Guidelines for Performing a Comprehensive Pediatric Transthoracic Echocardiogram: Recommendations From the American Society of Echocardiography.

Echocardiography is a fundamental component of pediatric cardiology, and appropriate indications have been established for its use in the setting of suspected, congenital, or acquired heart disease in children. Since the publication of guidelines for pediatric transthoracic echocardiography in 2006 and 2010, advances in knowledge and technology have expanded the scope of practice beyond the use of traditional modalities such as two-dimensional, M-mode, and Doppler echocardiography to evaluate the cardiac segmental structures and their function. Adjunct modalities such as contrast, three-dimensional, and speckle-tracking echocardiography are now used routinely at many pediatric centers. Guidelines and recommendations for the use of traditional and newer adjunct modalities in children are described in detail in this document. In addition, suggested protocols related to standard operations, infection control, sedation, and quality assurance and improvement are included to provide an organizational structure for centers performing pediatric transthoracic echocardiograms.

Myocardial Strain during Surveillance Screening Is Associated with Future Cardiac Dysfunction among Survivors of Childhood, Adolescent and Young Adult-Onset Cancer.

Cardiovascular disease is a leading contributor to mortality among childhood, adolescent and young adult (C-AYA) cancer survivors. While serial cardiovascular screening is recommended in this population, optimal screening strategies, including the use of echocardiography-based myocardial strain, are not fully defined. Our objective was to determine the relationship between longitudinal and circumferential strain (LS, CS) and fractional shortening (FS) among survivors. This single-center cohort study retrospectively measured LS and CS among C-AYAs treated with anthracycline/anthracenedione chemotherapy. The trajectory of LS and CS values over time were examined among two groups of survivors: those who experienced a reduction of >5 fractional shortening (FS) units from pre-treatment to the most recent echocardiogram, and those who did not. Using mixed modeling, LS and CS were used to estimate FS longitudinally. A receiver operator characteristic curve was generated to determine the ability of our model to correctly predict an FS ≤ 27%. A total of 189 survivors with a median age of 14 years at diagnosis were included. Among the two survivor groups, the trajectory of LS and CS differed approximately five years from cancer diagnosis. A statistically significant inverse relationship was demonstrated between FS and LS -0.129, p = 0.039, as well as FS and CS -0.413, p < 0.001. The area under the curve for an FS ≤ 27% was 91%. Among C-AYAs, myocardial strain measurements may improve the identification of individuals with cardiotoxicity, thereby allowing earlier intervention.

Occlusion of the left main coronary artery os by a tethered aortic valve cusp.

We report a 4-month-old girl who presented with poor ventricular function and was found to have occlusion of the left main coronary artery os by a tethered aortic cusp. The patient underwent surgical delamination of the aortic valve leaflet, revealing a normal left coronary os. After operative repair, her left ventricular function improved significantly. This diagnosis should be included in the differential in all children presenting with cardiac dysfunction.

A clinical prediction model to estimate the risk for coarctation of the aorta in the presence of a patent ductus arteriosus.

BACKGROUND: Diagnosing coarctation of the aorta (CoA) in the presence of a patent ductus arteriosus (PDA) may require observation until PDA closure. The aim of this study was to create a model incorporating previously published indices to estimate the probability of neonatal CoA in the presence of a PDA. METHODS: A retrospective "investigation" cohort of 80 neonates was divided into two groups: (1) neonates with PDA and suspicion for CoA requiring observation to confirm the presence or absence of CoA and (2) neonates with PDA and confirmed diagnosis of either CoA or unobstructed aortic arch. Multivariate logistic regression was used to create the coarctation probability model (CPM), which was used to calculate a neonate's probability of CoA. The CPM was validated internally using bootstrapping and subsequently validated prospectively using a "validation" cohort of 74 neonates with PDA. RESULTS: The CPM had an area under the receiver operating characteristic curve of 0.96 and demonstrated good clinical significance in the risk stratification of neonates with PDA and CoA. No neonate with a CPM probability of <15% had CoA after PDA closure. Neonates with CPM probability < 15% were classified at low risk, between 15% and 60% at moderate risk, and >60% at high risk for CoA. CONCLUSIONS: On the basis of these results, the authors recommend measurement of the CPM in all neonates with PDA. Those with CPM probability < 15% no longer require observation, which could decrease observation in as many as half of neonates with unobstructed aortic arches; those with CPM probabilities between 15% and 60% require follow-up imaging, while those with CPM probabilities > 60% should be observed as inpatients until PDA closure.