Brian D. Soriano

Matrix-Array 3-Dimensional Echocardiographic Assessment of Volumes, Mass, and Ejection Fraction in Young Pediatric Patients With a Functional Single Ventricle A Comparison Study With Cardiac Magnetic Resonance

Background— Quantitative assessment of ventricular volumes and mass in pediatric patients with single-ventricle physiology would aid clinical management, but it is difficult to obtain with 2-dimensional echocardiography. The purpose of the present study was to compare matrix-array 3-dimensional echocardiography (3DE) measurements of single-ventricle volumes, mass, and ejection fraction with those measured by cardiac magnetic resonance (CMR) in young patients. Methods and Results— Twenty-nine patients (median age, 7 months) with a functional single ventricle undergoing CMR under general anesthesia were prospectively enrolled. The 3DE images were acquired at the conclusion of the CMR. Twenty-seven of 29 3DE data sets (93%) were optimal for 3DE assessment. Two blinded and independent observers performed 3DE measurements of volume, mass, and ejection fraction. The 3DE end-diastolic volume correlated well (r=0.96) but was smaller than CMR by 9% (P<0.01), and 3DE ejection fraction was smaller than CMR by 11% (P<0.01). There was no significant difference in measurements of end-systolic volume and mass. The 3DE interobserver differences for mass and volumes were not significant except for ejection fraction (8% difference; P<0.05). Intraobserver differences were not significant. Conclusions— In young pediatric patients with a functional single ventricle, matrix-array 3DE measurements of mass and volumes compare well with those obtained by CMR. 3DE will provide an important modality for the serial analysis of ventricular size and performance in young patients with functional single ventricles.

Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience.

OBJECTIVES The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type. BACKGROUND Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested. METHODS An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses. RESULTS Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy. CONCLUSIONS Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Validation of 3D echocardiographic assessment of left ventricular volumes, mass, and ejection fraction in neonates and infants with congenital heart disease: a comparison study with cardiac MRI.

Background—Quantitative assessment and validation of left ventricular (LV) volumes and mass in neonates and infants with complex congenital heart disease (CHD) is important for clinical management but has not been undertaken. We compared matrix-array 3D echocardiography (3D echo) measurements of volumes, mass, and ejection fraction (EF) with those measured by cardiac MRI in young patients with CHD and small LVs because of either young age or LV hypoplasia. Methods and Results—Thirty-five patients aged <4 years (median, 0.8 years) undergoing MRI were prospectively enrolled. Three-dimensional echo was acquired immediately after MRI, and volume, mass, and EF measurements, using summation of discs methodology, were compared with MRI. Three-dimensional echo end-diastolic volume (24.4±15.7 versus 24.8±46.4 mL; P=0.01; intraclass correlation coefficient [ICC], 0.96) and end-systolic volume (12.3±8.6 versus 9.6±6.8 mL; P<0.001; ICC, 0.90) correlated with MRI with small mean differences (−0.49 mL [P=0.6] and 2.7 mL [P=0.001], respectively). Three-dimensional echo EF was smaller than MRI by 9.3% (P<0.001), and 3D echo LV mass measurements were comparable to MRI (17.3±10.3 versus 17.6±12 g; P<0.77; ICC, 0.93), with a small mean difference (1.1 g; P=0.28). There was good intra- and interobserver reliability for all measurements. Conclusions—In neonates and infants with CHD and small LVs (age appropriate or hypoplastic), matrix-array 3D echo measurements of mass and volumes compare well with MRI, providing an important modality for ventricular size and performance analysis in these patients, particularly in those with left-side heart obstructive lesions.

