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Background: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. Methods: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. Results: Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2–9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (− 0.61%/year vs. − 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). Conclusions: Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance. [ABSTRACT FROM AUTHOR]

Seven patients (16%) had nonspecific ST-segments or T-wave abnormalities, 9 patients (20%) had LV hypertrophy, and 2 patients (5%) had a prolonged QTc interval. Two patients (5%) had nonspecific ST-segment or T-wave abnormalities, 7 patients (16%) had LV hypertrophy, and 1 patient had an intraventricular conduction abnormality. Keywords: disease progression; progeria; ventricular dysfunction; left EN disease progression progeria ventricular dysfunction left 1782 1784 3 06/02/23 20230606 NES 230606 Hutchinson-Gilford Progeria syndrome (HGPS) is an ultrarare systemic disorder that causes accelerated aging and premature death, often due to cardiovascular disease.[1],[2] Because of its rarity, the characterization of cardiac abnormalities has been limited to cross-sectional assessment of small cohorts, and data on the progression of these abnormalities are lacking.[3] As a follow-up to our previous cross-sectional report, we evaluated longitudinal changes in cardiac abnormalities in patients with HGPS. [Extracted from the article]

Assessment of the size and function of a functional single ventricle (FSV) is a key element in the management of patients after the Fontan procedure. Measurement variability of ventricular mass, volume, and ejection fraction (EF) among observers by echocardiography and cardiac magnetic resonance imaging (CMR) and their reproducibility among readers in these patients have not been described. From the 546 patients enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study (mean age 11.9 ± 3.4 years), 100 echocardiograms and 50 CMR studies were assessed for measurement reproducibility; 124 subjects with paired studies were selected for comparison between modalities. Interobserver agreement for qualitative grading of ventricular function by echocardiography was modest for left ventricular (LV) morphology (κ = 0.42) and weak for right ventricular (RV) morphology (κ = 0.12). For quantitative assessment, high intraclass correlation coefficients were found for echocardiographic interobserver agreement (LV 0.87 to 0.92, RV 0.82 to 0.85) of systolic and diastolic volumes, respectively. In contrast, intraclass correlation coefficients for LV and RV mass were moderate (LV 0.78, RV 0.72). The corresponding intraclass correlation coefficients by CMR were high (LV 0.96, RV 0.85). Volumes by echocardiography averaged 70% of CMR values. Interobserver reproducibility for the EF was similar for the 2 modalities. Although the absolute mean difference between modalities for the EF was small (<2%), 95% limits of agreement were wide. In conclusion, agreement between observers of qualitative FSV function by echocardiography is modest. Measurements of FSV volume by 2-dimensional echocardiography underestimate CMR measurements, but their reproducibility is high. Echocardiographic and CMR measurements of FSV EF demonstrate similar interobserver reproducibility, whereas measurements of FSV mass and LV diastolic volume are more reproducible by CMR. [Copyright &y& Elsevier]

Background: Right ventricular (RV) late gadolinium enhancement (LGE) occurs due to surgical scarring and RV remodeling, and has been shown to be associated with clinical outcomes in Tetralogy of Fallot (TOF). However, it is not known if cardiovascular magnetic resonance (CMR) LGE extent progresses over time, and therefore, it is not known if serial reassessment of LGE is necessary. We determined the rate of progression in the extent of RV LGE on serial CMR examinations in repaired TOF. Methods: Retrospective review of 127 patients after TOF repair (49% male, median age at first CMR 18.9 years (Interquartile range (IQR) 13.3,27.0) who had at least two CMRs (median follow-up duration of 4.0 years (IQR 2.1,5.9)) was performed. 84/127 patients had no interventions between serial CMRs (Group 1) while 43/127 patients had transcatheter or surgical intervention between CMRs (Group 2). The extent of RV LGE was assessed using 2 methods: a semiquantitative RV LGE score and a quantitative RV LGE extent expressed as % of RV mass. Mixed effects linear regression modeling to estimate changes in LGE over time. Results: RV LGE was present in all patients on the first CMR. % RV LGE extent and LGE score did not increase over time in either patient group. The mean 5 year rates of change were small and negative for both % RV LGE extent [− 2.3 (95% CI − 2.9, − 1.8, p < 0.001) in Group 1, and − 1.9 (95% CI − 3.2, − 0.7, p = 0.004) in Group 2], and RV LGE score [− 0.9 (95% CI − 1.1, − 0.6, p < 0.001) in Group 1, and − 0.5 (95% CI − 1.1, − 0.0, p = 0.047) in Group 2]. Conclusions: In this serial CMR evaluation of children and adults with repaired TOF, no significant progression in the extent of RV LGE was seen on intermediate term follow-up. Given recent concerns regarding the safety of gadolinium-based contrast agents, frequent assessment of LGE may not be necessary in follow-up. [ABSTRACT FROM AUTHOR]

Background: Aortic stiffness has been shown to be abnormal in patients with bicuspid aortic valve (BAV), and is considered a component of the aortopathy associated with this condition. Progressive aortic stiffening associated with aging has been previously described in normal adults. However, it is not known if aging related aortic stiffening occurs at the same rate in BAV patients. We determined the longitudinal rate of decline in segmental distensibility in BAV patients using serial cardiovascular magnetic resonance (CMR) studies, and compared to previously published results from a group of patients with connective tissue disorders (CTD). Methods: A retrospective review of CMR and clinical data on children and adults with BAV (n = 49, 73% male; 23 ± 11 years) with at least two CMRs (total 98 examinations) over a median follow-up of 4.1 years (range 1–9 years) was performed to measure aortic distensibility at the ascending (AAo) and descending aorta (DAo). Longitudinal changes in aortic stiffness were assessed using linear mixed-effects modeling. The comparison group of CTD patients had a similar age and gender profile (n = 50, 64% male; 20.6 ± 12 years). Results: Compared to CTD patients, BAV patients had a more distensible AAo early in life but showed a steeper decline in distensibility on serial examinations [mean 10-year decline in AAo distensibility (× 10−3 mmHg−1) 2.4 in BAV vs 1.3 in CTD, p = 0.005]. In contrast, the DAo was more distensible in BAV patients throughout the age spectrum, and DAo distensibility declined with aging at a rate similar to CTD patients [mean 10 year decline in DAo distensibility (× 10−3 mmHg−1) 0.3 in BAV vs 0.4 in CTD, p = 0.58]. Conclusions: On serial CMR measurements, AAo distensibility declined at significantly steeper rate in BAV patients compared to a comparison group with CTDs, while DAo distensibility declined at similar rates in both groups. These findings offer new mechanistic insights into the differing pathogenesis of the aortopathy seen in BAV and CTD patients. [ABSTRACT FROM AUTHOR]

The ventriculoarterial coupling (VAC) ratio, the ratio of arterial elastance (Ea) to ventricular end-systolic elastance (Ees), reflects cardiovascular efficiency. Little is known about this ratio in patients who have undergone the Fontan procedure. Our aim was to assess the VAC ratio in a cohort of Fontan patients using a cardiac magnetic resonance (CMR) method, and to examine its relation to outcomes. We retrospectively assessed VAC from CMR data on 195 Fontan patients (age 19.6 ± 10.7 years) and 42 controls (age 15.2 ± 2.2 years). The VAC ratio was calculated as Ea/Ees (Ea = mean arterial blood pressure (MBP)/ventricular stroke volume; Ees = MBP/end-systolic volume). Compared with controls, Fontan patients had lower body surface area-adjusted median Ees (1.54 vs. 2.4, p < 0.001) and Ea (1.35 vs. 1.48, p = 0.01), and a higher median VAC ratio (0.88 vs. 0.62, p < 0.001). After a median follow-up of 4 years (range 1-10), 20 patients reached a composite endpoint of death or heart transplant listing. On multivariable modeling, being in the lowest tertile of the VAC ratio was independently associated with the composite endpoint (odds ratio 11.39, p = 0.02), and inclusion of the VAC ratio in the model improved prediction compared to traditional risk factors. In patients without ventricular dilation, the VAC ratio was the only factor predictive of the composite endpoint (p = 0.02). In conclusion, we found evidence for inefficient ventriculoarterial coupling in Fontan patients. The VAC ratio improved prediction of outcomes and was especially useful in patients without ventricular dilation. Further investigation into the clinical significance of ventriculoarterial coupling in this patient population is warranted. [ABSTRACT FROM AUTHOR]

