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Pediatric heart disease is a large and diverse field with an overall prevalence estimated at 6 to 13 per 1,000 live births. This document discusses appropriateness of advanced imaging for a broad range of variants. Diseases covered include tetralogy of Fallot, transposition of great arteries, congenital or acquired pediatric coronary artery abnormality, single ventricle, aortopathy, anomalous pulmonary venous return, aortopathy and aortic coarctation, with indications for advanced imaging spanning the entire natural history of the disease in children and adults, including initial diagnosis, treatment planning, treatment monitoring, and early detection of complications. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation., (Copyright © 2023 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

Background and Aims: Anthracyclines can cause cancer therapy-related cardiac dysfunction (CTRCD). We aimed to assess whether statins prevent decline in left ventricular ejection fraction (LVEF) in anthracycline-treated patients at increased risk for CTRCD., Methods: In this multicenter double-blinded, placebo-controlled trial, patients with cancer at increased risk of anthracycline-related CTRCD (per ASCO guidelines) were randomly assigned to atorvastatin 40 mg or placebo once-daily. Cardiovascular magnetic resonance (CMR) imaging was performed before and within 4 weeks after anthracyclines. Blood biomarkers were measured at every cycle. The primary outcome was post-anthracycline LVEF, adjusted for baseline. CTRCD was defined as a fall in LVEF by >10% to <53%. Secondary endpoints included left ventricular (LV) volumes, CTRCD, CMR tissue characterization, high sensitivity troponin I (hsTnI), and B-type natriuretic peptide (BNP)., Results: We randomized 112 patients (56.9 ± 13.6 years, 87 female, and 73 with breast cancer): 54 to atorvastatin and 58 to placebo. Post-anthracycline CMR was performed 22 (13-27) days from last anthracycline dose. Post-anthracycline LVEF did not differ between the atorvastatin and placebo groups (57.3 ± 5.8% and 55.9 ± 7.4%, respectively) when adjusted for baseline LVEF (P = 0.34). There were no significant between-group differences in post-anthracycline LV end-diastolic (P = 0.20) or end-systolic volume (P = 0.12), CMR myocardial edema and/or fibrosis (P = 0.06-0.47), or peak hsTnI (P ≥ 0.99) and BNP (P = 0.23). CTRCD incidence was similar (4% versus 4%, P ≥ 0.99). There was no difference in adverse events., Conclusions: In patients at increased risk of CTRCD, primary prevention with atorvastatin during anthracycline therapy did not ameliorate early LVEF decline, LV remodeling, CTRCD, change in serum cardiac biomarkers, or CMR myocardial tissue changes., Trial Registration: NCT03186404., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Importance: There is a growing interest in understanding whether cardiovascular magnetic resonance (CMR) myocardial tissue characterization helps identify risk of cancer therapy-related cardiac dysfunction (CTRCD)., Objective: To describe changes in CMR tissue biomarkers during breast cancer therapy and their association with CTRCD., Design, Setting, and Participants: This was a prospective, multicenter, cohort study of women with ERBB2 (formerly HER2)-positive breast cancer (stages I-III) who were scheduled to receive anthracycline and trastuzumab therapy with/without adjuvant radiotherapy and surgery. From November 7, 2013, to January 16, 2019, participants were recruited from 3 University of Toronto-affiliated hospitals. Data were analyzed from July 2021 to June 2022., Exposures: Sequential therapy with anthracyclines, trastuzumab, and radiation., Main Outcomes and Measures: CMR, high-sensitivity cardiac troponin I (hs-cTnI), and B-type natriuretic peptide (BNP) measurements were performed before anthracycline treatment, after anthracycline and before trastuzumab treatment, and at 3-month intervals during trastuzumab therapy. CMR included left ventricular (LV) volumes, LV ejection fraction (EF), myocardial strain, early gadolinium enhancement imaging to assess hyperemia (inflammation marker), native/postcontrast T1 mapping (with extracellular volume fraction [ECV]) to assess edema and/or fibrosis, T2 mapping to assess edema, and late gadolinium enhancement (LGE) to assess replacement fibrosis. CTRCD was defined using the Cardiac Review and Evaluation Committee criteria. Fixed-effects models or generalized estimating equations were used in analyses., Results: Of 136 women (mean [SD] age, 51.1 [9.2] years) recruited from 2013 to 2019, 37 (27%) developed CTRCD. Compared with baseline, tissue biomarkers of myocardial hyperemia and edema peaked after anthracycline therapy or 3 months after trastuzumab initiation as demonstrated by an increase in mean (SD) relative myocardial enhancement (baseline, 46.3% [16.8%] to peak, 56.2% [18.6%]), native T1 (1012 [26] milliseconds to 1035 [28] milliseconds), T2 (51.4 [2.2] milliseconds to 52.6 [2.2] milliseconds), and ECV (25.2% [2.4%] to 26.8% [2.7%]), with P <.001 for the entire follow-up. The observed values were mostly within the normal range, and the changes were small and recovered during follow-up. No new replacement fibrosis developed. Increase in T1, T2, and/or ECV was associated with increased ventricular volumes and BNP but not hs-cTnI level. None of the CMR tissue biomarkers were associated with changes in LVEF or myocardial strain. Change in ECV was associated with concurrent and subsequent CTRCD, but there was significant overlap between patients with and without CTRCD., Conclusions and Relevance: In women with ERBB2-positive breast cancer receiving sequential anthracycline and trastuzumab therapy, CMR tissue biomarkers suggest inflammation and edema peaking early during therapy and were associated with ventricular remodeling and BNP elevation. However, the increases in CMR biomarkers were transient, were not associated with LVEF or myocardial strain, and were not useful in identifying traditional CTRCD risk.

