Faculty Bibliography

Cardiomyopathies represent a diverse group of heart diseases that can be broadly classified into ischemic and nonischemic etiologies, each requiring distinct diagnostic approaches. Noninvasive imaging modalities, such as computed tomography (CT) and magnetic resonance imaging (MRI), play a pivotal role in the diagnosis, risk stratification, and prognosis of these conditions. This paper reviews the characteristic CT and MRI findings associated with ischemic cardiomyopathy (ICM) and nonischemic cardiomyopathy (NICM), focusing on their ability to provide detailed anatomical, functional, and tissue characterization. In ICM, CT and MRI reveal myocardial scarring, infarct size, and coronary artery disease, while MRI further distinguishes tissue viability through late gadolinium enhancement (LGE). Conversely, nonischemic cardiomyopathies demonstrate a wide array of findings, with MRI's LGE pattern analysis being particularly critical for identifying specific subtypes, such as restrictive, hypertrophic, or dilated cardiomyopathies. By comparing the strengths and limitations of these modalities, this paper highlights their complementary roles in improving diagnostic accuracy, risk stratification, prognosis, and therapeutic decision making in both ischemic and nonischemic cardiomyopathies.

Thanks to recent developments in Cardiovascular magnetic resonance (CMR), cardiac diffusion-weighted magnetic resonance is fast emerging in a range of clinical applications. Cardiac diffusion-weighted imaging (cDWI) and diffusion tensor imaging (cDTI) now enable investigators and clinicians to assess and quantify the 3D microstructure of the heart. Free-contrast DWI is uniquely sensitized to the presence and displacement of water molecules within the myocardial tissue, including the intra-cellular, extra-cellular and intra-vascular spaces. CMR can determine changes in microstructure by quantifying: a) mean diffusivity (MD) -measuring the magnitude of diffusion; b) fractional anisotropy (FA) - specifying the directionality of diffusion; c) helix angle (HA) and transverse angle (TA) -indicating the orientation of the cardiomyocytes; d) E2A and E2A mobility - measuring the alignment and systolic-diastolic mobility of the sheetlets, respectively. This document provides recommendations for both clinical and research cDWI and cDTI, based on published evidence when available and expert consensus when not. It introduces the cardiac microstructure focusing on the cardiomyocytes and their role in cardiac physiology and pathophysiology. It highlights methods, observations and recommendations in terminology, acquisition schemes, post-processing pipelines, data analysis and interpretation of the different biomarkers. Despite the ongoing challenges discussed in the document and the need for ongoing technical improvements, it is clear that cDTI is indeed feasible, can be accurately and reproducibly performed and, most importantly, can provide unique insights into myocardial pathophysiology.

BACKGROUND/UNASSIGNED:There is controversy about risk of malignant arrhythmias and stroke in patients with apical aneurysms in hypertrophic cardiomyopathy (HCM). OBJECTIVES/UNASSIGNED:The aim of this study was to estimate the associations of aneurysm size and major HCM risk factors with the incidence of lethal and potentially lethal arrhythmias and to estimate incidence of unexplained stroke. METHODS/UNASSIGNED:In 108 patients (age 57.4 ± 13.5 years, 37% female) from 3 HCM centers, we assessed American Heart Association/American College of Cardiology guidelines risk factors and initial aneurysm size by echocardiography and cardiac magnetic resonance imaging and assessed outcomes after median 5.9 (IQR: 3.7-10.0) years. RESULTS/UNASSIGNED:and also without risk factors VT, VF, or SCD occurred in only 2.5%. Clinical atrial fibrillation (AF) was prevalent, occurring in 49 (45%). Stroke was commonly associated with AF. Stroke without conventional cause had an incidence of 0.5%/year. Surgery in 19% was effective in reducing symptoms. VT ablation and surgery were moderately effective in preventing recurrent VT. CONCLUSIONS/UNASSIGNED:Risk factors and aneurysm size were associated with subsequent VT, VF, or SCD. Patients with aneurysms in the lowest tercile of size have a low cumulative 5-year risk. Clinical AF occurred frequently. Stroke prevalence in absence of known stroke etiologies is uncommon and comparable to risk of severe bleeding.

