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A 60-year-old woman with a past medical history of asthma presented with fulminant myocarditis 9 days after testing positive for SARS-CoV-2 and 16 days after developing symptoms consistent with COVID-19. Her hospital course was complicated by the need for veno-arterial extracorporeal membrane oxygenation, ventricular arrhythmias, and pseudomonas bacteremia. She ultimately recovered and was discharged to home with normal left ventricular systolic function. Thereafter, she developed symptomatic ventricular tachycardia, for which she received an implantable cardioverter-defibrillator and antiarrhythmic drug therapy.

OBJECTIVE. A spectrum of pathophysiologic mechanisms can lead to the development of myocardial disorders including ischemia, genetic abnormalities, and systemic disorders. Cardiac MRI identifies different myocardial disorders, provides prognostic information, and directs therapy. In comparison with other imaging modalities, cardiac MRI has the advantage of allowing both functional assessment and tissues characterization in a single examination without the use of ionizing radiation. Newer cardiac MRI techniques including mapping can provide additional information about myocardial disease that may not be detected using conventional techniques. Emerging techniques including MR spectroscopy and finger printing will likely change the way we understand the pathophysiology mechanisms of the wide array of myocardial disorders. CONCLUSION. Imaging of myocardial disorders encompasses a large variety of conditions including both ischemic and nonischemic diseases. Cardiac MRI sequences, such as balanced steady-state free precession and late gadolinium enhancement, play a critical role in establishing diagnosis, determining prognosis, and guiding therapeutic management. Additional sequences-including perfusion imaging, T2*, real-time cine, and T2-weighted sequences-should be performed in specific clinical scenarios. There is emerging evidence for the use of mapping in imaging of myocardial disease. Multiple other new techniques are currently being studied. These novel techniques will likely change the way myocardial disorders are understood and diagnosed in the near future.

A 60-year-old woman with a past medical history of asthma presented with fulminant myocarditis 9 days after testing positive for SARS-CoV-2 and 16 days after developing symptoms consistent with COVID-19. Her hospital course was complicated by the need for veno-arterial extracorporeal membrane oxygenation, ventricular arrhythmias, and pseudomonas bacteremia. She ultimately recovered and was discharged to home with normal left ventricular systolic function. Thereafter, she developed symptomatic ventricular tachycardia, for which she received an implantable cardioverter-defibrillator and antiarrhythmic drug therapy.

Aortic stenosis with concomitant chronic kidney disease (CKD) represents a clinical challenge. Aortic stenosis is more prevalent and progresses more rapidly and unpredictably in CKD, and the presence of CKD is associated with worse short-term and long-term outcomes after aortic valve replacement. Because patients with advanced CKD and end-stage kidney disease have been excluded from randomized trials, clinicians need to make complex management decisions in this population that are based on retrospective and observational evidence. This statement summarizes the epidemiological and pathophysiological characteristics of aortic stenosis in the context of CKD, evaluates the nuances and prognostic information provided by noninvasive cardiovascular imaging with echocardiography and advanced imaging techniques, and outlines the special risks in this population. Furthermore, this statement provides a critical review of the existing literature pertaining to clinical outcomes of surgical versus transcatheter aortic valve replacement in this high-risk population to help guide clinical decision making in the choice of aortic valve replacement and specific prosthesis. Finally, this statement provides an approach to the perioperative management of these patients, with special attention to a multidisciplinary heart-kidney collaborative team-based approach.