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultrarare disorder of segmental premature aging that is associated with the development of advanced atherosclerosis and significant cardiovascular and cerebrovascular disease. Treatment with lonafarnib has improved survival in patients with HGPS; however, in extended longitudinal follow-up, there has been an increase in the prevalence of rapidly progressive calcific aortic stenosis. The evolving course of HGPS has prompted reconsideration of conservative management and led to the development of strategies for anatomic treatment. In this case report, we describe the anesthetic management of patients with HGPS undergoing surgical management of aortic stenosis with cardiopulmonary bypass., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 International Anesthesia Research Society.)

Background: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF)., Methods: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length., Results: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p < 0.001 for both), but this was not associated with rate of growth. More rapid RVOT enlargement was noted in patients with larger right ventricular end-diastolic volume (RVEDV), higher PR fraction, and greater rates of increases in RVEDV and PR (p < 0.001 for all) CONCLUSIONS: in patients with repaired TOF, using serial CMR data, we found that RVOT size increased progressively at all ages, but the rate was more rapid in the first decade after repair. More rapid RVOT enlargement was noted in patients with a larger RV, more PR, and greater rates of increases in RV size and PR severity. These results may be important in considering timing of referral for transcatheter pulmonary valves, in planning transcatheter and surgical valve replacement, and in designing future valves for the native RVOT., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests. Ashwin Prakash reports a relationship with Moderna Inc that includes: equity or stocks. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision. The handling editor NL declared a past collaboration with the author EA., (© 2024 Gordon, Basso, Maestranzi, Aikawa, Clift, Cammardella, Danesi, del Nido, Edelman, Hamdy, Hegde, Kleinman, Maschietto, Mehra, Mukundan, Musumeci, Russo, Rybicki, Shah, Suarez, Tuminelli, Zaleski, Prakash and Gerhard-Herman.)

Background: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics. The effect of age-related changes in caval inflows on HFD was evaluated using cardiac magnetic resonance data and patient-specific computational fluid dynamics modeling., Methods: SVC and IVC flow from 414 patients with Fontan were collected to establish a relationship between SVC:IVC flow ratio and age. Computational fluid dynamics modeling was performed in 60 (30 extracardiac and 30 lateral tunnel) patient models to quantify the HFD that corresponded to patient ages of 3, 8, and 15 years, respectively., Results: SVC:IVC flow ratio inverted at ≈8 years of age, indicating a clear shift to lower body flow predominance. Our data showed that variation of HFD in response to age-related changes in caval inflows (SVC:IVC, 2, 1, and 0.5 corresponded to ages, 3, 8, and 15+, respectively) was not significant for extracardiac but statistically significant for lateral tunnel cohorts. For all 3 caval inflow ratios, a positive correlation existed between the IVC flow distribution to both the lungs and the HFD. However, as the SVC:IVC ratio changed from 2 to 0.5 (age, 3-15+) years, the correlation's strength decreased from 0.87 to 0.64, due to potential flow perturbation as IVC flow momentum increased., Conclusions: Our analysis provided quantitative insights into the impact of the changing caval inflows on Fontan's long-term HFD, highlighting the importance of SVC:IVC variations over time on Fontan's long-term hemodynamics. These findings broaden our understanding of Fontan hemodynamics and patient outcomes., Competing Interests: Disclosures Dr Govindarajan reports research funding from the American Heart Association (19TPA34860013), the National Heart, Lung, and Blood Institute (NHLBI)/National Institutes of Health (NIH; R01HL161507) and in-kind research support from National Science Foundation (high-performance computing resources from Texas Advanced Computing Center). A. Sahni, E. Eickhoff, and Drs Hoganson, Hammer, and Rathod report research funding from the NHLBI/NIH (R01HL161507). In addition, Dr Govindarajan reports collaborative research funding from Polyvascular Corp, Houston, TX.

