Fetal echocardiographic features associated with bicuspid aortic valve.

BACKGROUND: Prenatal diagnosis of bicuspid aortic valve is challenging. Bicuspid aortic valve is often associated with aortic dilation. METHODS: Fetuses with postnatally confirmed bicuspid aortic valve were gestational age-matched with normal controls. Complex lesions were excluded. Aortic valve and arch measurements by two blinded investigators were compared. RESULTS: We identified 27 cases and 27 controls. Estimated fetal weight percentile was lower in cases than controls. Seven cases had one or more significant lesions including perimembranous ventricular septal defects (n = 2), isolated annular hypoplasia (n = 2), and/or arch hypoplasia/coarctation (n = 4). Fetuses with bicuspid aortic valves had significantly smaller median z-scores of the aortic annulus (-1.60 versus -0.53, p < 0.001) and root (-1.10 versus -0.53, p = 0.040), and larger ratios of root to annulus (1.32 versus 1.21, p < 0.001), sinotubular junction to annulus (1.07 versus 0.99, p < 0.001), ascending aorta to annulus (1.29 versus 1.18, p < 0.001), and transverse aorta to annulus (1.04 versus 0.96, p = 0.023). Leaflets were "doming" in 11 cases (41%) and 0 controls (p = 0.010), "thickened" in 10 cases (37%) and 0 controls (p = 0.002). We noted similar findings in the subgroup without significant additional cardiac defects. CONCLUSIONS: The appearance of doming or thickened aortic valve leaflets on fetal echocardiogram is associated with bicuspid aortic valve. Compared to controls, fetuses with bicuspid aortic valve had smaller aortic annulus sizes (possibly related to smaller fetal size) without proportionally smaller aortic measurements, resulting in larger aortic dimension to annulus ratios. Despite inherent challenges of diagnosing bicuspid aortic valve prenatally, these findings may increase suspicion and prompt appropriate postnatal follow-up.

Identification of research priorities in CHD: empowering patients and families through participation in the development of formal research agendas.

BACKGROUND: Conquering CHD, formerly known as the Pediatric Congenital Heart Association (PCHA), is the leading congenital heart disease (CHD) patient advocacy organisation in the United States of America, and places high priority on patient engagement in the research process. Participatory design is an approach to problem-solving that utilises the knowledge and opinions of groups of people to generate plans and new ideas. Utilising this mode of patient engagement, patients and families engaged with Conquering CHD assisted in developing a list of research priorities which was then distributed to the larger membership with instructions to rank the priorities in order of importance. Upon completion, these items were compared to the current scientific literature to assess correlation with current publications. This cross-sectional study and literature review aimed to assess the priorities of patients and families in CHD research and to determine the reflection of these areas in the current body of scientific literature. METHODS: This cross-sectional study utilised a survey asking participants to rank the importance of research items within categories including "Technology Advances," "Genetic and Cellular Research," "Broad Understanding of CHD," and "Psychosocial Outcomes" which was distributed through social media and email to 43,168 accounts across all platforms. Respondents were asked to place each item in a ranked order in each category, with the value "1" representing the most preferred for each participant. Anyone engaged with Conquering CHD was eligible to complete the study, including patients and families. Subsequently, a literature review of the largest medical databases including PubMed, Scopus, and ScienceDirect was undertaken to determine the number of articles published per each topic which was then assessed to determine if there is a correlation between patient-ranked priorities and the current body of literature. RESULTS: The study generated a total response of 527 participants. Regarding "Technology Advances," valve replacement was the preferred topic (mean rank 2.07, IQR 2). Stem cell research was the favoured topic in "Genetic and Cellular Research" (mean rank 2.53, IQR 2). Access to care was the priority in the "Broadening Understanding of CHD" (mean rank 1.24, IQR 1). Pertaining to "Psychosocial Outcomes", psychological/emotional effects was the highest ranked topic (mean rank 1.46, IQR 1). The literature review returned a total of 135,672 articles in the areas of interest. For "Valve Replacement", 8361 articles resulted reflecting a proportion of 0.097 of total articles. For "Stem Cell Research", 9921 articles resulted reflecting a proportion of 0.115 of total articles. For "Access to Care", 7845 articles resulted reflecting a proportion of 0.091 of total articles. For "Psychological/Emotional Effects", 6422 articles resulted reflecting a proportion of 0.074 of total articles. A Spearman's correlation demonstrated no correlation between the preferred domain of CHD research and the number of articles published for that domain (rs = 0.02, p = 0.94). CONCLUSIONS: This process demonstrates the effectiveness of participatory design, using a patient and family network to determine the research items of concern to those affected by CHD. The cross-sectional survey was effective in assessing patient and family priorities but was limited by access to reliable internet and delivery only in English. Though the study had a large response rate, it was limited to patients already engaged with Conquering CHD. For these reasons, it may not completely reflect the opinions of the total population affected by CHD. However, this offers valuable insight into patient-determined priorities and reveals that the current scientific literature does not correlate with these items. These data serve to inform individual and institutional research agendas to better reflect the needs and desires of this population.

