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Reference values for cardiac magnetic resonance imaging (cMRI) in children and young adults are scarce. This leads to risk stratification of patients with congenital heart diseases being based on volumes indexed to body surface area (BSA). We aimed to produce cMRI Z score equations for ventricular volumes in children and young adults and to test whether indexing to BSA resulted in an incorrect assessment of ventricular dilation according to sex, body composition, and growth.We retrospectively included 372 subjects aged26 years with either normal hearts or conditions with no impact on ventricular volumes (reference group), and 205 subjects with repaired tetralogy of Fallot (TOF) aged26 years. We generated Z score equations by means of multivariable regression modelling. Right ventricular dilation was assessed with the use of Z scores and compared with indexing to BSA in TOF subjects.Ventricular volume Z scores were independent from age, sex, and anthropometric measurements, although volumes indexed to BSA showed significant residual association with sex and body size. In TOF subjects, indexing overestimated dilation in growing children and underestimated dilation in female compared with male subjects, and in overweight compared with lean subjects.Indexed ventricular volumes measured with cMRI did not completely adjust for body size and resulted in a differential error in the assessment of ventricular dilation according to sex and body size. Our proposed Z score equations solved this problem. Future studies should evaluate if ventricular volumes expressed as Z scores have a better prognostic value than volumes indexed to BSA.

Standardized methods to evaluate atrial properties in single ventricles are lacking. To determine the feasibility of quantifying right atrial volumes and function in hypoplastic left heart using MRI. We studied 15 infants with hypoplastic left heart prior to Glenn surgery (mean age 4.2 months [standard deviation 0.3]) who underwent cardiac MRI with evaluation of atrial volumes and emptying fraction using monoplane two-chamber, monoplane four-chamber, and biplane methods, all of which were compared to the atrial short-axial oblique stack method. We compared atrial end-diastolic volume, end-systolic volume and emptying fraction among these methods. We analyzed reproducibility of the methods using Bland‒Altman plots. Both four-chamber and biplane methods showed high correlations for atrial end-diastolic volume (r = 0.7 and r = 0.8, respectively; P

Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction. It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect (PM-VSD). We herein present 5 adult patients with concomitant DCRV and PM-VSD who varied in their symptomatic presentations and the ways of management.

Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8.4 ± 4.3 years and 5.4 ± 4.1 years post-Fontan) and 8 adults (average age 31.5 ± 8.9 years and 21.1 ± 4 years post-Fontan) using standard serum laboratory investigations assessing hepatic integrity and function, the FibroTest, liver ultrasound and transient elastography (FibroScan). In adult Fontan patients, hemoglobin, C-reactive protein and gamma glutamyl transpeptidase were significantly increased and white blood cell and platelet counts were significantly decreased in comparison to the pediatric cohort. International normalized ratio was mildly elevated in both children and adults. FibroTest results were suggestive of cirrhosis regardless of time post-Fontan. FibroScan measurements were significantly correlated with time post-Fontan but the frequency of ultrasound-detected liver abnormalities were not. No cases of hepatocellular carcinoma were identified. Abnormalities suggestive of FALD occur in both children and adults post-Fontan. Select laboratory tests, and possibly ultrasound and FibroScan in some patients, appear to have the most promise for the non-invasive detection of FALD.

Background Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined. Methods Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated. Results To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults. Conclusions CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.

Cardiovascular magnetic resonance (CMR) imaging is a rapidly developing technology that is becoming increasingly important in the diagnostic assessment of heart disease. Recognizing the need for recommendations to optimize the use of this technique, the Canadian Society for Cardiovascular Magnetic Resonance developed a task force to generate recommendations on the clinical use of parameters acquired by CMR imaging and how they should be reported. This article is the consensus report generated by the task force. The online material of this report provides such parameters for all relevant clinical settings, including pediatric and congenital applications. It considers the current clinical role of CMR, general requirements for CMR imaging, components of CMR studies, quantitative CMR image analysis, and appropriate contents of CMR reports. The recommendations are based on previously published recommendations on analysis and reporting and are the first of their kind. It is hoped that the use of these recommendations to guide daily clinical routine will help institutions offering CMR to adhere to high standards of quality according to the present state of the art.

