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Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is a rare congenital cardiac malformation. We reviewed the current literature and found only 31 patients with ARCAPA. We report the first case that was diagnosed and followed on a noninvasive basis with cardiovascular magnetic resonance after surgical re-implantation. This report of a patient with ARCAPA showed resolving coronary artery sizes secondary to decreased pulmonary steal. Cardiovascular magnetic resonance is an accurate and reliable imaging modality that allows serial noninvasive follow up in patients with coronary artery anomalies.

Background Adenosine has traditionally been utilized for coronary hyperemia during myocardial perfusion assessment in adults, however regadenoson has recently become more popular [1]. With improved survival of congenital heart disease (CHD), and increased diagnosis of acquired heart disease (AHD), there’s an increasing need for assessment of myocardial perfusion in pediatrics [2]. Only dipyridamole and adenosine have reportedly been utilized as hyperemia stressors in CHD patients. As a selective A2A agonist, regadenoson has a more favorable side-effect profile and less stimulation of receptors associated with bronchospasm, and decreased chronotropy [1]. It is administered as an intravenous (IV) bolus and thus only a single IV is required. Peak onset is at 60-90 seconds, with hyperemia lasting up to 6 minutes allowing additional wall motion assessment [3].

Background Rarely, patients with normally sized RV and LV will undergo total cavopulmonary connection (TCPC) due to the complexity of their intracardiac anatomy giving them a dual ventricle (DV) for a single cardiac output. The ventricular function in this unique physiology compared to SRV, SLV and normal hearts remains poorly understood, with few studies performed. In this study, we perform a comprehensive comparison of global and regional strain in both the circumferential (ecc) and longitudinal (eL) dimensions to conventional SV hearts and normal hearts.

Background Accurate measurement of maximal aortic root dimensions is important for informed decision making on the timing/ nature of aortic valve replacement surgeries in pediatric patients. Currently, the observer manually measures the following metrics to quantify maximum aortic root dimensions: 1) Cusp to Commisure (Cu-Co), and 2) Cusp to Cusp (Cu-Cu) lengths. This introduces significant interobserver variability (IOV), especially if a followup study is performed in a different institution than the previous

Methods Five patients with CHD were included in this pilot study. The MR studies were performed on a Philips Acheiva 1.5T magnet using a multielement phased array coil with the following sequences in this order: 1. Free-breathing, respiratory synchronized [1], time-resolved MRA following injection of 0.03 mmol/kg of Gadofosveset, and injection rate 2-4cc/second using a power injector. Duration: < 1 minute 2. Free breathing equilibrium phase MRA, acquired voxel size 0.8 × 0.8 × 1.6 mm, 2 NEX. Duration: 2.5-4 minutes 3. Free breathing 3D cine SSFP with respiratory triggering (TR/TE/flip angle: 3/1.5/60; acquired voxel size: 1.5-1.9 × 1.5-2.1 × 7-8 mm3; SENSE acceleration factor: 1.5-2 × 1.5-2; temporal resolution: 30-45 ms). Duration: 4.5-7 minutes 4. Free breathing sagittal 4D phase contrast (PC) imaging with respiratory navigator (18-26 phases/cardiac cycle, Venc 150 cm/sec, spatial resolution 1.6-2.8 mm3.) Duration: 6-12 minutes 5. Free breathing 3D SSFP with respiratory navigator. (acquired voxel size 1 × 1 × 2 mm3) Duration: 5-7 minutes Comparative data was obtained using conventional 2D cine respiratory triggered SSFP sequences (2) in the VLA, 4 chamber and short axis planes, and 2D PC imaging. Data Analysis: Image quality assessment and quantitative volumetric and flow analysis was performed by a single blinded user. MRA images were graded using a semi-quantitative scale from 1-5 for relevant imaging targets in CHD [1], with 1: excellent, no limitations, and 5: non-diagnostic. The clinical scoring system for 2D and 3D cine SSFP was based on blood-myocardial contrast, endocardial edge definition and inter-slice alignment[2]. Paired t-test analysis was performed on LV and RV volumes.

