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There is emerging evidence that children with complex congenital heart defects (CHDs) are at increased risk for childhood lymphoma, but the mechanisms underlying this association are unclear. Thus, we sought to evaluate the role of DNA methylation patterns on "CHD-lymphoma" associations.From3 million live births (1988-2004) in California registry linkages, we obtained newborn dried bloodspots from eight children with CHD-lymphoma through the California BioBank. We performed case-control epigenome-wide association analyses (EWAS) using two comparison groups with reciprocal discovery and validation to identify differential methylation associated with CHD-lymphoma.After correction for multiple testing at the discovery and validation stages, individuals with CHD-lymphoma had differential newborn methylation at six sites relative to two comparison groups. Our top finding was significant in both EWAS and indicates PPFIA1 cg25574765 was hypomethylated among individuals with CHD-lymphoma (mean beta = 0.04) relative to both unaffected individuals (mean beta = 0.93, p = 1.5 × 10We identified associations between molecular changes present in the genome at birth and risk of childhood lymphoma among those with CHD. Our findings also highlight novel perinatal exposures that may underlie methylation changes in CHD predisposing to lymphoma.

This study aimed to develop objective diagnostic criteria for early onset Marfan syndrome (eoMFS) to facilitate early diagnosis and timely interventions.On the basis of an extensive literature review and the responses from a survey distributed among providers with expertise in the diagnosis and management of eoMFS, we developed an age-based, diagnostic scoring system encompassing 10 features common to eoMFS (9 clinical + 1 laboratory) and divided them into cardiac, systemic, and FBN1 (on the basis of the location of the pathogenic FBN1 variant) scores.In total, 77 individuals with eoMFS (13 newly reported) and 49 individuals diagnosed with classical Marfan syndrome during early childhood were used to validate the criteria. Median cardiac (8 vs 0, P.001), systemic (11 vs 3, P.001), FBN1 (5 vs 0, P.001), and total (23 vs 4, P.001) scores were significantly higher in individuals with eoMFS than in those without. A proposed clinical score (cardiac + systemic) cutoff of ≥14 points showed excellent sensitivity (100%), specificity (92%), and reliability (correctly classified = 94%).Distinct from classical Marfan syndrome in phenotype and morbidity, eoMFS can be diagnosed clinically using an objective scoring system encompassing the typical physical features and cardiac disease manifestations. Although genetic testing can be suggestive of eoMFS, genetic testing alone is insufficient for diagnosis.

Even in the absence of significant valvular disease, patients with Marfan syndrome (MFS) have evidence of impaired left ventricular (LV) performance, suggestive of a primary cardiomyopathy. However, the true prevalence and long-term outcomes of this disease process remain largely unknown. We performed a retrospective analysis of all adult patients with confirmed MFS followed at Stanford Health Care. Those with significant valvular regurgitation, coronary artery disease, or previous cardiac surgery were excluded. LV systolic dysfunction was defined as a LV ejection fraction (LVEF)55% on transthoracic echocardiography. A total of 753 patients with confirmed MFS were followed up over a median duration of 8 years (interquartile range 4 to 13). Of those, 241 patients (53% women, 71% White) met inclusion criteria and comprised the study cohort. LV systolic dysfunction was present in 30 patients (12%), with a median age of onset of 25 years (interquartile range 19 to 37), median EF of 52% (interquartile range 48 to 54), and evidence of clinical heart failure (New York Heart Association functional class ≥II) in 10% of patients. LV systolic dysfunction was more common in patients with larger aortic root diameters (≥4.0 cm: Odds ratio = 4.5, 95% confidence interval = 1.2 to 17.1) but was not associated with other cardiovascular manifestations of MFS or traditional atherosclerotic risk factors. In conclusion, apart from significant valvular pathology, LV systolic dysfunction was prevalent in MFS from a young age, suggestive of a primary cardiomyopathy. LV dysfunction was typically mild and subclinical and occurred more commonly in patients with more pronounced aortopathies.

