Cardiac Computed Tomography in Congenital Heart Disease.

Computed tomography (CT) has emerged as a leading imaging modality in the evaluation of congenital heart disease (CHD). With ever-faster acquisition speed, decreasing radiation exposure, impeccable anatomic detail, optional functional data, and numerous post-processing tools, CT offers broad utility in CHD diagnosis, preoperative planning, and postoperative assessment. In this article, the far-reaching role of CT in CHD is reviewed, focusing on technical imaging considerations and key clinical applications.

Abbreviated cardiac magnetic resonance imaging versus echocardiography for interval assessment of systolic function in Duchenne muscular dystrophy: patient satisfaction, clinical utility, and image quality.

PURPOSE: Poor acoustic windows make interval assessment of systolic function in patients with (Duchenne Muscular Dystrophy) DMD by echocardiography (echo) difficult. Cardiac magnetic resonance imaging (CMR) can be challenging in DMD patients due to study duration and patient discomfort. We developed an abbreviated CMR (aCMR) protocol and hypothesized that aCMR would compare favorably to echo in image quality and clinical utility without significant differences in exam duration, patient satisfaction, and functional measurements. METHODS: DMD patients were recruited prospectively to undergo echo and aCMR. Modalities were compared with a global quality assessment score (GQAS), clinical utility score (CUS), and patient satisfaction score (PSS). Results were compared using Wilcoxon signed-rank tests, Spearman correlations, intraclass correlations, and Bland-Altman analyses. RESULTS: Nineteen DMD patients were included. PSS scores and exam duration were equivalent between modalities, while CUS and GQAS scores favored aCMR. ACMR scored markedly higher than echo in RV visualization and assessment of atrial size. Older age was negatively correlated with echo GQAS and CUS scores, as well as aCMR PSS scores. Higher BMI was positively correlated with aCMR GQAS scores. Nighttime PPV requirement and non-ambulatory status were correlated with worse echo CUS scores. Poor image quality precluding quantification existed in five (26%) echo and zero (0%) aCMR studies. There was moderate correlation between aCMR and echo for global circumferential strain and left ventricular four chamber global longitudinal strain. CONCLUSION: The aCMR protocol resulted in improved clinical relevance and quality scores relative to echo, without significant detriment to patient satisfaction or exam duration.

Compact pediatric cardiac magnetic resonance imaging protocols.

Cardiac MRI is in many respects an ideal modality for pediatric cardiovascular imaging, enabling a complete noninvasive assessment of anatomy, morphology, function and flow in one radiation-free and potentially non-contrast exam. Nonetheless, traditionally lengthy and complex imaging acquisition strategies have often limited its broader use beyond specialized centers. In this review, the author presents practical cardiac MRI imaging protocols to facilitate the performance of succinct yet successful exams that provide the most salient clinical data for the majority of congenital and acquired pediatric cardiac disease. In addition, the author reviews newer and evolving techniques that permit more rapid but similarly diagnostic MRI, including compressed sensing and artificial intelligence/machine learning reconstruction, four-dimensional flow acquisition and blood pool contrast agents. With the modern armamentarium of cardiac MRI methods, the goal of compact yet comprehensive exams in children can now be realized.

Pediatric cardiothoracic vasculitis: multimodality imaging review.

The pediatric vasculitides are a relatively uncommon and heterogeneous group of disorders characterized by vessel inflammation, often with cardiothoracic involvement. Diagnosis and monitoring are often clinically challenging because of the nonspecific symptoms and laboratory markers. Thus, imaging has assumed increasing importance for early detection of disease activity, extent and complications as well as long-term monitoring pre- and post-treatment. Herein, we review the major pediatric vasculitides with frequent chest manifestations, including Takayasu arteritis, Kawasaki disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, Behçet disease and potential mimics. We highlight key clinical features and management considerations, emphasizing the central role of imaging.

Postoperative Acute Kidney Injury in Williams Syndrome Compared With Matched Controls.