Background Ventricular dysfunction in patients with a single right ventricle (SRV) or as ingle left ventricle (SLV) is a known risk factor for morbidity and mortality. However, the differences in SRV and SLV function remain poorly understood, with only a few studies performed1-3. In this study, we measure the strain using cardiac MRI and perform comprehensive comparison of the global and regional strain in both the circumferential (ecc) and longitudinal (eL) dimension. Purpose: In normal subjects and asymptomatic patients with SLV and SRV after total cavopulmonary connection (TCPC), compare: 1) Global ecc and eL strain, 2) Regional circumferential and longitudinal strains at free wall (ecc-free, eL-free) and septum (ecc-sept, eL-sept), 3) ecc and eL across the ventricle from apex to base. Methods We performed a prospective analysis of 18 subjects (7 normal age: 11.8 +/- 3; 6 SRV age: 11.4 +/- 2.3; 5 SLV age: 12.7 +/- 4.2). Acquisition Protocol: Strain information was acquired at three short axis slices at basal, mid-cavity, and apical locations in all 18 subjects in a 1.5T MRI scanner (Philips Acheiva) using: a) Complementary Spatial Modulation of Magnetization (CSPAMM)4 images: Used for generating ecc; and b) Fast-Strain Encoded (fSENC)5 images: Used for generating eL. Data Analysis: ecc and eL across all cardiac phases and slices were calculated from SAX slices using DiagnosoftTM. The ventricular regions at each slice were assigned based upon the AHA 16 segment model. ecc-sept, eL-sept, ecc-free, and eL-free were also calculated for each slice and compared. Results

Background Abnormal myocardial rotation and torsion have been investigated earlier as markers of ventricular failure1-2. However, there are no previous studies on global or regional torsion in functional single ventricle (SV) or the contribution of torsion to biomechanical differences between single RV (SRV) and single LV (SLV) hearts. Additionally, regional variations in rotation between the ventricular free wall and the septal wall which borders the hypoplastic chamber in SV have not been studied. Purpose: In normal subjects and in asymptomatic patients with SLV and SRV after total cavopulmonary connection (TCPC), to compare: 1) Global rotation (ROTglo). 2) Regional rotation in systole at free wall (ROTfree) and septal wall (ROT sept) for each slice location. 3) Torsion across the ventricle from apex to base.

Background Turner syndrome is associated with bicuspid aortic valve (BAV), coarctation of the aorta, aortic dilation, and aortic dissection. Vertebral artery tortuosity, as demonstrated by magnetic resonance angiography (MRA), is increased in other disorders associated with thoracic aortic dilation and dissection, including Marfan syndrome and Loeys-Dietz syndrome, and increased tortuosity is associated with earlier adverse cardiovascular outcomes in those groups. We investigated the association between vertebral artery tortuosity and aortic pathology in patients with Turner syndrome.

Background Cine steady-state free precession (SSFP) is the preferred sequence for ventricular function evaluation. However, SSFP demands uninterrupted RF excitation to maintain steady-state (SS) during suspended respiration. This is feasible in adults who can perform breath-holding (BH), but is difficult to accomplish in sedated or uncooperative children. To overcome this, many pediatric groups routinely perform multi-NSA acquisitions (MN) during free breathing. In this work, we validate a respiratory triggered (RT) SSFP sequence that drives the magnetization to steady-state before commencing cardiac gated cine acquisition in sedated pediatric population.

Background Compliance is an important physical property of the aorta, reflecting its ability to work as a buffer during the systolic phase of cardiac cycle. There is a great interest in studying ventriculoarterial interations and aortic properties like stiffness and distensibility in patients with congenital heart disease due to their ability to predict cardiovascular mortality [1]. A surrogate marker of arterial stiffness is pulse wave velocity (PWV), which has been linked to prognostically adverse cardiac phenotypes in adults. There is little data on PWV in patients who have undergone single ventricle repair.