Magnetic resonance imaging (MRI) provides accurate and valuable information regarding cardiac shunts including their location, size, and flow. We describe the MRI findings of a restrictive atrial septal defect within a complex case of congenital heart disease: univentricular atrioventricular connection of left ventricular type with right-sided discordant atrioventricular connection (single), absent left atrioventricular connection, and ventriculoarterial discordance. Few similar cases are reported in the literature. Magnetic resonance imaging may add valuable information regarding restrictive atrial shunts in univentricular hearts.

Starting as a research method little more than a decade ago, cardiovascular magnetic resonance (CMR) imaging has rapidly evolved to become a powerful diagnostic tool used in routine clinical cardiology. The contrast in CMR images is generated from protons in different chemical environments and, therefore, enables high-resolution imaging and specific tissue characterization in vivo, without the use of potentially harmful ionizing radiation. CMR imaging is used for the assessment of regional and global ventricular function, and to answer questions regarding anatomy. State-of-the-art CMR sequences allow for a wide range of tissue characterization approaches, including the identification and quantification of nonviable, edematous, inflamed, infiltrated or hypoperfused myocardium. These tissue changes are not only used to help identify the etiology of cardiomyopathies, but also allow for a better understanding of tissue pathology in vivo. CMR tissue characterization may also be used to stage a disease process; for example, elevated T2 signal is consistent with edema and helps differentiate acute from chronic myocardial injury, and the extent of myocardial fibrosis as imaged by contrast-enhanced CMR correlates with adverse patient outcome in ischemic and nonischemic cardiomyopathies. The current role of CMR imaging in clinical cardiology is reviewed, including coronary artery disease, congenital heart disease, nonischemic cardiomyopathies and valvular disease.

Cardiac MRI Volumetric Assessment by Short-Axis has Better Reproducibility than the Axial Orientation in Functionally Single Right Ventricle Hearts Prior to Bidirectional Cavopulmonary Anastomosis Background: Cardiovascular Magnetic resonance imaging (CMR) is the standard for assessment of ventricular volumes and function. However, few reports exist regarding methodology for measurements of functionally single right ventricular (RV) volumes in congenital heart disease. This study aims to determine which imaging plane, the short axis oblique (SAO) or the axial orientation (AX), provides greater reproducibility in evaluation of RV volumes and function in patients with hypoplastic left heart syndrome (HLHS) prior to bidirectional cavopulmonary anastomosis (BCPA). Methods: CMR was performed under general anesthesia in 23 patients (5 ± 3.4 months) with single RV prior to BCPA to obtain ventricular volumes from the axial and/or short-axis cine orientations. Postprocessing (cmr42 - Circle Imaging, Calgary, Alberta) was performed by two independent observers to obtain end-diastolic (EDV), end-systolic (ESV) volumes and ejection fraction (EF) in both SAO (n=23) and AX (n=16) planes. Absolute differences (mean ± SD), repeatability values, intraclass correlation coefficients (ICC), coefficient of variation, and Bland Altman plots were used to assess the reproducibility between methods, and interobserver and intraobserver variability. Results: Comparisons of SAO versus AX volumes revealed small absolute differences, with better repeatability for ESV (4.6 ml) compared to EDV (8.5 ml), and high agreement between the 2 methods. Interobserver variability showed high correlations and narrow limits of agreements for SAO and AX measured ESV. However, AX EDV had poorer repeatability (11 ml) and wider limits of agreement than SAO EDV (6.4 ml). Intraobserver testing showed higher correlations for all measurements, but SAO EDV (2.7 ml) showed better repeatability than AX EDV (5.7 ml). Conclusion: CMR assessment of single RV volumes showed better inter and intra-observer reproducibility for the SAO than the AX methods suggesting that the addition of an axial stack does not provide any benefit over the short axis orientation in the assessment of single RV volumes and function.

