Search

BackgroundAbnormal substrate on invasive electroanatomic mapping (EAM) correlates with areas of myocardial thinning and fibrofatty replacement in Arrhythmogenic Cardiomyopathy (ACM). However, EAM parameters are absent from all sets of diagnostic criteria for ACM.Case summaryA 41-year-old female with no significant family history was referred for evaluation of frequent premature ventricular complexes (PVCs). Twelve-lead ECG showed diffuse low-voltage QRS complexes. Holter monitor showed 28% burden of PVCs with various morphologies consistent with right ventricular (RV) inflow and outflow tract exits. Transthoracic echocardiogram revealed normal biventricular function and dimension. Cardiac magnetic resonance revealed a mildly increased indexed RV end-diastolic volume with normal RV systolic function and no dyssynchrony, akinesia, dyskinesia, or late gadolinium enhancement. Electrophysiologic study demonstrated 2 predominant PVC morphologies that were targeted with ablation, in addition to extensive abnormality with low-voltage and fractionated electrograms in the peri-tricuspid and right ventricular outflow tract free wall regions with septal sparing, suggestive of RV cardiomyopathy. Subsequent genetic testing revealed two pathogenic variants in the desmoplakin and plakophilin-2 genes, confirming the diagnosis of ACM.ConclusionAdvanced RV electropathy can precede RV structural changes in ACM. Invasive evaluation of the electroanatomic substrate should be considered in select cases even when imaging findings are not diagnostic. Future iterations of ACM guidelines may need to consider EAM substrate as one of the diagnostic criteria. A high index of diagnostic suspicion for ACM should be maintained in patients with multifocal RV ectopy.

Abstract Hemodynamic assessment is an integral part of the diagnosis and management of cardiovascular disease. Four-dimensional cardiovascular magnetic resonance flow imaging (4D Flow CMR) allows comprehensive and accurate assessment of flow in a single acquisition. This consensus paper is an update from the 2015 ‘4D Flow CMR Consensus Statement’. We elaborate on 4D Flow CMR sequence options and imaging considerations. The document aims to assist centers starting out with 4D Flow CMR of the heart and great vessels with advice on acquisition parameters, post-processing workflows and integration into clinical practice. Furthermore, we define minimum quality assurance and validation standards for clinical centers. We also address the challenges faced in quality assurance and validation in the research setting. We also include a checklist for recommended publication standards, specifically for 4D Flow CMR. Finally, we discuss the current limitations and the future of 4D Flow CMR. This updated consensus paper will further facilitate widespread adoption of 4D Flow CMR in the clinical workflow across the globe and aid consistently high-quality publication standards.

IntroductionVolume overload from mitral regurgitation can result in left ventricular systolic dysfunction. To prevent this, it is essential to operate before irreversible dysfunction occurs, but the optimal timing of intervention remains unclear. Current echocardiographic guidelines are based on 2D linear measurement thresholds only. We compared volumetric CT-based and 2D echocardiographic indices of LV size and function as predictors of post-operative systolic dysfunction following mitral repair.MethodsWe retrospectively identified patients with primary mitral valve regurgitation who underwent repair between 2005 and 2021. Several indices of LV size and function measured on preoperative cardiac CT were compared with 2D echocardiography in predicting post-operative LV systolic dysfunction (LVEFecho

Cardiovascular magnetic resonance (CMR) is a proven imaging modality for informing diagnosis and prognosis, guiding therapeutic decisions, and risk stratifying surgical intervention. Patients with a cardiac implantable electronic device (CIED) would be expected to derive particular benefit from CMR given high prevalence of cardiomyopathy and arrhythmia. While several guidelines have been published over the last 16 years, it is important to recognize that both the CIED and CMR technologies, as well as our knowledge in MR safety, have evolved rapidly during that period. Given increasing utilization of CIED over the past decades, there is an unmet need to establish a consensus statement that integrates latest evidence concerning MR safety and CIED and CMR technologies. While experienced centers currently perform CMR in CIED patients, broad availability of CMR in this population is lacking, partially due to limited availability of resources for programming devices and appropriate monitoring, but also related to knowledge gaps regarding the risk-benefit ratio of CMR in this growing population. To address the knowledge gaps, this SCMR Expert Consensus Statement integrates consensus guidelines, primary data, and opinions from experts across disparate fields towards the shared goal of informing evidenced-based decision-making regarding the risk-benefit ratio of CMR for patients with CIEDs.

