Use of Everolimus to treat cardiac rhabdomyomas and incessant arrhythmias in a newborn: Benefits and complications.

We report treating a term neonate with tuberous sclerosis and giant rhabdomyomas who presented with incessant supraventricular tachycardia with Everolimus. The treatment was efficient in reducing tumor size and assisted as an adjunct therapy in controlling arrhythmia and limiting preexcitation. Treatment was challenged by difficulty to achieve stable drug level and limited by neutropenia as a serious side effect.

A unique cardiovascular presentation of Marfan syndrome.

We report a neonate with severe Marfan syndrome (MS), prenatally identified to have persistent atrial tachycardia, biventricular dysfunction, and an unusual structure within the atria. Detailed postnatal echocardiographic evaluation and cross-sectional imaging confirmed congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa. Emergent testing by next-generation sequencing identified a FBN1 pathological variant, key to establishing goals of care.

Persistent Cardiac Magnetic Resonance Imaging Findings in a Cohort of Adolescents with Post-Coronavirus Disease 2019 mRNA Vaccine Myopericarditis.

We describe the evolution of cardiac magnetic resonance imaging findings in 16 patients, aged 12-17 years, with myopericarditis after the second dose of the Pfizer mRNA coronavirus disease 2019 vaccine. Although all patients showed rapid clinical improvement, many had persistent cardiac magnetic resonance imaging findings at 3- to 8-month follow-up.

The Diagnostic Role of Echocardiographic Strain Analysis in Patients Presenting with Chest Pain and Elevated Troponin: A Multicenter Study.

Background: Myocarditis presenting as acute chest pain with elevated troponins without significant cardiac compromise is rare in previously healthy children, often referred to as myopericarditis. Diagnosis is challenging, as conventional echocardiographic measures of systolic function can be normal. The aim of this study was to demonstrate the diagnostic utility of strain imaging in this scenario.

Echocardiographic assessment of right ventricular volume in repaired tetralogy of Fallot: a novel approach to an older technique.

Background: In repaired tetralogy of Fallot (rTOF), right ventricular (RV) enlargement leads to poor outcomes. However, evaluating the RV has limitations; cardiac magnetic resonance (CMR) and 3D echocardiography have barriers including cost and accessibility. Traditional echocardiography is limited given the complex geometry and anterior location of the RV.

Multisystem Inflammatory Syndrome in Children: Survey of Protocols for Early Hospital Evaluation and Management.

Objective: To describe the similarities and differences in the evaluation and treatment of multisystem inflammatory syndrome in children (MIS-C) at hospitals in the US.

Study Design: We conducted a cross-sectional survey from June 16 to July 16, 2020, of US children's hospitals regarding protocols for management of patients with MIS-C. Elements included characteristics of the hospital, clinical definition of MIS-C, evaluation, treatment, and follow-up.

Tripartite Assessment of Right Ventricular Systolic Function in Persistent Pulmonary Hypertension of the Newborn.

Non-invasive evaluation of right ventricular (RV) systolic function in neonates with pulmonary hypertension (PH) with traditional metrics including RV fractional area change (FAC) and tricuspid annular systolic plane excursion (TAPSE) has improved outcomes. Apical three-chamber (3C) RV-FAC, a novel tripartite assessment of the RV, has recently been described in healthy infants. We assess the utility of 3C RV-FAC and biplane RV-FAC in delayed transitioning and neonatal PH.

Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management.

Tetralogy of Fallot (ToF) with pulmonary atresia (ToF-PA) is a complex congenital heart defect at the extreme end of the spectrum of ToF, with no antegrade flow into the pulmonary arteries. Patients differ with regard to the sources of pulmonary blood flow. In the milder spectrum of disease, there are confluent branch pulmonary arteries fed by ductus arteriosus.

The Early "Unnatural" History Following Surgical Repair of Ventricular Septal Defects.

Introduction: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed.

Methods: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed.

Preoperative Physiology, Imaging, and Management of Coarctation of Aorta in Children.

Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs.

Coarctation of Aorta in Children.

Coarctation of aorta (CoA) is a discrete narrowing in aorta causing obstruction to the flow of blood. It accounts for 6-8% of all congenital heart diseases. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally.

Common Arterial Trunk: Physiology, Imaging, and Management.

Common arterial trunk (CAT), or truncus arteriosus, is a rare form of cyanotic congenital heart disease and is highly associated with DiGeorge syndrome (microdeletion 22q11.2). Prenatal diagnosis is highly feasible, allowing proper delivery planning and postnatal management.

