Leo Lopez

All Publications

• Guidelines for Performing a Comprehensive Pediatric Transthoracic Echocardiogram: Recommendations From the American Society of Echocardiography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Lopez, L., Saurers, D. L., Barker, P. C., Cohen, M. S., Colan, S. D., Dwyer, J., Forsha, D., Friedberg, M. K., Lai, W. W., Printz, B. F., Sachdeva, R., Soni-Patel, N. R., Truong, D. T., Young, L. T., Altman, C. A. 2024; 37 (2): 119-170

  Abstract

  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.

  View details for DOI 10.1016/j.echo.2023.11.015

  View details for PubMedID 38309834

• Defining the Spectrum of Hypoplastic Left Heart Syndrome (HLHS). World journal for pediatric & congenital heart surgery Jacobs, J. P., Bleiweis, M. S., Cohen, M. S., Lopez, L., Peek, G. J., Franklin, R. C., Tchervenkov, C. I. 2023; 14 (6): 741-745

  Abstract

  The 2021 International Paediatric and Congenital Cardiac Code and the Eleventh Revision of the International Classification of Diseases provide the following definition for hypoplastic left heart syndrome (HLHS): "Hypoplastic left heart syndrome (HLHS) is defined as a spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch." Although HLHS with intact ventricular septum (HLHS+IVS) and HLHS with ventricular septal defect (HLHS+VSD) are different cardiac phenotypes, both of these lesions are part of the spectrum of HLHS.

  View details for DOI 10.1177/21501351231189277

  View details for PubMedID 37933696

• Fetal Echocardiographic Z-Score Pilot Project: Study Design and Impact of Gestational Age and Variable Type on Reproducibility of Measurements Within and Across Investigators. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Moon-Grady, A. J., Lee, H., Lopez, L., Fatusin, O., Freud, L. R., Hogan, W., Krishnan, A., McFarland, C., LuAnn Minich, L., Morris, S. A., Pinto, N., Presson, A. P., Tacy, T. A., Donofrio, M. T. 2023

  Abstract

  Fetal echocardiography is widely available but normative data are not robust. In this pilot study, we evaluated 1) the feasibility of pre-specified measurements in a normal fetal echocardiogram to inform study design and 2) measurement variability to assign thresholds of clinical significance and guide analyses in larger fetal echocardiography Z-score initiatives.Images from pre-defined gestational age groups (16-20 weeks; >20-24 weeks; >24-28 weeks; and >28-32 weeks) were retrospectively analyzed. Fetal echocardiography expert raters attended online group training then independently analyzed 73 fetal studies (18 per age group) in a fully crossed design of 53 variables; each observer repeated measures for 12 fetuses. Kruskal-Wallis tests were used to compare measurements across centers and age groups. Coefficients of variation (CoV) were calculated at the subject level for each measurement as the ratio of standard deviation (SD) to mean. Intraclass correlation coefficients (ICC) were used to show inter- and intrarater reliabilities. Cohen's d>0.8 was used to define clinically important differences. Measurements were plotted against gestational age, biparietal diameter, and femur length.Expert raters completed each set of measurements in a mean (SD) of 23(9) minutes per fetus. Missingness ranged from 0% to 29%. CoV was similar across age groups for all variables (P<0.05), except ductus arteriosus mean velocity and left ventricular ejection time which were both higher at older gestational age. CoV was >15% for right ventricular systolic and diastolic widths despite fair to good repeatability (ICC>0.5); ductal velocities and 2D measures, left ventricular short axis dimensions, and isovolumic times all had high CoV and high interobserver variability despite good to excellent intraobserver agreement (ICC>0.6). CoV did not improve when ratios (e.g. tricuspid:mitral annulus) were used instead of linear measurements. Overall, 27 variables had acceptable inter- and intraobserver repeatability, while 14 had excessive variability between readers despite good intraobserver agreement.There is considerable variability in fetal echocardiographic quantification in clinical practice that may affect the design of multicenter fetal echocardiography Z-score studies, and not all measurements may be feasible for standard normalization. As missingness was substantial, a prospective design will be needed. Data from this pilot study may aid in the calculation of sample sizes and inform thresholds for distinguishing clinically significant from statistically significant effects.

  View details for DOI 10.1016/j.echo.2023.05.010

  View details for PubMedID 37302438

• Appropriateness of cardiovascular computed tomography and magnetic resonance imaging in patients with conotruncal defects. Journal of cardiovascular computed tomography Pickard, S. S., Armstrong, A. K., Balasubramanian, S., Buddhe, S., Crum, K., Kong, G., Lang, S. M., Lee, M. V., Lopez, L., Natarajan, S. S., Norris, M. D., Parra, D. A., Parthiban, A., Powell, A. J., Priromprintr, B., Rogers, L. S., Sachdeva, S., Shah, S. S., Smith, C. A., Stern, K. W., Xiang, Y., Young, L. T., Sachdeva, R. 2023

  Abstract

  To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications.Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. To incorporate patient characteristics and center-level effects, a hierarchical generalized linear mixed model was used.Of the 1753 studies (80% CMR, and 20% CCT), 16% were rated M/R. Center M/R ranged from 4 to 39%. Infants accounted for 8.4% of studies. In multivariable analyses, patient- and study-level factors associated with M/R rating included: age <1 year (OR 1.90 [1.15-3.13]), truncus arteriosus (vs. tetralogy of Fallot, OR 2.55 [1.5-4.35]), and CCT (vs. CMR, OR 2.67 [1.87-3.83]). None of the provider- or center-level factors reached statistical significance in the multivariable model.Most CMRs and CCTs ordered for the follow-up care of patients with conotruncal defects were rated appropriate. However, there was significant center-level variation in appropriateness ratings. Younger age, CCT, and truncus arteriosus were independently associated with higher odds of M/R rating. These findings could inform future quality improvement initiatives and further exploration of factors resulting in center-level variation.

