Search

Assessment of left ventricular dilation plays an important role in the management of left ventricular volume overload lesions. Various echocardiographic methods exist, such as the 5/6 area-length and biplane Simpsons, but their agreement with cardiac magnetic resonance imaging in patients with chronic left ventricular volume overload from a young age has not been assessed. This was a retrospective review of patients with moderate or worse aortic regurgitation, mitral regurgitation, or ventricular septal defect who underwent both studies within 6 months. End-diastolic and systolic volumes and dimensions were measured by echocardiography using the 5/6 area-length, biplane Simpsons, and M-mode methods, and compared to cardiac MRI-derived volumes. The 5/6 area-length method showed the best agreement with MRI and remained consistent with increasing ventricular dilation. The biplane Simpsons and M-mode-based Teichholz method underestimated ventricular dilation and performed worse with increasing dilation. When comparing ventricular dimensions by M-mode, there was a non-linear relationship between linear dimension and MRI-derived volume. Linear dimension appeared to plateau with increasing ventricular dilation, leading to underestimation in severity of dilation. The 5/6 area-length method was superior to other echocardiographic methods of ventricular volume quantification when compared with MRI.

Objectives Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications. Background Large defect size and deficient rims have been described as predictors for complications and unsuccessful device placement in closure of interatrial communications. MASP is an underappreciated morphologic atrial septal variation whose prevalence in isolated atrial communications and its influence on percutaneous device closure has not been systematically assessed. Methods We retrospectively evaluated echocardiographic data of 328 patients scheduled for percutaneous secundum atrial septal defect (ASD) and patent foramen ovale (PFO) closure between January 2006 and January 2013. In ASD patients, defect size was measured by balloon stretch diameter while the length of the surrounding rims was measured in standard transthoracic and transesophageal views. Furthermore, the distance between septum primum and septum secundum as it attaches to the left atrial roof was evaluated in both, ASD and PFO patients. Septal anatomy of patients with procedural failure was compared to the anatomy of patients in whom the procedure was successful. Results Transcatheter defect closure was successful in 131 (92.3%) of 142 ASD patients (32.3 years ± 24.7 years) and in all 186 PFO patients (49.6 years ± 16.2 years). Thirteen (4%) patients were found to have MASP (1.6 mm–13 mm). Smaller retroaortic rim, inferior rim, and the presence of a MASP were independently associated with procedural failure in ASD patients (P = 0.02, P = 0.03, and P = 0.003, respectively). Conclusion Smaller retroaortic and inferior rims, as well as MASP are independent risk factors for unsuccessful transcatheter ASD closure. © 2015 Wiley Periodicals, Inc.

Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset and the 3D model. Complicated ventriculo-arterial relationships in patients with complex conotruncal malformations make preoperative assessment of possible repair pathways difficult. Patients were chosen with double outlet right ventricle for the complexity of intracardiac anatomy and potential for better delineation of anatomic spatial relationships. Virtual 3D models were generated from CMR 3D datasets. Measurements were made on the source CMR as well as the 3D model for the following structures: aortic diameter in orthogonal planes, VSD diameter in orthogonal planes and long axis of right ventricle. A total of six patients were identified for inclusion. The path from the ventricles to each respective outflow tract and the location of the VSD with respect to each great vessel was visualized clearly in all patients. Measurements on the virtual model showed excellent correlation with the source CMR when all measurements were included by Pearson coefficient, r = 0.99 as well as for each individual structure. Construction of virtual 3D models in patients with complex conotruncal defects from 3D CMR datasets allows for simultaneous visualization of anatomic relationships relevant for surgical repair. The availability of these models may allow for a more informed preoperative evaluation in these patients.

