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Objectives This study investigated an enriched cohort of patients with heart failure and preserved ejection fraction (HFpEF) in TOPCAT (Treatment of Preserved Cardiac Function Heart Failure With an Aldosterone Antagonist) with an echocardiographic phenotype of cardiac amyloidosis. Background There is a high prevalence of increased interventricular septal (IVS) thickness and decreased mitral annular systolic (s′) velocity in cardiac amyloidosis. In addition, clinical trials of neurohormonal blockade are missing in this population. Methods TOPCAT randomized patients with HFpEF to spironolactone or placebo therapy with a primary endpoint of cardiovascular death, HF hospitalization, or aborted cardiac arrest. Patients with IVS and s′ velocity measurements were included, and adjusted Cox models assessed the effect of echocardiographic variables and spironolactone on the primary endpoint. Results Among 590 patients, mean s′ velocity was 6.4 ± 2.1 cm/s and IVS thickness was 1.2 ± 0.2 cm. The enriched cohort with characteristics of cardiac amyloidosis (s′ velocity ≤6 cm/s and IVS thickness ≥1.2 cm) included 135 patients (23% of the cohort). After a median follow-up of 2.6 years (1.5-3.9 years), these patients had the worst prognosis (adjusted HR: 2.10; 95% CI: 1.26-3.50; P = 0.004). Both s′ velocity and IVS thickness were individually associated with the primary endpoint, and abnormalities in these parameters were additive as lower s′ velocity was particularly prognostic in those with greater IVS thickness (interaction: P = 0.013). Spironolactone was associated with improved outcomes in the overall cohort (P = 0.024), and patients in the enriched cohort had a benefit similar to that in other groups (interaction: P = 0.382). Conclusions An enriched subset of patients with structural and functional echocardiographic features of cardiac amyloidosis had the worst prognosis in the TOPCAT study, but they benefitted similarly from spironolactone therapy. Future studies of mineralocorticoid receptor antagonists in patients with cardiac amyloidosis are warranted.

Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy caused by the extracellular deposition of amyloid fibrils in the myocardium. Although cardiac amyloidosis patients primarily present with heart failure symptoms, arrhythmias and conduction system disease are frequently encountered. Atrial fibrillation (AF) is observed in up to 70% of patients at the time of diagnosis, and patients typically have controlled ventricular rates caused by concomitant conduction system disease. Thromboembolic risk is particularly high in patients with CA and AF, and left atrial thrombi have been observed even in the absence of clinically diagnosed AF. Atrioventricular nodal and infra-Hisian disease are common, and permanent pacemakers are frequently required. The use of implantable cardioverter-defibrillators in this population is controversial. This review summarizes the published data and therapeutic strategies surrounding arrhythmias and conduction system disease with the goal of aiding clinicians managing the clinical complexities of CA., Central Illustration, Highlights • Cardiac arrhythmias and conduction system disease are common in CA. • Management of AF is complicated by difficulty in maintaining sinus rhythm and thromboembolic complications. • Atrioventricular nodal and infra-Hisian disease are common, and the optimal choice of device is controversial. • Prospective studies are required to establish optimal management strategies for arrhythmias and conduction disease.

Cardiogenic shock (CS) is associated with high mortality and often requires involvement of a multidisciplinary provider team to deliver timely care. Care coordination is more difficult on weekends, which may lead to a delay in care. We sought to assess the effect of weekend admissions on outcomes in patients admitted with CS. Patients admitted with CS were identified from 2005 to 2014 in the National Inpatient Sample using ICD9 code 785.51. Baseline demographics, in-hospital procedures, and outcomes were obtained and compared by day of admission. A multivariable model was used to assess the impact of weekend admission on in-hospital mortality. A total of 875,054 CS admissions were identified (age 67.4 ± 15.1 years, 40.2% female, 72.1% Caucasian), with 23% of patients being admitted on weekends. Baseline co-morbidities were similar between groups. Weekend admissions were associated with higher in-hospital mortality (40.6% vs 37.5%) and cardiac arrest (20.3% vs 18.1%, p < 0.001 for both) consistently over the study period. Use of temporary and permanent mechanical support devices and heart transplantation were slightly less common for weekend admissions. In a multivariable model adjusting for relevant confounders, weekend admission was associated with a 10% increased mortality in patients with CS. In conclusion, patients with CS admitted on weekends had higher in-hospital mortality and were slightly less likely to receive mechanical support and advanced therapies compared with those admitted on weekdays. Future studies and health system initiatives should focus on rectifying these disparities with around-the-clock multidisciplinary coordinated care for CS.

BACKGROUND: The role of myocardial perfusion imaging (MPI) in patients with suspected coronary artery disease (CAD) presenting with syncope is controversial. We aimed to determine diagnostic yield of MPI for evaluation of syncope in patients without known CAD, as a function of pre-test patient risk and test modality (PET vs SPECT). METHODS: Between 1/2010 and 12/2016, 1324 consecutive patients presenting with syncope without known CAD underwent MPI with PET (n=640) or SPECT (n=684). Rates of abnormal MPI (summed difference score (SDS) >2 or left ventricular ejection fraction (LVEF) reserve ≤0 for PET and SDS > 2 or post-stress LVEF ≤ 45% for SPECT) were determined among patients stratified by pre-test risk. In patients who were referred for coronary angiography, diagnostic yield of obstructive CAD was calculated in the overall cohort as well as in a propensity-matched cohort compared to patients without syncope. RESULTS: Abnormal MPI was noted in 36.5% (201/551) of patients who had PET compared with 13.0% (87/671) who had SPECT (p

Aortic stenosis (AS) and cardiac amyloidosis (CA) are disease processes that are more prevalent in the aged population. Through active calcification and inflammation, AS eventually leads to signifi...

