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Objectives The goals of this study were to: 1) evaluate the cardiac magnetic resonance (CMR) characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. Background After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric CMR-based diagnostic criteria. Methods CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. Results Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. Conclusions CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Background Relationships between right ventricular (RV) and left ventricular (LV) shape and function may be useful in determining optimal timing for pulmonary valve replacement in patients with repaired tetralogy of Fallot (rTOF). However, these are multivariate and difficult to quantify. We aimed to quantify variations in biventricular shape associated with pulmonary regurgitant volume (PRV) in rTOF using a biventricular atlas. Methods In this cross-sectional retrospective study, a biventricular shape model was customized to cardiovascular magnetic resonance (CMR) images from 88 rTOF patients (median age 16, inter-quartile range 11.8–24.3 years). Morphometric scores quantifying biventricular shape at end-diastole and end-systole were computed using principal component analysis. Multivariate linear regression was used to quantify biventricular shape associations with PRV, corrected for age, sex, height, and weight. Regional associations were confirmed by univariate correlations with distances and angles computed from the models, as well as global systolic strains computed from changes in arc length from end-diastole to end-systole. Results PRV was significantly associated with 5 biventricular morphometric scores, independent of covariates, and accounted for 12.3% of total shape variation (p Conclusion A biventricular atlas of rTOF patients quantified multivariate relationships between left–right ventricular morphometry and wall motion with pulmonary regurgitation. Regional RV dilation, LV reduction, LV septal-lateral flattening and increased RV strain were all associated with increased pulmonary regurgitant volume. Morphometric scores provide simple metrics linking mechanisms for structural and functional alteration with important clinical indices.

Biventricular statistical atlases are useful dimensionality reduction tools that have demonstrated utility for discovering improved diagnostic and prognostic markers. Patient-specific geometries used to generate these atlases are obtained from in-vivo tomographic images of end diastole (ED) and/or end-systole (ES). However, because these are pressure loaded states, variations in hemodynamic loading may impact downstream shape analysis. In this project, we used a computationally expensive, iterative method to estimate the ventricular geometry in the absence of hemodynamic loading and quantified the shape difference between pressure loaded and unloaded states using a statistical atlas. Next, we assessed whether these atlas-based shape differences could be used as an accurate initial guess for the iterative method, thereby improving the convergence time. In a cohort of 23 patients with chronic thromboembolic pulmonary hypertension (CTEPH), hemodynamic unloading produced atlas-based z-score changes that were highly conserved between patients, with absolute differences between the loaded and unloaded states of 1.0 ± 0.14, 1.2 ± 0.27, −0.1 ± 0.08, and 0.5 ± 0.08 for the first 4 shape modes, respectively. When we used these atlas-based z-score changes as an initial guess, we observed a 72% decrease in required CPU time to solve for patient-specific unloaded geometries.

Modeling the hearts of patients with hypoplastic left heart syndrome has remained an ongoing challenge. Thus, in this work, we developed a HLHS-specific biventricular template to capture the single ventricle morphology in this cohort accurately and robustly. We have demonstrated that using the HLHS-specific template to reconstruct patient-specific models from cardiac magnetic resonance images result in significantly higher accuracy comparing to that of using the generic template. Additionally, element distortion in meshes created using the HLHS-specific template is compatible with meshes currently used for finite element analysis. Accurate 3D patient-specific models will serve as powerful tools for subsequent statistical shape and biomechanics analyses to provide insights into remodeling of ventricular shape and function and their potential association with clinical outcomes.

Background: Neonatal myocardial infarction is rare and is associated with a high mortality of 40% to 50%. We report our experience with neonatal myocardial infarction, including presentation, management, outcomes, and our current patient management algorithm. Methods: We reviewed all infants admitted with a diagnosis of coronary artery thrombosis, coronary ischemia, or myocardial infarction between January 2015 and May 2021. Results: We identified 21 patients (median age, 1 [interquartile range (IQR), 0.25–9.00] day; weight, 3.2 [IQR, 2.9–3.7] kg). Presentation included respiratory distress (16), shock (3), and murmur (2). Regional wall motion abnormalities by echocardiogram were a key criterion for diagnosis and were present in all 21 with varying degrees of depressed left ventricular function (severe [8], moderate [6], mild [2], and low normal [5]). Ejection fraction ranged from 20% to 54% (median, 43% [IQR, 34%–51%]). Mitral regurgitation was present in 19 (90%), left atrial dilation in 15 (71%), and pulmonary hypertension in 18 (86%). ECG was abnormal in 19 (90%). Median troponin I was 0.18 (IQR, 0.12–0.56) ng/mL. Median BNP (B-type natriuretic peptide) was 2100 (IQR, 924–2325) pg/mL. Seventeen had documented coronary thrombosis by cardiac catheterization. Seventeen (81%) were treated with intracoronary tPA (tissue-type plasminogen activator) followed by systemic heparin, AT (antithrombin), and intravenous nitroglycerin, and 4 (19%) were treated with systemic heparin, AT, and intravenous nitroglycerin alone. Nineteen of 21 recovered. One died (also had infradiaphragmatic total anomalous pulmonary venous return). One patient required a ventricular assist device and later underwent heart transplant; this patient was diagnosed late at 5 weeks of age and did not respond to tPA. Nineteen of 21 (90%) regained normal left ventricular function (ejection fraction, 60%–74%; mean, 65% [IQR, 61%–67%]) at latest follow-up (median, 6.8 [IQR, 3.58–14.72] months). Two of 21 (10%) had residual trivial mitral regurgitation. After analysis of these results, we present our current algorithm, which developed and matured over time, to manage neonatal myocardial infarction. Conclusions: We experienced a lower mortality rate for infants with neonatal infarction than that reported in the literature. We propose a post hoc algorithm that may lead to improvement in patient outcomes following coronary artery thrombus.

Background Adverse cardiac remodeling is an important precursor to anthracycline-related cardiac dysfunction, however conventional remodeling indices are limited. We sought to examine the utility of statistical atlas-derived measures of ventricular shape to improve the identification of adverse anthracycline-related remodeling in childhood cancer survivors. Methods We analyzed cardiac magnetic resonance imaging from a cross-sectional cohort of 20 childhood cancer survivors who were treated with low (2 [N = 10]) or high (≥250 mg/m2 [N = 10]) dose anthracyclines, matched 1:1 by sex and age between dose groups. We reconstructed 3D computational models of left ventricular end-diastolic shape for each subject and assessed the ability of conventional remodeling indices (volume, mass, and mass to volume ratio) vs. shape modes derived from a statistical shape atlas of an asymptomatic reference population to stratify anthracycline-related remodeling. We compared conventional parameters and five atlas-based shape modes: 1) between survivors and the reference population (N = 1991) using multivariable linear regression, and 2) within survivors by anthracycline dose (low versus high) using two-sided T-tests, multivariable logistic regression, and receiver operating characteristic curves. Results Compared with the reference population, survivors had differences in conventional measures (lower volume and mass) and shape modes (corresponding to lower overall size and lower sphericity; all p p = 0.039). Collectively, atlas-based shape modes in conjunction with conventional measures discriminated survivors who received low vs. high anthracycline dosage (area under the curve [AUC] 0.930, 95% confidence interval 0.816, 1.00) significantly better than conventional measures alone (AUC 0.710, 95% confidence interval 0.473, 0.947; AUC comparison p = 0.0498). Conclusions Compared with a reference population, heart size is smaller in anthracycline-exposed childhood cancer survivors. Atlas-based measures of left ventricular shape may improve the detection of anthracycline dose-related remodeling differences.

Graphical abstract, Highlights • Double aortic arch (DAA) typically occurs in isolation. • DAA with bilateral coarctation (COA) is very rare with three prior documented cases. • In addition to DAA with bilateral COA our case also has complex intracardiac disease. • Echocardiography served as the primary diagnostic tool with advanced imaging as a useful adjunct.

