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Background As a formative step toward development of adult congenital heart disease (ACHD) risk-adjusted metrics, we describe the STS ACHD population, procedural frequency, and early mortality. Methods Adults ( > 18 years) with CHD (2014-2019) were identified in the STS Adult Cardiac Surgery (ASCD) and the Congenital Heart Surgery (CHSD) Databases. Following deduplication, variable mapping, data concatenation, and harmonization of pre-procedure factors, procedures were grouped and unadjusted mortality catalogued for overall cohort and the cohort excluding patients with isolated bicuspid aortic valve (BAV). Results Among 171,186 ACSD and 18,281 CHSD records, 152,731 unique records met inclusion criteria. Twenty-eight congenital diagnoses accounted for 86% of the overall cohort, and prevalence of ACHD increased over the study timeframe. ACHD patients underwent operations to treat both acquired and CHD. Most common procedures overall and after excluding isolated BAV were: aortic valve replacement (AVR), ascending aortic surgery, and coronary artery bypass grafting (CABG). After excluding isolated BAV, major procedure combinations occurred in 46% (31% had 2 and 3% > 4 major procedures) and most prevalent combinations included: AVR + CABG (n=2,352), AVR + subaortic stenosis repair (n=1,481) and AVR + ascending aortic surgery (n=1,239). Unadjusted 30-day mortality was procedure dependent, 2.8% overall and 3.6% with isolated BAV excluded. Conclusions The ACHD surgical population is heterogenous, and patients undergo surgery for CHD-related and adult/acquired procedure combinations. Early mortality is variable and influenced by surgical complexity. Excluding isolated BAV patients and developing procedure-based ACHD mortality risk models may be ideal but will require empirically-derived grouping and collaboration.

A newborn presented with tetralogy of Fallot (TOF), right aortic arch (RAA), and isolated left brachiocephalic artery. The RAA supplied the right common carotid artery, right vertebral artery, and right subclavian artery, in that order. The left common carotid and left subclavian arteries were in continuity with no aortic origin. Ultrasound demonstrated retrograde flow in the left vertebral artery supplying antegrade flow to the diminutive left subclavian artery (ie, “steal phenomenon”). The patient underwent repair of TOF without intervention on the left common carotid or left subclavian arteries and is being followed conservatively.

Every effort must be made by the leaders in our field, as well as by every individual cardiothoracic surgeon, to assure equal opportunity for all cardiothoracic surgeons, regardless of race, gender or any other sociodemographic source of bias. Every effort must be made by every surgeon, not just those in particular leadership roles. Opportunities for advancement must be equal in multiple domains, including clinical practice, patient referral, clinical leadership, academic leadership, institutional leadership and leadership in professional medical and surgical societies. Such actions to minimize bias and promote inclusivity will also ensure that cardiothoracic surgical care is provided by a workforce that represents the diversity of patients whom we serve. In the final analysis, it is an absolute fact that gender differences in payments to cardiothoracic surgeons are absolutely unacceptable and cannot be tolerated.

Extracorporeal life support (ECLS) may be life saving for patients with status asthmaticus (SA), a difficult-to-treat, severe subset of asthma. Contemporary ECLS outcomes for SA in teens and young adults are not well described. The Extracorporeal Life Support Organization (ELSO) Registry was reviewed (2009-2019) for patients (15-35 years) with a primary diagnosis of SA. In-hospital mortality and complications were described. Multivariable logistic regression was used to identify independent risk factors for hospital mortality. Overall, 137 patients, (26 teens and 111 young adults; median age 25 years) were included. Extracorporeal life support utilization for SA sharply increased in 2010, coinciding with increased ECLS utilization overall. Median ECLS duration and length of stay were 97 hours and 11 days, respectively. In-hospital mortality and major complication rates were 10% and 11%, respectively. Nonsurvivors were more likely to have experienced ECLS complications, compared to survivors (86% vs. 42%, p = 0.003). Independent risk factors for in-hospital mortality included pre-ECLS arrest and any renal and/or neurologic complication. Prospective studies designed to evaluate complications and subsequent failure to rescue may help optimize quality improvement efforts.

