Impact of ferumoxytol vs gadolinium on 4D flow cardiovascular magnetic resonance measurements in small children with congenital heart disease.

BACKGROUND: Cardiovascular magnetic resonance (CMR) allows for time-resolved three-dimensional phase-contrast (4D Flow) analysis of congenital heart disease (CHD). Higher spatial resolution in small infants requires thinner slices, which can degrade the signal. Particularly in infants, the choice of contrast agent (ferumoxytol vs. gadolinium) may influence 4D Flow CMR accuracy. Thus, we investigated the accuracy of 4D Flow CMR measurements compared to gold standard 2D flow phase contrast (PC) measurements in ferumoxytol vs. gadolinium-enhanced CMR of small CHD patients with shunt lesions. METHODS: This was a retrospective study consisting of CMR studies from complex CHD patients less than 20 kg who had ferumoxytol or gadolinium-enhanced 4D Flow and standard two-dimensional phase contrast (2D-PC) flow collected. 4D Flow clinical software (Arterys) was used to measure flow in great vessels, systemic veins, and pulmonary veins. 4D Flow accuracy was defined as percent difference or correlation against conventional measurements (2D-PC) from the same vessels. Subgroup analysis was performed on two-ventricular vs single-ventricular CHD, arterial vs venous flow, as well as low flows (defined as < 1.5 L/min) in 1V CHD. RESULTS: Twenty-one ferumoxytol-enhanced and 23 gadolinium-enhanced CMR studies were included, with no difference in age (2.1 ± 1.6 vs. 2.3 ± 1.9 years, p = 0.70), patient body surface area (0.50 ± 0.2 vs. 0.52 ± 0.2 m2, p = 0.67), or vessel diameter (11.4 ± 5.2 vs. 12.4 ± 5.6 mm, p = 0.22). Ten CMR studies with single ventricular CHD were included. Overall, ferumoxytol-enhanced 4D flow CMR measurements demonstrated less percent difference to 2D-PC when compared to gadolinium-enhanced 4D Flow CMR studies. In subgroup analyses of arterial vs. venous flows (high velocity vs. low velocity) and low flow in single ventricle CHD, ferumoxytol-enhanced 4D Flow CMR measurements had stronger correlation to 2D-PC CMR. The contrast-to-noise ratio (CNR) in ferumoxytol-enhanced studies was higher than the CNR in gadolinium-enhanced studies. CONCLUSIONS: Ferumoxytol-enhanced 4D Flow CMR has improved accuracy when compared to gadolinium 4D Flow CMR, particularly for infants with small vessels in CHD.

Cardiac echocardiogram findings of severe acute respiratory syndrome coronavirus-2-associated multi-system inflammatory syndrome in children.

BACKGROUND: A novel paediatric disease, multi-system inflammatory syndrome in children, has emerged during the 2019 coronavirus disease pandemic. OBJECTIVES: To describe the short-term evolution of cardiac complications and associated risk factors in patients with multi-system inflammatory syndrome in children. METHODS: Retrospective single-centre study of confirmed multi-system inflammatory syndrome in children treated from 29 March, 2020 to 1 September, 2020. Cardiac complications during the acute phase were defined as decreased systolic function, coronary artery abnormalities, pericardial effusion, or mitral and/or tricuspid valve regurgitation. Patients with or without cardiac complications were compared with chi-square, Fisher's exact, and Wilcoxon rank sum. RESULTS: Thirty-nine children with median (interquartile range) age 7.8 (3.6-12.7) years were included. Nineteen (49%) patients developed cardiac complications including systolic dysfunction (33%), valvular regurgitation (31%), coronary artery abnormalities (18%), and pericardial effusion (5%). At the time of the most recent follow-up, at a median (interquartile range) of 49 (26-61) days, cardiac complications resolved in 16/19 (84%) patients. Two patients had persistent mild systolic dysfunction and one patient had persistent coronary artery abnormality. Children with cardiac complications were more likely to have higher N-terminal B-type natriuretic peptide (p = 0.01), higher white blood cell count (p = 0.01), higher neutrophil count (p = 0.02), severe lymphopenia (p = 0.05), use of milrinone (p = 0.03), and intensive care requirement (p = 0.04). CONCLUSION: Patients with multi-system inflammatory syndrome in children had a high rate of cardiac complications in the acute phase, with associated inflammatory markers. Although cardiac complications resolved in 84% of patients, further long-term studies are needed to assess if the cardiac abnormalities (transient or persistent) are associated with major cardiac events.