Methods 18 patients age range 0-10 years (12/18 were

Background In pilot studies, increased vascular tortuosity is associated with adverse outcomes in children and young adults withheritable thoracic aortic disease, specifically Marfan syndrome (MFS) and Loeys Dietz syndrome (LDS). It is known that vertebral artery tortuosity is highly prevalent in older adults who do not have heritable thoracic aortic disease. We hypothesized that tortuosity increases with age in patients with aortic disease

Introduction Dilated aortic roots occur in patients with congenital heart disease (CHD), bicuspid aortic valve (BAV), or with connective tissue disease. When severe, root dilation can lead to significant morbidity and mortality. Decisions regarding aortic valve/root replacement are based on root size, rate of change over time, and the degree of aortic stenosis and regurgitation, so accurate quantification of root size and progression is vital to patient management. Cardiovascular MRI provides more reproducible and accurate measurement of aortic roots than echocardiography, but the optimal imaging sequences have not been well studied.

Background Cine balanced steady-state free precession (SSFP), the preferred sequence for ventricular function, demands uninterrupted radio frequency (RF) excitation to maintain the steady-state during suspended respiration. This is difficult to accomplish in sedated children. In this work, we validate a respiratory triggered (RT) SSFP sequence that drives the magnetization to steady-state before commencing retrospectively cardiac gated cine acquisition in a sedated pediatric population. Methods This prospective study was performed on 20 sedated children with congenital heart disease (8.6 ± 4 yrs). Identical imaging parameters were used for multiple number of signal averages (MN) and RT cine SSFP sequences covering both the ventricles in short-axis (SA) orientation. Image quality assessment and quantitative volumetric analysis was performed on the datasets by two blinded observers. One-sided Wilcoxon signed rank test and Box plot analysis were performed to compare the clinical scores. Bland-Altman (BA) analysis was performed on LV and RV volumes. Results Scan duration for SA stack using RT-SSFP (3.9 ± 0.8 min) was slightly shorter than MN-SSFP (4.6 ± 0.9 min) acquisitions. The endocardial edge definition was significantly better for RT than MN, blood to myocardial contrast was better for RT than MN without reaching statistical significance, and inter slice alignment was comparable. BA analysis indicates that the variability of volumetric indices between RT and MN is comparable to inter and intra-observer variability reported in the literature. Conclusions The free breathing RT-SSFP sequence allows diagnostic images in sedated children with significantly better edge definition when compared to MN-SSFP, without any penalty for total scan time.

Background This is a pilot study to evaluate the performance of whole-heart isotropic RT-3D cine SSFP sequence as an efficient single solution for assessment of morphology and function in CHD by comparing it to: 1. Short axis (SA) 2D cine SSFP for LV volumetry and function 2. Multiplanar 2D cine SSFP for intracardiac morphology 3. Cine 2D SSFP and gadolinium (Gd)-enhanced 3D MRA for aortic root size 4. Gd-3D MRA for vessel diameter (VD) 5. Gd-3D MRA, and cine 2D SSFP for morphology of extracardiac vasculature 6. Gd-3D MRA and multiplanar cine 2D SSFP for overall diagnosis.

Background Myocardial tissue characterization with both native T1 mapping and T1 mapping following gadolinium based contrast agents (T1 enhanced) has emerged as an important asset of CMR imaging [1]. However, there is only minimal experience in pediatrics [2]. Native T1 has shown to be a marker of myocardial edema, and may play a role in pathologic states such as myocarditis [3]. T1 enhanced mapping has shown to be a useful biomarker for disease sates with diffuse fibrosis, such as hypertrophic cardiomyopathy (HCM), and is comparable to myocardial biopsy [4]. Purpose: To examine the effectiveness of CMR myocardial characterization by native T1 and T1 enhanced mapping in a heterogenous group of pediatric patients with signs concerning for cardiomyopathy or myocarditis.