Prior studies with single-photon emission computed tomography (SPECT) myocardial perfusion imaging (MPI) have shown a survival benefit with early revascularization in patients with10% to 12.5% ischemic myocardium. The relationship among positron emission tomography (PET)-derived extent of ischemia, early revascularization, and survival is unknown.The purpose of this study was to evaluate the association among percent ischemia on PET MPI, revascularization, and survival.A total of 16,029 unique consecutive patients who were undergoing Rubidium-82 rest-stress PET MPI from 2010 to 2016 were included. Patients with known cardiomyopathy and nondiagnostic perfusion results were excluded. Percent ischemic myocardium was estimated from a 17-segment model. Propensity scoring was used to account for nonrandomized referral to early revascularization (90 days of PET). A Cox model was developed, adjusting for propensity scores for early revascularization and percent ischemia, and an interaction between ischemia and early revascularization was tested.Median follow-up was 3.7 years. Overall, 1,277 (8%) patients underwent early revascularization and 2,493 (15.6%) died (738 cardiac). Nearly 37% of patients (n = 5,902) had ischemia, with 13.5% (n = 2,160) having ≥10%. In propensity-adjusted analyses, there was a significant interaction between ischemia and early revascularization (p 0.001 for all-cause and cardiac death), such that patients with greater ischemia had improved survival with early revascularization, with a potential ischemia threshold at 5% (upper limit 95% confidence interval at 10%). There was no differential association between ischemia and early revascularization on death based on history of known coronary artery disease (interaction p = 0.72).In a contemporary cohort of patients undergoing PET MPI, patients with greater ischemia had a survival benefit from early revascularization. On exploratory analyses, this threshold was lower than that previously reported for SPECT. These findings require future validation in prospective cohorts or trials.

Aims Positron emission tomography (PET) myocardial perfusion imaging (MPI) can non-invasively measure myocardial blood flow reserve (MBFR). We aimed to examine whether MBFR identifies patients with a survival benefit after revascularization, helping to guide post-test management. Methods and results We examined all-cause mortality in 12 594 consecutive patients undergoing Rb82 rest/stress PET MPI from January 2010 to December 2016, after excluding those with cardiomyopathy, prior coronary artery bypass surgery (CABG), and missing MBFR. Myocardial blood flow reserve was calculated as the ratio of stress to rest absolute myocardial blood flow. A Cox model adjusted for patient and test characteristics, early revascularization (percutaneous coronary intervention or CABG ≤90 days of MPI), and the interaction between MBFR and early revascularization was developed to identify predictors of all-cause mortality. After a median follow-up of 3.2 years, 897 patients (7.1%) underwent early revascularization and 1699 patients (13.5%) died. Ischaemia was present in 4051 (32.3%) patients, with 1413 (11.2%) having ≥10% ischaemia. Mean MBFR was 2.0 ± 1.3, with MBFR Conclusion Myocardial blood flow reserve on PET MPI is associated with all-cause mortality and can identify patients who receive a survival benefit with early revascularization compared to medical therapy. This may be used to guide revascularization, and prospective validation is needed.

Funding Acknowledgements Type of funding sources: None. Background Both the Coronary Artery Calcium Score (CACS), a non-invasive surrogate for atherosclerotic burden, and reduced myocardial blood flow reserve (MBFR) with normal perfusion, a non-invasive surrogate for coronary vasomotor dysfunction, independently predict future cardiovascular events. The relationship between CACS and MBFR, and potential clinical factors affecting it, is not well understood. Methods Among 9467 consecutive patients without known history of CAD who had normal perfusion on 82Rb PET-CT and a concomitantly measured CACS between 01/2010 - 06/2020 within our health system, we assessed the relationship between CACS and MBFR. Multiple linear regression was used to predict MBFR using CACS, adjusted for age, sex, BMI, risk factors, symptoms, resting LVEF and vital signs. Interactions of age, sex, diabetes, and symptoms with CACS were assessed to evaluate if they modified the relationship of CACS with MBFR. Results Mean age (SD) of the study cohort was 66.4 (12.6) years, 64% were women, 64% had chest pain and 47% had dyspnea. Reduced MBFR (0 in 74% of patients. There was a modest inverse correlation between MBFR and CACS, r= - 0.18, p = < 0.0001 (Figure). In adjusted analyses, CACS (β for CAC per 100 = -0.013 [95% CI: -0.015, -0.010]) was weakly associated with MBFR, and age, sex, diabetes, or symptoms did not modify this relationship (all interaction p-values >0.1). Older age, female sex, presence of hypertension, diabetes, dyspnea, lower LVEF, higher baseline HR and higher CACS independently predicted reduced MBFR, but explained only 20% of the variance in MBFR (R2 =0.20). Conclusion There is a weak relationship between CACS and MBFR, which is not modified by age, sex, symptoms, or other CV risk factors. Coronary calcium burden does not completely reflect the overall disease activity within the coronary circulation, and measures of coronary vasomotor function such as MBFR may offer complementary information on CAD risk to that provided by the total burden of calcified atherosclerosis.