STRUCTURED ABSTRACT Background Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and complex form of congenital heart disease. Unifocalization of MAPCAs has been advocated by some groups for the treatment of this condition. The purpose of this study was to assess the mid-term fate of unifocalized MAPCAs in a cohort of patients with retro-esophageal MAPCAs. Methods This was a retrospective review of 37 patients who underwent a unifocalization procedure. All patients in this study had one or more retro-esophageal MAPCAs, and detailed mapping of the MAPCAs was made based on a combination of the cardiac catheterization and surgical findings. Results The 37 patients had a total of 166 MAPCAs, or 4.5 MAPCAs per patient. One hundred twenty nine (78%) MAPCAs were unifocalized, while 37 (22%) were ligated as they were dual supply. Median follow-up was 69 months. At follow-up cardiac catheterization, evaluation of the 129 unifocalized MAPCAs demonstrated that 123 (95%) had antegrade flow while six were occluded. For the 123 MAPCAs with antegrade flow, 97 (80%) were widely patent while 26 were stenotic. Thirteen of the 37 patients have subsequently undergone re-intervention on MAPCAs that were determined to be stenotic following unifocalization. Seven of these patients had mild disease and had complete resolution with balloon (n=5) or surgical revision (n=2). Six patients with moderate or severe disease underwent surgical revision with confirmed resolution in four of six. Conclusions The data demonstrate that the majority of unifocalized MAPCAs remain widely patent following unifocalization. However, one-quarter of unifocalized MAPCAs develop stenoses or occlusion. These results suggest the fate for most unifocalized MAPCAs is favorable but highlight the need for close vigilance.

The cellular lipidome is comprised of thousands of unique lipid species. Herein, using mass spectrometry-based targeted lipidomics we have characterized the lipid landscape of human and mouse immune cells (www.cellularlipidatlas.com). Using this resource, we show that immune cells have unique lipidomic signatures and that processes such as activation, maturation and development impact immune cell lipid composition. To demonstrate the potential of this resource to provide new insights into immune cell biology, we determined how a cell-specific lipid trait, differences in the abundance of poly-unsaturated fatty acid-containing glycerophospholipids (PUFA-PLs), influenced immune cell biology. We firstly show that differences in PUFA-PL content underpins the differential susceptibility of immune cells to ferroptosis, and secondly, that low PUFA-PL content promotes resistance to ferroptosis in activated neutrophils. In summary, we show that the lipid landscape is a defining feature of immune cell identity and cell-specific lipid phenotypes underpin aspects of immune cell physiology.

BackgroundAortic dilation in tetralogy of Fallot (TOF) is primarily attributed to increased aortic flowin utero. An alternative hypothesis is abnormal neural crest cell migration, with unequal septation of the truncus arteriosus resulting in a larger aorta and inherently hypoplastic pulmonary artery (PA). If so, we hypothesize the aorta to PA ratio (Ao:PA) in TOF is stable throughout gestation, and the total sum of dimensions of the great arteries is similar to controls.MethodsWe performed a single-center retrospective study of all fetuses with TOF (2014-2020) and gestational age-matched controls. We compared sums of diameters, circumferences, and cross-sectional areas of the aorta and PA and evaluated the Ao:PA across gestation in TOF with pulmonary stenosis (TOF-PS) and atresia (TOF-PA). We analyzed data with two-tailed t-tests and Pearson’s correlation.ResultsThere were 100 fetuses with TOF (36% TOF-PA) with median gestational age of 31 weeks [IQR 26.5, 34.4] and median maternal age of 34 years [IQR 30, 37]. There were no differences in sums of great artery dimensions between TOF-PS and controls. In TOF-PA, sums were significantly lower than controls. The Ao:PA was stable throughout gestation.ConclusionsThe aorta in fetal TOF is large but grows proportionally throughout gestation, with a sum of great artery dimensions similar to controls. TOF-PA appears to be distinct from TOF-PS (with overall smaller dimensions), and is a group that warrants further investigation. In conclusion, our findings do not support the flow-mediated model of aortic dilation in TOF, and instead suggest an intrinsic developmental mechanism.Clinical PerspectiveWhat’s New?The aorta in fetal Tetralogy of Fallot (TOF) is large, but grows proportionally throughout gestation with a total sum of great artery dimensions similar to controls.Fetuses with TOF with pulmonary atresia have smaller great artery dimensions than TOF with pulmonary stenosis; this distinct group warrants further investigation.Our findings suggest that aortic dilation in TOF may be secondary to an intrinsic developmental mechanism, rather than from increased flow to the aortain utero.What are the clinical implications?The mechanisms of aortic dilation in fetal TOF have not been previously investigated.While aortic dilation is commonly seen in TOF, the degree of its progression over time and risk of dissection are not well understood.A better understanding of the etiology of aortic dilation in TOF could help to inform management decisions, particularly regarding the need for prophylactic surgical repair.

