Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

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

Application of Virtual Three-Dimensional Models for Simultaneous Visualization of Intracardiac Anatomic Relationships in Double Outlet Right Ventricle.

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.

Malattached septum primum and deficient septal rim predict unsuccessful transcatheter closure of atrial communications.

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.

Two-dimensional speckle-tracking-derived segmental peak systolic longitudinal strain identifies regional myocardial involvement in patients with myocarditis and normal global left ventricular systolic function.

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.

Applicability of published guidelines for assessment of left ventricular diastolic function in adults to children with restrictive cardiomyopathy: an observational study.

Guidelines for diagnosis and grading of diastolic dysfunction (DD) in children have not been established. The applicability of adult parameters of DD to children has been questioned by recent studies. Although normal diastolic parameters in children have been published, the data to support application of these indices for the non-invasive diagnosis of DD and quantifying its degree are still being developed. Restrictive cardiomyopathy (RCM) is the only recognized disease entity in children that presents with isolated, irreversible DD as the predominant finding. The aim of this study was to investigate the applicability of current diastolic indices used for assessment of diastolic function in adults as reliable indicators of DD in children with established RCM. Retrospective review of institutional clinical database for the period of 2002-2010 was performed to identify patients with RCM who had had a comprehensive echocardiographic assessment of diastolic function. The following parameters were obtained from apical four chamber view: mitral valve (MV) inflow Doppler early filling velocity (E), late filling velocity (A), deceleration time (DT), color M-mode flow propagation from MV to apex (Vp), Doppler tissue imaging derived early diastolic velocity E' and late diastolic velocity A' at the LV lateral wall at MV annulus, RV at the tricuspid valve annulus, septum, and LA area. All parameters were compared to age and gender matched controls using student t test. : LA area/BSA was significantly larger in RCM group than the control group, median 22.8 cm(2)/m(2) (range 16.9-28.6) versus 10.3 cm(2)/m(2) (range 8.3-12.3), p value <0.001. MV inflow E and A were lower, and DT was shorter in the RCM group (p = 0.04, 0.02, and 0.005, respectively). A wave was absent in 3 of 9 patients in the RCM group. Ratio of E to A (E/A) was not different between the two groups. E' was significantly lower at all three sites in RCM group; however, there was some overlap between the two groups. E/septal E' ratio was statistically significantly higher in RCM group. A' was absent either at lateral wall or at septum in five patients. 7 of 9 patients in RCM group had L' wave (at lateral wall or septum) defined as negative deflection during diastasis. Vp was higher in RCM group than in the control group 81.4 ± 44.5 versus 52.9 ± 10.9, p value <0.01. Combination of increased left atrial size, septal E/E', and lack of A wave and presence of mid-diastolic L'-wave are the noted abnormalities in this group. Individual cut-offs for Doppler indices have very poor sensitivity in identifying restrictive physiology. These findings suggest that poor LV compliance is the hallmark of restrictive cardiomyopathy in children even in the presence of normal early relaxation and ventricular filling. These findings support the need for development of guidelines for diagnosis and physiologic grading of diastolic dysfunction in children.

Prevalence and outcomes of coronary artery ectasia associated with isolated congenital coronary artery fistula.

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.

The role of right ventricular function in paediatric idiopathic dilated cardiomyopathy.