Objectives LMNA variants have been previously associated with cardiac abnormalities independent of lipodystrophy. We aimed to assess cardiac impact of familial partial lipodystrophy (FPLD) to understand the role of laminopathy in cardiac manifestations. Study design Retrospective cohort study. Methods Clinical data from 122 patients (age range: 13-77, 101 females) with FPLD were analysed. Mature human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from a patient with an LMNA variant were studied as proof-of-concept for future studies. Results Subjects with LMNA variants had a higher prevalence of overall cardiac events than others. The likelihood of having an arrhythmia was significantly higher in patients with LMNA variants (OR: 3.77, 95% CI: 1.45-9.83). These patients were at higher risk for atrial fibrillation or flutter (OR: 5.78, 95% CI: 1.04-32.16). The time to the first arrhythmia was significantly shorter in the LMNA group, with a higher HR of 3.52 (95% CI: 1.34-9.27). Non-codon 482 LMNA variants were more likely to be associated with cardiac events (vs. 482 LMNA: OR: 4.74, 95% CI: 1.41-15.98 for arrhythmia; OR: 17.67, 95% CI: 2.45-127.68 for atrial fibrillation or flutter; OR: 5.71, 95% CI: 1.37-23.76 for conduction disease). LMNA mutant hiPSC-CMs showed a higher frequency of spontaneous activity and shorter action potential duration. Functional syncytia of hiPSC-CMs displayed several rhythm alterations such as early afterdepolarizations, spontaneous quiescence and spontaneous tachyarrhythmia, and significantly slower recovery in chronotropic changes induced by isoproterenol exposure. Conclusions Our results highlight the need for vigilant cardiac monitoring in FPLD, especially in patients with LMNA variants who have an increased risk of developing cardiac arrhythmias. in addition, hiPSC-CMs can be studied to understand the basic mechanisms for the arrhythmias in patients with lipodystrophy to understand the impact of specific mutations., NIH Clinical and Translational Science AwardsUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [UL1TR000433]; Nutrition Obesity Research Centers [P30 DK089503]; NIH institutional grantUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [DK034933]; White Point Foundation of Turkey; Morton S. and Henrietta K. Sellner Professorship in Human Genetics, Infrastructure and data management support has been provided by the NIH Clinical and Translational Science Awards grant UL1TR000433, the Nutrition Obesity Research Centers grant P30 DK089503, and NIH institutional grant DK034933. Finally, the work was supported by generous gifts to the Lipodystrophy Fund at the University of Michigan made by the Sopha family, and the White Point Foundation of Turkey. JWI acknowledges support from the Morton S. and Henrietta K. Sellner Professorship in Human Genetics.

To highlight imaging features of coronary artery anomalies, especially those that may require surgical intervention, and review various management options. The clinical presentation of coronary artery anomalies ranges from clinically silent lesions to those leading to sudden cardiac death. With the evolution and increasing use of advanced imaging techniques such as computed tomography, more cases are being identified on scans either done specifically for this purpose or discovered incidentally. Management decisions can be complex and often require a multidisciplinary approach. In this article, we review advances in imaging techniques, the spectrum of imaging appearances of coronary artery anomalies, and the role of imaging in directing appropriate management as well as post-operative follow-up.

Introduction: Pulmonary hypertension is common among patients with end-stage renal disease, although data regarding the impact of right ventricular (RV) failure on postoperative outcomes remain limited. We hypothesized that echocardiographic findings of RV dilation and dysfunction are associated with adverse clinical outcomes after renal transplant. Methods: A retrospective review of adult renal transplant recipients at a single institution from January 2008 to June 2010 was conducted. Patients with transthoracic echocardiograms (TTEs) within 1 year leading up to transplant were included. The primary end point was a composite of delayed graft function, graft failure, and all-cause mortality. Results: Eighty patients were included. Mean follow-up time was 9.4 ± 0.8 years. Eight patients (100%) with qualitative RV dysfunction met the primary end point, while 39/65 patients (60.0%) without RV dysfunction met the end point ( p = 0.026). Qualitative RV dilation was associated with a significantly shorter time to all-cause graft failure ( p = 0.03) and death ( p = 0.048). RV systolic pressure was not measurable in 45/80 patients (56%) and was not associated with outcomes in the remaining patients. Conclusion: RV dilation and dysfunction are associated with adverse outcomes after renal transplant. TTE assessment of RV size and function should be a standard part of the pre-kidney transplant cardiovascular risk assessment.