OBJECTIVES: Cardiovascular manifestations occur in over 80% of Williams syndrome (WS) patients and are the leading cause of morbidity and mortality. One-third of patients require cardiovascular surgery. Renal artery stenosis (RAS) is common in WS. No studies have assessed postoperative cardiac surgery-related acute kidney injury (CS-AKI) in WS. Our objectives were to assess if WS patients have higher risk of CS-AKI postoperatively than matched controls and if RAS could contribute to CS-AKI. DESIGN: This was a retrospective study of all patients with WS who underwent cardiac surgery at our center from 2010 to 2020. The WS study cohort was compared with a group of controls matched for age, sex, weight, and surgical procedure. SETTING: Patients underwent cardiac surgery and postoperative care at Lucile Packard Children's Hospital Stanford. PATIENTS: There were 27 WS patients and 43 controls (31% vs 42% female; p = 0.36). Median age was 1.8 years (interquartile range [IQR], 0.7-3.8 yr) for WS and 1.7 years (IQR, 0.8-3.1 yr) for controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative hemodynamics, vasopressor, total volume input, diuretic administration, and urine output were collected in the first 72 hours. Laboratory studies were collected at 8-hour intervals. Multivariable analysis identified predictors of CS-AKI.Controlled for renal perfusion pressure (RPP) and vasoactive inotrope score (VIS), compared with controls, the odds ratio (OR) of CS-AKI in WS was 4.2 (95% CI, 1.1-16; p = 0.034). Higher RPP at postoperative hours 9-16 was associated with decreased OR of CS-AKI (0.88 [0.8-0.96]; p = 0.004). Increased VIS at hour 6 was associated with an increased OR of CS-AKI (1.47 [1.14-1.9]; p = 0.003). Younger age was associated with an increased OR of CS-AKI (1.9 [1.13-3.17]; p = 0.015). CONCLUSIONS: The OR of CS-AKI is increased in pediatric patients with WS compared with controls. CS-AKI was associated with VIS at the sixth postoperative hour. Increases in RPP and mean arterial pressure were associated with decreased odds of CS-AKI.

Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention.

Increasing the Utilization of Moderate Sedation Services for Pediatric Imaging.

Performing motion-free imaging is frequently challenging in children. To bridge the gap between examinations performed in children who are awake and in those under general anesthesia, a moderate sedation program was implemented at our institution but was seldom used despite substantial eligibility. In conjunction with a 5-month quality improvement (QI) course, a multidisciplinary team was assembled and, by using an A3 approach, sought to address the most important key drivers of low utilization, namely the need for clear moderate sedation eligibility criteria, reliable protocol routing order, consistent moderate sedation screening performed by registered nurses (RNs), and enhanced visibility of moderate sedation services to ordering providers. Initial steps focused on developing better-defined criteria and protocoling standard work for technologists and RNs, with coaching and audits. Modality-specific forecasting was then implemented to reroute profiles of patients who were awaiting scheduling or already scheduled for an examination with general anesthesia to the moderate sedation queue to identify more eligible patients. These manual efforts were coupled with higher reliability but more protracted electronic health record changes, facilitating automated protocol routing on the basis of moderate sedation eligibility and order entry constraints. As a result, scheduled imaging examinations requiring moderate sedation increased from a mean of 1.2 examinations per week to a sustained 6.1 examinations per week (range, 4-8) over the 5-month period, exceeding the team SMART (specific, measurable, achievable, relevant, and time bound) goal to achieve an average of five examinations per week by the QI course end. By targeting the most high-impact yet modifiable process deficiencies through a multifaceted team approach and initially investing in manual efforts to gain cultural buy-in while awaiting higher-reliability interventions, the project achieved success and may serve as a more general model for workflow change when there is organizational resistance. ©RSNA, 2021.

Free-breathing Accelerated Cardiac MRI Using Deep Learning: Validation in Children and Young Adults.