A 34-year-old man with complete transposition of the great arteries (D-TGA) presented for the first time to our clinic. He previously underwent balloon atrial septostomy (Rashkind procedure) early after birth followed by a Senning procedure at the age of 2 months together with ligation of a patent ductus arteriosus. He has been asymptomatic since his surgery. Transthoracic echocardiogram was technically challenging yet revealed a mildly dilated, hypertrophied systemic right ventricle with mild systolic dysfunction, normal sub-pulmonary left ventricular size and systolic function with no baffle leak or stenosis. No significant valvulopathy was noted (Movie). Cardiovascular magnetic resonance (CMR) and cardiac computed tomography (CCT) showed a moderately hypertrophied systemic right ventricle with normal systolic function (right ventricular ejection fraction 48 %). The sub-pulmonic left ventricle was dilated with normal systolic function. Systemic venous baffles were patent. The pulmonary venous portion of the baffle was patent. The main pulmonary artery and both branches were dilated (Fig. 1). Fig. 1 a CMR short-axis steady-state free precession (SSFP) coronal image showing hypertrabeculated systemic RV with dilated LV. b CMR angiography revealing the left lower PV draining superiorly to a larger common left-side PV ... CMR angiography and CCT uncovered a tortuous varix of the left lower pulmonary vein (PV) draining superiorly to a larger common left-side PV which in turn connected to the superior aspect of the left atrium to the left of the Senning baffle (residual left-to-right shunt). Qp:Qs ratio was estimated at 1.9 by flow data and 2.1 by stroke volume analysis. The right PVs were dilated (Fig. 1). Due to the presence of significant residual left-to-right shunt with a dilated sub-pulmonic ventricle, cardiac catheterisation and potential corrective surgery were recommended. However, the patient declined the procedure. To the best of our knowledge, this is the first case in the literature exploring an unusual anomalous PV connection in a patient with D‑TGA following atrial switch procedure. We speculate that the patient had this anomalous connection since birth but may have been overlooked during surgical correction [1–3].

Background The extent of surgical scarring in Tetralogy of Fallot (TOF) may be a marker of adverse outcomes and provide substrate for ventricular arrhythmia. In this study we evaluate the feasibility of high resolution three dimensional (3D) late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) for volumetric scar quantification in patients with surgically corrected TOF. Methods Fifteen consecutive patients underwent 3D LGE imaging with 3 Tesla CMR using a whole-heart, respiratory-navigated technique. A novel, signal-histogram based segmentation technique was tested for the quantification and modeling of surgical scar. Total scar volume was compared to the gold standard manual expert segmentation. The feasibility of segmented scar fusion to matched coronary CMR data for volumetric display was explored. Results Image quality sufficient for 3D scar segmentation was acquired in fourteen patients. Mean patient age was 32.2 ± 11.9 years (range 21 to 57 years) with mean right ventricle (RV) ejection fraction (EF) of 53.9 ± 9.2% and mean RV end diastolic volume of 117.0 ± 41.5 mL/m2. The mean total scar volume was 11.1 ± 8.2 mL using semi-automated 3D segmentation with excellent correlation to manual expert segmentation (r = 0.99, bias = 0.89 mL, 95% CI -1.66 to 3.44). The mean segmentation time was significantly reduced using the novel semi-automated segmentation technique (10.1 ± 2.6 versus 45.8 ± 12.6 minutes). Excellent intra-observer and good inter-observer reproducibility was observed. Conclusion 3D high resolution LGE imaging with semi-automated scar segmentation is clinically feasible among patients with surgically corrected TOF and shows excellent accuracy and reproducibility. This approach may offer a valuable clinical tool for risk prediction and procedural planning among this growing population.

Atrial shunts are a common finding in both paediatric and adult populations. Recent developments in advanced imaging have widened the options for diagnosis and evaluation of such shunts. This paper reviews the various types of interatrial communications, discusses the features of clinical presentation in adults and children, and provides an overview of the clinical assessment including physical examination, electrocardiography, echocardiography, cardiac catheterisation, computed tomography, and magnetic resonance imaging. Focus will be placed on recent developments in magnetic resonance imaging that may improve the non-invasive evaluation of atrial shunts.

Dilated cardiomyopathy as seen in children is clinically and genetically heterogeneous, with an increasing proportion of cases known to be caused by disorders of single genes. An autosomal recessive syndrome with a high incidence of dilated cardiomyopathy was recently described in the Canadian Dariusleut Hutterite population. It is caused by homozygous mutations in a novel gene, DNAJC19, presumed to play a role in importation of mitochondrial proteins. We discuss the cardiac features of this syndrome, and its relationship to cardiac mitochondrial function.

Giant aneurysm of the left atrial appendage (LAA) is a rare condition typically presenting in adulthood. This case report describes the investigations and surgical management of a giant LAA aneurysm in a neonate, emphasizing the role of echocardiography and cardiovascular magnetic resonance imaging in preoperative evaluation as well as challenges in surgical repair of this defect.

Children with right ventricular outflow tract obstructive (RVOTO) lesions require precise quantification of pulmonary artery (PA) size for proper management of branch PA stenosis. We aimed to determine which cardiovascular magnetic resonance (CMR) sequences and planes correlated best with cardiac catheterization and surgical measurements of branch PA size. Fifty-five children with RVOTO lesions and biventricular circulation underwent CMR prior to; either cardiac catheterization (n = 30) or surgery (n = 25) within a 6 month time frame. CMR sequences included axial black blood, axial, coronal oblique and sagittal oblique cine balanced steady-state free precession (bSSFP), and contrast-enhanced magnetic resonance angiography (MRA) with multiplanar reformatting in axial, coronal oblique, sagittal oblique, and cross-sectional planes. Maximal branch PA and stenosis (if present) diameter were measured. Comparisons of PA size on CMR were made to reference methods: (1) catheterization measurements performed in the anteroposterior plane at maximal expansion, and (2) surgical measurement obtained from a maximal diameter sound which could pass through the lumen. The mean differences (Δ) and intra class correlation (ICC) were used to determine agreement between different modalities. CMR branch PA measurements were compared to the corresponding cardiac catheterization measurements in 30 children (7.6 ± 5.6 years). Reformatted MRA showed better agreement for branch PA measurement (ICC > 0.8) than black blood (ICC 0.4–0.6) and cine sequences (ICC 0.6–0.8). Coronal oblique MRA and maximal cross sectional MRA provided the best correlation of right PA (RPA) size with ICC of 0.9 (Δ −0.1 ± 2.1 mm and Δ 0.5 ± 2.1 mm). Maximal cross sectional MRA and sagittal oblique MRA provided the best correlate of left PA (LPA) size (Δ 0.1 ± 2.4 and Δ −0.7 ± 2.4 mm). For stenoses, the best correlations were from coronal oblique MRA of right pulmonary artery (RPA) (Δ −0.2 ± 0.8 mm, ICC 0.9) and sagittal oblique MRA of left pulmonary artery (LPA) (Δ 0.2 ± 1.1 mm, ICC 0.9). CMR PA measurements were compared to surgical measurements in 25 children (5.4 ± 4.8 years). All MRI sequences demonstrated good agreement (ICC > 0.8) with the best (ICC 0.9) from axial cine bSSFP for both RPA and LPA. Maximal cross sectional and angulated oblique reformatted MRA provide the best correlation to catheterization for measurement of branch PA’s and stenosis diameter. This is likely due to similar angiographic methods based on reformatting techniques that transect the central axis of the arteries. Axial cine bSSFP CMR was the best surgically measured correlate of PA branch size due to this being a measure of stretched diameter. Knowledge of these differences provides more precise PA measurements and may aid catheter or surgical interventions for RVOTO lesions.

We report a patient with chronic Q-fever endocarditis who was treated with Bentall procedure on two occasions due to persistent endocarditis. A chronic pseudoaneurysm of the aortic root was discovered incidentally by cardiac magnetic resonance and computed tomography eight years post-operatively. Due to chronicity of the pseudoaneurysm and great risk of re-intervention, conservative management was recommended.

McArdle's disease (glycogen storage disease type V) is a rare autosomal recessive metabolic myopathy due to myophosphorylase deficiency. It classically manifests by exercise intolerance, leg cramps, muscle pain and occasionally exercise induced myoglobinuria. The onset of exercise intolerance is typically in the second or third decades of life. It has a specific predilection to skeletal muscle involvement, yet cardiac muscle involvement is very rare. This report describes an unusual case of a 33 year-old man with known McArdle's disease who presented with an incidental finding of severe obstructive hypertrophic cardiomyopathy.