INTRODUCTION: The prevalence of right ventricular dysfunction in idiopathic dilated cardiomyopathy is incompletely studied in children. Furthermore, right ventricular function may signal worse outcomes. We evaluated recently published right ventricular function echocardiographic indices in identifying dysfunction in children with idiopathic dilated cardiomyopathy and the impact of right ventricular dysfunction on long-term prognosis. METHODS: A retrospective database review of right ventricular function indices in 30 patients with idiopathic dilated cardiomyopathy was compared with 60 age- and sex-matched controls from January, 2001 until December, 2010. Right ventricular function was assessed by Doppler tissue peak systolic S', early and late diastolic E' and A' waves and isovolumic acceleration at the tricuspid valve annulus; pulsed wave Doppler tricuspid valve inflow E and A waves; right ventricular myocardial performance index; tricuspid annular plane systolic excursion; right ventricular fractional area change. RESULTS: Right ventricular systolic and diastolic function in idiopathic dilated cardiomyopathy was significantly impaired. All measured indices except for isovolumic acceleration and fractional area change were significantly reduced, with a p-value less than 0.05. There was no right ventricular index predictive of death or transplantation. Patients with poor outcome were significantly more likely to need inotropic support (p-value equal to 0.018), be placed on a ventricular assist device (p equal to 0.005), and have a worse left ventricular ejection fraction z-score (p-value equal to 0.002). CONCLUSION: Right ventricular dysfunction is under-recognised in children presenting with idiopathic dilated cardiomyopathy. The need for clinical circulatory support and left ventricular ejection fraction z-score less than minus 8 were primary determinants of outcome, independent of the degree of derangement in right ventricular function.

Imaging of congenital coronary artery anomalies.

Imaging of the coronary arteries is an important part of the evaluation of children with congenital heart disease and isolated congenital coronary artery anomalies. Echocardiography remains the main imaging modality and is complemented by MRI and CT angiography in the older or difficult-to-image child. We review echocardiography, MRI, and CT angiography for coronary artery imaging, with emphasis on techniques. The clinical implications of isolated congenital coronary artery anomalies are also addressed, along with a discussion about the current consensus on optimal management of these anomalies.

Normal values for left ventricular volume in infants and young children by the echocardiographic subxiphoid five-sixth area by length (bullet) method.

BACKGROUND: Left ventricular (LV) end-diastolic volume (LVEDV) can be estimated by the formula (5/6) x area x 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 < .01). There was no residual relationship between LVEDV/BSA(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.

The accuracy of echocardiographic assessment of left ventricular size in children by the 5/6 area x length (bullet) method.

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 x area x length ("bullet" method), by comparing echocardiographic (ECHO) LVEDV against cardiac magnetic resonance (CMR). METHODS: ECHO and CMR data from 72 consecutive subjects with CHD < or =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.

A comparison of echocardiographic techniques in determination of arterial elasticity in the pediatric population.

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.

Frequency of aberrant subclavian artery, arch laterality, and associated intracardiac anomalies detected by echocardiography.

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.

Echocardiographic diagnosis of clinically silent congenital coronary artery anomalies.

BACKGROUND: The incidence of congenital coronary anomalies is mainly derived from autopsy series and pre-participation exams in athletes. Limited data exist regarding the spectrum of coronary anomalies that can be detected in asymptomatic patients. We sought to describe echocardiographically detected congenital coronary artery anomalies in asymptomatic children after implementing a screening protocol mandating identification of coronary artery origin and proximal course in all initial studies. METHODS: Our database was searched from 1/1/1993 to 3/31/2006 and all echocardiograms coded for coronary anomalies were identified. Clinically "silent" congenital coronary anomalies were culled from that group. RESULTS: Of the 168 "silent" coronary anomalies detected, 111 were anomalies of aortic origin, including 59 patients with "high coronary takeoff" and 30 patients with "wrong sinus" origin of either the left or right coronary artery. Small coronary fistulas were seen in 57. Associated congenital heart defects were found in 53% of individuals with coronary anomalies. CONCLUSIONS: This study comprises the largest group of echocardiographically detected, "silent" but potentially clinically significant, congenital coronary anomalies in children. Prospective echocardiographic diagnosis of "high coronary takeoff", a risk factor for injury during cardio-pulmonary bypass, and asymptomatic intraseptal coronary stenosis are described for the first time.

Congenital muscular ventricular septal aneurysm: report of four cases and review of the literature.

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.

Left atrial appendage: variations in morphology and position causing pitfalls in pediatric echocardiographic diagnosis.

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.

Color Doppler tissue imaging for evaluation of right ventricular systolic function in patients with congenital heart disease.

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.

Regional differences in right ventricular systolic function as determined by cine magnetic resonance imaging after infundibulotomy.

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.