Objectives: Optimal aortic sizing during aortic arch reconstruction remains unknown. Negative effects of arch under- or oversizing are well-published. We aimed to characterize longitudinal aortic growth after patch-augmented arch reconstruction to identify the initial reconstructed arch size that results in normal mid-term arch dimensions., Methods: Single-centre, retrospective review of infants undergoing Damus-Kaye-Stansel (DKS) or non-DKS patch-augmented aortic arch reconstruction between 2000 and 2021. Ascending aorta, proximal and distal transverse arch, aortic isthmus (AIsth) and descending aorta dimensions were measured in postoperative echocardiograms (<3 months from index operation) and cross-sectional imaging (>12 months). Longitudinal changes to aortic dimensions and z-scores were analysed. Secondary outcomes included reintervention, valve and ventricular function, mortality and transplantation., Results: Fifty-four patients (16 DKS, 38 non-DKS) were included. At 6.3 [2.2, 12.0]-year follow-up, all aortic segments grew significantly in both groups, while z-scores remained unchanged except for non-DKS proximal and distal transverse arch z-scores, which significantly increased (P < 0.05 each). When stratified by initial postoperative z-score (z < -1, -1 ≤ z ≤ 1, z > 1), non-DKS patients with initial AIsth z-score <-1 had a final z-score significantly smaller than both the targeted z-score zero (P = 0.014) and final z-score in a group with initial postoperative z-score ±1 (P = 0.009). Valve and ventricular function remained stable. Eighteen patients required reintervention, 1 died and 1 underwent transplant., Conclusions: Over mid-term follow-up, aortic growth after arch reconstruction with patch augmentation was proportional when repaired to normal z-score dimensions, aside from proximal transverse arch, which disproportionately dilated. AIsth undersizing prevailed mid-term and trended towards a higher reintervention rate. Initial reconstruction between z-score 0 and +1 resulted in maintenance of that z-score size at mid-term follow-up. Overall, it is crucial to achieve targeted aortic sizing at index operation to maintain appropriate aortic dimensions over time and reduce reintervention risk with specific focus on the AIsth., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied., Objective: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology., Methods: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics., Results: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads., Conclusion: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