Valvular involvement is common in antiphospholipid syndrome (APS) with increased risk of thrombo-embolic events. We report a patient with APS and multiple cerebral infarcts. Echocardiography demonstrated verrucous vegetations of the mitral valve in keeping with marantic endocarditis. The patient underwent successful mitral valve replacement. Post-operative clinical and echocardiographic follow-up showed excellent short term results.

Tumoral calcinosis is uncommon in toddlers, and rare within the subscapular area. Although typically benign, tumoral calcinosis is often incorrectly diagnosed prior to biopsy. We present a case of subscapular tumoral calcinosis in a 16-month old girl and discuss the radiological findings on X-ray, ultrasound, computed tomography and magnetic resonance imaging, including the first description of T1-weighted post contrast imaging, which demonstrate the fibrotic components of tumoral calcinosis.

A 48-year-old woman presented with progressive exercise intolerance and paroxysmal atrial fibrillation. The past history was remarkable for severe Ebstein's anomaly and hypoplastic right ventricle, for which she had undergone placement of a 30 mm Carpentier-Edwards right atrial to right ventricular outflow tract conduit at 20 years of age. She subsequently required conduit revision with a 26 mm pulmonary cryopreserved homograft due to symptomatic conduit obstruction at the age of 24 years. Physical examination …

Paediatric myocarditis remains challenging from the perspectives of diagnosis and management. Multiple aetiologies exist and the majority of cases appear to be related to viral illnesses. Enteroviruses are believed to be the most common cause, although cases related to adenovirus may be more frequent than suspected. The clinical presentation is extremely varied, ranging from asymptomatic to sudden unexpected death. A high index of suspicion is crucial. There is emerging evidence to support investigations such as serum N-terminal B-type natriuretic peptide levels, as well as cardiac magnetic resonance imaging as adjuncts to the clinical diagnosis. In the future, these may reduce the necessity for invasive methods, such as endomyocardial biopsy, which remain the gold standard. Management generally includes supportive care, consisting of cardiac failure medical management, with the potential for mechanical support and cardiac transplantation. Treatments aimed at immunosuppression remain controversial. The paediatric literature is extremely limited with no conclusive evidence to support or refute these strategies. This article summarises the current literature regarding aetiology, clinical presentation, diagnosis, and management of myocarditis in paediatric patients.

A 25-year-old man was evaluated due to progressive exercise intolerance and desaturation in the setting of previously documented Tetralogy of Fallot (TOF). He was diagnosed with TOF shortly after birth but his family declined surgery. Three years earlier, he had undergone left sided modified Blalock-Taussig shunt as a palliative procedure due to progressive shortness of breath and desaturation (O2 saturation between 80-85%). Initially, in the first few months after the procedure, he noticed slight improvement of his symptoms. However, he developed progressive exercise intolerance and desaturation which mandated complete repair. Physical examination was remarkable for desaturation (O2 saturation between 85-90%), clubbing and systolic ejection murmur (grade II-III/VI) over the pulmonic area. Electrocardiogram showed right axis deviation and right ventricular (RV) hypertrophy. Chest X-ray revealed dilated right side of the heart. Transthoracic echocardiogram (TTE) was technically difficult still showed dilated hypertrophied RV with normal systolic function, malalignment ventricular septal defect (VSD) and overriding of the aorta (Supplementary movie 1 and 2). Transesophageal echocardiogram (TEE) was carried out for further delineation of the RV outflow tract (RVOT) which was hypoplastic with a long (~4 cm) segment of narrowing and significant infundibular narrowing (by hypertrophied muscle bundle). Peak gradient across the RVOT was approximately 35-40 mm Hg (Fig. 1, Supplementary movie 3 and 4). Cardiac magnetic resonance (CMR) was technically challenging due to patient's non-compliance with breathing instructions, yet confirmed the findings of TTE/TEE. Additionally, CMR uncovered hypoplastic proximal main pulmonary artery (PA), dilated left PA and mild proximal right PA stenosis with hypoplasia throughout the course (Fig. 2, Supplementary movie 5). The patient underwent successful complete repair with RV to PA conduit, resection of the native RVOT muscle bundles, arterioplasty of the right PA and VSD pericardial patch closure. Surgery was uneventful. Fig. 1 A: Transesophageal echocardiogram (TEE) transgastric view showing dilated right ventricle with significant hypertrophy. B: TEE apical long axis view showing overriding of the ascending aorta with malalignment ventricular septal defect (arrow). C: TEE ... Fig. 2 A: Cardiac magnetic resonance (CMR) sagittal image showing overriding of the ascending aorta with malalignment ventricular septal defect (arrow). B: CMR sagittal image showing hypoplastic right ventricular outflow tract and proximal main pulmonary artery ... An amalgamated approach of echocardiogram and CMR offers a precise detailed assessment of the RV, RVOT, and PA, which is critical for decision making regarding surgical intervention in this unusual case of unrepaired TOF presenting late in adult life.1),2)