The presence of myocardial late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) imaging in concert with electrocardiography and elevated biomarkers helps support the diagnosis of acute myocarditis. Two-dimensional echocardiography is limited to global and qualitative regional function assessment and may not contribute to the diagnosis, especially in the presence of normal LV systolic function. Two-dimensional speckle-tracking (2D-STE)-derived segmental peak systolic (pkS) longitudinal strain (LS) may identify segmental myocardial involvement in myocarditis. We sought to identify an association between segmental pkS, LGE, and troponin levels in patients with myocarditis. Retrospective analysis of myocardial segmental function by 2D-STE segmental strain was compared to the presence of LGE and admission peak troponin levels in patients with acute myocarditis and preserved global LV systolic function. American Heart Association 17-segment model was used for comparison between imaging modalities. Global function was assessed by m-mode-derived shortening fraction (SF). Descriptive statistics and regression analysis were utilized. Forty-four CMRs performed to evaluate for myocarditis were identified. Of the 44, 10 patients, median age 17.5 years (14-18.5 years) and median SF 35 % (28-44 %), had paired CMR and 2D-STE data for analysis, and 161/170 segments could be analyzed by both methods for comparison. PkS LS was decreased in 51 % of segments that were positive for LGE with average pkS of -14.7 %. Segmental pkS LS abnormalities were present in all but one patient who had abnormal pkS circumferential strain. Global pkS LS was decreased in patients with myocarditis. There is a moderate correlation between decreased pkS LS and the presence of LGE by CMR, 2D-STE for myocardial involvement in acute myocarditis can serve as an useful noninvasive adjunct to the existing tests used for the diagnosis of acute myocarditis and might have a role in prognostication.

Isolated congenital coronary artery fistula (CAF) is rare and varies with respect to size and hemodynamic significance. The prevalence of coronary artery ectasia in association with isolated congenital CAF, regardless of size, and after closure of large fistulae has not been systematically evaluated in the literature. This study aimed to characterize the demographic and echocardiographic differences between patients with large and small fistulae and to describe outcomes with respect to coronary ectasia in those who underwent closure. This is a retrospective review of an echocardiographic database that identified patients coded for CAF (1995 to 2012) and excluded those associated with complex cardiac disease and/or coronary anomalies and cardiomyopathy. Small fistulae were noted to arise mostly from the left anterior descending artery, drain into the pulmonary artery, and have a very low incidence of ectasia (n = 3 of 92), with a mean coronary artery diameter z score in these 3 patients of 3.45 ± 1.15. Larger fistulae had a female predominance, with most originating from the right coronary artery and draining into the right atrium; among the 12 patients who underwent procedural closure of large CAF, all feeding coronary arteries remained ectatic after closure, with a mean coronary artery diameter z score of 9.54 ± 5.66 after a total mean follow-up time of 3.95 ± 4.07 years. In conclusion, the occurrence of coronary dilatation justifies long-term follow-up irrespective of fistula size and successful closure of large CAF.

Background Decreased right ventricular (RV) ejection fraction (EF) increases morbidity in repaired tetralogy of Fallot (rToF). Cardiac magnetic resonance (CMR) is the accepted reference standard for RV EF measurement. There are no established color Doppler tissue imaging (cDTI) values that identify decreased RV EF in rToF. We sought to assess accuracy of cDTI myocardial deformation and velocity indices in predicting CMR-derived RV EF. Methods Retrospective evaluation of CMR and echocardiography studies performed on the same day. cDTI-derived peak systolic velocity (pkS), isovolumic acceleration (IVA) of the tricuspid valve annulus, strain (e) and strain rate (SR) of the RV free wall, adjusted for age, volume and pressure overload were used to compute RV EF. The probability of correctly classifying CMR measured RV EF

Restrictive cardiomyopathy (RCM) is characterized by irreversible diastolic dysfunction with preserved systolic function. The aim of this study was to investigate the presence of impaired ventricular contractility even in the presence of normal ejection fraction (EF) in children with RCM. Longitudinal Doppler tissue velocities were obtained from apical 4-chamber view at three locations—the left-ventricular (LV) lateral wall, the septum, and the right ventricle—in 8 children age 3–17 years old with RCM who had LV EF >55%. Peak systolic velocity (S′), acceleration during isovolumic contraction (IVA), and myocardial performance index (MPI) were measured. Data from the RCM group were compared with those from 24 age- and sex-matched controls. Both S′ and IVA were markedly lower at the septum (S′ 6.2 ± 1.7 vs. 9.2 ± 1.6, P < 0.001; IVA 1.8 ± 0.5 vs. 3.9 ± 1.5, P < 0.001). MPI, a measure of both diastolic and systolic function, was statistically significantly greater in the RCM group at all 3 locations (P < 0.005). S′ and IVA identify global subclinical systolic dysfunction in RCM with normal EF. These findings suggest that pre-ejection abnormality and subclinical systolic dysfunction coexist with diastolic dysfunction in children with RCM.

