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This cross-sectional analysis of a national sample of nonprofit, acute care hospital organizations assesses how these organizations distribute spending for community-building activities among the 9 domains allowed by the Internal Revenue Service.

Patient selection criteria for echocardiography with sedation in children are not well defined. We attempted to identify predictors of unplanned repeat echocardiography with sedation. This was a single-center, case–control study of echocardiograms performed in children aged 1–36 months. Cases underwent unplanned repeat examinations with sedation, while controls did not. Patient variables and study indications were compared. Logistic regression identified the most significant predictors. Cases (n = 104, median time to repeat echocardiogram 17 days, median age 12.9 months) were older than controls (n = 212, median age 5.0 months, P

Objective Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. Design Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. Setting Tertiary care children's hospital. Patients Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. Interventions Not applicable. Outcome Measures Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. Results One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). Conclusions In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Item does not contain fulltext Cardiovascular imaging is essential to providing excellent clinical care for girls and women with Turner syndrome (TS). Congenital and acquired cardiovascular diseases are leading causes of the lifelong increased risk of premature death in TS. Non-invasive cardiovascular imaging is crucial for timely diagnosis and treatment planning, and a systematic and targeted imaging approach should combine echocardiography, cardiovascular magnetic resonance and, in select cases, cardiac CT. In recent decades, evidence has mounted for the need to perform cardiovascular imaging in all females with TS irrespective of karyotype and phenotype. This is due to the high incidence of outcome-determining lesions that often remain subclinical and occur in patterns specific to TS. This review provides an overview of state-of-the-art cardiovascular imaging practice in TS, by means of a review of the most recent literature, in the context of a recent consensus statement that has highlighted the role of cardiovascular diseases in these females.

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 Society), is used widely, but has spawned many “short list” versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various “short lists”. In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the “short list” for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.

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 α ) 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 α ) 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.

Background Recently published appropriate use criteria (AUC) for initial pediatric outpatient transthoracic echocardiography (TTE) have not yet been evaluated for clinical applicability. Objectives This study sought to determine the appropriateness of TTE as currently performed in pediatric cardiology clinics, diagnostic yield of TTE for various AUC indications, and any gaps in the AUC document. Methods Data were prospectively collected from patients undergoing initial outpatient TTE in 6 centers. TTE indications (appropriate [A], may be appropriate [M], or rarely appropriate [R]) and findings (normal, incidental, or abnormal) were recorded. Results Of the 2,655 studies ordered by 102 physicians, indications rated A, M, and R were found in 1,876 (71%), 316 (12%), and 319 studies (12%), respectively, and 144 studies (5%) were unclassifiable. Twenty-four of 113 indications (21%) were not used. Innocent murmur and syncope or palpitations with no other indications of cardiovascular disease, a benign family history, and normal electrocardiogram accounted for 75% of indications rated R. Pathologic murmur had the highest yield of abnormal findings (40%). Odds of an abnormal finding in an A or M TTE were 6 times that of R (95% confidence interval [CI]: [2.8 to 12.8]). Abnormal findings were more common in patients 10 years of age (odds ratio: 6.4; 95% CI: 4.7 to 8.7). Age was a significant predictor of an abnormal finding after adjusting for indication and site (p Conclusions Most TTEs ordered in pediatric cardiology clinics were for indications rated A. AUC ratings successfully stratified indications based on the yield of abnormal findings. This study identified differences in the yield of TTE based on patient age and most common indications rated R. These findings should inform quality improvement efforts and future revisions of the AUC document.

Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS, Chair [∗][1] Pamela S. Douglas, MD, MACC, FAHA, FASE, Moderator [∗][1] Louis I. Bezold, MD, FACC, FAAP, FASE[†][2] William B. Blanchard, MD, FACC, FAHA, FAAP[∗][1] Jeffrey R. Boris, MD, FACC[∗][1] Bryan Cannon, MD[‡][3] Gregory J.