A 25-year-old woman with complete transposition of the great arteries presented for routine follow-up one year post Mustard procedure. She previously underwent balloon atrial septostomy (Rashkind procedure) early after birth, but her family declined further surgery. She developed progressive pulmonary hypertension early in life, shortness of breath on moderate exertion, followed by progressive exercise intolerance and desaturation (80-85%) over the preceding two years. Pre-operative pulmonary pressure was estimated at approximately 90 mmHg by cardiac catheterization. Subsequently, she underwent a palliative Mustard procedure. Recovery was uneventful with improvement of her functional status and oxygen saturation between 90-95%. Routine transthoracic echocardiogram (TTE) one year post-procedure revealed a severely dilated, hypertrabeculated systemic right ventricle with mild systolic dysfunction and normal sub-pulmonary left ventricular size and systolic function. Additionally, color Doppler showed turbulence at the junction of the upper and lower limbs of the systemic venous baffles suggestive of significant stenosis without baffle leak (Fig. 1, Supplementary movie 1-3). Cardiovascular magnetic resonance confirmed the TTE findings of significant narrowing of the superior vena cava (SVC) and inferior vena cava (IVC) baffles at the venoatrial junction. The pulmonary venous portion of the baffle was patent. The main pulmonary artery and both branches were severely dilated (Fig. 2, Supplementary movie 4-7). Fig. 1 Transthoracic echocardiogram apical 4-chamber view (A) with zoom mode (B) demonstrating the connections of the systemic venous circulation with significant color turbulence suggestive of baffle stenosis at the veno-atrial junction (arrow). LV: left ventricle, ... Fig. 2 A: Cardiovascular magnetic resonance 4-chamber steady-state free precession (SSFP) image showing the trabeculated dilated systemic RV and patent pulmonary venous baffle (arrow). B: Cardiovascular magnetic resonance coronal SSFP image showing the typical ... Due to the presence of significant narrowing of the SVC/IVC baffles, cardiac catheterization was recommended to assess the gradients across the stenotic areas and to potentially perform balloon angioplasty/stenting. However, the patient declined as she felt noticeable improvement of her symptoms following the Mustard procedure. Herein we present a rare case describing a known complication of Mustard procedures as early as one year post-operatively, in addition to the Mustard procedure itself, which was delayed until 24 years of age. The etiology of baffle stenosis post-Mustard procedure is not clear however, speculations include difficult sizing of the baffles in adult heart, localized scarring of the patch at the suture line or perhaps anatomical distortion. Surprisingly, the patient had an improvement in symptoms after the procedure despite this significant subsequent obstruction of the systemic venous pathways.1-3)

Herein we report a 21 year-old woman with a previously documented patent ductus arteriosus and Eisenmenger physiology. She presented with increasing cyanosis and exercise intolerance which could be explained by a new finding of right to left shunting through an interatrial communication. She was started on Bosentan therapy aiming to reduce the pulmonary pressure with consideration for heart-lung transplantation should any further deterioration occur.