Introduction With limited options for rate control of atrial fibrillation in the setting of low-output heart failure (HF), digoxin may appear to be a reasonable option for patients with cardiac amyloidosis (CA). However, current guidelines, based on historical case reports and an in vitro study, state that digoxin should generally be avoided in CA due to increased sensitivity for digoxin toxicity. A recent study of 107 patients with AL amyloidosis given digoxin showed relative safety with cautious use, with 11% of patients developing significant arrhythmias. We describe our institution's experience with digoxin in patients with CA and the suspected digoxin-related arrhythmias and toxicity. Methods This is a retrospective examination of patients diagnosed with CA seen between November 1995 and October 2018 at the Cleveland Clinic. CA patients were screened for a history of digoxin use; patients with less than 7 days of digoxin use were excluded, as well as those with incomplete medical records. Suspected digoxin-related arrhythmias and toxicity events were defined as: unexplained altered mental status, use of digoxin immune fab, ventricular tachycardia/fibrillation, paroxysmal atrial tachycardia with 2:1 block, new-onset junctional rhythm, and symptomatic bradycardia. Results Of 756 patients with CA identified, 71 patients were treated with digoxin for ≥7 days: 42 with transthyretin (ATTR) CA and 29 with light chain (AL) CA. Median duration of digoxin therapy was 10 months (IQR 3-38) for the ATTR-CA cohort and 2 months (IQR 1-7) for the AL-CA cohort. Digoxin was used for rate control of atrial fibrillation in 66% of the total population (64% ATTR, 69% AL) and management of symptomatic HF in 34% (36% ATTR, 31% AL). Suspected digoxin-related arrhythmias and toxicity occurred in 10%, described in detail in Table 1. 21% of the digoxin population died while on therapy, but only one death may have been attributed to digoxin use or toxicity. Conclusions In carefully-selected patients with CA treated with digoxin, 90% did not have a digoxin-related adverse event over a median treatment duration of 10 months. 10% had an adverse event potentially attributable to digoxin, with one death that may or may not have been related. Despite current guidelines, digoxin may be used cautiously in selected patients with ATTR- or AL-CA who have no good options for rate control in atrial fibrillation or for low output heart failure. Risks and benefits of using digoxin in CA should be evaluated on a patient by patient basis.

Objectives This study sought to evaluate the incremental value of quantifying the extent and severity of myocardial perfusion and 18F-labeled fluorodeoxyglucose (FDG) abnormalities in predicting adverse outcomes among patients with suspicion for cardiac sarcoidosis (CS). Background Positron emission tomography (PET) with FDG is a key component of the noninvasive assessment of patients with suspected CS. However, the optimal method for image interpretation has not been defined. Methods A retrospective analysis was performed of 203 patients who underwent perfusion and FDG-PET imaging to evaluate for CS. Imaging findings were scored by conventional 3-category methods (normal perfusion and metabolism, abnormal perfusion or metabolism, abnormal perfusion and metabolism) and by summed scores using the 17-segment model to represent extent and severity of disease. Heterogeneity of metabolism was quantified using the coefficient of variation (SD divided by the mean) of FDG uptake. Multivariable Cox models were developed to assess associations between imaging findings and adverse events (death, heart transplant, or ventricular arrhythmia requiring defibrillation). Results The indication for FDG-PET was ventricular arrhythmia in 69 (34%), heart block in 16 (8%), cardiomyopathy in 54 (27%), and other indications in 64 (32%). There were 63 patients who developed adverse events over a mean follow-up of 1.8 years. After robust adjustment, only the summed score in segments with a perfusion–metabolism mismatch and the coefficient of variation were important prognostically (p = 0.029 and p = 0.041, respectively). Conclusions Quantitative measures of extent and severity of perfusion–metabolism mismatch and coefficient of variation of FDG uptake provide an incremental prognostic advantage in patients undergoing FDG-PET for CS. These results support the use of a more detailed analysis of imaging findings, as is conventional in coronary artery disease.

Purpose To determine differences in donor transmitted atherosclerosis or rapidly progressive Cardiac Allograft Vasculopathy at 1-year post cardiac transplantation in patients with congenital heart disease. Methods This is a retrospective study of patients who underwent heart transplantation at a single center from 2009-2016 who had subsequent coronary angiography with protocol-driven IVUS assessment of the left anterior descending artery. Non-congenital cardiac transplantation patients (N=237) were compared to a cohort of patients with adult congenital heart disease (N=12). Five IVUS sections were prospectively analyzed for intimal thickness. Donor transmitted atherosclerosis was defined as maximum intimal thickness ≥0.5 mm at baseline and rapidly progressive CAV as an increase in maximum intimal thickness by ≥0.5 mm at 1-year. Results Donor transmitted atherosclerosis was present in 121 (51.1%) patients with non-congenital etiology vs. 7 (58.3%) patients with congenital etiology. Rapidly progressive CAV at 1-year post-transplantation was present in 54 (23.0%) vs. 2 (18.2%) patients, respectively. Over a median follow up of 6.1 years, 39 patients died or required re-transplantation. ACHD as an etiology of heart failure was not associated with age- and sex-adjusted outcomes. In a subsequent analysis, there were 45 patients who reached a composite outcome of coronary revascularization, epicardial CAV, or cardiovascular death. Likewise, there was no association between ACHD and this composite endpoint. Conclusion Pre-transplant adult congenital heart disease was not associated with a significant difference in donor transmitted atherosclerosis or rapidly progressive CAV in patients with heart transplantation. Long term outcomes including all-cause mortality and cardiovascular events were not different in patients with versus without ACHD.

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.

Left ventricular mechanical dispersion (LVMD), the variability in the timing of segmental peak negative longitudinal strains, is associated with ventricular arrhythmias in patients with decreased left ventricular (LV) ejection fraction and may be useful in risk-stratifying patients with pathological

Background: Cardiomyopathies with increased left ventricular wall thickness such as cardiac amyloidosis, septal hypertrophic cardiomyopathy (HCM), and apical HCM exhibit characteristic regional longitudinal strain (LS) patterns. However, between-vendor agreement of segmental and regional LS has not been tested in these diseases. We sought to assess LS values among vendors in specific cardiomyopathies that exhibit regional strain variation: cardiac amyloidosis, septal HCM, and apical HCM. Methods: This was a prospective, cross-sectional study of 69 patients (18 amyloidosis, 30 septal HCM, 6 apical HCM, and 15 controls) who underwent clinically indicated outpatient echocardiography at the Cleveland Clinic. Peak systolic segmental, regional (basal, mid, and apical), and global LS were evaluated using GE (EchoPAC), Siemens (Velocity Vector Imaging), and Phillips (QLab) systems in the same imaging session. Between-vendor, differences were analyzed using correlation coefficients, Bland Altman plots, and a mixed model. Results: Global LS was highly correlated among the 3 software packages and most abnormal in patients with amyloidosis ( P P Conclusions: Global and regional variations in LS exist between-vendors in patients with cardiomyopathies with increased left ventricular wall thickness (amyloidosis, septal HCM, and apical HCM). It is important to be aware of these differences for diagnosis, prognosis, and serial examinations in these conditions.

