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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.

The effects of recipient body mass index (BMI) on waitlist strategies, waitlist outcomes, and post-transplant outcomes among adult patients listed for heart transplantation under the updated 2018 allocation system have not been well characterized.The United Network of Organ Sharing data set between October 2015 and March 2021 was analyzed, and patients were grouped based on recipient BMI and whether listing occurred in the old (pre-October 2018) or new allocation system.Listing strategies differed by BMI group, but trends of increased use of temporary mechanical support and decreased use of durable support remained among all BMI groups, except those with BMI 35 kg/mThe 2018 change to the heart transplant allocation system was associated with similar changes in the use of mechanical support for listing strategy across BMI ranges, except in the most obese, and improved waitlist outcomes across all BMI ranges. Post-transplant outcomes in the new allocation system are worse for patients with BMI 30 kg/m

This information statement from the Society of Nuclear Medicine and Molecular Imaging, American Society of Nuclear Cardiology, and European Association of Nuclear Medicine describes the performance, interpretation, and reporting of hot spot imaging in nuclear cardiology. The field of nuclear cardiology has historically focused on cold spot imaging for the interpretation of myocardial ischemia and infarction. Hot spot imaging has been an important part of nuclear medicine, particularly for oncology or infection indications, and the use of hot spot imaging in nuclear cardiology continues to expand. This document focuses on image acquisition and processing, methods of quantification, indications, protocols, and reporting of hot spot imaging. Indications discussed include myocardial viability, myocardial inflammation, device or valve infection, large vessel vasculitis, valve calcification and vulnerable plaques, and cardiac amyloidosis. This document contextualizes the foundations of image quantification and highlights reporting in each indication for the cardiac nuclear imager.

Objectives This study sought to determine whether the increased use of telehealth was associated with a difference in outcomes for outpatients with heart failure. Background The COVID-19 pandemic led to dramatic changes in the delivery of outpatient care. It is unclear whether increased use of telehealth affected outcomes for outpatients with heart failure. Methods In March 2020, a large Midwestern health care system, encompassing 16 cardiology clinics, 16 emergency departments, and 12 hospitals, initiated a telehealth-based model for outpatient care in the setting of the COVID-19 pandemic. A propensity-matched analysis was performed to compare outcomes between outpatients seen in-person in 2018 and 2019 and via telemedicine in 2020. Results Among 8,263 unique patients with heart failure with 15,421 clinic visits seen from March 15 to June 15, telehealth was employed in 88.5% of 2020 visits but in none in 2018 or 2019. Despite the pandemic, more outpatients were seen in 2020 (n = 5,224) versus 2018 and 2019 (n = 5,099 per year). Using propensity matching, 4,541 telehealth visits in 2020 were compared with 4,541 in-person visits in 2018 and 2019, and groups were well matched. Mortality was similar for telehealth and in-person visits at both 30 days (0.8% vs 0.7%) and 90 days (2.9% vs 2.4%). Likewise, there was no excess in hospital encounters or need for intensive care with telehealth visits. Conclusions A telehealth model for outpatients with heart failure allowed for distanced encounters without increases in subsequent acute care or mortality. As the pressures of the COVID-19 pandemic abate, these data suggest that telehealth outpatient visits in patients with heart failure can be safely incorporated into clinical practice., Central Illustration

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.

Amyloidosis is a multisystem disease which continues to present in later stages due to delayed diagnosis. Once the disease is identified, the coordination of ongoing care and treatment becomes complex and often involves multiple specialists. As knowledge of the disease grows, healthcare providers within institutions have organized to create comprehensive amyloidosis programs to better serve patients in the region. In this review, we present considerations in starting a cardiac amyloidosis program from two institutions that have recently started such programs. Identification of multidisciplinary stakeholders, development of overarching program goals, creation of institutional buy-in, and emphasis on program growth and development are tenets of a successful program. The creation and growth of an amyloidosis program has the potential to raise awareness for the disease and benefit patients and institutions alike.