Coronavirus disease 2019 (COVID-19) is an ongoing global pandemic that has affected nearly 600 million people to date across the world. While COVID-19 is primarily a respiratory illness, cardiac injury is also known to occur. Cardiovascular magnetic resonance (CMR) imaging is uniquely capable of characterizing myocardial tissue properties in-vivo, enabling insights into the pattern and degree of cardiac injury. The reported prevalence of myocardial involvement identified by CMR in the context of COVID-19 infection among previously hospitalized patients ranges from 26 to 60%. Variations in the reported prevalence of myocardial involvement may result from differing patient populations (e.g. differences in severity of illness) and the varying intervals between acute infection and CMR evaluation. Standardized methodologies in image acquisition, analysis, interpretation, and reporting of CMR abnormalities across would likely improve concordance between studies. This consensus document by the Society for Cardiovascular Magnetic Resonance (SCMR) provides recommendations on CMR imaging and reporting metrics towards the goal of improved standardization and uniform data acquisition and analytic approaches when performing CMR in patients with COVID-19 infection., (© 2023. The Author(s).)

Dyspnea is the symptom of perceived breathing discomfort and is commonly encountered in a variety of clinical settings. Cardiac etiologies of dyspnea are an important consideration; among these, valvular heart disease (Variant 1), arrhythmia (Variant 2), and pericardial disease (Variant 3) are reviewed in this document. Imaging plays an important role in the clinical assessment of these suspected abnormalities, with usually appropriate procedures including resting transthoracic echocardiography in all three variants, radiography for Variants 1 and 3, MRI heart function and morphology in Variants 2 and 3, and CT heart function and morphology with intravenous contrast for Variant 3. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment., (Copyright © 2022 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

Background: Although insufficient maternal cardiac output (CO) has been implicated in poor outcomes in mothers with heart disease (HD), maternal-fetal interactions remain incompletely understood. We sought to quantify maternal-fetal hemodynamics with the use of magnetic resonance imaging (MRI) and explore their relationship with adverse events., Methods: Pregnant women with moderate or severe HD (n = 22; mean age 32 ± 5 years) were compared with healthy control women (n = 21; 34 ± 3 years). An MRI was performed during the third trimester at peak output (maternal-fetal) and 6 months postpartum with return of maternal hemodynamics to baseline (reference). Phase-contrast MRI was used for flow quantification and was combined with T1/T2 relaxometry for derivation of fetal oxygen delivery/consumption., Results: Third-trimester CO and cardiac index (CI) measurements were similar in HD and control groups (CO 7.2 ± 1.5 vs 7.3 ± 1.6 L/min, P = 0.79; CI 4.0 ± 0.7 vs 4.3 ± 0.7 L/min/m, 2 P = 0.28). However, the magnitude of CO/CI increase (Δ, peak pregnancy - reference) in the HD group exceeded that in the control group (CO 46 ± 24% vs 27 ± 16% [P = 0.007]; CI 51 ± 28% vs 28 ± 17% [P = 0.005]). Fetal growth and oxygen delivery/consumption were similar between groups. Adverse cardiovascular outcomes (nonmutually exclusive) in 6 HD women included arrhythmia (n = 4), heart failure (n = 2), and hypertensive disorder of pregnancy (n = 1); premature delivery was observed in 2 of these women. The odds of a maternal cardiovascular event were inversely associated with peak CI (odds ratio 0.10, 95% confidence interval 0.001-0.86; P = 0.04) and Δ,CI (0.02, 0.001-0.71; P = 0.03)., Conclusions: Maternal-fetal hemodynamics can be well characterised in pregnancy with the use of MRI. Impaired adaptation to pregnancy in women with HD appears to be associated with development of adverse outcomes of pregnancy., (Copyright © 2021 Canadian Cardiovascular Society. All rights reserved.)