Magnetic Resonance Imaging-Guided Cardiac Catheterization Evacuation Drills.

BACKGROUND: The interventional cardiac magnetic resonance imaging suite combines a cardiac catheterization x-ray laboratory with a magnetic resonance imaging suite. At the study institution, interventional cardiac magnetic resonance imaging procedures (ie, magnetic resonance imaging-guided cardiac catheterizations) have been performed under institutional review board-approved research protocols since 2015. Because the workplace incorporates x-ray and magnetic resonance imaging in a highly technical environment, education about the importance of magnet safety is crucial to ensure the safety of patients and staff. OBJECTIVE: To promote magnetic resonance imaging safety and staff preparedness to respond in emergency situations in a specialized interventional cardiac magnetic resonance imaging environment. METHODS: Quarterly in situ evacuation drills with a live volunteer were implemented. A retrospective participant survey using a Likert scale was conducted. Evacuations were timed from the cardiac arrest code alert to safe evacuation or defibrillation if appropriate. RESULTS: Over 4 years, 14 drills were performed. Twenty-nine of 48 participants responded to the survey, a 60% response rate. Most participants agreed or strongly agreed that the drills were a positive experience (90%) and that the drills increased their confidence in their ability to perform in an evacuation scenario (100%). Room evacuation times improved from 71 to 41 seconds. No patient or staff safety events occurred in the interventional cardiac magnetic resonance imaging environment. CONCLUSION: Magnetic resonance imaging-guided cardiac catheterization evacuation drills promote preparedness, ensure patient and staff safety, and improve evacuation time in the interventional cardiac magnetic resonance imaging environment.

Motion-corrected cardiac MRI is associated with decreased anesthesia exposure in children.

BACKGROUND: The benefits of cardiac magnetic resonance imaging (MRI) in the pediatric population must be balanced with the risk and cost of anesthesia. Segmented imaging using multiple averages attempts to avoid breath-holds requiring general anesthesia; however, cardiorespiratory artifacts and prolonged scan times limit its use. Thus, breath-held imaging with general anesthesia is used in many pediatric centers. The advent of free-breathing, motion-corrected (MOCO) cines by real-time re-binned reconstruction offers reduced anesthesia exposure without compromising image quality. OBJECTIVE: This study evaluates sedation utilization in our pediatric cardiac MR practice before and after clinical introduction of free-breathing MOCO imaging for cine and late gadolinium enhancement. MATERIALS AND METHODS: In a retrospective study, patients referred for a clinical cardiac MR who would typically be offered sedation for their scan (n=295) were identified and divided into two eras, those scanned before the introduction of MOCO cine and late gadolinium enhancement sequences and those scanned following their introduction. Anesthesia use was compared across eras and disease-specific cohorts. RESULTS: The incidence of non-sedation studies performed in children nearly tripled following the introduction of MOCO imaging (25% [pre-MOCO] to 69% [post-MOCO], P<0.01), with the greatest effect in patients with simple congenital heart disease. Eleven percent of the post-MOCO cohort comprised infants younger than 3 months of age who could forgo sedation with the combination of MOCO imaging and a "feed-and-bundle" positioning technique. CONCLUSION: Implementation of cardiac MR with MOCO cine and late gadolinium enhancement imaging in a pediatric population is associated with significantly decreased sedation utilization.

Abnormal Pulmonary Artery Bending Correlates With Increased Right Ventricular Afterload Following the Arterial Switch Operation.

