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Objective: To characterize patterns in the geospatial distribution of pre- and postnatally diagnosed congenital heart disease (CHD) across 6 surgical centers., Study Design: A retrospective, multicenter case series from the Fetal Heart Society identified patients at 6 centers from 2012 through 2016 with prenatally (PrND) or postnatally (PoND) diagnosed hypoplastic left heart syndrome (HLHS) or d-transposition of the great arteries (TGA). Geospatial analysis for clustering was done by the average nearest neighbor (ANN) tool or optimized hot spot tool, depending on spatial unit and data type. Both point location and county case rate per 10 000 live births were assessed for geographic clustering or dispersion., Results: Of the 453 CHD cases, 26% were PoND (n = 117), and 74% were PrND (n = 336). PrND cases, in all but one center, displayed significant geographic clustering by the ANN. Conversely, PoND cases tended toward geographic dispersion. Dispersion of PoND HLHS occurred in 2 centers (ANN = 1.59, P < .001; and 1.47, P = .016), and PoND TGA occurred in 2 centers (ANN = 1.22, P < .05; and ANN = 1.73, P < .001). Hot spot analysis of all CHD cases (TGA and HLHS combined) revealed clustering near areas of high population density and the tertiary surgical center. Hot spot analysis of county-level case rate, accounting for population density, found variable clustering patterns., Conclusion: Geographic dispersion among postnatally detected CHD highlights the need for a wider reach of prenatal cardiac diagnosis tailored to the specific needs of a community. Geospatial analysis can support centers in improving the equitable delivery of prenatal care., Competing Interests: Declaration of Competing Interest American Heart Association (Grant #821139 to JK). The authors declare no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Balloon atrial septostomy (BAS) is an essential catheterization procedure for congenital heart lesions. Recently, a balloon catheter for static BAS was approved for the first time in Japan as an alternative to the conventional pull-through BAS. Despite the expected increase in the use of static BAS, reports on its safety are scarce worldwide., Methods and results: Data on static and pull-through BAS registered in a national registry between 2016 and 2018 were collected. During the study period, 247 sessions of static BAS and 588 sessions of pull-through BAS were performed on a total of 674 patients. Patients who underwent static BAS were older (P<0.001). The incidence of serious adverse events (4.3% vs. 0.9%, P=0.03) and the overall incidence of adverse events (8.1% vs. 3.2%, P=0.03) were higher in static BAS than in pull-through BAS. Among patients who underwent static BAS, the risk factor for adverse events was a body weight <3 kg at the time of the procedure (odds ratio: 4.3 [confidence interval: 1.7-11], P=0.003)., Conclusions: This nationwide study revealed differences in patient background between static and pull-through BAS, as well as a higher incidence of adverse events related to static BAS. Patients weighing <3 kg are at high risk for adverse events after static BAS and may require surgical and circulatory support backup.

Juxtaposition of the atrial appendages is known to occur in specific congenital heart lesions. Recognition of these variants is critical in balloon atrial septostomy and atrial switch operations. There remains little clinical data on the prevalence and associations of these lesions. This is the largest echocardiographic study of juxtaposition of the atrial appendages. This is a retrospective study using the Mt. Sinai echocardiogram database (EchoLAN), which contains echocardiogram reports performed or reviewed at Mt. Sinai Hospital (New York, NY) between 1992 and 2019. Each report was reviewed for associated intracardiac and extracardiac anomalies and the prevalence of juxtaposed atrial appendage among specific diagnosis, including tricuspid atresia, transposition of the great arteries, and double outlet right ventricle, was calculated. Descriptive and analytical statistics were performed as applicable, including Fisher's exact test, with p value < 0.05 considered statistically significant. Forty-nine patients had juxtaposed atrial appendages (1.2/1000 patients): Thirty-eight had LJRAA (0.9 per 1000 patients) and eleven had RJLAA (0.3 per 1000 patients). LJRAA was seen in 22% of tricuspid atresia, 6% of d-TGA, 1% of l-TGA, and 5% of DORV. RJLAA was seen in 2% of DORV, and none of the other lesions. Of associated lesions, dextrocardia, mesocardia, transposition, tricuspid atresia, double inlet left ventricle, and pulmonary valve stenosis were more likely to be present in LJRAA, while bicuspid aortic valve and left ventricular hypoplasia were more likely to be present in RJLAA. This study reaffirms our understanding of juxtaposition of the atrial appendages, its prevalence, and clinical importance., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: The arterial switch operation (ASO) has excellent early outcomes in the modern era. We sought to determine the long-term outcomes in patients who underwent an ASO at a single institution., Methods: Patients who underwent an ASO between 1983 and 2015 were identified from the hospital database and retrospectively reviewed using hospital records., Results: From 1983 to 2015, 844 patients with a biventricular circulation underwent an ASO. There were 28 (3.3%, 28/844) early deaths. Follow-up was available for 94% (729/774) of local patients after hospital discharge. Median follow-up was 15 years (interquartile range, 8-20 years). There were 187 (26%, 187/729) patients with more than 20 years of follow-up and 95 (13%, 95/729) patients with more than 25 years of follow-up. Overall survival was 95% (95% confidence interval [CI], 94%-97%) at 10 and 25 years after the ASO. At 25 years after ASO, freedom from overall reintervention was 77% (95% CI, 73%-81%), freedom from reoperation on the neoaortic root or neoaortic valve was 92% (95% CI, 88%-95%), and freedom from coronary reoperation was 99% (95% CI, 98%-99.7%). Left ventricular (LV) systolic function was normal in 595 of 609 (98%) of patients who had LV function quantified at latest follow-up. Of the 95 patients with more than 25 years of follow-up after ASO, 6 (6.3%) had at least moderate neoaortic valve regurgitation (AR) and 8 (8.4%) had undergone replacement of the neoaortic valve., Conclusions: Overall, survivors of ASO have excellent late survival and normal LV systolic function into adult life. However, AR and reoperation on the neoaortic valve remains an issue for older patients., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

Objective: To determine presence of spatial clustering or dispersion of pre and postnatally detected hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (TGA) cases., Study Design: This retrospective study examined all patients with a prenatal or postnatal diagnosis of HLHS or TGA who had an initial visit or hospitalization at our tertiary care center over a 5-year period from 2012 to 2016 (n = 105). Using geographic information systems software, the nearest neighbor ratio (NNR) tool was used to determine whether statistically significant clustering or dispersion occurred., Results: Geographic clustering was observed among prenatally diagnosed pooled cases of HLHS and TGA and all total cases (NNR = 0.73 and 0.66, respectively), but not postnatally detected cases (NNR = 1.08). Notably, there was significant dispersion of postnatally detected TGA cases (NNR = 1.22) There was no pattern for prenatally detected TGA or HLHS when analyzed individually., Conclusions: The spatial distribution of HLHS and TGA is not random; these conditions occur in geographic clusters. Clustering of all patients in the study population and dispersion of postnatal diagnosis of TGA represent opportunities for improved delivery of fetal cardiac care., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background The aim of this study was to evaluate long-term survival and freedom from coronary artery reintervention after the arterial switch operation (ASO). Methods and Results This single-center nationwide retrospective study included consecutive children who underwent ASO between 1990 and 2016 (n=605). Long-term outcomes were obtained by cross-mapping individual data with the National Death Registry and the National Registry of Cardiovascular Interventions for adults. A control group was randomly retrieved at a 1:10 ratio from the National Birth and Death Registries. Early mortality was 3.3% and late mortality was 1.7% during a median follow-up of 10 (interquartile range, 5-16) years. The probability of overall survival at 20 years after ASO was 94.9% compared with 99.5% in the background population (hazard ratio [HR] 15.6; 95% CI, 8.9-27.5, P <0.001). Independent multivariable predictors of worse survival were an intramural coronary artery (HR, 5.2; 95% CI, 1.8-15.2, P =0.002) and period of ASO 1990 to 1999 (HR, 4.6; 95% CI, 1.5-13.6, P <0.001). Fourteen patients (2.3%) required 16 coronary artery reoperations. Freedom from coronary artery reintervention at 20 years after ASO was 96%. The only independent multivariable predictor associated with a higher hazard for coronary artery reintervention was an intramural coronary artery (HR, 33.9; 95% CI, 11.8-97.5, P <0.001). Conclusions Long-term survival after ASO is excellent. Coronary artery reinterventions are rare. An intramural coronary artery was an independent predictor associated with a higher risk for coronary artery reintervention and death, regardless of the surgical period.