Nonischemic cardiomyopathies encompass a broad spectrum of myocardial disorders with mechanical or electrical dysfunction without evidence of ischemia. There are five broad variants of nonischemic cardiomyopathies; hypertrophic cardiomyopathy (Variant 1), restrictive or infiltrative cardiomyopathy (Variant 2), dilated or unclassified cardiomyopathy (Variant 3), arrhythmogenic cardiomyopathy (Variant 4), and inflammatory cardiomyopathy (Variant 5). For variants 1, 3, and 4, resting transthoracic echocardiography, MRI heart function and morphology without and with contrast, and MRI heart function and morphology without contrast are the usually appropriate imaging modalities. For variants 2 and 5, resting transthoracic echocardiography and MRI heart function and morphology without and with contrast are the usually appropriate imaging modalities. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment., (Copyright © 2021 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

OBJECTIVE . Cardiac involvement is the leading cause of mortality in Fabry disease. Noninvasive markers of cardiac involvement are needed to identify patients at high risk. The purpose of this study was to evaluate the diagnostic potential of segmental native T1 spread as an imaging biomarker in Fabry disease. SUBJECTS AND METHODS. In this prospective study, 43 patients with confirmed Fabry disease (mean ± SD age, 46±14 years; 70% women) and 17 healthy control subjects (mean ± SD age, 44 ±13 years; 53% women) underwent 3-T cardiac MRI including modified Look-Locker inversion recovery T1 mapping. Segmental native T1 spread was calculated as the difference between maximum and minimum segmental native T1 values, expressed as an absolute value and as a relative percentage of global native T1. RESULTS. Absolute and relative segmental native T1 spreads were significantly higher in patients with Fabry disease than in healthy control subjects (absolute median, 115 vs 98 ms [ p = 0.004]; relative median, 9.9% vs 8.0% [ p < 0.001]) and correlated positively with quantitative late gadolinium enhancement (absolute, r = 0.434, p < 0.001; relative, r = 0.436, p < 0.001), indexed left ventricular mass (absolute, r = 0.316, p = 0.01; relative, r = 0.347, p = 0.007), and global longitudinal strain (absolute, r = 0.289, p = 0.03; relative, r = 0.277, p = 0.03). Relative segmental native T1 spread differentiated patients with Fabry disease from healthy control subjects (odds ratio, 1.44 [95% CI, 1.10-1.89]; p = 0.009). Interob-server agreement was excellent for both absolute (intraclass correlation coefficient, 0.932) and relative (intraclass correlation coefficient, 0.926) segmental native T1 spread. CONCLUSION. Increased native T1 spread is a reproducible imaging biomarker of cardiac involvement in Fabry disease and may be particularly useful in the evaluation of patients who cannot undergo late gadolinium enhancement imaging.

Purpose: To evaluate the diagnostic utility of the Look Locker inversion time (TI) sequence on cardiac magnetic resonance imaging in patients with suspected cardiac amyloidosis and to evaluate whether there are differences in the nulling pattern between amyloid types., Materials and Methods: A total of 144 patients with suspected cardiac amyloidosis who had undergone cardiac magnetic resonance imaging were included in this retrospective study. Sixty-four had cardiac amyloidosis (62.1±9.2 y, 70.3% male, 68.8% had light chain amyloid [AL], 18.8% had familial transthyretin amyloid caused by mutant genes [ATTRm], and 12.5% had wild-type transthyretin amyloid [ATTRwt]) and 80 did not have cardiac amyloidosis (61.3±13.3 y, 58.8% male). Time to myocardial and blood pool nulling on the Look Locker TI sequence was classified as normal if blood pool nulled before myocardium or abnormal if blood pool nulling was coincident with or after myocardial nulling., Results: The nulling pattern was abnormal in 26 patients with cardiac amyloidosis compared with none of the patients without cardiac amyloidosis (40.6% vs. 0.0%, P<0.0001). Abnormal nulling had 40.6% sensitivity and 100% specificity for cardiac amyloidosis (area under the receiver operating characteristic curve: 0.703, 95% confidence interval: 0.642-0.764). All patients with cardiac amyloidosis with an abnormal nulling pattern demonstrated late gadolinium enhancement. Among patients with cardiac amyloidosis, there was no significant difference in abnormal nulling between AL, ATTRm, and ATTRwt amyloid types (31.8%, 58.3%, 62.5%, respectively, P=0.10)., Conclusions: An abnormal nulling pattern on the Look Locker TI sequence is highly specific for cardiac amyloidosis when present. However, abnormal nulling is a late finding with low sensitivity and does not differentiate between amyloid types.