Background Obtaining ventricular volumetry and mass is key to most cardiac MRI but challenged by long multibreath-hold acquisitions. Purpose To assess the image quality and performance of a highly accelerated, free-breathing, two-dimensional cine cardiac MRI sequence incorporating deep learning (DL) reconstruction compared with reference standard balanced steady-state free precession (bSSFP). Materials and Methods A DL algorithm was developed to reconstruct custom 12-fold accelerated bSSFP cardiac MRI cine images from coil sensitivity maps using 15 iterations of separable three-dimensional convolutions and data consistency steps. The model was trained, validated, and internally tested in 10, two, and 10 adult human volunteers, respectively, based on vendor partner-supplied fully sampled bSSFP acquisitions. For prospective external clinical validation, consecutive children and young adults undergoing cardiac MRI from September through December 2019 at a single children's hospital underwent both conventional and highly accelerated short-axis bSSFP cine acquisitions in one MRI examination. Two radiologists scored overall and volumetric three-dimensional mesh image quality of all short-axis stacks on a five-point Likert scale and manually segmented endocardial and epicardial contours. Scan times and image quality were compared using the Wilcoxon rank sum test. Measurement agreement was assessed with intraclass correlation coefficient and Bland-Altman analysis. Results Fifty participants (mean age, 16 years ± 4 [standard deviation]; range, 5-30 years; 29 men) were evaluated. The mean prescribed acquisition times of accelerated scans (non-breath-held) and bSSFP (excluding breath-hold time) were 0.9 minute ± 0.3 versus 3.0 minutes ± 1.9 (P < .001). Overall and three-dimensional mesh image quality scores were, respectively, 3.8 ± 0.6 versus 4.3 ± 0.6 (P < .001) and 4.0 ± 1.0 versus 4.4 ± 0.8 (P < .001). Raters had strong agreement between all bSSFP and DL measurements, with intraclass correlation coefficients of 0.76 to 0.97, near-zero mean differences, and narrow limits of agreement. Conclusion With slightly lower image quality yet much faster speed, deep learning reconstruction may allow substantially shorter acquisition times of cardiac MRI compared with conventional balanced steady-state free precession MRI performed for ventricular volumetry. © RSNA, 2021 Online supplemental material is available for this article.

Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis.

BACKGROUND: Complications from liver cirrhosis are a leading cause of death in children with cystic fibrosis. Identifying children at risk for developing liver cirrhosis and halting its progression are critical to reducing liver-associated mortality. OBJECTIVE: Quantitative US imaging, such as shear-wave elastography (SWE), might improve the detection of liver fibrosis in children with cystic fibrosis (CF) over gray-scale US alone. We incorporated SWE in our pediatric CF liver disease screening program and evaluated its performance using magnetic resonance (MR) elastography. MATERIALS AND METHODS: Ninety-four children and adolescents with CF underwent 178 SWE exams, aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT) and platelet measurements. Of these, 27 children underwent 34 MR elastography exams. We evaluated SWE performance using 6-MHz and 9-MHZ point SWE, and 9-MHz two-dimensional (2-D) SWE. RESULTS: The 6-MHz point SWE was the only method that correlated with MR elastography (r=0.52; 95% confidence interval [CI] 0.20-0.74; P=0.003). SWE of 1.45 m/s distinguished normal from abnormal MR elastography (79% sensitivity, 100% specificity, 100% positive predictive value [PPV], 55% negative predictive value [NPV], area under the receiver operating characteristic [AUROC] curve 0.94). SWE of 1.84 m/s separated mild-moderate (3.00-4.77 kPa) from severe (>4.77 kPa) MR elastography (88% sensitivity, 86% specificity, 78% PPV, 93% NPV, AUROC 0.79). Elevations of AST, ALT, GGT and thrombocytopenia were associated with higher SWE. AST-to-platelet ratio index of 0.42, fibrosis-4 of 0.29, and GGT-to-platelet ratio of 1.43 all had >95% NPV for SWE >1.84 m/s. CONCLUSION: Given its correlation with MR elastography, SWE might be a clinically useful predictor of liver fibrosis. We identified imaging criteria delineating the use of SWE to identify increased liver stiffness in children with CF. With multicenter validation, these data might be used to improve the detection and monitoring of liver fibrosis in children with CF.

Subsuns and rainbows during solar eclipses.