The American College of Cardiology (ACC) and the American Heart Association (AHA) support their members’ goal to improve the care of patients with cardiovascular disease through professional education, research, and development of guidelines and standards and by fostering policies that support optimal patient outcomes. The ACC and AHA recognize the importance of the use of clinical data standards for patient management, assessment of outcomes, and conduct of research, and the importance of defining the processes and outcomes of clinical care, whether in randomized trials, observational studies, registries, or quality improvement initiatives. Clinical data standards strive to define and standardize data relevant to clinical concepts, with the primary goal of facilitating uniform data collection by providing a platform of clinical terms with corresponding definitions and data elements. Broad agreement on a …

Background Syncope is a common reason for outpatient transthoracic echocardiography (TTE). We studied the applicability of pediatric appropriate use criteria (AUC) on initial outpatient evaluation of children (≤18 years) with syncope. Methods Data were obtained before (Phase I, April–September 2014) and after (Phase II, January-April 2015) the release of the AUC document from six participating pediatric cardiology centers. Site investigators determined the indication for TTE and assigned appropriateness rating based on the AUC document: Appropriate (A), May Be Appropriate (M), Rarely Appropriate (R), or “unclassifiable” (U) if it did not fit any scenario in the AUC document. Results Of the total 4562 TTEs, 310 (6.8%) were performed for syncope: 174/2655 (6.6%) Phase I and 136/1907 (7.1%) Phase II, P=.44. Overall, 168 (50.5%) were for indications rated A, 63 (18.9%) for M, 79 (23.7%) for R, and 23 (6.9%) for U. Release of AUC did not change the appropriateness of TTEs [A=51.6% vs 49.0%, P=.63, R=20.2% vs 28.3%, P=.09]. Overall syncope-related R indications formed 15.7% of R indications for all the echocardiograms performed in the entire Pediatric Appropriate Use (PAUSE) study (11.9% Phase I and 22.4% Phase II, P=.002). TTEs were normal in majority of the patients except 7 that had incidental findings. Conclusions In conclusion, syncope is a common reason for indications rated R and release of the AUC document did not improve appropriate utilization of TTE in syncope. Targeted educational interventions are needed to reduce unnecessary TTEs in children with syncope.

We reviewed echocardiography literature for the assessment and management of semilunar valve disease in children. A search was performed within the National Library of Medicine using the keywords aortic stenosis (AS), aortic regurgitation, pulmonary stenosis (PS), and pulmonary regurgitation in children. The search was further refined adding the keywords-pediatric, neonates, echocardiographic definition, classification, evaluation. Thirty-eight studies were included. For stenotic lesions, there were sufficient consistencies between Doppler and invasive gradients (especially for PS), while other quantitative parameters used in adults showed significant limitations when applied to children. Heterogeneities remain in the range of Doppler measurements utilized to define mild vs moderate vs severe AS/PS, and to guide management. There is sufficient consensus regarding indications for interventions. In regurgitant lesions, there is weak evidence supporting the use of quantitative or semiquantitative parameters after correction for body surface area; clear indications for intervention are lacking. Because adult echocardiographic recommendations cannot be simply translated to the pediatric age, more specific pediatric guidelines and standards for the assessment of semilunar valve disease are needed.

After heart transplantation (HT) in infants and young children, environmental and intrinsic factors may lead to changes in the geometry and compliance of the donor heart. Serial demographic, clinical, hemodynamic, and echocardiographic data were obtained from HT recipients younger than 4 years of age. Echocardiographic chamber measurement z-scores were compared using recipient body surface area from the time of HT to 1 week, 3 months, and last follow-up visit. Left ventricular end-diastolic volume (LVEDV) z-scores were correlated with pulmonary capillary wedge pressure (PCWP) at each time point. Heart transplantation was performed for 13 children between March 2009 and December 2012, 9 of whom (69%) were boys. The median age at HT was 8 months (range, 4-43 months), and the mean follow-up period was 13 ± 7 months. Left ventricular end-diastolic dimension z-scores decreased significantly (p = 0.03) between HT and 1 week, then increased from 1 week to 3 and 12 months. (-1.32 ± 1.7, -0.71 ± 1.8, 0.41 ± 2.1, 0.79 ± 2.3, respectively). A positive relationship (R(2) = 0.48) between the LVEDV z-score and PCPW was present at the last follow-up visit. For infants and young children, the allograft demonstrates appropriate growth by 1 year after HT. Left ventricular compliance improves over time.