Multisystem inflammatory syndrome in children and Kawasaki disease: a critical comparison.

Children and adolescents infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are predominantly asymptomatic or have mild symptoms compared with the more severe coronavirus disease 2019 (COVID-19) described in adults. However, SARS-CoV-2 is also associated with a widely reported but poorly understood paediatric systemic vasculitis. This multisystem inflammatory syndrome in children (MIS-C) has features that overlap with myocarditis, toxic-shock syndrome and Kawasaki disease. Current evidence indicates that MIS-C is the result of an exaggerated innate and adaptive immune response, characterized by a cytokine storm, and that it is triggered by prior SARS-CoV-2 exposure. Epidemiological, clinical and immunological differences classify MIS-C as being distinct from Kawasaki disease. Differences include the age range, and the geographical and ethnic distribution of patients. MIS-C is associated with prominent gastrointestinal and cardiovascular system involvement, admission to intensive care unit, neutrophilia, lymphopenia, high levels of IFNγ and low counts of naive CD4+ T cells, with a high proportion of activated memory T cells. Further investigation of MIS-C will continue to enhance our understanding of similar conditions associated with a cytokine storm.

Six Month Follow-up of Patients With Multi-System Inflammatory Syndrome in Children.

BACKGROUND AND OBJECTIVES: Myocardial dysfunction and coronary abnormalities are prominent features of multisystem inflammatory syndrome in children (MIS-C). In this study we aim to evaluate the early and midterm outcomes of MIS-C. METHODS: This is a longitudinal 6-month cohort study of all children admitted and treated for MIS-C from April 17 to June 20, 2020. Patients were followed ∼2 weeks, 8 weeks, and 6 months postadmission, with those with coronary aneurysms evaluated more frequently. RESULTS: Acutely, 31 (62%) patients required intensive care with vasoactive support, 26 (52%) had left ventricular (LV) systolic dysfunction, 16 (32%) had LV diastolic dysfunction, 8 (16%) had coronary aneurysms (z score ≥2.5), and 4 (8%) had coronary dilation (z score <2.5). A total of 48 patients (96%) received immunomodulatory treatment. At 2 weeks, there was persistent mild LV systolic dysfunction in 1 patient, coronary aneurysms in 2, and dilated coronary artery in 1. By 8 weeks through 6 months, all patients returned to functional baseline with normal LV systolic function and resolution of coronary abnormalities. Cardiac MRI performed during recovery in select patients revealed no myocardial edema or fibrosis. Some patients demonstrated persistent diastolic dysfunction at 2 weeks (5, 11%), 8 weeks (4, 9%), and 6 months (1, 4%). CONCLUSIONS: Children with MIS-C treated with immunomodulators have favorable early outcomes with no mortality, normalization of LV systolic function, recovery of coronary abnormalities, and no inflammation or scarring on cardiac MRI. Persistence of diastolic dysfunction is of uncertain significance and indicates need for larger studies to improve understanding of MIS-C. These findings may help guide clinical management, outpatient monitoring, and considerations for sports clearance.

Continuous and intermittent administration of intravenous sildenafil in critically ill infants with pulmonary hypertension.

