Insights into the effects of Friedreich ataxia on the left ventricle using T1 mapping and late gadolinium enhancement.

Background: The left ventricular (LV) changes which occur in Friedreich ataxia (FRDA) are incompletely understood.

Methods: Cardiac magnetic resonance (CMR) imaging was performed using a 1.5T scanner in subjects with FRDA who are homozygous for an expansion of an intron 1 GAA repeat in the FXN gene.

A Deep Learning Pipeline for Assessing Ventricular Volumes from a Cardiac MRI Registry of Patients with Single Ventricle Physiology.

Purpose To develop an end-to-end deep learning (DL) pipeline for automated ventricular segmentation of cardiac MRI data from a multicenter registry of patients with Fontan circulation (Fontan Outcomes Registry Using CMR Examinations [FORCE]). Materials and Methods This retrospective study used 250 cardiac MRI examinations (November 2007-December 2022) from 13 institutions for training, validation, and testing. The pipeline contained three DL models: a classifier to identify short-axis cine stacks and two U-Net 3+ models for image cropping and segmentation.

Cerebral Blood Flow, Brain Injury, and Aortic-Pulmonary Collateral Flow After the Fontan Operation.

Patients with a single ventricle develop aortopulmonary collaterals (APCs) whose flow has been shown to be inversely proportional to cerebral blood flow (CBF) in a previous cross-sectional study. Longitudinal CBF and APC flow in patients with Fontan physiology adjusting for brain injury (BI) has never been reported. Decreased CBF and BI may adversely impact neurodevelopment.

Cardiac magnetic resonance predictors for successful primary biventricular repair of unbalanced complete common atrioventricular canal.

Background: Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal.

Patients with Post-COVID-19 Vaccination Myocarditis Have More Favorable Strain in Cardiac Magnetic Resonance Than Those With Viral Myocarditis.

There have been reports of myocarditis following vaccination against COVID-19. We sought to describe cardiac magnetic resonance (CMR) findings among pediatric patients. Retrospective review at a large academic center of patients clinically diagnosed with post-vaccine myocarditis (PVM) undergoing CMR.

Skeletal muscle deficits are associated with worse exercise performance in pediatric pulmonary hypertension.

Background: Skeletal muscle deficits are associated with worse exercise performance in adults with pulmonary hypertension (PH) but the impact is poorly understood in pediatric PH.

Objective: To study muscle deficits, physical inactivity, and performance on cardiopulmonary exercise test (CPET) and exercise cardiac magnetic resonance (eCMR) in pediatric PH.

Methods: Youth 8-18 years participated in a prospective, cross-sectional study including densitometry (DXA) for measurement of leg lean mass Z-score (LLMZ), handheld dynamometer with generation of dominant and non-dominant handgrip Z-scores, Physical Activity Questionnaire (PAQ), CPET, and optional eCMR.

Longitudinal Trends of Vascular Flow and Growth in Patients Undergoing Fontan Operation.

Background: 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.

Free-running cardiac and respiratory motion-resolved 5D whole-heart coronary cardiovascular magnetic resonance angiography in pediatric cardiac patients using ferumoxytol.

Background: Coronary cardiovascular magnetic resonance angiography (CCMRA) of congenital heart disease (CHD) in pediatric patients requires accurate planning, adequate sequence parameter adjustments, lengthy scanning sessions, and significant involvement from highly trained personnel. Anesthesia and intubation are commonplace to minimize movements and control respiration in younger subjects. To address the above concerns and provide a single-click imaging solution, we applied our free-running framework for fully self-gated (SG) free-breathing 5D whole-heart CCMRA to CHD patients after ferumoxytol injection.

Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the Use of Cardiac Magnetic Resonance in Pediatric Congenital and Acquired Heart Disease: Endorsed by The American Heart Association.

Cardiovascular magnetic resonance has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of cardiovascular magnetic resonance in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking.

Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the use of cardiovascular magnetic resonance in pediatric congenital and acquired heart disease : Endorsed by The American Heart Association.

Cardiovascular magnetic resonance (CMR) has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of CMR in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking.

Determination of Contrast Timing by Time-Resolved Magnetic Resonance Angiography in Patients With Bidirectional Glenn and Hemi-Fontan Anastomoses.

Purpose: Children with single-ventricle congenital heart disease undergo a series of operations to maintain their pulmonary circulation including bidirectional Glenn (BDG) or hemi-Fontan in the second stage to create a superior cavopulmonary anastomosis. We aimed to optimize cardiovascular angiography protocols by determining optimal contrast timing of pulmonary and systemic circulation on magnetic resonance angiography (MRA) performed with the technique of time-resolved imaging with interleaved stochastic trajectories (TWIST).

Methods And Materials: Cardiac TWIST MRA with lower extremity (LE) contrast injection was analyzed in 92 consecutive patients with a BDG or hemi-Fontan anastomosis.

Comparison of serum biomarkers of myocardial fibrosis with cardiac magnetic resonance in patients operated for tetralogy of Fallot.

Background: Serum biomarkers of myocardial fibrosis are considered markers of adverse outcome in adults with heart disease. Associations between biomarkers and clinical parameters in tetralogy of Fallot (TOF) has been understudied. We compared serum biomarker profiles with clinical and cardiac magnetic resonance (CMR) parameters of ventricular remodeling in patients with repaired TOF.

Computational Investigation of Anastomosis Options of a Right-Heart Pump to Patient Specific Pulmonary Arteries.

Patients with Fontan circulation have increased risk of heart failure, but are not always candidates for heart transplant, leading to the development of the subpulmonic Penn State Fontan Circulation Assist Device. The aim of this study was to use patient-specific computational fluid dynamics simulations to evaluate anastomosis options for implanting this device. Simulations were performed of the pre-surgical anatomy as well as four surgical options: a T-junction and three Y-grafts.

Fontan Geometry and Hemodynamics Are Associated With Quality of Life in Adolescents and Young Adults.

Background: Despite favorable short-term outcomes, Fontan palliation is associated with comorbidities and diminished quality of life (QOL) in the years after completion. We hypothesized that poor Fontan hemodynamics and ventricular function are associated with worse QOL.

Methods: This was a single-center study of Fontan survivors aged more than 12 years.

Longitudinal Assessment of Cardiac Outcomes of Multisystem Inflammatory Syndrome in Children Associated With COVID-19 Infections.

Background In multisystem inflammatory syndrome in children, there is paucity of longitudinal data on cardiac outcomes. We analyzed cardiac outcomes 3 to 4 months after initial presentation using echocardiography and cardiac magnetic resonance imaging. Methods and Results We included 60 controls and 60 cases of multisystem inflammatory syndrome in children.

Optimizing neonatal cardiac imaging (magnetic resonance/computed tomography).

Magnetic resonance imaging (MRI) and CT perform an important role in the evaluation of neonates with congenital heart disease (CHD) when echocardiography is not sufficient for surgical planning or postoperative follow-up. Cardiac MRI and cardiac CT have complementary applications in the evaluation of cardiovascular disease in neonates. This review focuses on the indications and technical aspects of these modalities and special considerations for imaging neonates with CHD.

Engineering Perspective on Cardiovascular Simulations of Fontan Hemodynamics: Where Do We Stand with a Look Towards Clinical Application.

Background: Cardiovascular simulations for patients with single ventricles undergoing the Fontan procedure can assess patient-specific hemodynamics, explore surgical advances, and develop personalized strategies for surgery and patient care. These simulations have not yet been broadly accepted as a routine clinical tool owing to a number of limitations. Numerous approaches have been explored to seek innovative solutions for improving methodologies and eliminating these limitations.

Four-dimensional Multiphase Steady-State MRI with Ferumoxytol Enhancement: Early Multicenter Feasibility in Pediatric Congenital Heart Disease.

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.

Imposition of Fontan physiology: Effects on strain and global measures of ventricular function.

Objective: We sought to evaluate contractile function in single-ventricle patients before and after imposition of Fontan physiology.

Methods: Single right ventricle (SRV; n = 38) and single left ventricle (SLV; n = 11) patients underwent cardiac magnetic resonance imaging pre and post Fontan operation. Global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain were measured along with ejection fraction (EF) and atrioventricular valve regurgitation (AVVR).