Background— Girls and women with Turner syndrome are at risk for aortic dissection and rupture. However, the size of the aorta and the clinical characteristics among those with Turner syndrome and dissection have received little attention. Methods and Results— We obtained medical records from 20 individuals who voluntarily participated in the International Turner Syndrome Aortic Dissection Registry. Type A dissections occurred in 17 of 20 (85%) cases, and type B occurred in 3 cases of which 1 occurred after coarctation stent placement. Of those with spontaneous aortic dissections, 18 of 19 (95%) had an associated cardiac malformation that included a bicuspid aortic valve. In 1 individual there was no predisposing finding other than the presence of Turner syndrome. Associated pregnancy was documented in 1 of 19 (5%). More than half (13/19, 68%) came to medical attention >24 hours after the onset of symptoms. For those with type A dissections, the mean ascending aortic size index was 2.7±0.6 cm/m 2 (n=9). Conclusions— Aortic dissection in Turner syndrome occurs in young individuals at smaller aortic diameters than in the general population or other forms of genetically triggered aortopathy. The absence of aortic valve or other cardiac malformations appears to markedly reduce the risk of aortic dissection However, aortic dissection can occur in Turner syndrome without cardiac malformations or hypertension. Individuals with Turner syndrome who are >18 years of age with an ascending aortic size index >2.5 cm/m 2 should be considered for an aortic operation to prevent aortic dissection.

The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics.Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE.Pediatric cardiology clinics at six centers.Those ≤18 years old, receiving initial outpatient TTE.EI included (i) sharing the pre-EI appropriateness ratings with providers, (ii) lecture on AUC, (iii) providers self-assigning indications, and (iv) monthly e-mail feedback by SI to individual providers.The primary outcome measure was a change in the proportion of studies for indications rated R following EI.Of the 4542 TTEs (1907 pre-EI, 2635 post-EI) ordered by 90 physicians, overall comparison of appropriateness ratings before and after EI showed an increase in Appropriate (72.5%-76.2%, P = .004), no change in May Be Appropriate, and a decline in Rarely Appropriate (R) from 9.6% to 7.4%, P = .008. Following EI, a significant decline in R was observed only in three centers and EI did not affect the variation in TTEs ordered for R indications among physicians (P = .467). Physicians with the highest proportion of TTEs ordered for R before EI, showed the most significant decline in R.Appropriateness of pediatric outpatient TTE varies substantially by center. A customized EI resulted in modest improvement in the appropriateness of TTEs in the PAUSE study, with an increase in Appropriate and a decrease in R TTEs. Multifaceted EIs are required to improve adherence to national standards such as AUC.

Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time.Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery.Tertiary care children's hospital.Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries.Not applicable.Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis.One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P .05).In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Three-dimensional echocardiography (3DE) has become important in the management of patients with congenital heart disease (CHD), particularly with pre-surgical planning, guidance of catheter intervention, and functional assessment of the heart. 3DE is increasingly used in children because of good acoustic windows and the non-invasive nature of the technique. The aim of this paper is to provide a review of the optimal application of 3DE in CHD including technical considerations, image orientation, application to different lesions, procedural guidance, and functional assessment.

Obesity and left ventricular hypertrophy (LVH) have been identified as independent risk factors for cardiovascular events. The definition of LVH depends on the geometric algorithm used to calculate LV mass (LVM) by echocardiography and the method used to normalize LVM for body size. This study evaluates the effect of these methods on the prevalence of LVH in obese children. LVM for 109 obese and 109 age-matched non-obese children was calculated using M-mode or two-dimensional echocardiography (2DE). LVM was then normalized to height 2.7 as indexed LVM (LVMI), to body surface area (BSA), height, and lean body mass (LBM) as LVM Z-scores. LVH was defined as LVMI >95th ‰ using age-specific normal reference values or LVM Z-scores ≥2. The prevalence of LVH by LVMI and LVM Z-scores was compared. There was a correlation between LVM determined by M-mode and by 2DE (R (2) = 0.91), although M-mode LVM was greater than 2DE LVM. However, the difference between these values was greater in obese children than in non-obese children. Based on the method of normalization, the prevalence of LVH among obese children was 64 % using LVMI, 15 % using LVM Z-scores for height, 8 % using LVM Z-scores for BSA and 1 % using LVM Z-scores for LBM. Height-based normalization correlates with obesity and hypertension. The methods used to measure and normalize LVM have a profound influence on the diagnosis of LVH in obese children. Further study is needed to determine which method identifies children at risk for cardiovascular morbidity and mortality.

The American Society of Echocardiography has recently published guidelines for chamber and vessel quantification during a pediatric echocardiogram. Because the sizes of cardiovascular structures increase with somatic growth, pediatric quantification requires the adjustment of measurements for the effects of body size in order to determine when a measurement is outside the normal range. A universal pediatric normative database of measurements from a normal population encompassing the full range of body sizes encountered in pediatrics would provide clinicians and researchers a powerful tool to distinguish normal from abnormal values and aid in the management of children with heart disease. However, there are still challenges in the complete acceptance of the recommended pediatric protocols for each cardiac segment, mostly because of historical standard practices, differences with the quantification guidelines for adults, and varying methodologies utilized in currently available pediatric normative databases.

