Regional Arch Measurements Differ Between Imaging Modalities in Infants With Aortic Coarctation.

BACKGROUND: Aortic arch measurements provide a framework for surgical decision-making in neonatal aortic coarctation, specifically in the determination of approach for arch repair by lateral thoracotomy vs median sternotomy. The purpose of this study was to evaluate our experience with transthoracic echocardiography (TTE) and computed tomography angiography (CTA) in the preoperative evaluation of infants with aortic coarctation, specifically comparing arch dimensions as a function of imaging modality. METHODS: Imaging data were reviewed for all infants undergoing surgical repair of aortic coarctation at our institution from 2012 to 2022. Infants with both TTE and CTA evaluations were included. Aortic measurements were compared at predefined anatomic regions including ascending aorta, proximal arch, distal arch, and isthmus. RESULTS: During the study period, 372 infants underwent surgical coarctation repair; 72 (19.4%) infants had TTE and CTA arch evaluations preoperatively. Significant discrepancies between imaging modalities were defined by poor correlation coefficients and absolute measurement differences and were most prominent in the proximal aortic arch (R2 = 0.23 [-4.4 to 3.2 mm]) and isthmus regions (R2 = 0.11 [-4.2 to 1.7 mm]). Improved correlation was demonstrated in the ascending aorta (R2 = 0.63) and distal aortic arch (R2 = 0.54). CONCLUSIONS: Significant variability exists between TTE- and CTA-derived aortic measurements in infants with coarctation, with proximal arch measurements demonstrating the poorest correlation. This anatomic location represents a commonly used arch region for the determination of approach for repair of neonatal aortic coarctation. Thus, these findings have important implications for current preoperative surgical decision-making paradigms and future prospective study to minimize the risk of residual or recurrent arch obstruction.

Feasibility of gray-blood late gadolinium enhancement evaluation in young patients with congenital and acquired heart disease.

Background: Late gadolinium enhancement (LGE) sequences have become common in pediatric cardiovascular magnetic resonance (CMR) to assess for myocardial fibrosis. Bright-blood late gadolinium enhancement (BB-LGE) by conventional phase-sensitive inversion recovery (PSIR) is commonly utilized, but similar inversion time (TI) value of fibrosis and left ventricular (LV) blood pool can make subendocardial areas difficult to assess. A gray-blood LGE (GB-LGE) technique has been described, targeting nulling of the LV blood pool and demonstrating improvement in ischemic scar detection over BB-LGE in adult patients. We sought to evaluate the feasibility of the GB-LGE technique in a young population with congenital and acquired heart disease and compare its ability to detect subendocardial scar to conventional BB-LGE. Methods: Seventy-six consecutive patients referred for clinical CMR underwent both BB-LGE and GB-LGE on 1.5 T and 3 T scanners. Conventional PSIR sequences were obtained with TI to null the myocardium (BB-LGE) in short-axis and horizontal long-axis stacks. Same PSIR stacks were immediately repeated with TI to null the blood pool (GB-LGE). Both sequences were reviewed separately a week apart by two readers, blinded to the initial clinical interpretation. Studies were analyzed for overall image quality, confidence in scar detection, confidence in detection of LGE, LGE class, inter- and intra-observer agreement for the presence of scar, and intraclass correlation coefficient (ICC) for total scar burden. Results: Overall confidence in myocardial scar detection by BB-LGE or GB-LGE as well as grading of image quality were not statistically different [(p = 1 and p = 1) and (p = 0.53, p = 0.18), respectively]. There was very good inter-observer agreement for the presence of scar on BB-LGE (K = 0.88, 95% CI 0.77-0.99) and GB-LGE (K = 0.84, 95% CI 0.7-0.96), as well as excellent intra-observer agreement for both readers (K = 0.93, 95% CI 0.87-0.99; and K = 0.81, 95% CI 0.69-0.95). Interclass correlation coefficient for total scar burden was excellent for BB-LGE (ICC = 0.98, 95% CI 0.96-0.99) and GB-LGE (ICC = 0.94, 95% CI 0.91-0.97). Conclusions: The GB-LGE technique is feasible in the pediatric population with congenital and acquired heart disease. It can detect subendocardial/ischemic scar similar to conventional bright-blood PSIR sequences in the pediatric population.

Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.

BACKGROUND: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. OBJECTIVE: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. MATERIALS AND METHODS: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. RESULTS: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. CONCLUSION: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.

Unexpected extracardiac findings in cardiac computed tomography from neonates to young adults.

BACKGROUND: Modern CT scanners with lower radiation doses have resulted in large numbers of cardiac CTs being performed in children. As seen in adults, pediatric cardiac CT has the potential to demonstrate extracardiac variants and pathology that can occur in conjunction with congenital heart disease (CHD). Prior publications demonstrated a high incidence of extracardiac findings in various locations but the prevalence of urgent unexpected extracardiac findings in children is unknown. OBJECTIVE: The purpose of this study was to describe the incidence, distribution and clinical significance of the extracardiac findings on pediatric cardiac CT at a tertiary referral center. MATERIALS AND METHODS: We reviewed all reports (n = 648) for 554 children through young adults who received a cardiac CT study between Jan. 2, 2018, and March 10, 2020, at our tertiary referral pediatric hospital. We interrogated CT reports for extracardiac findings and categorized them by system (airway, pulmonary, abdomen, malpositioned lines and musculoskeletal). We then subclassified each of these findings by level of clinical importance based upon the need for intervention or treatment into low, medium or high importance. High-importance findings were confirmed with a focused chart review. If a patient had more than one CT with a persistent extracardiac finding, the finding was only counted once. RESULTS: We identified 562 individual extracardiac findings, with one or more extracardiac findings present in 91% of the study population. Extracardiac findings with high clinical importance, requiring urgent attention or intervention, were present in 10% (57/554) of cases. The most common location of extracardiac findings was pulmonary (50%; 280/562), followed by airway (22%; 125/562) and abdomen (9%; 52/562). CONCLUSION: Unexpected highly important extracardiac findings were found in 10% of patients. Therefore, extracardiac structures should be scrutinized for the timely identification of potentially highly important findings.

Imaging of pediatric cardiac tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Cardiac tumors in children are rare and the majority are benign. The most common cardiac tumor in children is rhabdomyoma, usually associated with tuberous sclerosis complex. Other benign cardiac masses include fibromas, myxomas, hemangiomas, and teratomas. Primary malignant cardiac tumors are exceedingly rare, with the most common pathology being soft tissue sarcomas. This paper provides consensus-based imaging recommendations for the evaluation of patients with cardiac tumors at diagnosis and follow-up, including during and after therapy.

Computed Tomographic Angiography Provides Reliable Coronary Artery Evaluation in Infants With Pulmonary Atresia Intact Ventricular Septum.

Evaluate the use of coronary CTA as an initial assessment for determining Right Ventricle Dependent Coronary Circulation (RVDCC) in neonates with Pulmonary Atresia with Intact Ventricular Septum (PA IVS). Retrospective review of cases with coronary CTA and compare with available catheter angiography, pathology, surgical reports, and outcomes from Mar 2015 to May 2022. In our cohort of 16 patients, 3 were positive for RVDCC, confirmed by pathologic evaluation, and there was concordance for presence or absence of RVDCC with catheter angiography in 5 patients (4 negatives for RVDCC, 1 positive). Clinical follow up for the 8 patients that underwent RV decompression had no clinical evidence of myocardial ischemia. Our findings suggest that coronary CTA is reliable as first-line imaging for determination of RVDCC in neonates with PA IVS. These findings, if supported by further prospective study, may reserve invasive coronary angiography for cases with diagnostic uncertainty or at the time of necessary transcatheter interventions.

Imaging Evaluation of Kawasaki Disease.

PURPOSE OF REVIEW: This review provides the summary of the appropriate use of these modalities when caring for patients with Kawasaki disease at diagnosis and for long-term management. RECENT FINDINGS: Kawasaki disease is an inflammatory syndrome of unknown etiology that can result in coronary artery dilations or aneurysms if left untreated in 25% of the patients and 3-5% in treated patients. In addition to coronary artery aneurysms, patients can have ventricular dysfunction, valvular regurgitation, aortic root dilation, and pericardial effusion due to inflammation of the myocardium. Noninvasive imaging modalities are important to these assessments. Echocardiography is the first-line noninvasive evaluation of coronaries and function. Cardiac magnetic resonance imaging is useful for functional assessment in long-term follow-up. Distal coronaries, thromboses, and stenoses are best evaluated by cardiac computed tomography. Future research should demonstrate the effectiveness of advanced functional imaging in patients with Kawasaki disease and decreased radiation from cardiac computed tomography.

Chest computed tomography findings of ground-glass nodules with enhancing central vessel/nodule in pediatric patients with BMPR2 mutations and plexogenic arteriopathy.

BACKGROUND: Germline mutation in bone morphogenetic protein type II (BMPR2) is the most common cause of idiopathic/heritable pulmonary hypertension in pediatric patients. Despite the discovery of this gene there are no known descriptions of the CT or CT angiography findings in these children. OBJECTIVE: To correlate the clinical presentation, pathology and chest CT findings in pediatric patients with pulmonary hypertension caused by mutations in the BMPR2 gene. MATERIALS AND METHODS: We performed a search to identify pediatric patients with a BMPR2 mutation and CT or CT angiography with the clinical history of pulmonary hypertension. Three pediatric radiologists reviewed the children's CT imaging findings and ranked the dominant findings in order of prevalence via consensus. RESULTS: We identified three children with pulmonary hypertension and confirmed germline BMPR2 mutations, two of whom had undergone lung biopsy. We then correlated the imaging findings with histopathology and clinical course. CONCLUSION: All of our patients with BMPR2 mutations demonstrated a distinct CT pattern of ground-glass nodules with a prominent central enhancing vessel/nodule. These findings correlated well with the pathological findings of plexogenic arteriopathy.

Free-breathing magnetic resonance imaging with radial k-space sampling for neonates and infants to reduce anesthesia.

BACKGROUND: Conventional chest and abdominal MRI require breath-holds to reduce motion artifacts. Neonates and infants require general anesthesia with intubation to enable breath-held acquisitions. OBJECTIVE: We aimed to validate a free-breathing approach to reduce general anesthesia using a motion-insensitive radial acquisition with respiratory gating. MATERIALS AND METHODS: We retrospectively enrolled children <3 years old who were referred for MRI of the chest or abdomen. They were divided into two groups according to MRI protocol: (1) breath-held scans under general anesthesia with T2-weighted single-shot fast spin-echo (SSFSE) and contrast-enhanced T1-weighted modified Dixon, and (2) free-breathing scans using radial sequences (T2-W MultiVane XD and contrast-enhanced T1-W three-dimensional [3-D] Vane XD). Two readers graded image quality and motion artifacts. RESULTS: We included 23 studies in the free-breathing cohort and 22 in the breath-hold cohort. The overall imaging scores for the free-breathing radial T2-W sequence were similar to the scores for the breath-held T2-W SSFSE sequence (chest, 3.6 vs. 3.2, P=0.07; abdomen, 3.9 vs. 3.7, P=0.66). The free-breathing 3-D radial T1-W sequence also had image quality scores that were similar to the breath-held T1-W sequence (chest, 4.0 vs. 3.0, P=0.06; abdomen, 3.7 vs. 3.9, P=0.15). Increased motion was seen in the abdomen on the radial T2-W sequence (P<0.001), but increased motion was not different in the chest (P=0.73) or in contrast-enhanced T1-W sequences (chest, P=0.39; abdomen, P=0.15). The mean total sequence time was longer in free-breathing compared to breath-held exams (P<0.01); however, this did not translate to longer overall exam times (P=0.94). CONCLUSION: Motion-insensitive radial sequences used for infants and neonates were of similar image quality to breath-held sequences and had decreased sedation and intubation.

Coronary computed tomography angiography in children.

Imaging the coronary arteries of children, with their faster heart rates, small vessel size and common inability to lie still or breath-hold, has been a major challenge. With numerous advances in technology, CT examinations can now be performed quickly, often with children free-breathing and with much lower radiation doses than previously. This has led to increased use in children. Care must be taken with technique and choice of electrocardiogram (ECG)-gating technique to obtain adequate imaging for a diagnosis while keeping radiation dose as low as reasonably achievable (ALARA). In this paper, we discuss techniques and tips for CT imaging of the coronary arteries in children, including use of dual-source- and ultrawide-detector CT scanners.