Background: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada., Methods: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers., Results: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND., Conclusions: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Background In the past 3 decades, the arterial switch procedure has replaced the atrial switch procedure as treatment of choice for transposition of the great arteries. Although survival is superior after the arterial switch procedure, data on pregnancy outcomes are scarce and transposition of the great arteries after arterial switch is not yet included in the modified World Health Organization classification of maternal cardiovascular risk. Methods and Results The ROPAC (Registry of Pregnancy and Cardiac disease) is an international prospective registry of pregnant women with cardiac disease, part of the European Society of Cardiology EURObservational Research Programme. Pregnancy outcomes in all women after an arterial switch procedure for transposition of the great arteries are described. The primary end point was a major adverse cardiovascular event, defined as combined end point of maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischemic coronary events, and thromboembolic events. Altogether, 41 pregnant women (mean age, 26.7±3.9 years) were included, and there was no maternal mortality. A major adverse cardiovascular event occurred in 2 women (4.9%): heart failure in one (2.4%) and ventricular tachycardia in another (2.4%). One woman experienced fetal loss, whereas no neonatal mortality was observed. Conclusions Women after an arterial switch procedure for transposition of the great arteries tolerate pregnancy well, with a favorable maternal and fetal outcome. During counseling, most women should be reassured that the risk of pregnancy is low. Classification as modified World Health Organization risk class II seems appropriate.

Objectives: To assess potential risk factors and their effect on the development of transposition of the great arteries (TGA). Methods: A retrospective case-control study of all patients diagnosed with TGA between 1999 to 2016 at King Abdulaziz Medical City, Riyadh, Saudi Arabia. Age and gender-matched controls were selected. Risk factors, including consanguinity, gestational diabetes, family history of congenital heart disease, parental age, and maternal parity, were collected. Regression modeling was used to analyze the effects of risk factors on the development of TGA. Results: A total of 206 patients with transposition of the great arteries were enrolled in the study. Transposition of the great arteries  cases were divided into simple and complex TGA. Selected healthy controls were 446. In the studied cases, consanguinity was found in 95 (46%) of cases, gestational diabetes was diagnosed in 36 (17.5%) mothers, and 35 (17%) had a confirmed family history of congenital heart disease. When risk factors of the cases were compared to the controls, consanguinity, gestational diabetes, maternal age, and parity were found to significantly increase the incidence of TGA. Conclusion: Our study revealed significant risk factors for the development of transposition of great arteries including first degree consanguineous marriages, gestational diabetes, family history of congenital cardiac anomalies, and increasing maternal age and parity. These factors increased the risk by at least 2 folds.

Objective: To examine the association between fetal major heart defects and increased nuchal translucency thickness (NT), tricuspid regurgitation and abnormal flow in the ductus venosus in a large population of singleton pregnancies undergoing routine ultrasound examination at 11-13 weeks' gestation., Methods: This was a retrospective study of prospectively collected data from singleton pregnancies attending for a routine ultrasound scan at 11-13 weeks' gestation, which included examination of fetal anatomy, measurement of NT and assessment of blood flow across the tricuspid valve and in the ductus venosus, according to a standardized protocol. The incidence of fetal NT ≥ 95 th and ≥ 99 th percentiles, tricuspid regurgitation and reversed a-wave in the ductus venosus in fetuses with and those without a major heart defect was determined and the performance of each marker and their combination in the detection of major heart defects was calculated., Results: The study population of 93 209 pregnancies with no apparent chromosomal abnormality included 211 (0.23%) with a fetal major heart defect and 92 998 morphologically normal neonates. In 113 (53.6%) cases with a major heart defect, the diagnosis was made at the 11-13-week scan, in 82 (38.9%) at the 18-24-week scan, in 10 (4.7%) at the third-trimester scan and in six (2.8%) postnatally. At the 11-13-week scan, we diagnosed all cases of tricuspid or pulmonary atresia and polyvalvular dysplasia, > 90% of cases of hypoplastic left heart syndrome or atrioventricular septal defect, about 60% of complex heart defects and cases of left atrial isomerism (interrupted inferior vena cava with normal intracardiac anatomy), 30-40% of cases of tetralogy of Fallot and arch abnormalities, 25% of tricuspid valve abnormalities and about 15% of cases of transposition of the great arteries, but none of aortic or pulmonary stenosis or common arterial trunk. Fetal NT ≥ 95 th or ≥ 99 th percentile, tricuspid regurgitation or abnormal ductus venosus flow was observed in 77 (36.5%), 45 (21.3%), 61 (28.9%) and 58 (27.5%) fetuses with a major heart defect, respectively, and in 5678 (6.1%), 857 (0.9%), 1136 (1.2%) and 1644 (1.8%) of those without a heart defect. Any one of NT ≥ 95 th percentile, tricuspid regurgitation or abnormal flow in the ductus venosus was found in 117 (55.5%; 95% CI, 48.5-62.3%) fetuses with a heart defect and in 8166 (8.8%; 95% CI, 8.6-9.0%) of those without a heart defect. Any one of NT ≥ 99 th percentile or the other two markers was found in 99 (46.9%; 95% CI, 40.0-53.9%) fetuses with a heart defect and in 3517 (3.8%; 95% CI, 3.7-3.9%) of those without a heart defect., Conclusion: At 11-13 weeks' gestation, measurement of fetal NT and assessment of flow across the tricuspid valve and in the ductus venosus can lead to early diagnosis of major heart defect. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.)

Objective: There are scarce data about the prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. The purpose of this study is to provide a multi-institutional description and comparison of the overall prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease., Design: Retrospective multi-institutional study., Setting: The Pediatric Health Information System database., Patients: Neonates with congenital heart disease between 2004 and 2014., Interventions: None., Measurements and Main Results: The primary study measure is the prevalence of necrotizing enterocolitis. Secondary measures include in-hospital mortality, hospital charges, ICU length of stay, hospital length of stay, and 30-day readmission. The prevalence of necrotizing enterocolitis was 3.7% (1,448/38,770) and varied significantly among different congenital heart disease diagnoses. The lowest prevalence of necrotizing enterocolitis was in transposition of the great arteries (n = 104, 2.1%). Compared with transposition of the great arteries, necrotizing enterocolitis occurred more frequently in neonates with hypoplastic left heart syndrome (odds ratio, 2.7; 95% CI, 2.1-3.3), truncus arteriosus (odds ratio, 2.6; 95% CI, 1.9-3.5), common ventricle (odds ratio, 2.1; 95% CI, 1.5-2.8), and aortic arch obstruction (odds ratio, 1.4; 95% CI, 1.1-1.7). Prematurity is a significant risk factor for necrotizing enterocolitis and for mortality in neonates with necrotizing enterocolitis, conferring varying risk by cardiac diagnosis. Unadjusted mortality associated with necrotizing enterocolitis was 24.4% (vs 11.8% in neonates without necrotizing enterocolitis; p < 0.001), and necrotizing enterocolitis increased the adjusted mortality in neonates with transposition of the great arteries (odds ratio, 2.5; 95% CI, 1.5-4.4), aortic arch obstruction (odds ratio, 1.8; 95% CI, 1.3-2.6), and tetralogy of Fallot (odds ratio, 1.6; 95% CI, 1.1-2.4). Necrotizing enterocolitis was associated with increased hospital charges (p < 0.0001), ICU length of stay (p = 0.001), and length of stay (p = 0.001)., Conclusions: The prevalence of necrotizing enterocolitis among neonates with congenital heart disease is 3.7% and is associated with increased in-hospital mortality, length of stay, and hospital charges. The prevalence and associated mortality of necrotizing enterocolitis in congenital heart disease vary among different heart defects.