The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.

Neonates with critical heart disease are at risk of significant deficiencies in systemic oxygen delivery. The incidence and clinical pattern of hyperlactatemia in neonates presenting with critical heart disease has not been described. We reviewed the lactate pattern of neonates transferred to our cardiac intensive care unit for surgical management of their heart disease over a 1-year period. Stabilization of these neonates began in the referring institutions. From 8/4/03 to 8/4/04, 75 neonates with critical heart disease were transferred to our unit for stabilization and subsequent surgery. Blood lactate was measured on admission and subsequently in any patient thought to be at risk of low systemic oxygen delivery. Lactate was measured in 59 patients on admission and in 63 patients within the first 48 h of admission. Median age on admission was 1 day (range 0–13). Median age at surgery was 8 days (range 1–30). Median length of stay was 20 days. Peak lactate was noted on admission in 51 patients, and at 12–24 h in 8 patients. Mild hyperlactatemia (2.3–5 mmol/l) was present in 30 patients on admission and moderate-to-severe hyperlactatemia (≧5 mmol/l) was present in 8 patients. Mean lactate level on admission was 3.1 ± 0.6 mmol/l, and this did not return to normal (

Recently published appropriate use criteria (AUC) for initial pediatric outpatient transthoracic echocardiography (TTE) have not yet been evaluated for clinical applicability.This study sought to determine the appropriateness of TTE as currently performed in pediatric cardiology clinics, diagnostic yield of TTE for various AUC indications, and any gaps in the AUC document.Data were prospectively collected from patients undergoing initial outpatient TTE in 6 centers. TTE indications (appropriate [A], may be appropriate [M], or rarely appropriate [R]) and findings (normal, incidental, or abnormal) were recorded.Of the 2,655 studies ordered by 102 physicians, indications rated A, M, and R were found in 1,876 (71%), 316 (12%), and 319 studies (12%), respectively, and 144 studies (5%) were unclassifiable. Twenty-four of 113 indications (21%) were not used. Innocent murmur and syncope or palpitations with no other indications of cardiovascular disease, a benign family history, and normal electrocardiogram accounted for 75% of indications rated R. Pathologic murmur had the highest yield of abnormal findings (40%). Odds of an abnormal finding in an A or M TTE were 6 times that of R (95% confidence interval [CI]: [2.8 to 12.8]). Abnormal findings were more common in patients1 year of age than in those10 years of age (odds ratio: 6.4; 95% CI: 4.7 to 8.7). Age was a significant predictor of an abnormal finding after adjusting for indication and site (p0.001).Most TTEs ordered in pediatric cardiology clinics were for indications rated A. AUC ratings successfully stratified indications based on the yield of abnormal findings. This study identified differences in the yield of TTE based on patient age and most common indications rated R. These findings should inform quality improvement efforts and future revisions of the AUC document.

Indicators of quality services in pediatric and congenital echocardiography include structural, process, and outcome factors. Structural indicators involve the available resources related to the facility, the equipment, and the staff. Process indicators relate primarily to the activities and tasks associated with echocardiography, and these include patient selection as well as study performance, interpretation, and reporting. Outcome indicators involve the results of quality services and are intimately related to quality improvement activities as well as quality metrics. Current quality improvement activities in the field of pediatric and congenital echocardiography include development of the accreditation process for sonographers, physicians, and echocardiography laboratories; evaluation of productivity standards within echocardiography laboratories; identification of appropriate use criteria specific to pediatric and congenital echocardiography; and establishment of a robust database of normal reference values for cardiovascular measurements in children. Candidate quality metrics currently in development involve reporting of critical results, adverse events with sedated studies, variability of echocardiographic measurements, diagnostic errors, study completeness, and image quality.

A set of dicephalous parapagus twins was born at 36 wk gestational age. Twin A had complex congenital heart disease in the form of a single ventricle that would be fatal without surgical intervention. Twin B had normal intracardiac anatomy. The twins were deemed surgically inseparable and the parents requested palliative open-heart surgery on Twin A. The twins shared a common circulatory system to the lower half of the body so that physiological manipulations in one twin adversely affected the other's hemodynamic condition. Twin A underwent successful single ventricle palliation; however, after a prolonged hospitalization, the infants died secondary to severe respiratory disease in Twin B.