PURPOSE: In transposition of great arteries, increased right ventricular (RV) afterload is observed following arterial switch operation (ASO), which is not always related to pulmonary artery (PA) stenosis. We hypothesize that abnormal PA bending from the Lecompte maneuver may affect RV afterload in the absence of stenosis. Thus, we sought to identify novel measurements of three-dimensional cardiac magnetic resonance (CMR) images of the pulmonary arteries and compare with conventional measurements in their ability to predict RV afterload. METHODS: Conventional measurements and novel measurements of the pulmonary arteries were performed using CMR data from 42 ASO patients and 13 age-matched controls. Novel measurements included bending angle, normalized radius of curvature (Rc), and normalized weighted radius of curvature (Rc-w). Right ventricular systolic pressures (as the surrogate for RV afterload) were measured by either recent echocardiogram or cardiac catheterization. RESULTS: Conventional measurements of proximal PA size correlated with differential pulmonary blood flow (r = 0.49, P = .001), but not with RV peak systolic pressures (r = -0.26, P = .18). In ASO patients, Rc-w correlated with higher RV systolic pressures (r = -0.57, P = .002). Larger neoaortic areas and rightward bending angles correlated with smaller right pulmonary artery Rc (r = -0.48, P = .001; r = 0.41, P = .01, respectively). Finally, both pulmonary arteries had significantly smaller Rc compared to normal controls. CONCLUSIONS: Pulmonary arteries exhibit abnormal bends following ASO that correlate with increased RV afterload, independent of PA stenosis. Future work should focus on clinical and hemodynamic contributions of these shape parameters.

Updates in Congenital Heart Disease: Can Outcomes Improve?

Despite numerous advances in medical and surgical management, congenital heart disease (CHD) remains the number one cause of death in the first year of life from congenital malformations. The current strategies used to approach improving outcomes in CHD are varied. This article will discuss the recent impact of pulse oximetry screening for critical CHD, describe the contributions of advanced cardiac imaging in the neonate with CHD, and highlight the growing importance of quality improvement and safety programs in the cardiac intensive care unit.

X-ray fused with MRI guidance of pre-selected transcatheter congenital heart disease interventions.

OBJECTIVES: To determine whether X-ray fused with MRI (XFM) is beneficial for select transcatheter congenital heart disease interventions. BACKGROUND: Complex transcatheter interventions often require three-dimensional (3D) soft tissue imaging guidance. Fusion imaging with live X-ray fluoroscopy can potentially improve and simplify procedures. METHODS: Patients referred for select congenital heart disease interventions were prospectively enrolled. Cardiac MRI data was overlaid on live fluoroscopy for procedural guidance. Likert scale operator assessments of value were recorded. Fluoroscopy time, radiation exposure, contrast dose, and procedure time were compared to matched cases from our institutional experience. RESULTS: Forty-six patients were enrolled. Pre-catheterization, same day cardiac MRI findings indicated intervention should be deferred in nine patients. XFM-guided cardiac catheterization was performed in 37 (median age 8.7 years [0.5-63 years]; median weight 28 kg [5.6-110 kg]) with the following prespecified indications: pulmonary artery (PA) stenosis (n = 13), aortic coarctation (n = 12), conduit stenosis/insufficiency (n = 9), and ventricular septal defect (n = 3). Diagnostic catheterization showed intervention was not indicated in 12 additional cases. XFM-guided intervention was performed in the remaining 25. Fluoroscopy time was shorter for XFM-guided intervention cases compared to matched controls. There was no significant difference in radiation dose area product, contrast volume, or procedure time. Operator Likert scores indicated XFM provided useful soft tissue guidance in all cases and was never misleading. CONCLUSIONS: XFM provides operators with meaningful three-dimensional soft tissue data and reduces fluoroscopy time in select congenital heart disease interventions.

Anesthetic considerations for magnetic resonance imaging-guided right-heart catheterization in pediatric patients: A single institution experience.

Cardiac catheterization is an integral part of medical management for pediatric patients with congenital heart disease. Owing to age and lack of cooperation in children who need this procedure, general anesthesia is typically required. These patients have increased anesthesia risk secondary to cardiac pathology. Furthermore, multiple catheterization procedures result in exposure to harmful ionizing radiation. Magnetic resonance imaging-guided right-heart catheterization offers decreased radiation exposure and diagnostic imaging benefits over traditional fluoroscopy but potentially increases anesthetic complexity and risk. We describe our early experience with anesthetic techniques and challenges for pediatric magnetic resonance imaging-guided right-heart catheterization.

Validation of cardiac magnetic-resonance-derived left ventricular strain measurements from free-breathing motion-corrected cine imaging.