Background Left ventricular (LV) end-diastolic volume (LVEDV) can be estimated by the formula (5/6) × area × length, or the "bullet" method. The aim of this study was to determine the range of normal LVEDV values in infants and young children (aged 0–3 years) by the subxiphoid bullet method. Methods Echocardiograms from 100 normal subjects aged ≤3 years were retrospectively analyzed. Subjects with systemic disease, abnormal body size, cardiovascular disease, or nondiagnostic subxiphoid images were excluded. Measurements of LV short-axis cross-sectional diastolic area at the midventricular level and LV length were made offline from subxiphoid images. LVEDV was indexed to body surface area (BSA) to the powers of 1.0 and 1.38. Relationships between indexing methods, age, and gender were explored. Results The median age was 0.98 years (range, 0–2.9 years), the median weight was 9.5 kg (range, 3.1–16.0 kg), and the median BSA was 0.45 m 2 (range, 0.21–0.66 m 2 ). The mean LVEDV/BSA 1.38 was 70.4 ± 9.1 mL/m 2.6 , with an excellent correlation between LVEDV and BSA 1.38 ( r = 0.96, P 1.38 and BSA ( r = 0.06, P = NS) and no significant relationship between LVEDV/BSA 1.38 and age ( r = 0.10, P = NS) or LVEDV/BSA 1.38 and gender. Conclusions The normal range for LVEDV by the subxiphoid echocardiographic bullet method is reported for newborns, infants, and young children. LVEDV should be indexed to BSA 1.38 , which is consistent with the known relationship between LV size and body size. In children aged ≤3 years, these data can be used to calculate Z scores for LVEDV by the subxiphoid bullet technique independent of age or gender.

Introduction: Left ventricular end-diastolic volume (LVEDV) calculation is important in decision making in children with congenital heart disease (CHD). This study examined the accuracy of LVEDV calculation by the formula: 5/6 × area × length (“bullet” method), by comparing echocardiographic (ECHO) LVEDV against cardiac magnetic resonance (CMR). Methods: ECHO and CMR data from 72 consecutive subjects with CHD ≤10 years of age were retrospectively reviewed. Exclusion criteria included: >3 months between examinations; unavailable or poor-quality images. Subxiphoid short- and long-axis echocardiographic images were analyzed. Bullet LVEDV was compared to CMR volumes. Results: Seventeen subjects (median age 5.5 years, range 1–10 years) comprised the study group. ECHO and CMR LVEDV showed an excellent correlation (r = 0.97) and a good agreement with a mean difference (limits of agreement) of −1.0 mL (−13.9 to 11.9 mL). Conclusions: Subxiphoid echocardiographic bullet method of measuring LVEDV shows a good agreement with CMR obtained LV size in young children. (Echocardiography 2010;27:691-695)

The left atrial appendage is a complex structure that exhibits great variability in structure and form. Hence, it may masquerade as several other cardiac structures by echocardiography and cause errors in diagnosis. This report critically reviews the pitfalls that result in misinterpretation of normal and abnormal anatomy in a pediatric echocardiogram, and discusses and illustrates potential misdiagnoses and methods to avoid them.