A photographic observation sequence was obtained of a subsun before, during, and after the total phase of the 2016 solar eclipse. The time-resolved images were obtained from a high-altitude jet aircraft. The image sequence was searched for the possible presence of a solar corona-generated subsun during totality. Although the subsun-creating conditions apparently persisted during totality, the drop in signal intensity compared to the local background prevented its detection. Separately, we document a visual observation from the 1977 total solar eclipse of a rainbow that faded, in the last a few seconds before totality, from being normally multicolored to monochromatic red from water drops then predominantly illuminated by light from the solar chromosphere. A similar transition in the final seconds before, and after, totality is expected to occur for parhelia. The posited short-living monochromatic red parhelion resulting from the momentary illumination of ice crystals by the solar chromosphere is still waiting to be observed.

Image-based scaling laws for somatic growth and pulmonary artery morphometry from infancy to adulthood.

Pulmonary artery (PA) morphometry has been extensively explored in adults, with particular focus on intra-acinar arteries. However, scaling law relationships for length and diameter of extensive preacinar PAs by age have not been previously reported for in vivo human data. To understand preacinar PA growth spanning children to adults, we performed morphometric analyses of all PAs visible in the computed tomography (CT) and magnetic resonance (MR) images from a healthy subject cohort [n = 16; age: 1-51 yr; body surface area (BSA): 0.49-2.01 m2]. Subject-specific anatomic PA models were constructed from CT and MR images, and morphometric information-diameter, length, tortuosity, bifurcation angle, and connectivity-was extracted and sorted into diameter-defined Strahler orders. Validation of Murray's law, describing optimal scaling exponents of radii for branching vessels, was performed to determine how closely PAs conform to this classical relationship. Using regression analyses of vessel diameters and lengths against orders and patient metrics (BSA, age, height), we found that diameters increased exponentially with order and allometrically with patient metrics. Length increased allometrically with patient metrics, albeit weakly. The average tortuosity index of all vessels was 0.026 ± 0.024, average bifurcation angle was 28.2 ± 15.1°, and average Murray's law exponent was 2.92 ± 1.07. We report a set of scaling laws for vessel diameter and length, along with other morphometric information. These provide an initial understanding of healthy structural preacinar PA development with age, which can be used for computational modeling studies and comparison with diseased PA anatomy.NEW & NOTEWORTHY Pulmonary artery (PA) morphometry studies to date have focused primarily on large arteries and intra-acinar arteries in either adults or children, neglecting preacinar arteries in both populations. Our study is the first to quantify in vivo preacinar PA morphometry changes spanning infants to adults. For preacinar arteries > 1 mm in diameter, we identify scaling laws for vessel diameters and lengths with patient metrics of growth and establish a healthy PA morphometry baseline for most preacinar PAs.

Rosette Trajectories Enable Ungated, Motion-Robust, Simultaneous Cardiac and Liver T2 * Iron Assessment.

BACKGROUND: Quantitative T2 * MRI is the standard of care for the assessment of iron overload. However, patient motion corrupts T2 * estimates. PURPOSE: To develop and evaluate a motion-robust, simultaneous cardiac and liver T2 * imaging approach using non-Cartesian, rosette sampling and a model-based reconstruction as compared to clinical-standard Cartesian MRI. STUDY TYPE: Prospective. PHANTOM/POPULATION: Six ferumoxytol-containing phantoms (26-288 µg/mL). Eight healthy subjects and 18 patients referred for clinically indicated iron overload assessment. FIELD STRENGTH/SEQUENCE: 1.5T, 2D Cartesian and rosette gradient echo (GRE) ASSESSMENT: GRE T2 * values were validated in ferumoxytol phantoms. In healthy subjects, test-retest and spatial coefficient of variation (CoV) analysis was performed during three breathing conditions. Cartesian and rosette T2 * were compared using correlation and Bland-Altman analysis. Images were rated by three experienced radiologists on a 5-point scale. STATISTICAL TESTS: Linear regression, analysis of variance (ANOVA), and paired Student's t-testing were used to compare reproducibility and variability metrics in Cartesian and rosette scans. The Wilcoxon rank test was used to assess reader score comparisons and reader reliability was measured using intraclass correlation analysis. RESULTS: Rosette R2* (1/T2 *) was linearly correlated with ferumoxytol concentration (r2 = 1.00) and not significantly different than Cartesian values (P = 0.16). During breath-holding, ungated rosette liver and heart T2 * had lower spatial CoV (liver: 18.4 ± 9.3% Cartesian, 8.8% ± 3.4% rosette, P = 0.02, heart: 37.7% ± 14.3% Cartesian, 13.4% ± 1.7% rosette, P = 0.001) and higher-quality scores (liver: 3.3 [3.0-3.6] Cartesian, 4.7 [4.1-4.9] rosette, P = 0.005, heart: 3.0 [2.3-3] Cartesian, 4.5 [3.8-5.0] rosette, P = 0.005) compared to Cartesian values. During free-breathing and failed breath-holding, Cartesian images had very poor to average image quality with significant artifacts, whereas rosette remained very good, with minimal artifacts (P = 0.001). DATA CONCLUSION: Rosette k-sampling with a model-based reconstruction offers a clinically useful motion-robust T2 * mapping approach for iron quantification. J. MAGN. RESON. IMAGING 2020;52:1688-1698.

