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This focused review will highlight the results of HELIOS-B, the first randomized outcomes trial evaluating a gene silencing treatment for transthyretin cardiac amyloidosis (ATTR-CM). In HELIOS-B, vutrisiran was tested against placebo and demonstrated a 28% reduction in the composite of all-cause mortality and recurrent cardiovascular events. Additionally, there were clinically significant benefits on the 6-min walk test, Kansas City Cardiomyopathy Questionnaire, and NYHA class. Discontinuation rates and adverse events were similar between treatment and control arms, suggesting that vutrisiran is well tolerated. In this review, these promising results are explored and compared with other treatment trials in ATTR-CM.

ObjectiveCardiac amyloidosis (CA) is a rapidly progressive disease that portends poor prognosis. Our objective was to evaluate the prognostic impact of relative regional strain ratio (RRSR, a measure of the relative apical sparing of longitudinal strain (LS)) in CA.MethodsThis is a retrospective study evaluating 97 patients with CA from 2004 to 2013. Patients were included if they met criteria for CA based on endomyocardial biopsy or advanced imaging criteria coupled with either extracardiac biopsy or genetic analysis. Baseline clinical and imaging data were collected and compared between light-chain amyloidosis (AL) (n=59) and transthyretin amyloidosis (ATTR) (n=38) subtypes. RRSR was defined as the average apical LS divided by the sum of the average mid and basal LS values. A Cox proportional hazards model was used to assess the effects of clinical and echocardiographic characteristics, including RRSR, on the outcome of time to death or heart transplantation.ResultsDespite younger age, the AL subtype had a statistically significant association with the composite outcome as compared with ATTR (p=0.022). Log-transformed RRSR was independently associated with the composite outcome at 5 years (HR 2.45 (1.36 to 4.40), p=0.003). Patients with low ejection fraction and high RRSR had the worst prognosis. In multivariable analysis, RRSR remained predictive of the primary outcome (p=0.018). Addition of covariates related to systolic function (global LS and ejection fraction) to the model attenuated this effect.ConclusionsHigh RRSR is adversely prognostic in patients with cardiac amyloid. This novel tool is both diagnostic and prognostic and may have implications in management and suitability for treatment.

Post-systolic shortening index (PSI) is defined as myocardial shortening that occurs after aortic valve closure, and is an emerging measure of regional LV contractile dysfunction. PSI measurement variability amongst software vendor and its relationship with mechanical dyssynchrony and mechanical dispersion index (MDI) remains unknown. We evaluated PSI by speckle-tracking echocardiography from several vendors in patients with increased left ventricular wall thickness, and associations with MDI. This is a prospective cross-sectional study of 70 patients (36 hypertrophic cardiomyopathy [HCM], 18 cardiac amyloidosis and 16 healthy controls) undergoing clinically indicated echocardiography. PSI was measured using QLAB/aCMQ (Philips), QLAB/LV auto-trace (Philips), EchoPAC (GE), Velocity Vector Imaging (Siemens), and EchoInsight (EPSILON) software packages, and calculated as 100%x(post systolic strain-end-systole strain)/post systolic strain. There was a significant difference in mean PSI among controls 2.1±0.6%, HCM 6.1±2.6% and cardiac amyloidosis 6.8±2.7% (p <0.001). Variations between software vendors were significant in patients with pathologic increases in LV wall thickness (for HCM p = 0.03, for amyloidosis p = 0.008), but not in controls (p = 0.11). Furthermore, there were moderate correlations between PSI and both MDI (r = 0.77) and left ventricular global longitudinal strain (r = 0.69). PSI was greater in HCM and cardiac amyloidosis patients than controls, and a valuable tool for dyssynchrony evaluation, with moderate correlations to MDI and strain. However, there were significant variations in PSI measurements by software vendor especially in patients with pathological increase in LV wall thickness, suggesting that separate vendor-specific thresholds for abnormal PSI are required.

BackgroundTechnetium-based bone scintigraphy is rapidly becoming the most common non-invasive imaging tool in the diagnosis of Transthyretin cardiac amyloidosis (ATTR). Skeletal muscle uptake has been described with technetium-99m-3,3-diphosphono-1,2-propanodicarboxylic acid (TcDPD), and may account for masking of bony uptake. We sought to investigate skeletal muscle uptake of technetium-99m-pyrophosphate (TcPYP) in patients with ATTR.Methods and ResultsThis was a retrospective analysis of 57 patients diagnosed with ATTR who underwent TcPYP scintigraphy. Cardiac uptake was assessed on whole-body planar imaging using a semiquantitative scale (grades 0 to 3) and on single-photon emission computed tomography (SPECT) with CT attenuation correction using total myocardial counts per voxel after a 3-hour incubation. Skeletal muscle (psoas and biceps), vertebral body, LV myocardium, and blood pool mean counts were calculated. In the cohort (age 78 ± 9 years, 77% male, and 30% hereditary ATTR), there was no visualized tracer uptake in skeletal muscle or soft tissue on qualitative SPECT assessment. Total and blood pool-corrected uptake in the muscle groups were significantly less than myocardium and bone (P < 0.001). Blood pool-corrected muscle uptake was not associated with semiquantitative grade 3 vs 2 uptake (psoas P = 0.66, biceps P = 0.13) or presence of hereditary ATTR (psoas P = 0.43, biceps P = 0.69). As bony uptake decreased, there was no corresponding increase in skeletal muscle uptake.ConclusionsIn patients with ATTR cardiac amyloidosis, skeletal muscle uptake of TcPYP is minimal when assessed by qualitative and quantitative metrics, and is not significantly different in patients with grade 2 vs 3 semiquantitative uptake. The properties of this tracer may be different than TcDPD with respect to non-cardiac uptake.

