Santosh Uppu

Non-invasive cardiac imaging like echocardiogram, cardiac magnetic resonance imaging (CMR), and computed tomography angiography (CTA) play a key role in the diagnosis, aid in management and follow-up of congenital heart disease patients. Normative data for intracardiac and extracardiac vascular structures in children are currently available for ech...

Purpose Cardiac Magnetic resonance (CMR) derived left ventricular longitudinal and circumferential strain is known to be abnormal in myocarditis. CMR strain is a useful additional tool that can identify subclinical myocardial involvement and may help with longitudinal follow-up. Right ventricular strain derived by CMR in children has not been studi...

Purpose: CMR derived left ventricular longitudinal and circumferential strain is known to be abnormal in myocarditis. CMR strain is an useful additional tool that can identify subclinical myocardial involvement and may help with longitudinal follow-up. Right ventricular strain derived by CMR in children has not been studied. We sought to evaluate C...

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...

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:...

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...

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: 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...

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.

Background: Cardiovascular magnetic resonance (CMR) is increasingly used to diagnose myocarditis in adults but its use in children is not well-established. We sought to describe the presentation, CMR protocol and findings, and outcomes in a multicenter cohort of children with myocarditis. Methods: Thirteen hospitals retrospectively identified patie...

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...

Racial variability in certain prenatal risk factors, such as prenatal vitamin supplementation and termination of pregnancy for fetal anomaly, has altered the racial prevalence of congenital malformation (CM). Analysis of a single large representative population is required to analyze current racial differences in prevalence of CM in the United Stat...

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...

Background Congenital malformation (CM) is a leading cause of infant mortality. We hypothesized that the current estimates of the prevalence of CM are obsolete because of the increased rate of terminating fetuses with severe CMs and the widespread use of prenatal vitamins. Methods This population-based cross-sectional study analyzed the effect of...

This work aimed to analyze a single large representative population to determine racial/ethnic differences in the prevalence of congenital anomalies (CA). This is a population-based comprehensive analysis of racial/ethnic differences in the prevalence of CA diagnoses. We reviewed all live births in the 2008 Nationwide Inpatient Sample (NIS) databas...

Although the epidemiology of congenital heart disease (CHD) has been described, the authors believe changes in prenatal factors such as termination of pregnancy for fetal anomaly and prenatal vitamin supplementation have altered the birth prevalence of severe CHD. This population-based study reviewed the Nationwide Inpatient Sample (NIS) database a...

The authors hypothesized that changes in prenatal factors such as termination of pregnancy for fetal anomalies and prenatal vitamin supplementation have altered the epidemiology of patients with multiple congenital anomalies and may have had an impact on their prevalence in the current era. This study reviewed the Nationwide Inpatient Sample databa...

Background: There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other...

This is a longitudinal analysis of the largest and most comprehensive inpatient care database in the United States to determine temporal variation of birth prevalence of congenital heart disease (CHD) diagnosis, adjusting for potentially confounding factors. We compared all entries of CHD diagnoses in the Nationwide Inpatient Sample (NIS) database...

Current estimates of the incidence of congenital heart disease (CHD) are derived from small clinical studies and metaanalyses. For the true incidence of CHD in the United States of America to be estimated, a single large representative population must be analyzed. All the data in this study were derived from the Nationwide Inpatient Sample database...

Cardiovascular magnetic resonance (CMR) is increasingly used to diagnose myocarditis in adults but its use in children is not well-established. We sought to describe the presentation, CMR protocol and findings, and outcomes in a multicenter cohort of children with myocarditis. Thirteen hospitals retrospectively identified patients meeting the follo...

Background: Primary repair of tetralogy of Fallot has low surgical mortality, but some patients still experience significant postoperative morbidity. Our objectives were to review our institutional experience with primary tetralogy of Fallot repair, and identify predictors of intensive care unit morbidity. Methods: We reviewed all patients with...

A 2-year-old boy with an incidental finding of massive cardiomegaly on a chest X-ray was diagnosed with a giant right atrial aneurysm upon further investigation with echocardiography. The patient underwent successful surgical reduction of the right atrium and closure of the patent foramen ovale to prevent thromboembolic complications and to lower t...

Our objective was to evaluate morbidity and mortality associated with extracorporeal membrane oxygenation (ECMO) in children with genetic syndromes and heart disease. We conducted a retrospective review of all children with heart disease and genetic syndromes receiving ECMO during the period January 2000 and March 2012 at Arkansas Children's Hospit...

Background: Some neonates with tetralogy of Fallot (TOF) have rapid progression of right ventricular outflow tract obstruction, requiring early repair irrespective of Doppler gradient as measured in the neonatal period. The aim of this study was to test the hypothesis that infundibular morphology in neonates with TOF is associated with the occurre...

The occurrence of spontaneous subarachnoid haemorrhage (SAH) in association with coarctation of thoracic aorta and absence of intracranial aneurysm is a rare association. In spontaneous SAH, the predominant cause is intracranial aneurysmal rupture. This report describes a case of a 40 year-old male who presented with SAH and was incidentally diagno...

The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1 day-18 years who received cardiac surgery for interrupted aortic arch (IAA), tetra...

Interrupted aortic arch is a rare congenital heart disease, whereas right-sided interrupted aortic arch is an extremely rare disorder with few reported cases in the literature. We report our institutional experience with two such patients and review the recent literature.

Ventricular tachycardia (VT) arising from the coronary sinus cusps and mimicking right-ventricular morphology is a rare entity. In this report, we report the successful cryoablation of left-coronary sinus cusp VT.

This is a report of a 49-year-old male, who presented with typical signs and symptoms of constrictive pericarditis. He was diagnosed with sinus venosus atrial septal defect (ASD) and anomalous right upper pulmonary venous return during his adolescence, which was elected not to be repaired. During the attempted repair of the ASD it was noted there w...