Environmental factors may influence the development of tetralogy of Fallot (TOF), and DNA methylation patterns may reveal specific chemical signatures of perturbations during cardiac development. We investigated whether blood and buccal cells could be viable surrogates for myocardium.We measured epigenome-wide DNA methylation at 866,895 5'-cytosine-phosphate-guanine-3' (CpG) sites in blood (n=3), buccal cells (n=3), and right ventricular myocardium (n=4) collected from infants with TOF and compared the percent of differentially methylated CpG sites across tissue types. Gene-specific DNA methylation profiles were also analyzed for ten representative genes associated with heart development. Welch's ANOVAs compared general methylation between tissue types.Comparison of DNA methylation profiles across blood, buccal, and myocardium suggested myocardium and buccal samples were most similar, differing in DNA methylation at only 1.3% (11,386) of CpG sites whereas myocardium and blood were most dissimilar, having 146,857 statistically dissimilar methylated CpG sites (~17% dissimilarity; adjusted p0.01 for each site). Buccal swabs were significantly more variable (p.001) than either blood or myocardial samples. In gene-specific analyses, SCO2, GATA4, NOTCH4, WNT7A, and DKK2 showed conserved DNA methylation profiles across tissue types, while HAND1, JAG1, NKX2-5, TBX5 and TBX20 showed more distinctive tissue-specific patterns of DNA methylation.Compared with blood, buccal tissue more closely mirrors the myocardial methylome, with10-fold similarity. Nevertheless, both buccal and blood tissue capture highly conserved DNA methylation patterns at specific genetic loci related to cardiac development. Buccal cheek swabs may be a useful surrogate tissue type for future investigations of TOF-specific epigenetic profiles.

Background Contemporary practice patterns and outcomes for aortic valve replacement (AVR) among young and middle-aged adults are unknown given guideline modifications for surgical AVR (SAVR) and increasing transcatheter AVR (TAVR) acceptance. This study describes SAVR and TAVR use and outcomes using The Society of Thoracic Surgeons (STS) National Databases. Methods Adults 18 to 55 years of age in the Congenital Heart Surgery Database (CHSD) and the Adult Cardiac Surgery Database (ACSD) who underwent SAVR or TAVR from 2013 to 2018 were included. Perioperative characteristics and early outcomes were described by valve type. Multivariable regression identified determinants of death, length of hospital stay, and a composite end point of renal failure, persistent neurologic deficit, readmission, and reoperation. Results The study analyzed 1580 unique CHSD and 44,173 ACSD operations, 16% of which were performed in patients with congenital heart disease. Valve use included the following: TAVR, 1%; mechanical, 42%; bioprosthetic, 55%; autograft, 0.6%; homograft, 1.2%; and Ozaki, 0.4%. Over time, TAVR volumes increased by 167%. The 30-day mortality was as follows: TAVR, 3.8%; mechanical, 3.2%; bioprosthetic, 3.7%; autograft, 0.6%; homograft, 9%; and Ozaki, 3.4%. Stroke rate was lower for isolated SAVR vs isolated TAVR (0.9% vs 2.4%; P = .002). In multivariable analyses, mortality risk was lower with mechanical valves, congenital morbidity risk was higher with TAVR, and length of stay was shorter with TAVR. Conclusions TAVR is increasingly used for adults younger than 55 years of age. Given the uniformly excellent results with SAVR, including both mortality and morbidity—particularly regarding stroke, our data favor SAVR in this population, but a prospective trial is needed. Ongoing efforts to harmonize variables and outcomes definitions between the ACSD and CHSD are valuable.

Case Presentation A previously healthy 22-month-old girl presented to the ED with a 3-week history of dyspnea on exertion. A chest radiograph showed a right upper-lobe opacity suspicious for pneumonia ( Fig 1 A). The patient was prescribed amoxicillin but returned to the ED 7 days later with cough and persistent dyspnea and tachypnea. At that time, a repeat chest radiograph was concerning for worsening pneumonia ( Fig 1 B). Treatment with azithromycin and albuterol was initiated, and amoxicillin was discontinued. Her symptoms briefly improved; however, she returned to the ED 10 days later because of worsening cough and tachypnea, and a 2-day history of increased irritability, decreased oral intake, decreased urine output, and intermittent perioral cyanosis. She was afebrile throughout this period per parent report and vital sign documentation at each ED visit.