  View details for DOI 10.1016/j.jcct.2023.01.044

  View details for PubMedID 36868899

• Video-Based Deep Learning for Automated Assessment of Left Ventricular Ejection Fraction in Pediatric Patients. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Reddy, C. D., Lopez, L., Ouyang, D., Zou, J. Y., He, B. 2023

  Abstract

  Significant interobserver and interstudy variability occurs for left ventricular functional indices despite standardization of measurement techniques. Artificial intelligence models trained on adult echocardiograms are not likely to be applicable to a pediatric population. We present EchoNet-Peds, a video-based deep learning algorithm, which matches human expert performance of left ventricular (LV) segmentation and ejection fraction (EF).A large pediatric dataset of 4,467 echocardiograms were used to develop EchoNet-Peds. EchoNet-Peds was trained on 80% of the data for segmentation of the left ventricle and estimation of left ventricular EF. The remaining 20% was used to fine tune and validate the algorithm.In both apical 4-chamber (A4C) and parasternal short-axis views (PSAX), EchoNet-Peds segments the left ventricle with a Dice similarity coefficient of 0.89. EchoNet-Peds estimates EF with a mean absolute error of 3.66% and can routinely identify pediatric patients with systolic dysfunction (area under the curve of 0.95). EchoNet-Peds was trained on pediatric echocardiograms and performed significantly better to estimate EF (p < 0.001) than an adult model applied to the same data.Accurate, rapid automation of EF assessment and recognition of systolic dysfunction in a pediatric population are feasible using EchoNet-Peds with the potential for far-reaching clinical impact. In addition, the first large pediatric dataset of annotated echocardiograms is now publicly available for efforts to develop pediatric-specific artificial intelligence algorithms.

  View details for DOI 10.1016/j.echo.2023.01.015

  View details for PubMedID 36754100

• Quality Improvement in a Pediatric Echocardiography Laboratory: A Collaborative Process. Children (Basel, Switzerland) Sganga, D., Behera, S., Beattie, M. J., Stauffer, K. J., Burlinson, A., Lopez, L., Tierney, E. S. 2022; 9 (12)

  Abstract

  Transthoracic echocardiography (TTE) is an essential tool for diagnosis and management of congenital heart disease. Pediatric echocardiography presents unique challenges including complex anatomy, variable patient cooperation and provider expertise. Diagnostic errors inevitably occur. We designed a collaborative and stepwise quality improvement (QI) process to address diagnostic errors within our laboratory. We retrospectively reviewed medical records to identify diagnostic TTE errors in 100 consecutive cardiac surgery patients ≤ 5 years old (July 2020-January 2021). We identified 18 diagnostic errors. Most errors had minor impact (14/18), and 13 were preventable or possibly preventable. We presented these results to our sonographers and faculty and requested input on preventing and managing diagnostic errors. Our root cause analysis based on their responses yielded 7 areas for improvement (imaging, reporting, systems, time, environment, people, QI processes). Our faculty and sonographers chose QI processes and imaging as initial areas for intervention. We defined our SMART goal as a 10% reduction in diagnostic errors. We implemented interventions focused on QI processes. On initial follow up in May 2022, we identified 7 errors in 70 patients (44% reduction in error rate). Utilizing a stepwise and team-based approach, we successfully developed QI initiatives in our echocardiography laboratory. This approach can serve as a model for a collaborative QI process in other institutions.

  View details for DOI 10.3390/children9121845

  View details for PubMedID 36553289

  View details for PubMedCentralID PMC9776848

• Targeted Neonatal Echocardiography in Patients With Hemodynamic Instability. Pediatrics Rahde Bischoff, A., Bhombal, S., Altman, C. A., Fraga, M. V., Punn, R., Rohatgi, R. K., Lopez, L., McNamara, P. J. 2022; 150 (Suppl 2)

  Abstract

  Targeted neonatal echocardiography (TNE) has been increasingly used at the bedside in neonatal care to provide an enhanced understanding of physiology, affecting management in hemodynamically unstable patients. Traditional methods of bedside assessment, including blood pressure, heart rate monitoring, and capillary refill are unable to provide a complete picture of tissue perfusion and oxygenation. TNE allows for precision medicine, providing a tool for identifying pathophysiology and to continually reassess rapid changes in hemodynamics. A relationship with cardiology is integral both in training as well as quality assurance. It is imperative that congenital heart disease is ruled out when utilizing TNE for hemodynamic management, as pathophysiology varies substantially in the assessment and management of patients with congenital heart disease. Utilizing TNE for longitudinal hemodynamic assessment requires extensive training. As the field continues to grow, guidelines and protocols for training and indications are essential for ensuring optimal use and providing a platform for quality assurance.

  View details for DOI 10.1542/peds.2022-056415I

  View details for PubMedID 36317979

• Intraoperative epicardial echocardiography or transoesophageal echocardiography in CHD: how much does it matter? Cardiology in the young Stauffer, K. J., Brabender, J., Reddy, C. D., Selamet Tierney, E. S., Lopez, L., Maeda, K., Navaratnam, M., Punn, R. 2022: 1-8

  Abstract

  BACKGROUND: Intraoperative imaging determines the integrity of surgical repairs. Transoesophageal echocardiography represents standard care for intraoperative imaging in CHD. However, some conditions preclude its use, and epicardial echocardiography is used alternatively. Minimal literature exists on the impact of epicardial echocardiography versus transoesophageal echocardiography. We aimed to evaluate accuracy between the two modalities and hypothesised higher imaging error rates for epicardial echocardiography.METHODS: We retrospectively reviewed all epicardial echocardiograms performed over 16 years and compared them to an age- and procedure-matched, randomly selected transoesophageal echocardiography cohort. We detected un- or misidentified cardiac lesions during the intraoperative imaging and evaluated patient outcomes. Data are presented as a median with a range, or a number with percentages, with comparisons by Wilcoxon two-sample test and Fisher's exact test.RESULTS: Totally, 413 patients comprised the epicardial echocardiography group with 295 transoesophageal echocardiography matches. Rates of imaging discrepancies, re-operation, and incision infection were similar. About 13% of epicardial echocardiography patients had imaging discrepancies versus 16% for transoesophageal (p = 0.2352), the former also had smaller body sizes (p < 0.0001) and more genetic abnormalities (33% versus 19%, p < 0.0001). Death/mechanical support occurred more frequently in epicardial echocardiography patients (16% versus 6%, p < 0.0001), while hospitalisations were longer (25 versus 19 days, p = 0.0003).CONCLUSIONS: Diagnostic accuracy was similar between patients undergoing epicardial echocardiography and transoesophageal echocardiography, while rates of death and mechanical support were increased in this inherently higher risk patient population. Epicardial echocardiography provides a reasonable alternative when transoesophageal echocardiography is not feasible.

  View details for DOI 10.1017/S1047951122001536

  View details for PubMedID 35747949

• Leftward and Posterior Deviation of the Septum Primum Predicts Morbidity in Patients with Hypoplastic Left Heart Syndrome. Pediatric cardiology Gill, K., Lopez, L., Taylor, T., Muniz, J. C. 2022

  Abstract

  Leftward posterior deviation of the atrial septum primum (LDSP) has been reported in up to 64% of patients with hypoplastic left heart syndrome (HLHS) but there are no published data on its impact on neonatal outcomes. We reviewed the prevalence of LDSP and its correlation with neonatal outcomes in our institution. This was a single-center retrospective study of neonates with HLHS from 2001 to 2019. Echocardiograms were reviewed and the presence or absence of LDSP was noted. To quantify the degree of deviation in patients with LDSP, a new measurement, the deviation index (DI) was calculated using both the subcostal long and short-axis views. Of ninety-four patients with HLHS, fifty-seven (61%) patients were noted to have LDSP. There was no statistically significant difference in gestational age (GA), birth weight (BW), or mortality between patients with and without LDSP. Patients with LDSP had an increased incidence of unplanned reoperation (p<0.01), post-operative cardiac catheterization (p<0.05), and post-operative infection (p<0.05). After correction for GA, BW, HLHS subtype, and type of surgery, LDSP predicted reoperation (OR=3.6, p<0.01), catheterization (OR=2.7, p=0.05), and infection (OR=3.4, p<0.05). Higher degree of deviation predicted reoperation (DI>0.17), catheterization (DI>0.07), and infection (DI>0.12). There was excellent inter-observer reproducibility of the DI (ICCabsolute-agreement=0.82, ICCconsistency=0.90). Patients with LDSP have a higher prevalence of post-operative morbidity. The degree of deviation was found to be predictive of post-operative complications. Pre-operative echocardiographic evaluation of LDSP in patients with HLHS may be helpful in risk stratification and counseling.

  View details for DOI 10.1007/s00246-022-02860-w

  View details for PubMedID 35316357

• Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies. Pediatric cardiology Duong, S. Q., Zaniletti, I., Lopez, L., Sutherland, S. M., Shin, A. Y., Collins, R. T. 1800

  Abstract

  Heterotaxy is a complex, multisystem disorder associated with single ventricle heart disease and decreased survival. Ciliary dysfunction is common in heterotaxy and other situs abnormalities (H/SA) and may increase post-operative complications. We hypothesized that patients with H/SA have increased respiratory and renal morbidities and increased in-hospital mortality after Fontan procedure. We queried the Pediatric Health Information System database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 through 11years from 2004 to 2019. H/SA was identified by codes for dextrocardia, situs inversus, asplenia/polysplenia, or atrial isomerism and compared to non-H/SA controls. Outcomes were in-hospital mortality or heart transplantation, ECMO, hemodialysis, length of stay (LOS), and mechanical ventilation or vasoactive medication use≥4days. We adjusted estimates with multivariable logistic regression. Of 7897 patients at 50 centers, 1366 (17%) met criteria for H/SA. H/SA had worse outcomes for all study measures: death/transplantation (1.9 vs 1.1%, OR 1.74 (95% CI 1.01-3.03); p=0.047), ECMO (3.7 vs 2.3%, OR 1.74 (1.28-2.35); p<0.001), hemodialysis (2.1 vs 1.2%, OR 1.66 (1.06-2.59); p=0.026), prolonged mechanical ventilation (13.2% vs 7.6%, OR 1.85 (1.53-2.25); p<0.001) and vasoactive medication use (29.4 vs 19.7%, OR 1.65 (1.43-1.90), and longer LOS (11 (8-17) vs 9 (7-14) days; p<0.001). H/SA is associated with increased cardiovascular, renal, and respiratory morbidity, as well as in-hospital mortality after Fontan procedure. Attention to renal and respiratory needs may improve outcomes in this difficult population. The relationship between ciliary dysfunction and lung and renal morbidity should be explored further.

  View details for DOI 10.1007/s00246-021-02804-w

  View details for PubMedID 35064275

• Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). World journal for pediatric & congenital heart surgery Jacobs, J. P., Franklin, R. C., Beland, M. J., Spicer, D. E., Colan, S. D., Walters, H. L., Bailliard, F., Houyel, L., St Louis, J. D., Lopez, L., Aiello, V. D., Gaynor, J. W., Krogmann, O. N., Kurosawa, H., Maruszewski, B. J., Stellin, G., Weinberg, P. M., Jacobs, M. L., Boris, J. R., Cohen, M. S., Everett, A. D., Giroud, J. M., Guleserian, K. J., Hughes, M. L., Juraszek, A. L., Seslar, S. P., Shepard, C. W., Srivastava, S., Cook, A. C., Crucean, A., Hernandez, L. E., Loomba, R. S., Rogers, L. S., Sanders, S. P., Savla, J. J., Tierney, E. S., Tretter, J. T., Wang, L., Elliott, M. J., Mavroudis, C., Tchervenkov, C. I. 2021: 21501351211032919

  Abstract

  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 worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC. The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

  View details for DOI 10.1177/21501351211032919

  View details for PubMedID 34304616

• Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). Cardiology in the young Jacobs, J. P., Franklin, R. C., Beland, M. J., Spicer, D. E., Colan, S. D., Walters, H. L., Bailliard, F., Houyel, L., St Louis, J. D., Lopez, L., Aiello, V. D., Gaynor, J. W., Krogmann, O. N., Kurosawa, H., Maruszewski, B. J., Stellin, G., Weinberg, P. M., Jacobs, M. L., Boris, J. R., Cohen, M. S., Everett, A. D., Giroud, J. M., Guleserian, K. J., Hughes, M. L., Juraszek, A. L., Seslar, S. P., Shepard, C. W., Srivastava, S., Cook, A. C., Crucean, A., Hernandez, L. E., Loomba, R. S., Rogers, L. S., Sanders, S. P., Savla, J. J., Tierney, E. S., Tretter, J. T., Wang, L., Elliott, M. J., Mavroudis, C., Tchervenkov, C. I. 2021; 31 (7): 1057-1188

  Abstract

  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 worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

  View details for DOI 10.1017/S104795112100281X

  View details for PubMedID 34323211

• Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database. Pediatric cardiology Mahgerefteh, J., Lai, W., Colan, S., Trachtenberg, F., Gongwer, R., Stylianou, M., Bhat, A. H., Goldberg, D., McCrindle, B., Frommelt, P., Sachdeva, R., Shuplock, J. M., Spurney, C., Troung, D., Cnota, J. F., Camarda, J. A., Levine, J., Pignatelli, R., Altmann, K., van der Velde, M., Thankavel, P. P., Chowdhury, S., Srivastava, S., Johnson, T. R., Lopez, L., Pediatric Heart Network Investigators 2021

  Abstract

  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 area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18years of age enrolled in the Pediatric Heart Network Echo Z-Score Project. The echocardiographic study parameters included proximal aortic diameters at 3 locations, LV end-diastolic volume, and LV mass. Using the statistical methodology described in the original project, Z-scores based on height and BSA were determined for the study parameters and tested for any clinically significant relationships with age, sex, race, ethnicity, and body mass index (BMI). Normalization models based on height versus BSA were compared among underweight, normal weight, and overweight (but not obese) children in the study population. Z-scores based on height and BSA were calculated for the 5 study parameters and revealed no clinically significant relationships with age, sex, race, and ethnicity. Normalization based on height resulted in lower Z-scores in the underweight group compared to the overweight group, whereas normalization based on BSA resulted in higher Z-scores in the underweight group compared to the overweight group. In other words, increasing BMI had an opposite effect on height-based Z-scores compared to BSA-based Z-scores. Allometric normalization based on height and BSA for aortic and LV sizes is feasible. However, height-based normalization results in higher cardiovascular Z-scores in heavier children, and BSA-based normalization results in higher cardiovascular Z-scores in lighter children. Further studies are needed to assess the performance of these approaches in obese children with or without cardiac disease.