Background Pathogenic variants in Lamin A/C (LMNA) gene are the most common monogenic etiology in Familial Partial Lipodystrophy (FPLD) causing FPLD2. LMNA pathogenic variants have been previously associated with cardiomyopathy, familial arrhythmias or conduction system abnormalities independent of lipodystrophy. We aimed to assess cardiac impacts of FPLD, and to explore the extent of overlap between cardiolaminopathies and FPLD. Methods We conducted a retrospective review of an established cohort of 122 patients (age range: 13-77, M/F 21/101) with FPLD from Michigan (n = 83) and Turkey (n = 39) with an accessible cardiac evaluation. Also, functional syncytia of mature human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from a FLPD2 patient was studied for assessment of autonomous rhythm and action potential duration with optical mapping using a voltage sensitive dye. Results In the whole study cohort, 95 (78%) patients had cardiac alterations (25% ischemic heart disease, 36% arrhythmia, 16% conduction abnormality, 20% prolonged QT interval, 11% cardiomyopathy, and 15% congestive heart failure). The likelihood of having an arrhythmia (OR; 3.95, 95% CI: 1.49-10.49) and conduction disease (OR: 3.324, 95% CI: 1.33-8.31) was significantly higher in patients with LMNA pathogenic variants. Patients with LMNA pathogenic variants were at high risk for atrial fibrillation/flutter (OR: 6.77, 95% CI: 1.27- 39.18). The time to first arrhythmia was significantly shorter in the LMNA group with a higher hazard rate of 3.04 (95% CI: 1.29-7.17, p = 0.032). Non-482 LMNA pathogenic variants were more likely to be associated with cardiac events (vs. 482 LMNA: OR: 4.74, 95% CI: 1.41- 15.98 for arrhythmia; OR: 17.67, 95% CI: 2.44- 127.68 for atrial fibrillation/flutter; OR: 5.71, 95% CI: 1.37- 23.76 for conduction disease. hiPSC-CMs from a FPLD2 patient had higher frequency of autonomous activity, and shorter Fridericia corrected action potential duration at 80% repolarization compared to control cardiomyocytes. Furthermore, FPLD2 functional syncytia of mature hiPSC-CMs presented several rhythm alterations such as early after-depolarizations, spontaneous quiescence and spontaneous tachyarrhythmia; none of those were observed in the control cell lines. Finally, FPLD2 hiPSC-CMs presented significantly slower recovery in chronotropic changes induced by isoproterenol exposure; which indicates disrupted beta-adrenergic response. Conclusion Our results suggest the need for vigilant cardiac monitoring in FPLD, especially in patients with FPLD2 who have an increased risk to develop cardiac arrhythmias and conduction system diseases. In addition, study of human induced pluripotent stem cell-derived cardiomyocytes may prove useful to understand the mechanism of cardiac disease and arrhythmias and to create precision therapy opportunities in the future.

End-stage kidney disease (ESKD) and heart failure (HF) often coexist and must be managed simultaneously. Multidisciplinary collaboration between nephrology and cardiology is critical when treating patients with such complicated physiology. There is no "one-size-fits-all" approach to the evaluation of patients with new left ventricular systolic dysfunction, and diagnostic testing should be adapted to an individual's risk factors. Guideline-directed medical therapy (GDMT) for systolic heart failure should be employed in these patients. While limited randomized data exist, observational data and post hoc analyses suggest that GDMT, including renin angiotensin aldosterone system inhibitors, is associated with improved cardiovascular outcomes and can be safely initiated at low doses with close monitoring of kidney function in this population. Volume status is typically managed through ultrafiltration, so close communication between cardiology and nephrology is necessary to achieve a patient's optimal dry weight and mitigate intradialytic hypotension. Patient education and engagement regarding sodium and fluid restriction is crucial, and symptom burden should be reassessed following changes to the dialysis regimen.

Objective: Lipodystrophy represents a group of rare diseases characterized by loss of body fat. While patients with generalized lipodystrophy exhibit near-total lack of fat, partial lipodystrophy is associated with selective fat loss affecting certain parts of the body. Although classical familial partial lipodystrophy (FPLD) is a well-described entity, recent reports indicate phenotypic heterogeneity among carriers of LMNA pathogenic variants. Methods: We have encountered 2 unique cases with complex phenotypes, generalized fat loss, and very low leptin levels that made the distinction between generalized versus partial lipodystrophy quite challenging. Results: We present a 61-year-old female with generalized fat loss, harboring the heterozygous pathogenic variant p.R541P (c.1622G>C) on the LMNA gene. The discovery of the pathogenic variant led to correct clinical diagnosis of her muscle disease, identification of significant heart disease, and a recommendation for the implantation of a defibrillator. She was able to start metreleptin based on her generalized fat loss pattern and demonstration of the genetic variant. Secondly, we report a 40-year-old Turkish female with generalized fat loss associated with a novel heterozygous LMNA pathogenic variant p.K486E (c.1456A>G), who developed systemic B cell follicular lymphoma. Conclusion: Clinicians need to recognize that the presence of an LMNA variant does not universally lead to FPLD type 2, but may lead to a phenotype that is more complex and may resemble more closely generalized lipo-dystrophy. Additionally, providers should recognize the multisystem features of laminopathies and should screen for these features in affected patients, especially if the variant is not at the known hotspot for FPLD type 2. © 2020 Elsevier Inc., Bristol-Myers Squibb, BMS; AstraZeneca; University of Michigan, U-M; Ionis Pharmaceuticals; Akebia Therapeutics, E.A.O. and J.E.A.W. were partially supported by the Lipodystrophy Fund at the University of Michigan which is graciously funded by the Sopha Family and the White Point Foundation of Turkey. E.A.O. received grant support from and served as an advisor to Amylin Pharmaceuticals LLC, Bristol-Myers Squibb, and AstraZeneca in the past and is currently receiving grant support from Gemphire Therapeutics, Aegerion Pharmaceuticals, Ionis Pharmaceuticals, and Akcea Therapeutics and is serving as an advisor to Aegerion Pharmaceuticals, Akcea Therapeutics, and Regeneron Pharmaceuticals through funding paid to the University of Michigan. E.A.O. recently started grant support from GI Dynamics. B.A. has attended scientific advisory board meetings organized by Aegerion Pharmaceuticals and has received honoraria as a speaker from AstraZeneca, Lilly, MSD, Novartis, Novo Nordisk, Boehringer-Ingelheim, Servier, and Sanofi-Aventis. The other authors have no multiplicities of interest to disclose.

In diagnosing and following patients with acute aortic syndromes and thoracic aortic aneurysms, high-quality imaging of the thoracic aorta is indispensable. Mainstay modalities for thoracic aortic imaging are echocardiography, computed tomographic angiography, and magnetic resonance angiography. For any given clinical scenario, the imaging modality and protocol chosen will have a significant impact on sensitivity and specificity for the aortic diagnosis of concern. Imaging can also provide important ancillary information regarding myocardial performance, aortic valve morphology and function, and end-organ perfusion. Surveillance of patients following thoracic aortic surgery with serial imaging studies can identify complications that may require reintervention, and imaging has played an integral role in development of new surgical and interventional methods. Emerging techniques in thoracic aortic imaging include positron emission tomography, which addresses vessel wall inflammation, and 4-dimensional magnetic resonance angiography, which illustrates flow dynamics.

Background: Lipodystrophy syndromes are characterized by loss of body fat. Although classical Familial Partial Lipodystrophy (FPLD) and Congenital Generalized Lipodystrophy (CGL) have different clinical presentations, we have encountered a unique case where the distinction was quite challenging. Clinical Case: Our patient first presented to an endocrinologist at age 19 with secondary amenorrhea and hypothyroidism. After normalization of TSH, further investigation revealed central hypogonadism, hypertriglyceridemia (>3,000 mg/dL, normal

Author(s): Saran, Rajiv; Robinson, Bruce; Abbott, Kevin C; Agodoa, Lawrence YC; Bragg-Gresham, Jennifer; Balkrishnan, Rajesh; Bhave, Nicole; Dietrich, Xue; Ding, Zhechen; Eggers, Paul W; Gaipov, Abduzhappar; Gillen, Daniel; Gipson, Debbie; Gu, Haoyu; Guro, Paula; Haggerty, Diana; Han, Yun; He, Kevin; Herman, William; Heung, Michael; Hirth, Richard A; Hsiung, Jui-Ting; Hutton, David; Inoue, Aya; Jacobsen, Steven J; Jin, Yan; Kalantar-Zadeh, Kamyar; Kapke, Alissa; Kleine, Carola-Ellen; Kovesdy, Csaba P; Krueter, William; Kurtz, Vivian; Li, Yiting; Liu, Sai; Marroquin, Maria V; McCullough, Keith; Molnar, Miklos Z; Modi, Zubin; Montez-Rath, Maria; Moradi, Hamid; Morgenstern, Hal; Mukhopadhyay, Purna; Nallamothu, Brahmajee; Nguyen, Danh V; Norris, Keith C; O'Hare, Ann M; Obi, Yoshitsugu; Park, Christina; Pearson, Jeffrey; Pisoni, Ronald; Potukuchi, Praveen K; Repeck, Kaitlyn; Rhee, Connie M; Schaubel, Douglas E; Schrager, Jillian; Selewski, David T; Shamraj, Ruth; Shaw, Sally F; Shi, Jiaxiao M; Shieu, Monica; Sim, John J; Soohoo, Melissa; Steffick, Diane; Streja, Elani; Sumida, Keiichi; Kurella Tamura, Manjula; Tilea, Anca; Turf, Megan; Wang, Dongyu; Weng, Wenjing; Woodside, Kenneth J; Wyncott, April; Xiang, Jie; Xin, Xin; Yin, Maggie; You, Amy S; Zhang, Xiaosong; Zhou, Hui; Shahinian, Vahakn

This document is the second of 2 companion appropriate use criteria (AUC) documents developed by the American College of Cardiology, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. The first document