To assess the association between hypertrophic cardiomyopathy (HCM) and mortality among infants of diabetic mothers (IDMs).We performed a retrospective cohort study of hospitalized IDMs admitted at ≤14-days-old in the Pediatric Health Information System (years 2004 - 2019). Multivariable logistic regression was used to evaluate the association between HCM and mortality; covariates in the model were prematurity, sex, and congenital malformations of the cardiovascular, nervous, urinary and musculoskeletal systems.Among 32,993 IDMs, there were 203 (0.6%) with HCM. Black and Hispanic children were disproportionately represented among children with HCM compared to those without HCM (23.2 vs. 14.9%,We identify HCM as a contributor to in-hospital mortality. These data reinforce the need for more specific diagnostic criteria, better prevention of maternal diabetes, and effective therapies for HCM in IDMs.

Supravalvar aortic stenosis (SVAS) severity guides management, including decisions for surgery. Physiologic and technical factors limit the determination of SVAS severity by Doppler echocardiography and cardiac catheterization in Williams syndrome (WS). We hypothesized SVAS severity could be determined by the sinotubular junction-to-aortic annulus ratio (STJ:An). We reviewed all preintervention echocardiograms in patients with WS with SVAS cared for at our center. We measured STJ, An, peak and mean Doppler gradients, and calculated STJ:An. We created 2 mean gradient prediction models. Model 1 used the simplified Bernoulli's equation, and model 2 used computational fluid dynamics (CFD). We compared STJ:An to Doppler-derived and CFD gradients. We reviewed catheterization gradients and the waveforms and analyzed gradient variability. We analyzed 168 echocardiograms in 54 children (58% male, median age at scan 1.2 years, interquartile range [IQR] 0.5 to 3.6, median echocardiograms 2, IQR 1 to 4). Median SVAS peak Doppler gradient was 24 mm Hg (IQR 14 to 46.5). Median SVAS mean Doppler gradient was 11 mm Hg (IQR 6 to 21). Median STJ:An was 0.76 (IQR 0.63 to 0.84). Model 1 underpredicted clinical gradients. Model 2 correlated well with STJ:An through all severity ranges and demonstrated increased pressure recovery distance with decreased STJ:An. The median potential variability in catheterization-derived gradients in a given patient was 14.5 mm Hg (IQR 7.5 to 19.3). SVAS severity in WS can be accurately assessed using STJ:An. CFD predicts clinical data well through all SVAS severity levels. STJ:An is independent of physiologic state and has fewer technical limitations than Doppler echocardiography and catheterization. STJ:An could augment traditional methods in guiding surgical management decisions.

Residential proximity to greenspace is associated with various health outcomes.We estimated associations between maternal residential proximity to greenspace (based on an index of vegetation) and selected structural birth defects, including effect modification by neighborhood-level factors.Data were from the National Birth Defects Prevention Study (1997-2011) and included 19,065 infants with at least one eligible birth defect (cases) and 8925 without birth defects (controls) from eight Centers throughout the United States. Maternal participants reported their addresses throughout pregnancy. Each address was systematically geocoded and residences around conception were linked to greenspace, US Census, and US Department of Agriculture data. Greenspace was estimated using the normalized difference vegetation index (NDVI); average maximum NDVI was estimated within 100 m and 500 m concentric buffers surrounding geocoded addresses to estimate residential NDVI. We used logistic regression to estimate odds ratios (ORs) and 95% confidence intervals comparing those in the highest and lowest quartiles of residential NDVI and stratifying by rural/urban residence and neighborhood median income.After multivariable adjustment, for the 500 m buffer, inverse associations were observed for tetralogy of Fallot, secundum atrial septal defects, anencephaly, anotia/microtia, cleft lip ± cleft palate, transverse limb deficiency, and omphalocele, (aORs: 0.54-0.86). Results were similar for 100 m buffer analyses and similar patterns were observed for other defects, though results were not significant. Significant heterogeneity was observed after stratification by rural/urban for hypoplastic left heart, coarctation of the aorta, and cleft palate, with inverse associations only among participants residing in rural areas. Stratification by median income showed heterogeneity for atrioventricular and secundum atrial septal defects, anencephaly, and anorectal atresia, with inverse associations only among participants residing in a high-income neighborhood (aORs: 0.45-0.81).Our results suggest that perinatal residential proximity to more greenspace may contribute to a reduced risk of certain birth defects, especially among those living in rural or high-income neighborhoods.

22q11.2 deletion syndrome (22q11) and trisomy 21 (T21) are frequently associated with tetralogy of Fallot (TOF). We hypothesized that there are differences in postoperative length of stay (LOS) and occurrence of postoperative interventions after complete repair of TOF when comparing children with 22q11 to those with T21. Using the Pediatric Health Information System, we performed a retrospective cohort study of patients who underwent complete repair of TOF from 2004 to 2019. Three groups were identified: 22q11, T21, and controls (those without a coded genetic syndrome). Outcomes were postoperative LOS and composite occurrence (yes/no) of at least one postoperative intervention. Bivariate and multivariate comparisons were made among groups; odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using the control group as the comparator. There were 6924 subjects (n = 493 22q11, n = 455 T21, n = 5976 controls). In bivariate analysis, 22q11 was associated with a longer LOS compared to T21 (OR 2.37 [2.16, 2.60] vs. 1.25 [1.12, 1.39], p

Objective Patients with Williams and elastin arteriopathy syndromes often have similar cardiac phenotypes characterized by supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPAS), and coronary artery ostial stenosis (CAOS). SVAS and/or PPAS result in a marked increase in myocardial oxygen demand, whereas CAOS may limit myocardial oxygen supply. This combination predisposes to myocardial ischemic events and sudden cardiac arrest. The purpose of this study was to review our experience with the surgical repair of CAOS in patients with Williams and elastin arteriopathy syndromes. Methods This was a retrospective review of 16 patients with Williams (n = 11) or elastin arteriopathy (n = 5) who underwent surgical repair of CAOS as a concomitant procedure with SVAS and/or PPAS repair. Eleven patients had moderate or severe SVAS, and 10 had PPAS. The median age at surgery was 9 months (range, 3-108 months). Results Seven patients had repair of the left main and right coronary ostia, 6 the left main, and 3 the right coronary ostium. Median duration of aortic crossclamp was 56 minutes and cardiopulmonary bypass time was 454 minutes. The median SVAS gradient decreased from 70 to 12 mm Hg and pulmonary artery pressure decreased from 120 to 30 mm Hg. There was 1 operative mortality (6%). The remaining 15 patients were discharged from the hospital and are alive at a median of 17 months' follow-up. Conclusions The data demonstrate that patients with Williams and elastin arteriopathy syndromes presenting with SVAS and/or PPAS plus CAOS can undergo successful repair of all hemodynamic issues simultaneously.

The value of structured development processes has been recognized and implemented in formal physician training programs such as residencies and fellowships. Physicians are seemingly viewed as a "finished product" upon completing formal training. In recent years, a number of academic medical centers have implemented formalized early-career development programs for physicians, largely those who have a major research focus. However, beyond the early stage of physicians' careers, formalized and intentional physician career development programs are rare. The lack of a philosophy of intentional, career-long individual development at academic medical centers reflects a narrow understanding of the implicit contract between employers and employees. The resulting gap leads the vast majority of physicians to fall short of their potential, further leading to long-term loss for the academic medical centers, their physicians, and society as a whole. Based on the framework of analyze-design-develop-implement-evaluate, the authors propose a robust, iterative model for physician career development that goes beyond skills and knowledge maintenance toward leveraging a broad range of individual capabilities, needs, and contexts along the career lifespan. The model provides a means for harnessing physicians' strengths and passions in concert with the needs of their organization to create greater physician fulfillment and success, which in turn would benefit the patients they care for and the academic medical centers in which they work.

Patients with Williams syndrome (WS) have a 25- to 100-fold higher risk of sudden death and prolonged heart rate-corrected QT (QTc). A recent study using the Fridericia formula for QT correction suggested that prolongation is principally an issue of heart rate. We used multiple published heart rate correction formulas to reevaluate the prevalence of QTc prolongation in our original dataset from our 2010 study at the Children's Hospital of Philadelphia. The ninety-eighth centile for QTc and corrected JT Interval (JTc) of the control population for each formula were used to set the threshold for prolongation. Prevalence comparison was done with Fisher's exact test. Predictors of longer QTc/JTc were assessed using linear regression models adjusting for age, gender, and heart rate. Adjusted odds of QTc/JTc prolongation were evaluated with conditional logistic regression models matched based on age and heart rate. There were 482 electrocardiograms from 188 patients with WS and 1,522 from normal controls. Patients with WS were younger, with higher heart rates and shorter RR and QRS intervals. WS was associated with longer QTc/JTc compared with controls. There were higher odds of prolonged QTc/JTc in patients with WS than controls using both Bazett and Fridericia formulas. In conclusion, this study confirms the higher prevalence of QTc prolongation in WS compared with controls and highlights the importance of setting appropriate formula-specific upper thresholds for QTc prolongation for accurate diagnosis.