Single ventricle physiology and palliation via the Fontan operation lead to a series of cardiovascular changes. In addition, organs such as the kidneys and liver have been shown to experience insults and subsequent injury. This has led to routine surveillance of patients. We present findings from a small cohort of patients that was deeply phenotyped to illustrate the need for comprehensive evaluation. A cohort of four Fontan patients with fairly high cardiovascular function was recruited 5–10 years post-Fontan. Patients underwent a rigorous clinical work-up after which a research MRI scan was performed during which (I) data were obtained during exercise to evaluate changes in stroke volume during supine exercise and (II) magnetic resonance angiograms with phase-contrast images were obtained for computational modeling of flows through the Fontan circulation at rest. Clinical measures were consistent with a fairly homogeneous high function cohort (peak oxygen consumption >20 mL/kg/min, robust response to exercise, peak ventilatory efficiency below levels associated with heart failure, MR-derived ejection fraction >50%). Liver evaluation did not reveal clear signs of cirrhosis or extensive fibrosis. However, we observed considerable variability (27–162%) in the increase in stroke index with exercise [100%±64% increase, 53.9±17.4 mL/beat m(2) (rest), 101.1±20.7 mL/beat m(2), (exercise)]. Computational flow modeling at rest in two patients also showed marked differences in flow distribution and shear stress. We report marked differences in both changes in stroke index during an exercise MRI protocol as well as computational flow patterns at rest suggesting different compensation strategies may be associated with high functioning Fontan patients. The observed heterogeneity illustrates the need for deep phenotyping to capture patient-specific adaptive mechanisms.

Introduction: Current indications for pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) rely on global ventricular measurements but are inconsistently applied, lead to mixed outcomes, and are widely debated. Hypothesis: We aimed to test the hypothesis that specific regional markers of biventricular shape can discriminate differences in ventricular remodeling between rTOF patients that were and were not designated for follow-up PVR better than standard clinical indices. Methods: In this cross-sectional retrospective study, biventricular shape models were fit to CMR images from 84 rTOF patients. A statistical atlas of end-diastolic shape was constructed using principal component analysis. Multivariate regression was used to quantify shape marker and clinical index associations with subsequent intervention status (PVR, n=48 vs. No-PVR, n=36) while accounting for confounders. Clustering analysis was then used to test the ability of statistically significant shape markers and clinical indices to discriminate PVR status as evaluated by Matthews correlation coefficient (MCC). Geometric strain analysis was also conducted to assess shape marker associations with systolic function. Results: PVR status correlated with shape markers associated with RV apical dilation and LV dilation (p Conclusions: Biventricular shape markers discriminated PVR status better than standard and current use clinical indices for PVR. These specific, regional features of cardiac morphology can provide mechanistic insight into adaptive vs. maladaptive types of ventricular remodeling that provide the foundation for clinical decision-making but may be overlooked when relying solely on global ventricular measurements.

Patients with repaired tetralogy of Fallot (rTOF) are at risk of long-term left ventricular (LV) dysfunction associated with poor outcomes. In this study, we examined the association of LV end-diastolic (ED) shape with components of systolic wall motion (SWM) that contribute to global systolic dysfunction in an rTOF patient cohort. Features of LV shape associated with conicity and septal wall curvature correlated with components of SWM. The effect of ED shape perturbations on SWM were examined in a finite element analysis of systolic ventricular mechanics. Variations in the combination of ED shape and myocardial contractility were able to match predicted measures of SWM. From these results, we hypothesize that greater LV conicity and a flatter septal wall are markers of reduced global myocardial contractility and should be examined further for clinical prognostic utility to improve patient outcomes.

Increased availability and access to medical image data has enabled more quantitative approaches to clinical diagnosis, prognosis, and treatment planning for congenital heart disease. Here we present an overview of long-term clinical management of tetralogy of Fallot (TOF) and its intersection with novel computational and data science approaches to discovering biomarkers of functional and prognostic importance. Efforts in translational medicine that seek to address the clinical challenges associated with cardiovascular diseases using personalized and precision-based approaches are then discussed. The considerations and challenges of translational cardiovascular medicine are reviewed, and examples of digital platforms with collaborative, cloud-based, and scalable design are provided.

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.

After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria.The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information.CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis.Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis.CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Background/purpose Patients with a functional single ventricle undergo multiple, palliative open-heart surgeries. This includes a superior cavopulmonary anastomosis or bidirectional Glenn shunt. A less-invasive transcatheter approach may reduce morbidity. Methods/materials We analyzed pre-Glenn X-ray contrast angiography (XA), cardiac computed tomography (CT), and cardiac magnetic resonance (CMR) studies. Results Over an eleven-year period (1/2007 – 6/2017), 139 Glenn surgeries were performed at our institution. The typical age range at surgery was 59 – 371 days (median = 163; IQR = 138 – 203). Eight-nine XA, ten CT, and ten CMR studies obtained from these patients were analyzed. Cephalad SVC measurements (millimeters) were 7.3 ± 1.7 (XA), 7.7 ± 1.6 (CT) and 6.9 ± 1.8 (CMR). RPA measurements were 7.3 ± 1.9 (XA), 7.4 ± 1.6 (CT) and 6.6 ± 1.9 (CMR). Potential device lengths were 10.9 ± 6 – 17.4 ± 6.4 (XA), 10.1 ± 2.1 – 17.7 ± 2.4 (CT) and 17.3 ± 4. - 23.7 ± 5.5 (CMR). SVC-RPA angle (degrees) was 132.9 ± 13.2 (CT) and 140 ± 10.2 (MRI). Image quality of all CT (100%), almost all XA (SVC 100%, RPA 99%), and most MRI (SVC 80%, RPA 90%) were deemed sufficient. Parametric modeling virtual fit device with 10 mm diameter and 20 – 25 mm length was ideal. Conclusions Ideal transcatheter cavopulmonary shunt device for the typical patient would be 10 mm in diameter and 20–25 mm in length.