Abstract Background Quantitative evaluation of mitral regurgitation (MR) in hypertrophic cardiomyopathy (HCM) by cardiovascular magnetic resonance (CMR) relies on an indirect volumetric calculation. The aim of this study was to directly assess and quantify MR jets in patients with HCM using 4D flow CMR jet tracking in comparison to standard-of-care CMR indirect volumetric method. Methods This retrospective study included patients with HCM undergoing 4D flow CMR. By the indirect volumetric method from CMR, MR volume was quantified as left ventricular stroke volume minus forward aortic volume. By 4D flow CMR direct jet tracking, multiplanar reformatted planes were positioned in the peak velocity of the MR jet during systole to calculate through-plane regurgitant flow. MR severity was collected for agreement analysis from a clinical echocardiograms performed within 1 month of CMR. Inter-method and inter-observer agreement were assessed by intraclass correlation coefficient (ICC), Bland–Altman analysis, and Cohen’s kappa. Results Thirty-seven patients with HCM were included. Direct jet tracking demonstrated good inter-method agreement of MR volume compared to the indirect volumetric method (ICC = 0.80, p = 0.004) and fair agreement of MR severity (kappa = 0.27, p = 0.03). Direct jet tracking showed higher agreement with echocardiography (kappa = 0.35, p = 0.04) than indirect volumetric method (kappa = 0.16, p = 0.35). Inter-observer reproducibility of indirect volumetric method components revealed the lowest reproducibility in end-systolic volume (ICC = 0.69, p = 0.15). Indirect volumetric method showed good agreement of MR volume (ICC = 0.80, p = 0.003) and fair agreement of MR severity (kappa = 0.38, p

Abstract Aims While right ventricular (RV) dysfunction is associated with worse prognosis in co‐morbid pulmonary hypertension and heart failure with preserved ejection fraction (PH‐HFpEF), the mechanisms driving RV dysfunction are unclear. We evaluated the extent and clinical correlates of diffuse RV myocardial fibrosis in PH‐HFpEF, as measured by cardiovascular magnetic resonance‐derived extracellular volume (ECV). Methods and results We prospectively enrolled participants with PH‐HFpEF (n = 14), pulmonary arterial hypertension (PAH; n = 13), and controls (n = 8). All participants underwent high‐resolution cardiovascular magnetic resonance, and case subjects (PH‐HFpEF and PAH) additionally underwent right heart catheterization. T1 mapping was performed using high‐resolution modified look‐locker inversion recovery with a 1 × 1 mm2 in‐plane resolution. RV free wall T1 values were quantified, and ECV was calculated. Participants with PH‐HFpEF were older and carried higher rates of hypertension and obstructive sleep apnoea than those with PAH. While RV ECV was similar between PH‐HFpEF and PAH (33.1 ± 8.0 vs. 34.0 ± 4.5%; P = 0.57), total pulmonary resistance was lower in PH‐HFpEF compared with PAH [PH‐HFpEF: 5.68 WU (4.70, 7.66 WU) vs. PAH: 8.59 WU (8.14, 12.57 WU); P = 0.01]. RV ECV in PH‐HFpEF was associated with worse indices of RV structure (RV end‐diastolic volume: r = 0.67, P = 0.01) and RV function (RV free wall strain: r = 0.59, P = 0.03) but was not associated with RV afterload (total pulmonary resistance: r = 0.08, P = 0.79). Conversely, there was a strong correlation between RV ECV and RV afterload in PAH (r = 0.57, P = 0.04). Conclusions Diffuse RV fibrosis, as measured by ECV, is present in PH‐HFpEF and is associated with adverse RV structural and functional remodelling but not degree of pulmonary vasculopathy. In PH‐HFpEF, diffuse RV fibrosis may occur out of proportion to the degree of RV afterload.

Abstract Background Chronic iliac vein occlusion can result in the development of a variety of collateral venous drainage pathways. While several drainage pathways have been well documented, autologous sapheno-saphenous bypass collateral drainage has not been described. This novel collateral drainage pathway is readily visible on cross sectional imaging, may serve as a diagnostic indicator of chronic obstructive venous pathology, and may hint at the underlying etiology. Case presentation This brief report depicts findings and technical considerations in two cases of venous recanalization of sapheno-saphenous collaterals in the setting of chronic unilateral iliac vein occlusion. In both cases at one-month follow-up, the patients’ pain had resolved, edema had improved, and computed tomographic venography demonstrated stent patency. Conclusions Identification of a sapheno-saphenous collateral can provide an important clue to the underlying venous obstructive pathology, therefore guiding corrective intervention.