We sought to perform a comprehensive review of reported cases of conjoined thoracopagus twins with cardiovascular conjunction to determine the overall survival after separation and factors that might be associated with survival. We performed a systematic review of cases of thoracopagus twins with conjoined cardiovascular systems reported in the English medical literature using Embase, PubMed, Web of Science, and Scopus. We employed standard statistical methods to analyze differences among groups. We identified 102 unique cases of thoracopagus twins (69% female) with some degree of cardiovascular conjunction who had undergone surgical separation. We identified 6 distinct types of cardiovascular union. Median age was 47 days (IQR 12, 120). Survival to at least hospital discharge occurred in 51% (105/204). Median age at separation was lower in emergent (9 days, IQR 1, 25) versus elective cases (93 days, IQR 49, 180) (p

The aim of this article was to synopsize platelet-rich plasma (PRP) use in musculoskeletal pathologies through evidence-based assessment of the preparation, classification, mechanism of action and applications of PRP, thereby answering which PRP type is best for each clinical indication. The literature search was performed using Medline, EMBASE and Cochrane Reviews databases for papers containing the key terms “platelet-rich plasma” AND “orthopaedics” AND (“classification” OR “mechanism of action” OR “preparation” OR “clinical application”). Generated papers were evaluated for pertinence in following areas: preparation, classification, mechanism of action, clinical application within orthopaedics. Non-English papers were excluded. Included studies were evaluated for quality. Sixty studies were included in our review. There are many commercial PRP preparation kits with differing component concentrations. There is no consensus on optimal component concentrations. Multiple PRP classifications exist but none have been validated. Platelet-rich plasma acts via growth factors (GFs) released from α-granules within platelets. Growth factors have been shown to be beneficial in healing. Grossly elevated concentrations of GFs may have inhibitory effects on healing. Multiple systematic reviews show efficacy of PRP in tendinopathies, early osteoarthritis, acute muscle injuries and in combination with rotator cuff repair and anterior cruciate ligament reconstruction. The literature suggests leukocyte-rich PRP (L-PRP) is more beneficial in tendinopathies and pure PRP (P-PRP) is more beneficial in cartilage pathology. However, different PRP preparations have not been directly compared in any pathology. Classification of PRP type is frequently not stated in research. Standardization of PRP research parameters is needed to streamline findings and generate clear indications for PRP types to yield maximum clinical benefit. Cite this article: EFORT Open Rev 2021;6:225-235. DOI: 10.1302/2058-5241.6.200017

Objective. Low-dose nonselective β blockade is an effective treatment for problematic infantile hemangioma (PIH). Screening electrocardiograms (ECG) are performed prior to the initiation of propranolol to minimize the risk of exacerbating undiagnosed heart block. How ECG results affect subsequent propranolol usage and patient management remains unclear. We examined the value of ECG prior to propranolol therapy in a quaternary pediatric hospital. Methods. A retrospective chart review was performed on all infants who received propranolol (2 mg/kg/day divided three times daily) to treat PIH at Arkansas Children’s Hospital from Sept. 2008 to Sept. 2015. All available demographic, historical, and clinical data were obtained. ECGs and echocardiographic data were reviewed and summarized. A pediatric cardiologist read all ECGs. Results. A total of 333 patients (75% female) received propranolol therapy. ECG information was available for 317 (95%). Abnormal findings were present on 44/317 (13.9%) of study ECGs. The most common abnormal finding was “voltage criteria for ventricular hypertrophy” ( n = 35 , 76.1%). Two patients had abnormal rhythms; one had first-degree atrioventricular (AV) block, and one had occasional premature atrial contractions. Of the 31 patients who underwent echocardiograms, 20 (35%) were abnormal. 2.9% of infants with PIH treated with propranolol required a follow-up with a cardiologist. No patient was precluded from taking propranolol due to the findings on screening ECG. Conclusions. Screening ECGs prior to propranolol therapy are abnormal in nearly 14% of patients with PIH but are unlikely to preclude therapy. In the absence of prior cardiac history, this cohort offers further evidence suggesting that screening ECGs may be of limited value in determining the safety of propranolol in otherwise healthy infants with PIH.