Background: Patients with end-stage kidney disease (ESKD) on dialysis were excluded from clinical trials of direct oral anticoagulants for atrial fibrillation (AF). Recent data have raised concerns regarding the safety of dabigatran and rivaroxaban, but apixaban has not been evaluated despite current labeling supporting its use in this population. The goal of this study was to determine patterns of apixaban use and its associated outcomes in dialysis-dependent patients with ESKD and AF. Methods: We performed a retrospective cohort study of Medicare beneficiaries included in the United States Renal Data System (October 2010 to December 2015). Eligible patients were those with ESKD and AF undergoing dialysis who initiated treatment with an oral anticoagulant. Because of the small number of dabigatran and rivaroxaban users, outcomes were only assessed in patients treated with apixaban or warfarin. Apixaban and warfarin patients were matched (1:3) based on prognostic score. Differences between groups in survival free of stroke or systemic embolism, major bleeding, gastrointestinal bleeding, intracranial bleeding, and death were assessed using Kaplan–Meier analyses. Hazard ratios (HRs) and 95% CIs were derived from Cox regression analyses. Results: The study population consisted of 25 523 patients (45.7% women; 68.2±11.9 years of age), including 2351 patients on apixaban and 23 172 patients on warfarin. An annual increase in apixaban prescriptions was observed after its marketing approval at the end of 2012, such that 26.6% of new anticoagulant prescriptions in 2015 were for apixaban. In matched cohorts, there was no difference in the risks of stroke/systemic embolism between apixaban and warfarin (HR, 0.88; 95% CI, 0.69–1.12; P =0.29), but apixaban was associated with a significantly lower risk of major bleeding (HR, 0.72; 95% CI, 0.59–0.87; P P =0.04 for stroke/systemic embolism; HR, 0.64; 95% CI, 0.45–0.92; P =0.01 for death) or warfarin (HR, 0.64; 95% CI, 0.42–0.97; P =0.04 for stroke/systemic embolism; HR, 0.63; 95% CI, 0.46–0.85; P =0.003 for death). Conclusions: Among patients with ESKD and AF on dialysis, apixaban use may be associated with a lower risk of major bleeding compared with warfarin, with a standard 5 mg twice a day dose also associated with reductions in thromboembolic and mortality risk.

Author(s): Saran, Rajiv; Robinson, Bruce; Abbott, Kevin C; Agodoa, Lawrence YC; Bhave, Nicole; Bragg-Gresham, Jennifer; Balkrishnan, Rajesh; Dietrich, Xue; Eckard, Ashley; Eggers, Paul W; Gaipov, Abduzhappar; Gillen, Daniel; Gipson, Debbie; Hailpern, Susan M; Hall, Yoshio N; Han, Yun; He, Kevin; Herman, William; Heung, Michael; Hirth, Richard A; Hutton, David; Jacobsen, Steven J; Jin, Yan; Kalantar-Zadeh, Kamyar; Kapke, Alissa; Kovesdy, Csaba P; Lavallee, Danielle; Leslie, Janet; McCullough, Keith; Modi, Zubin; Molnar, Miklos Z; Montez-Rath, Maria; Moradi, Hamid; Morgenstern, Hal; Mukhopadhyay, Purna; Nallamothu, Brahmajee; Nguyen, Danh V; Norris, Keith C; O'Hare, Ann M; Obi, Yoshitsugu; Park, Christina; Pearson, Jeffrey; Pisoni, Ronald; Potukuchi, Praveen K; Rao, Panduranga; Repeck, Kaitlyn; Rhee, Connie M; Schrager, Jillian; Schaubel, Douglas E; Selewski, David T; Shaw, Sally F; Shi, Jiaxiao M; Shieu, Monica; Sim, John J; Soohoo, Melissa; Steffick, Diane; Streja, Elani; Sumida, Keiichi; Tamura, Manjula K; Tilea, Anca; Tong, Lan; Wang, Dongyu; Wang, Mia; Woodside, Kenneth J; Xin, Xin; Yin, Maggie; You, Amy S; Zhou, Hui; Shahinian, Vahakn

This document is 1 of 2 companion appropriate use criteria (AUC) documents developed by the American College of Cardiology, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. This document addresses the evaluation and use of multimodality imaging in the diagnosis and management of valvular heart disease, whereas the second, companion document addresses this topic with regard to structural heart disease. Although there is clinical overlap, the documents addressing valvular and structural heart disease are published separately, albeit with a common structure. The goal of the companion AUC documents is to provide a comprehensive resource for multimodality imaging in the context of valvular and structural heart disease, encompassing multiple imaging modalities. Using standardized methodology, the clinical scenarios (indications) were developed by a diverse writing group to represent patient presentations encountered in everyday practice and included common applications and anticipated uses. Where appropriate, the scenarios were developed on the basis of the most current American College of Cardiology/American Heart Association guidelines. A separate, independent rating panel scored the 92 clinical scenarios in this document on a scale of 1 to 9. Scores of 7 to 9 indicate that a modality is considered appropriate for the clinical scenario presented. Midrange scores of 4 to 6 indicate that a modality may be appropriate for the clinical scenario, and scores of 1 to 3 indicate that a modality is considered rarely appropriate for the clinical scenario. The primary objective of the AUC is to provide a framework for the assessment of these scenarios by practices that will improve and standardize physician decision making. AUC publications reflect an ongoing effort by the American College of Cardiology to critically and systematically create, review, and categorize clinical situations where diagnostic tests and procedures are utilized by physicians caring for patients with cardiovascular diseases. The process is based on the current understanding of the technical capabilities of the imaging modalities examined.