COVID-19 has impacted the healthcare system across the globe. The study will span three pandemic waves in 2020, 2021, and 2022. The goal is to learn how the pandemic affects antenatal care (ANC) and emergency delivery care for pregnant women in Tamil Nadu, India, and how medical services respond. The study employs counterfactual analysis to evaluate the causal impact of the pandemic. A feedforward in combination with a simple auto-regressive neural network (AR-Net) is used to predict the daily number of calls for ambulance services (CAS). Three categories of the daily CAS count between January 2016 and December 2022 are utilised. The total CAS includes all types of medical emergencies; the second group pertains to planned ANC for high-risk pregnant women and the third group comprises CAS from pregnant women for medical emergencies. The second wave's infection and mortality rates were up to six times higher than the first. The phases in wave-II, post-wave-II, wave-III, and post-wave-III experienced a significant increase in both total IFT (inter-facility transfer) and total non-IFT calls covering all emergencies relative to the counterfactual, as evidenced by reported effect sizes of 1 and a range of 0.65 to 0.85, respectively. This highlights overwhelmed health services. In Tamil Nadu, neither emergency prenatal care nor planned prenatal care was affected by the pandemic. In contrast, the increase in actual emergency-related IFT calls during wave-II, post-wave-II, wave-III, and post-wave-III was 62%, 160%, 141%, and 165%, respectively, relative to the counterfactual. During the same time periods, the mean daily CAS related to prenatal care increased by 47%, 51%, 38%, and 38%, respectively, compared to pre-pandemic levels. The expansion of ambulance services and increased awareness of these services during wave II and the ensuing phases of Covid-19 pandemic have enhanced emergency care delivery for all, including obstetric and neonatal cohorts., Competing Interests: The authors have declared that no competing interests exist, (Copyright: © 2023 Paramasivan et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Cardiovascular computed tomography (CCT) is rated appropriate by published guidelines for the initial evaluation and follow up of congenital heart disease (CHD) and is an essential modality in cardiac imaging programs for patients of all ages. However, no recommended core competencies exist to guide CCT in CHD imaging training pathways, curricula development, or establishment of a more formal educational platform. To fill this gap, a group of experienced congenital cardiac imagers, intentionally inclusive of adult and pediatric cardiologists and radiologists, was formed to propose core competencies fundamental to the expert-level performance of CCT in pediatric acquired and congenital heart disease and adult CHD. The 2020 SCCT Guideline for Training Cardiology and Radiology Trainees as Independent Practitioners (Level II) and Advanced Practitioners (Level III) in Cardiovascular Computed Tomography (1) for adult imaging were used as a framework to define pediatric and CHD-specific competencies. Established competencies will be immediately relevant for advanced cardiac imaging fellowships in both cardiology and radiology training pathways. Proposed future steps include radiology and cardiology society collaboration to establish provider certification levels, training case-volume recommendations, and continuing medical education (CME) requirements for expert-level performance of CCT in pediatric and adult CHD., Competing Interests: Declaration of competing interest There are no relevant financial disclosures or conflict of interest for any author related to the content of this manuscript., (Copyright © 2023 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Background: Coarctation of the aorta (COA) is associated with reduced aortic distensibility and systemic hypertension (HTN). 60-85% of COA patients have a bicuspid aortic valve (BAV). It is not known if the presence of a BAV accentuates the aortopathy and HTN in CoA patients. We examined whether patients with COA and a BAV had lower aortic distensibility by CMR, and a higher prevalence of systemic HTN compared with COA patients with a tricuspid aortic valve (TAV)., Methods: In successfully repaired COA patients excluding those with residual COA, ascending aorta (AAO) and descending aorta (DAO) distensibility was calculated by CMR. HTN was assessed using standard pediatric and adult criteria., Results: Among 215 COA patients (median age 25.3 years), 67% had a BAV, and 33% had a TAV. Median AAO distensibility z-score was lower in the BAV group (- 1.2 versus - 0.7; p = 0.014) but DAO distensibility was similar in BAV and TAV patients. HTN prevalence was similar in BAV (32%) and TAV groups (36%, p = 0.56). On multivariable analysis controlling for confounders, HTN was not associated with BAV but was associated with male gender (p = 0.003) and older age at follow-up (p = 0.004)., Conclusions: In young adults with treated COA, those with a BAV had stiffer AAO compared to those with a TAV, but DAO stiffness was similar. HTN was not related to BAV. These results suggest that although the presence of a BAV in COA exacerbates the AAO aortopathy, it does not exacerbate the more generalized vascular dysfunction and associated HTN., (© 2023. The Author(s).)