Validation of 3-Dimensional Echocardiographic Assessment of Left Ventricular Volumes, Mass, and Ejection Fraction in Neonates and Infants with Congenital Heart Disease: A Comparison Study with Cardiac Magnetic Resonance Imaging

Background—Quantitative assessment and validation of left ventricular (LV) volumes and mass in neonates and infants with complex congenital heart disease (CHD) is important for clinical management but has not been undertaken. We compared matrix-array 3D echocardiography (3D echo) measurements of volumes, mass, and ejection fraction (EF) with those measured by cardiac MRI in young patients with CHD and small LVs because of either young age or LV hypoplasia. Methods and Results—Thirty-five patients aged <4 years (median, 0.8 years) undergoing MRI were prospectively enrolled. Three-dimensional echo was acquired immediately after MRI, and volume, mass, and EF measurements, using summation of discs methodology, were compared with MRI. Three-dimensional echo end-diastolic volume (24.4±15.7 versus 24.8±46.4 mL; P=0.01; intraclass correlation coefficient [ICC], 0.96) and end-systolic volume (12.3±8.6 versus 9.6±6.8 mL; P<0.001; ICC, 0.90) correlated with MRI with small mean differences (−0.49 mL [P=0.6] and 2.7 mL [P=0.001], respectively). Three-dimensional echo EF was smaller than MRI by 9.3% (P<0.001), and 3D echo LV mass measurements were comparable to MRI (17.3±10.3 versus 17.6±12 g; P<0.77; ICC, 0.93), with a small mean difference (1.1 g; P=0.28). There was good intra- and interobserver reliability for all measurements. Conclusions—In neonates and infants with CHD and small LVs (age appropriate or hypoplastic), matrix-array 3D echo measurements of mass and volumes compare well with MRI, providing an important modality for ventricular size and performance analysis in these patients, particularly in those with left-side heart obstructive lesions.

Etanercept as adjunctive treatment for acute kawasaki disease: Study design and rationale

BACKGROUND Kawasaki disease (KD) is a multisystem vasculitis affecting coronary arteries in children. Patients, refractory to standard treatment with intravenous immunoglobulin and aspirin, show higher rates of coronary artery dilation. Early tumor necrosis factor α receptor antagonism in KD may provide effective adjunctive therapy. STUDY DESIGN The EATAK trial is a multicenter, double-blind, randomized, and placebo-controlled trial intended to assess the efficacy of etanercept in reducing the intravenous immunoglobulin refractory rate during treatment of acute KD. Each arm will enroll 110 patients who will receive 3 doses of study drug over 2 weeks in conjunction with standard therapy. Coronary artery dilation parameters will serve as secondary end points. DISCUSSION The EATAK trial will test a new paradigm for treatment of acute KD involving tumor necrosis factor α antagonism by etanercept.

Transcatether closure of an atrial redirection baffle leak

We present the case of a woman born with transposition of the great arteries, who was surgically repaired with the Mustard technique. Because of interatrial shunting, she was brought to the cardiac catheterization laboratory for device closure. Matrix-array 3D transesophageal echocardiography enabled visualization of both baffle leaks and demonstrated its orientation in a fashion superior to 2D imaging, had it been used alone. The leaks were successfully closed with a single transcatheter device.

Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and EthnicityCLINICAL PERSPECTIVE: The Pediatric Heart Network Normal Echocardiogram Database

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 for body surface area (BSA) and stratified by age, sex, race, and ethnicity. Methods and Results— Data collected from healthy nonobese children ⩽18 years of age at 19 centers with a normal echocardiogram included age, sex, race, ethnicity, height, weight, echocardiographic images, and measurements performed at the Core Laboratory. Z score models involved indexed parameters (X/BSA&agr;) that were normally distributed without residual dependence on BSA. The models were tested for the effects of age, sex, race, and ethnicity. Raw measurements from models with and without these effects were compared, and <5% difference was considered clinically insignificant because interobserver variability for echocardiographic measurements are reported as ≥5% difference. Of the 3566 subjects, 90% had measurable images. Appropriate BSA transformations (BSA&agr;) were selected for each measurement. Multivariable regression revealed statistically significant effects by age, sex, race, and ethnicity for all outcomes, but all effects were clinically insignificant based on comparisons of models with and without the effects, resulting in Z scores independent of age, sex, race, and ethnicity for each measurement. Conclusions— Echocardiographic Z scores based on BSA were derived from a large, diverse, and healthy North American population. Age, sex, race, and ethnicity have small effects on the Z scores that are statistically significant but not clinically important.

