ventricle pathway may need heart transplantation. Patients who have undergone hybrid Norwood or those with visceral heterotaxy who have undergone ductal stenting and bilateral PA bands represent a technically challenging group of patients for heart transplantation, but it appears to be a favorable approach and we describe our experience with three patients who underwent heart transplant after a hybrid Norwood procedure.

Background There has been much focus on factors influencing medical students' career choice, prompted by such concerns as a sufficient future surgical workforce, declining applicant pool, changing gender composition, and a cultural shift in values and priorities. Once in a surgical residency, there are little data on factors influencing general surgery (GS) residents' final specialty choice. Study Design A survey instrument was developed and content validated in conjunction with the Association of American Medical Colleges Center for Workforce Studies. The final instrument was distributed electronically between March 24 and May 2, 2008, through 251 GS program directors to all ACGME-accredited GS residents (n = 7,508). Results Response rate was 29% (2,153 residents; 89% programs). Half of GS residents remained undecided about specialty choice through the 2nd year, declining to 2% by year 5. Of the two-thirds who decided on a specialty, 16.5% chose to remain in GS, 14.6% chose plastics, 9.3% cardiothoracic, and 8.5% vascular. The specialty choice factors most likely to be very important were type of procedures and techniques, exposure to positive role model, and ability to balance work and personal life. Relative importance of factors in specialty choice varied by gender and chosen specialty. Mentors play a key role in specialty choice (66% decided had mentors versus 47% undecided). Work schedule was the most frequently selected shortcoming in every specialty except plastics. Cardiothoracic surgery followed by GS had the highest shortcomings. Conclusions The majority of GS residents plan to subspecialize. Three factors dominate specialty choice. Faculty need to understand their impact potential to modify or change perceptions of their specialty.

Background Applications to cardiothoracic surgery (CTS) training programs have declined precipitously. The viewpoints of potential applicants, general surgery residents, have not yet been assessed. Their perceptions are crucial to understanding the cause and formulating appropriate changes in our educational system. Methods An initial survey instrument was content-validated, and the final instrument was distributed electronically between March 24 and May 2, 2008 through 251 general surgery program directors to all Accreditation Council for Graduate Medical Education-accredited general surgery residents (7,508). Results The response rate was 29% (2153 residents; 89% programs). Respondent's demographics matched existing data; 6% were committed to CTS, and 26% reported prior or current interest in CTS. Interest waned after postgraduate year 3. Interest correlated with CTS rotation duration. Of the respondents committed to CTS, 76% had mentors (71% were cardiothoracic surgeons). CTS had the most shortcomings among 9 subspecialties. Job security and availability accounted for 46% of reported shortcomings (3 to 14 times higher than other subspecialties). Work schedule accounted for 25%. Length of training was not a very important factor, although it was identified as an option to increase interest in CTS. Residents who were undecided or uninterested in CTS were twice as likely to cite the ability to balance work and personal life as important than residents who chose CTS. Conclusions The dominant concern documented in the survey is job security and availability. The importance of mentorship and exposure to CTS faculty in promoting interest was also evident. Decision makers should consider these findings when planning changes in education and the specialty.

Exsanguinating hematemesis secondary to an aortoesophageal fistula from an impacted foreign body occurred in a patient with a vascular ring. This report describes successful resuscitation and repair in a 6-year-old boy who was transferred from an outside hospital in extremis with an aortoesophageal fistula from a foreign body in the presence of a vascular ring.

This study's objective was to evaluate insertion techniques and device fit of the pediatric version of the Impella ventricular assist device in swine which had similar sized carotids and left ventricles (LVs) as children weighing 10-20 kg.Options for minimally invasive circulatory support in children are limited. A modified device based on the current Impella 2.5 platform was created in an effort to provide minimally invasive circulatory support for children.Animal studies (n = 10) were performed to determine technical feasibility of device implant via the right common carotid artery (RCCA) in swine with a carotid and LV size similar to children with a BSA 1 mAll animals underwent successful device insertion. Mean carotid artery diameter by ultrasound was 3.5 ± 0.3 mm. There was no LV or aortic/mitral valve damage with a minimum LV length of 5.4 cm.Minimally invasive circulatory support is needed in small children. Limitations are primarily related to vessel and chamber size. The Impella Pediatric catheter was safely and successfully implanted in carotid arteries similar in size to children weighing 10-20 kg with minimal complications. © 2016 Wiley Periodicals, Inc.

Although nesiritide has been used in adults with left heart failure, the experience in the paediatric population is limited. We reviewed and analysed our experience with continuous nesiritide infusion as adjunct therapy in children with biventricular dysfunction due to diverse aetiologies and suffering from oliguria despite intravenous diuretics and inotropic therapies for heart-failure management.

The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed. Hospital records were reviewed, and follow-up data were obtained to evaluate outcomes. Overall, a total of 19 patients underwent a Senning procedure between August 2005 and July 2014. Median age at repair was 594 days (range: 5 days to 15 years). Of those, 7 patients underwent a combined Senning-Rastelli operation and 10 patients underwent a double switch operation (arterial switch or atrial switch). Primary diagnosis was congenitally corrected transposition of the great arteries (cc-TGA) in 17 patients. Associated lesions included ventricular septal defect (n = 10), pulmonary stenosis or atresia (n = 7), and Ebstein׳s anomaly of the tricuspid valve (n = 4). There was an operative death (5%). In all, 2 patients required superior vena cava baffle revision in the operating room. No new pulmonary venous or systemic venous baffle obstruction was observed during follow-up. A total of 5 (26%) patients developed heart block requiring pacemaker implantation. Actuarial survival was 81% at 5 years with a median follow-up of 38 months. The 2 patients in the cc-TGA group were subsequently transplanted. Surviving patients (n = 15) are all well at the last clinical follow-up, most with normal biventricular function. In conclusion, the Senning procedure offers excellent outcomes with regard to systemic and pulmonary venous baffle patency. Results in patients with cc-TGA, however, are affected by significant early mortality after Senning-Rastelli and incidence of left ventricular dysfunction after double switch; which should be considered during patient selection and surgical planning.