Systemic immunoglobulin light chain (AL) amyloidosis is a rare but fatal disease. It results from clonal proliferation of plasma cells with excessive production of insoluble misfolded proteins that aggregate in the extracellular matrix, causing damage to the normal architecture and function of various organs. For decades, treatment for AL amyloidosis was based mainly on therapeutic agents previously studied for its more common counterpart, multiple myeloma. As the prevalence and incidence of AL amyloidosis have increased, ongoing research has been conducted with treatments typically used in myeloma with varying success. In this review, we focus on current treatment strategies and updates to clinical guidelines and therapeutics for AL amyloidosis.

Statins are recommended in heart transplant patients, but are sometimes poorly tolerated. Alternative agents are often considered including proprotein convertase subtilisin/kexin type-9 inhibitors (PCSK9i). We sought to investigate the use of PCSK9i after heart transplantation.We identified patients who received a heart transplant from 1999 to 2019 and were started on PCSK9i at our institution. Clinical, laboratory, and coronary angiography with intravascular ultrasound results were compared. Among 65 patients initiated on PCSK9i (48 for statin intolerance and 17 for refractory hyperlipidemia), the median time from transplant was 5.5 years (interquartile range [IQR], 2.8-9.9 years) with a median PCSK9 treatment duration of 1.6 years (IQR, 0.8-3.2 years) and 80% still on treatment. Evolocumab was used in 73.8%, alirocumab in 12.3%, and both in 13.8% owing to insurance coverage. All patients required prior authorization; initial denial occurred in 18.5% and 32.3% had denials in subsequent years. The median low-density lipoprotein cholesterol decreased from 130 mg/dL (IQR, 102-148 mg/dL) to 55 mg/dL (IQR, 35-74 mg/dL) after starting PCSK9i (P.001), with 72% of patients achieving a low-density lipoprotein cholesterol of70 mg/dL after treatment. There were also significant reductions of total cholesterol, non-high-density lipoprotein cholesterol, total/high-density lipoprotein cholesterol ratio, and triglycerides, with a modest increase in high-density lipoprotein cholesterol. These changes were durable at latest follow-up. In 33 patients with serial coronary angiography and intravascular ultrasound, PCSK9i were associated with stable coronary plaque thickness and lumen area.Among heart transplant recipients, PCSK9i are effective in lowering cholesterol levels and stabilizing coronary intimal hyperplasia with minimal side effects. Despite favorable effects, access and affordability remain a challenge.

Type 2 diabetes mellitus (T2D) is a common comorbidity among patients who have undergone heart transplantation. Recently two classes of glucose-lowering medications (sodium-glucose cotransporter type-2 inhibitors [SGLT-2Is] and glucagon-like-peptide-1 receptor agonists [GLP-1RAs]), have been shown to significantly improve cardiovascular outcomes. There is a paucity of data regarding their use in immunosuppressed patients, with many studies specifically excluding this population. We retrospectively evaluated the safety and efficacy of GLP-1RAs and SGLT-2Is in patients who had undergone orthotopic heart transplant at a high-volume center. Among 21 patients, we found significant weight loss, reductions in insulin use, hemoglobin A1c, and low-density lipoprotein-cholesterol. Moreover, both SGLT-2Is and GLP-1RAs were well tolerated with no adverse events leading to discontinuation of either therapy. While larger studies of patients after solid organ transplant are needed, this small hypothesis-generating study demonstrates that SGLT-2Is and GLP-1RAs appear safe and effective therapies among patients with T2D after heart transplant.

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.

The COVID-19 pandemic underscored our healthcare system's unpreparedness to manage an unprecedented pandemic. Heart failure (HF) physicians from 14 different academic and private practice centers share their systems' challenges and innovations to care for patients with HF, heart transplantation, and patients on LVAD support during the COVID-19 pandemic. We discuss measures implemented to alleviate the fear in seeking care, ensure continued optimization of guideline directed medical therapy (GDMT), manage the heart transplant waiting list, continue essential outpatient monitoring of anticoagulation in LVAD patients and surveillance testing post-heart transplant, and prevent physician burnout. This collaborative work can build a foundation for better preparation in the face of future challenges.