Background The effect of implantable cardioverter defibrillators ( ICD ) in patients with continuous flow left ventricular assist devices ( LVAD s) on outcomes has not been evaluated in a randomized clinical trial. Methods and Results This is a retrospective single‐center study that included patients who underwent continuous flow LVAD implantation at the Cleveland Clinic between October 2004 and March 2017. Patients were evaluated according to the presence or absence of ICD at the time of LVAD insertion. Among 486 patients in the study cohort, 387 (79.6%) had an ICD before LVAD insertion. Patients with ICD before LVAD were older and had lower use of pre‐ LVAD inotropes, extracorporeal membrane oxygenation, and mechanical ventilation. There were 81 patients (21.4% of patients with ICD ) who required 93 procedures after LVAD : 74 generator exchanges, 12 lead revisions, and 7 complete system removals because of infection. Of the 99 patients without ICD , 52 (53%) underwent ICD implantation: 29 for primary prevention and 23 for secondary prevention. Patients were followed for a median of 401 (interquartile range 150–966) days. The presence of a pre‐ LVAD ICD was not associated with mortality in a multivariable model (hazard ratio 1.19, 95% CI 0.73–1.93, P =0.492), nor was the presence of an ICD at any point when analyzed as a time‐varying covariate (hazard ratio 1.05, 95% CI 0.50–2.20, P =0.907). Conclusions There is no apparent mortality benefit associated with an ICD in a contemporary cohort of patients with continuous flow LVAD s to balance considerable morbidity involving ICD ‐related procedures and complications.

Cardiac amyloidosis is an increasingly recognized cause of heart failure with preserved or mildly reduced ejection fraction with emerging treatment options. We sought to analyze the temporal trends and impact of hospital admissions in patients with amyloidosis. The National Inpatient Sample was queried to identify patients from 2005 to 2014 who were hospitalized with a diagnosis of amyloidosis using ICD9 codes. Trends over time of prevalence, demographics, co-morbidities, and outcomes were described. Propensity-matching was used to assess the impact of amyloidosis on in-hospital outcomes, including mortality. A total of 156,914 admissions in patients with amyloidosis (age 69.86 +/- 12.33 years, 45.7% female, 68.5% Caucasian) were identified. Hospitalizations more than doubled with a peak of 21,740 per year and 62 per 100,000 admissions in 2014. Over time, patients admitted with amyloidosis were older and more likely to have co-morbid medical conditions. A diagnosis of heart failure was present in 34.7% of patients, increased over time (p = 0.001), and was associated with further morbidity and mortality. In a propensity-matched analysis, patients admitted with amyloidosis had a longer length of stay (7.5 vs 6.2 days), were less likely to be discharged home (43.6% vs 48.7%), and were more likely to die during the hospitalization (7.4% vs 4.9%, p0.001 for all). In conclusion, inpatient hospitalizations in the United States in patients with amyloidosis have increased over time and are associated with high morbidity and mortality, particularly when there is concomitant heart failure.

Rubidium-82 PET MPI measures of rest and peak stress perfusion, myocardial flow reserve (MFR), and changes in ejection fraction (LVEF). We compared the relative association of MFR, perfusion defects, and LVEF reserve with prognosis in ICM and NICM. Between 2010-2016, 1,679 consecutive patients with

MPI evaluates myocardial perfusion defect (PD) and changes in blood flow (MBF) that occur early in the ischemic cascade, eventually leading to angina. Few data exist on link between MPI findings and health status. Health status was collected using the Seattle Angina Questionnaire (SAQ-7, 0-100

PET Myocardial Perfusion Imaging (MPI) uniquely measures myocardial blood flow in addition to perfusion and function. The prevalence of abnormal PET MPI, if it helps guide treatment and its relation to outcomes among asymptomatic patients (pts) with diabetes (DM) is not well understood. We included

AimsImplantable cardioverter-defibrillators (ICDs) implantation in heart failure (HF) patients with reduced ejection fraction improves survival by reducing mortality secondary to arrhythmic events. Whether advanced HF patients treated with continuous-flow left ventricular assist devices (CF-LVADs) derive similar benefit is controversial.Methods and resultsWe searched PubMed, Cochrane Central Register of Controlled Trials, Embase, and Scopus from inception through November 2018 for studies examining the association between ICD implantation and all-cause mortality in patients with advanced HF and CF-LVADs. Analyses were performed using a random-effects model. Hazard ratios (HRs) were calculated with 95% confidence intervals (CIs). Heterogeneity and publication bias were formally assessed, using I2 and funnel plots, respectively. Eight observational studies with a total of 6416 patients (ICD group = 3450, no ICD group = 2966) met inclusion criteria. The majority of patients (84.6%) came from the two largest observational studies. There was no difference in mortality in the ICD and no ICD groups (HR 0.96, 95% CI 0.73–1.27, P = 0.79, I2 = 42%), and ICD implantation post-CF-LVAD was not associated with an improvement in mortality (HR 0.87, 95% CI 0.48–1.57, P = 0.64, I2 = 0%). Additionally, there was no significant difference in the likelihood of transplantation (HR 1.10, 95% CI 0.93–1.30, P = 0.28, I2 = 26%) or non-mortality adverse events between the two groups.ConclusionImplantable cardioverter-defibrillator use was not associated with improved survival in advanced HF patients with CF-LVADs. These findings underscore the need to formally study the efficacy of ICDs in this population in a dedicated randomized controlled study.