BACKGROUND: Fontan-associated liver disease (FALD) is increasingly recognized as a serious complication post-Fontan resulting from the chronically elevated venous pressure created by this abnormal circulation. This study was designed to: define the prevalence and severity of FALD in our local population of pediatric (age

Background CMR is increasingly used to diagnose myocarditis in adults but its use in pediatric-age pts is not well estab- lished. We sought to describe the clinical presentation, CMR imaging protocols, CMR findings, and outcomes in a multicenter cohort of children with myocarditis. Methods 71 studies (66%) and was abnormal in 49 (69%). First pass contrast perfusion (FPP) imaging was performed in 42 studies (45%) and was abnormal in 4 (10%). T1- weighted imaging for early gadolinium enhancement (EGE) was performed in 35 studies (37%) and was abnor- mal in 19 (51%). Late gadolinium enhancement (LGE) imaging was performed in all studies, and was abnormal in 93 (83%) with the following reported distributions: 89% subepicardial or midwall, 6% patchy, 3% sub-endo- cardial, 1% transmural, and 1% diffuse. The CMR study was interpreted as positive for myocarditis in 96 pts (87%), negative in 11 (10%), and equivocal in 4 (4%), yielding a sensitivity of 86% for an ultimate clinical diag- nosis of myocarditis. There was significant practice varia- tion in the use of T2W, FPP, and EGE imaging among the participating institutions (Figure 1). At a median fol- low-up time from CMR of 6 mo (0.2-217), all patients were alive and 3 had undergone cardiac transplantation, all of whom had CMR studies positive for myocarditis. Conclusions This is the largest study to date describing the CMR findings in children with myocarditis. The CMR techni- ques used, from most to least common, were LGE, T2W, FPP, and EGE. Abnormalities were most often seen with LGE followed by T2W, EGE, and FPP. There was significant practice variation in the CMR protocol between institutions. The information from this study should be useful in planning a prospective study to eval- uate the diagnostic and predictive performance of CMR in children with suspected myocarditis. Funding None.

Background Increased left atrial volume and reduced atrial emptying are predictors of adverse cardiac events in adults. The atrium in single ventricle hearts contributes to ventricular filling to a greater degree than in normal hearts. Standardized methods of determining atrial function in single ventricles are lacking. We aimed to assess the feasibility and reproducibility of quantifying atrial volumes and function in hypoplastic left heart (HLH) prior to the Glenn operation.

Background Accurate evaluation of branch pulmonary artery size in children with single ventricle is important for surgical planning and has prognostic implications after Fontan surgery. Various cardiac MRI sequences are employed to evaluate these sometimes hypoplastic pulmonary arteries, which increases imaging time in anesthetised children. The aim of this study was to evaluate which MRI sequence(s) correlate with angiography measure- ments of pulmonary artery size made at cardiac cathe- terization in children with single ventricle. Methods regression with 95% confidence intervals were used to compare MRI with the "gold" standard catheterization angiography measurements (p

A 28-year-old woman, with known Scimitar syndrome, was evaluated for moderate exercise intolerance. Examination was notable for grade 2/6 systolic murmur over the pulmonary area. The electrocardiogram was unremarkable. Chest X-ray demonstrated dextroposition of the heart with a shadow suggestive of the scimitar vein. Transthoracic echocardiogram was technically challenging with no evidence of pulmonary hypertension or intracardiac shunts. Cardiovascular magnetic resonance showed moderately dilated right …

We retrospectively reviewed the records of patients who underwent redo carotid endarterectomies during a 5-year period. Patients were followed by duplex ultrasound to assess late patency of the carotid artery. Reoperations for recurrent carotid stenosis were performed in 46 of 973 patients who had carotid endarterectomies (5%). Indications for surgery were hemispheric transient ischemic attacks (TIA) in 33 (72%) and asymptomatic greater than 80% stenosis in 13 (28%). Pathologic findings revealed that the cause of recurrence was myointimal hyperplasia in 11 patients (24%), with a mean recurrence interval of 12.8 postoperative months, and atherosclerosis in 35 (76%), with a mean recurrence interval of 84 postoperative months ( P = 0.0002). Redo endarterectomy with patch angioplasty was used for reconstruction in 32 cases (70%), patch angioplasty alone in 11 (24%), and endarterectomy with primary closure in 3. There were 3 perioperative strokes (7%). Late follow-up mean 30.9 months) revealed no strokes and 1 TIA. Of 40 patients, 34 (85%) were alive and stroke free. Although six late deaths occurred, none were stroke related. One patient (2%) had late significant second recurrent carotid stenosis. Redo carotid endarterectomy for symptomatic patients and asymptomatic high-grade stenosis is safe and durable.