Additional file 1: Supplemental Table 1. Individual Survivor Characteristics. Supplemental Table 2. Reproducibility and Validity of Left Ventricular Shape Models. Supplemental Table 3. Reference Population Characteristics by Age Group*. Supplemental Table 4. Comparison of Left Ventricular Size and Shape Between the Survivor Cohort and Younger Individuals in the Reference Population.*

Background In the care of patients with congenital heart disease, percutaneous interventional treatments have supplanted many surgical approaches for simple lesions, such as atrial septal defect. By contrast, complex congenital heart defects continue to require open-heart surgery. In single-ventricle patients, a staged approach is employed, which requires multiple open-heart surgeries and significant attendant morbidity and mortality. A nonsurgical transcatheter alternative would be attractive. Objectives The authors sought to show the feasibility of catheter-only, closed-chest, large-vessel anastomosis (superior vena cava and pulmonary artery [PA] or bidirectional Glenn operation equivalent) in a patient. Methods In preclinical testing over a decade, the authors developed the techniques and technology needed for nonsurgical crossing from a donor (superior vena cava) to a recipient (PA) vessel and endovascular stent-based anastomosis of those blood vessels. The authors undertook this transcatheter approach for an adult with untreated congenital heart disease with severe cyanosis and significant surgical risk. They rehearsed the procedure step by step using contrast-enhanced cardiac computed tomography and a patient-specific 3-dimensional printed heart model. Results The authors describe a first-in-human, fully percutaneous superior cavopulmonary anastomosis (bidirectional Glenn operation equivalent). The patient, a 35-year-old woman, was homebound due to dyspnea and worsening cyanosis. She was diagnosed with functional single ventricle and very limited pulmonary blood flow. The heart team believed surgical palliation conferred high operative risk due to the patient’s complete condition. With the percutaneous procedure, the patient recovered uneventfully and remained improved clinically after 6 months. Conclusions This procedure may provide a viable alternative to one of the foundational open-heart surgeries currently performed to treat single-ventricle congenital heart disease.

While early and intermediate results of Fontan palliation have greatly improved the survival and quality of life of patients with single ventricle physiology, late complications remain concerning, and leave room for further improvement. In addition to recent advances in surgical techniques and post-operative care, advances in Medical Imaging and related technologies can further improve pre-surgical planning and post-operative management of these complex patients. Novel technologies including advanced MRI, computer aided design, three-dimensional printing, Virtual Surgery and Computational Fluid Dynamics may provide better patient-specific surgical plans, and continue to improve long-term outcomes.

Congenital heart disease is associated with abnormal ventricular shape that can affect wall mechanics and may be predictive of long-term adverse outcomes. Atlas-based parametric shape analysis was used to analyze ventricular geometries of eight adolescent or adult single-ventricle CHD patients with tricuspid atresia and Fontans. These patients were compared with an “atlas” of non-congenital asymptomatic volunteers, resulting in a set of Z-scores which quantify deviations from the control population distribution on a patient-by-patient basis. We examined the potential of these scores to: (1) quantify abnormalities of ventricular geometry in single ventricle physiologies relative to the normal population; (2) comprehensively quantify wall motion in CHD patients; and (3) identify possible relationships between ventricular shape and wall motion that may reflect underlying functional defects or remodeling in CHD patients. CHD ventricular geometries at end-diastole and end-systole were individually compared with statistical shape properties of an asymptomatic population from the Cardiac Atlas Project. Shape analysis-derived model properties, and myocardial wall motions between end-diastole and end-systole, were compared with physician observations of clinical functional parameters. Relationships between altered shape and altered function were evaluated via correlations between atlas-based shape and wall motion scores. Atlas-based shape analysis identified a diverse set of specific quantifiable abnormalities in ventricular geometry or myocardial wall motion in all subjects. Moreover, this initial cohort displayed significant relationships between specific shape abnormalities such as increased ventricular sphericity and functional defects in myocardial deformation, such as decreased long-axis wall motion. These findings suggest that atlas-based ventricular shape analysis may be a useful new tool in the management of patients with CHD who are at risk of impaired ventricular wall mechanics and chamber remodeling.