The prevalence of cardiac sarcoidosis has exponentially increased over the past decade, primarily due to increased awareness and diagnostic modalities for the disease entity. Despite an expanding patient cohort, the optimal management of cardiac sarcoidosis remains yet to be established with a significant lack of prospective trials to support current practice. Corticosteroids remain first-line treatment of this disorder, and we recommend that immunosuppressive therapy should be initiated in all patients diagnosed with cardiac sarcoidosis. Additional pharmacotherapy may be necessary based on disease manifestations and response to treatment. The use of nuclear imaging with 18fluorodeoxyglucose (18FDG) positron emission tomography (PET) to guide treatment has become more common, but lacks rigorous data from larger cohorts. Whether an improvement in inflammatory burden as assessed by 18FDG-PET is correlated with clinical outcomes is as yet unproven. Device therapy with implantable-cardioverter defibrillators should be considered in all cardiac sarcoidosis patients for either primary or secondary prevention of ventricular arrhythmias and cardiac death.

Background Accurate assessment of volume status is essential in diagnosis and guidance of decongestive therapy in patients with acute heart failure. We sought to compare peripheral venous pressure (PVP) with central venous pressure (CVP), as well as other invasive hemodynamic measurements, in patients hospitalized with an acute heart failure syndrome. Methods and Results PVP-HF (Peripheral Venous Pressure Measurements in Patients With Acute Decompensated Heart Failure) was a single-center prospective study, which enrolled patients admitted with acute heart failure, regardless of ejection fraction or disease pathogenesis. PVP and intracardiac pressures were obtained by transducing a peripheral intravenous and pulmonary artery catheter, respectively, after zeroing at the phlebostatic axis. Data were compared using Pearson’s correlation coefficient and Bland–Altman plots. A total of 30 patients (median age 64 years, 73% male, 30% ischemic pathogenesis) were enrolled. Mean ejection fraction was 31%, and 60% had moderate or greater right ventricular dysfunction. Median PVP was 9.5 (6–17) mm Hg, CVP was 8.5 (6–18) mm Hg, and pulmonary capillary wedge pressure was 18 (14–21) mm Hg. PVP and CVP were found to be highly correlated ( r =0.947), while PVP and pulmonary capillary wedge pressure were found to be moderately correlated ( r =0.565). The mean difference between PVP and CVP was 0.4 mm Hg and between PVP and pulmonary capillary wedge pressure was 7.5 mm Hg. Conclusions In patients with acute heart failure syndromes, a simple assessment of PVP demonstrates a high correlation with CVP. These findings suggest that PVP may be useful in the standard bedside clinical assessment of volume status in these patients to help guide decongestive therapy.

Background The use of implantable cardioverter defibrillators (ICDs) in patients with continuous flow left ventricular assist devices (LVADs) has not been evaluated in randomized clinical trials and the observational data published so far is controversial. To our knowledge, the burden of ICD related procedures after LVAD has not been previously described. Methods This is a retrospective single center study that included patients who underwent continuous flow LVAD implantation at the Cleveland Clinic between October 2004 and March 2017. Patients were grouped according to the presence or absence of ICD at the time of LVAD insertion. Outcomes after LVAD insertion were assessed including revisions of existing and subsequent implantation of ICDs. Results There were 486 patients in the study cohort; of those, 387 (79.6%) had an ICD prior to LVAD insertion. Patients with ICD prior to LVAD were older and had lower use of inotropes, extracorporeal membrane oxygenation, and mechanical ventilation before LVAD. There were 4 patients with leads cut or removed during LVAD, and 12 that had post-operative lead dysfunction due to dislodgment. There were 80 patients (21% of patients with ICD) who required 96 procedures after LVAD: 74 generator exchanges because of end of life or technical failure, 15 lead revisions, and 7 complete system removals because of infection. Of the 99 patients without ICD, 52 underwent ICD implantation: 38 for primary prevention and 14 for secondary prevention. Of those 4, had additional procedures: 2 generator changes due to end of life, 1 lead revision, and 1 system extraction due to pseudomonas endocarditis. The median follow ICD data follow up was 227 days in the pre-LVAD ICD group and 390 days in the post LVAD ICD group. There were 115(30%) and 13 (25%) patients that experimented ICD shocks in the pre-LVAD and post-LVAD ICD groups respectively. In both groups approximately 80% were categorized as appropriate.The unadjusted 30-day mortality was 5.2% and 10.1% in patients with and without ICD respectively (p=0.097). Conclusion In this cohort, ICD related procedures are common in patients with continuous flow LVADs and occur in about 1/5th of patients. During LVAD implantation displacement or cut of the ICD lead because of tricuspid valve intervention is not infrequent. More than half of patients without an ICD at baseline had an ICD implanted after LVAD. A substantial proportion of patients experience ICD shocks. The impact of shocks in outcomes remains to be determined.

Purpose Among patients who undergo orthotopic heart transplant (OHT), intravascular ultrasound (IVUS) detection of coronary allograft vasculopathy (CAV), defined as ≥ 0.5 mm increase in maximal intimal thickness (Δ MIT), is associated with worse outcomes at 5 years. Other IVUS-derived measurements of arterial plaque, while validated in studies of coronary atherosclerosis, have not been well studied in CAV. Methods From a single-center registry of 279 patients who underwent OHT from 1/09-1/16, a cohort of 10 randomly selected patients who experienced a cardiac event (defined as myocardial infarction or need for percutaneous coronary intervention) within 36 months post-OHT was compared with a control group of 10 randomly selected patients without post-OHT cardiac events. As a proof of concept analysis, IVUS data from baseline & 1 year were assessed to determine evidence of CAV development by Δ MIT & to measure change in Percent Atheroma Volume (PAV) & Total Atheroma Volume Normalized (TAVN). Presence of CAV by Δ MIT & median differences in PAV & TAVN were tested for association with cardiac events, using appropriate univariate statistics. Results Of 20 total patients, 30% were female, and median (IQR) age was 52.5 (42.5, 60.5) years. More patients in the non-event group had CAV at 1 year based on Δ MIT (4 vs. 3). Presence of CAV by Δ MIT & change in TAVN at 1 year were not significantly associated with cardiac events. However, a significant difference in PAV was present in patients within the event cohort, compared to the non-event cohort (median % change [IQR] = 7.6 [1.5, 10.9] vs. 0.6 [0.2, 4.8], p=0.049) (Table). Conclusion While increase in MIT at 1 year predicts worse outcomes at 5 years among patients after OHT, this retrospective proof of concept analysis suggests that PAV could have a stronger association with cardiac events than MIT or TAVN. Further studies are needed to investigate the accuracy & predictive utility of PAV as a measure of total allograft plaque burden.