Double aortic arch associated with atresia of the left arch proximal to the left common carotid artery has been considered a theoretical possibility. To our knowledge, we report the first patient with this anatomy confirmed by surgical observation., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Myocarditis is a rare complication of the COVID-19 mRNA vaccine. We previously reported a case series of 15 adolescents with vaccine-associated myocarditis, 87% of whom had abnormalities on initial cardiac magnetic resonance (CMR), including late gadolinium enhancement (LGE) in 80%. We performed follow-up CMRs to determine the trajectory of myocardial recovery and better understand the natural history of vaccine-associated myocarditis. Case series of patients age < 19 years admitted to Boston Children's Hospital with acute vaccine-associated myocarditis following the BNT162b2 vaccine who had abnormal CMR at the time of initial presentation, and underwent follow-up testing. CMR assessment included left ventricular (LV) ejection fraction, T2-weighted myocardial imaging, LV global native T1, LV global T2, extracellular volume (ECV), and late gadolinium enhancement (LGE). Ten patients (9 male, median age 15 years) with vaccine-associated myocarditis underwent follow-up CMR at a median of 92 days (range 76-119) after hospital discharge. LGE was persistent in 80% of patients, though improved from prior in all cases. Two patients (20%) had abnormal LV global T1 at presentation, which normalized on follow-up. ECV decreased between acute presentation and follow-up in 6/10 patients; it remained elevated at follow-up in 1 patient and borderline in 3 patients., Conclusion: CMR performed ~3 months after admission for COVID-19 vaccine-associated myocarditis showed improvement of LGE in all patients, but persistent in the majority. Follow-up CMR 6-12 months after acute episode should be considered to better understand the long-term cardiac risks., What Is Known: • Myocarditis is a rare side effect of COVID-19 mRNA vaccine. •Late gadolinium enhancement is present on most cardiac magnetic resonance at the time of acute presentation., What Is New: •Late gadolinium enhancement improved on all repeat cardiac magnetic resonance at 3-month follow-up. •Most patients still had a small amount of late gadolinium enhancement, the clinical significance of which is yet to be determined., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Advances in cardiac CT (CCT) scanner technology allow imaging without anesthesia, and with low radiation dose, making it an attractive technique in infants with congenital heart disease. However, the utility of CCT using a dual-source scanner with respect to diagnostic performance and impact on management has not been systematically studied in this population., Methods: Retrospective review of infants who underwent CCT to determine the utility of CCT with respect to the following: answering the primary diagnostic question, providing new diagnostic information, prompting a change in management, and concordance with catheterization or surgical inspection., Results: A total of 156 infants underwent 172 scans at a median age of 64 days, (IQR 4-188) from Jan 2016-Dec 2019. The most frequent diagnostic question was related to the pulmonary arteries (43%), followed by the aortic arch (30%), pulmonary veins (26%), coronary arteries (17%), patent ductus arteriosus (10%) and others (9%). A high-pitch spiral scan was frequently used (90%). The median effective radiation dose was low (0.66 ​mSv) and general anesthesia was used infrequently (23%). CCT answered the primary diagnostic question in 168/172 (98%) and added to the diagnostic information already available by echocardiography in 161/172 (96%) scans. CCT led to a change in management following 78/172 (53%) scans and had an impact on management following 167/172 (97%) scans. On follow-up, after 107/172 (62%) scans, subjects underwent cardiac surgery, and after 55/172 (32%) scans, they had cardiac catheterization. CCT findings were concordant with catheterization and/or surgical inspection in 156/159 (98%) scans., Conclusions: In infants with complex congenital heart disease, CCT was accurate, answered the diagnostic questions in nearly all cases, and frequently added diagnostic information that impacted management. Radiation exposure was low, and anesthesia was needed infrequently., Competing Interests: Declaration of competing interest None., (Copyright © 2021 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Adults with repaired coarctation of the aorta (CoA) suffer reduced long-term survival compared with the general population, in part due to coronary artery disease (CAD). There is conflicting evidence as to whether or not CoA is an independent risk factor for CAD. The primary aim was to determine if CoA is independently associated with premature myocardial infarction (MI) in the contemporary era. The secondary aim was to determine if CoA is independently associated with early coronary intervention. In a cross-sectional study using the National Inpatient Sample database from 2005 to 2014, we compared the age at MI and the age at coronary intervention (coronary artery bypass grafting or percutaneous coronary intervention, in the absence of MI diagnosis) in patients with and without CoA using weighted linear regression. Among 5,472,416 observations with a primary diagnosis of MI, 174 had a diagnosis of CoA. Patients with CoA had MI 7.2 years younger than those without CoA, after adjusting for potential confounders (95% CI -11.3, -3.1, p = 0.001). Among 3,631,718 patients without a diagnosis of MI who underwent coronary artery bypass grafting or percutaneous coronary intervention, 279 had a diagnosis of CoA. Patients with CoA who underwent coronary intervention were 15.6 years younger than those without CoA, after adjusting for potential confounders (95% CI -18.3, -12.9, p < 0.001). In conclusion, patients with CoA have MI at a slightly younger age and undergo coronary intervention at a significantly younger age than those without CoA in the contemporary era. Our findings support continued close surveillance for and treatment of modifiable risk factors for CAD., (Copyright © 2018. Published by Elsevier Inc.)