Background Right ventricular (RV) strain is a potentially useful prognostic marker in patients with pulmonary arterial hypertension (PAH). However, published reports regarding the accuracy of two-dimensional echocardiography (2DE)-derived RV strain against an independent reference in this patient population are limited. The aims of this study were: (1) to study the relationship between 2DE RV longitudinal strain and cardiovascular magnetic resonance (CMR)-derived RV ejection fraction (RVEF) in patients with PAH; (2) to compare 2DE-derived and CMR-derived RV longitudinal strain in these patients; and (3) to determine the reproducibility of these measurements. Methods Thirty patients with PAH underwent 2DE and CMR imaging within a 2-hour time period. 2DE RV longitudinal strain was measured from a focused RV apical four-chamber view using speckle tracking software. CMR RV longitudinal strain was measured from short-axis slices acquired using fast-strain-encoded sequence. Global peak systolic RV longitudinal strain was calculated for both 2DE and CMR. Results RV longitudinal strain using 2DE software correlated well with CMR-derived RVEF (R = 0.69, P = 0.0006). There was moderate agreement when comparing 2DE to CMR RV longitudinal strain (R = 0.74, P = 0.0002; bias −1%, limits of agreement −9 to 7%). Inter-observer variability and intra-observer variability for RV longitudinal strain were lower for 2DE than CMR. Conclusions RV longitudinal strain by 2DE provides a good alternative for CMR-derived RVEF in patients with PAH. The moderate agreement in strain measurements between 2DE and CMR suggests that further software improvements are needed before these measurements can be used interchangeably in clinical practice.

with the advent of real-time 3-dimensional transesophageal echocardiography (3d tee), rapid creation of high-quality, true-to-life images of mitral valve anatomy and pathology has become possible in the vast majority of patients ([1][1]). In addition to common mitral valve lesions, such as

The aim of this study was to compare appropriateness designations as determined by the updated 2011 appropriate use criteria (AUC) for echocardiography with prior versions of the AUC for transthoracic echocardiographic (TTE) imaging, transesophageal echocardiographic (TEE) imaging, and stress echocardiographic (SE) imaging. An additional goal was to define relationships between appropriateness determinations and echocardiographic findings for each modality.Previously published data sets of TTE, TEE, and SE studies were reclassified according to the 2011 AUC, and indication representation, appropriateness designations, and echocardiographic findings were compared with prior classifications according to the 2007 AUC for TTE and TEE imaging and the 2008 AUC for SE imaging.Overall, 2,247 echocardiographic studies were analyzed. The 2011 AUC addressed the vast majority of studies (98%), a marked increase compared with prior versions of the AUC (89%) (P.001). An increase in addressed studies was present in each echocardiographic modality (TTE imaging: n = 1,525, 98% vs 89%, P .001; TEE imaging: n = 405, 99.7% vs 91%, P.01; SE imaging: n = 289, 97% vs 88%, P.01). Among all echocardiographic procedures, the 2011 AUC found a lower frequency of appropriate studies compared with prior AUC (82% vs 88%, P.01), primarily because of new uncertain indications for TTE imaging. The frequency of inappropriate echocardiographic studies was unchanged (11%). Among all echocardiographic procedures, the 2011 AUC found appropriate studies to have more new abnormal echocardiographic findings compared with inappropriate studies (45% vs 13%, P.001). Interestingly, 2011 AUC inappropriate TTE studies had fewer major new echocardiographic abnormalities than 2007 AUC inappropriate TTE studies (9% vs 17%, P = .04).The updated 2011 AUC for echocardiography encompass the vast majority of echocardiographic procedures in a university hospital practice, filling virtually all of the gaps identified in prior versions of the AUC for TTE, TEE, and SE imaging. The 2011 AUC also reasonably stratify the likelihood of finding an echocardiographic abnormality, demonstrating improvement compared with the prior AUC.