Double aortic arch with circumflex aorta is a rare type of vascular ring. Symptoms result from external compression of the trachea and esophagus. The best surgical approach for patients with double arch and circumflex aorta is debated, and options include the highly complex aortic uncrossing procedure. Herein we describe the surgical treatment of a patient with concurrent double aortic arch and circumflex aorta by division of the non-dominant arch and ligamentum arteriosum, plication and posterior tacking of the diverticulum of Kommerell. This left thoracotomy approach provided complete symptom resolution.

To identify opportunities for enhanced data collection for adults with congenital heart disease (ACHD), a structured review of existing variables in The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) and the STS Adult Cardiac Surgery Database (ACSD) was conducted.A working group was assembled representing multiple STS Workforces and Task Forces. The ACSD was reviewed systematically over eight 90-minute calls. ACSD version 4.20.2 and CHSD version 3.41 were used, and the ACSD was approached in sections. ACSD variables were classified as (1) represented in identical form in the CHSD (no further discussion), (2) represented in similar form in the CHSD (discussed for potential harmonization of definitions), or (3) not represented in the CHSD (discussed for potential inclusion). Variables felt to be relevant to ACHD were noted, and special consideration was given to STS required fields and variables used in existing STS adult risk models. Other factors that were examined were the frequency, use, and capture of existing ACSD variables.Over 22 weeks (8 calls), the existing 1069 variables in version 4.20.2 of the ACSD were discussed. Ultimately, 539 total variables were found to be both (1) relevant to ACHD and (2) not currently collected in the CHSD. These were recommended for inclusion in the next CHSD upgrade for patients aged ≥18 years.For adult patients having case records entered into the CHSD, the inclusion of a limited set of additional data fields from the ACSD should enhance capture of comorbidities and other clinical data relevant to the ACHD population.

Aberrant right subclavian artery, or arteria lusoria, is a common congenital anomaly of the aortic arch and may be associated with dysphagia. Surgical treatment, particularly in children, remains controversial. Current surgical interventions include supraclavicular and thoracotomy approaches, as well as hybrid endovascular techniques. However, these techniques have significant limitations and varying degrees of success. This case report describes a 2-site operation whereby the arteria lusoria is transected via a left thoracotomy and subsequently re-implanted into the right common carotid artery via a median sternotomy. This approach has provided complete symptom resolution for dysphagia lusoria in 2 children.

An increasing number of pediatric heart centers are utilizing early extubation protocols to “fast-track” patients after congenital heart surgery. Early postoperative arrhythmias are an important recognized complication of pediatric heart surgery, but the arrhythmia burden among candidates for extubation immediately after bypass surgery is unknown. This study evaluated the incidence and risk factors associated with postoperative arrhythmias in surgical fast-track candidates. Postoperative rhythm status and potential perioperative risk factors were retrospectively analyzed in all patients undergoing surgery for congenital heart disease from 2012 to 2014 who were considered early extubation candidates. Overall, 199 operations in 196 individual patients (30 days–19 years) were included. The overall incidence of arrhythmias was 9% (18/199). The most common arrhythmia was junctional ectopic tachycardia (5%; 10/199), followed by atrial tachycardia (1.5%; 3/199) and complete heart block (1.5%; 3/199). Two patients with junctional ectopic tachycardia had permanent complete heart block. Compared to patients with no arrhythmias, patients with arrhythmias were more likely to be younger (0.5 vs. 2.4 years, U = 783, p Early postoperative arrhythmias are common among fast-track candidates. Patient age and operation complexity may predict risk of early arrhythmia. These data provide important information to help guide programmatic and clinical decision-making when contemplating fast tracking children after congenital heart surgery.

