Background Congenital Bicuspid Aortic Valve (BAV) is a significant risk factor for serious complications including valve dysfunction, aortic dilatation, dissection, and sudden death. Clinical tools for identification and monitoring of BAV patients at high risk for development of aortic dilatation, an early complication, are not available. Methods This paper reports an investigation in 18 pediatric BAV patients and 10 normal controls of links between abnormal blood flow patterns in the ascending aorta and aortic dilatation using velocity-encoded cardiovascular magnetic resonance. Blood flow patterns were quantitatively expressed in the angle between systolic left ventricular outflow and the aortic root channel axis, and also correlated with known biochemical markers of vessel wall disease. Results The data confirm larger ascending aortas in BAV patients than in controls, and show more angled LV outflow in BAV (17.54 ± 0.87 degrees) than controls (10.01 ± 1.29) (p = 0.01). Significant correlation of systolic LV outflow jet angles with dilatation was found at different levels of the aorta in BAV patients STJ: r = 0.386 (N = 18, p = 0.048), AAO: r = 0.536 (N = 18, p = 0.022), and stronger correlation was found with patients and controls combined into one population: SOV: r = 0.405 (N = 28, p = 0.033), STJ: r = 0.562 (N = 28, p = 0.002), and AAO r = 0.645 (N = 28, p < 0.001). Dilatation and the flow jet angle were also found to correlate with plasma levels of matrix metallo-proteinase 2. Conclusions The results of this study provide new insights into the pathophysiological processes underlying aortic dilatation in BAV patients. These results show a possible path towards the development of clinical risk stratification protocols in order to reduce morbidity and mortality for this common congenital heart defect.

Background: Improved delineation of vascular structures is a common indication for cardiovascular magnetic resonance (CMR) in children and requires high spatial resolution. Currently, pre-contrast 3D, respiratory navigated, T2-prepared, fat saturated imaging with a bSSFP readout (3D bSSFP) is commonly used; however, these images can be limited by blood pool inhomogeneity and exaggeration of metal artifact. We compared image quality of pediatric vasculature obtained using standard 3D bSSFP to 3D, respiratory navigated, inversion recovery prepared imaging with a gradient echo readout (3D IR GRE) performed after administration of gadofosveset trisodium (GT), a blood pool contrast agent. Methods: For both sequences, VCG triggering was used with acquisition during a quiescent period of the cardiac cycle. 3D bSSFP imaging was performed pre-contrast, and 3D IR GRE imaging was performed 5 min after GT administration. We devised a vascular imaging quality score (VIQS) with subscores for coronary arteries, pulmonary arteries and veins, blood pool homogeneity, and metal artifact. Scoring was performed on axial reconstructions of isotropic datasets by two independent readers and differences were adjudicated. Signal- and contrast-to-noise (SNR and CNR) calculations were performed on each dataset. Results: Thirty-five patients had both 3D bSSFP and 3D IR GRE imaging performed. 3D IR GRE imaging showed improved overall vascular imaging compared to 3D bSSFP when comparing all-patient VIQS scores (n = 35, median 14 (IQR 11-15), vs 6 (4-10), p < 0.0001), and when analyzing the subset of patients with intrathoracic metal (n = 17, 16 (14-17) vs. 5 (2-9), p < 0.0001). 3D IR GRE showed significantly improved VIQS subscores for imaging the RCA, pulmonary arteries, pulmonary veins, and blood pool homogeneity. In addition, 3D IR GRE imaging showed reduced variability in both all-patient and metal VIQS scores compared to 3D bSSFP (p < 0.05). SNR and CNR were higher with 3D IR GRE in the left ventricle and left atrium, but not the pulmonary arteries. Conclusions: Respiratory navigated 3D IR GRE imaging after GT administration provides improved vascular CMR in pediatric patients compared to pre-contrast 3D bSSFP imaging, as well as improved imaging in patients with intrathoracic metal. It is an excellent alternative in this challenging patient population when high spatial resolution vascular imaging is needed.