Deep learning to automate Brasfield chest radiographic scoring for cystic fibrosis.

BACKGROUND: The aim of this study was to evaluate the hypothesis that a deep convolutional neural network (DCNN) model could facilitate automated Brasfield scoring of chest radiographs (CXRs) for patients with cystic fibrosis (CF), performing similarly to a pediatric radiologist. METHODS: All frontal/lateral chest radiographs (2058 exams) performed in CF patients at a single institution from January 2008-2018 were retrospectively identified, and ground-truth Brasfield scoring performed by a board-certified pediatric radiologist. 1858 exams (90.3%) were used to train and validate the DCNN model, while 200 exams (9.7%) were reserved for a test set. Five board-certified pediatric radiologists independently scored the test set according to the Brasfield method. DCNN model vs. radiologist performance was compared using Spearman correlation (ρ) as well as mean difference (MD), mean absolute difference (MAD), and root mean squared error (RMSE) estimation. RESULTS: For the total Brasfield score, ρ for the model-derived results computed pairwise with each radiologist's scores ranged from 0.79-0.83, compared to 0.85-0.90 for radiologist vs. radiologist scores. The MD between model estimates of the total Brasfield score and the average score of radiologists was -0.09. Based on MD, MAD, and RMSE, the model matched or exceeded radiologist performance for all subfeatures except air-trapping and large lesions. CONCLUSIONS: A DCNN model is promising for predicting CF Brasfield scores with accuracy similar to that of a pediatric radiologist.

Lumbar Spine MRI: Missed Opportunities for Abdominal Aortic Aneurysm Detection.

PURPOSE: The US Preventive Services Task Force (USPSTF) recommends 1-time sonographic screening for abdominal aortic aneurysms (AAAs) in male smokers ages 65-75 and other selected individuals in this age group based on risk factors. Patients in this age range are frequent utilizers of lumbar spine MRI, in which the abdominal aorta is typically fully imaged. The purpose of this study was to assess the potential detection rate of AAAs on lumbar spine MRI performed in the USPSTF screening age range with systematic aortic measurement and the frequency with which AAAs are currently reported in practice. MATERIALS AND METHODS: All consecutive lumbar spine MRI exams performed without contrast at a single academic tertiary care center over a 1-year period (4/1/2016-3/31/2017) in patients ages 65-75 were retrospectively reviewed. Maximal anteroposterior, and transverse dimensions of the abdominal aorta were measured using axial T2-weighted images, supplemented with sagittal T2-weighted images if assessment was limited by field-of-view or artifact. The detection rate of AAA, defined as dilation of the aorta to a diameter of ≥3 cm, size of AAAs detected, and frequency with which AAAs were reported, were assessed. Differences in aortic diameters and aneurysm detection rates between genders were compared with the unpaired 2-sample t test. RESULTS: Three hundred and ninety-five lumbar spine MRIs were reviewed, 240 (60.8%) in women and 155 (39.2%) in men, with mean ± standard deviation (SD) age of 70.2 ± 3.2 years. AAAs were detected in 38/395 (9.6%) cases, most (33/38, 86.8%) of which were <4 cm. Of these, only 4 (10.5%) were reported by the interpreting radiologist; 3/4 (75%) corresponded to aneurysms ≥4 cm. CONCLUSION: Lumbar spine MRI performed in the USPSTF AAA screening age range, especially in men, facilitates frequent detection of AAA when the aorta is systematically measured. However, in typical lumbar spine assessment, AAAs are often underreported, particularly for smaller aneurysms.

