Coronary Artery Origins Pattern in Pediatric Patients with Right-Left Fusion Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV) is a common congenital heart defect associated with coronary artery (CA) variants, including higher incidence of left CA dominance and shorter left main CA length. We observed by transthoracic echocardiography that left and right CA origins appear closer together in pediatric patients with right-left fusion (R/L) BAV compared to patients with trileaflet aortic valves. We sought to objectively confirm this observation. A retrospective review of pediatric echocardiograms with R/L BAV at a single institution (12/2010-11/2018) was performed. The 'coronary angle' was defined as the angle between the left and right coronary artery origins in the parasternal short axis view relative to the center of the aortic valve orifice. Values were compared to age-matched controls. Patients with inadequate images, anomalous coronary origins, or predefined significant congenital heart defects were excluded. We compared 191 R/L BAV patients (64% male) to 136 controls (57% male). Coronary angle was significantly more acute in R/L BAV than in controls (117.9° ± 16.7° vs 139.0° ± 10.1°, p < 0.0001). This was independent of age and gender. The difference persisted when BAV patients with abnormal aortic annulus/root diameters were removed from analysis (119.5° ± 15.1° vs 139.0° ± 10.1°, p < 0.0001). CA origins are closer together in R/L BAV independent of age, gender, or annulus/root size. This new anatomical description may aid in the diagnosis of subtle ('forme fruste') R/L BAV, assist in interventional planning, and improve understanding of the relationship between BAV and CA development.

Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database.

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 18 years 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.

Challenges and lessons learned from the Pediatric Heart Network Normal Echocardiogram Database study.

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.

Bilateral congenital coronary ostial obstruction: Exertional syncope in an otherwise healthy 8-year-old.

Congenital stenosis/atresia of a coronary artery is an exquisitely rare anomaly (Congenit Heart Dis, 2, 2007, 347) with increased risk of sudden death. Bilateral coronary obstruction is even more unusual but has been reported in conjunction with aortic valve disease, syphilis, and Takayasu's arteritis. To the best of our knowledge, obstruction of both coronaries in a pediatric patient has only been reported once (Ann Thorac Surg, 55, 1993, 1564). We present a patient with an intramural, anomalous aortic origin of the right coronary artery from the contralateral sinus (AAORCA) with near atresia of the left main coronary ostium. The diagnosis was made by echocardiogram and confirmed by catheterization and magnetic resonance imaging.

Isolated Mitral Cleft in Trisomy 21: An Initially 'Silent' Lesion.

Congenital cardiac anomalies are common in trisomy 21, and transthoracic echocardiogram within the first month of life is recommended. While a cleft mitral valve associated with atrioventricular septal defect has been well defined in this population, the prevalence of isolated mitral valve cleft has not been previously reported. The aim of our study was to define the occurrence of isolated mitral cleft in the first echocardiogram of patients with trisomy 21. This retrospective chart review examined echocardiographic data on all Trisomy 21 patients <1 year of age obtained during January 1, 2010, to May 1, 2014, at our institution. Images were reviewed by one of the authors with no knowledge of the official diagnosis. In addition to evaluation for isolated mitral valve cleft, data obtained included presence of additional congenital heart defects and need for surgical intervention. A total of 184 patients (median age 5 days) were identified. Isolated mitral cleft was identified in 12 patients (6.5 %). Four were diagnosed retrospectively (33 %). Only one had mitral regurgitation on initial echocardiogram. Seven required surgery for closure of ventricular septal defects. Isolated mitral cleft is present in an important number of neonates with Trisomy 21. Mitral regurgitation is often absent in the neonatal period and should not be used as a reliable indicator of absence of valve abnormality. Careful attention should be directed toward the mitral valve during the first echocardiogram to exclude an isolated cleft, which can lead to progressive mitral regurgitation.

Early echocardiographic predictor of heart failure in cerebral arteriovenous malformations.

Neonatal congestive heart failure in cerebral arteriovenous malformations carries a high risk of mortality. Severe heart failure leading to end-organ dysfunction may be a contraindication to early intervention. Although echocardiographic markers of haemodynamic instability exist, an objective echocardiographic predictor of severe heart failure is not available. We present a ratio of antegrade to retrograde flow in the aortic arch that objectively identifies those at the highest risk of mortality. This measurement can be easily obtained and has prognostic value at presentation, before clinical deterioration. Outcomes remain poor even when early intervention is offered, which may reflect irreversible neurological abnormalities.

Anomalous left pulmonary artery origin from internal carotid artery: prospective echocardiographic diagnosis of a previously unknown variant.

We present a neonate with dextrocardia, tetralogy of Fallot, right arch, and aberrant left subclavian artery with left pulmonary artery origin from the left internal carotid artery, which is previously unreported.

