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Patients with many forms of congenital heart disease (CHD) and hypertrophic cardiomyopathy undergo surgical intervention to relieve left ventricular outflow tract obstruction (LVOTO). Cardiovascular Computed Tomography (CCT) defines the complex pathway from the ventricle to the outflow tract and can be visualized in 2D, 3D, and 4D (3D in motion) to help define the mechanism and physiologic significance of obstruction. Advanced cardiac visualization may aid in surgical planning to relieve obstruction in the left ventricular outflow tract, aortic or neo-aortic valve and the supravalvular space. CCT scanner technology has advanced to achieve submillimeter, isotropic spatial resolution, temporal resolution as low as 66 msec allowing high-resolution imaging even at the fast heart rates and small cardiac structures of pediatric patients ECG gating techniques allow radiation exposure to be targeted to a minimal portion of the cardiac cycle for anatomic imaging, and pulse modulation allows cine imaging with a fraction of radiation given during most of the cardiac cycle, thus reducing radiation dose. Scanning is performed in a single heartbeat or breath hold, minimizing the need for anesthesia or sedation, for which CHD patents are highest risk for an adverse event. Examples of visualization of complex left ventricular outflow tract obstruction in the subaortic, valvar and supravalvular space will be highlighted, illustrating the novel applications of CCT in this patient subset. [ABSTRACT FROM AUTHOR]

This consensus document for the performance of Cardiovascular Computed Tomography (CCT) to guide intervention in the right ventricular outflow tract (RVOT) in patients with congenital disease (CHD) was developed collaboratively by pediatric and adult interventionalists, surgeons and cardiac imagers with expertise specific to this patient subset. The document summarizes definitions of RVOT dysfunction as assessed by multi-modality imaging techniques and reviews existing consensus statements and guideline documents pertaining to indications for intervention. In the context of this background information, recommendations for CCT scan acquisition and a standardized approach for reporting prior to surgical or transcatheter pulmonary valve replacement are proposed and presented. It is the first Imaging for Intervention collaboration for CHD patients and encompasses imaging and reporting recommendations prior to both surgical and percutaneous pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

Cardiovascular computed tomography (CCT) is rated appropriate by published guidelines for the initial evaluation and follow up of congenital heart disease (CHD) and is an essential modality in cardiac imaging programs for patients of all ages. However, no recommended core competencies exist to guide CCT in CHD imaging training pathways, curricula development, or establishment of a more formal educational platform. To fill this gap, a group of experienced congenital cardiac imagers, intentionally inclusive of adult and pediatric cardiologists and radiologists, was formed to propose core competencies fundamental to the expert-level performance of CCT in pediatric acquired and congenital heart disease and adult CHD. The 2020 SCCT Guideline for Training Cardiology and Radiology Trainees as Independent Practitioners (Level II) and Advanced Practitioners (Level III) in Cardiovascular Computed Tomography (1) for adult imaging were used as a framework to define pediatric and CHD-specific competencies. Established competencies will be immediately relevant for advanced cardiac imaging fellowships in both cardiology and radiology training pathways. Proposed future steps include radiology and cardiology society collaboration to establish provider certification levels, training case-volume recommendations, and continuing medical education (CME) requirements for expert-level performance of CCT in pediatric and adult CHD. [ABSTRACT FROM AUTHOR]

Cardiac computed tomography (CCT) has increasingly been used in the assessment of both children and adults with congenital heart disease (CHD), in part due to advances in CCT technology and an increased prevalence of adults with palliated CHD. It serves as a complimentary modality to echocardiography, cardiac magnetic resonance imaging and cardiac catheterization. CCT can provide unique diagnostic information, is less invasive and less likely to require sedation compared to other modalities. Detailed knowledge of individual patient cardiac anatomy, physiology, surgical repair and possible residual lesions are paramount to optimal CCT imaging. This comprehensive review details the use of CCT both pre- and postoperatively for the most common CHD diagnoses. We also aim to highlight some new and innovative technologies that have become available and can further optimize CCT imaging for CHD patients. [ABSTRACT FROM AUTHOR]

