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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 < 0.0001). Survival to hospital discharge was higher in those who underwent elective separation (70%, 93/132 total children versus 17%, 12/72) (p < 0.0001). Survival was associated with the type of cardiovascular union (p < 0.0001). The separation of thoracopagus twins with cardiovascular conjunction is higher than expected. Increased survival is associated with elective separation, older age, and shared pericardium only. These findings suggest survival could be improved with better delineation of degree of union and delayed separation. [ABSTRACT FROM AUTHOR]

In children with congenital heart defects, Doppler ultrasound is the standard, bedside imaging modality. However, precise characterization of blood flow is challenging due to angle-dependent and one-dimensional velocity estimation. Contrast agent free Vector Flow Imaging is a new ultrasound technology that enables angle-independent visualization of the detailed flow field. Two piglets, one with normal cardiac anatomy and one with congenital heart disease comprised of valvular pulmonary stenosis, a dilated main pulmonary artery, and an incomplete atrioventricular canal defect, were imaged transthoracically and epicardially using a BK Ultrasound bk5000 with built-in vector flow imaging and a 5 MHz linear probe. Subsequently, two children, one with normal cardiac anatomy and one with congenital heart disease comprised of aortic valve stenosis and coarctation of the aorta were imaged transthoracically. Transthoracic two-dimensional echocardiography and vector flow imaging were readily performed in both animals and were limited only by the geometry of the porcine thorax. In addition, transthoracic vector flow imaging was successfully performed in both children, and abnormal flow secondary to cardiac anomalies was visible. Adequate penetration was obtained to a depth of 6.5 cm. Our group has previously demonstrated for the first time that transthoracic vector flow imaging echocardiography is feasible and practicable in pediatric-sized patients, and this paper describes examples of these concepts and in-depth comparisons with traditional imaging modalities. This paper demonstrates that commercially available vector flow imaging technology can be utilized in pediatric cardiac applications as a bedside transthoracic imaging modality, providing advanced detail of blood flow patterns within the cardiac chambers, across valves, and in the great arteries. • Vector Flow Imaging (VFI), a new, ultrasound-based imaging method, provides real-time, angle-independent flow visualization. • VFI is non-invasive and provides advanced hemodynamic flow assessment along with bedside availability and mobility. • This is the first report demonstrating transthoracic VFI is feasible and practicable in pediatric-sized piglets and children. [ABSTRACT FROM AUTHOR]

The article focuses on Williams syndrome (WS) or Williams Beuren syndrome, a congenital and multisystem disorder of the connective tissue, cardiovascular, and central nervous systems. It says that the clinical manifestations of WS were elf-like facial features, ebullient personality or cocktail personality, as well as hypercalcemia. It also highlights the cardiovascular features of WS such as arterial stenosis, supravalvar aortic stenosis (SVAS), and pulmonary arterial stenosis (PAS).

This document is a response to a letter to the editor regarding an article titled "Left Ventricular Dysfunction Following Repair of Ventricular Septal Defects in Infants." The authors of the response appreciate the critique and agree with the primary finding that decreased left ventricular shortening fraction (LVSF) is common following surgical repair of ventricular septal defects (VSD) and normalizes over time. They respectfully disagree with the critique that their results align entirely with a previous study, and instead, they argue that their findings are consistent with other studies that observed a transient depression in left ventricular ejection fraction (LVEF) following VSD closure. The authors acknowledge that the presence of transient left ventricular dysfunction post-operatively may have implications for outcomes such as arrhythmias and neurodevelopmental outcomes, but they hope that their study will help alleviate some concerns and reduce the need for frequent follow-up visits. [Extracted from the article]

Williams syndrome (WS) is a multisystem congenital disorder affecting 1/8000 live births. Our objective was to review our experience with stenosis of the thoracic aorta (STA) in these patients. A retrospective review was undertaken of consecutive WS patients at The Children’s Hospital of Philadelphia from January 1, 1980, through December 31, 2007. WS was diagnosed by an experienced medical geneticist and/or by fluorescence in situ hybridization. Stenosis was diagnosed with either echocardiography or cardiac catheterization. Freedom from intervention was determined using Kaplan–Meier analysis. From a total cohort of 270 patients, 37 (14%) patients with STA were identified and comprised the study group. Age at presentation was 2.1 ± 4.0 years, and follow-up was 11.8 ± 12.6 years (range 0–51). Long-segment STA was more common (89%) than discrete STA. Severity of STA was mild in 18, moderate in 10, and severe in 9 patients. Branch pulmonary artery stenosis was seen in 62% (23 of 37) of STA patients, and supravalvar aortic stenosis was seen in 54% (20 of 37) STA patients. Nine (24%) patients underwent intervention for STA: 8 cases were severe, and 1 case was moderate. Restenosis resulting in reintervention occurred in 5 of 9 (56%) patients, with 4 of 5 (80%) patients undergoing multiple reinterventions. Freedom from intervention was 89, 82, and 73% at 1, 5, and 20 years, respectively. One patient died. STA is common in WS and is generally the long-segment type. In patients with STA, interventions are common and usually occur by 5 years of age. Reintervention for STA occurs frequently. [ABSTRACT FROM AUTHOR]

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. We reviewed all patients ≤ 18 years who received digoxin while admitted at our institution from 2014 to 2021. Admissions < 2 nights were excluded. We compared patients with a hemodynamically significant arrhythmia to those without. We performed adjusted mixed-effects logistic regression with arrhythmia as the outcome variable and potassium status as the predictor variable; adjusting for weight, route of digoxin administration, digoxin indication, serum creatinine, and number of interacting drugs prescribed. Abnormal potassium was defined as serum levels < 3.5 mmol/L or > 6.0 mmol/L. There were 268 encounters in 171 patients. Potassium levels were abnormal in 75.5% of patients who experienced an arrhythmia during digoxin administration, compared to 42.6% who did not (p < 0.001). Odds of arrhythmia was 138% higher in patients with abnormal potassium receiving digoxin (AOR = 2.38, 95% CI 1.07–5.29, p = 0.03). Receiving intravenous digoxin was also associated with a 7.35 odds of cardiac arrhythmia (AOR 7.35, p = 0.006, 95% CI 1.79–30.26). 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. [ABSTRACT FROM AUTHOR]

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 < 0.001), and 22q11 more often underwent postoperative intervention (OR 3.42 [CI 2.56, 4.57] vs. 1.38 [CI 0.91, 2.11]; p < 0.001). In multivariate analysis, 22q11 was also associated with longer LOS (adjusted OR 1.35 [1.26, 1.44] vs. 1.12 [1.04, 1.20]; p < 0.001), but there was no difference in the adjusted odds of postoperative intervention. Children with 22q11 are more likely to experience adverse outcomes after repair of TOF compared to those with T21; the differences are most pronounced for LOS. [ABSTRACT FROM AUTHOR]

In the setting of physician shortages, nurse practitioner (NP) roles have evolved, with increasing independence across most healthcare settings. We sought to characterize referring clinician perceptions of NP-performed outpatient pediatric cardiology consultations. We electronically distributed to pediatric and family medicine physicians and NPs in Arkansas our 11-item survey assessing the acceptability of pediatric cardiology consultations being completed by an NP under varying circumstances. Circumstances included seven common referral indications, and the scale offered five answer choices ranging from "definitely unacceptable" to "definitely acceptable". A total of 292 of 1756 (16.6% response rate) referring clinicians responded to the survey. Overall, 57% of responses indicated that NP-completed pediatric cardiology evaluations were either definitely or probably unacceptable. Acceptability was varied by referral indication and referring clinician characteristics. Unacceptability of NP-completed pediatric cardiology evaluations was greatest among family medicine physicians (81%), pediatricians (66%), and clinicians working in solo or two-physician practices (77%) or community hospitals/clinics (71%). If NP evaluation of a murmur included required review with a cardiologist, the unacceptability rate dropped from 50 to 24% (p < 0.0001). Unacceptability was higher in physicians who do not work with NPs (69%) compared to those who do (60%) (pp < 0.0001). Many referring physicians were willing to send patients ≥ 100 miles to ensure evaluation by a pediatric cardiologist. Most referring physicians find pediatric cardiology evaluations performed by NPs to be unacceptable. Requisite review with a cardiologist improved acceptability of NP evaluations. Many referring physicians would send patients much farther to guarantee evaluation by a cardiologist. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Mitral Valve Diseases in Williams Syndrome: Case Report and Review of the Literature” by Bajracharya et al., which appeared in the September 2011 issue.