Objectives: Improved survival has led to interest in functional health status (FHS) as patients with dextro-transposition of the great arteries (d-TGA) transition to adulthood. Our primary objectives were (1) evaluation of The Medical Outcomes Study Short Form-36 Health Survey (SF-36) results; (2) comparison with results of patients who completed the Child Health Questionnaire-Child Form 87 (CHQ-CF87) previously, or the PedsQL Generic Core Scales (PedsQL) survey subsequently; and (3) determination of factors associated with SF-36 domains., Methods: Survivors from the d-TGA Congenital Heart Surgeons' Society cohort (1985-1989) completed the SF-36 (2010) as a measure of FHS (n = 210; age 21-26 years). Patient characteristics, medical history, psychosocial factors, and previous adolescent CHQ-CF87 FHS assessment (2000) were explored for association with SF-36 domains, along with comparison with recent PedsQL data (2017)., Results: Patients scored themselves the same/higher than published normative data in 10 of 10 SF-36 summary scores/domains and similar in 5 of 6 PedsQL summary scores/domains. Factors commonly associated with lower summary scores/domains of the SF-36 were presence of cardiac symptoms, heart condition impacting physical activity/overall health/quality of life, unemployment, and lack of postsecondary education. Less commonly associated factors were lower birth weight, greater total medication number, female sex, shorter procedure-free interval, poor health knowledge, lower family income, younger age at SF-36, living with parents, and being married. These factors accounted for 17% to 47% of the variation in FHS summary scores/domains. FHS was minimally related to d-TGA morphology and repair type., Conclusions: Patients with d-TGA surviving into adulthood, regardless of morphology or repair type, can primarily expect normal FHS. Addressing the challenges of patients with d-TGA entering adulthood requires consideration of psychosocial factors and clinical management., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate whether a nationwide prenatal anomaly screening programme improves detection rates of univentricular heart (UVH) and transposition of great arteries (TGA), and whether maternal risk factors for severe fetal heart disease affect prenatal detection., Design: Population-based cohort study., Setting: Nationwide data from Finnish registries 2004-14., Population: A total of 642 456 parturients and 3449 terminated pregnancies due to severe fetal anomaly., Methods: Prenatal detection rates were calculated in three time periods (prescreening, transition and screening phase). The effect of maternal risk factors (obesity, in vitro fertilisation, pregestational diabetes and smoking) was evaluated., Main Outcome Measures: Change in detection rates and impact of maternal risk factors on screening programme efficacy., Results: In total, 483 cases of UVH and 184 of TGA were detected. The prenatal detection rate of UVH increased from 50.4% to 82.8% and of TGA from 12.3% to 41.0% (P < 0.0001). Maternal risk factors did not affect prenatal detection rate, but detection rate differed substantially by region., Conclusions: A nationwide screening programme improved overall UVH and TGA detection rates, but regional differences were observed. Obesity or other maternal risk factors did not affect the screening programme efficacy. The establishment of structured guidelines and recommendations is essential when implementing the screening programme. In addition, a prospective screening register is highly recommended to ensure high quality of screening., Tweetable Abstract: Implementation of a nationwide prenatal anomaly screening improved detection rates of UVH and TGA., (© 2018 Royal College of Obstetricians and Gynaecologists.)

Background: The aim of this study was to evaluate the incidence and risk factors for the development of right ventricular outflow tract obstruction (RVOTO) after the arterial switch operation (ASO)., Methods: Between 1983 and 2014, a total of 688 patients underwent ASO. RVOTO was defined as any obstruction of the right ventricular outflow tract (RVOT) requiring reintervention., Results: RVOTO developed in 79 patients (11%) at a median time of 3.8 years (range, 1 day-23.6 years) after ASO. Freedom from RVOT reintervention was 96 ± 1, 89 ± 1, and 83 ± 2% at 1, 10, and 25 years, respectively. Independent risk factors for the development of RVOTO in a Cox's regression model were side-by-side great arteries ( p  < 0.001), aortic arch anomalies ( p  < 0.001), use of a pericardial patch for augmentation of the coronary buttons ( p  < 0.001), and a peak gradient more than 20 mm Hg over the RVOT at discharge ( p  < 0.001)., Conclusion: The incidence of RVOTO after ASO is not negligible. Complex morphology, such as side-by-side great arteries and aortic arch anomalies influences the development of RVOTO., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: The physiological repair of the congenitally corrected transposition of the great arteries (ccTGA) has been associated with a long-term risk of the right ventricular dysfunction and tricuspid valve regurgitation. On the other side, the anatomical repair with the restoration of the left ventricle in a systemic position has been hypothesized to improve long-term outcomes. The aim of this study was to determine the results of the anatomical repair., Methods: Between 1990 and 2015, 39 patients underwent an anatomical repair of a ccTGA. The median age was 7 years (range from 8 days to 42.8 years). Eighteen (46%) patients had a double switch. Of these, 21 (54%) patients had an atrial switch with a Rastelli technique (n = 16, 41%), with a réparation à l'étage ventriculaire (REV) procedure (n = 4, 10%) or a truncal rotation (n = 1, 3%). The follow-up was 100% complete at 2 years with a median follow-up of 3.17 years (range 0-26 years)., Results: The early mortality was 10% (n = 4) and the late mortality was 20% (n = 8). The long-term survival was 77 ± 10% at 10 years for the double switch group and 62 ± 11% at 10 years for the Rastelli group (P = 0.25). Eight (20%) patients required reintervention. Freedom from reoperation at 10 years was 82 ± 11% in the double switch group and 88 ± 11% in the Rastelli group. At the last follow-up, 23 (82%) patients were in New York Heart Association (NYHA) functional class I and 2 (7%) patients in NYHA class II., Conclusions: The anatomical repair of the ccTGA is associated with significant early mortality and morbidity. However, the long-term functional status is satisfactory. The anatomical repair of the ccTGA should be reserved for a carefully selected group of patients.

The increasing survival to adulthood of patients with congenital heart disease (CHD) has changed the epidemiology of adult CHD (ACHD) patients and has led to an increment in hospitalization rates due to heart failure (HF). ACHD patients hospitalized for HF have a five-fold higher risk of death than those compensated. HF occurs predominantly in patients with tetralogy of Fallot, single ventricles, and after the Mustard operation for transposition of the great arteries. Diagnostic strategies applied in acquired HF patients are usually used to evaluate ACHD patients, but sometimes this can postpone the identification of HF that can become manifest with unusual and peculiar signs or symptoms. In the same way, therapeutic management resembles the acquired HF one, even if no large randomized clinical trials have been conducted in ACHD patients. Therefore, a close monitoring in dedicated units is mandatory in order to identify in time HF manifestations and manage them adequately.

