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Graphical abstract, Highlights • Double aortic arch (DAA) typically occurs in isolation. • DAA with bilateral coarctation (COA) is very rare with three prior documented cases. • In addition to DAA with bilateral COA our case also has complex intracardiac disease. • Echocardiography served as the primary diagnostic tool with advanced imaging as a useful adjunct.

Graphical abstract, Highlights • Abnormal right coronary artery origin and course are demonstrated by FE. • Color flow Doppler is essential for visualizing fetal coronary artery anatomy. • Color-compare images help identify coronary artery origin and course.

Coronary artery inflammation and aneurysm formation are the most common complications of Kawasaki disease (KD). Valvulitis and myocarditis are also well described and may lead to valvar regurgitation and left ventricular dysfunction. However, functional changes in the right heart have rarely been reported. We noted several acute KD patients with dilated pulmonary arteries (PA) and thus sought to systematically characterize PA size and right-heart function in an unselected cohort of KD patients cared for at a single clinical center. Clinical, laboratory, and echocardiographic data from 143 acute KD subjects were analyzed. PA dilation was documented in 23 subjects (16.1 %); these subjects had higher median right ventricle myocardial performance index (RV MPI), higher ratio of early tricuspid inflow velocity to tricuspid annular early diastolic velocity (TV E/e'), and lower median TV e' velocity compared to the non-PA dilation group (0.50 vs 0.38 p < 0.01, 4.2 vs 3.6 p < 0.05, and 13.5 vs 15.2 cm/s p < 0.01, respectively). Almost all subjects with PA dilation had improved PA Z-score, RV MPI, and TV E/e' in the subacute phase (p < 0.01). There were no significant differences in indices of left ventricle function between PA dilation group and non-PA dilation group. In summary, PA dilation was documented in 16 % of acute KD subjects. These subjects were more likely to have echocardiographic indices consistent with isolated RV dysfunction that improved in the subacute phase. The long-term consequence of these findings will require longitudinal studies of this patient population.

Objective The aim of this study was to characterize the cardiovascular and musculoskeletal systems of elite volleyball players, including aortic dimensions. Previous studies have shown that the upper limit of normal aortic sinus diameter for male and female athletes is 4 and 3.4 cm, respectively. Design Cross-sectional analysis. Setting United States Olympic Volleyball Training Facility and Rady Children's Hospital San Diego. Participants Seventy (37 male) members of the US national volleyball team. Main outcome measures Athletes underwent evaluation that included medical and family histories, targeted physical examinations specifically focusing on abnormalities present in Marfan syndrome (MFS), and transthoracic echocardiograms. Cardiac chamber and great artery size, valve function, and coronary artery origins were assessed. Results Three male athletes (8%) had an aortic sinus diameter ≥4 cm, one of whom also had an ascending aorta >4 cm. Two female athletes (6%) had aortic sinus diameter ≥3.4 cm, and another had an ascending aorta of 3.4 cm. There were no other intracardiac or arterial abnormalities. Individual musculoskeletal characteristics of MFS were common among the athletes but not more frequent or numerous in those with aortic dilation. Conclusions The prevalence of aortic root dilation in this population of athletes was higher than what has previously been reported in other similar populations. Further study is needed to determine whether these represent pathological changes or normal variations in tall athletes. Clinical relevance This study adds to the existing knowledge base of athlete's heart, with specific attention to aortic dimensions in elite volleyball players. The data are relevant to similar athletes' medical care and to preparticipation cardiac screening in general.

In the absence of a specific test, the diagnosis of clinically incomplete Kawasaki disease (KD) can be challenging. The 2004 American Heart Association guidelines state that the diagnosis of KD is supported by the presence of coronary artery dilation documented by echocardiography. However, the specificity of coronary artery dilation and its prevalence in children with other acute illnesses associated with fever has not been studied.A 2-center, prospective study compared the internal diameters and Z-scores (standard deviation [SD] units from the mean internal diameter normalized for body surface area) of the proximal left anterior descending and right coronary arteries measured by echocardiography in febrile children (FC) and children with KD.The median Z-score (interquartile range) of the left anterior descending coronary artery was -0.05 (-0.86, 0.62) and 1.06 (0.36, 2.06) SD units for the 45 FC and the 145 KD patients, respectively (P0.0001). For the right coronary artery, the median Z-score was 0.21 (-0.20, 0.87) and 1.04 (0.31, 1.85) SD units for the FC and KD patients, respectively (P0.0001). There was no correlation between body temperature at the time of echocardiography and coronary artery Z-score. No febrile child had a coronary artery Z-score ≥ 2.5 SD units.Z-scores ≥ 2.5 SD units were not observed in our cohort of FC. Therefore, echocardiographic evidence of coronary artery dilation can be used to support the diagnosis of KD.

We report an infant who had tetralogy of Fallot, hypoplastic pulmonary arteries, and membranous pulmonary atresia who underwent successful perforation of the atretic valve and subsequent balloon pulmonary valvuloplasty. Because of the inability to access the pulmonary arteries via a patent ductus arteriosus, two-dimensional echocardiography was used to confirm wire position prior to perforation. The branch pulmonary arteries initially measured 1.5 mm in diameter and enlarged to 2.8 mm immediately after valvuloplasty. Four months postprocedure, the patient underwent a successful repeat balloon pulmonary valvuloplasty. The pulmonary arteries had grown to approximately 6 mm in diameter. Although it is a rare occurrence, patients with tetralogy of Fallot and membranous pulmonary atresia can be dilated with successful growth of the pulmonary arteries.

Early left ventricular (LV) remodeling following pediatric cardiac transplantation has not been described. To identify patterns and determinants of change in left ventricular mass and volume posttransplant, we studied 125 consecutive children who underwent cardiac transplantation between January 1, 1989 and July 31, 1993. Two-dimensional imaging-directed M-mode echocardiograms were studied weekly until 26 weeks post-transplant. LV mass and volume (indexed to BsA 1.5 ) were measured. LV mass index increased until 3 weeks post-transplant, and then decreased. The mean decrement in LV mass index after 8 weeks post-transplant (relative to baseline) was significantly larger in patients with donor-recipient weight ratio >1.5 compared with patients with donor-recipient weight ratio ≤1.5 (-2.2 g/m 3 compared with 33.4 g/m 3 , respectively, P

To the Editor: We read with interest the recent article by Lytrivi et al. and would like to thank the investigators for this important reference material. For clarification, we hope that they will respond to these questions: 1. The units of measure used for the relationship volume/(body surface area) are expressed as mL/m. Using the rule of exponents, the exponent 2 raised to the power 1.38 should result in 2 1.38 = 2.76. Could the authors explain the derivation of the exponent used in this model? 2. Inspection of the model (Figure 2) at the extreme small end (

Although the videotape method (VTM) is commonly used to record and intrepret ecocardiographic images, many pediatric echocardiographers are considering acquiring to, interpreting from, and storing their images to digital disk using the single-beat digital acquisition method (SBM). The paucity of image redundancy using SBM should translate into improved laboratory efficiency compared with VTM, but hard data are lacking. The purpose of this study was to test the hypothesis that the time to acquire images to videotape using VTM and to disk using SBM would be the same for normal hearts and corresponding congenital heart diseases, but interpretation times would be shorter using SBM.We measured the times to acquire and interpret 403 echocardiograms using standard VTM from Children's Hospital in Cincinnati, Ohio, and 352 echocardiograms acquired using SBM from Children's Hospital in San Diego, Calif. Diagnostic categories at each site included: (1) normal, (2) simple shunt or isolated valve disease, and (3) multiple-lesion disease.As a group, SBM echocardiograms included more hemodynamic measurements and took more time to acquire (P.037), but less time to read (P.001) than corresponding images acquired using VTM. Using SBM, it took more time to acquire normals and isolated valve or shunt lesions, whereas the average time to acquire multiple-lesion disease was the same using both VTM and SBM. With SBM, in contrast, interpretation times were significantly less for all corresponding diagnoses.SBM studies took longer to acquire because more hemodynamic measurements were acquired, but they were read in less time than corresponding VTM studies even though all videotapes were replayed in search fast-forward mode. Pediatric echocardiographers can increase their laboratory efficiency by converting from VTM to SBM.

Objectives. The purpose of this study was to determine the feasibility, safety and diagnostic accuracy of dobutamine stress echocardiography (DSE) for evaluating posttransplant coronary artery disease (TxCAD) in children, and to determine the frequency of selected cardiac events after normal or abnormal DSE. Background. Posttransplant coronary artery disease is the most common cause of graft loss (late death or retransplantation) after cardiac transplantation (CTx) in children. Coronary angiography, routinely performed to screen for TxCAD, is an invasive procedure with limited sensitivity. The efficacy of DSE for detecting atherosclerotic coronary artery disease is established, but is unknown in children after CTx. Methods. Of the 78 children (median age 5.7 years, range 3 to 18) entered into the study, 72 (92%) underwent diagnostic DSE by means of a standard protocol, 4.6 ± 1.9 years after CTx. The results of coronary angiography performed in 70 patients were compared with DSE findings. After DSE, subjects were monitored for TxCAD-related cardiac events, including death, retransplantation and new angiographic diagnosis of TxCAD. Results. No major complications occurred. Minor complications, most often hypertension, occurred in 11% of the 72 subjects. The sensitivity and specificity of DSE were 72% and 80%, respectively, when compared with coronary angiography. At follow-up (21 ± 8 months), TxCAD-related cardiac events occurred in 2 of 50 children (4%) with negative DSE, versus 6 of 22 children (27%) with positive DSE (p Conclusions. DSE is a feasible, safe and accurate screening method for TxCAD in children. Positive DSE identifies patients at increased risk of TxCAD-related cardiac events. Negative DSE predicts short-term freedom from such events.

Although infracardiac total anomalous pulmonary venous connection (TAPVC) is almost always associated with venous obstruction, the incidence and location of obstruction in supracardiac TAPVC has not been completely delineated. This report summarizes our experience with 20 cases of supracardiac TAPVC diagnosed by transthoracic echocardiography from Jan. 1989 to Mar. 1997. Fifty percent were obstructed, and five different sites of narrowing were found. The most common sites of obstruction were at the level of the left pulmonary artery (left vertical vein) and at the insertion into the superior vena cava (right vertical vein). Because nonobstructed Doppler flow patterns are present proximal to the actual site of obstruction in the anomalous pathway, a thorough interrogation of the entire venous channel with two-dimensional and Doppler echocardiography is essential to provide complete preoperative anatomic and hemodynamic details to determine the nature and timing of surgery in this condition. (J Am Soc Echocardiogr 1998;11:289-93).