Background 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. Methods 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%×(post systolic strain–end-systole strain)/post systolic strain. Results 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 Conclusion 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.

Background: Coronary artery calcium score (CACS) is an anatomic measure of calcified atherosclerosis. Myocardial perfusion defects and reduced myocardial blood flow reserve (MBFR) are physiological measures of ischemia and coronary circulatory health. We aimed to assess the relative prognostic importance of MBFR, perfusion defects, and CACS in patients with suspected coronary artery disease. Methods: A total of 5983 consecutive patients without known history of coronary artery disease or cardiomyopathy, who underwent a CACS and 82 Rb positron emission tomography myocardial perfusion imaging between 2010 and 2016, were followed for all-cause death (n=785) over median of 3 years. Prognostic value was assessed using multivariable Cox regression models, and incremental risk discrimination for imaging variables was evaluated by comparing model c-indices after adjusting for clinical risk factors (RF). Results: Mean age was 67.1 years, 60% were female, and 83% were symptomatic. CACS was 0 in 22%, abnormal perfusion in 19%, and MBFR P P =0.02). Adding CACS to a model with RF, perfusion, and MBFR did not provide incremental prognostic value (c=0.785 for both, P =0.16). CACS and MBFR both had independent prognostic value in patients with normal and abnormal myocardial perfusion imaging. Even among patients with CACS of 0, MBFR P Conclusions: Use of anatomic testing such as CACS of 0 to avoid myocardial perfusion imaging in symptomatic patients could lead to missing microvascular dysfunction in 4 out of 10 patients, a finding associated with a high mortality risk. Higher CACS was independently associated with the risk of death but did not provide incremental prognostic value over positron emission tomography with MBFR.

Multi-societal consensus recommendations endorse both planar and single photon emission tomographic (SPECT) image acquisitions for the evaluation of cardiac amyloidosis. However, the correlation between planar and SPECT findings and the optimal timing of image acquisitions remain uncertain. This is an analysis of 109 consecutive patients who underwent technetium pyrophosphate nuclear scintigraphy for the evaluation of cardiac amyloidosis. Patients were imaged at 1 and 3 hours after radiotracer injection using both planar and SPECT/CT, and the correlations between imaging protocols were compared. In the overall cohort (median age 77 years, 75% male), 33 patients had radiotracer localized to the myocardium on SPECT/CT images. There was strong correlation between 1- and 3-hour planar heart-to-contralateral lung ratios (mean difference 0.07, r = 0.94). However, there was discordance between planar image interpretation (based upon semiquantitative score and H/CL ratio) and myocardial localization of radiotracer on SPECT/CT in 17 patients (16%). The pattern of SPECT/CT uptake was identical at 1 and 3 hours in all cases (32 diffuse, 1 focal). These data support the recommendation that SPECT/CT should be obtained in addition to planar images when performing nuclear scintigraphy for the detection of cardiac amyloidosis. A 1-hour planar and SPECT/CT protocol appears optimal.