Intravenous (IV) sildenafil may be administered as a continuous infusion or intermittent bolus dosing in infants with pulmonary hypertension (PH). We aimed to compare these delivery methods. METHODS: We retrospectively evaluated subjects less than 12 months old treated with IV sildenafil for PH. Vital signs, oxygen requirement, vasoactive-inotropic score (VIS), and echocardiogram results before and after sildenafil initiation, and the need for discontinuation due to side effects, were noted. RESULTS: Forty-three subjects were identified (23 continuous, 20 intermittent). There were clinically significant differences in PH classifications between groups. The continuous group was significantly younger (p = 0.010) with higher baseline severity of illness suggested by higher inspired oxygen (FiO2 ) and VIS (p = 0.012). After sildenafil initiation, there were no significant differences in changes in blood pressure, oxygen saturation, FiO2 , or VIS between groups, and no difference in the number of subjects requiring discontinuation due to side effects (4 continuous, 1 intermittent, p = 0.351). Eight continuous group subjects (34.8%) and 3 intermittent group subjects (15.0%) died (p = 0.024), but echocardiographic improvement in PH degree was more common in the continuous group (77.8% vs. 33.3%, p = 0.007). CONCLUSION: In this small cohort of infants treated with continuous or intermittent IV sildenafil, in the setting of different baseline characteristics between groups, there were no significant differences in changes in vital signs, VIS, FiO2 , or need for discontinuation of therapy due to side effects. Higher continuous group mortality may be explained by greater baseline illness severity, but larger prospective, randomized studies are required to investigate these different delivery methods.

Didactic education in paediatric cardiology during the COVID-19 pandemic: a national fellow survey.

The coronavirus disease 2019 (COVID-19) pandemic has had a profound impact on medical educational curricula. We aimed to examine the impact of these unprecedented changes on the formal education of paediatric cardiology fellows through a nationwide survey. A REDCap™-based voluntary anonymous survey was sent to all current paediatric cardiology fellows in the United States of America in May, 2020. Of 143 respondents, 121 were categorical fellows, representing over one-fourth of all categorical paediatric cardiology fellows in the United States of America. Nearly all (140/143, 97.9%) respondents utilised online learning during the pandemic, with 134 (93.7%) reporting an increase in use compared to pre-pandemic. The percentage of respondents reporting curriculum supplementation with outside lectures increased from 11.9 to 88.8% during the pandemic. Respondents considered online learning to be "equally or more effective" than in-person lectures in convenience (133/142, 93.7%), improving fellow attendance (132/142, 93.0%), improving non-fellow attendance (126/143, 88.1%), and meeting individual learning needs (101/143, 70.6%). The pandemic positively affected the lecture curriculum of 83 respondents (58.0%), with 35 (24.5%) reporting no change and 25 (17.5%) reporting a negative effect. A positive effect was most noted by those whose programmes utilised supplemental outside lectures (62.2 versus 25.0%, p = 0.004) and those whose lecture frequency did not decrease (65.1 versus 5.9%, p < 0.001). Restrictions imposed by the COVID-19 pandemic have greatly increased utilisation of online learning platforms by medical training programmes. This survey reveals that an online lecture curriculum, despite inherent obstacles, offers advantages that may mitigate some negative consequences of the pandemic on fellowship education.

Cardiac Dysfunction and Shock in Pediatric Patients With COVID-19.

Coronavirus disease-2019 (COVID-19) has been reported to cause significant morbidity in adults, with reportedly a lesser impact on children. Cardiac dysfunction has only been described in adults thus far. We describe 3 cases of previously healthy children presenting with shock and COVID-19-related cardiac inflammation. (Level of Difficulty: Intermediate.).

Highly Sensitive Blocker Displacement Amplification and Droplet Digital PCR Reveal Low-Level Parental FOXF1 Somatic Mosaicism in Families with Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins.

Detection of low-level somatic mosaicism [alternate allele fraction (AAF) ≤ 10%] in parents of affected individuals with the apparent de novo pathogenic variants enables more accurate estimate of recurrence risk. To date, only a few systematic analyses of low-level parental somatic mosaicism have been performed. Herein, highly sensitive blocker displacement amplification, droplet digital PCR, quantitative PCR, long-range PCR, and array comparative genomic hybridization were applied in families with alveolar capillary dysplasia with misalignment of pulmonary veins. We screened 18 unrelated families with the FOXF1 variant previously determined to be apparent de novo (n = 14), of unknown parental origin (n = 1), or inherited from a parent suspected to be somatic and/or germline mosaic (n = 3). We identified four (22%) families with FOXF1 parental somatic mosaic single-nucleotide variants (n = 3) and copy number variant deletion (n = 1) detected in parental blood samples and an AAF ranging between 0.03% and 19%. In one family, mosaic allele ratio in tissues originating from three germ layers ranged between <0.03% and 0.65%. Because the ratio of parental somatic mosaicism have significant implications for the recurrence risk, this study further implies the importance of a systematic screening of parental samples for low-level and very-low-level (AAF ≤ 1%) somatic mosaicism using methods that are more sensitive than those routinely applied in diagnostics.

