David Saudek

Multicenter study comparing shunt type in the norwood procedure for single-ventricle lesions: three-dimensional echocardiographic analysis.

Background—The Pediatric Heart Network’s Single Ventricle Reconstruction (SVR) trial randomized infants with single right ventricles (RVs) undergoing a Norwood procedure to a modified Blalock-Taussig or RV-to-pulmonary artery shunt. This report compares RV parameters in the 2 groups using 3-dimensional echocardiography. Methods and Results—Three-dimensional echocardiography studies were obtained at 10 of 15 SVR centers. Of the 549 subjects, 314 underwent 3-dimensional echocardiography studies at 1 to 4 time points (pre-Norwood, post-Norwood, pre–stage II, and 14 months) for a total of 757 3-dimensional echocardiography studies. Of these, 565 (75%) were acceptable for analysis. RV volume, mass, mass:volume ratio, ejection fraction, and severity of tricuspid regurgitation did not differ by shunt type. RV volumes and mass did not change after the Norwood, but increased from pre-Norwood to pre–stage II (end-diastolic volume [milliliters]/body surface area [BSA]1.3, end-systolic volume [milliliters]/BSA1.3, and mass [grams]/BSA1.3 mean difference [95% confidence interval]=25.0 [8.7–41.3], 19.3 [8.3–30.4], and 17.9 [7.3–28.5], then decreased by 14 months (end-diastolic volume/BSA1.3, end-systolic volume/BSA1.3, and mass/BSA1.3 mean difference [95% confidence interval]=−24.4 [−35.0 to −13.7], −9.8 [−17.9 to −1.7], and −15.3 [−22.0 to −8.6]. Ejection fraction decreased from pre-Norwood to pre–stage II (mean difference [95% confidence interval]=−3.7 [−6.9 to −0.5]), but did not decrease further by 14 months. Conclusions—We found no statistically significant differences between study groups in 3-dimensional echocardiography measures of RV size and function, or magnitude of tricuspid regurgitation. Volume unloading was seen after stage II, as expected, but ejection fraction did not improve. This study provides insights into the remodeling of the operated univentricular RV in infancy. Clinical Trial Registration—URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Quantitative echocardiographic measures in the assessment of single ventricle function post‐Fontan: Incorporation into routine clinical practice

Quantitative echocardiographic measurements of single ventricular (SV) function have not been incorporated into routine clinical practice.

Regional Implementation of a Pediatric Cardiology Syncope Algorithm Using Standardized Clinical Assessment and Management Plans (SCAMPS) Methodology

Background Pediatric syncope is common. Cardiac causes are rarely found. We describe and assess a pragmatic approach to these patients first seen by a pediatric cardiologist in the New England region, using Standardized Clinical Assessment and Management Plans (SCAMPs). Methods and Results Ambulatory patients aged 7 to 21 years initially seen for syncope at participating New England Congenital Cardiology Association practices over a 2.5‐year period were evaluated using a SCAMP. Findings were iteratively analyzed and the care pathway was revised. The vast majority (85%) of the 1254 patients had typical syncope. A minority had exercise‐related or more problematic symptoms. Guideline‐defined testing identified one patient with cardiac syncope. Syncope Severity Scores correlated well between physician and patient perceived symptoms. Orthostatic vital signs were of limited use. Largely incidental findings were seen in 10% of ECGs and 11% of echocardiograms. The 10% returning for follow‐up, by design, reported more significant symptoms, but did not have newly recognized cardiac disease. Iterative analysis helped refine the approach. Conclusions SCAMP methodology confirmed that the vast majority of children referred to the outpatient pediatric cardiology setting had typical low‐severity neurally mediated syncope that could be effectively evaluated in a single visit using minimal resources. A simple scoring system can help triage patients into treatment categories. Prespecified criteria permitted the effective diagnosis of the single patient with a clear cardiac etiology. Patients with higher syncope scores still have a very low risk of cardiac disease, but may warrant attention.

MRI validated echocardiographic technique to measure total cardiac volume: a tool for donor-recipient size matching in pediatric heart transplantation.