Utility and importance of new echocardiographic screening methods in diagnosis of anomalous coronary origins in the pediatric population: assessment of quality improvement.

Anomalous aortic origin of coronaries from the contralateral sinus (AAOCA) is rare but an important cause of cardiac death in the otherwise healthy young athlete. This necessitates prompt, accurate identification; transthoracic echocardiography (TTE) remains the primary screening tool. Assessment of accuracy is difficult since the true prevalence of the disease is unknown, with estimates at 0.3-1.07%. The incidence by TTE remains much lower, between 0.09 and 0.17% even with sophisticated equipment and a high index of suspicion. Our goal was to incorporate two new screening views to our standard TTE protocol and assess improvement in diagnosis of AAOCA by TTE in our laboratory. Recently (2011), we incorporated two new screening methods to standard protocol. The parasternal short axis sweep is extended to visualize the anomalous segment arising superiorly from the ascending aorta before exiting the root at a site close to a 'normal' origin. Secondly, the anomalous, interarterial coronary demonstrates an anterior, steep-angled course visualized in the parasternal long axis between the aorta and pulmonary artery. The echocardiogram database was searched for patients newly diagnosed with AAOCA in 2010 (prior to incorporation of new methods) and 2012. AAOCA incidence in our patient population improved from 0.02% (2010) to 0.22% (2012), age range from 4 days to 17 years. Teenagers and symptomatic patients with anomalous right coronary origin (6) underwent additional confirmatory imaging, and three underwent surgery. One patient with anomalous left coronary origin underwent surgical repair. Addition of the screening views can significantly increase the sensitivity of TTE in diagnosing AAOCA in the asymptomatic patients. We propose that these views be incorporated into the standard TTE evaluation of coronary arteries.

Unruptured sinus of valsalva aneurysm in a neonate with hypoplastic left heart syndrome: echocardiographic diagnosis and features.

An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications in regards to medical management and surgical options.

Elevated troponin levels in previously healthy children: value of diagnostic modalities and the importance of a drug screen.

BACKGROUND: Myocardial injury in previously healthy children is rare, with a wide range of aetiologies. It is increasingly being identified on the basis of elevated troponin levels during routine evaluation of cardiorespiratory symptoms. Establishing the aetiology remains challenging because of the lack of an accepted work-up algorithm. Our objective was to delineate the contribution of diagnostic modalities and troponin patterns towards the final diagnosis. METHODS: A retrospective chart review of previously healthy patients admitted to the Pediatric Cardiology Service with myocardial injury was carried out. Data analysed included echocardiograms, electrocardiograms, cardiac catheterisations, magnetic resonance imaging, drug screen tests, troponin values, and final diagnosis. RESULTS: A total of 32 patients were identified. The diagnoses were: myocarditis in 16 patients, vasospasm due to drug use in seven, myopericarditis in six, anomalous coronary artery origins in two, and Prinzmetal's angina in one patient. The electrocardiograms were abnormal in 27 of the 32 patients (84%), echocardiograms in 18 of the 32 patients (56%), cardiac magnetic resonance imaging in two of the four patients (50%), urine drug screen in five of the 25 patients (20%), and cardiac catheterisations in two of the 15 patients (13%). CONCLUSIONS: Myocarditis is the most common aetiology of myocardial injury in children. Clinical history remains the basic screening tool; drug screens help identify coronary vasospasms secondary to drug use (22% of our cohort). Patients with anomalous coronaries had exertional symptoms. Initial troponin levels and progression were not diagnostic or prognostic. Catheterisation is of limited value and did not change management. Magnetic resonance imaging with gadolinium enhancement is probably the most useful test when initial evaluation is not diagnostic.

Anomalous left main coronary artery origin from the right sinus of Valsalva: a novel echocardiographic screening method.

Anomalous origin of the left main coronary artery from the right sinus (ALMCA) is a rare entity associated with an increased risk for sudden cardiac death. Although transthoracic echocardiography often is the primary screening tool for the diagnosis, its accuracy remains undefined, resulting in the use of more expensive methods such as cardiac catheterization and magnetic resonance imaging when clinical suspicion exists. This report aims to describe a novel echocardiographic screening method for detecting ALMCA and to apply it retrospectively for patients with confirmed findings intraoperatively and/or on autopsy. All patients with a confirmed diagnosis of ALMCA who presented to our institution from 2004 to 2012 were included in the study. Eight patients had adequate images, including two patients with an initial diagnosis of normal coronary origins. A control group consisted of 35 patients with normal coronaries shown by echocardiogram. Five of these patients had normal coronary artery origins confirmed by magnetic resonance imaging (MRI). Echocardiographic images were reviewed, and the left main coronary was identified in the parasternal long-axis view between the aorta and pulmonary trunk. The angle of the left main coronary course was measured. In patients with ALMCA, the proximal course of the vessel was steeper as it coursed posteriorly. In contrast, the course was almost horizontal in patients with normal coronary origins. Based on these findings, a cutoff angle of 28° is proposed. In the parasternal long-axis view, ALMCA can be identified by its anomalous proximal course. This screening method is reliable and increases the accuracy of transthoracic echocardiograms.