Fungal endocarditis resembling primary cardiac malignancy in a patient with B-cell ALL with culture confirmation.

Fungal endocarditis is a rare subtype of infective endocarditis that often presents with nonspecific symptoms in patients with complex medical histories, making diagnosis challenging. Patients with a history of ALL may present with congestive heart failure, chemo-induced cardiomyopathy, acute coronary syndrome, cardiac lymphomatous metastasis, or infections. We present the case of a patient with a history of ALL who presented with acute coronary syndrome and imaging concerning for primary cardiac lymphoma, when in fact the patient ended up suffering from culture proven fungal endocarditis.

Erratum: Author Correction: Human-machine partnership with artificial intelligence for chest radiograph diagnosis.

[This corrects the article DOI: 10.1038/s41746-019-0189-7.].

Human-machine partnership with artificial intelligence for chest radiograph diagnosis.

Human-in-the-loop (HITL) AI may enable an ideal symbiosis of human experts and AI models, harnessing the advantages of both while at the same time overcoming their respective limitations. The purpose of this study was to investigate a novel collective intelligence technology designed to amplify the diagnostic accuracy of networked human groups by forming real-time systems modeled on biological swarms. Using small groups of radiologists, the swarm-based technology was applied to the diagnosis of pneumonia on chest radiographs and compared against human experts alone, as well as two state-of-the-art deep learning AI models. Our work demonstrates that both the swarm-based technology and deep-learning technology achieved superior diagnostic accuracy than the human experts alone. Our work further demonstrates that when used in combination, the swarm-based technology and deep-learning technology outperformed either method alone. The superior diagnostic accuracy of the combined HITL AI solution compared to radiologists and AI alone has broad implications for the surging clinical AI deployment and implementation strategies in future practice.

Cardiac MRI-guided interventional occlusion of ventricular septal rupture in a patient with cobalt alloy stent.

The case of a 68-year-old man with chest pain for 3 days is presented. Coronary angiography demonstrated subtotal occlusion of the mid-left anterior descending artery. A drug-eluting cobalt alloy stent was implanted after balloon dilation. On the 3rd postoperative day, echocardiography showed a ventricular septal rupture (VSR) (7 mm diameter) near the cardiac apex and ventricular aneurysm. On cardiac magnetic resonance imaging (MRI), the VSR was shown to be 11 mm in diameter. The membranous septum was 32 and 27.8 mm along the anteroposterior and superoinferior axes, respectively. The left-to-right shunt was apparent. Four weeks later, interventional therapy was performed to occlude the VSR according to the result of the MRI. The symptoms improved rapidly, and the patient was discharged. At the 4-month follow up visit, cardiac MRI revealed no shunt at the occlusion site, and the edge of the occluder was secured in the adjacent normal cardiac tissues. In conclusion, cardiac MRI could be considered for patients with a newly implanted cobalt alloy stent to provide an accurate assessment of VSR.

Cross-sectional imaging of congenital pulmonary artery anomalies.

Congenital pulmonary artery (PA) anomalies comprise a rare and heterogeneous spectrum of disease, ranging from abnormal origins to complete atresia. They may present in early infancy or more insidiously in adulthood, often in association with congenital heart disease such as tetralogy of Fallot or other syndromes. In recent years, cross-sectional imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), has become widely utilized for the noninvasive assessment of congenital PA diseases, supplementing echocardiography and at times supplanting invasive angiography. In this article, modern CT and MRI techniques for imaging congenital PA disorders are summarized. The key clinical features, cross-sectional imaging findings, and treatment options for the most commonly encountered entities are then reviewed. Emphasis is placed on the ever-growing role of cross-sectional imaging options in facilitating early and accurate diagnosis and tailored treatment.