Magnetic resonance imaging findings following button battery ingestion.

BACKGROUND: Lithium button battery ingestions have been increasing in frequency since the early 2000s and can develop severe and sometimes fatal complications from caustic injury even after rapid battery removal. To aid in clinical decision-making, we began obtaining magnetic resonance imaging (MRI)/MR angiography in these patients. OBJECTIVE: Our goal was to review MRI/MR angiography imaging in button battery ingestion cases and compare with other imaging, clinical data and outcomes in these patients. MATERIALS AND METHODS: In this retrospective institutional-review-board-approved study, we reviewed all button battery ingestion cases with MRI from April 2012 to September 2018. Clinical data, endoscopic findings and all imaging studies were rereviewed. MRIs were evaluated for inflammation, blooming artifact and complications including vascular injury, tracheoesophageal fistula, esophageal perforation and spondylodiscitis, and compared to endoscopy, esophagram and bronchoscopy. RESULTS: Twenty-three patients with button battery ingestions had a total of 51 MRI/MR angiograms. Seventy percent of the cohort was male with a median age of 2 years (range: 0.94-17 years). Severe complications were found in 48% of patients (11/23), including esophageal perforation (n=11), tracheoesophageal fistula (n=3) and spondylodiscitis (n=1). No patients had vascular injury. Cervical location of the battery was significantly associated with severe complications (10/11 cases). The length of the blooming artifact was greater than 2 cm in those with severe complications and, in most cases, <2 cm in those without severe complications. All complications were seen on initial screening MR exam with serial exams showing decreased inflammation. CONCLUSION: MRI/MR angiography can provide valuable information about complications, including esophageal perforation, tracheoesophageal fistula and spondylodiscitis. Decreasing inflammation surrounding the esophagus and vasculature is believed to be clinically reassuring and aids in managing button battery ingestion patients.

Use of compressed sensing to reduce scan time and breath-holding for cardiac cine balanced steady-state free precession magnetic resonance imaging in children and young adults.

BACKGROUND: Conventional pediatric volumetric MRI acquisitions of a short-axis stack typically require multiple breath-holds under anesthesia. OBJECTIVE: Here, we aimed to validate a vendor-optimized compressed-sensing approach to reduce scan time during short-axis balanced steady-state free precession (bSSFP) cine imaging. MATERIALS AND METHODS: Imaging was performed in 28 patients (16±9 years) in this study on a commercial 3-tesla (T) scanner using retrospective electrocardiogram-gated cine bSSFP. Cine short-axis images covering both ventricles were acquired with conventional parallel imaging and a vendor-optimized parallel imaging/compressed-sensing approach. Qualitative Likert scoring for blood-myocardial contrast, edge definition, and presence of artifact was performed by two experienced radiologists. Quantitative comparisons were performed including biventricular size and function. A paired t-test was used to detect significant differences (P<0.05). RESULTS: Scan duration was 7±2 s/slice for conventional imaging (147±33 s total) vs. 4±2 s/slice for compressed sensing (83±28 s total). No significant differences were found with qualitative image scores for blood-myocardial contrast, edge definition, and presence of artifact. No significant differences were found in volumetric analysis between the two sequences. The number of breath-holds was 10±4 for conventional imaging and 5±3 for compressed sensing. CONCLUSION: Compressed sensing allowed for a 50% reduction in the number of breath-holds and a 43% reduction in the total scan time without differences in the qualitative or quantitative measurements as compared to the conventional technique.

Visualization of proximal coronary arteries on high-pitch electrocardiogram-triggered computed tomography in pediatric congenital heart disease: effects of heart rate and body surface area.

