Shubhika Srivastava

AI DuPont Nemours Heart Center Delaware

Background The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. Methods and Results The American College of Cardiology Quality Network ass...

Entrustable professional activities (EPAs) are “observable essential tasks expected to be performed by a physician for safe patient care in practice.” Six Pediatric Cardiology (PC) EPAs and their level of supervision (LOS) scales were developed by medical educators in PC using a modified Delphi process and reviewed by the Subspecialty Pediatrics In...

Introduction: Cardiovascular (CV) involvement in multisystem inflammatory syndrome in children (MISC) is frequent (80-85%). Data on 2D speckle tracking echocardiography and its value in predicting CV outcomes are limited. Hypothesis: We hypothesized that an association exists between 2D speckle tracking strain/strain rate parameters at admission an...

Background: Entrustable Professional Activities (EPAs) are observable, essential tasks that subspecialists are expected to perform for safe and effective patient care. Pediatric Cardiology (PC) specific EPAs have been developed, but their general use in assessment for PC fellows for graduation requirements has yet to be studied. Objective: To dete...

The premise of the recommendations listed in the Society for Maternal-Fetal Medicine (SMFM) Consensus Statement #641 regarding the risk of fetal atrioventricular block (AVB) owing to maternal anti-Ro/SSA (anti–Sjögren-syndrome related antigen A) antibodies is that surveillance by echocardiography is unwarranted and unnecessary because treatment is...

Left ventricular vortex formation optimizes the effective transport of blood volume while minimizing energy loss (EL). Vector flow mapping (VFM) derived EL patterns have not been described in children < 1 year. A prospective cohort of 66 (0 days - 22 years, 14 patients ≤ 2 months) cardiovascularly normal children was used to determine LV vortex num...

Estimation of the right ventricular systolic pressure (RVSP) using echocardiographically derived maximal instantaneous gradient across the ventricular septal defect is a well-utilized tool, which can potentially underestimate the RVSP in patients with conduction abnormalities as such its utility in these patients is questionable.

Background The FUEL trial (Fontan Udenafil Exercise Longitudinal) demonstrated statistical improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle function in this cohort has not been studied. Methods The 400 enrolled participants wer...

Appropriate use criteria (AUC) for the performance of outpatient transthoracic echocardiography are based on history, physical examination and electrocardiograms. Telehealth provides access to clinical care in the absence of a physical examination. We assessed application of AUC for performance of echocardiography following a telehealth visit. In t...

Background: Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions. Methods:...

Background: Understanding the clinical course and short-term outcomes of suspected myocarditis following COVID-19 vaccination has important public health implications in the decision to vaccinate youth. Methods: We retrospectively collected data on patients <21 years-old presenting before 7/4/2021 with suspected myocarditis within 30 days of COVID-...

FOXP1 syndrome is a neurodevelopmental disorder caused by mutations or deletions that disrupt the forkhead box protein 1 (FOXP1) gene, which encodes a transcription factor important for the early development of many organ systems, including the brain. Numerous clinical studies have elucidated the role of FOXP1 in neurodevelopment and have character...

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA COVID-19 vaccine-associated myopericarditis (VAM) was noted. Only one adolescent with life-threatening complications was reported with no deaths at any of the participating institutions.

Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is rare with varied associated cardiac defects and rhythm abnormalities. We aimed to describe the natural history, associated anomalies and prenatal outcome in a cohort of prenatally diagnosed patients in which biventricular repair is anticipated. Methods: A retrospect...

Background Obesity is associated with left ventricular (LV) hypertrophy and cardiac dysfunction. In obese adults with congenital heart disease (ACHD), a sensitive echocardiographic marker like strain might enable early detection of subclinical cardiac changes. Objectives To assess the association of elevated body mass index (BMI) with global longi...

Background and objectives: Entrustable Professional Activities (EPAs) were developed to assess pediatric fellows. We previously showed that fellowship program directors (FPDs) may graduate fellows who still require supervision. How this compares with their expectations for entrustment of practicing subspecialists is unknown. Methods: We surveyed...

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, with an incidence of 32.6 per 100,000live births. The embryologic theory supports Van Praagh et al.'s hypothesis that the primary morphologic abnormality in TOF is an underdeveloped subpulmonary infundibulum. Genetic syndromes in the absence of abnormal pulmonary artery...

This chapter reviews the imaging of coronary artery anomalies of importance to the pediatric cardiologist with a focus on echocardiography. Embryologic development of the coronary arteries extends from the endocardial sinuses to the epicardial network. There are normally two major coronary arteries, which originate from aortic sinuses that “face” t...