The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.

Coronary heart disease and stroke are two leading causes of morbidity and mortality. Risk factors have included obesity, metabolic syndrome, hypertension, and diabetes mellitus, and they appear to influence left ventricular mass, geometry, and systolic and diastolic function as well as left atrial size. Echocardiography is a non-invasive tool to assess these effects in children and should be utilized as a preventive as well as a diagnostic modality, as measurements from echocardiography may define an intermediate phenotype between risk and clinical endpoints. Information regarding the pathophysiologic mechanisms of the echocardiographic changes, the risk for future cardiovascular morbidity and mortality, and the effects of intervention can be derived from research.

n Society of Echocardiography designates this educational activity for of 1.5 AMA PRA Category 1 Credits . Physicians should only claim credit te with the extent of their participation in the activity. CCI recognize the ASE’s certificates and have agreed to honor the credit their registry requirements for sonographers. n Society of Echocardiography is committed to ensuring that its educan and all sponsored educational programs are not influenced by the special y corporation or individual, and itsmandate is to retain only those authors ial interests can be effectively resolved to maintain the goals and educaty of the activity. Although amonetary or professional affiliationwith a cors not necessarily influence an author’s presentation, the Essential Areas and e ACCME require that any relationships that could possibly conflict with al value of the activity be resolved prior to publication and disclosed to . Disclosures of faculty and commercial support relationships, if any, dicated. ience: is designed for all cardiovascular physicians and cardiac sonographers with erest and knowledge base in the field of echocardiography; in addition, reschers, clinicians, intensivists, and other medical professionals with a spein cardiac ultrasound will find this activity beneficial.

We report the first implantation of two Helex septal occluder devices in a 10-year-old boy with complex atrial septal anatomy. The unique characteristics of this new device are discussed in the following case report.

Anomalies of the aortic and pulmonary outflow tracts are usually associated with obstruction, regurgitation, and/or aneurysmal dilation of the proximal great arteries, and they represent some of the conditions most frequently encountered by congenital heart disease specialists. In most instances, a full preoperative diagnosis is performed by standard transthoracic echocardiography and other imaging modalities such as cardiac catheterization and magnetic resonance imaging. However, transesophageal echocardiography (TEE) serves an important role in the perioperative management of these patients. Preoperative TEE can provide information regarding the morphology of the outflow tracts and the degree of obstruction and regurgitation; postoperative TEE can evaluate the success of a surgical procedure and exclude residual obstruction, regurgitation, or other potential complications. In addition, TEE is sometimes necessary outside of the operating room setting for older patients with poor transthoracic echocardiographic windows, particularly if the patient has undergone prior surgery. This chapter discusses the use of TEE for the evaluation of both right and left sided outflow tract anomalies.

Haemodynamically insignificant left-to-right shunts are frequently discovered when screening for congenital heart disease, resulting in significant economic and psychosocial impact. A literature search was performed within the National Library of Medicine using the keywords small/insignificant/silent atrial septal defect (ASD) , ventricular septal defect (VSD) , patent ductus arteriosus (PDA) and patent foramen ovale (PFO). The search was refined by adding the keywords definition , classification and follow-up . Our analysis revealed significant heterogeneity in the evaluation and management of innocent left-to-right shunts. The definitions for small defects vary greatly, making it difficult to distinguish between physiologic and pathologic lesions (eg, a PFO vs a true ASD). Most small defects will partially or completely resolve spontaneously early in life. If spontaneous resolution does not occur, the risk for long-term complications (such as embolic events and endocarditis) is low but poses several practical and ethical issues: immediate discharge versus long-term follow-up, duration and frequency of follow-up, and content and method of discussions with the parents. Additionally, there is controversy pertaining to treatment for PDAs and VSDs, particularly among interventional cardiologists, even though risk/benefit analyses are lacking. Standards and guidelines using consensus opinion for the management of insignificant left-to-right shunts are needed to address the heterogeneity in diagnosis and management as well as use of resources, ethical and psychosocial issues.