Enamel hypoplasia is a dental pathology that forms when an individual is exposed to physiological stress in early life while tooth crowns are developing. Biological anthropologists utilize these enamel defects as indicators of growth interruption and interpret them as reflective of factors pertaining to health status and cultural practices that influence health. Over decades of research, numerous studies have noted a pattern in the distribution of linear enamel hypoplasia across the dentition. It is suggested that the anterior dentition presents the highest frequency of defects, followed by the premolars, with molars most rarely exhibiting hypoplasia. This apparent differential susceptibility pattern has resulted in the preferential study of the anterior dentition in anthropology, however little research has been conducted into the validity or cause of this developmental phenomenon. Through examination of the literature, the observed higher frequency of enam2el hypoplasia in the anterior dentition substantiates the existence of this differential distribution pattern. Further investigation reveals that the cause of this varying susceptibility has not been sufficiently explored, leading to a number of inconclusive explanations. Examination of these theories – ranging from the chronology of tooth development, specifics of crown morphology, and variations in genetic control – indicate that there is no single causal variable, but that a multitude of factors are responsible. From this research it is apparent that further study is necessary to fully understand why the anterior teeth appear to be more susceptible to hypoplastic defects than their posterior counterparts.

BACKGROUND New drugs may further decrease the need for lung transplant (LTx) in pediatric patients with cystic fibrosis (CF), but few studies highlight pediatric non-CF LTx characteristics and outcomes. METHODS The ISHLT registry was used to report morbidity, graft failure, and survival for primary pediatric (

OBJECTIVES The aim of this study is to explore growth discrepancies in the dentition of impoverished children and examine how dental development is impacted by environmental influences throughout childhood, thereby identifying which teeth are more sensitive to the effects of biocultural factors and are consequently less useful to predict age. METHODS Length measurements of developing teeth (deciduous and permanent) were taken from individuals of known age and sex (n = 61) from the Certosa collection, a 19th century skeletal assemblage representing Italian children of low socioeconomic status. Discrepancies between age estimates based on tooth length and chronological age were calculated, and the accuracy and precision of age prediction between earlier forming teeth and later forming teeth were compared. RESULTS Deciduous teeth produced more precise dental age estimates (mean age discrepancy -0.092 years), while discrepancies between chronological age and age based on developing permanent dentition were larger (-0.628 years). The difference between these discrepancies in age estimates for deciduous and permanent teeth was significant (p

Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2).From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years. Of those, 355 underwent surgical replacement (PC2) of that initial conduit. Competing risk methodology and multiphase parametric hazard analyses were used to identify factors associated with time-related risk of PC3 or TPVI.Of 355 PC2 patients (median follow-up, 5.3 years), 65 underwent PC3 and 41 TPVI. Factors at PC2 associated with increased time-related risk of PC3 were smaller PC2 Z score (hazard ratio [HR] 1.6, P.001), concomitant aortic valve intervention (HR 7.6, P = .009), aortic allograft (HR 2.2, P = .008), younger age (HR 1.4, P.001), and larger Z score of PC1 (HR 1.2, P = .04). Factors at PC2 associated with increased time-related risk of TPVI were aortic allograft (HR: 3.3, P = .006), porcine unstented conduit (HR 4.7, P.001), and older age (HR 2.3, P = .01).Aortic allograft as PC2 was associated with increased time-related risk of both PC3 and TPVI. Surgeons may reduce risk of these subsequent procedures by not selecting an aortic homograft at PC2, and by oversizing the conduit when anatomically feasible.

There is evidence that reducing opioid exposure in children undergoing cardiac surgery may enhance postoperative recovery. We aimed to describe a minimal opioid postoperative management protocol in children undergoing cardiac surgery and our early outcomes with this strategy. We reviewed the medical records of children (6 months-18 years) who underwent elective cardiac surgery through a median sternotomy with cardiopulmonary bypass at our institution between 2016 and 2018. All patients were managed postoperatively using a standardized protocol. 101 children (median age 5 years) were included and 85% were extubated in the operating room. Although most patients (96%) received opioids postoperatively, opioid requirements decreased steadily over time, with 88%, 58%, and 18% of children receiving opioids on postoperative day 1, 2, and 3, respectively; 41% received no opioids after postoperative day 1. The median cumulative opioid exposure was 0.25 morphine milligram equivalents per kg (interquartile range, 0.10-0.75). Greater than mild pain was rare (10%) at each time point. The rates of operative mortality and major complication were 0% and 3%, respectively. The median postoperative length of stay was 3 days, and 13% required readmission within 30 days. Age, cardiopulmonary bypass time, and number of benzodiazepine doses were independently associated with cumulative opioid exposure. Any complication, chest tube time, and higher STAT Category were independently associated with prolonged postoperative length of stay. A minimal opioid postoperative management protocol can be safe and effective in children undergoing cardiac surgery. Future prospective studies are needed to determine optimal practice and patient selection.