  View details for DOI 10.1007/s00246-021-02609-x

  View details for PubMedID 33877418

• Aortic size in children: Systolic measurements are different from diastolic measurements ANNALS OF PEDIATRIC CARDIOLOGY Al-Mousily, M. F., Lopez, L., Muniz, J., Sasaki, N., Seltzer, I., Gruber, J., Welch, E. 2021; 14 (2): 165-169

  Abstract

  Current guidelines recommended aortic measurements during diastole in adults and during systole in children. Recent studies in adults have demonstrated noteworthy differences in aortic measurements during systole and diastole in the same subjects. In the present study, we aimed to characterize systolic and diastolic differences in aortic measurements in healthy children.This retrospective study included 272 children who had a complete echocardiogram and no heart disease. Aortic measurements at the annulus (ANN), aortic root (AOR), sinotubular junction (STJ), and ascending aorta (AAO) were performed. Systolic and diastolic values were compared by calculating the mean systolic to diastolic (SD) percent difference for each segment; if the SD difference was >5%, it was considered clinically important. Similar measurements were conducted by another observer in 18% of the subjects.Systolic measurements were larger than diastolic measurements with mean SD percent differences >5% (P < 0.001) for the AOR (7.3% ± 5.5%), STJ (10.24% ± 7.1%), and AAO (9.8% ± 7.4%). There was no clinically significant SD difference for the ANN. There was an excellent intraclass correlation coefficient between observers (0.982-0.995).Systolic measurements for the AOR, STJ, and AAO were larger than diastolic measurements. Normal reference values are utilized to design treatment for patients with abnormal aortic sizes, and the timing in the cardiovascular cycle used to decide the reference values should be equivalent to the timing used to make measurements in clinical practice. This is particularly imperative as patients transition their care from a pediatric to an adult cardiologist.

  View details for DOI 10.4103/apc.APC_157_19

  View details for Web of Science ID 000649437900006

  View details for PubMedID 34103855

  View details for PubMedCentralID PMC8174627

• Pediatric Heart Network Echocardiographic Z Scores: Comparison with Other Published Models. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Lopez, L., Frommelt, P. C., Colan, S. D., Trachtenberg, F. L., Gongwer, R., Stylianou, M., Bhat, A., Burns, K. M., Cohen, M. S., Dragulescu, A., Freud, L. R., Frommelt, M. A., Lytrivi, I. D., Mahgerefteh, J., McCrindle, B. W., Pignatelli, R., Prakash, A., Sachdeva, R., Soslow, J. H., Spurney, C., Taylor, C. L., Thankavel, P. P., Thorsson, T., Tretter, J. T., Young, L. T., LuAnn Minich, L., Pediatric Heart Network Investigators 2020

  Abstract

  BACKGROUND: Different methods have resulted in variable Z scores for echocardiographic measurements. Using the measurements from 3,215 healthy North American children in the Pediatric Heart Network (PHN) echocardiographic Z score database, the authors compared the PHN model with previously published Z score models.METHODS: Z scores were derived for cardiovascular measurements using four models (PHN, Boston, Italy, and Detroit). Model comparisons were performed by evaluating (1) overlaid graphs of measurement versus body surface area with curves at Z=-2, 0, and+2; (2) scatterplots of PHN versus other Z scores with correlation coefficients; (3) Bland-Altman plots of PHN versus other Z scores; and (4) comparison of median Z scores for each model.RESULTS: For most measurements, PHN Z score curves were similar to Boston and Italian curves but diverged from Detroit curves at high body surface areas. Correlation coefficients were high when comparing the PHN model with the others, highest with Boston (mean, 0.99) and lowest with Detroit (mean, 0.90). Scatterplots suggested systematic differences despite high correlations. Bland-Altman plots also revealed poor agreement at both extremes of size and a systematic bias for most when comparing PHN against Italian and Detroit Z scores. There were statistically significant differences when comparing median Z scores between the PHN and other models.CONCLUSIONS: Z scores from the multicenter PHN model correlated well with previous single-center models, especially the Boston model, which also had a large sample size and similar methodology. The Detroit Z scores diverged from the PHN Z scores at high body surface area, possibly because there were more subjects in this category in the PHN database. Despite excellent correlation, significant differences in Z scores between the PHN model and others were seen for many measurements. This is important when comparing publications using different models and for clinical care, particularly when Z score thresholds are used to guide diagnosis and management.

  View details for DOI 10.1016/j.echo.2020.09.019

  View details for PubMedID 33189460

• Physician variation in ordering of transthoracic echocardiography in outpatient pediatric cardiac clinics. Echocardiography (Mount Kisco, N.Y.) Stern, K. W., McCracken, C. E., Gillespie, S. E., Lang, S. M., Statile, C. J., Lopez, L., Verghese, G. R., Choueiter, N. F., Sachdeva, R. 2020

  Abstract

  BACKGROUND: The pediatric Appropriate Use Criteria (AUC) for outpatient transthoracic echocardiography (TTE) aim to reduce practice variation. Little is known on variation in TTE use between physicians. Understanding this variation will help identify areas for improvement in standardization of TTE use.METHODS AND RESULTS: This is a retrospective review of initial outpatient visits at 6 pediatric cardiology centers in the United States prior to AUC release. Variation in TTE use was examined using multilevel generalized mixed effects models. Forward selection identified combinations of variables that explained the most variance in TTE use between physicians. Due to collinearity, physician compensation model and center were analyzed separately. Of 2883 encounters, the most common indication was murmur (36%), followed by chest pain (15.2%). Overall TTE use was 41.9%, and varied widely between centers (22.9%-52.6%), and between physicians within centers. Center alone explained 29% of this physician variance. Adding physician characteristics increased the variance explained to 57%, which only minimally improved by adding patient characteristics. The variance explained was driven by subspecialty. The center-based multivariable model explained more variance over compensation model.CONCLUSIONS: Center was the single largest determinant of physician variance in TTE use, followed by physician subspecialty. Efforts to reduce practice variation, such as the AUC, should be employed across centers and all pediatric cardiac providers. Center appears to have a stronger impact on variance than compensation model, though in this dataset the effect of center and compensation are hard to separate from each other and deserve further evaluation.