Strategies for monitoring patients recovering after congenital heart surgery have evolved considerably as technology continues to progress. Monitoring techniques traditionally centered around the comprehensive physical examination have been replaced by a number of revolutionary technologies developed to objectively evaluate various components of the cardiovascular system. Despite scant evidence that these methodologies actually improve outcomes, some have been embraced by clinicians. We developed an Internet survey designed to describe current practices of clinicians who care for patients after congenital heart surgery. There were 162 respondents to our survey with the majority from the United States. The views of cardiologists, intensivists, those dual trained in both cardiology and critical care medicine, and surgeons are all robustly represented in the results. Serial lactate monitoring was the strategy that was utilized most often by respondents (94%), followed by multisite near-infrared spectrometry (NIRS, 67%). There were 78% who utilized the combination of serial lactate and NIRS monitoring. Serial lactate monitoring was the technique that was thought to best represent cardiovascular well-being after heart surgery (40%). The results of this survey suggest that despite the paucity of evidence that clinical outcomes of patients recovering after congenital heart surgery are improved by any of these monitoring techniques, there is almost universal acceptance to monitor patients with serial lactate monitoring, NIRS monitoring, or a combination of these techniques.

OBJECTIVE: The aim of this study was to evaluate the diagnostic and prognostic accuracy of brain natriuretic peptide assay in neonates and children undergoing surgery for congenital heart disease. DESIGN: Prospective, observational study. SETTING: Single center. PATIENTS: We enrolled 336 consecutive children (median age, 6 mo [range, 0-37 mo]) undergoing cardiac surgery (87 neonates; age, 7 d [5-12]; median, 25th-75th percentile; 24 infants and children; age, 11 mo [4-60]) and 436 healthy controls. INTERVENTIONS: Brain natriuretic peptide was measured preoperatively, on every postoperative day in the ICU, and at discharge. Intubation time was the primary outcome. MEASUREMENTS AND MAIN RESULTS: Preoperative brain natriuretic peptide values in patients with congenital heart disease were higher than those in controls (p < 0.01). Brain natriuretic peptide had a good diagnostic accuracy in discriminating between patients with congenital heart disease and healthy controls with an area under the curve = 0.918 for neonates and area under the curve = 0.894 for older children. The best cutoff values, calculated by receiver operating characteristic analysis, were different for the two age subgroups with cutoff values of 363.5 ng/L for neonates and 23.5 ng/L for older children. At 24 hours after surgery, although brain natriuretic peptide decreased in neonates (baseline 2723 vs 1290 ng/L, p < 0.001), it increased in children (60 vs 365 ng/L at 24 hours, p < 0.001). Multivariable analysis identified the preoperative level of brain natriuretic peptide in infant/children and the difference in brain natriuretic peptide value (baseline 24 hours) in neonates, as independent predictors of intubation time. Furthermore, body surface area, Aristotle score, and cardiopulmonary bypass time had an independent significant effect on the endpoint in either group. CONCLUSIONS: Baseline cardiac endocrine function and its response to surgical stress are dependent on age in neonates and children, undergoing cardiac surgery for congenital heart disease. Brain natriuretic peptide shows a good diagnostic and prognostic accuracy in this setting, with different features in either neonates or infants/children subsets.

Interest in diastolic function in children has increased recently. However, the strengths and limitations of published pediatric nomograms for echocardiographic diastolic parameters have not been critically evaluated, especially in the neonatal population. A literature search was performed within the National Library of Medicine using the keywords normal/reference values, power Doppler/tissue Doppler velocities, and children/neonates. The search was further refined by adding the keywords diastolic function, myocardial, mitral/tricuspid inflow, pulmonary vein, and Tei index. Thirty-three published studies evaluating diastolic function in normal children were included in this review. In many studies, sample sizes were limited, particularly in terms of neonates. There was heterogeneity in the methodologies to perform and normalize measurements and to express normalized data (Z scores, percentiles, and mean values). Although most studies adjusted measurements for age, classification by specific age subgroups varied, and few addressed the relationships of measurements to body size and heart rate (especially with higher neonatal heart rates). Although reference values were reproducible in older children, they varied significantly in neonates and infants. Pediatric diastolic nomograms are limited by small sample sizes and inconsistent methodologies for the performance and normalization of measurements, with few data on neonates. Some studies do reveal reproducible patterns in diastolic function in older children. A comprehensive pediatric nomogram of diastolic function involving a large population of normal infants and older children and using standardized methodology is warranted and would have tremendous impact in the care of children with acquired and congenital heart disease.

Syncope is a common reason for referral to pediatric cardiologists and to perform transthoracic echocardiography (TTE). We studied the applicability of pediatric appropriate use criteria (AUC) on initial outpatient evaluation of children with syncope before and after the release of the AUC document