Key Clinical Message Left pulmonary artery slings and vascular rings are rare congenital anomalies definable by fetal echocardiography. Left pulmonary artery slings are associated with high respiratory morbidity and mortality. Prenatal diagnosis of a left pulmonary artery sling should prompt delivery planning for postnatal management at a pediatric tertiary care center.

Noninvasive measurement of cardiac output (CO) and particularly stroke volume (SV) remain difficult but potentially valuable. These variables can be particularly challenging to measure in children with congenital heart disease (CHD). Impedance cardiography (IC) is a technique shown to be accurate in measuring SV in adults and in children with structurally normal hearts. The ease of use and rapidity of SV measurement using IC makes it potentially attractive for young patients with CHD. Advances in IC technology have led to more sophisticated signal-morphology IC (SMIC) devices that may further improve accuracy. We tested the accuracy of SMIC to measure SV in 21 subjects with CHD by comparing measurements with those from cardiac magnetic resonance (CMR) imaging. There was good agreement between SMIC and CMR in measurement of SV: mean difference = 1.7 ml (p = 0.47); r = 0.89. The agreement and correlation persisted when controlling for the differences in blood pressure and heart rate during the two testing methods. We conclude that SMIC is accurate at measuring SV and thus CO when compared to CMR in a variety of forms of CHD.

Neonatal myocardial infarction (MI), in the absence of congenital heart disease or cardiac surgery involving the coronaries, is a rare condition with associated high mortality. A cluster of neonatal myocardial infarction cases was observed, leading to an investigation of causes and contributors. We performed a single-center review of neonates >37 weeks between 2011 and 2017 to identify neonates with myocardial infarction. Neonates with prior cardiac surgery, congenital anomalies of the coronaries, or sepsis were excluded. Diagnosis of MI was based on ECG changes, elevated troponin, decreased function or regional wall abnormality, and abnormal coronary angiography. There were 4,583 admissions to the NICU without interval changes in referrals or the incidence of neonatal asphyxia. There were six cases of neonatal myocardial infarction. Of 2,155 admissions in the first three years, there were no cases. The incidence in the next three years was 1.3/1,000, 2.7/1,000, and 3.3/1,000. There was a significant difference (0.03, Fishers exact) between the first and second halves of the study period. All cases underwent catheterization, revealing coronary artery thrombosis requiring thrombolytic therapy. Additionally, obstetric providers were surveyed to determine changes in umbilical cord handling practices. Of 48 obstetric providers, 57% changed their practice of cord clamping since 2014: 13% reported cord milking then clamping; 47% delayed cord clamping for a few minutes; 28% delayed cord clamping until the cord stopped pulsating; and 13% immediate clamping. Only 40% reported routine documentation of cord clamping. In conclusion, there was an increased incidence of neonatal myocardial infarction due to coronary thrombosis. Early diagnosis and initiation of therapy of neonates with myocardial infarction can improve outcomes. A larger study cohort could lead to better understanding of risk factors and outcomes of myocardial infraction in neonates.

