H. Helen Ko

Multimodality Imaging Guidelines for Patients with Repaired Tetralogy of Fallot: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology

1. Executive Summary 112 Goals of Imaging 112 Imaging Modalities 112 Echocardiography 113 CMR 113 Cardiovascular CT 113 Nuclear Scintigraphy 113 X-Ray Angiography 113 Multimodality Imaging 113 2. Background 113 3. General Considerations 114 4. Goals of Imaging 114 5. Echocardiography 114 a. Overview of Modality 114 b. Strength and Limitations 115 c. Assessment of Repaired TOF with Echocardiography 115 RVOT 115 PAs 115

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.

The Accuracy of Echocardiographic Assessment of Left Ventricular Size in Children by the 5/6 Area × 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 × 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)

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 Students t‐test and Pitmans 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.

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.

Echocardiographic Myocardial Deformation Evaluation of Right Ventricular Function in Comparison with CMRI in Repaired Tetralogy of Fallot: A Cross‐Sectional and Longitudinal Validation Study

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.

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

Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications.