Design and implementation of multicenter pediatric and congenital studies with cardiovascular magnetic resonance: Big data in smaller bodies.

Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.

Right ventricle-dependent coronary circulation diagnosed by non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

Normative Echocardiographic Values for Right and Left Ventricular Function in Extremely Premature Neonates.

OBJECTIVE: To identify the measures of biventricular function and surrogates of pulmonary vascular resistance that can be expected in relatively stable extremely premature neonates, and evaluate maturational changes in myocardial performance in this patient population. STUDY DESIGN: This was a prospective observational study. Clinically stable extremely preterm newborns were divided into 3 cohorts based on gestational age: cohort 1 (240/7-256/7 weeks), cohort 2 (260/7-276/7 weeks), and cohort 3 (280/7-296/7 weeks). Serial echocardiograms were obtained on day of life 3-5, 7-10, and just prior to discharge. RESULTS: In total, 46 subjects met the criteria aimed at capturing only the most clinically healthy and stable newborns less than 296/7 weeks of gestational age. Myocardial performance was reliably assessed by echocardiography with high inter-reader correlation. Normative values were identified for right ventricular function, left ventricular function, and surrogates of pulmonary vascular resistance. CONCLUSIONS: Biventricular systolic performance is significantly different in the clinically stable extremely premature neonate when compared with healthy full-term newborns. All participants had evidence of poor biventricular compliance at birth which improved with maturation. Extreme prematurity does not appear to adversely affect myocardial functional maturation at the time of term corrected age and/or discharge.

Right Ventricular Function and T1-Mapping in Boys With Duchenne Muscular Dystrophy.

BACKGROUND: Clinical management of boys with Duchenne muscular dystrophy (DMD) relies on in-depth understanding of cardiac involvement, but right ventricular (RV) structural and functional remodeling remains understudied. PURPOSE: To evaluate several analysis methods and identify the most reliable one to measure RV pre- and postcontrast T1 (RV-T1) and to characterize myocardial remodeling in the RV of boys with DMD. STUDY TYPE: Prospective. POPULATION: Boys with DMD (N = 27) and age-/sex-matched healthy controls (N = 17) from two sites. FIELD STRENGTH/SEQUENCE: 3.0 T using balanced steady state free precession, motion-corrected phase sensitive inversion recovery and modified Look-Locker inversion recovery sequences. ASSESSMENT: Biventricular mass (Mi), end-diastolic volume (EDVi) and ejection fraction (EF) assessment, tricuspid annular excursion (TAE), late gadolinium enhancement (LGE), pre- and postcontrast myocardial T1 maps. The RV-T1 reliability was assessed by three observers in four different RV regions of interest (ROI) using intraclass correlation (ICC). STATISTICAL TESTS: The Wilcoxon rank sum test was used to compare RV-T1 differences between DMD boys with negative LGE(-) or positive LGE(+) and healthy controls. Additionally, correlation of precontrast RV-T1 with functional measures was performed. A P-value <0.05 was considered statistically significant. RESULTS: A 1-pixel thick RV circumferential ROI proved most reliable (ICC > 0.91) for assessing RV-T1. Precontrast RV-T1 was significantly higher in boys with DMD compared to controls. Both LGE(-) and LGE(+) boys had significantly elevated precontrast RV-T1 compared to controls (1543 [1489-1597] msec and 1550 [1402-1699] msec vs. 1436 [1399-1473] msec, respectively). Compared to healthy controls, boys with DMD had preserved RVEF (51.8 [9.9]% vs. 54.2 [7.2]%, P = 0.31) and significantly reduced RVMi (29.8 [9.7] g vs. 48.0 [15.7] g), RVEDVi (69.8 [29.7] mL/m2 vs. 89.1 [21.9] mL/m2 ), and TAE (22.0 [3.2] cm vs. 26.0 [4.7] cm). Significant correlations were found between precontrast RV-T1 and RVEF (ß = -0.48%/msec) and between LV-T1 and LVEF (ß = -0.51%/msec). DATA CONCLUSION: Precontrast RV-T1 is elevated in boys with DMD compared to healthy controls and is negatively correlated with RVEF. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 2.

T1-Mapping and extracellular volume estimates in pediatric subjects with Duchenne muscular dystrophy and healthy controls at 3T.

BACKGROUND: Cardiovascular disease is the leading cause of death in patients with Duchenne muscular dystrophy (DMD)-a fatal X-linked genetic disorder. Late gadolinium enhancement (LGE) imaging is the current gold standard for detecting myocardial tissue remodeling, but it is often a late finding. Current research aims to investigate cardiovascular magnetic resonance (CMR) biomarkers, including native (pre-contrast) T1 and extracellular volume (ECV) to evaluate the early on-set of microstructural remodeling and to grade disease severity. To date, native T1 measurements in DMD have been reported predominantly at 1.5T. This study uses 3T CMR: (1) to characterize global and regional myocardial pre-contrast T1 differences between healthy controls and LGE + and LGE- boys with DMD; and (2) to report global and regional myocardial post-contrast T1 values and myocardial ECV estimates in boys with DMD, and (3) to identify left ventricular (LV) T1-mapping biomarkers capable of distinguishing between healthy controls and boys with DMD and detecting LGE status in DMD. METHODS: Boys with DMD (N = 28, 13.2 ± 3.1 years) and healthy age-matched boys (N = 20, 13.4 ± 3.1 years) were prospectively enrolled and underwent a 3T CMR exam including standard functional imaging and T1 mapping using a modified Look-Locker inversion recovery (MOLLI) sequence. Pre-contrast T1 mapping was performed on all boys, but contrast was administered only to boys with DMD for post-contrast T1 and ECV mapping. Global and segmental myocardial regions of interest were contoured on mid LV T1 and ECV maps. ROI measurements were compared for pre-contrast myocardial T1 between boys with DMD and healthy controls, and for post-contrast myocardial T1 and ECV between LGE + and LGE- boys with DMD using a Wilcoxon rank-sum test. Results are reported as median and interquartile range (IQR). p-Values < 0.05 were considered significant. Receiver Operating Characteristic analysis was used to evaluate a binomial logistic classifier incorporating T1 mapping and LV function parameters in the tasks of distinguishing between healthy controls and boys with DMD, and detecting LGE status in DMD. The area under the curve is reported. RESULTS: Boys with DMD had significantly increased global native T1 [1332 (60) ms vs. 1289 (56) ms; p = 0.004] and increased within-slice standard deviation (SD) [100 (57) ms vs. 74 (27) ms; p = 0.001] compared to healthy controls. LGE- boys with DMD also demonstrated significantly increased lateral wall native T1 [1322 (68) ms vs. 1277 (58) ms; p = 0.001] compared to healthy controls. LGE + boys with DMD had decreased global myocardial post-contrast T1 [565 (113) ms vs 635 (126) ms; p = 0.04] and increased global myocardial ECV [32 (8) % vs. 28 (4) %; p = 0.02] compared to LGE- boys. In all classification tasks, T1-mapping biomarkers outperformed a conventional biomarker, LV ejection fraction. ECV was the best performing biomarker in the task of predicting LGE status (AUC = 0.95). CONCLUSIONS: Boys with DMD exhibit elevated native T1 compared to healthy, sex- and age-matched controls, even in the absence of LGE. Post-contrast T1 and ECV estimates from 3T CMR are also reported here for pediatric patients with DMD for the first time and can distinguish between LGE + from LGE- boys. In all classification tasks, T1-mapping biomarkers outperform a conventional biomarker, LVEF.

Multimodality cardiovascular imaging in the diagnosis and management of prosthetic valve infective endocarditis in children report of two cases and brief review of the literature.

Diagnosing prosthetic valve infective endocarditis in children is challenging. Transthoracic and transesophageal echocardiography can yield false-negative results. Data are lacking in paediatric multimodality imaging in prosthetic valve infective endocarditis. We present two children with repaired CHD where initial echocardiogram was non-diagnostic, while CT angiogram and 18F-fluorodeoxyglucose positron emission tomography in combination with CT angiography, respectively, confirmed the diagnosis of endocarditis affecting clinical management.

Myocardial T1 mapping for patients with implanted cardiac devices using wideband inversion recovery spoiled gradient echo readout.

PURPOSE: To develop and validate a technique for myocardial T1 mapping in patients with implantable cardioverter defibrillators (ICDs). METHODS: A MOLLI-based pulse sequence, named Wideband-FLASH-MOLLI, was developed by incorporating a fast low angle shot (FLASH) readout and a wideband inversion pulse. The performance of Wideband-FLASH-MOLLI was evaluated using phantom studies and validated in eight healthy volunteers and ten patients with ICDs at 1.5 Tesla. Comparisons were made between Wideband-FLASH-MOLLI, FLASH-MOLLI, and bSSFP-MOLLI sequences. RESULTS: In phantom studies, the maximum T1 estimation errors using Wideband-FLASH-MOLLI with and without an ICD were less than 3% for T1 range from 212 to 1673 ms. In all healthy volunteers, there was no significant native myocardial T1 estimation difference using Wideband-FLASH-MOLLI before and after the external attachment of an ICD to the body coil (1178 ± 27 ms versus 1174 ± 33 ms; P = 0.41). Due to the presence of an ICD, the magnitude images acquired using bSSFP-MOLLI and FLASH-MOLLI showed severe artifacts within the myocardium. In contrast, no or negligible device-induced artifacts were noted within the myocardial regions of the healthy volunteers or the patients with ICDs when using Wideband-FLASH-MOLLI. CONCLUSION: This study demonstrates the feasibility of using Wideband-FLASH-MOLLI to mitigate image artifacts and to produce accurate myocardial T1 maps in patients with ICDs. Magn Reson Med 77:1495-1504, 2017. © 2016 International Society for Magnetic Resonance in Medicine.

Aortic Root Dilatation in Mucopolysaccharidosis I-VII.

The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children's Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were extracted by retrospective chart review and echocardiographic measurements. Descriptive statistics, ANOVA, and paired post-hoc t-tests were used to summarize the aortic dimensions. Exact binomial 95% confidence intervals (CIs) were constructed for ARD, defined as a z-score greater than 2 at the SoV. The patient age ranged from 3.4-25.9 years (mean 13.3 ± 6.1), the height from 0.87-1.62 meters (mean 1.24 ± 0.21), and the weight from 14.1-84.5 kg (mean 34.4 ± 18.0). The prevalence of dilation at the AVA was 41% (14/34; 95% CI: 25%-59%); at the SoV was 35% (12/34; 95% CI: 20%-54%); and at the STJ was 30% (9/30; 95% CI: 15%-49%). The highest prevalence of ARD was in MPS IVa (87.5%). There was no significant difference between mean z-scores of MPS patients who received treatment with hematopoietic stem cell transplantation (HSCT) or enzyme replacement therapy (ERT) vs. untreated MPS patients at the AVA (z = 1.9 ± 2.5 vs. z = 1.5 ± 2.4; p = 0.62), SoV (z = 1.2 ± 1.6 vs. z = 1.3 ± 2.2; p = 0.79), or STJ (z = 1.0 ± 1.8 vs. z = 1.2 ± 1.6; p = 0.83). The prevalence of ARD was 35% in our cohort of MPS I-VII patients. Thus, we recommend screening for ARD on a routine basis in this patient population.

High-field MR imaging in pediatric congenital heart disease: initial results.

BACKGROUND: Comprehensive assessment of pediatric congenital heart disease (CHD) at any field strength mandates evaluation of both vascular and dynamic cardiac anatomy for which diagnostic quality contrast-enhanced magnetic resonance angiography (CEMRA) and cardiac cine are crucial. OBJECTIVE: To determine whether high-resolution (HR) CEMRA and steady-state free precession (SSFP) cine can be performed reliably at 3.0 T in children with CHD and to compare the image quality to similar techniques performed at 1.5 T. MATERIALS AND METHODS: Twenty-eight patients with a median age of 5 months and average weight 9.0 ± 7.8 kg with suspected or known CHD were evaluated at 3.0 T. SSFP cine (n = 86 series) and HR-CEMRA (n = 414 named vascular segments) were performed and images were scored for image quality and artifacts. The findings were compared to those of 28 patients with CHD of similar weight who were evaluated at 1.5 T. RESULTS: Overall image quality on HR-CEMRA was rated as excellent or good in 96% (397/414) of vascular segments at 3.0 T (k = 0.49) and in 94% (349/371) of vascular segments at 1.5 T (k = 0.36). Overall image quality of SSFP was rated excellent or good in 91% (78/86) of cine series at 3.0 T (k = 0.55) and in 81% (87/108) at 1.5 T (k = 0.47). Off-resonance artifact was common at both field strengths, varied over the cardiac cycle and was more prevalent at 3.0 T. At 3.0 T, off-resonance dark band artifact on SSFP cine was absent in 3% (3/86), mild in 69% (59/86), moderate in 27% (23/86) and severe in 1% (1/86) of images; at 1.5 T, dark band artifact was absent in 16% (17/108), mild in 69% (75/108), moderate in 12% (13/108) and severe in 3% (3/108) of cine images. The signal-to-noise ratio and contrast-to-noise ratio of both SSFP cine and HR-CEMRA images were significantly higher at 3.0 T than at 1.5 T (P < 0.001). CONCLUSION: Signal-to-noise ratio and contrast-to-noise ratio of high-resolution contrast-enhanced magnetic resonance angiography and SSFP cine were higher at 3.0 T than at 1.5 T. Artifacts on SSFP cine were cardiac phase specific and more prevalent at 3.0 T such that frequency-tuning was required in one-third of exams. In neonates, high spatial resolution CEMRA was highly reliable in defining extracardiac vascular anatomy.

Ferumoxytol-Enhanced Cardiac Magnetic Resonance Angiography and 4D Flow: Safety and Utility in Pediatric and Adult Congenital Heart Disease.

Cardiac magnetic resonance imaging and angiography have a crucial role in the diagnostic evaluation and follow up of pediatric and adult patients with congenital heart disease. Although much of the information required of advanced imaging studies can be provided by standard gadolinium-enhanced magnetic resonance imaging, the limitations of precise bolus timing, long scan duration, complex imaging protocols, and the need to image small structures limit more widespread use of this modality. Recent experience with off-label diagnostic use of ferumoxytol has helped to mitigate some of these barriers. Approved by the U.S. FDA for intravenous treatment of anemia, ferumoxytol is an ultrasmall superparamagnetic iron oxide nanoparticle that has a long blood pool residence time and high relaxivity. Once metabolized by macrophages, the iron core is incorporated into the reticuloendothelial system. In this work, we aim to summarize the evolution of ferumoxytol-enhanced cardiovascular magnetic resonance imaging and angiography and highlight its many applications for congenital heart disease.

Reproducibility of Left Ventricular CINE DENSE Strain in Pediatric Subjects with Duchenne Muscular Dystrophy.

Cardiomyopathy is the leading cause of mortality in boys with Duchenne muscular dystrophy (DMD). Left ventricular (LV) peak mid-wall circumferential strain (Ecc) is a sensitive early biomarker for evaluating both the subtle and variable onset and the progression of cardiomyopathy in pediatric subjects with DMD. Cine Displacement Encoding with Stimulated Echoes (DENSE) has proven sensitive to changes in Ecc, but its reproducibility has not been reported in a pediatric cohort or a DMD cohort. The objective was to quantify the intra-observer repeatability, and intra-exam and inter-observer reproducibility of global and regional Ecc derived from cine DENSE in DMD patients (N = 10) and age-and sex-matched controls (N = 10). Global and regional Ecc measures were considered reproducible in the intra-exam, intra-observer, and inter-observer comparisons. Intra-observer repeatability was highest, followed by intra-exam reproducibility and then inter-observer reproducibility. The smallest detectable change in Ecc was 0.01 for the intra-observer comparison, which is below the previously reported yearly decrease of 0.013 ± 0.015 in Ecc in DMD patients.

A Case of Lemierre Syndrome in the Era of COVID-19: All That Glitters Is Not Gold.

We report a case of a 15-year-old female presenting with a serious multisystemic inflammatory illness during a surge of SARS-CoV-2 (COVID-19) cases in our county. The initial clinical findings of sore throat and neck stiffness, followed by signs of sepsis, raised suspicion of Lemierre syndrome early in her hospital course. However, the presence of severe respiratory distress, multifocal pneumonia with pleural effusion on chest radiograph, acute kidney injury, and the discovery of coronary artery ectasia, pointed to the new entity "multisystem inflammatory syndrome in children (MIS-C)." Immune modulatory treatment was thus considered. However, progressive neck pain and swelling, coupled with the eventual growth of Fusobacterium necrophorum on blood culture, eventually led to the correct diagnosis of Lemierre syndrome.

Danon disease with typical early-onset cardiomyopathy in a male: focus on a novel LAMP-2 mutation.

We report a case of a 16-yr-old male with Danon disease caused by a novel mutation in the LAMP-2 gene. Mutations in the LAMP-2 gene result in the absence of LAMP-2 on immunohistochemical staining of muscle tissue, thus defining Danon disease, a rare X-linked myopathy. It is characterized clinically by HCM or left ventricular hypertrophy, a WPW pattern on ECG, variable degrees of muscular weakness (skeletal myopathy), mental retardation, and retinal changes. The patient presented with severe skeletal muscular weakness and respiratory failure. He also had a history of two OHTs, the first one for severe HCM and the second for allograft rejection. The patient's myopathy was initially presumed to be exclusively related to steroid-induced "critical care myopathy." However, further evaluation with a thigh muscle biopsy revealed autophagic vacuoles with sarcolemnal features suggestive of a lysosomal storage disorder. DNA analysis ultimately identified a previously unreported hemizygous IVS6+3_+6delGAGT splice site deletion mutation in the LAMP-2 gene located within the 5' splice site of intron 6, consistent with Danon disease.

Cardiac MRI biomarkers for Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a fatal inherited genetic disorder that results in progressive muscle weakness and ultimately loss of ambulation, respiratory failure and heart failure. Cardiac MRI (MRI) plays an increasingly important role in the diagnosis and clinical care of boys with DMD and associated cardiomyopathies. Conventional cardiac MRI biomarkers permit measurements of global cardiac function and presence of fibrosis, but changes in these measures are late manifestations. Emerging MRI biomarkers of myocardial function and structure include the estimation of rotational mechanics and regional strain using MRI tagging; T1-mapping; and T2-mapping, a marker of inflammation, edema and fat. These emerging biomarkers provide earlier insights into cardiac involvement in DMD, improving patient care and aiding the evaluation of emerging therapies.

Prognostic Significance of Left Ventricular Fibrosis in Patients With Congenital Bicuspid Aortic Valve.

This study sought to evaluate the prognostic value of left ventricular (LV) fibrosis assessed by late gadolinium enhancement (LGE) of the myocardium during cardiac magnetic resonance (CMR) imaging in patients with bicuspid aortic valve (BAV), which is associated with early aortic valve fibrosis and calcification. To what degree the LV myocardial wall is affected by fibrosis and its prognostic value is currently unknown. This is a retrospective, single-center study evaluating all adult patients with BAV who had CMR and followed from March 2002 to March 2016. CMR and transthoracic echocardiogram images were reviewed. Clinical data were abstracted from the electronic medical record. A total of 29 patients were included in the study, of which 11 (38%) had CMR studies that demonstrated the presence of LGE. Patients with LGE had significantly higher aortic valve mean gradients by echocardiography when compared with LGE-negative patients (30.3 ± 7.2 mm Hg vs 14.7 ± 3.6 mm Hg, p = 0.049). They were also more likely to have LV hypertrophy. Patients with LGE were 10 times more likely to need aortic valve replacement within 1 year of the CMR study than did patients without LGE (55% vs 5.5%, p = 0.0028). In conclusion, evaluation of LGE by CMR as a marker of LV myocardial fibrosis can have additional prognostic value when evaluating patients with aortic stenosis secondary to BAV.