Performing coronary computed tomography angiography (CCTA) using third-generation dual source computed tomography (3G-DSCT) scanners results in radiation dose reduction without sacrificing image quality in adults. The largest dose reductions have been reported with prospectively gated, high-pitch imaging. However, there are limited data to determine if these benefits extend to pediatric patients. We evaluated image quality and radiation dose range of CCTA performed in children using a 3G-DSCT scanner. A retrospective review of 44 children (median age 10years, range 0.6 to 17) who underwent imaging to evaluate coronary artery origins (n = 27), Kawasaki disease (n = 12) or other coronary abnormalities (n = 5) were performed. General anesthesia was used in 9/44 (20%) patients and a β blocker was administered in 19/44 (43%). Prospectively gated high-pitch scanning was most frequently used (n = 24). Other techniques used included prospectively-gated "step and shoot" (n = 14), retrospectively gated (n = 2) and nongated high-pitch scan (n = 4). Median effective radiation doses were lowest for prospectively gated high-pitch scans (0.5mSv, range 0.4 to 0.7). Overall coronary artery image quality grade (1-excellent and 4-nondiagnostic) was acceptable for all electrocardiography-gated techniques, with no significant differences between high-pitch and "step-and-shoot" scan types (median 1, range 1 to 3 vs median 1, range 1 to 4, p = 0.22). Image quality grade was diagnostic (1 to 3) for all proximal coronary segments but rare distal segments were nondiagnostic (0.8% segments for gated high-pitch scan). In conclusion, CCTA can be performed in children using 3G-DSCT scanners withacceptable image quality. Prospectively gated high-pitch scans deliver the lowest radiation dose without reduction in image quality compared with conventional scan techniques., (Copyright © 2018. Published by Elsevier Inc.)

Background: The cumulative exposure and risk of anesthesia, vascular access, contrast agents and radiation is emerging as a significant lifelong burden in patients with congenital heart disease (CHD). Latest generation computerized tomographic (CT) scanners are increasingly used for high resolution cardiovascular imaging and have both hardware and post processing radiation dose reduction strategies that can be implemented. Currently, these dose reduction strategies are not uniformly applied and there is a large variability in radiation dose used for the performance of CT in CHD., Methods: We propose the development and implementation of a prospective, multi-center and multi-specialty consortium to measure the variability of use, risk and image quality of CT scans in patients of all ages with CHD. The primary goals of this collaboration are 1) define variability of use, diagnostic quality, and risk of cardiac CT 2) establish best practice guidelines designed to optimize diagnostic image quality with appropriate use of radiation and anesthesia exposure 3) provide institution specific feedback compared with the group norm across participating centers 4) improve the level of evidence for the use of CT in CHD through the collection of prospective and multi-institutional data., Conclusions: Prospective multi-institutional data is needed to inform risk estimates of CT in CHD using current generation scanners and aggressive dose optimization techniques. This registry will provide a platform for future collaboration establishing a multi-modality risk assessment tool specific to patients with CHD., (Copyright © 2018. Published by Elsevier Inc.)