Our aim is to develop and validate an accurate method for estimating TCV using standard echocardiographic imaging that can be easily employed to aid in donor–recipient size matching in pediatric heart transplantation. Thirty patients who underwent Echo and cardiac magnetic resonance imaging (cMRI) were identified. TCV was measured on cMRI. TCV was determined echocardiographically by two methods: a volume measurement using the modified Simpsons method on a four‐chamber view of the heart; and a calculated volume measurement which assumed a true‐elliptical shape of the heart. These two methods where compared with the value obtained by cMRI using the concordance correlation coefficient (CCC). TCV by method 1 correlated well with cMRI (CCC = 0.98%, CI = 0.97, 0.99). TCV by method 2 had a CCC = 0.90 (CI = 0.9464, 0.9716) when compared to cMRI. Left ventricular end‐diastolic volume (LVEDV) also correlated as a predictor of TCV in patients with structurally normal hearts and could be described by the equation: TCV = 6.6 (LVEDV) + 12 (R2 = 0.97). Echocardiographic assessment of TCV for recipients and their potential donors is a simple process and can be prospectively applied as part of donor evaluation.

Delayed Contrast-Enhanced Magnetic Resonance Imaging in the Evaluation of Takayasu Arteritis

![Figure][1] A 12-year-old Hispanic girl was referred for evaluation of hypertension and a heart murmur. Transthoracic echocardiogram revealed long-segment coarctation of the aorta. (A) Gadolinium-enhanced magnetic resonance angiography confirmed long-segment narrowing of the aortic arch

Single-Stage Repair of Coarctation of the Aorta and Ventricular Septal Defect: A Comparison of Surgical Strategies and Resource Utilization:

Background: We sought to compare clinical outcomes and resource utilization for two surgical approaches for single-stage repair of coarctation of the aorta and ventricular septal defect (VSD). Methods: This was a retrospective chart review of 21 consecutive neonates and infants undergoing single-stage repair of coarctation of the aorta and VSD. Group 1 included 13 patients with both arch repair and VSD repair completed via sternotomy. Group 2 included eight patients with off-pump arch repair via left thoracotomy followed by repositioning and VSD repair via sternotomy. Primary clinical outcome was arch reintervention. Secondary outcomes included various measures of resource utilization. Results: Group 1 patients demonstrated younger age at repair (median of 10 days vs 57 days for group 2; P = .05) and lower proximal arch z scores (−4.2 vs −2.3 for group 2; P = .003). Arch reintervention occurred in 0 of 8 patients in group 2 and 1 (7.7%) of 13 patients in group 1 (P = nonsignificant). Group 2 was associated with lower total charges (US

Transplacental Treatment of Fetal Tachycardia: A Systematic Review and Meta-analysis

68,301 vs US

Utility of speckle tracking echocardiography in measuring systemic right ventricular systolic function for patients with d-transposition of the great arteries status post atrial switch procedure: a comparison with cardiac magnetic resonance imaging

211,723 for group 1; P = .0007), shorter length of stay (8 days vs 23 days for group 1; P = .004), and shorter duration of postoperative mechanical ventilation (0.5 days vs 4.0 days for group 1; P = .0008). Group 2 was also associated with shorter total cardiopulmonary bypass time (86 minutes vs 201 minutes for group 1; P = .0009). Conclusion: Single-stage two-incision repair of coarctation and VSD in appropriately selected patients may be associated with higher value of care. Confirmation of this finding will require further study based on larger numbers of patients.

Impact of initial norwood shunt type on right ventricular deformation: The single ventricle reconstruction trial

Multiple transplacental medications can be used to treat fetal tachycardia. We sought to perform a systematic review and meta‐analysis to determine whether digoxin, flecainide, or sotalol was the most efficacious therapy for converting fetal tachycardia to sinus rhythm.

496 MRI Validated Echocardiographic Technique To Measure Total Cardiac Volume: A Tool for Donor Recipient Size Matching

Background Patients with d-transposition of the great arteries (D-TGA), status post atrial switch, are at risk for developing systemic right ventricular (RV) dysfunction. Echocardiographic (Echo) assessment of RV function is subjective because complex RV geometry does not allow accurate determination of ejection fraction (EF). RVEF measured by cardiac magnetic resonance imaging (MRI) is the gold standard for quantitative assessment of systemic RV function. New Echo measures of ventricular deformation allow for quantitative assessment of RV function. The primary aim was to explore the correlation of global peak longitudinal strain (GPLS) of the systemic RV with MRI RVEF for patients with D-TGA status post atrial switch. The secondary aim was to characterize MRI and clinical findings in this population.