Continuous Murmur in a Child: Sometimes It Is a Zebra.

• Most AVFs present with a continuous murmur with radiation to the back. • There is little evidence to guide management of thoracic AVF. • Management options include surgical repair, embolization, or conservative management.

Reference data for left ventricular filling and atrial function in children using cardiovascular magnetic resonance.

Background: Diastolic dysfunction is associated with morbidity and mortality in multiple pediatric disease processes. Cardiovascular magnetic resonance (CMR) provides a non-invasive method of studying left ventricular (LV) diastolic dysfunction through the assessment of LV filling curves and left atrial (LA) volume and function. However, there are no normative data for LV filling curves and the standard method is time-intensive.

Association of cardiovascular magnetic resonance diastolic indices with arrhythmia in repaired Tetralogy of Fallot.

Background: Patients with repaired Tetralogy of Fallot (rTOF) experience a high burden of long-term morbidity, particularly arrhythmias. Cardiovascular magnetic resonance (CMR) is routinely used to assess ventricular characteristics but the relationship between CMR diastolic function and arrhythmia has not been evaluated. We hypothesized in rTOF, left ventricular (LV) diastolic dysfunction on CMR would correlate with arrhythmias and mortality.

Congenitally Corrected Transposition Cardiac Surgery: Society of Thoracic Surgeons Database Analysis.

Background: Congenitally corrected transposition of the great arteries (ccTGA) has many management strategies, with the emergence of anatomic repair increasing the available surgical options. Contemporary surgical practices have not been described in multicenter analyses. This study describes the distribution of heart surgery in patients with ccTGA and defines contemporary outcomes in a large multicenter cohort.

Comparison of Strain-Encoding and Feature-Tracking Derived Myocardial Deformation Assessment of Left Ventricular Function in a Pediatric and Adult Congenital Heart Disease Cohort.

Background: Cardiac magnetic resonance (CMR) strain can be assessed with feature-tracking (FT), which utilizes a post-processing algorithm to quantify myocardial deformation on routine cine images, and strain-encoding magnetic resonance imaging (SENC), which uses parallel magnetization tags combined with out-of-plane phase-encoding gradients to quantify deformation. Assessing agreement is critical to determine whether results can be translated between methods. We compared SENC to FT in the assessment of left ventricle (LV) global longitudinal strain (GLS) and global circumferential strain (GCS) in a cohort of pediatric and adult congenital heart disease (ACHD) patients.

Characteristics of COVID-19 Myocarditis With and Without Multisystem Inflammatory Syndrome.

Multisystem inflammatory syndrome (MIS) is a severe complication described in a minority of patients with COVID-19. Myocarditis has been reported in patients with COVID-19, including MIS. In this study, we compared the clinical characteristics and cardiac magnetic resonance (CMR) findings of COVID-19 myocarditis in patients with and without MIS.

Assessment of gadolinium deposition in the brain tissue of pediatric and adult congenital heart disease patients after contrast enhanced cardiovascular magnetic resonance.

Background: Contrast enhanced magnetic resonance imaging (MRI) is an important tool for the assessment of extracardiac vasculature and myocardial viability. Gadolinium (Gd) brain deposition after contrast enhanced MRI has recently been described and resulted in a warning issued by the United States Food and Drug Administration. However, the prevalence of brain deposition in children and adults with congenital heart disease (CHD) undergoing cardiovascular magnetic resonance (CMR) is unclear.

Tissue characterisation and myocardial mechanics using cardiac MRI in children with hypertrophic cardiomyopathy.

Introduction: Distinguishing between hypertrophic cardiomyopathy and other causes ofleft ventricular hypertrophy can be difficult in children. We hypothesised that cardiac MRI T1 mapping could improve diagnosis of paediatric hypertrophic cardiomyopathy and that measures of myocardial function would correlate with T1 times and extracellular volume fraction.

Methods: Thirty patients with hypertrophic cardiomyopathy completed MRI with tissue tagging, T1-mapping, and late gadolinium enhancement.

Congenital Heart Surgery Outcomes in Turner Syndrome: The Society of Thoracic Surgeons Database Analysis.

Background: Turner syndrome (TS) is a genetic syndrome characterized by monosomy X (45,XO) in phenotypic females and is commonly associated with congenital heart disease. We sought to describe the distribution, mortality, and morbidity of congenital heart surgery in TS and compare outcomes to individuals without genetic syndromes.

Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to evaluate index cardiovascular operations performed from 2000 to 2017 in pediatric patients (aged 0-18 years) with and without TS.

Left main coronary artery stent placement in a 7.0 kg infant with Williams Syndrome.

We report the case of a 9-month-old male with Williams syndrome who underwent patch augmentation of supravalvar aortic stenosis and pulmonary artery stenosis, and required emergent drug-eluting left coronary artery stenting on post-operative day 1 for severe left ventricular dysfunction related to myocardial ischemia.

The Role of Matrix Metalloproteinases and Tissue Inhibitors of Metalloproteinases in Duchenne Muscular Dystrophy Cardiomyopathy.

Background: Cardiomyopathy is the leading cause of death in Duchenne muscular dystrophy (DMD). Standard cardiac biomarkers are poor indicators of DMD cardiovascular disease. Matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) regulate collagen turnover.

Assessment of brain-derived neurotrophic factor and osteopontin in a healthy pediatric population.

Biomarkers are routinely used for noninvasive identification or monitoring of disease processes in clinical practice, as well as surrogate end points for drug development. There is a significant lack of data regarding biomarkers in children. An understanding of biomarker levels in a healthy pediatric cohort is essential as more studies begin to apply noninvasive biomarkers to pediatric populations.

Heart Transplantation in Children with Turner Syndrome: Analysis of a Linked Dataset.

Turner syndrome (TS) patients with hypoplastic left heart syndrome (HLHS) have poor single ventricle palliation outcomes; therefore, consideration of other potential management strategies is important. Little is known about heart transplantation (HTx) in this group, as standard HTx databases do not allow for identification of TS. This study describes experiences and outcomes of HTx in TS using a unique linkage between the Scientific Registry of Transplant Recipients and the Pediatric Health Information System databases.

Genetics of Pulmonary Arterial Hypertension.

Tremendous progress has been made in understanding the genetics of pulmonary arterial hypertension (PAH) since its description in the 1950s as a primary disorder of the pulmonary vasculature. Heterozygous germline mutations in the gene coding bone morphogenetic receptor type 2 (BMPR2) are detectable in the majority of cases of heritable PAH, and in approximately 20% of cases of idiopathic pulmonary arterial hypertension (IPAH). However, recent advances in gene discovery methods have facilitated the discovery of additional genes with mutations among those with and without familial PAH.

Percutaneous Removal of Intravascular Pellet Following Penetrating Cardiac Trauma.

There is controversy regarding the management of projectile embolization, a rare complication of penetrating trauma. We present the case of a 5-year-old, 20 kg male with retrograde venous projectile embolization following traumatic injury with a pellet gun. The projectile was successfully removed utilizing a novel, percutaneous approach.

Macrophage EP4 deficiency increases apoptosis and suppresses early atherosclerosis.

Prostaglandin (PG) E(2), a major product of activated macrophages, has been implicated in atherosclerosis and plaque rupture. The PGE(2) receptors, EP2 and EP4, are expressed in atherosclerotic lesions and are known to inhibit apoptosis in cancer cells. To examine the roles of macrophage EP4 and EP2 in apoptosis and early atherosclerosis, fetal liver cell transplantation was used to generate LDLR(-/-) mice chimeric for EP2(-/-) or EP4(-/-) hematopoietic cells.