Objective: We previously identified a pathogenic germline DICER1 variant in a child with transposition of the great arteries who was a member of a family with DICER1 syndrome. In view of a report linking Dicer1 knockout in murine cardiomyocytes to cardiac outflow defects, we investigated the involvement of DICER1 in transposition of the great arteries., Design: We used Fluidigm access array followed by next generation sequencing to screen for variants in the coding exons, their exon/intron boundaries and the 3' untranslated region of DICER1 in patient DNA., Cases: Germline DNA was collected from 129 patients with either sporadic or familial forms of transposition of the great arteries from two sites in Australia and Italy., Results: Most cases (85%) did not have any germline DICER1 variants. In the remaining 15% of cases, we identified 16 previously reported variants (5 synonymous, 6 intronic, and 5 missense) and 2 novel variants (1 intronic and 1 missense). None of the identified variants were predicted to be pathogenic., Conclusions: Here, we report that neither likely pathogenic nor pathogenic variants in DICER1 appear to play a major role in transposition of the great arteries., (© 2018 Wiley Periodicals, Inc.)

Objectives: Dextro-transposition of the great arteries (d-TGA) is one of the most common critical neonatal heart defects, with a low detection rate antenatally. We sought to evaluate trends in the prenatal detection of d-TGA with or without ventricular septal defect (VSD) in Alberta over the past 13 years, examining the potential impact of ultrasound guidelines incorporating screening of cardiac outflow tracts, updated in 2009-2010 and in 2013, and factors affecting detection of the condition., Methods: All fetuses and neonates with d-TGA, with or without VSD, encountered between 2003 and 2015 in the province of Alberta, were identified retrospectively. Clinical records including obstetric ultrasound reports were reviewed. Pregnancy outcome, common referral indications and associated maternal and fetal pathology in affected pregnancies were assessed., Results: From 2003 to 2015, 127 cases with d-TGA were encountered in Alberta, of which 47 (37%) were detected prenatally. Prenatal detection improved over the study period, from 14% in 2003-2010, to 50% in 2011-2013, and to 77% in 2014-2015. Of the 47 fetuses with a prenatal diagnosis of d-TGA, an indication for fetal echocardiography included abnormal or poorly visualized cardiac outflows with normal four-chamber view in 46 (98%). Comorbidities were identified in 12 mothers, only five of which represented an additional reason for fetal echocardiography referral, and four fetuses had extracardiac pathology., Conclusion: Substantial improvement in the prenatal detection of d-TGA has been observed in Alberta over the past few years, owing to improved screening of cardiac outflow tracts on routine obstetric ultrasound examination in otherwise healthy pregnancies, and has been temporally associated with updated obstetric ultrasound guidelines suggesting that these contributed to optimized screening of affected pregnancies. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.)

Objective: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO)., Design: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an "open comment period." Final approval of each QM was provided by a vote of the ACC ACPC Council., Patients: Patients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded., Results: Twelve candidate QMs were generated. Seven metrics passed the RAND-UCLA process. Four passed the "open comment period" and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age., Conclusions: Application of the RAND-UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers., (© 2017 Wiley Periodicals, Inc.)

Objective: To assess the impact of prenatal diagnosis of transposition of the great arteries (TGA) on postnatal outcome., Methods: Hundred and fifty-four patients with either simple (n = 101) or complex forms (n = 53) of TGA, diagnosed prenatally (G1, n = 88) or postnatally (G2, n = 66), who were admitted and underwent surgical correction in our centre between 1998 and 2014, were analysed., Results: Prostaglandin E 1 (PgE 1 ) infusion and balloon atrial septostomy (BAS) were performed in the first 48 h after birth more commonly in G1. The hospital mortality rate for the whole group was 7.1%, higher for complex forms (13.2%) than for simple TGA (3.9%), (p = 0.034). The overall mortality rate was similar in G1 and G2. The mortality for simple TGA was higher when PgE 1 infusion and BAS were implemented after the first 48 h (p = 0.001). All deaths in G2 occurred in patients first receiving PgE 1 and BAS beyond 48 h. PgE 1 was initiated in the first 48 h in most patients (83%) with simple TGA postnatally diagnosed., Conclusions: Adequate measures in the first 48 h after birth are essential to reduce the early mortality in TGA, especially in the simple form. This can be provided by prenatal diagnosis or by early neonatal clinical suspicion and prompt measures.