During repair of atrioventricular septal defect (AVSD), surgeons might leave an atrial level shunt when concerned about postoperative physiology, or as part of routine practice. However, the association of fenestration with outcomes is unclear. We sought to determine factors associated with mortality after biventricular repair of AVSD.We included 581 patients enrolled from 32 Congenital Heart Surgeons' Society institutions from January 1, 2012, to June 1, 2020 in the Congenital Heart Surgeons' Society AVSD cohort. Parametric multiphase hazard analysis was used to identify factors associated with mortality. A random effect model was used to account for possible intersite variability in mortality.An atrial fenestration was placed during repair in 133/581 (23%) patients. Overall 5-year survival after repair was 91%. Patients who had fenestration had an 83% 5-year survival versus 93% for those not fenestrated (P .001). Variables associated with mortality in multivariable hazard analysis included institutional diagnosis of ventricular unbalance (hazard ratio [HR], 2.7 [95% confidence interval (CI): 1.5-4.9]; P = .003), preoperative mechanical ventilation (HR, 4.1 [95% CI, 1.3-13.1]; P = .02), atrial fenestration (HR, 2.8 [95% CI, 1.5-4.9]; P .001), and reoperation for ventricular septal defect (HR, 4.0 [95% CI, 1.3-13.1]; P = .002). There was no difference in measures of ventricular unbalance for comparisons of fenestrated with nonfenestrated patients. No significant interinstitution variability in mortality was observed on the basis of the random effect model (P = .7).An atrial communication at biventricular repair of AVSD is associated with significantly reduced long-term survival after adjusting for other known associated factors, including unbalance. These findings might challenge the routine practice of fenestration.

Antibiotic prophylaxis following delayed sternal closure in pediatric cardiac surgery is not standardized. We systematically reviewed relevant literature published between 1990 and 2019 to aid future trial design. Patient characteristics, antimicrobial prophylaxis regimens, and postoperative incidence of infection were collected. Twenty-eight studies described 36 different regimens in over 3,000 patients. There were 11 single-drug regimens and 25 multidrug regimens. Cefazolin-only was the most common regimen (9/36, 25%). The overall incidence of surgical site infection was 7.5% (217/2,910 patients) and bloodstream infection was 7.4% (123/1,667 patients). In the 2010s, multidrug regimens were associated with a significantly lower incidence of both surgical site infections (4.6% vs. 20%, P < .001) and bloodstream infections (6.0% vs. 50%, P < .001) compared to single-drug regimens.

Granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody–associated vasculitis that primarily affects small vessels. The disease typically affects the respiratory tract and kidneys but has also been known to involve the gastrointestinal tract, genitourinary tract, thyroid, and liver. Cardiac involvement is rare. Coronary artery aneurysms (CAAs) are an extremely uncommon finding, with only 1 reported case in an adult patient and no pediatric cases reported to date. Here, we report the unique case of a child with granulomatosis with polyangiitis who initially presented with fever of unknown origin and pulmonary and renal symptoms with no cardiac complaints. An echocardiogram revealed severe bilateral fusiform CAAs. Because of the high risk of mortality posed by the severity of her renal and cardiac disease, the patient was managed with intensive induction immunosuppression with steroids, rituximab, and cyclophosphamide. She is maintained on steroids, rituximab, aspirin, and warfarin with improved renal function but no change in her CAAs.

There are no established neuroprotective guidelines during pediatric extracorporeal membrane oxygenation (ECMO) after cardiac arrest. We report a case of unknown duration, out-of-hospital cardiac arrest after drowning in a toddler. The patient experienced severe cardiopulmonary failure shortly after return of spontaneous circulation and required veno-arterial ECMO. Clinical outcome was excellent. This report outlines a neuroprotective strategy using high-dose erythropoietin, dexmedetomidine, and therapeutic hypothermia that was implemented during ECMO. Risk factors related to pediatric drowning with cardiac arrest are also discussed.