In 2007 we began a hybrid program for hypoplastic left heart syndrome (HLHS) variants to potentially improve outcome in high-risk patients. During implementation we offered both hybrid and Norwood approaches to all risk categories. The purpose of this study was to perform a comparative analysis of intermediate survival.Newborns were evaluated jointly for high-risk characteristics, including birth weight less than 2.5 kg, prematurity (especially35 weeks), central nervous system abnormalities, multiorgan failure, intact or severely restrictive atrial septum, severe ventricular dysfunction, and severe atrioventricular valve regurgitation. We prefer Norwood for standard risk and hybrid for high risk, but all groups crossed over into all treatment pathways resulting in the following 5 treatment groups: standard risk Norwood; high-risk Norwood; standard risk hybrid ductal stent (HDS); high-risk hybrid DS; and high-risk hybrid prostaglandin E1 (HPGE). We reviewed all consecutive patients from 2007 to 2012, obtained follow-up, and analyzed the results.Sixty-eight newborns presented (median 2.96 kg, 8 days); 29 (43%) were high and 39 (57%) were standard risk. There were 14 stage I hospital deaths strongly associated with risk: 3 of 39 standard (7.7%) and 11 of 29 high (38%, p = 0.002). Stage I discharge mortality was highest for high-risk Norwood and high-risk HPGE groups (p0.001). Actuarial survival up to 5 years demonstrated superior survival for Norwood versus hybrid (78.1% vs 56.4%, p = 0.0182). With risk stratification there was suboptimal survival for all 3 high-risk groups (p = 0.003); HDS fared better than HPGE but had higher birth weight (p0.001).While a risk-stratified approach for HLHS variant patients with selective use of hybrid palliation resulted in acceptable stage I mortality, the longer term mortality for high-risk patients remains higher than for standard risk regardless of treatment modality. Intrinsic patient risk factors (rather than treatment modality) likely determine long-term outcome in experienced centers. Our current high-risk approach has evolved to HPGE application with Norwood conversion whenever deemed medically possible.

Approximately 1% of all babies are born with some form of congenital heart defect. Many serious forms of CHD can now be surgically corrected after birth, which has lead to improved survival into adulthood. However, many patients require serial monitoring to evaluate progression of heart failure and determine timing of interventions. Accurate multidimensional quantification of regional heart shape and function are required for characterizing these patients. A computational atlas of single ventricle and biventricular heart shape and function enables quantification of remodeling in terms of z-scores in relation to specific reference populations. Progression of disease can then be monitored effectively by longitudinal evaluation of z-scores. A biomechanical analysis of cardiac function in relation to population variation enables investigation of the underlying mechanisms for developing pathology. Here, we summarize recent progress in this field, with examples in single ventricle and biventricular congenital pathologies.

Congenital heart disease is the most common birth defect, with an incidence of 75 in every 1000 births. As a result of improved interventions, 90% of people with congenital heart disease now survive to adulthood. They must undergo regular imaging to assess their biventricular (left and right ventricular) function. Analysis of the images is problematic due to the large variety of shapes and complex geometry. In this paper we extend a biventricular modeling method to improve the analysis of MR images from congenital heart disease patients. We used a subdivision surface method to create three customizable exemplars, representing common manifestations of anatomy, and incorporated these as priors into an interactive biventricular customization procedure. The CHD-specific priors were tested on 60 cases representing a variety of congenital heart diseases for which the gold standard manual contours were available. The introduction of multiple priors showed a significant decrease in analysis time while maintaining good correlation between the two methods (R2 >.82).

Background: Secundum atrial septal defect (ASD) closure devices were granted approval based on industry-sponsored, prospective, nonrandomized, single device studies, demonstrating acceptable efficacy and safety in selected patients. We sought to report community practice and outcomes. Methods and Results: Procedure specific data was collected on cases considered for ASD closure in the congenital cardiac catheterization project on outcomes (C3PO) between February 1, 2007 and June 31, 2010. Eight centers contributed data during this time period. All adverse events (AE) were independently reviewed and classified by a five level severity scale. In 40 months (2/07–6/10), 653 of 688 ASDs were occluded with a single device using an AMPLATZER® Septal Occluder (ASO) in 566 (87%), GORE® HELEX® Septal Occluder (HSO) in 33 (5%), and a CardioSEAL® or STARFlex™ device (CSD) in 54 (8%). Most patients had an isolated ASD (93%). 85% were >2 years of age. The ASD median diameter was 12 mm [8,16] for ASO, with smaller diameters in HSO 8 mm [7,10] and CSD 8 mm [5,10] (P