Background: Coronary anomalies are frequently present in children with transposition of the great arteries (TGA). Such anomalies significantly increase the complexity of arterial switch operations and may have an effect on postoperative outcomes. In this study, we aimed to assess the frequency of coronary anomalies in children with TGA and describe their effect on postoperative outcomes., Methods: All patients with TGA who underwent an arterial switch operation between 1991 and 2015 were consecutively enrolled in this study. Patient coronary patterns were obtained from corresponding operative reports and analyzed to determine their associations with adverse postoperative outcomes., Results: The study included 715 patients with isolated and complex TGA. Coronary anomalies were present in 33.7% of patients and were significantly correlated with the side-by-side configuration of the great arteries. Coronary sinuses with more than 1 ostium were associated with a significantly increased risk of postoperative death (hazard ratio [HR], 2.58), and coronary arteries with a single ostium were associated with an increased risk of postoperative reinterventions (HR, 2.49). In contrast, the trap-door technique of coronary artery transplantation was associated with a reduced risk of reinterventions during postoperative follow-up (HR, 0.40). Complex coronary anomalies were significantly associated with postoperative coronary events (HR, 2.12)., Conclusions: With the exception of patients whose circumflex artery branches off of the right coronary artery, an anomaly that clearly has no effect on postoperative follow-up, patients with unusual coronary patterns are at higher risk for adverse postoperative outcomes than patients with normal coronary anatomy and must be monitored carefully., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The hemi-Mustard and bidirectional Glenn (BDG) procedures combined with the Rastelli procedure have been applied to selected cases of congenitally corrected transposition of the great arteries (ccTGA) for potential benefit over the classic atrial switch procedure. The aim of this study was to analyse our experience with the hemi-Mustard, BDG and Rastelli procedures as an anatomical correction for patients with ccTGA/left ventricular outflow tract obstruction (LVOTO) with positional heart anomalies., Methods: In this retrospective study, 31 consecutive patients with corrected transposition underwent the hemi-Mustard/BDG procedures with the Rastelli operation between 2011 and 2015. The median age was 5.4 (range: 0.75-12) years. Positional anomalies were present in all patients. Eleven patients underwent BDG initially; they then had the second-stage hemi-Mustard and Rastelli procedures; 'one-stage repair' (hemi-Mustard/BDG and the Rastelli procedures) was performed in 20 cases., Results: There were no in-hospital deaths, and 3 patients received a pacemaker. One patient had an atrial baffle obstruction that was observed in the early postoperative period; 7 patients had prolonged pleural effusions that developed more frequently in the one-stage repair group (7/20 vs 0/11, P =  0.033). During the mean follow-up of 3.3 years, 1 late death was noted, and no conduit replacements were required. Twenty-five (83.3%) patients are in New York Heart Association classes I and II at the latest follow-up., Conclusions: Hemi-Mustard, BDG and the Rastelli procedures are technically feasible for correction of ccTGA/left ventricular outflow tract obstruction (LVOTO) and cardiac malposition. Nevertheless, postoperative pleural effusion is the most prevalent complication in the one-stage repair. Two-stage repair may reduce the risk of pleural effusion-related complications., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The atrial switch operations, the Mustard and Senning procedures, performed for dextro-transposition of the great arteries, have largely been supplanted by the arterial switch operation. As such, affected patients will only exist for approximately 30 more years. The main arrhythmias in these patients include sinoatrial node dysfunction, intraatrial reentry tachycardia, and sudden death. Device therapy for these patients is well-established, and catheter ablation for atrial tachycardias is highly efficacious. The application of meticulous procedural planning, customization of catheter courses, and electrophysiologic principles to this patient group may be extended to all postoperative complex congenital heart patients., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined. We defined outcomes utilizing records from 2 centres that were cross-checked with data from a bi-national Fontan Registry for completeness and accuracy., Methods: Two hundred and eleven patients were identified, 59 TA-TGA,152 DILV. Median follow-up was 17 years (range 4 days to 49.8 years). The Kaplan-Meier method was used for all of the time to event analyses and the log-rank test was used to compare the time-to-events. Cox proportional hazard models were used to test the association between potential predictors and time-to-event end-points., Results: TA-TGA had reduced survival compared to DILV (cumulative risk of death 28.8% vs 11%, hazard ratio (HR) 3.1 (95% confidence interval (CI) 1.6-6.1), P  = 0.001). In both groups, SOTO at birth carried a worse prognosis HR 3.54 (1.36-9.2, P  = 0.01). SOTO was not more common in either morphology at birth ( P  = 0.20). Periprocedural mortality accounted for 40% of deaths. Fontan was achieved in 82%, DILV were more likely to achieve Fontan than TA-TGA (91% vs 60%, P <0.001). After Fontan there were 9 deaths (4%) with no difference according to morphology., Conclusions: Patients with TA-TGA have poorer outcomes than those with DILV, affecting survival and likelihood of achieving Fontan. SOTO at birth carries a high risk of mortality suggesting that, when present, initial surgical management should address this., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Little has been published regarding surgery for transposition of the great arteries (TGA) in the developing world., Objectives: This study sought to identify patient characteristics, surgical interventions, institutional characteristics, risk factors for mortality, and outcomes among patients undergoing surgery for TGA in this setting., Methods: Developing world congenital heart surgical programs submitted de-identified data to a novel international collaborative database as part of a quality improvement project. We conducted a retrospective cohort study that included all cases of TGA with intact ventricular septum and TGA with ventricular septal defect performed from 2010 to 2013. Demographic, surgical, and institutional characteristics and their associations with in-hospital mortality were identified., Results: There were 778 TGA operations performed at 26 centers, 480 (62%) for TGA with intact ventricular septum and 298 (38%) for TGA with ventricular septal defect. Most (80%) were single-stage arterial switch operations, but 20% were atrial baffling procedures (atrial switch operation) or 2-stage repairs (pulmonary artery band followed by arterial switch operation). Age at operation was >30 days in one-half of the cases and did not vary significantly with operation type. Survival was 85% and did not significantly vary with age at operation or operation type. Preceding septostomy was infrequently reported (16%) and was not associated with surgical mortality. Mortality was associated with lower World Health Organization weight/body mass index-for-age percentile and lower institutional volume of TGA repair., Conclusions: Surgical repair of TGA performed in the developing world is associated with an early survival of 85%. Type of surgical repair and age at operation varied considerably, but no associations with mortality were identified. In contrast, poor nutrition and small surgical volume were most strongly associated with mortality. Multicenter collaborative quality improvement efforts may benefit patients with TGA in the developing world., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Introduction: Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth., Patients and Methods: We included fetuses diagnosed with dTGA between January 2000 and December 2014. We assessed pre- and postnatal appearance of the foramen ovale, ductus arteriosus and pulmonary veins. Both the diagnostic findings at the time of last prenatal echocardiogram and those findings deriving from a retrospective reevaluation of stored videos were considered. BAS was defined as urgent if performed in neonates with restrictive foramen ovale and severe hypoxemia., Results: We reviewed 40 fetuses with dTGA. 20/40 fetuses received urgent BAS at birth. Not only the restrictive but also the hypermobile and the redundant appearance of the foramen ovale was significantly associated with urgent BAS (p < 0.0001, p = 0.002 and p = 0.0001, respectively)., Conclusions: Prenatal evaluation of the foramen ovale appearance in fetuses with dTGA is still challenging. Based on our experience, also the redundant foramen ovale appearance may need urgent BAS at birth., (© 2016 S. Karger AG, Basel.)

Background: Australian Aboriginal children have increased infant and childhood mortality compared with Caucasian children, but their mortality related to congenital heart defects (CHDs) throughout life is unknown., Methods: We conducted a retrospective cohort study using data on 8,110 live born, singleton infants with CHDs born January 1980 to December 2010 from the Western Australian Register of Developmental Anomalies. Vital status was determined from death and medical records. Data for infants with chromosomal anomalies (except Down syndrome) were excluded. Kaplan-Meier Product-Limit estimates and 95% confidence intervals (CIs) were computed by Aboriginality. Hazard ratios (HRs) and 95% CIs were calculated from multivariable Cox-Proportional Hazard Regression models., Results: Aboriginal children had lower survival than Caucasians for all CHDs combined but most notably during the neonatal period for functional single ventricle (50.0% vs. 86.1%; p = 0.015) and during the postneonatal period for tetralogy of Fallot (87.0% vs. 97.4%; p = 0.021) and atrioventricular septal defect (60.0% vs. 94.6%; p = 0.010). After adjusting for covariates except remoteness and socioeconomic status (SES), Aboriginal children with all CHDs combined (HR = 1.4; 95% CI, 1.0-1.9), with transposition of the great arteries (HR = 4.3; 95% CI, 1.0-18.9) or functional single ventricle (HR = 8.6; 95% CI, 1.3-57.9) had increased risk of mortality compared with Caucasian children. When remoteness and SES were included, the risks were not statistically significant., Conclusion: Long-term survival was lower for Aboriginal children with CHDs, and Aboriginal children with specific CHD phenotypes had increased risk of mortality throughout life. Increased risk may be due to SES and environmental factors. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:1016-1031, 2016. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Background: Transposition of great arteries (TGA) defined as the combination of concordant atrioventricular and discordant ventriculo-arterial connections is one of the most common congenital heart defects. Prenatal diagnosis of TGA remains difficult. To determine the impact of antenatal diagnosis we evaluated the sensitivity of antenatal detection and the neonatal mortality of TGA considering two study periods and two major types of TGA., Methods: A cross-sectional study was performed. Data were collected from a French population-based birth defect registry. From 1988 to 2012, 94 fetuses with TGA were registered. The study period was subdivided into the 1988 to 1999 period and the 2000 to 2012 period. Two types of TGA were considered: isolated TGA (n = 66) and associated TGA (n = 28). A stratified analysis was performed considering the study periods and the types of TGA., Results: Considering the study periods, the sensitivity of prenatal detection of TGA increased significantly (9.8% vs. 51.5%, p = 0.0001). The same trend was found for associated TGA (4.8% vs. 33.3%, p = 0.002) and isolated TGA (21.1% vs. 100%, p < 0.001). A late diagnosis of TGA (7 days after birth) was observed in 13.2% of cases. Neonatal mortality decreased significantly over time for isolated TGA (25.0% vs. 0 p = 0.01). Prenatal diagnosis of both types of TGA did not improve survival., Conclusion: We demonstrated that prenatal diagnosis and neonatal mortality of TGA varied greatly according to the malformation type and the study period. This could be explained by an improvement in terms of medical management., (© 2015 Wiley Periodicals, Inc.)