Examining the Utility of Coronary Artery Lack of Tapering and Perivascular Brightness in Incomplete Kawasaki Disease.

BACKGROUND: In 2017, the AHA published revised guidelines for the diagnosis of Kawasaki disease (KD). In the absence of compelling data supporting or refuting the utility of lack of tapering (LT) and perivascular brightness (PB), expert panel consensus removed LT and PB from consideration. We hypothesize that LT and PB are unreliable, subjective findings, non-specific to KD, which can be seen in systemic febrile illnesses without KD and in normal controls. METHODS: We performed a single-center retrospective study from 1/2008 to 12/2016. De-identified coronary artery (CA) echocardiographic clips from patients 0-10 years old were interpreted blindly by six pediatric cardiologists. Subjects were grouped as follows: (1) healthy: afebrile with benign murmur, (2) KD: IVIG treatment, 4-5 clinical criteria at presentation, (3) incomplete KD (iKD): IVIG, 1-3 clinical criteria, (4) Febrile: ≥3 days of fever, no IVIG, KD not suspected. The presence or absence of LT and PB was recorded. Inter-rater and intra-rater reliabilities were analyzed using intra-class correlation coefficient, Fleiss' Kappa and Cohen's Kappa coefficients. RESULTS: We interpreted 117 echocardiograms from healthy (27), KD (30), iKD (32), and febrile (28) subjects. Analysis showed moderate agreement in CA z score measurements. LT and PB were observed by most readers in control groups. LT exhibited fair inter-reader agreement (reliability coefficient 0.36) and PB slight inter-reader agreement (reliability coefficient 0.13). Intra-rater reliability was inconsistent for both parameters. CONCLUSIONS: LT and PB are subjective, poorly reproducible features that can be seen in febrile patients without KD and in healthy children.

Comparison of Echocardiographic Diagnostic Criteria of Left Ventricular Noncompaction in a Pediatric Population.

BACKGROUND: There is controversy regarding the best echocardiographic diagnostic criteria for left ventricular noncompaction (LVNC). We assessed the diagnostic utility and reproducibility of the previously proposed echocardiographic diagnostic criteria in a pediatric population using a segmental approach. METHODS: Echocardiograms were matched for patients with and without a clinical diagnosis of LVNC. Blinded reviews of echocardiograms measured (1) depths of intertrabecular recesses (X/Y), (2) noncompaction-to-compaction ratio (NC/C), and (3) number of trabeculations, using a segmental approach. Measurements were analyzed for area under the receiver operating characteristic curves (AUC), sensitivity, and specificity. RESULTS: There were 30 echocardiograms in the initial cohort (15 LVNC cases, 15 controls). Median age was 1.7 years (IQR 0.2-6.9 years) and systolic function was decreased in 40%. Comparison of diagnostic criteria demonstrated the best interrater agreement and AUC with an X/Y ratio measured in end-diastole in the parasternal short axis in the apical anterolateral segment (κ 0.72, CI 0.43-1.00, p value <0.001), yielding 100% sensitivity and 70-86% specificity, among readers. The least predictive and reproducible method was the NC/C ratio. A validation cohort confirmed the superiority of the X/Y ratio, although the interrater agreement and AUC decreased. CONCLUSION: Measurements according to existing LVNC diagnostic criteria vary by echocardiographic view and segment. Modification of the Chin et al. criteria (Circulation 82:507-513, 1990) using an X/Y ratio <0.5 had the greatest interrater reliability and predictive validity when measured in end-diastole in the parasternal short axis in the apical anterolateral segment. The NC/C ratio had the lowest reliability and predictive validity.

Subclinical Decrease in Myocardial Function in Asymptomatic Infants of Diabetic Mothers: A Tissue Doppler Study.