Small studies have shown discordant findings regarding the association between sex, coronary vasomotor dysfunction (measured by myocardial blood flow reserve, MBFR), and adverse cardiac events (MACE), with some suggesting that low MFR was only associated with higher risk of MACE in women. To verify

Physiologic parameters quantified by positron emission tomography (PET) myocardial perfusion imaging (MPI), such as coronary flow reserve (CFR) and left ventricular ejection fraction reserve (LVEFr), improve identification of patients with obstructive 3-vessel or left main (3VD/LM) coronary artery

Accurate and reproducible quantification of ventricular septal (VS) thickness in hypertrophic cardiomyopathy (HC) is essential for diagnosis, surgical planning, and risk stratification. We sought to compare VS thickness measurements using transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR) in patients with HC. Prospectively reported measurements of VS thickness were compared using analysis of variance and Bland-Altman plots in 90 consecutive patients with HC who underwent a TTE, TEE, and CMR within 3 months. A subset was re-measured on 2 separate occasions by 2 readers to assess inter- and intraobserver variability. There was modest correlation between modalities, with CMR and TTE measurements of VS thickness showing the greatest correlation (CMR vs TTE, r = 0.70; CMR vs TEE, r = 0.60; TTE vs TEE, r = 0.56). Smaller measurements were seen using CMR versus either echocardiographic technique (13% smaller vs TEE, 8% smaller vs TTE, p0.001 for both). The variability of measurement between modalities was not correlated with the degree of VS thickness. There was significantly lower intraobserver variability with CMR versus echocardiography (p = 0.01 for both), but no difference in interobserver variability. CMR delineated a different area of maximal VS thickness other than the basal anteroseptum more frequently than echocardiography (44% of cases vs 24% for TTE and 11% for TEE). In conclusion, CMR assessment of VS thickness differs significantly from echocardiography in patients with HC, with a systemic bias toward lower measurements seen with CMR.

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%, p0.01). 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 (p0.01 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.

Quantitative uptake of Technetium 99 m-pyrophosphate (TcPYP) is sensitive and specific for diagnosing transthyretin cardiac amyloidosis (ATTR). We sought to examine the association between TcPYP uptake intensity and echocardiographic measures of disease severity and clinical outcomes.A retrospective analysis was performed of 75 patients who underwent TcPYP scintigraphy. Planar images were evaluated semiquantitatively and using heart-to-contralateral lung (H/CL) ratio. The associations between H/CL ratio and echocardiographic parameters and outcomes were evaluated using linear regression and Cox models, respectively. There were 48 patients diagnosed with ATTR with mean H/CL ratio 1.58 ± 0.22 (vs 1.08 ± 0.09 if semiquantitative score = 0). The H/CL ratio was not associated with any measured echocardiographic parameter. Both semiquantitative uptake grade and H/CL ratio were associated with all-cause mortality (P = 0.009 and 0.007, respectively) and all-cause mortality or heart failure hospitalization (P = 0.001 and 0.020, respectively); however, neither were associated with outcomes when limited to patients with confirmed ATTR (P = 0.18 and 0.465, respectively).In patients with suspected ATTR, quantitative and semiquantitative uptake intensity of TcPYP is associated with all-cause mortality as well as all-cause mortality or heart failure hospitalization. However, in those with confirmed ATTR, there is no association with echocardiographic disease severity or outcomes.

Background Light chain ( AL ) and transthyretin ( ATTR ) amyloidosis have a similar effect on myocardial function but very different disease trajectories and survival. However, limited data are available evaluating subtype‐specific predictors of outcomes in a large contemporary cohort. Methods and Results We retrospectively investigated 360 patients at the time of initial diagnosis of cardiac amyloidosis (191 AL and 169 ATTR ) from 2002 to 2014. Clinical, laboratory, electrical, and morphologic covariates were evaluated based upon amyloid subtype. ATTR etiology was associated with older age, more chronic medical conditions, and the use of standard heart failure medical therapy. Left ventricular mass index and electrocardiographic voltage were higher in ATTR , while there was no difference in ejection fraction or markers of diastology between subtypes. A multivariable Cox model was generated using previously identified predictors of negative outcomes in cardiac amyloidosis and analyzed after stratification for subsequent amyloid‐specific treatment. An AL etiology was the most predictive variable (hazard ratio 3.143, P AL ( P =0.015). The magnitude of effect of other variables on mortality did not significantly differ between subtypes. Conclusions Clinical, morphological, electrical, and biomarker data do not significantly interact with amyloid subtype in its association with mortality, despite the fact that the prognosis in each subtype differs greatly. This suggests an additional factor or factors (such as light chain toxicity) contributing to poorer outcomes in AL amyloid.

Background Low voltage electrocardiography (ECG) coupled with increased ventricular wall thickness is the hallmark of cardiac amyloidosis. However, patient characteristics influencing voltage in the general population, including bundle branch block, have not been evaluated in amyloid heart disease. Methods A retrospective analysis was performed of patients with newly diagnosed cardiac amyloidosis from 2002 to 2014. ECG voltage was calculated using limb (sum of QRS complex in leads I, II and III) and precordial (Sokolow: S in V1 plus R in V5–V6) criteria. The associations between voltage and clinical variables were tested using multivariable linear regression. A Cox model assessed the association of voltage with mortality. Results In 389 subjects (transthyretin ATTR 186, light chain AL 203), 30% had conduction delay (QRS >120ms). In those with narrow QRS, 68% met low limb, 72% low Sokolow and 57% both criteria, with lower voltages found in AL vs ATTR. LV mass index as well as other typical factors that impact voltage (age, sex, race, hypertension, BSA, and smoking) in the general population were not associated with voltage in this cardiac amyloidosis cohort. Patients with LBBB and IVCD had similar voltages when compared to those with narrow QRS. Voltage was significantly associated with mortality (p Conclusion Classic predictors of ECG voltage in the general population are not valid in cardiac amyloidosis. In this cohort, the prevalence estimates of ventricular conduction delay and low voltage are higher than previously reported. Voltage predicts mortality after multivariable adjustment.