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface ar...

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who u...

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized world...

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code ( IPCCC ) is now utilized wor...

Objective(s) To determine if telehealth acceptance by parents of children with heart disease is predicted by sociodemographic and/or by parental digital literacy; and to assess parental perceptions of TH usability and reliability. Study design We conducted a single center study comparing TH acceptance versus visit cancellation/rescheduling for ped...

Introduction: The Pediatric Heart Network’s Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT 02741115) demonstrated improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle (SV) function in this cohort ha...

Background: The incidence of congenital mitral valve disease is 0.4%; Double Orifice Mitral Valve (DOMV) and Parachute Mitral Valve (PMV) are two morphologic pathologies that may result in mitral valve dysfunction. The objectives of this study are 1) To describe valve function and progression and 2) To define factors contributing to disease progres...

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic...

Background The accuracy of fetal echocardiography (FE) is not well defined, and reporting of diagnostic discrepancies (DDs) is not standardized. The authors applied a categorization scheme developed by the American College of Cardiology Quality Metric Working Group and applied it to FE. Methods A retrospective single-center study was conducted of...

We report one of the earliest known U.S. cases of multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C). This adolescent male presented prior to any known association between COVID-19 and immune mediated inflammatory syndrome in children. He presented in stable condition and without significant multisystem involvement. Duri...

Echocardiogram is the first-line and most used noninvasive imaging diagnostic modality and is convenient and reliable in its assessment of ventricular function in pediatrics. Ventricular function is an important physiological parameter representing myocardial performance in response to overall hemodynamic status. Here, we introduce basic principles...

The World Health Organization declared the novel coronavirus, or COVID-19, a pandemic in March 2020. Given the severity of COVID-19, appropriate use criteria have been implemented for fetal echocardiography. Screening low risk pregnancies for critical congenital heart disease has typically been a shared responsibility by pediatric cardiologists, ob...

The field of pediatric cardiology is rich with abnormal anatomy, unique congenital malformations and unusual surgical corrections that can be challenging to adequately image. The comprehensive pediatric echocardiogram is uniquely different from our adult colleagues and requires a systematic segmental approach to evaluate a wide range of structural...

A cyanotic neonate with tetralogy of Fallot was found to have a congenitally inverted pulmonary valve. Diagnosis was made via echocardiography and cardiac catheterization. The valve opened retrograde into the right ventricle, which allowed severe regurgitation and prevented anterograde flow. This report is the first description of this anomaly in m...

Background The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies. Methods Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations. Memoranda, Core Lab repor...

Congenital coronary artery anomalies, both in isolation and associated with other forms of congenital heart disease, have been recognized as important lesions with significant potential morbidity and mortality, including sudden cardiac death in children and adolescents. Multimodality imaging techniques have demonstrated increasing utility in the ch...

Background At the end of training, fellowship program directors (FPD) attest that graduating fellows are competent to practice without supervision, but there are no agreed upon criteria on which to base this decision. Entrustable Professional Activities (EPAs) may provide an approach to determine if fellows are meeting a set standard. We examined w...

Background: The reliability of left ventricular (LV) systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of LV size and systolic function in children wit...

Background In 2013, outpatient use of chloral hydrate (CH) was limited and other alternatives such as oral pentobarbital (PB) were explored to achieve conscious sedation in young children for transthoracic echocardiography (TTE). We aimed to assess efficacy and safety of the two medications. Methods Clinical information, from a computerized databa...

Objective: To assess health-related quality of life (HRQOL) in a large multicenter cohort of children and young adults with Marfan syndrome participating in the Pediatric Heart Network Marfan Trial. Study design: The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales were administered to 321 subjects with Marfan syndrome (5-25...

Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotationa...

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past nine...

Right atrial (RA) size is a prognostic indicator for heart failure and cardiovascular death in adults. Data regarding use of RA area (RAA) by two-dimensional echocardiography as a surrogate for RA size and allometric modeling to define appropriate indexing of the RAA are lacking. Our objective was to validate RAA as a reliable measure of RA size an...

Background: The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity was formed in 2011 to study institutional factors that could influence the clinical productivity of physicians and sonographers in academic pediatric echocardiography laboratories. In the previous two surveys, staff clinical product...

The mitral valve apparatus comprises of the annulus, leaflets, commissure, chordal apparatus, papillary muscles, and left ventricular myocardium, all of which work together as a single functional unit in conjunction with the atrium, which serves as the inflow, and the left ventricle, which supports the papillary muscle architecture. Abnormality in...