Infants of diabetic mothers (IDMs) with hypertrophic cardiomyopathy are recognized to have impaired myocardial performance, but less is known about ventricular function in IDMs without hypertrophy. We hypothesized that in asymptomatic newborns with normal two-dimensional echocardiographic evaluations, pulsed wave tissue Doppler imaging (TDI) would suggest a subclinical decrease in the cardiac function of IDMs compared to infants of non-diabetics (nIDMs). This is a retrospective cohort study of asymptomatic neonates ≥36 weeks gestation, at 0-7 days of life, with normal standard echocardiograms. Systolic (S'), early diastolic (E'), and late diastolic (A') TDI velocities were measured at the mitral valve (MV) annulus, basal interventricular septum (IVS), and tricuspid valve (TV) annulus, and averaged from three consecutive cardiac cycles. Demographic, perinatal, and echocardiographic variables were compared between IDM and nIDM groups. Of 631 subjects, 75 IDMs were identified. The mean gestational age of the entire cohort was 39.33 weeks (±1.26), birth weight 3.44 kg (±0.56), and body surface area (BSA) 0.21 m2 (±0.02). IDMs had significantly greater birth weight and BSA, lower gestational age, older maternal age, and higher incidence of maternal obesity and hypertension than nIDMs (p < 0.001). On multivariable analysis, IDMs had significantly lower S' (p ≤ 0.03) and E' (p < 0.001) velocities, and higher E/E' ratios (p < 0.001) at the MV, IVS, and TV than nIDMs. In asymptomatic newborn IDMs without cardiac hypertrophy, pulsed wave TDI suggests a subclinical decrease in systolic and diastolic myocardial function compared to nIDMs.

Assessing Myocardial Function in Infants with Pulmonary Hypertension: The Role of Tissue Doppler Imaging and Tricuspid Annular Plane Systolic Excursion.

Transthoracic echocardiography is the most common noninvasive method of evaluating pulmonary hypertension (PH) in infants. Identification of reliable, quantitative indices of myocardial function may enhance the diagnostic value of echocardiography in this population. We hypothesized that pulsed wave tissue Doppler imaging (TDI) and tricuspid annular plane systolic excursion (TAPSE) would be reproducible measurements and would suggest decreased ventricular function, in infants with PH. This retrospective case-control study involved subjects diagnosed clinically and echocardiographically with PH at <12 months of age, matched with controls by age and gestational age (GA). TAPSE was measured by M-mode in the apical 4-chamber view. TDI velocities were averaged from three consecutive cardiac cycles. Observers were blinded to patient identity and clinical status. Fifty-seven subjects, including 35 term or near-term infants ≥35 weeks GA [mean GA 39 weeks (±1.7), median age 1 day (range 0-2)] and 22 preterm infants [median GA 25.4 weeks (24.1-26.6), age 66 days (4-128)], were matched with 57 controls. Subjects with PH had lower TAPSE (term p < 0.001, preterm p = 0.03) and TAPSE indexed to body surface area (term p < 0.001, preterm p = 0.005). Mitral annular, septal, and tricuspid annular systolic (S') and early diastolic (E') TDI velocities were also decreased compared to controls (all p < 0.05). Intraclass correlation demonstrated 84-99% agreement between observers in measuring TDI and 92% for TAPSE. Intraobserver reliability for these measures was 98-99% and 96%, respectively. We concluded that TDI and TAPSE are reproducible indices of myocardial function and may serve as useful adjuncts to standard echocardiographic measures in infants with PH.

Pathogenetics of alveolar capillary dysplasia with misalignment of pulmonary veins.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal lung developmental disorder caused by heterozygous point mutations or genomic deletion copy-number variants (CNVs) of FOXF1 or its upstream enhancer involving fetal lung-expressed long noncoding RNA genes LINC01081 and LINC01082. Using custom-designed array comparative genomic hybridization, Sanger sequencing, whole exome sequencing (WES), and bioinformatic analyses, we studied 22 new unrelated families (20 postnatal and two prenatal) with clinically diagnosed ACDMPV. We describe novel deletion CNVs at the FOXF1 locus in 13 unrelated ACDMPV patients. Together with the previously reported cases, all 31 genomic deletions in 16q24.1, pathogenic for ACDMPV, for which parental origin was determined, arose de novo with 30 of them occurring on the maternally inherited chromosome 16, strongly implicating genomic imprinting of the FOXF1 locus in human lungs. Surprisingly, we have also identified four ACDMPV families with the pathogenic variants in the FOXF1 locus that arose on paternal chromosome 16. Interestingly, a combination of the severe cardiac defects, including hypoplastic left heart, and single umbilical artery were observed only in children with deletion CNVs involving FOXF1 and its upstream enhancer. Our data demonstrate that genomic imprinting at 16q24.1 plays an important role in variable ACDMPV manifestation likely through long-range regulation of FOXF1 expression, and may be also responsible for key phenotypic features of maternal uniparental disomy 16. Moreover, in one family, WES revealed a de novo missense variant in ESRP1, potentially implicating FGF signaling in the etiology of ACDMPV.