Cardiac computed tomography (CT) is increasingly used in pediatric patients with congenital heart disease (CHD). Variability of practice and of comprehensive diagnostic risk across institutions is not known. Four centers prospectively enrolled consecutive pediatric CHD patients <18 years of age undergoing cardiac CT from January 6, 2017 to 1/30/2020. Patient characteristics, cardiac CT data and comprehensive diagnostic risk were compared by age and institutions. Risk categories included sedation and anesthesia use, vascular access, contrast exposure, cardiovascular medication, adverse events (AEs), and estimated radiation dose. Cardiac CT was performed in 1045 pediatric patients at a median (interquartile range, IQR) age of 1.7 years (0.3, 11.0). The most common indications were arterial abnormalities, suspected coronary artery anomalies, functionally single ventricle heart disease, and tetralogy of Fallot/pulmonary atresia. Sedation was used in 8% and anesthesia in 11% of patients. Peripheral vascular access was utilized for 93%. Median contrast volume was 2 ​ml/kg. Beta blockers were administered in 11% of cases and nitroglycerin in 2% of cases. The median (IQR) total procedural dose length product (DLP) was 20 ​mGy∗cm (10, 50). Sedation, vascular access, contrast exposure, use of cardiovascular medications and radiation dose estimates varied significantly by institution and age (p ​< ​0.001). Seven minor adverse events (0.7%) and no major adverse events were reported. Cardiac CT for CHD is safe in pediatric patients when appropriate CT technology and expertise are available. Scans can be acquired at relatively low radiation exposure with few minor adverse events. We conducted a prospective multi-institutional evaluation of variability of practice and comprehensive diagnostic risk for cardiac CT in pediatric CHD patients. 1329 scans were performed in 1045 patients at four institutions from January 6, 2017 to 1/30/2020 in patients from 1 day to <18 years of age. Anesthesia and sedation were required in a minority of cases, with a very low rate of adverse events and low radiation exposure (median procedural DLP 20 ​mGy∗cm). However, variation exists between institutions, warranting future exploration and quality improvement initiatives to develop best practice guidelines and reduced practice variability. [ABSTRACT FROM AUTHOR]

The impact of the coronavirus disease (COVID-19) pandemic in the United States and around the world has required significant changes to medical practice. Amidst the rapidly evolving public health emergency, hospital centers have been required to postpone elective procedures, preserve personal protective equipment (PPE), practice social distancing and limit staff exposures. Patients with congenital heart disease (CHD) often need urgent evaluation, most commonly for preprocedural evaluation. We have stratified the most common indications for cardiac computed tomography (CCT) imaging in patients with CHD to help guide care for these patients during the COVID-19 pandemic including considerations for reopening. [ABSTRACT FROM AUTHOR]

Background The cumulative exposure and risk of anesthesia, vascular access, contrast agents and radiation is emerging as a significant lifelong burden in patients with congenital heart disease (CHD). Latest generation computerized tomographic (CT) scanners are increasingly used for high resolution cardiovascular imaging and have both hardware and post processing radiation dose reduction strategies that can be implemented. Currently, these dose reduction strategies are not uniformly applied and there is a large variability in radiation dose used for the performance of CT in CHD. Methods We propose the development and implementation of a prospective, multi-center and multi-specialty consortium to measure the variability of use, risk and image quality of CT scans in patients of all ages with CHD. The primary goals of this collaboration are 1) define variability of use, diagnostic quality, and risk of cardiac CT 2) establish best practice guidelines designed to optimize diagnostic image quality with appropriate use of radiation and anesthesia exposure 3) provide institution specific feedback compared with the group norm across participating centers 4) improve the level of evidence for the use of CT in CHD through the collection of prospective and multi-institutional data. Conclusions Prospective multi-institutional data is needed to inform risk estimates of CT in CHD using current generation scanners and aggressive dose optimization techniques. This registry will provide a platform for future collaboration establishing a multi-modality risk assessment tool specific to patients with CHD. [ABSTRACT FROM AUTHOR]

Children with congenital or acquired heart disease can be exposed to relatively high lifetime cumulative doses of ionizing radiation from necessary medical imaging procedures including radiography, fluoroscopic procedures including diagnostic and interventional cardiac catheterizations, electrophysiology examinations, cardiac computed tomography (CT) studies, and nuclear cardiology examinations. Despite the clinical necessity of these imaging studies, the related ionizing radiation exposure could pose an increased lifetime attributable cancer risk. The Image Gently "Have-A-Heart" campaign is promoting the appropriate use of medical imaging studies in children with congenital or acquired heart disease while minimizing radiation exposure. The focus of this manuscript is to provide a comprehensive review of radiation dose management and CT performance in children with congenital or acquired heart disease. [ABSTRACT FROM AUTHOR]

Background The use of cardiac computed tomography (CT) for quantification of ventricular function is limited by relatively high radiation dose. Objectives The goal of this study was to describe the radiation exposure and image quality of 70 kVp functional cardiac CT in patients with congenital heart disease (CHD). Methods A retrospective review of 70 kVp ECG gated functional CT scans using tube current modulation was performed in CHD patients at a single institution. Quantitative and qualitative (assessed by myocardial segment, 1 – 4; 1 = optimal) image quality was determined. Per segment image quality was compared between thin (1.5 mm) and thick (8 mm) reconstructions and by patient age and size. Scan DLP was used to estimate radiation dose. Results 72 scans were performed during the time of review (7/2013-6/2015). Median patient age was 19.5 years (8.0, 27.1) and BMI was 20.1 (16.6, 24.5) kg/m 2 . Median functional scan DLP was 78.8 (45.5, 98) and unadjusted and adjusted procedural mSv were 1.10 (0.64, 1.37) and 1.13 (0.90, 1.37). Image quality of 1 was achieved in all myocardial segments in >75% of scans. Patients with a weight ≥75 kg were more likely to have a scan achieve optimal image when using thick reconstructions compared to thin (81.3% vs. 43.8%; p = 0.028). Conclusions Imaging of ventricular function with 70 kVp in CHD patients can be done with low radiation doses and provides diagnostic image quality, particularly for patients <75 kg. In larger patients, thicker slice reconstruction improved image quality. [ABSTRACT FROM AUTHOR]