  View details for DOI 10.1111/echo.14756

  View details for PubMedID 32516460

• Challenges and lessons learned from the Pediatric Heart Network Normal Echocardiogram Database study. Cardiology in the young Truong, D., Lopez, L., Frommelt, P. C., Stelter, J., Ni, B., Cohen, M. S., Prakash, A., Colan, S. D., Spurney, C., Soslow, J., Pearson, G. D., Mahgerefteh, J., Sachdeva, R., Pignatelli, R., Trachtenberg, F., Stylianou, M., Altmann, K., Rathge, K. A., Camarda, J., Chowdhury, S., Dragulescu, A., Frommelt, M., Garuba, O., Soriano, B., Srivastava, S., Thankavel, P., van der Velde, M. E., Minich, L. L., Pediatric Heart Network Investigators 2020: 1–6

  Abstract

  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 reports, and adjudication logs were reviewed. A centre-level questionnaire provided information regarding local processes for data collection. Descriptive statistics were used, and chi-square tests determined differences in imaging quality.RESULTS: For the 19 participating centres, challenges with enrolment included variations in Institutional Review Board definitions of "retrospective" eligibility, overestimation of non-White participants, centre categorisation of Hispanic participants that differed from National Institutes of Health definitions, and exclusion of potential participants due to missing demographic data. Institutional Review Board amendments resolved many of these challenges. There was an unanticipated burden imposed on centres due to high numbers of echocardiograms that were reviewed but failed to meet submission criteria. Additionally, image transfer software malfunctions delayed Core Lab image review and feedback. Between the early and late study periods, the proportion of unacceptable echocardiograms submitted to the Core Lab decreased (14 versus 7%, p < 0.01). Most protocol violations were from eligibility violations and inadvertent protected health information disclosure (overall 2.5%). Adjudication committee reviews led to protocol changes.CONCLUSIONS: Numerous challenges encountered during the Normal Echocardiogram Database Study prolonged study enrolment. The retrospective design and flaws in image transfer software were key impediments to study completion and should be considered when designing future studies collecting echocardiographic images as a primary outcome.

  View details for DOI 10.1017/S1047951120000438

  View details for PubMedID 32180543

• ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Sachdeva, R., Valente, A., Armstrong, A. K., Cook, S. C., Han, B., Lopez, L., Lui, G. K., Pickard, S. S., Powell, A. J., Bhave, N. M., Pickard, S. S., Baffa, J. M., Banka, P., Cohen, S. B., Glickstein, J. S., Kanter, J. P., Kanter, R. J., Kim, Y. Y., Kipps, A. K., Latson, L. A., Lin, J. P., Parra, D. A., Rodriguez, F. H., Saarel, E. V., Srivastava, S., Stephenson, E. A., Stout, K. K., Zaidi, A. N., Gluckman, T. J., Aggarwal, N. R., Bhave, N. M., Dehmer, G. J., Gilbert, O. N., Kumbhani, D. J., Price, A. L., Winchester, D. E., Gulati, M., Dehmer, G. J., Doherty, J. U., Bhave, N. M., Daugherty, S. L., Dean, L. S., Desai, M. Y., Gillam, L. D., Mehrotra, P. 2020; 75 (6): 657–703

  View details for DOI 10.1016/j.jacc.2019.10.002

  View details for Web of Science ID 000512903000013

  View details for PubMedID 31918898

• Unilateral branch pulmonary artery origin from a solitary arterial trunk with major aortopulmonary collaterals to the contralateral lung: anatomic and developmental considerations. Seminars in thoracic and cardiovascular surgery Adamson, G. T., Houyel, L. n., McElhinney, D. B., Maskatia, S. A., Ma, M. n., Hanley, F. L., Lopez, L. n. 2020

  Abstract

  In both truncus arteriosus communis (TAC) and tetralogy of Fallot (TOF), there is a rare phenotype that includes a single branch pulmonary artery (PA) arising from a solitary great artery and major aortopulmonary collaterals (MAPCAs) supplying the contralateral lung. We describe the intracardiac and great vessel anatomy of infants with this phenotype, consider rationale for classifying patients as TOF vs. TAC, and describe surgical outcomes. Our institution's surgical database was reviewed for patients with a single branch PA from a solitary arterial trunk and contralateral MAPCAs from 2007 to 2019. Demographic, imaging, and surgical data were collected and described. All 11 patients underwent complete repair with a median right ventricular to aortic pressure ratio of 0.36 (range 0.26-0.50). At 0.1-9.1 years after repair (median 0.8 years) there was approximately balanced left-right lung perfusion (median 52% to the right lung, range 34-74%). The MAPCA lungs exemplified the full spectrum of PA and MAPCA anatomy, from absent intrapericardial PAs with all single supply MAPCAs to a normally arborizing PA with all dual supply MAPCAs and present pulmonary valve leaflet tissue. All patients had a systemic semilunar valve with 3 thin and similarly sized leaflets and fibrous continuity with the tricuspid valve, and all had coronary origins and outflow tract morphology more consistent with TOF. It is appropriate to classify all patients with a single anomalous PA from a solitary arterial trunk and MAPCAs to the contralateral lung as TOF rather than TAC Type A3. All variants were amenable to surgical repair.