A 46-year-old woman presented with progressive fatigue. Her past history was remarkable for partial atrio-ventricular septal defect repair at the age of 9 years. Physical examination was notable for obesity, positional desaturation (PaO2 = 92% standing vs. 88% supine) and Grade 2/6 apical pansystolic murmur. Transthoracic echocardiography showed an intact atrial septum and mild mitral regurgitation. Saline contrast injection from the left arm showed prompt filling of both atria with bubbles consistent with a significant extracardiac right-to-left shunt and suggesting communication at the caval level (see …

BACKGROUND: High spatial resolution contrast-enhanced magnetic resonance angiography (CEMRA) is routinely used to assess vascular morphology. In children, however, faster heart rates and variable arterial-to-venous transit times may produce venous contrast contamination and make CEMRA of the pulmonary artery (PA) and aorta (Ao) difficult using a single dose of gadolinium contrast. A newer technique, known as time-resolved imaging with interleaved stochastic trajectories (TWIST), allows rapid dynamic vascular imaging and may be used to determine optimal contrast arrival time in the great arteries for conventional CEMRA. The aim of this study is to assess TWIST bolus timing for optimizing CEMRA of the great arteries using a single dose of contrast in pediatric patients with congenital heart disease (CHD). METHODS/RESULTS: Patients underwent clinically indicated angiographyonaSiemensAvanto1.5Tsystem.TWISTwasperformed using a 4 mL (2 mmol) test bolus of gadopentetate. TWIST parameterswere:TR,2.3ms;TE,0.8ms;flipangle, 20°; rectangularfieldof view (rFOV), 300 320 mm; matrix, 120 128; 36 partitions; voxel size 2.5 2.5 3.5 mm^3; acceleration factor, 6; measurements, 24; temporal resolution per measurement, 1 second. Test bolus duration, acquisition time, and time to peak signal intensity in the PA and Ao were derived from TWIST datasets and used to perform twosequential conventionalCEMRAacquisitions in thegreatarteries following a single 0.1 mmol/kg contrast bolus. CEMRA parameters were: TR, 3.03 ms; TE, 1.11 ms; flip angle, 25°; rFOV, 332 380 mm;matrix,236 512;120partitions; voxel size0.99 0.99 1.0 mm^3; acceleration factor, 3. TWIST and CEMRA were evaluated for image quality and degree of separation of arterial and venous phases using a 4 point scale. To date, 3 patients (13 1 years; 53 11 kg, heart rate 69 20 / min) have been studied. TWIST yielded separate venous and arterial phases but with a greater degree of vessel-margin blurring. Two sequential CEMRA acquisitions based on TWIST bolus timing resulted in visualization of the great arteries with a distinct PA and Ao phase and without contrast contamination (Figure 1). CONCLUSION: TWIST bolus timing may be used to determine optimal contrast arrival time. This dynamic information allows sequential acquisition of conventional high spatial resolution CEMRA images of the PA and Ao using a single contrast bolus. This technique is valuable in performing angiography in pediatric patients with CHD. 048 THE IMPACT OF FETAL DIAGNOSIS ON DUCT-DEPENDENT CONGENITAL HEART LESIONS

Background Cardiovascular magnetic resonance (CMR) is increasingly used to diagnose myocarditis in adults but its use in children is not well-established. We sought to describe the presentation, CMR protocol and findings, and outcomes in a multicenter cohort of children with myocarditis. Methods Thirteen hospitals retrospectively identified patients meeting the following inclusion criteria: 1) diagnosis of myocarditis by the managing physicians, 2) age