This is an expert consensus document created to provide information about the current use of cardiovascular computed tomography (CT) in patients of all ages with proven or suspected congenital heart disease (CHD). The discussion and recommendations are based on available literature and the judgment of a diverse group of subspecialists with extensive experience in the use of CT imaging in CHD. The field of CHD CT imaging is evolving rapidly with the availability of new scanner technology. In addition, the prevalence of palliated CHD has increased with marked improvements in patient survival. We believe it is important to review the clinical indications, strengths, limitations, and risks of cardiovascular CT in this patient population. This is the first of two complementary documents. It will concentrate on the disease entities and circumstances in which CT may be used. The second document will focus on recommendations for the technical performance of cardiovascular CT in patients with CHD. Successful cardiovascular CT imaging of CHD requires an in depth understanding of the core teaching elements of both cardiology and radiology. The ability to perform and interpret high quality congenital cardiovascular CT in a clinical context requires focused time and effort regardless of the previous background of the cardiac imager. This is reflected by a writing committee that consists of pediatric and adult radiologists and cardiologists, all whom have extensive experience in performing CT in this patient population. Cardiovascular CT is complementary to other imaging modalities and its optimal use will be in centers where all diagnostic modalities are available. The choice of modality for an individual patient should be determined by age, diagnosis, clinical condition, clinical question and patient preference. 1–4 Use of CT in CHD should be reserved for situations in which it is expected to provide unique diagnostic information for the individual patient or clinical indication, and/or less risk than other modalities. This multi-disciplinary document is intended to guide the optimal selection of CHD patients for cardiovascular CT. The goals of this document apply to both pediatric and adult CHD patients and are to: 1) Review the current use of cardiovascular CT. 2) Assess the most up to date information on risks, benefits, as well as limitations of cardiovascular CT. 3) Provide disease-specific indications for cardiovascular CT imaging. 4) Outline a consensus opinion on the essential skills and knowledge needed to optimally perform and interpret cardiovascular CT. [ABSTRACT FROM AUTHOR]

Abstract: Background: Cardiac magnetic resonance imaging (MRI) and CT are available in the recent era at many pediatric cardiac centers. Objective: The aim was to provide a contemporary description of diagnostic imaging trends for definition of congenital heart disease (CHD). Methods: Echocardiography, cardiac catheterization, cardiac MRI, and cardiac CT use in patients with congenital heart disease at a single institution was retrospectively recorded (2005–2012). Surgical procedures were recorded. Total and modality-specific rates were estimated by Poisson regression and compared. The median age, studies in patients aged >17 years, and referral diagnosis were tabulated for the last year of review. Results: An average of 11,940 cardiovascular diagnostic tests was performed annually. The number of total studies, echocardiograms, catheterizations, and surgical procedures, did not change significantly across time. Echocardiography comprised 95% to 97% of all studies performed during each year of review. The use of cardiac MRI (2%) and cardiac CT (1%) increased linearly (P < .001), and the use of diagnostic catheterization decreased (0.7%; P = .0005). The median age was 3 years for echocardiography, 15 years for MRI, 11 years for CT, and 3 years for catheterization. The percentage of patients aged >17 years was 9% for echocardiography, 33% for cardiac MRI, 29% for cardiac CT, and 8% for catheterization. Most patients undergoing CT, MRI, and diagnostic catheterization had moderate or complex CHD. Conclusion: Cardiac CT is used increasingly in the recent era for evaluation of CHD. The increased use of both cardiac CT and cardiac MRI are temporally associated with a decrease in diagnostic cardiac catheterization. [Copyright &y& Elsevier]