BACKGROUND: As CT technology has advanced, techniques for pediatric cardiac CT in congenital heart disease have evolved from retrospective electrocardiography (ECG)-gating with relatively high radiation doses to lower-dose prospective ECG-gating and even single-beat gated scans. Despite these advances, coronary artery imaging in children remains challenging because of their small vessel size and high heart rates, often necessitating retrospective gating. OBJECTIVE: Evaluate coronary artery visualization in pediatric patients (<20 years) who underwent low-dose high-pitch ECG-triggered scans and stratify the probability of coronary artery visualization based upon heart rate and body surface area (BSA). MATERIALS AND METHODS: Two hundred eleven high-pitch ECG-triggered studies from April 2014 to November 2017 were reviewed by two pediatric cardiac imagers in this retrospective study. Patient age, gender, BSA, average heart rate, heart rate variability and use of general anesthesia were recorded as well as dose-length product (DLP) and volumetric CT dose index (CTDIvol). We assessed the coronary artery score using a 5-point scale, with score of ≥3 considered of diagnostic quality. We performed multivariate statistical analysis including logistic regression to analyze effects of heart rate and BSA. RESULTS: Patient age range was 1 day to 19 years (median age 3 years). Heart rate range was 49-188 beats per minute (bpm; median 122 bpm) and BSA range was 0.15-2.07 m2 (median 0.53 m2). The origin and proximal coronary arteries were confidently seen (score ≥3) in 61% of studies in this cohort. Coronary artery visualization scores further increased with increased BSA (P<0.002) and with decreased heart rate (P<0.001). At heart rates <100 bpm or in patients with BSA>0.58, adequate coronary artery visualization was present 72% of the time. CONCLUSION: While in many patients the coronary artery origins are visualized using high-pitch ECG-triggered technique, the importance of coronary artery visualization needs to be weighed with the radiation dose penalty in individual patients to achieve optimal imaging.

Abnormal aortic flow conduction is associated with increased viscous energy loss in patients with repaired tetralogy of Fallot.

OBJECTIVES: Aortopathy in tetralogy of Fallot (TOF) is characterized by increased aortic stiffness, dilation and reduced left ventricular (LV) function. Repair in infancy normalizes aortic dimensions in early childhood. Our prior work demonstrated that early TOF repair does not normalize aortic compliance and that abnormal ascending aortic flow patterns are prevalent. The objectives of this study were to: (i) determine whether proximal aortic flow-mediated viscous energy loss (EL') is elevated in patients with early TOF repair compared with healthy controls, and (ii) determine whether the degree of EL' is associated with LV function. METHODS: Forty-one patients post TOF repair with normalized aortic size and 15 healthy controls underwent 4-dimenisonal-flow magnetic resonance imaging flow analysis and EL' assessment. Correlations between EL', aortic size, and LV function were assessed. RESULTS: The TOF group had increased peak systolic thoracic aorta EL' (3.8 vs 1.5 mW, P = 0.004) and increased averaged EL' throughout the cardiac cycle (1.2 vs 0.5 mW, P = 0.003). Peak and mean systolic EL' in the ascending aorta was increased 2-fold in the TOF group compared with control (peak: 2.0 vs 0.9 mW, P = 0.007). Peak EL' measured along the entire thoracic aortic length correlated with LV ejection fraction (R = -0.45, P = 0.009), indexed LV end-systolic volume (R = -0.40, P = 0.010), and right ventricular end-systolic volume (R = -0.37, P = 0.034). CONCLUSIONS: Patients with repaired TOF exhibit abnormal aortic flow associated with increased EL' in the thoracic aorta. The magnitude of EL' is associated with LV function and volumes. Increased aortic EL' in TOF is likely due to inherently abnormal LV outflow geometry and or right ventricular interaction. Reduced aortic flow efficiency in TOF increases cardiac work and may be an important factor in long-term cardiac performance.

Children with kawasaki disease present elevated stiffness of great arteries: Phase-contrast MRI study.

BACKGROUND: Patients with diagnosed Kawasaki disease (KD) are known to develop extracardiac vascular lesions and are prone to accelerated stiffening of medium-size arteries. PURPOSE: To noninvasively evaluate great vessel (central aorta and main pulmonary artery (MPA)) stiffness using phase-contrast MRI (PC-MRI). STUDY TYPE: Retrospective review. SUBJECTS: Thirty-three patients with previously diagnosed KD and 15 control subjects underwent PC-MRI evaluation. FIELD STRENGTH/SEQUENCE: A free-breathing PC-MRI sequence was applied with Cartesian encoding and retrospective sorting using a 1.5 or 3.0T system. ASSESSMENT: We evaluated regionally specific vessel stiffness using pulse-wave velocity (PWV) and relative area change (RAC) at the ascending aorta, descending aorta, and MPA. STATISTICAL TESTS: Hemodynamics among patients with KD and controls were compared using Student's t-test, Wilcoxon Rank-sum, and χ2 . Additional group-specific comparisons were performed using Kruskal-Wallis or one-way analysis of variance (ANOVA). RESULTS: Patients with KD showed elevated PWV in both ascending (5.0 ± 1.2 vs. 2.4 ± 0.5, P < 0.001) and descending aorta (4.4 ± 2.1 vs. 2.8 ± 0.8, P < 0.001). RAC was correspondingly reduced in both segments (both P < 0.01). PWV measured in MPA was increased in KD patients (2.2 ± 0.5 vs. 1.5 ± 0.6, P = 0.045) while the RAC was reduced (34 ± 6 vs. 47 ± 3, P = 0.045). There were no associations between considered vessel stiffness indices and respective ventricular size and function, functional indices, and no correlations were observed with KD severity markers. DATA CONCLUSION: Patients with KD have elevated great vessel stiffness measured at the chronic stage of the disease. Accelerated stiffness process does not appear to affect biventricular function in youth Level of Evidence: 1 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1228-1236.

Aortic stiffness in adolescent Turner and Marfan syndrome patients.

OBJECTIVES: Turner syndrome (TS) and Marfan syndrome (MFS) are partially characterized by aortopathies with a risk of developing severe aortic dilation, stiffness and consequent dissection and aneurysm formation. The incidence of a bicuspid aortic valve (BAV) is also increased in TS. We investigated aortic stiffness in teenage TS and MFS patients and evaluated to what degree stiffness in TS patients is augmented by the presence of a BAV. METHODS: Fifty-seven patients with TS (n = 37) and MFS (n = 20), as well as 22 controls with similar age and size distribution underwent evaluation of thoracic aortic stiffness using phase-contrast magnetic resonance imaging. Calculated stiffness indices including pulse wave velocity (PWV), distensibility and relative area change (RAC) were collected to characterize the ascending aorta and descending aorta. PWV was also determined to evaluate global aortic arch stiffness. RESULTS: Patients with TS had reduced distensibility (0.43 vs 0.58%/mmHg, P < 0.05) and RAC (21 vs 29%, P < 0.01) in the ascending aorta when compared with normal controls. Similarly, patients with MFS had reduced ascending aortic distensibility (0.39 vs 0.58%/mmHg, P < 0.05) and RAC (22 vs 29%, P < 0.05). There were no differences in measured PWV in the ascending aorta. Patients with TS had significantly elevated PWV measured in the aortic arch when compared with controls (2.7 vs 1.9 m/s, P < 0.05). Patients with MFS had more prominent elevation in aortic arch PWV (4.2 vs 1.9 m/s, P < 0.01). The descending aortas had decreased distensibility (0.36 vs 0.55%/mmHg, P < 0.05) and RAC (18 vs 25%, P < 0.01) only in MFS patients. Additionally, 18 TS patients with a BAV were compared with 19 TS patients with a trileaflet aortic valve, without significant differences observed in any of the considered stiffness indices. CONCLUSIONS: TS and MFS teenage patients display evidence of increased aortic stiffness. In TS patients, this is focused in the ascending aorta and is independent of the presence of a BAV. MFS patients display a generalized reduction in compliance of the entire aorta.

Myocardial Perfusion Reserve Index in Children With Kawasaki Disease.