Background Clinical application of the American College of Cardiology Foundation Appropriate Use Criteria (AUC) represents a potentially feasible alternative to third-party pre-certification for imaging procedures and will soon be required as part of the accreditation process for imaging laboratories. Electronic tools that rapidly apply the AUC are needed in clinical practice. We developed and tested a web-based application of the AUC to track appropriateness of transthoracic echocardiography (TTE). Methods Indications for outpatient TTE studies performed in a university hospital echocardiography laboratory were assessed prospectively at the point of service using a prototype web-based AUC application (Echo AUC App). The Echo AUC App was developed on the basis of our own prior published data regarding indication frequency to minimize time and screens required for completion. Echo AUC App-determined indications were compared with blinded investigator-determined indications based on review of relevant medical records. Echo AUC App characteristics, including Echo AUC App entry time, were recorded. Results Of the 258 studies enrolled, Echo AUC App-determined TTE indications were Appropriate (A) in 77% ( n = 198), Inappropriate (I) in 9% ( n = 23), and Not Classified (NC) by the AUC in 14% ( n = 37). Agreement between Echo AUC App- and investigator-determined classifications was excellent (94%, kappa statistic 0.83). Mean Echo AUC App study entry time was 55 seconds (range 25-280 seconds). Conclusion The use of an electronic application allows rapid and accurate implementation of the AUC for TTE at the point of service. Such an application could be installed in echocardiography laboratories to track appropriateness in accordance with soon-to-be-implemented accreditation requirements. Further study of this Echo AUC App at the point of order may provide an alternative to third-party pre-certification procedures.

Background The aim of this study was to evaluate the clinical application of the American College of Cardiology Foundation and American Society of Echocardiography appropriateness criteria for stress echocardiography (SE) in a single-center university hospital. Methods Indications were determined for consecutive studies by two reviewers and categorized as appropriate, uncertain, or inappropriate. Results Of 477 studies for which primary indications could be determined, 188 specifically related to university transplantation programs were excluded. Of the remaining 289 studies, 88% were addressed in the appropriateness criteria for SE. Of these, 71% were appropriate, 9% were uncertain, and 20% were inappropriate. Inappropriate studies were more likely to be ordered on younger patients and women and were less likely to be ordered by cardiologists. Abnormal results on SE were more frequent among appropriate than inappropriate studies. Conclusions The appropriateness criteria for SE encompass and effectively characterize the majority of studies ordered in a single-center university hospital and appear to reasonably stratify the likelihood of abnormal results on SE. However, revisions will be required to fully capture and stratify appropriate clinical practice of SE.

Direct oral anticoagulants (DOACs) reduce the thromboembolic risk in general populations with atrial fibrillation (AF), but their safety and effectiveness in dialysis patients is not well known and their use is discouraged due to a lack of empirical data. We identified dialysis patients with non

International audience; Background: There is no accepted methodology for CT-based vasodilator stress myocardial perfusion imaging and analysis. We developed a technique for quantitative 3D analysis of CT images, which provides several indices of myocardial perfusion. We sought to determine the ability of these indices during vasodilator stress to identify segments supplied by coronary arteries with obstructive disease and to test the accuracy of the detection of perfusion abnormalities against SPECT.Methods: We studied 93 patients referred for CT coronary angiography (CTCA) who underwent regadenoson stress. 3D analysis of stress CT images yielded segmental perfusion indices: mean X-ray attenuation, severity of defect and relative defect volume. Each index was averaged for myocardial segments, grouped by severity of stenosis: 0%, 70%. Objective detection of perfusion abnormalities was optimized in 47 patients and then independently tested in the remaining 46 patients.Results: CTCA depicted normal coronary arteries or non-obstructive disease in 62 patients and stenosis of >50% in 31. With increasing stenosis, segmental attenuation showed a 7% decrease, defect severity increased 11%, but relative defect volume was 7-fold higher in segments with obstructive disease (p50% showed sensitivity 0.78, specificity 0.54, accuracy 0.59. When compared to SPECT in a subset of 21 patients (14 with abnormal SPECT), stress CT perfusion analysis showed sensitivity 0.79, specificity 0.71, accuracy 0.76.Conclusions: 3D analysis of vasodilator stress CT images provides quantitative indices of myocardial perfusion, of which relative defect volume was most robust in identifying segments supplied by arteries with obstructive disease. This study may have implications on how CT stress perfusion imaging is performed and analyzed.