Sarcoidosis is a multisystem inflammatory disease of unknown etiology that is characterized histologically by the presence of non-caseating granulomas. It most commonly affects the lungs and thoracic lymph nodes; however, autopsy studies have shown that up to 27% of patients with sarcoidosis have cardiac involvement.1 The diagnosis of cardiac sarcoidosis (CS) is challenging, because specimens from an endomyocardial biopsy (the gold standard) are confirmatory in only 32% of patients.2 Patients diagnosed with CS who present with heart failure symptoms have a worse prognosis than those who present with other symptoms3 and have acceptable long-term outcomes without recurrence after heart transplantation.4 The Heart Rhythm Society (HRS) recommends that patients with extracardiac sarcoidosis proven by biopsy specimen undergo advanced cardiac imaging if they have an abnormal electrocardiogram (ECG), abnormal echocardiogram, or symptoms (palpitations, syncope, or presyncope).5 We sought to determine how well this recommendation performed if applied to our population of patients with CS proven by biopsy specimen who initially presented with heart failure. A retrospective analysis was performed of 27 consecutive patients at the Cleveland Clinic with CS proven by myocardial biopsy specimen from 2002 to 2014. Patients were included with New York Heart Association Functional Classification ≥ II symptoms and a left ventricular ejection fraction of ≤50%. Baseline data were obtained on all patients before cardiac tissue diagnosis, including clinical characteristics, echocardiographic, ECG, and laboratory findings. Positron emission tomography with 18F-fluoro-2-deoxy-D-glucose (FDG-PET) and cardiac magnetic resonance imaging (CMR) were evaluated when available (Table). Table Clinical Characteristics and Japanese Ministry of Health and Welfare Criteria in Patients with Biopsy Specimen– Proven Cardiac Sarcoidosis and Cardiomyopathy Patients were on average in their sixth decade of life and more likely to be Caucasian and male. The ECGs in all patients showed abnormalities, as defined by a complete right or left bundle branch block, axis deviation, ventricular tachycardia, frequent premature ventricular contractions, or abnormal Q waves. Eighteen patients (67%) had class III or IV heart failure symptoms, 16 (59%) had ventricular tachycardia, and 15 (56%) had advanced heart block requiring pacing. All patients met at least 1 criterion needed to warrant further investigation with FDG-PET or CMR based on the HRS consensus statement.5 In contrast, only 8 of 27 patients (30%) met Japanese Ministry of Health and Welfare criteria for CS before cardiac biopsy. Of the 16 patients who underwent FDG-PET, 12 had matching segmental defects in perfusion and FDG and 12 had segments with reduced perfusion and enhanced FDG uptake (“mismatched segments”). All patients had matching or mismatched defects. In addition, the 10 patients who underwent contrast-enhanced CMR had late gadolinium enhancement in a pattern consistent with CS. Fourteen patients (52%) had isolated CS, defined as lack of extracardiac symptoms, diagnostic biopsy specimen, or imaging findings, including CMR and FDG-PET. There were 19 heart transplantations and 5 left ventricular assist devices (LVADs) in the cohort. Of the 5 patients with LVADs, all of whom went on to heart transplantation, 2 had apical core biopsy specimens that were nondiagnostic but were later confirmed to be sarcoidosis after analysis of the explanted heart. Eight of the 19 patients receiving heart transplantation or LVAD (42%) were previously unaware of a diagnosis of systemic or CS until the time of pathologic confirmation. Ten of the 19 heart transplant patients had isolated CS. All patients were maintained on low-dose prednisone after transplantation. There was no recurrence of CS on endomyocardial biopsy specimens in any of the patients after 25,993 total patient follow-up days with an average follow-up of 4.2 years per patient. This analysis highlights several important points regarding CS proven on biopsy specimen in patients presenting with cardiomyopathy. The LVAD apical core was positive in only 3 of the 5 patients who later went on to heart transplantation, highlighting the heterogeneous involvement of sarcoid granulomas in the myocardium as well as the difficulty in obtaining a positive biopsy sample, even in patients with severe cardiomyopathy. Isolated CS was present in 52% of the patients, validating the high prevalence seen in other studies of biopsy specimen–proven CS.3,4 However, this prevalence may be influenced by selection bias, because patients with extracardiac biopsy specimen–proven sarcoidosis are more likely to have a non-invasive evaluation of cardiac involvement. All patients had an abnormal ECG, reduced LVEF, and findings consistent with the diagnosis of CS on multimodality imaging, including CMR and FDG-PET. This corroborates the findings of the recently published HRS consensus statement on the diagnosis of CS in patients presenting with arrhythmias or heart block and suggests that patients with a cardiomyopathy severe enough to have a positive endomyocardial biopsy specimen or warrant advanced heart failure therapies will have findings consistent with the diagnosis on FDG-PET or CMR.5 Given the difficulty in sampling an affected area for a histologic diagnosis, we agree with the HRS guidelines that a non-invasive approach with CMR or PET imaging is favored. Currently, no consensus recommendations exist regarding the diagnosis and screening of CS in patients presenting with non-ischemic cardiomyopathy. Our results add to the small but growing body of literature in this field.