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

Background: In patients with transthyretin amyloid cardiomyopathy, tafamidis reduces all-cause mortality and cardiovascular hospitalizations and slows decline in quality of life compared with placebo. In May 2019, tafamidis received expedited approval from the US Food and Drug Administration as a breakthrough drug for a rare disease. However, at $225 000 per year, it is the most expensive cardiovascular drug ever launched in the United States, and its long-term cost-effectiveness and budget impact are uncertain. We therefore aimed to estimate the cost-effectiveness of tafamidis and its potential effect on US health care spending. Methods: We developed a Markov model of patients with wild-type or variant transthyretin amyloid cardiomyopathy and heart failure (mean age, 74.5 years) using inputs from the ATTR-ACT trial (Transthyretin Amyloidosis Cardiomyopathy Clinical Trial), published literature, US Food and Drug Administration review documents, healthcare claims, and national survey data. We compared no disease–specific treatment (“usual care”) with tafamidis therapy. The model reproduced 30-month survival, quality of life, and cardiovascular hospitalization rates observed in ATTR-ACT; future projections used a parametric survival model in the control arm, with constant hazards reduction in the tafamidis arm. We discounted future costs and quality-adjusted life-years by 3% annually and examined key parameter uncertainty using deterministic and probabilistic sensitivity analyses. The main outcomes were lifetime incremental cost-effectiveness ratio and annual budget impact, assessed from the US healthcare sector perspective. This study was independent of the ATTR-ACT trial sponsor. Results: Compared with usual care, tafamidis was projected to add 1.29 (95% uncertainty interval, 0.47–1.75) quality-adjusted life-years at an incremental cost of $1 135 000 (872 000–1 377 000), resulting in an incremental cost-effectiveness ratio of $880 000 (697 000–1 564 000) per quality-adjusted life-year gained. Assuming a threshold of $100 000 per quality-adjusted life-year gained and current drug price, tafamidis was cost-effective in 0% of 10 000 probabilistic simulations. A 92.6% price reduction from $225 000 to $16 563 would be necessary to make tafamidis cost-effective at $100 000/quality-adjusted life-year. Results were sensitive to assumptions related to long-term effectiveness of tafamidis. Treating all eligible patients with transthyretin amyloid cardiomyopathy in the United States with tafamidis (n=120 000) was estimated to increase annual healthcare spending by $32.3 billion. Conclusions: Treatment with tafamidis is projected to produce substantial clinical benefit but would greatly exceed conventional cost-effectiveness thresholds at the current US list price. On the basis of recent US experience with high-cost cardiovascular medications, access to and uptake of this effective therapy may be limited unless there is a large reduction in drug costs.

Background: Myocardial perfusion imaging (MPI) identifies abnormalities that occur early in the ischemic cascade leading to angina. Our aim was to study the association between ischemic measures on positron emission tomography MPI and patients’ health status; their symptoms, function, and quality of life. Methods: Health status was collected using the Seattle Angina Questionnaire (SAQ-7, 0–100, higher=better) and Rose Dyspnea Score (RDS) on 1515 outpatients with known or suspected coronary artery disease presenting for clinically indicated pharmacological 82 Rb positron emission tomography MPI from July 2018 to July 2019. Adjusted multivariable ordinal regression models were used to assess the association between MPI findings of ischemia and the SAQ physical limitation, angina frequency, quality of life, summary score, and the RDS. Results: The mean SAQ and RDS scores of the cohort (mean age 71.7 years, 55% male, 37.6% prior myocardial infarction or revascularization) were 73.8±28.6 (physical limitation), 87.4±21.7 (angina frequency), 79.0±26.1 (quality of life), 81.3±19.0 (summary score), and 2±2 (RDS). No perfusion, flow or function abnormalities were significantly associated with SAQ angina frequency scores. Low left ventricular ejection fraction reserve (≤0%), low global and regional myocardial blood flow reserve (P ≤0.01), but reversible perfusion defects were not. Conclusions: Impaired augmentation of left ventricular ejection fraction and myocardial blood flow with stress is associated with significant angina-associated functional limitation, health status, and dyspnea in patients who underwent positron emission tomography MPI, but not the frequency of their angina. Future studies should evaluate whether therapies that improve stress-induced abnormalities in systolic function and myocardial flow may improve patients’ health status.