The patient was a male infant with L-transposition of great arteries (L-TGA), Ebstein's anomaly of the tricuspid valve, subvalvar aortic stenosis, ventricular septal defect (VSD), hypoplastic right ventricle, arch hypoplasia, and congenital complete heart block. He underwent a Norwood procedure, aortic arch repair, permanent pacemaker implantation, and a 3.5-mm aortopulmonary shunt at 4 days of age. At the time of his surgery, left ventricular function was in the normal range (ejection fraction [EF] = 67%). However by 3 months of age, he was noted to have developed moderate-severe biventricular dysfunction (left ventricular ejection fraction [LVEF] = 34%). Atresia of the coronary sinus with a small left superior venacava (LSVC) and a bridging vein was discovered during cardiac catheterization at this time. The coronary sinus mean pressure was 17 mm Hg, and the common atrial mean pressure was 6 mmHg. We opened the atretic coronary sinus ostium using radiofrequency ablation and stent placement. There was dramatic improvement in ventricular function observed over a 2-month period. Follow-up cardiac catheterization 5 months later revealed the stent in the coronary sinus to be widely patent with no intimal buildup, and the ventricular function was normal (LVEF = 58%). The patient had a bidirectional Glenn procedure with an uncomplicated postoperative course and is currently awaiting Fontan completion.

This review discusses the current safety issues related to U.S. Food and Drug Administration approved atrial septal defect devices and proposes a potential avenue to gather additional safety data including factors, which may be involved in device erosion.

Item does not contain fulltext PURPOSE: To evaluate the feasibility and accuracy of magnetic resonance (MR) coronary angiography for the detection of coronary artery anomalies in infants and children by using surgical findings as a reference. MATERIALS AND METHODS: The data analysis was approved by the institutional review board. One hundred children with congenital heart disease underwent MR coronary angiography while under general anesthesia (mean age +/- standard deviation, 3.9 years +/- 3; age range, 0.2-11 years). A navigator-gated, T2-prepared, three-dimensional steady-state free precession whole-heart protocol (isotropic voxel size, 1.0-1.3 mm(3); mean imaging time, 4.6 minutes +/- 1.2; mean navigator efficiency, 70%; 3-mm gating window) was used after injection of gadopentetate dimeglumine. The cardiac rest period (end systole or middiastole) and acquisition window were prospectively assessed for each patient. Coronary artery image quality (score of 0 [nondiagnostic] to 4 [excellent]), vessel sharpness, and coronary artery anomalies were assessed by two observers. Surgery was performed in 58 patients, and those findings were used to define accuracy. Variables were assessed between age groups by using either analysis of variance or Kruskal-Wallis tests. RESULTS: Diagnostic image quality (score, >/=1 for all coronary artery segments) was obtained in 46 of the 58 patients (79%) who underwent surgery. The origin and course of the coronary artery anatomy depicted with MR imaging was confirmed at surgery in all 46 patients-including the four (9%) with substantial coronary artery anomalies. Diagnostic-quality images were obtained in 84 of the 100 patients. The rate of success improved significantly when patients were older than 4 months (88% for patients >4 months vs 17% for patients

Purpose The aim of this article was to assess the role of MRI in the diagnosis and management of a variety of complex aortic arch anomalies. Materials and methods Imaging was performed on a 1.5T Philips Gyroscan Intera. We retrospectively reviewed all cardiac MR scans performed from November 2003 to February 2007 at our institute to identify aortic arch anomalies. Magnetic resonance imaging included three-dimensional (3D) sagittal volume images, cine images, short axis ventricular volumes, phase contrast flow of great vessels, and 3D gadolinium magnetic resonance angiogram. Results Three major categories of anomalies were identified in 47 patients: double aortic arch (n = 9), right-sided aortic arch with mirror image branching (n = 28), and aberrant right subclavian artery (n = 10). In the double aortic arch group, six patients were symptomatic, and four of them underwent a surgical division. Magnetic resonance imaging was used to plan the surgical management. In the other two groups, no patient presented with symptoms of airway or esophageal compression, and the arch abnormalities were noticed during investigation for other associated cardiac disease. However, we noticed a strong correlation with well-defined subgroups of congenital heart disease. Right-sided aortic arch was seen in patients with pulmonary atresia with ventricular septal defect (46.4%), tetralogy of Fallot (32.1%), and double outlet right ventricle with right atrial isomerism (14.2%). Seventy percent of the patients with aberrant right subclavian artery had aortic coarctation, and another case presented a complete aortic arch interruption. Conclusion Magnetic resonance imaging is an important diagnostic tool in identifying anomalies of the aortic arch and its branches and can be considered the imaging technique of choice when planning surgical management, especially when there are associated cardiac anomalies.