Background: Right ventricular dysfunction occurs in many patients with significant pulmonary valve regurgitation late after initial total repair of tetralogy of Fallot. Methods to predict which of these patients are at increased risk of late morbidity and mortality are not yet known. Hypothesis: This study evaluated electrocardiographic (ECG) predictors of severe right ventricular dilatation determined by magnetic resonance imaging (MRI) volumes in patients with tetralogy of Fallot late after initial corrective repair. Methods: We retrospectively reviewed the ECGs and MRI right ventricular volume measurements of 20 patients (age 4.4 to 19.3 years, mean 10.0 years) with significant pulmonary valve regurgitation late after repair of tetralogy of Fallot. All patients had enlarged, hypokinetic right ventricles by echocardiography. The patients were grouped based on an indexed fight ventricular end-diastolic volume (RVEDV/BSA) of < 102 ml/m2 (Group 1) or ≥102 ml/m2 (Group 2). We determined the sensitivity, specificity, positive and negative predictive values of QRS duration, and mean frontal plane QRS axis for predicting right ventricular volumes. Results: A maximal QRS duration of ≥ 150 ms or a northwest quadrant frontal plane ORS axis had 85% sensitivity, 86% specificity, 92% positive predictive value, and 75% negative predictive value for predicting an RVEDV/BSA of ≥102 ml/m2. The mean QRS duration was significantly longer in Group 2 than in Group 1 patients (156 ms vs. 123 ms, p = 0.005). Conclusions: In patients late after repair of tetralogy of Fallot with significant pulmonary valve regurgitation, a maximal manually measured QRS duration of ≥ 150 ms and/or a frontal plane QRS northwest quadrant axis can predict patients with marked fight ventricular enlargement. The presence of either of these findings on the ECG signifies patients who require further evaluation and consideration for pulmonary valve replacement.

Background: Using a bifurcated Y-graft as the Fontan baffle is hypothesized to streamline and improve flow dynamics through the total cavopulmonary connection (TCPC). This study conducted numerical simulations to evaluate this hypothesis using postoperative data from 5 patients. Methods: Patients were imaged with cardiac magnetic resonance or computed tomography after receiving a bifurcated aorto-iliac Y-graft as their Fontan conduit. Numerical simulations were performed using in vivo flow rates, as well as 2 levels of simulated exercise. Two TCPC models were virtually created for each patient to serve as the basis for hemodynamic comparison. Comparative metrics included connection flow resistance and inferior vena caval flow distribution. Results: Results demonstrate good hemodynamic outcomes for the Y-graft options. The consistency of inferior vena caval flow distribution was improved over TCPC controls, whereas the connection resistances were generally no different from the TCPC values, except for 1 case in which there was a marked improvement under both resting and exercise conditions. Examination of the connection hemodynamics as they relate to surgical Y-graft implementation identified critical strategies and modifications that are needed to potentially realize the theoretical efficiency of such bifurcated connection designs. Conclusions: Five consecutive patients received a Y-graft connection to complete their Fontan procedure with positive hemodynamic results. Refining the surgical technique for implementation should result in further energetic improvements that may help improve long-term outcomes.

OBJECTIVE Optimizing flow and diminishing power loss in the Fontan circuit can improve hemodynamic efficiency potentially improving long-term outcomes. Computerized modeling has predicted improved energetics with a Y-graft Fontan. METHODS From August to December, 2010, six consecutive children had a completion Fontan (n=3) or a Fontan revision (n=3) using a bifurcated polytetrafluoroethylene Y-graft (18×9×9 mm in 2, 20×10×10 mm in 4) connecting the inferior vena cava (IVC) to the right and left pulmonary arteries (PAs) with separate graft limbs. Patents were imaged by magnetic resonance imaging (MRI; n-5) or computerized tomography (n=1). Computational fluid dynamics (CFD) assessed Fontan hemodynamics, power loss, and IVC flow splits to the branch PAs. Clinical parameters were compared with 12 patients immediately preceding this series who had a lateral Fontan procedure. RESULTS Despite longer crossclamp and bypass times (not statistically significant), the Y-graft Fontan patients had postoperative courses similar to the conventional Fontan patients. Other than two early readmissions for pleural effusions managed with diuretics, on 6–12 months follow-up (mean 8 months), all six patients have done well. Postoperative flow modeling demonstrated balanced distribution of IVC flow to both PAs with minimal flow disturbance. Improvements in hemodynamics and efficiency were noted when the Y-graft branches were anastomosed distally and aligned tangentially with the branch PAs. CONCLUSIONS This preliminary surgical experience demonstrates clinical feasibility of the bifurcated Y-graft Fontan. CFD shows acceptable hemodynamics with low calculated power losses and balanced distribution of IVC flow to the PAs as long as the branch grafts are anastomosed distally.