Objective We sought to explore the relationship of color Doppler tissue imaging–derived systolic indices of tricuspid valve annular motion and magnetic resonance imaging–derived right ventricular (RV) ejection fraction in patients with congenital heart disease. Methods Patients with congenital heart disease who underwent echocardiography and magnetic resonance imaging on the same day were included. The tricuspid valve annular color Doppler tissue imaging–derived parameters of peak velocity during isovolumic contraction, myocardial acceleration during isovolumic contraction, peak systolic velocity, and Tei index were compared with magnetic resonance imaging–derived RV ejection fraction. Results Peak systolic velocity and myocardial acceleration during isovolumic contraction correlated well with RV ejection fraction after adjusting for age, RV dilation, and pressure overload ( r = 0.65 and 0.73, respectively). Interobserver and intraobserver reliability were excellent for peak systolic velocity ( r = 0.95 and 0.97, respectively) and very good for myocardial acceleration during isovolumic contraction ( r = 0.93 and 0.85, respectively). Conclusions Color Doppler tissue imaging indices of tricuspid valve annular motion are reproducible and provide a potentially useful complementary tool for assessment of RV systolic function in patients with congenital heart disease.

Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications.Large defect size and deficient rims have been described as predictors for complications and unsuccessful device placement in closure of interatrial communications. MASP is an underappreciated morphologic atrial septal variation whose prevalence in isolated atrial communications and its influence on percutaneous device closure has not been systematically assessed.We retrospectively evaluated echocardiographic data of 328 patients scheduled for percutaneous secundum atrial septal defect (ASD) and patent foramen ovale (PFO) closure between January 2006 and January 2013. In ASD patients, defect size was measured by balloon stretch diameter while the length of the surrounding rims was measured in standard transthoracic and transesophageal views. Furthermore, the distance between septum primum and septum secundum as it attaches to the left atrial roof was evaluated in both, ASD and PFO patients. Septal anatomy of patients with procedural failure was compared to the anatomy of patients in whom the procedure was successful.Transcatheter defect closure was successful in 131 (92.3%) of 142 ASD patients (32.3 years ± 24.7 years) and in all 186 PFO patients (49.6 years ± 16.2 years). Thirteen (4%) patients were found to have MASP (1.6 mm-13 mm). Smaller retroaortic rim, inferior rim, and the presence of a MASP were independently associated with procedural failure in ASD patients (P = 0.02, P = 0.03, and P = 0.003, respectively).Smaller retroaortic and inferior rims, as well as MASP are independent risk factors for unsuccessful transcatheter ASD closure.

Left ventricular end-diastolic volume (LVEDV) calculation is important in decision making in children with congenital heart disease (CHD). This study examined the accuracy of LVEDV calculation by the formula: 5/6 x area x length ("bullet" method), by comparing echocardiographic (ECHO) LVEDV against cardiac magnetic resonance (CMR).ECHO and CMR data from 72 consecutive subjects with CHDor =10 years of age were retrospectively reviewed. Exclusion criteria included:3 months between examinations; unavailable or poor-quality images. Subxiphoid short- and long-axis echocardiographic images were analyzed. Bullet LVEDV was compared to CMR volumes.Seventeen subjects (median age 5.5 years, range 1-10 years) comprised the study group. ECHO and CMR LVEDV showed an excellent correlation (r = 0.97) and a good agreement with a mean difference (limits of agreement) of -1.0 mL (-13.9 to 11.9 mL).Subxiphoid echocardiographic bullet method of measuring LVEDV shows a good agreement with CMR obtained LV size in young children.

Background: Many methods are used to measure arterial elasticity in children using echocardiography. There is no data to support the equivalence of the different techniques. The goal of this study was to evaluate the reproducibility of several techniques used to measure arterial elasticity using echocardiography. Methods: Aortic distension in two different sites (arterial distension) through the cardiac cycle was measured by (four) two-dimensional (2D) and M-mode echocardiographic techniques in 20 children without significant structural heart disease. These measurements combined with noninvasive blood pressure measurements were used to calculate arterial elastic indices. Arterial elasticity was expressed in terms of distensibility and stiffness. Data were collected by two sonographers and interpreted by two reviewers. Paired Student's t-test and Pitman's test for equality of variance for correlated observations were used to detect differences between different sonographers, different reviewers, and different techniques. Results: No significant difference in the measured elasticity between sonographers or reviewers was observed. There was a somewhat increased variance in two of the four techniques evaluated. There was no significant difference in elasticity measured using different techniques to evaluate the same arterial site, although a significantly decreased elasticity was noted from measurements taken in the proximal ascending aorta as compared with the distal ascending aorta. Conclusions: Many echocardiographic techniques produce reproducible measurements of arterial elasticity. There may be intrinsic differences in arterial elasticity between different segments of the ascending aorta, which have not been previously described in children with normal cardiac anatomy. Comparisons of data from separate studies must take these differences into account.