A letter to the editor is presented in response to the article "Prevention of Pediatric Graft Coronary Artery Disease: Atorvastatin" in the June 2008 issue.

Aortic complications occur rarely after pediatric orthotopic heart transplantation, but are typically accompanied by catastrophic events. We describe the three cases of major aortic complications in our experience of 329 pediatric heart transplants. This case series and review highlight the important risk factors for aortic complications after heart transplantation. [ABSTRACT FROM AUTHOR]

Heterotaxy is a complex, multisystem disorder associated with single ventricle heart disease and decreased survival. Ciliary dysfunction is common in heterotaxy and other situs abnormalities (H/SA) and may increase post-operative complications. We hypothesized that patients with H/SA have increased respiratory and renal morbidities and increased in-hospital mortality after Fontan procedure. We queried the Pediatric Health Information System database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 through 11 years from 2004 to 2019. H/SA was identified by codes for dextrocardia, situs inversus, asplenia/polysplenia, or atrial isomerism and compared to non-H/SA controls. Outcomes were in-hospital mortality or heart transplantation, ECMO, hemodialysis, length of stay (LOS), and mechanical ventilation or vasoactive medication use ≥ 4 days. We adjusted estimates with multivariable logistic regression. Of 7897 patients at 50 centers, 1366 (17%) met criteria for H/SA. H/SA had worse outcomes for all study measures: death/transplantation (1.9 vs 1.1%, OR 1.74 (95% CI 1.01–3.03); p = 0.047), ECMO (3.7 vs 2.3%, OR 1.74 (1.28–2.35); p < 0.001), hemodialysis (2.1 vs 1.2%, OR 1.66 (1.06–2.59); p = 0.026), prolonged mechanical ventilation (13.2% vs 7.6%, OR 1.85 (1.53–2.25); p < 0.001) and vasoactive medication use (29.4 vs 19.7%, OR 1.65 (1.43–1.90), and longer LOS (11 (8–17) vs 9 (7–14) days; p < 0.001). H/SA is associated with increased cardiovascular, renal, and respiratory morbidity, as well as in-hospital mortality after Fontan procedure. Attention to renal and respiratory needs may improve outcomes in this difficult population. The relationship between ciliary dysfunction and lung and renal morbidity should be explored further. [ABSTRACT FROM AUTHOR]