Background Williams-Beuren syndrome (WS) is characterized by cardiovascular abnormalities associated with a multigene deletion on 7q11.23, in particular elastin (ELN). Peripheral pulmonary artery stenosis (PPAS) frequently affects pediatric patients with WS. Molecular investigation of WS pulmonary arterial (PA) tissue is limited by tissue scarcity. Methods We compared transcriptomes, tissue architecture, and localized changes in protein expression in PA tissue from patients with WS (n = 8) and donors (n = 5). Results Over 100 genes were differentially expressed at the ≥4-fold level, including genes related to the serotonin signaling pathway: >60-fold downregulation of serotonin transporter SLC6A4 and >3-fold upregulation of serotonin receptor HTR2A. Histologic examination revealed abnormal elastin distribution and smooth muscle cell morphology in WS PA, with markedly shorter, disorganized elastin fibers, and expanded proteoglycan-rich extracellular matrix between muscle layers. Conclusions There were significant abnormalities in the PA expression of genes regulating serotonin signaling, metabolism, and receptors in WS. Those changes were associated with distinct changes in the arterial structure and may play a role in the stenosis-promoting effects of elevated shear stress at PA bifurcations in WS. Impact Serotonin pathway signaling is significantly altered in the pulmonary arteries of patients with Williams syndrome and severe peripheral arterial stenosis. The present study compares the histological and biochemical characteristics of pulmonary arteries from patients with Williams syndrome to those of controls, something that has not, to our knowledge, been done previously. It demonstrates marked abnormalities in the pulmonary arteries of patients with Williams syndrome, especially significant pathologic alterations in the signaling of the serotonin pathway. The findings of this study provide direction for the development of potential therapies to treat pulmonary artery stenosis in patients with Williams syndrome.

Congenital heart disease is exceedingly prevalent in trisomy 13 and 18. Improved survival following congenital heart surgery has been reported, however, mortality remains significantly elevated. Utilizing inpatient data on trisomy 13 and 18 from the 2003-2016 Pediatric Health Information System database, a survival model was developed and validated using data from the California Perinatal Quality Care Collaborative and the California Office of Statewide Health Planning and Development. The study cohort included 1,761 infants with trisomy 13 and 18. Two models predicting survival to 6 months of age were developed and tested. The initial model performed excellently, with a c-statistic of 0.87 and a c-statistic of 0.76 in the validation cohort. After excluding procedures performed on the day of death, the revised model's c-statistic was 0.76. Certain variables, including cardiac surgery, gastrostomy, parenteral nutrition, and mechanical ventilation, are predictive of survival to 6 months of age. This study presents a model, which potentially can inform decision-making regarding congenital heart surgery.

Pericardial effusion (PCE) can be associated with Kawasaki disease (KD). We performed a multicenter, retrospective cohort study of the Pediatric Health Information System of children admitted with KD to determine the association between PCE and adverse outcomes. A total of 17 422 patients were in the cohort, of which 440 (3%) had PCE. PCE was associated with longer hospital length of stay (adjusted odds ratio [aOR] = 1.23; P < .01) and risk of readmission at 30 days (aOR = 1.42; P = .03). Black children were more likely to have a PCE (aOR = 1.54, P < .01) and longer length of stay (aOR = 1.05; P < .001). These data may support delayed discharge in children with PCE and KD in the hopes of preventing readmission. Special consideration needs to be given to how black children with KD are managed.

