Background Coarctation of the aorta (CoA) typically requires repair, but re‐interventions and vascular complications occur, particularly with associated defects like bicuspid aortic valve (BAV). Magnetic resonance imaging (MRI) may identify anatomic and hemodynamic factors contributing to clinical complications. Purpose To investigate 4D flow MRI characteristics in pediatric CoA to determine parameters for long‐term clinical surveillance. Study Type Retrospective. Population CoA (n = 21), CoA with BAV (n = 24), BAV alone (n = 29), and healthy control (n = 25). Field Strength/Sequence A 1.5 T, 3D CE IR FLASH MRA, 4D flow MRI using 3D time resolved PC‐MRI with velocity encoding. Assessment Thoracic aorta diameters were measured from 3D CE‐MRA. Peak systolic velocities and wall shear stress were calculated and flow patterns were visualized throughout the thoracic aorta using 4D flow. Repair characteristics, re‐interventions, and need for anti‐hypertensive medications were recorded. Statistics Descriptive statistics, ANOVA with post hoc t‐testing and Bonferroni correction, Kruskal–Wallis H, intraclass correlation coefficient, Fleiss' kappa. Results Patients with CoA with or without repair had smaller transverse arch diameters compared to BAV alone and control cohorts (P < 0.05), higher peak systolic flow velocities and wall shear stress compared to controls in the transverse arch and descending aorta (P < 0.05), and flow derangements in the descending aorta. The most common CoA repairs were extended end‐to‐end anastomosis (n = 22/45, 48.9%, age at repair 1 ± 2 years, seven re‐interventions) and stent/interposition graft placement (n = 10/45, 22.2%, age at repair 12 ± 3 years, one re‐intervention). Anti‐hypertensive medications were prescribed to 33.3% (n = 15/45) of CoA and 34.4% of BAV alone patients (n = 10/29). Data Conclusions Despite repair, CoA alters hemodynamics and flow patterns in the transverse arch and descending aorta. These findings may contribute to vascular remodeling and secondary complications. 4D flow MRI may be valuable in risk stratification, treatment selection and postintervention assessment. Long‐term, prospective studies are warranted to correlate patient and MRI factors with clinical outcomes. Evidence Level 3 Technical Efficacy Stage 3
Background: Early recognition of anthracycline-induced cardiomyopathy may reduce morbidity and mortality in children, but risk stratification tools are lacking. This study evaluates whether electrocardiogram (ECG) changes precede echocardiographic abnormalities in children with anthracycline-induced cardiomyopathy. Methods: We performed a retrospective analysis of 589 pediatric cancer patients who received anthracyclines at a tertiary referral center. ECG endpoints were sum of absolute QRS amplitudes in the 6 limb leads (ΣQRS(6 L)) and corrected QT interval (QTc). Cardiomyopathy was defined by echocardiogram as ejection fraction < 50%, shortening fraction < 26%, or left ventricular end-diastolic diameter z-score > 2.5. Results: Median age at start of therapy was 7.8 years (IQR 3.7-13.6); median follow-up time was 3.6 years (IQR 1.1-5.8). 19.5% of patients met criteria for cardiomyopathy. Male sex, race, older age at first dose, and larger body surface area were associated with development of cardiomyopathy. A 0.6 mV decrease in ΣQRS(6 L) and 10 ms increase in QTc were associated with an increased risk of developing cardiomyopathy with hazard ratios of 1.174 (95% CI = 1.057-1.304, p = 0.003) and 1.098 (95%CI = 1.027-1.173, p = 0.006) respectively. Kaplan-Meier estimates showed a lower chance of cardiomyopathy-free survival for QTc ≥ 440 ms and ΣQRS(6 L) ≤ 3.2 mV over time. After controlling for confounders, total anthracycline dose predicted a decrease in ΣQRS(6 L) and an increase in QTc independent of cardiomyopathy status (p = 0.01 and p < 0.001 respectively). Cardiotoxic radiation did not predict changes in ECG parameters. Cardiomyopathy was associated with increased mortality (34% versus 12%, p < 0.001). Conclusion: In children receiving anthracyclines, decrease in ΣQRS(6 L) and QTc prolongation are associated with increased risk of developing cardiomyopathy. ECG is a potential non-invasive risk stratification tool for prediction of anthracycline-induced cardiomyopathy and requires prospective validation.
A pregnant woman with KCNQ1 variant long QT syndrome (LQTS) underwent fetal magnetocardiography (fMCG) after atrioventricular (AV) block was noted during fetal echocardiogramatypical for LQTS type 1. Concern for fetal LQTS on fMCG prompted monitoring of maternal labs, change of maternal beta blocker therapy, and frequent fetal echocardiograms. Collaboration between obstetricians, neonatologists, and pediatric cardiologists ensured safe delivery. Beta blocker therapy was initiated after birth, and postnatal evaluation confirmed genotype and phenotype positive LQTS in the infant. Our experience suggests diagnosis and evaluation of fetal LQTS can alter antenatal management to reduce risk of poor fetal and postnatal outcomes. K E Y W O R D S fetal bradycardia, fetal echocardiography, fetal magnetocardiography, long QT syndrome 1
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