From Other Journals: A Review of Recent Articles by Our Editorial Team.

This review is meant to highlight recent publications from other journals that are relevant to pediatric cardiologists. The articles chosen for this edition look at the outcomes of catheter-based interventions for aortic stenosis, the effect of atherosclerotic cardiovascular disease risk factors on the adult congenital heart disease population, the difference in mortality from congenital heart disease between rural and urban America, preoperative NT-proBNP as a predictor of Fontan outcomes, and an overview of the utilization and outcomes of the Rastelli, Nikaidoh, and REV procedures.

Longitudinal Assessment of Cardiac Function Following Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Multisystem inflammatory syndrome in children (MIS-C) after COVID-19 is commonly associated with cardiac involvement. Studies found myocardial dysfunction, as measured by decreased ejection fraction and abnormal strain, to be common early in illness. However, there is limited data on longitudinal cardiac outcomes. We aim to describe the evolution of cardiac findings in pediatric MIS-C from acute illness through at least 2-month follow-up. A retrospective single-center review of 36 patients admitted with MIS-C from April 2020 through September 2021 was performed. Echocardiographic data including cardiac function and global longitudinal strain (GLS) were analyzed at initial presentation, discharge, 2-4-week follow-up, and at least 2-month follow-up. Patients with mild and severe disease, normal and abnormal left ventricular ejection fraction (LVEF), and normal and abnormal GLS at presentation were compared. On presentation, 42% of patients with MIS-C had decreased LVEF < 55%. In patients in whom GLS was obtained (N = 18), 44% were abnormal (GLS < |- 18|%). Of patients with normal LVEF, 22% had abnormal GLS. There were no significant differences in troponin or brain natriuretic peptide between those with normal and abnormal LVEF. In most MIS-C patients with initial LVEF < 55% (90%), LVEF normalized upon discharge. At 2-month follow-up, all patients had normal LVEF with 21% having persistently abnormal GLS. Myocardial systolic dysfunction and abnormal deformation were common findings in MIS-C at presentation. While EF often normalized by 2 months, persistently abnormal GLS was more common, suggesting ongoing subclinical dysfunction. Our study offers an optimistic outlook for recovery in patients with MIS-C and carditis, however ongoing investigation for longitudinal effects is warranted.

Breath-hold and free-breathing quantitative assessment of biventricular volume and function using compressed SENSE: a clinical validation in children and young adults.

BACKGROUND: Although the breath-hold cine balanced steady state free precession (bSSFP) imaging is well established for assessment of biventricular volumes and function, shorter breath-hold times or no breath-holds are beneficial in children and severely ill or sedated patients. METHODS: Clinical cardiovascular magnetic resonance (CMR) examinations from September 2019 to October 2019 that included breath-hold (BH) and free-breathing (FB) cine bSSFP imaging accelerated using compressed sensitivity encoding (C-SENSE) factor of 3 in addition to the clinical standard BH cine bSSFP imaging using SENSE factor of 2 were analyzed retrospectively. Patients with structurally normal hearts who could perform consistent BHs were included. Aortic flow measured by phase contrast acquisition was used as a reference for the left ventricular (LV) stroke volume. Comparative analysis was performed for evaluation of biventricular volumes and function, imaging times, quantitative image quality, and qualitative image scoring. RESULTS: There were 26 patients who underwent all three cine scans during the study period (16.7 ± 6.4 years, body surface area (BSA) 1.6 ± 0.4 m2, heart rate 83 ± 7 beats/min). BH durations of 8 ± 1 s with C-SENSE = 3 were significantly shorter (p < 0.001) by 33% compared to 12 ± 1 s with SENSE = 2. Actual scan time for BH SENSE (4.9 ± 1.2 min) was comparable to that with FB C-SENSE (5.2 ± 1.5 min; p= NS). Biventricular stroke volume and ejection fraction, and LV mass computed using all three sequences were comparable. There was a small but statistically significant (p < 0.05) difference in LV end-diastolic volume (- 3.0 ± 6.8 ml) between BH SENSE and FB C-SENSE. There was a small but statistically significant (p < 0.005) difference in end-diastolic LV (- 5.0 ± 7.7 ml) and RV (- 6.0 ± 8.5 ml) volume and end-systolic LV (- 3.2 ± 4.3 ml) and RV(- 4.2 ± 6.8 ml) volumes between BH C-SENSE and FB C-SENSE. The LV stroke volumes from all three sequences had excellent correlations (r = 0.96, slope = 0.98-1.02) with aortic flow, with overestimation by 2.7 (5%) to 4.6 (8%) ml/beat. The image quality score was Excellent (16 of 26) to Good (10 of 26) with BH SENSE, Excellent (13 of 26) to Good (13 of 26) with BH C-SENSE, and Excellent (3 of 26) to Good (21 of 26) to Adequate (2 of 26) with FB C-SENSE. CONCLUSIONS: Image quality and ventricular volumetric and functional indices using either BH or FB C-SENSE cine bSSFP imaging were comparable to standard BH SENSE cine bSSFP imaging while maintaining nominally identical spatio-temporal resolution. This accelerated image acquisition provides an alternative to accommodate patients with impaired BH capacity.