Background: Calculation of cardiovascular magnetic resonance (CMR) extracellular volume (ECV) requires input of hematocrit, which may not be readily available. The purpose of this study was to evaluate the diagnostic accuracy of ECV calculated using various noninvasive measures of hematocrit compared to ECV calculated with input of laboratory hematocrit as the reference standard., Methods: One hundred twenty three subjects (47.7 ± 14.1 years; 42% male) were prospectively recruited for CMR T1 mapping between August 2016 and April 2017. Laboratory hematocrit was assessed by venipuncture. Noninvasive hematocrit was assessed with a point-of-care (POC) device (Pronto-7 ® Pulse CO-Oximeter ® , Masimo Personal Health, Irvine, California, USA) and by synthetic derivation based on the relationship with blood pool T1 values. Left ventricular ECV was calculated with input of laboratory hematocrit (Lab-ECV), POC hematocrit (POC-ECV), and synthetic hematocrit (synthetic-ECV), respectively. Statistical analysis included Wilcoxon signed-rank test, Bland-Altman analysis, receiver-operating curve analysis and intra-class correlation (ICC)., Results: There was no significant difference between Lab-ECV and POC-ECV (27.1 ± 4.7% vs. 27.3 ± 4.8%, p = 0.106), with minimal bias and modest precision (bias - 0.18%, 95%CI [- 2.85, 2.49]). There was no significant difference between Lab-ECV and synthetic-ECV (26.7 ± 4.4% vs. 26.5 ± 4.3%, p = 0.084) in subjects imaged at 1.5 T, although bias was slightly higher and limits of agreement were wider (bias 0.23%, 95%CI [- 2.82, 3.27]). For discrimination of abnormal Lab-ECV ≥30%, POC-ECV had good diagnostic performance (sensitivity 85%, specificity 96%, accuracy 94%, and AUC 0.902) and synthetic-ECV had moderate diagnostic performance (sensitivity 71%, specificity 98%, accuracy 93%, and AUC 0.849). POC-ECV had excellent test-retest (ICC 0.994, 95%CI[0.987, 0.997]) and inter-observer agreement (ICC 0.974, 95%CI[0.929, 0.991])., Conclusions: Myocardial ECV can be accurately and reproducibly calculated with input of hematocrit measured using a noninvasive POC device, potentially overcoming an important barrier to implementation of ECV. Further evaluation of synthetic ECV is required prior to clinical implementation.

Non-electrocardiographic (ECG)-gated computed tomography (CT) of the chest is one of the most commonly performed imaging studies. Although the heart is included in every CT study, cardiac findings are commonly underreported in radiology reports. Left ventricular size is one of the most important prognostic markers in multiple cardiac diseases and can be measured on almost all non-ECG-gated multidetector chest CT studies. This review will discuss the available evidence on different measurements of left ventricular size obtained on non-ECG-gated CT of the chest. Measurement thresholds, technical issues, and potential problems are emphasized, with practical recommendations.