Tetralogy of Fallot is generally considered to be the most common congenital heart defect associated with an aberrant subclavian artery (ASA), but the prevalence of ASA in patients with other cardiac anomalies is not well described. The pediatric echocardiography database, with 15,871 initial echocardiograms, was searched for all patients with ASA. Arch laterality and associated intracardiac anomalies were documented for each patient. ASA was found in 226 patients, of whom 171 had a left aortic arch (LAA) and 55 had a right aortic arch (RAA). The occurrence of ASA was 1% in patients with LAA (171 of 15,650) and 25% in patients with RAA (55 of 221; p = 0.001). Intracardiac anatomy was normal in 32% of patients with ASA/LAA and 25% with ASA/RAA. Conotruncal anomalies occurred more frequently with ASA/RAA than ASA/LAA (36% vs 18%; p = 0.01). Atrioventricular canal defects accounted for 10% and left-sided cardiac obstructive lesions accounted for 11% of subjects with ASA/LAA. ASA was rarely associated with d-transposition of the great arteries (1 of 226) and double-outlet right ventricle (5 of 226). The prevalence of ASA was highest in patients with interrupted aortic arch (11 of 38; 29%). In patients with tetralogy of Fallot, the overall prevalence of ASA was 8% (34 of 447), but was higher with RAA (16 of 103; 16%). The highest prevalence of ASA occurred in the subgroup of patients with tetralogy of Fallot with pulmonary atresia and RAA (6 of 25; 24%). In conclusion, ASA was more common in patients with RAA, especially with conotruncal anomalies. In patients with LAA, hypoplastic left heart syndrome, aortic coarctation, and atrioventricular canal defects were commonly associated with ASA.

We describe a rare association between congenital muscular ventricular septal aneurysm (MuVSA) and right heart lesions, with emphasis on echocardiographic diagnosis, clinical presentation, and outcome. Our four cases of MuVSA occurred in combination with either pulmonary atresia with intact ventricular septum (PAIVS; three patients) or absent pulmonary valve syndrome with severe tricuspid stenosis (one patient). MuVSA and right heart lesions were detected in utero in three patients. The three patients with PAIVS and MuVSA survived with no significant morbidity from the septal aneurysm at 3- to 5-year follow-up. The presence of MuVSA did not negatively affect the outcome of our patients.

Aortic arch branching consisting of a common origin of the innominate and carotid arteries (COIC) is a normal variant of aortic arch branching in which the right innominate artery and the left common carotid artery share a common trunk and the left subclavian artery arises separately, leaving only two branches arising from the aortic arch. Adachi classified this branching pattern as type B in 1928, but it has also been referred to as a bovine arch, a common brachiocephalic trunk, and, at our institution, an ovine arch. The prevalence of COIC has been reported to be anywhere from 3% to 29% based on angiographic and autopsy studies that comprise relatively small sample sizes. This wide range suggests that the true prevalence is unclear. In addition, the prevalence in individuals with and without structural heart disease has not been clearly defined. Detailed and accurate preoperative anatomic diagnosis is critical to the surgical management of congenital heart disease. Knowledge of the brachiocephalic artery anatomy is relevant in certain surgical procedures and, thus, is routinely defined and communicated to the surgical team. The objectives of this study are to: determine the prevalence of COIC in a large population of individuals with and without structural heart disease; discuss the appropriate nomenclature of this variant; and describe the potential surgical implications.

The right ventricle of subjects after infundibulotomy (n = 20) and of subjects free of right ventricular disease (n = 19) was partitioned into the infundibular and sinus segments to examine the relative size and function of each. Excellent agreement was found between observers for measuring regional volume and ejection fraction. Although the surgical subjects had lower infundibular ejection fraction and higher indexed volumes, most had preserved sinus ejection fraction.