Objectives: Twenty years ago our institution published an analysis of a cluster of failures associated with the arterial switch operation (ASO). The concept of 'near miss' was explored to detect warning signs of suboptimal performance. The aim of the current study was to review the parameters best suited to monitoring early ASO outcomes in the modern setting and re-examine the concept of near misses as failure equivalents., Methods: All ASOs performed in our institution between 1983 and 2012 were reviewed. The experience was divided into three eras (1983-92, 1993-2002 and 2003-12). The cumulative sum graphic for sequential monitoring was used for early mortality. The need to re-establish cardiopulmonary bypass (CPB), CPB time >240 min and extracorporeal membrane oxygenation (ECMO) were explored as variables of near misses., Results: The cohort consisted of 606 patients. The 30-day mortality rate was 23% (n = 29) in Era 1, 6% (n = 14) in Era 2 and 1% (n = 3) in Era 3. There were further 4, 8 and 6 deaths between 30 and 90 days in the three eras, respectively. In Era 3, the majority of deaths occurred between 30 and 90 days. In the current era, ECMO and CPB time >240 min as a marker of near miss was associated with an increased risk of death both within 30 days and 90 days after ASO., Conclusions: The 30-day outcomes of ASO have significantly improved over the last 30 years. As life-saving mechanical support after surgery has been implemented more often, an extended 90-day window of reporting can offer a more realistic outcome indicator of performance., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Maternal nutritional status has been recognized as a contributor to conotruncal heart defects, but there is limited understanding of the specific nutrition-related factors involved. In this California case-control study of 296 conotruncal cases and 695 nonmalformed controls we explored whether weight loss during early pregnancy was associated with an increased risk of d-transposition of the great arteries (dTGA) and tetralogy of Fallot (TOF) conotruncal defects., Methods: During telephone interviews women were asked whether they were dieting to lose weight or using weight loss remedies during 2 months before or 2 months after conception, and how much weight they gained or lost in the first 2 months of pregnancy or during the year before pregnancy., Results: Odds ratios for dieting to lose weight and use of weight loss remedies for dTGA and TOF were not substantially elevated and all had confidence intervals that included 1.0. Mothers who had a loss of >5 lbs in the first 2 months of pregnancy as well as mothers who lost and gained >5 lbs in the first 2 months of pregnancy also did not show a significant increased risk of delivering case infants when compared with women with no weight change in the year before pregnancy., Conclusion: Given current recommendations about limited weight gain for obese pregnant women, these data indicate that dieting may not substantially increase a fetus' risk of having a conotruncal defect., (© 2015 Wiley Periodicals, Inc.)

Objective: Previous Hungarian intervention trials have shown an association between periconceptional folic-acid-containing multivitamin supplementation and significantly reduced risk of congenital heart defects (CHDs). These findings were confirmed in observational multivitamin studies in the USA, and studies in the Netherlands and China regarding folic acid. The objective of this observational population-based study was to estimate the possible preventive effect of folic acid supplementation for different CHDs during their critical period of development., Study Design: Evaluation of medically recorded use of folic acid (calculated daily average 5.6mg) during the critical period of development of eight types of CHD (verified through autopsy reports or after catheter examination and/or surgical correction) in the population-based Hungarian Case-Control Surveillance of Congenital Abnormalities (HCCSCA), 1980-1996, containing 22,843 cases with congenital abnormalities and 38,151 population controls without any CHDs, including 5395 matched controls of 3567 live-born cases with various CHDs. A conditional logistic regression model was used to estimate the relative risk/protection [odds ratio (OR) with 95% confidence intervals (CI)] of folic acid in the mothers of cases with various types of CHD and their matched controls., Results: There was a significant decrease in the prevalence of cases with ventricular septal defect (OR 0.57, 95% CI 0.45-0.73), tetralogy of Fallot (OR 0.53, 95% CI 0.17-0.94), d-transposition of great arteries (OR 0.47, 95% CI 0.26-0.86) and atrial septal defect secundum (OR 0.63, 95% CI 0.40-0.98) in infants born to mothers who had taken high doses of folic acid during the critical period of CHD development., Conclusions: The risk of development of certain types of CHD was significantly reduced in pregnant women who were supplemented with folic acid. Thus, CHDs should be included as a secondary assessment in neural-tube-defect preventive programs., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: Individuals with L-transposition of the great arteries and two normally sized ventricles are at risk for complete heart block. Little is known about the incidence of complete heart block in those with a single ventricle L-transposition of the great arteries. In this study, we compare the incidence of complete heart block in a modern cohort of patients with L-looped single ventricle anatomy to patients with L-transposition of the great arteries and two ventricles., Methods: We conducted a retrospective cohort study of patients with L-transposition of the great arteries who were seen at Yale-New Haven Hospital between 2001 and 2013. Patients were classified as having isolated L-transposition of the great arteries (group I), L-transposition of the great arteries and major cardiac defects with two-ventricle anatomy (group II), or L-transposition of the great arteries and single ventricle anatomy (group III). We recorded the age of onset and the circumstances of CHB in each group. We calculated the incidence rate of complete heart block and compared this between the groups., Results: We identified 64 patients with L-transposition of the great arteries, median age of 21 years (range 6 months-52 years): 21 in group I, 15 in group II, and 28 in group III. In total, 15 subjects developed complete heart block, incidence of 21.9% and rate of 1.3% per person years. Although group III patients were significantly less likely to develop complete heart block than dual ventricle patients (7.1% vs. 33% and 40%, P = .01), this difference is not significant when only spontaneous complete heart block was analyzed (P = .16)., Conclusion: All patients with L-transposition of the great arteries have similar risk of spontaneous complete heart block and should be routinely screened for this complication., (© 2015 Wiley Periodicals, Inc.)

Background: The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome., Methods and Results: All the 6 surgical centers that performed Mustard and Senning operations in Sweden and Denmark identified all operated TGA patients. Information about death was obtained in late 2007 and early 2008 from the Danish and Swedish Centralised Civil Register by using the patients' unique national Civil Registration Numbers. Four hundred sixty-eight patients undergoing the atrial switch operation were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Perioperative mortality was significantly increased by the presence of a ventricular septal defect, left ventricular outflow obstruction, surgery early in the Mustard and Senning era. However, only pacemaker implantation is predictive of long-term outcome (hazard ratio, 1.90; 95% confidence interval, 1.05-3.46, P=0.04), once the TGA patient has survived the perioperative period. The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed., Conclusions: The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve and not the timing of or the type of surgery in childhood. Cardiac function necessitating the implantation of a pacemaker is associated with an increase in mortality., (© 2015 American Heart Association, Inc.)

Dextro-transposition of the great arteries with intact ventricular septum (d-TGA) is the most frequent cyanotic congenital heart disease in neonates. In newborns affected by d-TGA, the pulmonary and systemic circulations are in parallel instead of being in series. The survival of babies affected by d-TGA is related to the level of mixing at the patent foramen ovale. The diagnosis of d-TGA is an indication for treatment due to scarce survival beyond the neonatal period if not corrected. The current surgical approach is the anatomical correction with the arterial switch operation, which has excellent early and medium-term results. In this review, we discuss treatment of d-TGA and the arterial switch operation, the medium- and long-term results following surgery, and the reasons that have led the arterial switch operation to its popularity compared to the former physiological correction of d-TGA, i.e. the Senning and Mustard procedures.