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Ec...

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Socie...

Background: Published nomograms of pediatric echocardiographic measurements are limited by insufficient sample size to assess the effects of age, sex, race, and ethnicity. Variable methodologies have resulted in a wide range of Z scores for a single measurement. This multicenter study sought to determine Z scores for common measurements adjusted f...

3D imaging datasets from cardiac computed tomography (CT), magnetic resonance imaging (CMR), or echocardiography can be used to create 3D virtual models for 3D printing. Although CT imaging is not considered first line to diagnose congenital heart disease, it has significant advantages when a 3D printed cardiac model is needed. Advantages over ultr...

Assessment of left ventricular dilation plays an important role in the management of left ventricular volume overload lesions. Various echocardiographic methods exist, such as the 5/6 area-length and biplane Simpsons, but their agreement with cardiac magnetic resonance imaging in patients with chronic left ventricular volume overload from a young a...

The number of pediatric cardiology trainees in the United States doubled between 2004 and 2015, but there is a lack of information on the current pediatric cardiology workforce. Therefore, a survey was administered to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and to non-duplicative board-certified or bo...

Background: The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity aimed to study factors that could influence the clinical productivity of physicians and sonographers and assess longitudinal trends for the same. The first survey results indicated that productivity correlated with the total volume...

Background: Antenatal diagnosis of congenital heart defects (CHD) can impact outcomes in neonates with severe CHD. Obstetric screening guidelines and the indications for fetal echocardiography (FE) have evolved in an attempt to improve the early prenatal detection of CHD. Analyzing yield for specific indications will help clinicians better stratif...

Background: Anomalous aortic origin of a coronary artery from the wrong Sinus of Valsalva (AAOCA) is a rare congenital anomaly and is associated with sudden cardiac death. Morphologic features considered to be "high risk" are significant luminal narrowing, acute coronary angulation at its origin, intramural course, and long interarterial course. A...

Cardiac magnetic resonance (CMR) assesses myocardial involvement in myocarditis (MYO). Current techniques are qualitative, subjective, and prone to interpretation error. Feature tracking (FT) analyzes myocardial strain using CMR and has not been examined in MYO. We hypothesize that regional left ventricular (LV) strain is abnormal in MYO. Regional...

Methods: All patients at a single centre from 2001 to 2012 with a diagnosis of cardiomyopathy who underwent echocardiography and catheterisation within 30 days were included in this study. The correlation between tricuspid regurgitation gradient and catheterisation-derived right ventricular systolic pressure and mean pulmonary artery pressure was...

19th Annual Update on Pediatric and Congenital Cardiovascular Disease. (Children’s Hospital of Philadelphia Meeting). Meeting attended by more than 800 pediatric cardiologists, intensivists, surgeons, nurses, and others involved in the care of patients with pediatric and congenital cardiovascular disease.

Survival of patients with tetralogy of Fallot (TOF)-the most common cyanotic heart defect- has improved dramatically in the past decades. Echocardiography is the primary imaging modality for diagnosing TOF and is crucial for anatomic and functional assessments before and after therapeutic interventions. Following a review of the developmental and g...

Background: The NHLBI Pediatric Heart Network randomized trial of atenolol vs. losartan in Marfan Syndrome demonstrated no significant treatment difference in the rate of change in body surface area adjusted maximum aortic root diameter z score (AoRz). Objectives: To report trial results on aortic stiffness and to determine whether aortic stiffness...

Objectives This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a “best practice” protocol for imaging and...

Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications. Large defect size and deficient rims have been described as predictors for complications and unsuccessful device placement in closure of interatrial communications. MASP is an underappreciated morphologic atrial septal variation whose prevalenc...

Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset a...

### 1.1 Document Development Process The Society of Pediatric Cardiology Training Program Directors (SPCTPD) board assembled a steering committee that nominated 2 chairs, 1 SPCTPD steering committee member, and 4 additional experts from a wide range of program sizes, geographic regions, and

The presence of myocardial late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) imaging in concert with electrocardiography and elevated biomarkers helps support the diagnosis of acute myocarditis. Two-dimensional echocardiography is limited to global and qualitative regional function assessment and may not contribute to the diagno...

Autism spectrum disorder (ASD) and intellectual disability (ID) can be caused by mutations in a large number of genes. One example is SHANK3 on the terminal end of chromosome 22q. Loss of one functional copy of SHANK3 results in 22q13 deletion syndrome or Phelan-McDermid syndrome (PMS) and causes a monogenic form of ASD and/or ID with a frequency o...