Hypoplastic left heart syndrome (HLHS) is a highly resource-intensive diagnosis. Geographic variation in cost and length of stay (LOS) in HLHS is not well described. Neonates diagnosed with HLHS between 2000 and 2012 were identified using the Kids’ Inpatient Database. Hospitalizations were stratified into two groups: (1) birth and (2) secondary. United States regional differences in hospital charges and LOS were compared using adjusted linear regression. Of 2431 birth hospitalizations, 449 neonates (18.5%) died while inpatient and mortality rates differed by region (p = 0.02). After birth, 40.5% (n = 985) of neonates were transferred; transfers were most common in the Midwest (p

BACKGROUND Differences in the prevalence of left and right congenital heart defects (CHD) across the United States are unclear. This study evaluated the overall prevalence and the distribution of right versus left CHD across US regions and divisions in neonates. METHODS Newborns born from 2000 to 2014 diagnosed with CHD were identified using the National Inpatient Sample. Heart defects were stratified into right, left, and "neither" subtypes. The risk of right and left heart diagnoses between US Census regions and divisions was compared using multivariable binomial regression, adjusting for infant, and hospital characteristics. RESULTS Two hundred forty thousand four hundred fifty-five newborns were included and 38,185 (15.9%) were classifiable as having either right or left subtypes. Between 2000 and 2014, the prevalence of right defects increased from 1.65 to 2.88 cases/1,000 live born infants (p

BackgroundPatient-reported outcomes and epidemiological studies in adults with tetralogy of Fallot are lacking. Recruitment and longitudinal follow-up investigation across institutions is particularly challenging. Objectives of this study were to assess the feasibility of recruiting adult patients with tetralogy of Fallot for a patient-reported outcomes study, describe challenges for recruitment, and create an interactive, online tetralogy of Fallot registry.MethodsAdult patients living with tetralogy of Fallot, aged 18–58 years, at the University of North Carolina were identified using diagnosis code query. A survey was designed to collect demographics, symptoms, history, and birth mother information. Recruitment was attempted by phone (Part I, n=20) or by email (Part II, n=20). Data analysis included thematic grouping of recruitment challenges and descriptive statistics. Feasibility threshold was 75% for recruitment and for data fields completed per patient.ResultsIn Part I, 60% (12/20) were successfully contacted and eight (40%) were enrolled. Demographics and birth mother information were obtained for all enrolled patients. In Part II, 70% (14/20) were successfully contacted; 30% (6/20) enrolled and completed all data fields linked to REDCap database; the median time for survey completion was 8 minutes. Half of the patients had cardiac operations/procedures performed at more than one hospital. Automatic electronic data entry from the online survey was uncomplicated.ConclusionsAlthough recruitment (54%) fell below our feasibility threshold, enrolled individuals were willing to complete phone or online surveys. Incorrect contact information, privacy concerns, and patient-reported time constraints were challenges for recruitment. Creating an online survey and linked database is technically feasible and efficient for patient-reported outcomes research.

Background A substantial proportion of infants born with tetralogy of Fallot (TOF) die in infancy. A better understanding of the heterogeneity associated with TOF, including extracardiac malformations and chromosomal anomalies is vital to stratifying risk and optimizing outcomes during infancy. Methods Using the North Carolina Birth Defects Monitoring Program, infants diagnosed with TOF and born between 2003 and 2012 were included. Kaplan-Meier survival curves were used to estimate cumulative 1-year mortality, stratified by the presence of concomitant birth defects (BDs) and chromosomal anomalies. Multivariable logistic regression was used to estimate the direct effect of each concomitant BD, after adjusting for all others. Results A total of 496 infants with TOF were included, and 15% (n = 76) died. The number of concomitant BD systems was significantly associated with the risk of death at 1-year, p