BACKGROUND: Coronary artery lesions in patients with Kawasaki disease (KD) can impair myocardial perfusion, yet evaluation of perfusion defects by cardiac magnetic resonance (MR) in children is often qualitative. PURPOSE: In this study we aimed to use a quantitative method of myocardial perfusion using stress cardiac MR-derived myocardial perfusion reserve index (MPRI) in children with KD and compare MPRI with ventricular mechanical performance evaluated by cardiac MR strain analysis. STUDY TYPE: This study was a retrospective review. SUBJECTS: Twenty-one children with a diagnosis of KD who underwent stress perfusion cardiac MR were compared with nine controls. FIELD STRENGTH/SEQUENCE: First-pass perfusion imaging using a T1 -weighted gradient echo sequence was performed at rest and stress after administration of adenosine with 1.5T or 3T magnets. ASSESSMENT: The MPRI was calculated as the ratio of maximum slope of myocardial enhancement during stress compared to rest and was evaluated with the American Heart Association 17 segment model. STATISTICAL TESTS: Demographic and clinical characteristics among KD patients and controls were compared using Student's t-test for normally distributed continuous variables, Wilcoxon-rank sum test for nonnormally distributed variables, and χ2 for categorical variables. RESULTS: There was a significant decrease in MPRI in Segment 7 (1.53 vs. 2.23, P = 0.0058) in KD patients compared with controls. The reduction in MPRI in Segment 12 approached statistical significance (1.58 vs. 2.31, P = 0.0636). Three patients who underwent serial studies had decreased MPRI longitudinally. No differences were seen in circumferential or radial strain. DATA CONCLUSION: MPRI shows impaired myocardial perfusion in patients with KD. MPRI can change over time, suggestive of progressive coronary artery changes, which may precede fibrosis and mechanical decline. MPRI can assess segmental and global perfusion defects in patients with KD and should be a part of routine cardiac MR evaluation in KD. LEVEL OF EVIDENCE: 3 Technical Efficacy Stage 3 J. Magn. Reson. Imaging 2017.

Preprocedural Risk Assessment Prior to PPVI with CMR and Cardiac CT.

Percutaneous pulmonary valve intervention (PPVI) is a less invasive and less costly approach to pulmonary valve replacement compared with the surgical alternative. Potential complications of PPVI include coronary compression and pulmonary arterial injury/rupture. The purpose of this study was to characterize the morphological risk factors for PPVI complication with cardiac MRI and cardiac CTA. A retrospective review of 88 PPVI procedures was performed. 44 patients had preprocedural cardiac MRIs or CTAs available for review. Multiple morphological variables on cardiac MRI and CTA were compared with known PPVI outcome and used to investigate associations of variables in determining coronary compression or right ventricular-pulmonary arterial conduit injury. The most significant risk factor for coronary artery compression was the proximity of the coronary arteries to the conduit. In all patients with coronary compression during PPVI, the coronary artery touched the conduit on the preprocedural CTA/MRI, whilst in patients without coronary compression the mean distance between the coronary artery and the conduit was 4.9 mm (range of 0.8-20 mm). Multivariable regression analysis demonstrated that exuberant conduit calcification was the most important variable for determining conduit injury. Position of the coronary artery directly contacting the conduit without any intervening fat may predict coronary artery compression during PPVI. Exuberant conduit calcification increases the risk of PPVI-associated conduit injury. Close attention to these factors is recommended prior to intervention in patients with pulmonary valve dysfunction.

Body MR angiography in children: how we do it.

Vascular pathology is ubiquitous in children. Common indications for angiographic imaging in the body include congenital anomalies, portal hypertension, assessing resectability of neoplasms, renovascular hypertension, vascular malformations, vasculitis, systemic vein thrombosis, and trauma. MR angiography, with or without the use of intravenous contrast agents, is therefore a mainstay in the repertoire of MR imaging in children. Pediatric contrast-enhanced MR angiography has benefited from several innovations in recent years, including improved hardware options like high-field-strength scanners and integrated high-density coil arrays, new sequences that combine parallel imaging, innovative k-space sampling and Dixon fat suppression with time-resolved imaging, new contrast agents with longer blood-pool residence time, and advanced post-processing solutions like image fusion. This article focuses on the principles of contrast-enhanced MR angiography of the body as it pertains to the physiologies and pathologies encountered in children. It also discusses tools to adapt the MR angiographic technique to the clinical indication, as well as pitfalls of post-processing and interpretation in commonly encountered vascular imaging scenarios in the pediatric body.