Mechanical tricuspid valve replacement in hypoplastic left heart syndrome: An institutional experience.

Objective: Atrioventricular valvar regurgitation in patients with single ventricles is associated with worse outcomes. Valve repair or replacement has been undertaken in an attempt to reduce mortality and morbidity. Current data on valve replacement in single ventricle patients are limited and derived from heterogenous populations. We describe our experience with repair and replacement of the tricuspid valve in children undergoing single ventricle palliation for hypoplastic left heart syndrome. Methods: We included 27 patients with hypoplastic left heart syndrome with at least moderate tricuspid regurgitation who underwent valve intervention between 2007 and 2021 at our institution; charts were retrospectively reviewed for data. Results: Eleven patients (73% male) underwent valve replacement (median age, 3 years). Preoperative ventricular systolic function was normal in 10 patients (91%). Median follow-up postoperatively was 4 years with no early mortality, 1 (9%) late mortality, and 1 heart transplant (9%). Morbidity consisted of complete heart block in 1 patient (9%), with no important hemorrhagic or thrombotic events. Among survivors of replacement (n = 9), ventricular function was preserved in all (n = 8) who had normal function preoperatively and improved in the remaining patient. Sixteen patients underwent valve repair (median age, 4 months) with no early mortality, 8 (50%) midterm mortalities, and 2 heart transplants (12%). Conclusions: Tricuspid valve replacement is a feasible option in hypoplastic left heart syndrome with significant tricuspid regurgitation, with favorable outcomes in the intermediate follow-up. When undertaken in the setting of normal function, ventricular function may be preserved in up to 80% of patients. Long-term follow-up is needed.

A neonate with left congenital diaphragmatic hernia and transient retrograde flow in the aortic arch.

Retrograde flow in the aortic arch identifies inadequate cardiac output in neonates with congenital heart disease. We present a left congenital diaphragmatic hernia (CDH) infant with retrograde flow in the arch that normalized after CDH repair. We hypothesize transient inadequacy of the left heart; prostaglandins might play a pivotal role.

Pediatric Heart Network Echocardiographic Z Scores: Comparison with Other Published Models.

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.

Echocardiographic evaluation of left ventricular systolic function by the M-mode lateral mitral annular plane systolic excursion in patients with Duchenne muscular dystrophy age 0-21 years.

BACKGROUND AND AIMS: Duchenne muscular dystrophy (DMD) results in cardiac fibrosis and dysfunction. These patients frequently have poor image quality. Mitral annular plane systolic excursion (MAPSE) is a reproducible and reliable method for determining function and can be a valuable tool in patients with poor images. Our study was performed to evaluate the feasibility of MAPSE and compare it to shortening fraction (SF) in patients with DMD. METHODS: Lateral M-mode MAPSE was obtained on all echocardiograms performed on DMD patients aged 0 to 21 years between October 2013 and April 2015. Retrospectively, interobserver and intraobserver variability was determined for these measurements and each measurement was compared to patient characteristics and measured values of SF. RESULTS: There was good interobserver (r 2 = .66, P = .0081) correlation. Seventeen of 59 echocardiograms (29%) had abnormal SF while 32 (54%) echocardiograms had an abnormal M-mode lateral MAPSE Z-score. There was no significant association between lateral MAPSE Z-score and SF. Age at the time of echocardiogram and time from diagnosis to echocardiogram both had a significant negative correlation with lateral MAPSE. CONCLUSIONS: Lateral M-mode MAPSE measurements are reproducible in young patients with Duchenne muscular dystrophy. M-mode lateral MAPSE may worsen over length of time with Duchenne muscular dystrophy. Further studies are necessary to provide absolute conclusions, but this study shows that lateral M-mode MAPSE may be a valuable additional tool at routine echocardiogram in these patients.

Ventricular Pseudoaneurysms After Perventricular Closure of Ventricular Septal Defect.

Perventricular ventricular septal defect closure has become an accepted method for treatment of some muscular and perimembranous ventricular septal defects. This report describes the case of a child who had left ventricular pseudoaneurysms after perventricular closure of a ventricular septal defect performed at 3 months of age. At 18 months, the ventricular pseudoaneurysms were closed surgically. Ventricular trauma from guidewire use during device placement was thought to be the cause. The potential for injury to the left ventricular free wall should be borne in mind and sought with serial imaging during and after perventricular septal defect closure.

Challenges With Left Ventricular Functional Parameters: The Pediatric Heart Network Normal Echocardiogram Database.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.