Abstract: Background: Neonates with complex congenital heart disease (CHD) are at risk of adverse events from anesthesia. CT angiography (CTA) performed free breathing and without sedation has not been reported for evaluation of complex CHD in neonates. Objectives: The aim was to evaluate the image quality and risk of free breathing, non-sedated cardiac CTA for definition of CHD in the neonatal period and to determine accuracy compared with interventional findings. Methods: This is a combined retrospective–prospective single institution review of all non-sedated, free breathing cardiac CT angiograms performed in patients <1 month of age with complex CHD. Diagnosis, scan acquisition parameters, image quality (1- to 4-point scale), adverse events, radiation dose estimates, and accuracy compared with operative and interventional catheterization findings were recorded. Results are reported as median and interquartile range. Results: Nineteen non-sedated, free breathing, neonatal cardiac CT angiograms were performed during the time of review. All studies were diagnostic with a mean image quality score of 1.1 ± 0.3. Median total procedural dose-length product was 11 (range, 10–14), CT dose index volume was 0.47 (range, 0.31–0.5). Median unadjusted radiation dose was 0.15 mSv (range, 0.14–0.2 mSv), age- and size-adjusted radiation dose was 0.86 mSv (range, 0.78–1.1 mSv). No adverse events and no discrepancies compared with surgical or catheterization findings were found in the 17 of 19 patients that had subsequent intervention. Conclusions: Cardiac CTA can be performed in the neonatal period free breathing and without sedation. Image quality is excellent, and there is high accuracy compared with surgical and catheterization findings at the time of intervention. [Copyright &y& Elsevier]

Abstract: Echocardiography and cardiac magnetic resonance imaging are the most commonly performed diagnostic studies in patients with congenital heart disease. A small percentage of patients with congenital heart disease will be referred to cardiac CT subsequent to echocardiography when magnetic resonance imaging is insufficient, contraindicated, or considered high risk. The most common complex lesions referred for CT at our institution are tetralogy of Fallot, transposition complexes, and single ventricle heart disease. This review discusses the most common surgical procedures performed in these patients and the technical considerations for optimal image acquisition on the basis of the prior procedure and the individual patient history. Cardiac CT can provide the functional and anatomic information required for decision making in complex congenital heart disease. Image interpretation is aided by knowledge of the common approaches to operative repair and the residual hemodynamic abnormalities. Acquisition and interpretation that is both individualized to the patient's underlying disease and the specific clinical question is likely to maintain diagnostic accuracy while decreasing the potential risk of cardiac CT. [Copyright &y& Elsevier]

Background: Pediatric cardiac patients often undergo repeat diagnostic testing, resulting in relatively high cumulative medical radiation exposure. Low-dose CT scanning techniques used to decrease radiation exposure may result in reduced image quality. Objective: This study evaluates a prototype iterative reconstruction algorithm, sinogram-affirmed iterative reconstruction (SAFIRE), to determine the effect on qualitative and quantitative measures of image quality in pediatric cardiac CT datasets, compared with a standard weighted filtered back projection (wFBP) algorithm. Methods: Seventy-four datasets obtained on a 128-slice dual-source CT system were evaluated for image quality using both the wFBP and the prototype iterative reconstruction algorithm. Contrast, noise, contrast-to-noise ratio, signal-to-noise ratio, and qualitative image quality were compared between groups. Data were analyzed as medians and 25th and 75th percentiles, and groups were compared with the use of the Wilcoxon singed-rank test or k sample equality of medians test. Results: There was a 34% decrease in noise, a 41% increase in contrast-to-noise ratio, and a 56% increase in signal-to-noise ratio in the prototype iterative reconstruction, compared with wFBP. All differences were statistically significant (P < 0.001). Qualitative measures of image noise and noise texture were also improved in the iterative reconstruction group (P < 0.001 for both). Diagnostic confidence was similar between reconstruction techniques. Median scan dose length product was 15.5 mGy · cm. Conclusion: The prototype iterative reconstruction algorithm studied significantly reduces image noise and improves qualitative and quantitative measures of image quality in low-dose pediatric CT datasets, compared with standard wFBP. [ABSTRACT FROM AUTHOR]

Recent years have witnessed exponential growth in cardiac imaging technologies, allowing better visualization of complex cardiac anatomy and improved assessment of physiology. These advances have become increasingly important as more complex surgical and catheter-based procedures are evolving to address the needs of a growing congenital heart disease population. This state-of-the-art review presents advances in echocardiography, cardiac magnetic resonance, cardiac computed tomography, invasive angiography, 3-dimensional modeling, and digital twin technology. The paper also highlights the integration of artificial intelligence with imaging technology. While some techniques are in their infancy and need further refinement, others have found their way into clinical workflow at well-resourced centers. Studies to evaluate the clinical value and cost-effectiveness of these techniques are needed. For techniques that enhance the value of care for congenital heart disease patients, resources will need to be allocated for education and training to promote widespread implementation. [Display omitted] • Cardiac imaging plays a crucial role in the evaluation and management of patients with congenital heart disease. • Advancements in cardiac imaging techniques will likely find rapid clinical application in the assessment of patients with congenital heart disease. • Strategies to assess clinical value and resources for training are needed before widespread implementation. [ABSTRACT FROM AUTHOR]