The aim of this study was to compare the size and geometry of the aorta in patients with 7q11.23 duplication (Dup7) to healthy controls. We retrospectively reviewed all echocardiograms in all patients with Dup7 evaluated at our institutions from June 2017 through September 2019. All standard aortic diameter measurements were made and recorded. Z-scores for the measurements were calculated. For comparison, a set of control echocardiograms was developed by randomly selecting 24 normal echocardiograms in age-matched patients who had undergone echocardiograms for an indication of either chest pain or syncope. In 58 echocardiograms from 21 Dup7 patients, all aortic measurements were increased compared to controls (p < 0.0001). Effacement of the sinotubular junction (STJ) of the aorta was present in all Dup7 patients. Our novel STJ-to-aortic annulus ratio of ≥ 1.15 had a 98.28% sensitivity (95% CI 90.76–99.96) and 100% specificity (95% CI 85.75–100) for distinguishing Dup7 from controls with a positive predictive value of 100% and a negative predictive value of 96.00% (95% CI 77.47–99.41). All patients in our study with Dup7 had echocardiographic evidence of aortopathy. Effacement of the STJ was present in all Dup7 patients. The STJ-to-annulus ratio is a better indicator of aortopathy in Dup7 than the aortic Z-score. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Congenital heart disease (CHD) is common in trisomy 13 (T13) and trisomy 18 (T18), but surgical repair has not been offered in most centers. Data on outcomes of congenital heart surgery (CHS) for T13 and T18 are lacking. We sought to determine the impact of CHS on in-hospital mortality in T13 and T18. METHODS: Data from the 2004 to 2015 Pediatric Health Information System database were used to identify inpatients with T13 or T18 and CHD. Data were restricted to newborns with T13 or T18 admitted at ≤14 days of age. Hospital readmissions were examined to analyze longer-term in-hospital mortality. In-hospital mortality and length of stay were compared between infants with and without CHD and with and without CHS. RESULTS: The study cohort included 1020 infants with T18 and 648 infants with T13. CHD was present in 91% of infants with T18 and 86% of infants with T13. CHS was performed in 7% of each group. In-hospital mortality was decreased in those who underwent CHS (64% lower in T18 [P <.001]; 45% lower in T13 [P = .003]) and remained decreased throughout the 24 months of follow-up. In-hospital mortality was decreased in infants with higher weight, female sex, and older age at admission. CONCLUSIONS: CHS is associated with decreased in-hospital mortality in T18 and T13. These results suggest CHS may be beneficial in select cases. [ABSTRACT FROM AUTHOR]

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. • We estimated associations between greenspace and select birth defects. • We observed inverse associations between more greenspace and various defects. • Associations were observed across multiple organ systems. • Heterogeneity was observed for rural vs. urban and high vs. low income neighborhoods. • Associations were generally stronger among those in rural or high-income areas. [ABSTRACT FROM AUTHOR]

Cardiovascular abnormalities in Williams syndrome (WS) are largely attributable to elastin haploinsufficiency resulting from a large deletion of the elastin-containing region on chromosome 7q11.23. The risk of sudden death in patients withWS is 25- to 100-fold greater than that in the general population. The corrected QT (QTc) interval is prolonged in 14% of patients with WS. Patients with nonsyndromic supravalvar aortic stenosis (NSVAS) have elastin mutations resulting in elastin haploinsufficiency and a vascular phenotype nearly identical to that of WS. No previous studies have evaluated the QTc duration in NSVAS. A retrospective review of all electrocardiograms (ECGs) performed on consecutive patients with NSVAS at Arkansas Children's Hospital from January 1, 1985 to January 1, 2012 was completed.ECGs with nonsinus rhythm or unmeasurable intervals were excluded. TheECGs were read by 1 reader who was unaware of previous readings. A QTc interval of ‡460 ms was defined as prolonged. The NSVAS cohort was compared to previously published WS and control groups using the mixed model for continuous electrocardiographic variables and the generalized estimating equation for binary indicators for prolonged QTc. The generalized estimating equation used bootstrapping with 1,000 replicates. A total of 300 ECGs (median 6, range 1 to 27) from the 35 identified patients with NSVAS met the inclusion criteria. A total of 482ECGs from patients withWSand 1,522ECGs from controls were included. The mean age of the patients with NSVAS at ECG was 7.3 - 6.9 years; 64% were male. The mean QTc duration was 409 - 20 ms in the NSVAS group, 418 - 17 ms in the control group (p <0.001), and 436 - 27 ms in theWSgroup (p <0.001 compared to the control group). The prevalence of QTc prolongation was 0.3% in the NSVAS group, 2.0% in the control group (p <0.001), and 14.8% in the WS group (p <0.001 compared to controls). No patients with NSVAS died. In conclusion, cardiac repolarization is normal in patients with NSVAS. Elastin haploinsufficiency does not appear to be the etiology of QTc prolongation in patients with WS. The possible contribution of other genes on 7q11.23 to QTc prolongation in WS should be investigated. [ABSTRACT FROM AUTHOR]