Ubiquitin C-Terminal Hydrolase 1 and Phosphorylated Axonal Neurofilament Heavy Chain in Infants Undergoing Cardiac Surgery: Preliminary Assessment as Potential Biomarkers of Brain Injury.

Background: There are no reliable markers to assess brain injury in neonates following cardiac surgery. We examine ubiquitin C-terminal hydrolase 1 (UCHL1) and phosphorylated axonal neurofilament heavy chain (pNF-H), neuronal-specific biomarkers released following axonal and cortical injury, in neonates undergoing cardiac surgery involving cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA).

Methods: Twenty-six patients younger than three months were prospectively enrolled (CPB only, n = 12 and DHCA, n = 14).

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy.

"Chorea": A Unique Presentation of Left Atrial Myxoma in a Pediatric Patient.

We report an unusual presentation of a large left atrial myxoma in an eight-year-old girl who presented with the sudden onset of chorea. This case illustrates the fact that the presentation of chorea in nonendemic areas for rheumatic fever should raise suspicion for a myxoma. The chorea resolved soon after removal of the myxoma, supporting the hypothesis of an immune-mediated mechanism, or manifestation of paraneoplastic syndrome secondary to the myxoma.

Variations in rotation of the aortic root and membranous septum with implications for transcatheter valve implantation.

Objective: It is intuitive to suggest that knowledge of the variation in the anatomy of the aortic root may influence the outcomes of transcatheter implantation of the aortic valve (TAVI). We have now assessed such variation.

Methods: We used 26 specimens of normal hearts and 78 CT data sets of adults with a mean age of 64±15 years to measure the dimensions of the membranous septum and to assess any influence played by rotation of the aortic root, inferring the relationship to the atrioventricular conduction axis.

Surgical planning for a complex double-outlet right ventricle using 3D printing.

Rapid prototyping may be beneficial in properly selected cases of complex congenital heart disease, providing detailed anatomical understanding that helps to guide potential surgical and cardiac catheterization interventions. We present a case of double-outlet right ventricle, where the decision to obtain a three-dimensional printed model helped for better understanding of the anatomy, with the additional advantage of surgical simulation in planning the surgical approach and type of surgical repair.

The Significance of the Interleaflet Triangles in Determining the Morphology of Congenitally Abnormal Aortic Valves: Implications for Noninvasive Imaging and Surgical Management.

A comprehensive understanding of the normal and abnormal aortic root is paramount if we are to improve not only our assessment of the aortic root and its components but also the surgical approach to reconstructing this complex structure when congenitally malformed. Most anatomic and imaging-based classifications of the normal root recognize and describe the basic components, which include the shape and size of the three aortic sinuses and their three valvar leaflets, as well as the sinutubular junction and proximal ascending aorta. However, the three interposing fibrous interleaflet triangles, which share an intimate relationship with all elements of the root, are often ignored.

Subacute left ventricular outflow tract obstruction after transapical closure of a mitral paravalvular leak in the region of the aortomitral curtain.

We report a rare case of progressive left ventricular outflow tract (LVOT) obstruction after percutaneous device closure of a mechanical prosthetic mitral valve (MV) paravalvular leak (PVL) in the region of aortomitral curtain in a patient who also had small mechanical aortic valve prosthesis with patient-prosthesis mismatch.

Understanding the spectrum of sinus venosus interatrial communications.

Background: It is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects.

Relationship of ambulatory blood pressure and body mass index to left ventricular mass index in pediatric patients with casual hypertension.

Both obesity and hypertension are associated with left ventricular hypertrophy (LVH) in children. Our objective was to compare the prevalence of LVH in obese and nonobese subjects with casual hypertension who underwent ambulatory blood pressure monitoring (ABPM). Untreated children (aged 6-20 years) underwent 24-hour ABPM, and left ventricular mass index (LVMI) was measured.

Retrograde transcatheter device closure of a complex paravalvular leak after bioprosthetic pulmonary valve replacement in a pediatric patient.

We report a case of retrograde transcatheter device closure of a complex paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement (PVR) in a 13-year-old patient with congenital pulmonary valve stenosis. There are prior reports of pulmonary PVL closure after PVR in adults (Seery and Slack, Congenit Heart Dis 2014;9:E19-F22), but indications for and technical considerations in PVL closure after bioprosthetic PVR, particularly in children, are not well defined. © 2015 Wiley Periodicals, Inc.