Background Geographic variation in congenital heart disease is not well-described. This study uses geographic information systems (GIS) to describe the spatial epidemiology of tetralogy of Fallot (TOF), in North Carolina (NC) and to compare travel time for cases to congenital heart centers in NC. Methods Using the NC Birth Defects Monitoring Program database, live births with TOF born between 2003 and 2012 were identified. Birth certificates provided demographic variables. A denominator of live births/zip code was obtained from the NC live births database. ArcGIS® software was used to illustrate TOF prevalence by zip code, and SatScanTM was used to identify spatial clusters of TOF cases and to identify changes in cluster location over time. Driving time to each of five NC congenital heart centers was predicted based on road systems information. Results A total of 496 infants were born with TOF between 2003 and 2012. The prevalence was 4.2/10,000 live births. A large cluster (330 zip codes, 306 cases) was identified in northeastern NC. Average driving time for each case to closest congenital heart center was: University of North Carolina 37 min, Vident Medical Center 64 min, Duke University 58 min, Carolina's Medical Center 89 min, and Wake Forest Baptist Health 57 min. Overall, average predicted driving time to the nearest congenital heart center was 61 min. Conclusion Approximately 50 infants/year were born with TOF in NC. One cluster was identified. Further study is necessary to explore potential explanations for the observed case cluster. As interest in regionalization of congenital heart surgery grows, GIS and spatial analysis can become increasingly useful tools for health care planning. Birth Defects Research (Part A) 106:881-887, 2016. © 2016 Wiley Periodicals, Inc.

We present the case of a 17-year-old boy with a cardiac venous malformation. This case highlights the diagnostic challenges of such tumours and demonstrates the potential efficacy of a watch-and-wait management approach.

Several options exist for aortic valve replacement (AVR) in children and teens, but contemporary practice patterns and outcome data are lacking. We describe national AVR practice patterns and early outcomes.Children (aged 1 to 18 years) in The Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing AVR from 2000 to 2016 were included. Preoperative characteristics, operative data, and outcomes were described. To evaluate practice patterns, centers were assigned tertiles by aortic valve surgical volume. Statistical comparisons included Mann-Whitney U statistic, Kruskal-Wallis, χIn total, 3446 operations (46% children aged 1 to 12 years; 54% teens aged 12 to 18 years) were included. Preoperative risk factors were present in 23%, and 46% had a prior sternotomy. Valve utilization included autograft (64% child, 37% teen), mechanical (19% child, 35% teen), bioprosthetic (8% child, 20% teen), and homograft (9% child, 7% teen). Autografts were utilized more often for teenage girls than for teenage boys (odds ratio 1.3, 95% confidence interval: 1.05 to 1.66, P.05). Overall, inpatient mortality and major complications affected 1% and 10%, respectively, and these rates were highest for homografts (5%, P.001, and 13%, P.05). Autograft utilization varied widely across centers (10th to 90th percentile: 21% to 71% of total AVR volume). More autografts were utilized at high-volume centers vs low- or medium-volume centers (53% ± 2.3% vs 46% ± 2.6%, P.001).Practice patterns for AVR in children and teens vary across centers, age groups, and sexes. Although early outcomes were similar across valve types, homografts had higher morbidity and mortality. Valve choice was related to aortic valve surgical volume. Further efforts are needed to understand and optimize AVR practice patterns and long-term outcomes.

Coarctation of the aorta is a common form of congenital heart disease. Presentation, evaluation, and treatment of coarctation of the aorta is different in neonates and infants compared with older children. Neonates may present in shock and require prostaglandin E1 to maintain ductal patentcy until the time of surgical repair. Older children usually present with upper extremity hypertension or murmur. The approach to surgical repair of coarctation in neonates and infants depends on the presence or absence of concomitant defects and the degree of associated arch hypoplasia. For older children, robust collaterals and limited mobility of the aorta may mandate patch repair, interposition graft, or bypass of the coarctation segment. Percutaneous techniques, including angioplasty and stent placement, are often viable options for older children and adolescents with native or recurrent coarctation. Surgical and interventional outcomes have improved over time. Reliable repair may be anticipated with a very low rate of morbidity and mortality, although lifetime follow-up is recommended.

In 1529, Peter Dell the Elder (1490–1552) made a relief sculpture of the Resurrection for Duke Heinrich of Saxony. At this time, Heinrich was shifting toward his wife Katharina's Lutheranism despite his elder brother Georg's disapproval. The relief's disjunctive, nonillusionistic appearance resonates with Lutheran image theology. Its maintenance of visual difference exemplifies a faith in sacred history's power to transcend historical difference; the relief depicts Heinrich's conversion as one sacrohistorical event among many. The relief's disjunctive appearance also provides an occasion to propose the general heuristic term “chimerism” to describe a specific kind of visual form: not a hybrid that fuses different visual modes, but a chimera that joins unlike features while preserving their differences.