Factors associated with in utero fetal demise (IUFD) of fetuses that have underlying cardiac pathologies are largely unknown. This case-control study aimed to define the prevalence of IUFD in fetuses with a diagnosis of cardiac pathologies and to identify prenatal predictors of IUFD. Between January 2004 and December 2010, 74 IUFD cases [4.6 %; 95 % confidence interval (CI) 3.7-5.8 %] were identified from 1,584 cases with a diagnosis of structural or functional cardiac lesions in the Hospital for Sick Children database. The cases were divided into right-sided (N = 28), left-sided (N = 23), great artery (N = 8), and miscellaneous (N = 15) groups. The control subjects (1:1 ratio) were fetuses that had cardiac pathology diagnosed within 48 h of the IUFD case. Multivariable regression models were used to determine echocardiographic predictors of IUFD. The prevalence of IUFD was greatest in hypertrophic cardiomyopathy (8/16, 50 %) and Ebstein's anomaly/tricuspid dysplasia (4/15, 27 %) and lowest in transposition of the great arteries (2/85, 1 %). The findings showed IUFD to be associated with hydrops in 17 (23 %) of the 74 cases and arrhythmia in 11 (15 %) of the 74 cases. The factors identified by univariable logistic regression analyses were right ventricular dysfunction [odds ratio (OR) 2.7; p = 0.001], left ventricular dysfunction (OR 1.8; p = 0.007), umbilical vein pulsations (OR 10.9; p = 0.002), and abnormal ductus venosus flow (OR 3.3; p = 0.01). The factors associated with IUFD in multivariable logistic regression models were cardiomegaly (OR 5.6; p = 0.01), hydrops (OR 29.5; p = 0.001), pericardial effusion (OR 4.1; p = 0.06), and extracardiac abnormalities (OR 7.2; p < 0.001). The prevalence of IUFD is greatest in conditions affecting the ventricular myocardium. The onset of IUFD appears to be related initially to right ventricular dysfunction. Closer surveillance is recommended for lesions at risk of IUFD.

Current estimates of the incidence of congenital heart disease (CHD) are derived from small clinical studies and metaanalyses. For the true incidence of CHD in the United States of America to be estimated, a single large representative population must be analyzed. All the data in this study were derived from the Nationwide Inpatient Sample database. The study determined the overall and lesion-specific incidences of CHD diagnoses among all birth hospitalizations in 2008, stratified by race, gender, socioeconomic status, and hospital geographic location. The study identified 13,093 CHD diagnoses among 1,204,887 birth hospitalizations, yielding an incidence of 10.8 per 1,000, with a predominance of mild lesions and septal defects. Atrial septal defect (ASD) and pulmonic stenosis were more common among females, whereas aortic stenosis, coarctation of the aorta, hypoplastic left heart syndrome, and d-transposition of great arteries were more common among males. No racial difference was observed in the overall CHD incidence. However, isolated patent ductus arteriosus (PDA) and ventricular septal defects (VSDs) were more common among Caucasians, whereas ASDs were more common among Hispanics. The incidences of CHD diagnoses were similar for all socioeconomic classes except the lowest socioeconomic class, which had a significantly lower CHD incidence. There was no geographic or seasonal variation in CHD incidence. This study demonstrated the incidence of echocardiographically confirmed CHD diagnosis to be 10.8 per 1,000 live births, marked by a high proportion of mild cardiac lesions and isolated PDAs. The high incidence of isolated PDAs in this study may be explained by the inclusion of only CHD diagnoses during birth hospitalization.

Background and Methods: Systemic right ventricular dysfunction and tricuspid regurgitation (TR) are frequently encountered in patients with congenitally corrected transposition of the great arteries (CCTGA). Studies using echocardiography have suggested a relationship between the degree of TR and systemic right ventricular dysfunction; however, assessment of systemic right ventricular function by echocardiography is limited. Cardiac MRI (CMR) is the gold standard for volumetric assessment of the systemic right ventricle. We performed a retrospective cohort study at our center evaluating all adult patients with CCTGA who underwent a CMR between 1/1999 and 1/2013 to determine the relationship between the degree of TR and systemic right ventricular function., Results: Of the 33 patients identified, 12 had ≤ mild TR (37%), 13 had moderate TR (40%), and 8 had severe TR (24%). Mean age at CMR was 38 years (23-64). Mean right ventricular ejection fraction (45% vs. 41% vs. 42%, p=0.68) and mean indexed right ventricular end diastolic volume (122 ml/m(2) vs. 136 ml/m(2) vs. 138 ml/m(2)p=0.36) were not significantly different for patients with ≤ mild TR, moderate TR or severe TR. The degree of TR was not associated with additional congenital lesions, prior procedures, presence of an intraventricular conduction delay, or decreased left ventricular function., Conclusion: No association between the degree of TR and right ventricular volume or ejection fraction by CMR was identified. Failure to show worsening function or increased volume with greater degrees of TR suggests that the degree of regurgitation alone may not fully explain the heterogeneity in right ventricular size and function., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objective: The objective of this study is to highlight the factors that may affect prenatal diagnosis of transposition of the great arteries (TGA) in order to improve it., Methods: This is a retrospective study performed between 2004 and 2009 in the maternity units from North of France. We identified a total of 68 cases of TGA (isolated or associated with only VSD or coarctation of aorta), of which 32 (47.1%) had prenatal diagnosis (PND+) and 36 did not (PND-). Maternal characteristics and ultrasound factors were studied in relation to PND., Results: Maternal weight and body mass index were significantly higher in the PND- group (70.4 kg and 26.5 kg/m(2) vs 63.6 kg and 23.6 kg/m(2) , respectively). Maternal obesity (body mass index >30) was significantly more frequent in the PND- group (27.8% vs 12.5%). More than a quarter of TGA (28.1%) were diagnosed during the third trimester., Conclusion: Obesity is the main cause of missed PND of TGA. Obese patients with suboptimal prenatal scans may benefit from reassessment of fetal cardiac anatomy and/or from referral for fetal echocardiography., (© 2014 John Wiley & Sons, Ltd.)

Background: The number of women with congenital heart disease reaching reproductive age has increased due to therapeutic advances. The aim of this study was to examine obstetric and cardiac problems during pregnancy after Mustard/Senning repair for transposition of the great arteries., Methods and Results: Sixty pregnancies in 34 women from 3 centers were studied. The women were interviewed, and their records reviewed for clinical status and diagnostic evaluation. Age range was 16-34 years during first pregnancy, and all were in a low functional class. There were 11 miscarriages and 5 abortions. Of 44 successful pregnancies, 20 were vaginal deliveries and 24, cesarean sections. A total of 25% were delivered prematurely. Thirteen babies had birth weight <2,500g. Deterioration in functional class occurred in 7 pregnancies, without recovery in 5. Deterioration in systolic function occurred in 4 of 44 echocardiographically documented pregnancies, without recovery in 75%. In 2 women resuscitation was necessary during delivery, in 1, supraventricular tachycardia occurred during labor., Conclusions: Pregnancy is usually well-tolerated, but outcome is unforeseeable and life-threatening problems can occur. These women belong in cardiac care conducted by experienced congenital cardiologists, who systematically check for typical residua. The pregnancy should be planned and gynecologists/obstetricians with special expertise integrated into the consultations. During delivery a congenital cardiologist, and an anesthetist experienced in congenital cardiology, should be present for possible severe cardiac events.