Background Iterative reconstruction (IR) in cardiac CT has been shown to improve confidence of interpretation of noninvasive coronary CT angiography (CTA). Objective We hypothesized that IR would also improve the quality of vasodilator stress coronary CT images acquired with low tube voltage to assess myocardial perfusion and the accuracy of the detection of perfusion abnormalities by using quantitative 3-dimensional (3D) analysis. Methods We studied 39 consecutive patients referred for coronary CTA (256-slice scanner; Philips), who underwent additional imaging (100 kV, prospective gating) with regadenoson (0.4 mg; Astellas). Stress images were reconstructed with different algorithms: filtered back projection (FBP) and IR (iDose; Philips). Image quality was quantified by signal-to-noise and contrast-to-noise ratios in the blood pool and the myocardium. Then, FBP and separately IR images were analyzed with custom 3D analysis software to quantitatively detect perfusion defects. Accuracy of detection was compared with perfusion abnormalities predicted by coronary stenosis >50% on coronary CTA. Results Five patients with image artifacts were excluded. In the remaining 34 patients, both signal-to-noise and contrast-to-noise ratios increased with IR, indicating improvement in image quality compared with FBP. For 3D perfusion analysis, 10 patients with normal coronary arteries were used as a reference to correct for x-ray attenuation variations in normal myocardium. In the remaining 24 patients, reduced noise levels in the IR images compared with FBP resulted in tighter attenuation distribution and improved detection of perfusion abnormalities. Conclusion IR significantly improves image quality on regadenoson stress CT images acquired with low tube voltage, leading to improved 3D quantitative evaluation of myocardial perfusion.

Background— Placebo-controlled trials for pulmonary arterial hypertension are no longer acceptable because new therapies must show clinically significant effects on top of standard treatment. The purpose of this study was to estimate sample sizes and imaging costs for the planning of a hypothetical pulmonary arterial hypertension drug trial using imaging to detect changes in right ventricular size and function in response to combined therapy. Methods and Results— Same-day cardiovascular MR (CMR) and 2-dimensional (2D) and 3D transthoracic echocardiography (2DTTE and 3DTTE) were performed in 22 patients with pulmonary arterial hypertension (54±13 years of age) twice, 6 months apart. Short-axis CMR cines and full-volume 3DTTE data sets of the right ventricle were used to measure end-diastolic volume and ejection fraction. Fractional area change was obtained from 2DTTE. Sample size calculations used a 2-sample t test model incorporating differences between baseline and 6-month measurements. Cost estimates were made using the Medicare fee schedule. No significant differences were noted between baseline and follow-up measurements. Large SDs reflected variable progression of disease in individual patients on standard therapy and measurement variability. These sources of variability resulted in intertechnique differences in sample sizes: to detect a change of 5% to 15% in 3DTTE-derived right ventricular ejection fraction and fractional area change or change of 15 to 30 mL in 3DTTE right ventricular end-diastolic volume; sample sizes were 2× to 2.5× those required by CMR. As a result, the total cost of a trial using complete TTE was greater than CMR, which was greater than limited TTE. Conclusions— Because of lower measurement variability, CMR is more cost saving in pulmonary arterial hypertension drug trials than echocardiography, unless limited TTE is used.

To summarize research on the use of 3-dimensional (3D) echocardiography for quantifying left ventricular (LV) volumes, ejection fraction, and mass, and to describe emerging applications of the technology.Several publications have provided reference values for LV volumes and ejection fraction by 3D transthoracic echocardiography (3DTTE) in normal patients.With real-time 3DTTE, one can acquire and display a 3D image encompassing the entire LV within seconds. Because 3DTTE aids in identification of the true LV apex, it provides more accurate LV volumes than its 2D counterpart. As compared with a cardiovascular magnetic resonance standard, 3DTTE tends to slightly underestimate LV volumes, in part because its spatial resolution is limited, making identification of the true endocardial border more difficult. As compared with 2DTTE, 3DTTE is advantageous for identifying and assessing the extent of regional wall motion abnormalities. For quantification of LV mass, 3DTTE is superior to both 2DTTE and M-mode echocardiography. Emerging applications of 3DTTE include speckle-tracking strain assessment, dyssynchrony analysis, and LV shape analysis, which appear to have prognostic value in patients with a variety of cardiac conditions.

Cardiac computed tomography (CCT) has become an important tool for the anatomic assessment of patients with suspected coronary disease. Its diagnostic accuracy for detecting the presence of underlying coronary artery disease and ability to risk stratify patients are well documented. However, the role of CCT for the physiologic assessment of myocardial perfusion during resting and stress conditions is only now emerging. With the addition of myocardial perfusion imaging to coronary imaging, CCT has the potential to assess both coronary anatomy and its functional significance with a single non-invasive test. In this review, we discuss the current state of CCT myocardial perfusion imaging for the detection of myocardial ischemia and myocardial infarction and examine its complementary role to CCT coronary imaging.

Cardiac chamber quantification from 3D transthoracic echocardiography (3D TTE) has been shown to be superior to measurements obtained from 2D studies. However, integration of 3D TTE into routine clinical practice has been limited by the time-consuming workflow and need for 3D expertise. We assessed