Diagnosis and follow-up of neonatal tetralogy of Fallot and hemitruncus with discontinuous pulmonary arteries noninvasively using awake ultra low-dose computed tomographic angiography.

We present a case of an ex-30 week premature male infant diagnosed postnatally with Tetralogy of Fallot, hemitruncus, and discontinuous pulmonary arteries (PAs) at 6 days of life. The patient was diagnosed by echocardiography, and the diagnosis was confirmed on subsequent dual-energy chest CT angiogram. In our patient, the left PA arose directly from the aorta, whereas the right PA originated normally from the right ventricular outflow tract. At 9 days of life, he underwent successful surgical palliation with placement of a modified Blalock-Taussig (aortopulmonary) shunt from the base of the left subclavian artery to the anomalously connected left PA along with anastomosis together of the right and left branch PAs to establish continuity with the main PA. Such cases have been described and are rare. The specific aim of this case report is to illustrate the added benefit of dual-energy electrocardiographically-triggered computed tomographic angiography (CTA) along with standard echocardiography. In addition, high quality images useful in preoperative planning were obtained noninvasively using an ultra low radiation dose without the need for sedation. The information obtained proved essential for confirmation of the diagnosis, preoperative planning, and post-surgical monitoring of branch PA development.

Feasibility and reproducibility of three-dimensional echocardiographic assessment of right ventricular size and function in pediatric patients.

BACKGROUND: Quantitative assessment of right ventricular (RV) size and function is important in congenital heart disease. Although three-dimensional (3D) echocardiography overcomes geometric challenges that limit two-dimensional echocardiography, its feasibility and reproducibility in children have not been systematically evaluated. The goal of this study was to determine the feasibility and reproducibility of 3D echocardiographic RV measurements in children. METHODS: Patients aged 0 to 20 years were prospectively enrolled. Full-volume 3D echocardiographic RV data sets were acquired in each from the subxiphoid and apical four-chamber views by a trained sonographer. Another sonographer then acquired a second image data set from the same patient. RV volumes and ejection fraction were then measured offline. Outcomes included successful acquisition, measurable data set, and observer and interstudy variability. RESULTS: Three-dimensional echocardiographic RV data sets were obtainable in 67 of 70 patients (96%) and were measurable from at least one view in 39 (58%). Factors associated with nonmeasurable data sets by univariate analysis included older age, larger body surface area and body mass index, no sedation, and female gender. Multivariate analysis identified older age (odds ratio, 1.34; 95% confidence interval, 1.17-1.55, P < .0001) and female gender (odds ratio, 6.06; 95% confidence interval, 1.45-25.3, P = .0135) as independently associated with nonmeasurable RV data sets. Intraobserver, interobserver, and interstudy agreement was excellent for analyzable RV volumes and modest for RV ejection fraction. CONCLUSIONS: Transthoracic 3D echocardiographic RV volumes were measurable in 58% of children with and without congenital heart disease. Older age and female gender were independently associated with nonanalyzable data sets. When feasible, measurements of RV volumes are highly reproducible.

Magnetic resonance angiography in paced complex heterotaxy syndrome with Fontan conduit obstruction and venovenous collateral decompression.

Imaging of complex congenital heart diseases (CHDs) in children is challenging. This article reviews the complementary role of high temporal and high spatial resolution magnetic resonance (MR) angiographic imaging techniques in evaluation of a patient with complex congenital cardiovascular disease and related postsurgical complications. A 4-year-old female patient with complex CHD and multiple previous palliative surgical procedures underwent MR angiography to evaluate the cause of refractory hypoxia. High-resolution MR angiography demonstrated the complex postsurgical cardiovascular anatomy and also assisted in the evaluation of cavopulmonary shunt patency and secondary venovenous shunt formation. Time-resolved MR angiography evaluated pulmonary perfusion and demonstrated a significant pulmonary arteriovenous malformation. This information guided physicians in planning further managements, which resulted in a satisfactory clinical outcome.