Purpose To quantify myocardial extracellular volume (ECV) by using cardiac magnetic resonance (MR) imaging in thalassemia major and to investigate the relationship between ECV and myocardial iron overload. Materials and Methods With institutional review board approval and informed consent, 30 patients with thalassemia major (mean age ± standard deviation, 34.6 years ± 9.5) and 10 healthy control subjects (mean age, 31.5 years ± 4.4) were prospectively recruited (clinicaltrials.gov identification number NCT02090699). Nineteen patients (63.3%) had prior myocardial iron overload (defined as midseptal T2* < 20 msec on any prior cardiac MR images). Cardiac MR imaging at 1.5 T included cine steady-state free precession for ventricular function, T2* for myocardial iron quantification, and unenhanced and contrast material-enhanced T1 mapping. ECV was calculated with input of the patient's hematocrit level. Peak systolic global longitudinal strain by means of speckle tracking was assessed with same-day transthoracic echocardiography. Statistical analysis included use of the two-sample t test, Fisher exact test, and Spearman correlation. Results Unenhanced T1 values were significantly lower in patients with prior myocardial iron overload than in control subjects (850.3 ± 115.1 vs 1006.3 ± 35.4, P < .001) and correlated strongly with T2* values (r = 0.874, P < .001). Patients with prior myocardial iron overload had higher ECV than did patients without iron overload (31.3% ± 2.8 vs 28.2% ± 3.4, P = .030) and healthy control subjects (27.0% ± 3.1, P = .003). There was no difference in ECV between patients without iron overload and control subjects (P = .647). ECV correlated with lowest historical T2* (r = -0.469, P = .010) but did not correlate significantly with left ventricular ejection fraction (r = -0.216, P = .252) or global longitudinal strain (r = -0.164, P = .423). Conclusion ECV is significantly increased in thalassemia major and is associated with myocardial iron overload. These abnormalities may potentially reflect diffuse interstitial myocardial fibrosis. (©) RSNA, 2015 Online supplemental material is available for this article.

Purpose: To evaluate the value of cardiac magnetic resonance imaging (MRI)-based measurements of inferior vena cava (IVC) cross-sectional area in the diagnosis of pericardial constriction., Methods: Patients who had undergone cardiac MRI for evaluation of clinically suspected pericardial constriction were identified retrospectively. The diagnosis of pericardial constriction was established by clinical history, echocardiography, cardiac catheterization, intraoperative findings, and/or histopathology. Cross-sectional areas of the suprahepatic IVC and descending aorta were measured on a single axial steady-state free-precession (SSFP) image at the level of the esophageal hiatus in end-systole. Logistic regression and receiver-operating curve (ROC) analyses were performed., Results: Thirty-six patients were included; 50% (n = 18) had pericardial constriction. Mean age was 53.9 ± 15.3 years, and 72% (n = 26) were male. IVC area, ratio of IVC to aortic area, pericardial thickness, and presence of respirophasic septal shift were all significantly different between patients with constriction and those without (P < .001 for all). IVC to aortic area ratio had the highest odds ratio for the prediction of constriction (1070, 95% confidence interval [8.0-143051], P = .005). ROC analysis illustrated that IVC to aortic area ratio discriminated between those with and without constriction with an area under the curve of 0.96 (95% confidence interval [0.91-1.00])., Conclusions: In patients referred for cardiac MRI assessment of suspected pericardial constriction, measurement of suprahepatic IVC cross-sectional area may be useful in confirming the diagnosis of constriction when used in combination with other imaging findings, including pericardial thickness and respirophasic septal shift., (Copyright © 2015 Canadian Association of Radiologists. Published by Elsevier Inc. All rights reserved.)

Background: The diagnosis of pericardial constriction remains challenging., Purpose: We sought to evaluate the predictive value of cardiovascular CT-based measurements of inferior vena cava (IVC) parameters in the diagnosis of pericardial constriction., Methods: Forty-two consecutive patients referred for assessment of pericardial constriction by 64-slice CT were evaluated. The diagnosis of pericardial constriction was confirmed by clinical history, echocardiography, cardiac catheterization, intraoperative findings, histopathology, or a combination. Diameter and cross-sectional area of the suprahepatic IVC and cross-sectional area of the aorta were measured on a single-axial CT image at the level of the esophageal hiatus. Maximum pericardial thickness was measured. Logistic regression and receiver operating curve analyses were performed., Results: Twenty-two patients had pericardial constriction. Mean age of the 42 patients was 57.1 ± 16.4 years, 57.1% were men. IVC diameter, IVC area, the ratio of IVC to aortic area, and pericardial thickness were all significantly greater in patients with constriction than in patients without (P < .05 for all). IVC-to-aortic area ratio had the highest odds ratio (51; 95% CI, 2.8-922) for the prediction of constriction and remained a significant predictor in multivariable analysis. In nested models, IVC-to-aortic area ratio had incremental value over pericardial thickness for the diagnosis of constriction. IVC-to-aortic area ratio discriminated between patients with and without constriction with an area under the curve of 0.88 on receiver operating curve analysis, with a value ≥ 1.6 having a sensitivity and specificity of 95% and 76%, respectively. Interobserver agreement for IVC-to-aortic area ratio was excellent (intraclass correlation coefficient, 0.98)., Conclusion: Assessment of IVC-to-aortic area ratio on CT aids with the diagnosis of pericardial constriction and has independent and incremental value over pericardial thickness alone., (Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.)