Conservative gadolinium administration to patients with Duchenne muscular dystrophy: decreasing exposure, cost, and time, without change in medical management.

Cardiac MR (CMR) is increasingly used to assess for cardiac involvement in patients with Duchenne muscular dystrophy (DMD). The frequent use of gadolinium based contrast agents (GBCAs) has been called into question with reports of intracranial gadolinium deposition in patients receiving multiple administrations. We adopted a conservative GBCA administration policy, limiting the frequency of GBCA exposure in patients with previously documented late gadolinium enhancement. The aim of our study was to evaluate the clinical effects of this policy change. Data were retrospectively reviewed on 405 consecutive patients with DMD who underwent CMR evaluation. Patients were grouped into conservative GBCA administration or historical control. CMR reports were evaluated and clinical reports were reviewed to determine actionable changes. Ohio Medicaid reimbursements were used to estimate costs. A total of 187 patients comprised the conservative GBCA group and 218 patients the historical cohort. The conservative GBCA group had lower contrast administration rates (84% vs. 99%, p < 0.0001), shorter scan times (35.2 vs. 39.0 min, p < 0.0001), and lower estimated medical costs ($339 vs. $351/study). There was no change regarding the initial presence of first-time late gadolinium enhancement, and no difference in actionable change. Contrast administration substantially decreased 7 months post-policy change (65%) compared to the initial 7 months (96%, p < 0.0001). In the current era with unclear concern for intracranial gadolinium deposition, thoughtful GBCA administration is warranted in patients anticipated to undergo multiple CMRs. Our updated approach has resulted in fewer patients receiving contrast, shorter scan times, and less medical costs, without appreciable changes to patient management.

Assessment of liver T1 mapping in fontan patients and its correlation with magnetic resonance elastography-derived liver stiffness.

OBJECTIVES: To explore the utility of liver T1 mapping in Fontan patients and its correlation to magnetic resonance elastography (MRE)-derived liver stiffness. BACKGROUND AND AIMS: Liver disease is a major long-term extra cardiac complication in the Fontan population. MRE is frequently used to quantify liver stiffness in Fontan patients; however, it has certain limitations. Native T1 mapping by cardiac magnetic resonance (CMR) is useful in assessment of cardiac fibrosis, but its potential in evaluating liver fibrosis and its correlation to MRE-derived liver stiffness in Fontan patients have not been reported. METHODS: Fontan patients who underwent CMR and MRE were included. Liver Native T1, extracellular volume (ECV) and delta coefficients were measured and correlated with MRE-derived liver stiffness in all Fontan patients. Native liver T1 in Fontan patients were compared to normal controls with biventricular circulation and no known liver disease. RESULTS: A total of 17 Fontan patients and 7 normal controls were included in this study. Fontan patients had significantly higher liver native T1 (690 ± 41 ms vs 620 ± 35 ms; p < 0.001) as compared to controls. There was strong positive correlation between MRE derived liver stiffness and liver native T1 (r = 0.81, p < 0.001). CONCLUSIONS: Liver native T1 was significantly elevated in Fontan patients compared to controls and strongly correlated with MRE-derived liver stiffness. This technique may prove to be a useful noninvasive imaging biomarker for assessing liver fibrosis in the Fontan population.

Prenatal heart block screening in mothers with SSA/SSB autoantibodies: Targeted screening protocol is a cost-effective strategy.

OBJECTIVE: Maternal anti-Ro/SSA and anti-La/SSB antibodies can lead to fetal complete heart block (CHB). Current guidelines recommend weekly echocardiographic screening between 16 and 28 weeks gestation. Given the cost of screening and the rarity of conduction abnormalities in fetuses of mothers with low anti-Ro levels (<50 U/mL), we sought to identify a strategy that optimizes resource utilization. DESIGN: Decision analysis cost-utility modeling was performed for three screening paradigms: "standard screening" (SS) in which mid-gestation mothers are screened weekly, "limited screening" (LS) in which fetal echocardiograms are avoided unless the fetus develops bradycardia, and "targeted screening by maternal antibody level" (TS) in which only high anti-Ro values warrant weekly screening. A systematic review of existing literature and institutional cost data were used to define model inputs. RESULTS: The average cost of LS, TS, and SS was $8566, $11 038, and $23 279, respectively. SS was cost-ineffective with an incremental cost-effectiveness ratio (ICER) of $322 756 while TS was cost-effective with an ICER of $43 445. CONCLUSION: While the efficacy of fetal intervention for first or second degree AV block remains unclear, this analysis supports utilizing antibody levels to stratify this population for optimized surveillance for CHB. SS is cost-ineffective and results in resource overutilization.

Systematic Reviews and Meta-analyses for Cardiology Fellows.

Participating in a scholarly activity is one of the training requirements for cardiology fellows. However, it can be very challenging to complete a research project during such a busy period of clinical training. To help the cardiology fellows in choosing and starting off a research project, a light has been shed on the process of conducting a systematic review, and the importance of this research activity, as well as its limitations.