Constriction of the ductus arteriosus, severe right ventricular hypertension, and a right ventricular aneurysm in a fetus after maternal use of a topical treatment for striae gravidarum.

Fetal constriction of the ductus arteriosus is a complication of maternal non-steroidal anti-inflammatory drug use and polyphenol-rich food intake. It is unclear as to whether polyphenol-containing topical treatments have similar effects. We present a case of fetal constriction of the ductus arteriosus, severe right ventricular hypertension, and a right ventricular aneurysm associated with maternal use of a topical treatment for striae gravidarum.

Repair of Anomalous Left Coronary Artery From the Right Pulmonary Artery: A Series of Nine Cases.

BACKGROUND: Repair of anomalous left coronary artery from the right pulmonary artery presents a particular technical challenge to the congenital cardiac surgeon. There is disagreement in the literature over the optimal technique for this defect, with some authors advocating for unroofing of the periaortic segment of coronary artery, while others prefer direct aortic reimplantation of the artery. METHODS: We performed a retrospective study examining outcomes of patients who were repaired for this anomaly at our institution. In-hospital and outpatient follow-up data were analyzed. RESULTS: Nine patients were identified. Most patients had poor left ventricular function at the time of surgery. All patients in our series were repaired using the direct coronary transfer technique. To date there were no mortalities among the study participants. At last follow-up, all patients with available echocardiograms had normal ventricular function. One patient required reoperation for anastomotic stenosis. CONCLUSIONS: We demonstrate that using the technique of direct coronary transfer to the aorta, we have achieved excellent results with repair of this defect.

Earlier arterial switch operation improves outcomes and reduces costs for neonates with transposition of the great arteries.

OBJECTIVES: This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries (d-TGA). BACKGROUND: Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates. METHODS: We conducted a retrospective cohort study of infants at ≥36 weeks' gestation, with d-TGA, with or without ventricular septal defects, admitted to our institution at 5 days of age or younger, between January 1, 2003 and October 1, 2012. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the effects of age at operation on major morbidity and hospital reimbursement. RESULTS: A total of 140 infants met inclusion criteria. Reimbursement data were available for them through January 1, 2012 (n = 128). The mortality rate was 1.4% (n = 2). Twenty percent (n = 28) experienced a major morbidity. The median costs were $60,000, in 2012 dollars (range: $25,000 to $549,000). The median age at operation was 5 days (range: 1 to 12 days). For every day later that surgery was performed, beyond day of life 3, the odds of major morbidity increased by 47% (range: 23% to 66%, p < 0.001) and costs increased by 8% (range: 5% to 11%, p < 0.001), after considering the effects of sex, birth weight, gestational age, year at which surgery was performed, transfer, weekend admission, insurance, surgeon, septostomy, bypass and cross-clamp times, and the presence of ventricular septal defects or abnormal coronary anatomy. CONCLUSIONS: Delay of neonatal arterial switch operation beyond 3 days is significantly associated with increased morbidity and healthcare costs.

Primary arterial switch operation as a strategy for total correction of Taussig-Bing anomaly: a 21-year experience.

BACKGROUND: Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the long-term outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly. METHODS AND RESULTS: A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011. Median age at arterial switch operation was 7 (range, 2-192) days, and median operative weight was 3.2 (1.4-6.2) kg. Aortic arch obstruction was present in 30 patients (70%). Hospital mortality was 7% (n=3). Follow-up was available for 37 hospital survivors at a mean of 8.1 (± 6.3) years. Late mortality was 2% (n=1). At follow-up, all patients were in New York Heart Association functional class I. Freedom from transcatheter or surgical reintervention was 73% at 1 year, 64% at 5 years, and 60% at 10 years. Eleven patients underwent 13 catheter reinterventions on the pulmonary arteries (n=8) or aortic arch (n=5). Seven patients underwent 11 reoperations, including relief of right ventricular outflow tract obstruction (n=5), pulmonary arterioplasty (n=3), recoarctation repair (n=2), and tricuspid valve repair (n=1). By multivariate analysis, a preoperative aortic valve annulus z score of ≤-2.5 was associated with reintervention (hazard ratio, 7.66 [95% confidence interval, 1.29-45.6], P=0.03). CONCLUSIONS: Although reintervention is common, primary correction of Taussig-Bing anomaly with arterial switch operation can be achieved in all patients with low mortality and good long-term outcomes.