BACKGROUND: Myocardial strain is an important measure of cardiac function and can be assessed on cardiac magnetic resonance (MR) through the current gold standard of breath-held segmented steady-state free precession (SSFP) cine imaging. Novel free-breathing techniques have been validated for volumetry and systolic function, allowing for evaluation of sicker and younger children who cannot reliably hold their breath. It is unclear whether strain measurements can be reliably performed on free-breathing, motion-corrected, re-binning cine images. OBJECTIVE: To compare strain analysis from motion-corrected retrospective re-binning images to the breath-held SSFP cine images to explore their validity. MATERIALS AND METHODS: Twenty-five children and young adults, ages (2.1-18.6 years) underwent breath-held and motion-corrected retrospective re-binning cine techniques during the same MR examination on a 1.5-tesla magnet. We measured endocardial end-systolic global circumferential strain and endocardial averaged segmental strain using commercial software (MEDIS QStrain 2.1). We used Pearson correlation coefficients to test agreement across techniques. RESULTS: Analysis was possible in all 25 breath-held and motion-corrected retrospective re-binning studies. Global circumferential strain and endocardial averaged segmental strain obtained by motion-corrected retrospective re-binning compared favorably to breath-held studies. Global circumferential strain linear regression models demonstrated acceptable agreement, with coefficients of determination of 0.75 for breath-held compared to motion-corrected retrospective re-binning (P<0.001) and for endocardial averaged segmental strain comparisons yielded 0.77 for breath-held vs. motion-corrected retrospective re-binning (P<0.001). Bland-Altman assessment demonstrated minimal bias for breath-held compared to motion-corrected retrospective re-binning (mean 2.4 and 1.9, respectively, for global circumferential strain and endocardial averaged segmental strain). CONCLUSION: Free-breathing imaging by motion-corrected retrospective re-binning cine imaging provides adequate spatial and temporal resolution to measure myocardial deformation when compared to the gold-standard breath-held SSFP cine imaging in children with normal or borderline systolic function.

Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called "scimitar vein" without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.

Reduced psychological distress in familial chylomicronemia syndrome after patient support group intervention.

Familial chylomicronemia syndrome (FCS) is a rare genetic disorder that is associated with severe hypertriglyceridemia and complications that often include recurrent pancreatitis beginning in childhood. Patients with FCS frequently struggle to maintain normality in their lives as a consequence of the necessity to severely restrict their intake of dietary fat coupled with the constant threat of recurrent pancreatitis. Patients typically face a high level of psychological stress and anxiety in association with reduced measures of quality of life. Routine medical care for affected patients usually does not adequately address the day-to-day struggles that accompany a diagnosis of FCS, resulting in ongoing suffering for many patients. We describe herein the highly beneficial effects of a support group interaction for a patient with FCS.

Radiation-free CMR diagnostic heart catheterization in children.

BACKGROUND: Children with heart disease may require repeated X-Ray cardiac catheterization procedures, are more radiosensitive, and more likely to survive to experience oncologic risks of medical radiation. Cardiovascular magnetic resonance (CMR) is radiation-free and offers information about structure, function, and perfusion but not hemodynamics. We intend to perform complete radiation-free diagnostic right heart catheterization entirely using CMR fluoroscopy guidance in an unselected cohort of pediatric patients; we report the feasibility and safety. METHODS: We performed 50 CMR fluoroscopy guided comprehensive transfemoral right heart catheterizations in 39 pediatric (12.7 ± 4.7 years) subjects referred for clinically indicated cardiac catheterization. CMR guided catheterizations were assessed by completion (success/failure), procedure time, and safety events (catheterization, anesthesia). Pre and post CMR body temperature was recorded. Concurrent invasive hemodynamic and diagnostic CMR data were collected. RESULTS: During a twenty-two month period (3/2015 - 12/2016), enrolled subjects had the following clinical indications: post-heart transplant 33%, shunt 28%, pulmonary hypertension 18%, cardiomyopathy 15%, valvular heart disease 3%, and other 3%. Radiation-free CMR guided right heart catheterization attempts were all successful using passive catheters. In two subjects with septal defects, right and left heart catheterization were performed. There were no complications. One subject had six such procedures. Most subjects (51%) had undergone multiple (5.5 ± 5) previous X-Ray cardiac catheterizations. Retained thoracic surgical or transcatheter implants (36%) did not preclude successful CMR fluoroscopy heart catheterization. During the procedure, two subjects were receiving vasopressor infusions at baseline because of poor cardiac function, and in ten procedures, multiple hemodynamic conditions were tested. CONCLUSIONS: Comprehensive CMR fluoroscopy guided right heart catheterization was feasible and safe in this small cohort of pediatric subjects. This includes subjects with previous metallic implants, those requiring continuous vasopressor medication infusions, and those requiring pharmacologic provocation. Children requiring multiple, serial X-Ray cardiac catheterizations may benefit most from radiation sparing. This is a step toward wholly CMR guided diagnostic (right and left heart) cardiac catheterization and future CMR guided cardiac intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT02739087 registered February 17, 2016.