While there have been many descriptions of characteristic motion findings in left bundle branch block (LBBB), there are few published descriptions of such findings in right bundle branch block (RBBB). The purpose of this study was to assess the frequency of particular regional motion findings in cardiac magnetic resonance imaging (CMR) studies of patients with RBBB, compared with normal subjects. We focused on three distinctive motion patterns that can be seen in RBBB during early systole: delayed apex-ward motion of the RV base, "reverse septal flash", and "basal bulge". The presence and relative magnitude of these findings were independently scored by four experienced observers, in 3-chamber and 4-chamber CMR cines, for both normal subjects and patients with RBBB. These motion patterns were found to be strongly associated with the presence of RBBB. While only moderately sensitive, they were quite specific for RBBB, when present. In particular, with ROC analysis, a combined feature set of the findings in the 4-chamber view had an area under the curve of 0.81.This previously undescribed set of RBBB-associated early-systolic regional motion features (delayed apex-ward motion of the RV base, "reverse septal flash", and "basal bulge") is strongly suggestive of RBBB when present, particularly in the 4-chamber view. Although here evaluated with CMR, it is also likely to be associated with RBBB when seen with other cardiac imaging modalities.

BACKGROUND:Late gadolinium enhancement (LGE) is a valuable part of cardiac magnetic resonance imaging (CMR). In particular, inversion-recovery imaging of LGE, with nulling of the signal from reference areas of myocardium, can have a distinctive pattern in some patients with cardiac amyloid, including both diffuse (relatively faint) subendocardial LGE and a relatively dark appearance of the blood. However, the underlying reasons for this distinctive appearance have not previously been well investigated. Pharmacokinetic modeling of myocardial contrast enhancement kinetics can potentially provide insight into the mechanisms of the distinctive LGE appearance that can be seen in cardiac amyloid, as well as why it may be unreliable in some patients. METHODS:An interactive three-compartment pharmacokinetic model of the dynamics of myocardial contrast enhancement in CMR was implemented, and used to simulate LGE dynamics in normal, scar, and cardiac amyloid myocardium; the results were compared with previously published values. RESULTS:The three-compartment model is able to capture the qualitative features of LGE, in patients with cardiac amyloid. In particular, the characteristic "dark blood" appearance of PSIR images of LGE in cardiac amyloid is seen to likely primarily reflect expansion of the extravascular extracellular space (EES) by amyloid in the "reference" myocardium; the cardiac amyloid contrast enhancement dynamics also reflect expansion of the body EES. CONCLUSION:The distinctive appearance of LGE in cardiac amyloid is likely due to a combination of diffuse expansion by amyloid of the EES of the reference myocardium and of the body EES.

Background: Obesity is prevalent among patients with hypertrophic cardiomyopathy (HCM). Obese HCM patients have greater wall thickness, LV mass, worse hemodynamic function and NYHA class. Weight loss may favorably influence the HCM phenotype. Case summary: We describe six patients with hypertrophic cardiomyopathy who lost weight through diet and lifestyle changes (n = 4) or bariatric surgery (n = 2). Radiographic imaging, with cardiac MRI or CT scan, was performed before and after their weight loss. There was a mean decrease in LV mass and indexed LV mass, and a mean numerical decrease in mean wall thickness in up to 14 out of 18 LV segments. There was also NYHA class reduction in symptoms. Discussion: In this case series, we have shown that substantial weight loss in HCM patients can be associated with a decrease in LV mass, wall thickness and improvement in symptoms. These observations indicate the potential for positive remodeling of the heart by weight loss. Prospective studies of the benefits of weight loss in HCM are needed.

BACKGROUND:Apical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%. OBJECTIVES/OBJECTIVE:The authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms. METHODS:The authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities). RESULTS:]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying. CONCLUSIONS:The great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

Modern medical imaging techniques, such as ultrasound (US) and cardiac magnetic resonance (MR) imaging, have enabled the evaluation of myocardial deformation directly from an image sequence. While many traditional cardiac motion tracking methods have been developed for the automated estimation of the myocardial wall deformation, they are not widely used in clinical diagnosis, due to their lack of accuracy and efficiency. In this paper, we propose a novel deep learning-based fully unsupervised method, SequenceMorph, for in vivo motion tracking in cardiac image sequences. In our method, we introduce the concept of motion decomposition and recomposition. We first estimate the inter-frame (INF) motion field between any two consecutive frames, by a bi-directional generative diffeomorphic registration neural network. Using this result, we then estimate the Lagrangian motion field between the reference frame and any other frame, through a differentiable composition layer. Our framework can be extended to incorporate another registration network, to further reduce the accumulated errors introduced in the INF motion tracking step, and to refine the Lagrangian motion estimation. By utilizing temporal information to perform reasonable estimations of spatio-temporal motion fields, this novel method provides a useful solution for image sequence motion tracking. Our method has been applied to US (echocardiographic) and cardiac MR (untagged and tagged cine) image sequences; the results show that SequenceMorph is significantly superior to conventional motion tracking methods, in terms of the cardiac motion tracking accuracy and inference efficiency. Code will be available at <uri>https://github.com/DeepTag/SequenceMorph</uri>.