Background: Targets for catheter ablation of atrial tachyarrhythmias (AT) in post-Mustard procedure patients are often located in the pulmonary venous atrium (PVA). Traditional access to this chamber is retrograde via the aorta. However trans-baffle puncture may be a key determinant of successful ablation in many cases., Methods: All AT ablations performed in patients late after Mustard and Senning operations by a single operator from 2007 to 2012 were reviewed., Results: Nine procedures were identified. In total, 12 ATs were treated, seven persistent, the remainder induced, consisting of counterclockwise cavotricuspid isthmus dependent flutter (5), macroreentrant with isthmus in the systemic venous atrium (SVA) (2), macroreentrant with isthmus in the PVA (1), focal from the PVA (3), and focal from the SVA (1). Ablation within the PVA was required in all procedures to treat AT. Retrograde access in one patient was impossible due to the presence of a Bjork-Shiley tricuspid valve replacement; retrograde access in another two patients was attempted but catheter manipulation was ineffective and AT could not be mapped and ablated. Trans-baffle puncture was performed with transoesophageal echocardiographic guidance in all cases without complications and resulted in successful ablation of AT., Conclusions: Access to the pulmonary venous atrium is essential for successful ablation of AT in many Mustard patients. Trans-baffle puncture remains a relevant technique to modern practice and can be performed safely and effectively., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist., Methods and Results: We studied 145 adult patients (age>16, median 25 years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (>45 mm in 3 patients, maximal-diameter 49 mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7-5.4, P<0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood., Conclusions: Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

The purpose of this review/editorial is to discuss how and when to treat the most common cyanotic congenital heart defects (CHDs); the discussion of acyanotic heart defects was presented in a previous editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. While some patients with acyanotic CHD may not require surgical or transcatheter intervention because of spontaneous resolution of the defect or mildness of the defect, the majority of cyanotic CHD will require intervention, mostly surgical. Total surgical correction is the treatment of choice for tetralogy of Fallot patients although some patients may need to be palliated initially by performing a modified Blalock-Taussig shunt. For transposition of the great arteries, arterial switch (Jatene) procedure is the treatment of choice, although Rastelli procedure is required for patients who have associated ventricular septal defect (VSD) and pulmonary stenosis (PS). Some of these babies may require Prostaglandin E1 infusion and/or balloon atrial septostomy prior to corrective surgery. In tricuspid atresia patients, most babies require palliation at presentation either with a modified Blalock-Taussig shunt or pulmonary artery banding followed later by staged Fontan (bidirectional Glenn followed later by extracardiac conduit Fontan conversion usually with fenestration). Truncus arteriosus babies are treated by closure of VSD along with right ventricle to pulmonary artery conduit; palliative banding of the pulmonary artery is no longer recommended. Total anomalous pulmonary venous connection babies require anastomosis of the common pulmonary vein with the left atrium at presentation. Other defects should also be addressed by staged correction or complete repair depending upon the anatomy/physiology. Feasibility, safety and effectiveness of treatment of cyanotic CHD with currently available medical, transcatheter and surgical methods are well established and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

Objective: To find the prevalence and pattern of congenital heart disease (CHD) at a tertiary care hospital in Uttarakhand, India., Methods: A thorough history and clinical examination was done for all the live births and children up to 18 y of age who presented to the institute over a period of 3 y from July 2008 through June 2011. Those suspected of having a CHD, were further evaluated with ECG, chest radiography and the diagnosis was confirmed by color Doppler echocardiography., Results: Of the 36541 children examined, 312 were identified as having congenital heart defects, thus giving a prevalence of 8.54 per 1000 children attending hospital. Only one fifth cases were diagnosed in neonatal period and the diagnosis was delayed beyond infancy in more than half of the cases. Ventricular septal defect (30.45%), atrial septal defect (17.63%), patent ductus arteriosus (9.62%), pulmonary stenosis (6.41%), tetralogy of Fallot (5.45%) and transposition of great arteries (5.13%) were the commonest defects observed. Only 9.29% of children with CHD underwent definitive treatment., Conclusion: The prevalence of CHD at a tertiary referral hospital in Uttarakhand India is 8.54 per 1000 children. VSD and TOF are the most common acyanotic and cyanotic congenital heart defects respectively.

Aims: The aim of the study was to examine the prevalence and management of outborn babies with a post-natally confirmed diagnosis of transposition of the great arteries (TGA) requiring transport by the Newborn and Paediatric Emergency Transport Service (NETS), New South Wales during the epoch 1991-2010., Method: A retrospective audit of NETS database and case notes. The physiological status, interventions and any complications encountered from the point of referral to NETS (pre-transport), stabilisation (transport) and subsequent admission to the receiving hospital (post-transport) were evaluated., Results: One hundred fifty-seven infants with TGA were transported, with an average of eight per year (1:11,598 births). Seven (4%) had an antenatal diagnosis, and 72 (46%) had a post-natal diagnosis prior to referral. Physiological and clinical parameters demonstrated overall clinical stability; however, 47% of the babies had a PaO(2) <30 mmHg, and approximately one-fifth had oxygen saturations <70%. Rates of mechanical ventilation and prostaglandin E(1) administration were approximately 50%. A quarter of transported babies encountered a transport-related event, including one death and two babies for which a decision was reached to forego life-sustaining treatment at the referring hospital., Conclusions: Most newborns with TGA remain stable or improve during transport. There is a rate of adverse events; however, this reinforces the need to facilitate delivery where there is ready access to interventional paediatric cardiology services., (© 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Juxtaposition of the atrial appendages is a rare congenital cardiac malformation, with the appendages both located on the left or right side of the great arteries. It is usually associated with cyanotic congenital heart disease. The aim of this report is to illustrate the anatomical features of normal and juxtaposed atrial appendages, with a review of the associated anomalies. In the Anatomical Collection of Congenital Heart Disease of the University of Padua, consisting in 1,526 specimens, we found 17 (1.1%) cases of atrial appendages juxtaposition with left juxtaposition in 15 (88%) and right juxtaposition in 2 (12%). Complete form was present in 11 cases and partial form in 6. In left juxtaposition, the situs was solitus in all, and the most frequent anomalies were complete transposition of great arteries in 9 (60%) and tricuspid atresia in 5 (33%); anomalies of position of the heart in the thorax (dextro-mesocardia) were present in 46% of cases, hypoplastic right ventricle in 73%, abnormal relation of the great arteries and subaortic or bilateral infundibulum in all. In right atrial juxtaposition, the atrial situs was solitus with mitral and pulmonary atresia in one case and left isomerism with aortic atresia and double-inlet right ventricle in the other. In describing this malformation, we propose to maintain the use of a positional definition using the terms right and left juxtaposition to describe the presence of both the appendages on the right or on the left side of the great arteries, respectively. The use of a morphological definition should be added in cases of situs inversus or isomerism, with description of the morphology of the appendage located in the wrong position., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Coarctation of the aorta (CoA) is a common congenital defect whose overall incidence is 5-8% of all congenital cardiac anomalies. Associated cardiac anomalies have been well described in previous studies examining specific subgroups of CoA patients, particularly infants and necropsy specimens. The majority of studies, conducted from the 1970s to 1980s, excluded older children, adolescents, and adults. Given the advent of improved surgical and interventional techniques, many CoA patients are surviving into adulthood. This study examined a population of 500 CoA patients in the authors' cardiovascular magnetic resonance imaging (MRI) database involving a population of CoA survivors 5-79 years of age. This was to give a new perspective on the prevalence of associated cardiovascular abnormalities including the bicuspid aortic valve, arch hypoplasia, intracardiac shunts, and subaortic stenosis. These associated abnormalities are less prevalent than in previous studies, reflecting a milder spectrum of CoA. Cardiovascular MRI with its multiplanar imaging capabilities and lack of ionizing radiation is safe and suitable for evaluation and follow-up assessment of CoA patients. Evaluation of CoA by MRI should not be confined to the arch, but should include the heart and mediastinal vessels to assess for the presence and severity of any expected or unexpected associated anomalies.