Myocarditis masquerading as acute coronary syndrome: diagnostic role of cardiac MRI

Background Myocarditis presenting as isolated acute chest pain with elevated troponins but normal systolic function by echocardiogram is rare in previously healthy children. Diagnosis is challenging in this situation with significant drawbacks even for the gold standard endomyocardial biopsy. Our study aim is to evaluate the diagnostic role of cardiac MRI in comparison with echocardiography in these patients.

Determinants of Physician, Sonographer, and Laboratory Productivity: Analysis of the Third Survey from the American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity

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 productivity remained stable while total echocardiography volumes increased. This third survey was designed to assess how clinical productivity is associated with laboratory infrastructure elements such as training, administrative tasks, quality improvement, research, and use of focused cardiac ultrasound (FCU). Methods: Survey questions were sent by e‐mail to North American laboratories. The aims were to assess (1) educational and training obligations, (2) academic productivity and research, (3) laboratory medical director satisfaction, (4) quality improvement, (5) laboratory leadership roles, and (6) impact and use of FCU. Survey responses were compared with clinical productivity metrics defined in the first two surveys. Results: There were 38 responses. Academic productivity was higher at institutions with more dedicated imaging personnel, personnel with dedicated protected academic time, and advanced imaging fellows. Academic productivity did not correlate with clinical productivity and was not significantly affected by the presence of dedicated research sonographers. The satisfaction level of laboratory medical directors was related to dedicated administrative time and an administrative stipend. The majority of administrative roles were tasked to the laboratory medical director with support of the technical director. FCU was listed as a hospital privilege at four institutions (13%). Twenty‐two (58%) were training FCU providers in one or more subspecialties. FCU was not associated with clinical or academic productivity. Conclusions: This third survey gathered supplemental data to complement the clinical productivity data collected from the first two surveys. Together, the results of these surveys further describe the range of factors that can affect North American academic pediatric echocardiography laboratories. HIGHLIGHTSA descriptive survey was sent to pediatric academic echocardiography laboratories in 2015.Clinical and academic productivity correlated with amount of resources, with some exceptions.Medical director satisfaction correlated with amount of support.Focused cardiac ultrasound is prevalent in over half of respondents’ centers.

Comparison of two-dimensional and three-dimensional echocardiographic strain in children with CHD

BACKGROUND In CHD, three-dimensional strain analysis may overcome limitations of Doppler and two-dimensional strain of the left ventricle. The aims of this study were to evaluate feasibility and reproducibility of three-dimensional longitudinal, circumferential, and radial systolic strain by three-dimensional speckle-tracking echocardiography compared with two-dimensional echocardiography. METHODS Patients with CHD, biventricular circulation with a systemic left ventricle, and who had two- and three-dimensional imaging performed on the same day from 2010 to 2014 were included. Quantitative two- and three-dimensional strain analyses were performed (two-dimensional cardiac performance analysis version 1.2 and four-dimensional left ventricular analysis version 3.1). Intra- and inter-observer variabilities were calculated on 25 studies. RESULTS A total of 30 patients, including 19 (61%) males, with a median age of 3.6 years (0.1-22 years) were included. The mean fractional shortening was 34.6±5.3%, and the mean ejection fraction was 62.0±6.4%. Measurement of two- and three-dimensional strain was feasible in >95% of segments. Good correlation was observed between longitudinal and circumferential strain (r=0.92, p⩽0.001 and r=0.87, p⩽0.001), but not radial strain (r=0.29, p=0.2). Intra- and inter-observer agreements were better for three-dimensional compared with two-dimensional strain, and better for both two- and three-dimensional longitudinal and circumferential strains compared with radial strain. CONCLUSION Left ventricular three-dimensional strain analysis is feasible in children with CHD. The reproducibility of longitudinal and circumferential strain by three-dimensional analyses is better. Further longitudinal studies are warranted for the potential clinical application of this new technology.