Background: Low voltage on ECG may be caused by various medical conditions. In cardiac amyloidosis, low voltage in the limb leads is found in approximately 50% with AL and only 20-25% of ATTR, whether senile or familial. Despite not meeting low voltage criteria on ECG, a reduction of voltage over time may signify amyloid cardiomyopathy. Methods: The electrocardiograms of consecutive patients at the time of a diagnosis of cardiac amyloidosis at our institution were analyzed. Patients with ventricular paced rhythms were excluded. An additional ECG at least one year prior to the diagnosis was also analyzed. The sum of voltage in all limb leads and the Sokolow voltage (S wave in V1 plus R wave in V5 or V6) index were calculated. Low limb voltage was defined as ≤ 5 mm in all limb leads and low precordial voltage as ≤ 10 mm in all precordial leads. A Cox proportional hazards model was used to assess the correlation between decreasing ECG voltage and 3 year mortality. Results: A total of 338 patients (192 AL, 146 ATTR, age 68.7 +/- 12.1 years, obese 57%, HTN 54%) were identified. At the time of diagnosis, 167 patients (49.4%) met either low limb or low precordial voltage criteria. The limb lead voltage decreased in 63 of 73 patients (86.3%) and the Sokolow voltage decreased in 62 of 73 patients (84.9%). The mean overall decrease in limb lead voltage was 10.6 +/- 12.6 mm overall or 3.3 +/- 4.6 mm per year. The Sokolow voltage decreased 6.5 +/- 7.1 mm overall or 1.8 +/- 2.3 mm per year. On univariable analysis, the annualized decrease in Sokolow voltage showed a non-significant trend towards an increase in mortality. Conclusion: A decrease in voltage on serial ECG’s is more prevalent than low voltage in cardiac amyloidosis. There is a trend towards increased mortality in patients who have a greater decrease in voltage over time. The evaluation of serial ECG’s for changes in voltage is important to recognize in the diagnosis of cardiac amyloidosis and may have prognostic value.

Introduction: Left bundle branch block (LBBB) with QRS duration > 120 msec is typically associated with increased ECG voltage without a necessary increase in left ventricular wall thickness. Studies assessing voltage criteria in amyloidosis and other cardiomyopathies have excluded patients with LBBB. We sought to assess the effects of QRS duration and LBBB on voltage criteria in cardiac amyloidosis. Methods: We performed a retrospective analysis of patients with newly diagnosed cardiac amyloidosis at our institution from 2001-2014. Low voltage in the precordial leads was defined by the Sokolow criteria (S wave in V1 plus R wave in V5 or V6 ≤ 15mm). Limb lead voltage was calculated by the sum of the entire QRS complex voltage of leads I, II and III with low voltage being defined as each lead ≤ 5 mm. Patients with left bundle branch block were propensity matched to assess voltage criteria based upon age, sex, history of HTN, amyloid type (AL vs TTR), anteroseptal thickness and BSA to those with QRS < 120msec. Results: In 299 subjects (age 69.6 +/- 11.7 years, male 69%, HTN 53%, AL 53%, IVS 18.1 +/- 4.1 mm, BSA 1.95 +/- 0.26, EF 48 +/- 14%), mean QRS duration was 107 +/- 25 msec with 71 patients (24%) having a QRS duration greater than 120 msec (17 LBBB). The average limb voltage was 15.1 +/- 7.3 mm with low limb lead voltage seen in 62% of patients. Low precordial lead voltage was found in 73% of patients with a mean Sokolow voltage of 12.9 +/- 7.5 mm. After propensity matching the 17 patients with LBBB on a 1:3 basis with patients with a narrow complex QRS, there was no difference in any measure of voltage criteria. Conclusion: LBBB is not associated with higher voltage and does not hinder the ability to detect low voltage in patients with cardiac amyloidosis.

Cardiac amyloidosis is an under recognized and underdiagnosed heart failure etiology characterized by protein misfolding, myocardial deposition, and restrictive cardiomyopathy. Cardiac involvement in elderly patients most commonly results from the transthyretin protein (ATTR) and carries a poor

Objective Cardiac 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. Methods This 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. Results Despite 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. Conclusions High 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.

Valvular aortic stenosis leads to compensatory myocyte hypertrophy that initially restores wall stress to normal levels. When left untreated, myocardial fibrosis and decreased systolic function ensue with development of a cardiomyopathy of pressure overload. Clinically, this may present as a low-gradient, low ejection fraction (EF) aortic stenosis, and management of this clinical entity is well defined. Subjects with contractile reserve in the setting of dobutamine stimulation have been shown to benefit from aortic valve replacement (AVR). In contrast, the lack of contractile reserve after dobutamine is a high-risk finding.1 AVR in this setting is related to high-operative mortality, and therefore, many patients are denied valve replacement. We present an extreme case of a patient with severe aortic stenosis and Stage D heart failure that recovered left ventricular (LV) function after left ventricular assist device (LVAD) implantation allowing for LVAD disconnection despite absent contractile reserve and pathological evidence of significant myocardial fibrosis. This case highlights the challenges of the conventional treatment paradigm and provides us unique insights into myocardial contractile recovery. The patient was a 53-year-old man who initially presented to a community hospital with 3 months of progressive dyspnea, orthopnea, edema, and decreased exercise tolerance. He had a past medical history of hypertension, hyperlipidemia, type 2 diabetes mellitus, and a murmur first noted in adolescence. He denied a history of alcohol, drug, or stimulant use and had no family history of cardiovascular disease. Results of a complete metabolic panel, blood count, thyroid stimulating hormone, and iron studies were unremarkable. Echocardiography demonstrated a bicuspid aortic valve with severe low EF low-gradient aortic stenosis and a dilated and hyptrophied …

Background: Cardiac amyloidosis (CA) creates a low flow state that may predispose patients to forming atrial thrombi. We sought to define the prevalence of left and right atrial thrombi in patients with CA. Methods: A retrospective analysis was performed of consecutive TEEs in patients with known

In patients with systemic sarcoidosis (CS), myocardial involvement is often analyzed by echocardiography and positron emission tomography with 18F-fluorodeoxyglucose (FDG-PET). However, global longitudinal strain (GLS) may be the earliest sign of myocardial involvement. We sought to compare GLS with