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Background The value of MRI in pediatric congenital heart disease (CHD) is well recognized; however, the requirement for expert oversight impedes its widespread use. Four-dimensional (4D) multiphase steady-state imaging with contrast enhancement (MUSIC) is a cardiovascular MRI technique that uses ferumoxytol and captures all anatomic features dynamically. Purpose To evaluate multicenter feasibility of 4D MUSIC MRI in pediatric CHD. Materials and Methods In this prospective study, participants with CHD underwent 4D MUSIC MRI at 3.0 T or 1.5 T between 2014 and 2020. From a pool of 460 total studies, an equal number of MRI studies from three sites (n = 60) was chosen for detailed analysis. With use of a five-point scale, the feasibility of 4D MUSIC was scored on the basis of artifacts, image quality, and diagnostic confidence for intracardiac and vascular connections (n = 780). Respiratory motion suppression was assessed by using the signal intensity profile. Bias between 4D MUSIC and two-dimensional (2D) cine imaging was evaluated by using Bland-Altman analysis; 4D MUSIC examination duration was compared with that of the local standard for CHD. Results A total of 206 participants with CHD underwent MRI at 3.0 T, and 254 participants underwent MRI at 1.5 T. Of the 60 MRI examinations chosen for analysis (20 per site; median participant age, 14.4 months [interquartile range, 2.3-49 months]; 33 female participants), 56 (93%) had good or excellent image quality scores across a spectrum of disease complexity (mean score ± standard deviation: 4.3 ± 0.6 for site 1, 4.9 ± 0.3 for site 2, and 4.6 ± 0.7 for site 3; P < .001). Artifact scores were inversely related to image quality (r = -0.88, P < .001) and respiratory motion suppression (P < .001, r = -0.45). Diagnostic confidence was high or definite in 730 of 780 (94%) intracardiac and vascular connections. The correlation between 4D MUSIC and 2D cine ventricular volumes and ejection fraction was high (range of r = 0.72-0.85; P < .001 for all). Compared with local standard MRI, 4D MUSIC reduced the image acquisition time (44 minutes ± 20 vs 12 minutes ± 3, respectively; P < .001). Conclusion Four-dimensional multiphase steady-state imaging with contrast enhancement MRI in pediatric congenital heart disease was feasible in a multicenter setting, shortened the examination time, and simplified the acquisition protocol, independently of disease complexity. Clinical trial registration no. NCT02752191 © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Roest and Lamb in this issue.

Single ventricle (SV) patients undergo multiple surgeries with subsequent changes in anatomy and hemodynamics. There are little cardiac magnetic resonance (CMR) data on serial changes in these patients. This study aimed to assess longitudinal changes of SV anatomy and hemodynamics in a large cohort. Anatomy and flow in SV patients with serial CMRs performed between 2008 and 2019 at a single institution were retrospectively reviewed. Mixed-effects linear regression was used to estimate changes over time at 3 to 9 months, 1 to 5 years, and >5 years after Fontan. A total of 119 patients were included (51% with hypoplastic left heart syndrome; 77% underwent extracardiac Fontan). A total of 88 patients had 3 serial CMRs. Indexed right superior vena cava, inferior vena cava, neoaortic valve, and descending aorta area decreased over time (beta = -0.19, -0.44, and -0.23, respectively; P <.01), as did indexed right superior vena cava, neoaorta and native aorta, and descending aorta flow (beta = -0.49, -0.53, and -0.59, respectively; P <.0001). Inferior vena cava flow and its contribution to total caval flow increased (beta = 0.33; P <.0001). Indexed right and left pulmonary artery flow did not change; however, indexed left pulmonary artery area decreased (beta = -0.16; P =.0014) with time. Systemic-to-pulmonary collateral flow remained unchanged before and early after Fontan (beta = -0.54; P =.42) but decreased with time from Fontan (beta = -0.22; P <.0001). In this cohort of longitudinally followed SV patients, there are significant trends in vascular size and flow over time from Fontan. These findings can be used as a framework to interpret serial CMR data in the SV and noninvasively identify deviations from expected patterns before the development of clinical symptoms. [Display omitted] [ABSTRACT FROM AUTHOR]

Introduction: New data suggest that lymphatic congestion due to elevated central venous pressure plays a role in development of post-Fontan complications. Conditions such as protein losing enteropathy and plastic bronchitis are a significant cause of late Fontan failure. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined. Methods: Retrospective cohort study of patients undergoing first-time Fontan completion at a single center from 2012-2017. Primary outcome was early Fontan complication , a composite of: pleural effusions >14 d, readmission, cardiac catheterization, ECMO, transplant, Fontan takedown or death; all ≤6 months of surgery. Etiologies of complications were assigned to 1 of 3 groups: Group 1, "structural failure" (obstruction in the Fontan circuit); Group 2, "pump failure" (> moderate ventricular dysfunction and/or > moderate AV valve regurgitation); and Group 3, "lymphatic failure" (development of chylothorax or plastic bronchitis) and those patients with persistent pleural effusions in the absence of structural or pump failure. When clinically utilized prior to Fontan completion, T2 weighted MRI sequences were used to assess low vs. high-grade lymphatic abnormality. Results: The cohort consisted of 238 patients, of which 57% had HLHS and 81% underwent extracardiac fenestrated Fontan. Nearly one-third (n=70; 29%) developed early complications: 20 (28%) in Group 1, 8 (11.5%) in Group 2 and 30 (43%) in Group 3. The remaining 12 had wound infection or non-cardiac cause of readmission. T2 imaging was available for 126 (53%) patients. Across all etiologies, patients with high-grade lymphatic abnormalities had 6 times greater odds of developing early complications (p = 0.001) Conclusions: There is substantial morbidity in the early post-Fontan period. The primary etiology of early complications in this cohort was lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. When performed, T2 imaging demonstrated that patients with higher-grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients prior to Fontan palliation. [ABSTRACT FROM AUTHOR]

Objective: Reintervention rates after patch-augmented reconstruction for hypoplastic aortic arch remain moderately high. We analyzed mid-term outcomes of aortic arch reconstruction to define modifiable reintervention risk factors., Methods: Excluding Damus-Kaye-Stansel anastomoses and previous arch repair, 338 patients underwent arch reconstruction from 2000-2021 at median age 6d (IQR 4-13d) and weight 3.2kg (IQR 2.8-3.7kg). Surgical technique was patch augmentation with coarctectomy ± interdigitation in 269 (80%), isolated patch aortoplasty in 41 (12%), and other reconstruction in 28 (8%). Risk factors for reintervention were assessed using competing risk models., Results: At median follow-up of 3.9y (IQR 1.1-8.0y), 35 (10.4%) patients required reintervention (30 endovascular, 12 surgical, 7 both). Ten-year cumulative incidence of death/transplant and reintervention were 10% (95%CI 4-20%) and 13% (95%CI 8-20%). On univariate analysis, isolated patch aortoplasty (p=0.002), aortic homograft patch material (p=0.006), and postoperative aortic size z-score ≤-2 for each segment were associated with greater risk of reintervention: ascending aorta (p=0.006), proximal (p=0.001) and distal (p=0.005) transverse arch, and aortic isthmus (p<0.001). On multivariable analysis, aortic homograft (HR 6.29, 95%CI 1.94-20.5, p=0.002) and postoperative isthmus z-score ≤-2 (HR 10.5, 95%CI 5.15-21.5, p<0.001) remained significant. Patients with repaired isthmus z-score ≤-2 had 72.8% (95%CI 44.6-94.4%) cumulative incidence of reintervention at 10 years, versus 6.8% (95%CI 4.1-11.4%) in those with z-score >-2., Conclusions: Aortic undersizing during patch-augmented reconstruction of hypoplastic aortic arch results in over 10% rate of reintervention at mid-term follow-up. Achieving adequate postoperative arch size is critical for preventing reintervention, with aortic isthmus size being of utmost importance., (Copyright © 2024. Published by Elsevier Inc.)