  View details for DOI 10.1053/j.semtcvs.2020.11.009

  View details for PubMedID 33181302

• Recommendations for Multimodality Assessment of Congenital Coronary Anomalies: A Guide from the American Society of Echocardiography: Developed in Collaboration with the Society for Cardiovascular Angiography and Interventions, Japanese Society of Echocardiography, and Society for Cardiovascular Magnetic Resonance. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Frommelt, P. n., Lopez, L. n., Dimas, V. V., Eidem, B. n., Han, B. K., Ko, H. H., Lorber, R. n., Nii, M. n., Printz, B. n., Srivastava, S. n., Valente, A. M., Cohen, M. S. 2020; 33 (3): 259–94

  View details for DOI 10.1016/j.echo.2019.10.011

  View details for PubMedID 32143778

• 2019 ACC/AHA/ASE Key Data Elements and Definitions for Transthoracic Echocardiography A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Data Standards (Writing Committee to Develop Clinical Data Standards for Transthoracic Echocardiography) and the American Society of Echocardiography JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Douglas, P. S., Carabello, B. A., Lang, R. M., Lopez, L., Pellikka, P. A., Picard, M. H., Thomas, J. D., Varghese, P., Wang, T. Y., Weissman, N. J., Wilgus, R., Bozkurt, B., Jneid, H., Al-Khatib, S. M., Anderson, H., Gilstrap, L., Graham, G. N., Jones, G. K., Kao, D., Lopez, L., Marcus, G., Rymer, J., Tcheng, J. E., Weintraub, W. S. 2019; 32 (9): 1161–1248

  View details for DOI 10.1016/j.echo.2019.05.006

  View details for Web of Science ID 000484074400016

  View details for PubMedID 31248777

• How Well Does the Neonatal Heart Measure Up? Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Lopez, L., Colan, S. D. 2019; 32 (7): 906–8

  View details for DOI 10.1016/j.echo.2019.04.423

  View details for PubMedID 31272594

• 2019 ACC/AHA/ASE Key Data Elements and Definitions for Transthoracic Echocardiography: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Data Standards (Writing Committee to Develop Clinical Data Standards for Transthoracic Echocardiography) and the American Society of Echocardiography CIRCULATION-CARDIOVASCULAR IMAGING Douglas, P. S., Carabello, B. A., Lang, R. M., Lopez, L., Pellikka, P. A., Picard, M. H., Thomas, J. D., Varghese, P., Wang, T. Y., Weissman, N. J., Wilgus, R. 2019; 12 (7): e000027

  View details for DOI 10.1161/HCI.0000000000000027

  View details for Web of Science ID 000475945900002

  View details for PubMedID 31233331

• Challenges With Left Ventricular Functional Parameters: The Pediatric Heart Network Normal Echocardiogram Database. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Frommelt, P. C., Minich, L. L., Trachtenberg, F. L., Altmann, K. n., Camarda, J. n., Cohen, M. S., Colan, S. D., Dragulescu, A. n., Frommelt, M. A., Johnson, T. R., Kovalchin, J. P., Lin, L. n., Mahgerefteh, J. n., Nutting, A. n., Parra, D. A., Pearson, G. D., Pignatelli, R. n., Sachdeva, R. n., Soriano, B. D., Spurney, C. n., Srivastava, S. n., Statile, C. J., Stelter, J. n., Stylianou, M. n., Thankavel, P. P., Tierney, E. S., van der Velde, M. E., Lopez, L. n. 2019

  Abstract

  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 with normal cardiac anatomy and qualitatively normal function.The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race. A core lab used two-dimensional echocardiograms to measure LV dimensions from which a separate data coordinating center calculated LV volumes and systolic functional indices. To evaluate interobserver variability, two independent expert pediatric echocardiographic observers remeasured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices.Of 3,215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF) < 25% and/or LV ejection fraction (EF) < 50%; the subjects were significantly younger and smaller than those with normal values. When the core lab and independent observer measurements were compared, individual LV size parameter intraclass correlation coefficients were high (0.81-0.99), indicating high reproducibility. The intraclass correlation coefficients were lower for SF (0.24) and EF (0.56). Comparing reviewers, 40/56 (71%) of those with an abnormal SF and 36/104 (35%) of those with a normal SF based on core lab measurements were calculated as abnormal from at least one independent observer. In contrast, an abnormal EF was less commonly calculated from the independent observers' repeat measures; only 9/47 (19%) of those with an abnormal EF and 8/113 (7%) of those with a normal EF based on core lab measurements were calculated as abnormal by at least one independent observer.Although blinded measurements of LV size show good reproducibility in healthy children, subsequently calculated LV functional indices reveal significant variability despite qualitatively normal systolic function. This suggests that, in clinical practice, abnormal SF/EF values may result in repeat measures of LV size to match the subjective assessment of function. Abnormal LV functional indices were more prevalent in younger, smaller children.

  View details for DOI 10.1016/j.echo.2019.05.025

  View details for PubMedID 31351792

• Echocardiographic surveillance in children after tetralogy of Fallot repair: Adherence to guidelines? International journal of cardiology Annavajjhala, V. n., Valente, A. M., Lopez, L. n., Sachdeva, R. n., Glickstein, J. S., Natarajan, S. S., Buddhe, S. n., Altmann, K. n., Soriano, B. D., Colquitt, J. L., Altman, C. A., Sasaki, N. n., Sakarovitch, C. n., Tacy, T. A., Geva, T. n., Selamet Tierney, E. S. 2019

  Abstract

  Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations.This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines.We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53-70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported.Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

  View details for DOI 10.1016/j.ijcard.2019.09.075

  View details for PubMedID 31668657

• Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The Annals of thoracic surgery Lopez, L., Houyel, L., Colan, S. D., Anderson, R. H., Béland, M. J., Aiello, V. D., Bailliard, F., Cohen, M. S., Jacobs, J. P., Kurosawa, H., Sanders, S. P., Walters, H. L., Weinberg, P. M., Boris, J. R., Cook, A. C., Crucean, A., Everett, A. D., Gaynor, J. W., Giroud, J., Guleserian, K. J., Hughes, M. L., Juraszek, A. L., Krogmann, O. N., Maruszewski, B. J., St Louis, J. D., Seslar, S. P., Spicer, D. E., Srivastava, S., Stellin, G., Tchervenkov, C. I., Wang, L., Franklin, R. C. 2018; 106 (5): 1578-1589

  Abstract

  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 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

  View details for DOI 10.1016/j.athoracsur.2018.06.020

  View details for PubMedID 30031844

• Cardiovascular imaging in Turner syndrome: state-of-the-art practice across the lifespan. Heart (British Cardiac Society) Mortensen, K. H., Young, L. n., De Backer, J. n., Silberbach, M. n., Collins, R. T., Duijnhouwer, A. L., Pandya, B. n., Gravholt, C. H., Lopez, L. n., Roos-Hesselink, J. W. 2018; 104 (22): 1823–31

  Abstract

  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.

  View details for PubMedID 30228249

• Cardiovascular Health in Turner Syndrome: A Scientific Statement From the American Heart Association. Circulation. Genomic and precision medicine Silberbach, M. n., Roos-Hesselink, J. W., Andersen, N. H., Braverman, A. C., Brown, N. n., Collins, R. T., De Backer, J. n., Eagle, K. A., Hiratzka, L. F., Johnson, W. H., Kadian-Dodov, D. n., Lopez, L. n., Mortensen, K. H., Prakash, S. K., Ratchford, E. V., Saidi, A. n., van Hagen, I. n., Young, L. T. 2018; 11 (10): e000048

  Abstract

  Girls and women with Turner syndrome face a lifelong struggle with both congenital heart disease and acquired cardiovascular conditions. Bicuspid aortic valve is common, and many have left-sided heart obstructive disease of varying severity, from hypoplastic left-sided heart syndrome to minimal aortic stenosis or coarctation of the aorta. Significant enlargement of the thoracic aorta may progress to catastrophic aortic dissection and rupture. It is becoming increasingly apparent that a variety of other cardiovascular conditions, including early-onset hypertension, ischemic heart disease, and stroke, are the major factors reducing the life span of those with Turner syndrome. The presentations and management of cardiovascular conditions in Turner syndrome differ significantly from the general population. Therefore, an international working group reviewed the available evidence regarding the diagnosis and treatment of cardiovascular diseases in Turner syndrome. It is recognized that the suggestions for clinical practice stated here are only the beginning of a process that must also involve the establishment of quality indicators, structures and processes for implementation, and outcome studies.

  View details for DOI 10.1161/HCG.0000000000000048

  View details for PubMedID 30354301

• Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and Ethnicity: The Pediatric Heart Network Normal Echocardiogram Database. Circulation. Cardiovascular imaging Lopez, L., Colan, S., Stylianou, M., Granger, S., Trachtenberg, F., Frommelt, P., Pearson, G., Camarda, J., Cnota, J., Cohen, M., Dragulescu, A., Frommelt, M., Garuba, O., Johnson, T., Lai, W., Mahgerefteh, J., Pignatelli, R., Prakash, A., Sachdeva, R., Soriano, B., Soslow, J., Spurney, C., Srivastava, S., Taylor, C., Thankavel, P., van der Velde, M., Minich, L. 2017; 10 (11)

  Abstract

  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.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/BSAα) 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 (BSAα) 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.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.

  View details for DOI 10.1161/CIRCIMAGING.117.006979

  View details for PubMedID 29138232

  View details for PubMedCentralID PMC5812349

• Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Lopez, L., Colan, S. D., Frommelt, P. C., Ensing, G. J., Kendall, K., Younoszai, A. K., Lai, W. W., Geva, T. 2010; 23 (5): 465-95; quiz 576-7

  View details for DOI 10.1016/j.echo.2010.03.019

  View details for PubMedID 20451803