Aortic stiffness measured by cardiac magnetic resonance (CMR) in connective tissue disorder (CTD) patients has been previously shown to be abnormal and to be associated with adverse aortic outcomes. The rate of increase in aortic stiffness with normal aging has been previously described. However, longitudinal changes in aortic stiffness have not been characterized in CTD patients. We examined longitudinal changes in CMR-derived aortic stiffness in children and young adults with CTDs. A retrospective analysis of 50 children and young adults (median age, 20 years; range, 0.2 to 49; 40% < 18 years old) with a CTD, and with at least 2 CMR examinations (total 152 examinations) over a median duration of 3.9 (1 to 13.2) years was performed. Aortic stiffness measures (strain, distensibility, and β stiffness index) were calculated on each examination at the aortic root (AoR), ascending aorta, and descending aorta. Longitudinal changes in parameters were analyzed using linear mixed-effects models. Aortic strain and distensibility decreased with age, whereas the β stiffness index increased at all aortic segments. The average rates of decline in distensibility (x10 -3  mm Hg -1 per 10-year increase in age) were 0.7, 1.3, and 1 at the AoR, ascending aorta, and descending aorta, respectively. The rates of decline in distensibility were not associated with the rates of AoR dilation or surgical AoR replacement. In conclusion, on serial CMR measurements in children and young adults with CTDs, aortic stiffness progressively increased with age, with rates of change only slightly higher than those previously reported in healthy adults., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Aims: Patients with repaired tetralogy of Fallot (rTOF) frequently have right bundle branch block. To better understand the contribution of cardiac dyssynchrony to dysfunction, we developed a method to quantify left (LV), right (RV), and inter-ventricular dyssynchrony using standard cine cardiac magnetic resonance (CMR)., Methods and Results: Thirty patients with rTOF and 17 healthy controls underwent cine CMR. Patients were imaged twice to assess inter-test reproducibility. Circumferential strain curves were generated with a custom feature-tracking algorithm for 12 LV and 12 RV segments in each of 4-7 short-axis slices encompassing the ventricles. Temporal offsets (TOs, in ms) of the strain curves relative to a patient-specific reference curve were calculated. The intra-ventricular dyssynchrony index (DI) for each ventricle was computed as the standard deviation of the TOs. The inter-ventricular DI was calculated as the difference in median RV and median LV TOs. Compared with controls, patients had a greater LV DI (21 ± 8 vs. 11 ± 5 ms, P < 0.001) and RV DI (60 ± 19 vs. 47 ± 17 ms, P = 0.02). RV contraction was globally delayed in patients, resulting in a greater inter-ventricular DI with the RV contracting 45 ± 25 ms later than the LV vs. 12 ± 29 ms earlier in controls (P < 0.001). Inter-test reproducibility was moderate with all coefficients of variation ≤22%. Both LV and RV DIs were correlated with measures of LV, but not RV, function., Conclusion: Patients with rTOF have intra- and inter-ventricular dyssynchrony, which can be quantified from standard cine CMR. This new approach can potentially help determine the contribution of dyssynchrony to ventricular dysfunction in future studies., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

In this paper we present a new automated method for detecting endocardial and epicardial borders in the left (LV) and right ventricles (RV) of the human heart. Our approach relies on morphological operations on both binary and grayscale images. First, the standard power-law transformation is applied on the image. Then, a region of interest (ROI) is selected semi-automatically, followed by automated endocardial and epicardial border extraction based on the selected ROI. In order to get the endocardial contour, the transformed image is thresholded and the maximum area, which indicates the cavity, is selected. Finally, the edge detection is performed and the papillary muscles (PMs) are excluded via a convex-hull method. The epicardial boundary is delineated through a threshold decomposition opening (TDO) approach along with morphological operations. The algorithm extracts the most precise myocardial and RV contours. Experimental results from three normal subjects are shown and quantitatively compared with manually traced contours by an expert. It is concluded that the method performs well in both endocardial and epicardial LV contouring as well as RV cavity detection.