Survival rates for infants with congenital heart disease (CHD) are improving, resulting in a growing population of adults with CHD. However, the analysis of left and right ventricular function is very time-consuming owing to the variety of congenital morphologies. Efficient customization of patient geometry and function depends on high quality shape templates specifically designed for the application. In this paper, we combine a method for creating finite element shape templates with an interactive template customization to patient MRI examinations. This enables different templates to be chosen depending on patient morphology. To demonstrate this pipeline, a new biventricular template with 162 elements was created and tested in place of an existing 82-element template. The method was able to provide fast interactive biventricular analysis with 0.31 sec per edit response time. The new template was customized to 13 CHD patients with similar biventricular topology, showing improved performance over the previous template and good agreement with clinical indices.

PURPOSE The lack of magnetic resonance (MR) safe catheters and guide wires remains an important obstacle to widespread clinical use of MR-guided endovascular procedures. The authors looked at the feasibility of using multiple tuned fiducial markers (TFM) and novel imaging sequences to track catheters reliably under MR and to evaluate the safety of such markers in terms of heating. MATERIALS AND METHODS The visualization and tracking of a catheter with six quadrature tuned fiducial coils was carried out in a special designed in-vitro setup within a 1.5-T MR imager simulating an MR-guided endovascular intervention. The fiducial markers were also tested for heating. RESULTS The excellent signal contrast between the fiducial and the background when using novel interleaved real time and interactive sequences allowed for rapid and reliable identification of the fiducial markers and therefore the catheter. No significant heating of the marker was noted. CONCLUSIONS The authors have shown that catheters with multiple tuned fiducial markers are superior to passive catheter designs in terms of visualization and do not carry the risk of heating that is commonly associated with active catheters.

The hybrid magnetic resonance (MR)/X-ray suite (XMR) is a recently introduced imaging solution that provides new possibilities for guidance of cardiovascular catheterization procedures. We have previously described and validated a technique based on optical tracking to register MR and X-ray images obtained from the sliding table XMR configuration. The aim of our recent work was to extend our technique by providing an improved calibration stage, real-time guidance during cardiovascular catheterization procedures, and further off-line analysis for mapping cardiac electrical data to patient anatomy. Specially designed optical trackers and a dedicated calibration object have resulted in a single calibration step that can be efficiently checked and updated before each procedure. An X-ray distortion model has been implemented that allows for distortion correction for arbitrary c-arm orientations. During procedures, the guidance system provides a real-time combined MR/X-ray image display consisting of live X-ray images with registered recently acquired MR derived anatomy. It is also possible to reconstruct the location of catheters seen during X-ray imaging in the MR derived patient anatomy. We have applied our registration technique to 13 cardiovascular catheterization procedures. Our system has been used for the real-time guidance of ten radiofrequency ablations and one aortic stent implantation. We demonstrate the real-time guidance using two exemplar cases. In a further two cases we show how off-line analysis of registered image data, acquired during electrophysiology study procedures, has been used to map cardiac electrical measurements to patient anatomy for two different types of mapping catheters. The cardiologists that have used the guidance system suggest that real-time XMR guidance could have substantial value in difficult interventional and electrophysiological procedures, potentially reducing procedure time and delivered radiation dose. Also, the ability to map measured electrical data to patient specific anatomy provides improved visualization and a path to investigation of cardiac electromechanical models.

Background— Right ventricular outflow tract (RVOT) reconstruction with valved conduits in infancy and childhood leads to reintervention for pulmonary regurgitation and stenosis in later life. Methods and Results— Patients with pulmonary regurgitation with or without stenosis after repair of congenital heart disease had percutaneous pulmonary valve implantation (PPVI). Mortality, hemodynamic improvement, freedom from explantation, and subjective and objective changes in exercise tolerance were end points. PPVI was performed successfully in 58 patients, 32 male, with a median age of 16 years and median weight of 56 kg. The majority had a variant of tetralogy of Fallot (n=36), or transposition of the great arteries, ventricular septal defect with pulmonary stenosis (n=8). The right ventricular (RV) pressure (64.4±17.2 to 50.4±14 mm Hg, P P P P −1 · m −2 , P −1 · m −2 , P =0.005) and effective RV stroke volume (37±7 versus 42±9 mL · beat −1 · m −2 , P =0.006) in 28 patients (age 19±8 years). A further 16 subjects, on metabolic exercise testing, showed significant improvement in V̇ o 2 max (26±7 versus 29±6 mL · kg −1 · min −1 , P Conclusions— PPVI is feasible at low risk, with quantifiable improvement in MRI-defined ventricular parameters and pulmonary regurgitation, and results in subjective and objective improvement in exercise capacity.