BackgroundWall shear stress (WSS) is a stimulus for vessel wall remodeling. Differences in ascending aorta (AAo) hemodynamics have been reported between bicuspid aortic valve (BAV) and tricuspid aortic valve patients with aortic dilatation, but the confounding impact of aortic valve stenosis (AS) is unknown. Methods and ResultsFive hundred seventy‐one subjects underwent 4‐dimensional flow magnetic resonance imaging in the thoracic aorta (210 right‐left BAV cusp fusions, 60 right‐noncoronary BAV cusp fusions, 245 tricuspid aortic valve patients with aortic dilatation, and 56 healthy controls). There were 166 of 515 (32%) patients with AS. WSS atlases were created to quantify group‐specific WSS patterns in the AAo as a function of AS severity. In BAV patients without AS, the different cusp fusion phenotypes resulted in distinct differences in eccentric WSS elevation: right‐left BAV patients exhibited increased WSS by 9% to 34% (P

Rosai-Dorfman disease (RDD) is a rare entity that usually involves the lymph nodes but extranodal involvements have been seen in numerous cases, although RDD with cardiovascular involvement is extremely rare. We describe a case of a young male who presented with intermittent palpitations and was found to have a left atrium mass. Our case not only emphasizes the rarity of the above lesion but also highlights the importance of modern-day imaging like computed tomography, Cardiac Magnetic Resonance Imaging (CMRI), and PET scan in characterizing such nonspecific lesions and directing appropriate line of treatment. RDD should be considered as one of the differentials even for isolated cardiac lesions.

Raghib Syndrome is a rare developmental complex, which consists of persistence of the left superior vena cava (PLSVC) along with coronary sinus ostial atresia and atrial septal defect. This Raghib complex anomaly has also been associated with other congenital malformations including ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. Our case demonstrates an isolated PLSVC draining into the left atrium along with coronary sinus atresia in a young patient presenting with cryptogenic stroke without the atrial septal defect. Majority of the cases reported in the literature were found to have the lesion during the postmortem evaluation or were characterized at angiography and/or echocardiography. We stress the importance of modern day imaging like the computed tomography (CT) angiography and cardiac MRI in diagnosis and surgical management of such rare lesions leading to cryptogenic strokes.

BackgroundPhysician engagement has become a key metric for healthcare leadership and is associated with better healthcare outcomes. However, engagement tends to be low and difficult to measure and improve. This study sought to efficiently characterize the professional cultural dynamics between physicians and administrators at an academic hospital and how those dynamics affect physician engagement.Materials and methodsA qualitative mixed methods analysis was completed in 6 weeks, consisting of a preliminary analysis of the hospital system's history that was used to purposefully recruit 20 physicians across specialties and 20 healthcare administrators across management levels for semi-structured interviews and observation. Participation rates of 77% (20/26) and 83% (20/24) were achieved for physicians and administrators, respectively. Cohorts consisted of equal numbers of men and women with experience ranging from 1 to 35 years within the organization. Field notes and transcripts were systematically analyzed using an iterative inductive-deductive approach. Emergent themes were presented and discussed with approximately 400 physicians and administrators within the organization to assess validity and which results were most meaningful.Results & discussionThis investigation indicated a professional cultural disconnect was undermining efforts to improve physician engagement. This disconnect was further complicated by a minority (10%) not believing an issue existed and conflicting connotations not readily perceived by participants who often offered similar solutions. Physicians and administrators felt these results accurately reflected their realities and used this information as a common language to plan targeted interventions to improve physician engagement. Limitations of the study included its cross-sectional nature with a modest sample size at a single institution.ConclusionsA qualitative mixed methods analysis efficiently identified professional cultural barriers within an academic hospital to serve as an institution-specific guide to improving physician engagement.

PURPOSE:In response to limited physician adoption of various healthcare initiatives, we sought to propose and assess a novel approach to policy development where one first characterizes diverse physician groups' common interests, using a medical student and constructivist grounded theory. METHODS:In 6 months, a medical student completed 36 semi-structured interviews with interventional radiologists, gynecologists, and vascular surgeons that were systematically analyzed according to constructivist grounded theory to identifying common themes. Common drivers of clinical decision making and professional values across 3 distinct specialty groups were derived from physicians' descriptions of their clinical decision making, stories, and concerns. RESULTS:Common drivers of clinical decision making included patient preference/benefit, experience, reimbursement, busyness/volume, and referral networks. Common values included honesty, trustworthiness, loyalty, humble service, compassion and perseverance, and practical wisdom. Although personal gains were perceived as important interests, such values were easily sacrificed for the good of patients or other non-financial interests. This balance was largely dependent on the incentives and security provided by physicians' environments. CONCLUSIONS:Using a medical student interviewer and constructivist grounded theory is a feasible means of collecting rich qualitative data to guide policy development. Healthcare administrators and medical educators should consider incorporating this methodology early in policy development to anticipate how value differences between physician groups will influence their acceptance of policies and other broad healthcare initiatives.