Background: Digoxin is used in children with heart failure and tachyarrhythmia. Its use in patients with single ventricle anatomy has increased following evidence of improved interstage survival after the Norwood procedure. Digoxin has a narrow therapeutic window and may alter serum potassium balance, inducing arrhythmias. We hypothesized digoxin use in the setting of abnormal serum potassium levels is associated with arrhythmias. Methods: We reviewed all patients ≤18 years who received digoxin while admitted at our institution from 2014-2021. Admissions 6.0 mmol/L.Results: There were 272 encounters in 172 patients. Potassium levels were abnormal in 72.9% of patients who experienced an arrhythmia during digoxin administration, compared to 40.3% who did not (p < 0.001). Odds of arrhythmia was 133% higher in patients with abnormal potassium receiving digoxin (AOR=2.33, 95% CI 1.16 – 4.68, p=0.02). Receiving intravenous digoxin was also associated with a 4.46 odds of cardiac arrhythmia (AOR 4.46, p=0.04, 95% CI 1.07 to 18.50).Conclusion: Odds of arrhythmia is increased during digoxin administration when pediatric patients have abnormal potassium levels. Vigilant attention to potassium levels is essential to prevent adverse outcomes during digoxin therapy.

Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy. This disease is characterized by stenoses at nearly all lobar and segmental ostia and results in systemic-level right ventricular pressures. The current study summarizes our experience with the surgical treatment of PPAS.This was a retrospective review of 145 patients who underwent surgical repair of PPAS. This included 43 patients with Williams syndrome, 39 with Alagille syndrome, and 21 with elastin arteriopathy. Other diagnoses include tetralogy of Fallot with PPAS (n = 21), truncus arteriosus (n = 5), transposition (n = 3), double-outlet right ventricle (n = 2), arterial tortuosity syndrome (n = 3), and other (n = 8).The median preoperative right ventricle to aortic peak systolic pressure ratio was 1.01 (range, 0.50-1.60) which was reduced to 0.30 (range, 0.17-0.60) postoperatively. The median number of ostial repairs was 17 (range, 6-34) and median duration of cardiopulmonary bypass was 398 minutes (range, 92-844). There were 3 in-hospital deaths (2.1%). The median duration of follow-up was 26 months (range, 1-220) with 4 late deaths (2.9%). Eighty-two patients have subsequently undergone catheterization and 74 had a pressure ratio0.50.The surgical treatment of PPAS resulted in a 70% reduction in right ventricular pressures. At 3 years, freedom from death was 94% and 90% of those evaluated maintained low pressures. These results suggest that the surgical treatment of PPAS is highly effective in most patients.

Background Supravalvar aortic stenosis (SVAS) may be an isolated defect of the proximal ascending aorta. However, more severe cases have extension of the arteriopathy into the transverse and proximal descending aorta. The purpose of this study was to review our experience with SVAS with and without aortic arch arteriopathy. Methods This was a retrospective review of 58 patients who underwent surgical repair of SVAS. The median age at repair was 18 months. A total of 37 patients had Williams syndrome. A total of 31 (53%) patients had associated peripheral pulmonary artery stenosis and 23 (39%) had coronary artery ostial stenosis (CAOS). Results A total of 37 of 58 (64%) patients had surgical repair of SVAS without the need for arch intervention while 21 (36%) patients had repair of the distal aortic arch. There were 3 (5.2%) operative deaths, 2 of whom had aortic arch involvement and one without arch involvement. There were 2 deaths after discharge from the hospital. Patients who needed arch surgery were more likely to have severe arch gradients compared to those without arch involvement (71% vs 30%, P < .05), were more likely to undergo concomitant procedures for peripheral pulmonary artery stenosis or CAOS (90% vs 62%, P < .05), and to have Williams syndrome (86% vs 51%, P < .05). Conclusions More than one-third of patients who had SVAS repair at our institution had procedures directed at the transverse or proximal descending aorta. Patients with arch involvement had more severe arch obstruction, required more concomitant procedures, and were more likely to have Williams syndrome.

Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality.This was a retrospective review of 938 consecutive patients undergoing congenital heart surgery at a single institution over a 2-year timeframe. The number of postoperative surgical and unplanned diagnostic procedures were counted and the impact on hospital length of stay and mortality was assessed.581 of the 938 (62%) patients had zero postoperative diagnostic or surgical procedures. These patients had a median length of stay of 6 days with a single operative mortality (0.2%). 357 of the 938 (38%) patients had one or more postoperative diagnostic or surgical procedures. These patients had a total of 1586 postoperative procedures. There was a significant correlation between the number of postoperative procedures and both hospital length of stay and mortality (p .001). Patients who required 10 or more postoperative procedures had a median hospital length of stay of 89 days and had a 50% mortality. There were no survivors in patients who had 15 or more postoperative procedures.The data demonstrate that the number of postoperative procedures was highly correlated with both hospital length of stay and mortality.