Global longitudinal strain (GLS) by speckle-tracking echocardiography is a sensitive measure of regional left and right ventricular (LV and RV) dysfunction, before onset of overt systolic dysfunction. We sought to evaluate the prognostic utility of measuring LV-GLS and RV free wall strain (FWS) in low risk patients at 1 year after orthotopic heart transplantation (OHT). We retrospectively studied 96 OHT recipients (age 52 ± 14 years, 64% men) free of antibody-mediated rejection or moderate to severe coronary allograft vasculopathy (CAV, grade 2 to 3) at 1 year after transplant. LV-GLS and RV-FWS were calculated using EchoPAC software. Cox models were developed after adjusting for the Index for Mortality Prediction After Cardiac Transplantation (IMPACT) score (post-transplant risk score), with the primary outcome of death, moderate to severe CAV, or treated rejection. At 1 year after transplant, LV ejection fraction and RV fractional area change (FAC) were 58 ± 7% and 42 ± 10%, respectively. LV-GLS was −17.0 ± 3.3% and RV-FWS −16.4 ± 4.5%. At an average follow-up of 4.5 years, 28 patients met the primary end point (10 death, 5 vasculopathy, 17 rejection). In sequential Cox models, markers of RV function were associated with the primary outcome (RV-FAC, p = 0.012; RV-FWS, p = 0.022), while LV ejection fraction and LV-GLS were not. We conclude that in low risk patients 1 year after OHT, markers of RV function (RV-FAC and RV-FWS) are independently associated with incident rejection, CAV, and death. Markers of RV dysfunction could potentially be incorporated into risk scores and future prospective studies to risk stratify patients after OHT.

Acute decompensated heart failure is a significant source of morbidity and mortality in the USA. It is the most common reason for admission in the Medicare population and the greatest cause of hospital readmission in both medical and surgical patients. As many of these readmissions are considered preventable, providers and hospital systems are seeking novel strategies to reduce rehospitalization. Several specific interventions have been shown to decrease readmission for heart failure. However, these are typically narrow in scope, focusing on one aspect of patient care and providing a one-size-fits-all approach. We review the data and propose integrating some of these interventions into a comprehensive patient-centered model that is organized into six categories: quality of medical management, early reassessment, health literacy, neuropsychological status, financial means and functional status. By screening for deficiencies in each of these categories, providers and hospital systems can use resources more efficiently to make targeted interventions to improve health outcomes and mitigate readmissions.

Amyloidosis has often been referred to as a “great masquerader,” mimicking other systemic and cardiac diseases. As diagnostic techniques such as echocardiography with longitudinal strain, cardiac magnetic resonance imaging, and nuclear scintigraphy have advanced, identification of cardiac amyloidosis has become less daunting. This review covers the differential diagnosis and workup of patients with transthyretin cardiac amyloidosis, with a specific focus on developing a clinical suspicion through demographic, clinical, and echocardiographic features of the disease. The most common mimics of cardiac amyloidosis, i.e., conditions that likewise cause heart failure with preserved or mildly reduced ejection fraction, are also explored with respect to differential diagnosis, both for patients with normal and increased ventricular wall thickness. Ultimately, this review aims to demystify the diagnostic process by offering an algorithmic approach to the identification of transthyretin cardiac amyloidosis.

In independent studies, abnormal global longitudinal strain (GLS) and myocardial inflammation or scar detected by 18F-fludeoxyglucose positron emission tomography (FDG-PET) are associated with poor prognosis among patients with high likelihood for cardiac sarcoidosis. However, commonly used imaging modalities have not been evaluated in the same population. Our goals were to examine the relation between GLS and FDG-PET, and to evaluate the incremental prognostic value of these imaging techniques for predicting major adverse cardiac events (MACE) in patients suspected to have cardiac sarcoidosis. We identified patients with systemic sarcoidosis who underwent an echocardiogram and FDG-PET within 60 days. Regional strain (average of base, mid, and apical segmental strains from each of 6 wall regions) was calculated and compared with regional FDG-PET findings. The associations among GLS, FDG-PET findings, and MACE (defined as death, ventricular tachycardia, heart failure hospitalization, or transplantation) were evaluated using a Cox model. Of 84 patients, 51 had abnormal FDG-PET. GLS was impaired in patients with abnormal versus normal FDG-PET (−14.2 ± 4.7% vs −17.9 ± 3.5%, p <0.01). After adjusting for clinical risk factors, both GLS and the number of segments with abnormal perfusion and metabolism on FDG-PET were associated with adverse cardiac events (p <0.01 for both). In conclusion, GLS and regional LS are impaired in patients with abnormal perfusion and metabolism detected using FDG-PET. Additionally, both GLS and abnormal FDG-PET have incremental prognostic value for predicting MACE in patients with systemic sarcoidosis.

Purpose of Review This article summarizes findings seen in various cardiomyopathies on myocardial perfusion imaging (MPI) with positron emission tomography (PET). Recent Findings MPI is the cornerstone for evaluation of coronary ischemia, and technological advancements have yielded improved imaging quality and reduction in radiation exposure, particularly with PET. Multi-specialty guidelines and appropriate use criteria provide guidance on utilization of PET MPI in various scenarios related to evaluation of chest pain, new onset cardiomyopathy, and other scenarios where coronary ischemia should be assessed. Various non-ischemic cardiomyopathies such as septal and apical hypertrophic cardiomyopathy, amyloidosis, sarcoidosis, takotsubo, and dilated cardiomyopathy have typical imaging findings on PET MPI and can be identified if these patterns are understood. Summary It is essential to recognize specific imaging patterns in non-ischemic cardiomyopathies which may aide in diagnosis. Ultimately, multimodality imaging, including echocardiography and cardiac magnetic resonance, complement PET MPI in diagnosing and guiding treatment options for these conditions.