Acute Cardiac MRI Assessment of Radiofrequency Ablation Lesions for Pediatric Ventricular Arrhythmia: Feasibility and Clinical Correlation.

BACKGROUND: Arrhythmia ablation with current techniques is not universally successful. Inadequate ablation lesion formation may be responsible for some arrhythmia recurrences. Periprocedural visualization of ablation lesions may identify inadequate lesions and gaps to guide further ablation and reduce risk of arrhythmia recurrence. METHODS: This feasibility study assessed acute postprocedure ablation lesions by MRI, and correlated these findings with clinical outcomes. Ten pediatric patients who underwent ventricular tachycardia ablation were transferred immediately postablation to a 1.5T MRI scanner and late gadolinium enhancement (LGE) imaging was performed to characterize ablation lesions. Immediate and mid-term arrhythmia recurrences were assessed. RESULTS: Patient characteristics include median age 14 years (1-18 years), median weight 52 kg (11-81 kg), normal cardiac anatomy (n = 6), d-transposition of great arteries post arterial switch repair (n = 2), anomalous coronary artery origin post repair (n = 1), and cardiac rhabdomyoma (n = 1). All patients underwent radiofrequency catheter ablation of ventricular arrhythmia with acute procedural success. LGE was identified at the reported ablation site in 9/10 patients, all arrhythmia-free at median 7 months follow-up. LGE was not visible in 1 patient who had recurrence of frequent premature ventricular contractions within 2 hours, confirmed on Holter at 1 and 21 months post procedure. CONCLUSIONS: Ventricular ablation lesion visibility by MRI in the acute post procedure setting is feasible. Lesions identifiable with MRI may correlate with clinical outcomes. Acute MRI identification of gaps or inadequate lesions may provide the unique temporal opportunity for additional ablation therapy to decrease arrhythmia recurrence.

Dark blood late enhancement imaging.

BACKGROUND: Bright blood late gadolinium enhancement (LGE) imaging typically achieves excellent contrast between infarcted and normal myocardium. However, the contrast between the myocardial infarction (MI) and the blood pool is frequently suboptimal. A large fraction of infarctions caused by coronary artery disease are sub-endocardial and thus adjacent to the blood pool. It is not infrequent that sub-endocardial MIs are difficult to detect or clearly delineate. METHODS: In this present work, an inversion recovery (IR) T2 preparation was combined with single shot steady state free precession imaging and respiratory motion corrected averaging to achieve dark blood LGE images with good signal to noise ratio while maintaining the desired spatial and temporal resolution. In this manner, imaging was conducted free-breathing, which has benefits for image quality, patient comfort, and clinical workflow in both adults and children. Furthermore, by using a phase sensitive inversion recovery reconstruction the blood signal may be made darker than the myocardium (i.e., negative signal values) thereby providing contrast between the blood and both the MI and remote myocardium. In the proposed approach, a single T1-map scout was used to measure the myocardial and blood T1 using a MOdified Look-Locker Inversion recovery (MOLLI) protocol and all protocol parameters were automatically calculated from these values within the sequence thereby simplifying the user interface. RESULTS: The contrast to noise ratio (CNR) between MI and remote myocardium was measured in n = 30 subjects with subendocardial MI using both bright blood and dark blood protocols. The CNR for the dark blood protocol had a 13 % loss compared to the bright blood protocol. The CNR between the MI and blood pool was positive for all dark blood cases, and was negative in 63 % of the bright blood cases. The conspicuity of subendocardial fibrosis and MI was greatly improved by dark blood (DB) PSIR as well as the delineation of the subendocardial border. CONCLUSIONS: Free-breathing, dark blood PSIR LGE imaging was demonstrated to improve the visualization of subendocardial MI and fibrosis in cases with low contrast with adjacent blood pool. The proposed method also improves visualization of thin walled fibrous structures such as atrial walls and valves, as well as papillary muscles.

Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked, inherited disorder causing dilated cardiomyopathy with variable onset and progression. Currently we lack objective markers of the effect of therapies targeted towards preventing progression of subclinical cardiac disease. Thus, our aim was to compare the ability of native T1 and extracellular volume (ECV) measurements to differentiate risk of myocardial disease in DMD and controls. METHODS: Twenty boys with DMD and 16 age/gender-matched controls without history predisposing to cardiac fibrosis, but with a clinical indication for cardiovascular magnetic resonance (CMR) evaluation, underwent CMR with contrast. Data points collected include left ventricular ejection fraction (LVEF), left ventricular mass, and presence of late gadolinium enhancement (LGE). Native T1, and ECV regional mapping were obtained using both a modified Look-Locker (MOLLI) and saturation recovery single shot sequence (SASHA) on a 1.5T scanner. Using ordinal logistic regression models, controlling for age and LVEF, LGE-free septal we evaluated the ability native T1 and ECV assessments to differentiate levels of cardiomyopathy. RESULTS: Twenty DMD subjects aged 14.4 ± 4 years had an LVEF of 56.3 ± 7.4 %; 12/20 had LGE, all confined to the lateral wall. Sixteen controls aged 16.1 ± 2.2 years had an LVEF 60.4 ± 5.1 % and no LGE. Native T1 and ECV values were significantly higher in the DMD group (p < 0.05) with both MOLLI and SASHA imaging techniques. Native T1 demonstrated a 50 % increase in the ability to predict disease state (control, DMD without fibrosis, DMD with fibrosis). ECV demonstrated only the ability to predict presence of LGE, but could not distinguish between controls and DMD without fibrosis. CONCLUSIONS: LGE-spared regions of boys with DMD have significantly different native T1 and ECV values compared to controls. Native T1 measurements can identify early changes in DMD patients without the presence of LGE and help predict disease severity more effectively than ECV. Native T1 may be a novel outcome measure for early cardiac therapies in DMD and other cardiomyopathies.

The Impact of a Designated Cardiology Team Involving Telemedicine Home Monitoring on the Care of Children with Single-Ventricle Physiology After Norwood Palliation.

We evaluated the effect of an interdisciplinary single-ventricle task force (SVTF) that utilizes a family-driven, telemedicine home monitoring program on clinical outcomes of stage II admissions and its acceptance by parents and cardiologists. Study population was divided into two cohorts, one with Norwood surgery dates before the SVTF (pre-SVTF) and one interventional (post-SVTF). Post-SVTF data also included surveys of parents and cardiologists on the efficacy of the SVTF. Comparative and multivariate statistical testing was performed. Compared to the pre-SVTF group, the post-SVTF group had lower complications after stage II (18.4 vs. 34.1 %, p = 0.02), higher weight-for-age z scores at stage II (-1.5 ± 0.97 vs. -1.58 ± 1.34, p = 0.02) and were less likely to have a stage II weight-for-age z score below -2 (26.5 vs. 31.7 %, p = 0.03). A multivariate regression analysis showed providing a written red-flag action plan to parents at discharge was independently associated with higher weight at stage II (ß = 0.42, p = 0.04) and higher weight-for-age z score (ß = 0.48, p = 0.02). Parents' satisfaction with SVTF (α = 0.97) was 4.34 ± 0.62; (95 % CI 4.01-4.67) and cardiologists' acceptance (α = 0.93) was 4.1 ± 0.7 (95 % CI 3.79-4.42). Development of SVTF was associated with a reduction in complications post-stage II and improved weight status at stage II. A written red-flag action plan provided to parents at the time of Norwood discharge was associated with higher weight status at stage II. Parents and cardiologists expressed satisfaction with the utility of SVTF and encouraged expansion to cover all children with congenital heart disease.