ImportanceTafamidis reduced all-cause mortality and cardiovascular-related hospitalizations and minimized patient-reported health status deterioration at 30 months in patients with transthyretin (ATTR) amyloidosis. However, the clinical significance of health status changes remains unclear, particularly in patients with New York Heart Association (NYHA) class III symptoms who experienced more cardiovascular-related hospitalizations than those with NYHA class I-II symptoms.ObjectiveTo evaluate the health status of patients taking tafamidis with baseline NYHA class III symptoms.Design, Setting, and ParticipantsThis randomized clinical trial post hoc analysis evaluated data for patients with transthyretin (ATTR) cardiac amyloidosis and NYHA class I-III symptoms at baseline who were enrolled in ATTR-ACT, a placebo-controlled study of tafamidis held at 48 sites in 13 countries.InterventionsTafamidis meglumine, 80 mg or 20 mg (pooled cohort), vs placebo.Main Outcomes and MeasuresEstablished thresholds for clinical benefit on the Kansas City Cardiomyopathy Questionnaire Overall Summary (KCCQ-OS) were used to define response groups (very large decline to very large improvement); the proportion of patients in each group was calculated within each baseline NYHA class.ResultsAmong 441 patients (264 tafamidis, 177 placebo), the mean (SD) age was 74.3 (7.0) years; 398 (90%) were male and 43 (10%) were female. Mean (SD) baseline KCCQ-OS scores were 67.3 (21.4) in the tafamidis group and 65.9 (21.7) in the placebo group (range: 0-100, with 100 indicating the best health). There was a significant shift toward better KCCQ-OS scores in patients receiving tafamidis (odds ratio for 10-point improvement 2.4; 95% CI, 1.6-3.4; P Conclusions and RelevanceIn ATTR-ACT, although patients with baseline NYHA class III symptoms had worse overall outcomes, treatment with tafamidis yielded better health status compared with placebo.Trial RegistrationClinicalTrials.gov Identifier: NCT01994889

Introduction: Left ventricular assist device (LVAD) is a lifesaving bridge to transplantation or destination therapy for patients with end-stage heart failure. More than one-third of patients suffer from gastrointestinal (GI) bleeding within one year of placement. We investigated the yield and safety of endoscopic screening for potential sources of GI bleeding before LVAD insertion. Hypothesis: Endoscopic screening prior to LVAD placement does not necessarily reduce the risk of GI bleed post LVAD. Methods: We performed a retrospective chart review study of patients at Saint Luke’s Mid America Heart Institute who underwent continuous-flow LVAD implantation from 2010 to 2020. Study participants were categorized based on whether or not they underwent screening endoscopy prior to LVAD implantation. Patients were followed for one year after implantation, and outcomes were collected to determine the yield and safety of these procedures. A multivariable logistic regression analysis was performed using SPSS® controlling for the following confounding factors: age, gender, race, and history of GI bleeding. Results: Among 205 patients with an LVAD, 169 patients were included in the study after excluding those with lack of data or those lost to follow up. 68 (40.23%) patients who received pre-LVAD screening by either upper and/or lower endoscopies, and 42 (24.85%) patients who had post-LVAD GI bleed. Patients who underwent pre-LVAD screening had similar rates of GI bleeding after LVAD compared to those who were not screened (odds ratio 1.66, 95% CI 0.648-4.264, P=0.2). Moreover, in patients with post LVAD GI bleed, endoscopy findings were typically different than those pre LVAD. No complications to the endoscopic interventions were reported. Conclusions: While endoscopic screening appears to be safe in patients before LVAD implantation, it had no association with GI bleeding risk or etiology after LVAD.

Background: Rubidium-82 positron emission tomography myocardial perfusion imaging provides measurements of perfusion, myocardial blood flow and reserve (MBFR), and changes in left ventricular ejection fraction (LVEF) at rest and peak stress. Although all of these variables are known to provide prognostic information, they have not been well studied in patients with heart failure due to reduced LVEF. Methods: Between 2010 and 2016, 1255 consecutive unique patients with LVEF≤40% were included in this study who underwent rubidium-82 positron emission tomography myocardial perfusion imaging and did not have subsequent revascularization within 90 days. Perfusion assessment was scored semiquantitatively, and LVEF reserve (stress-rest LVEF) and global MBFR (stress/rest MBF) were quantified using automated software. Cox proportional hazards models adjusted for 14 clinical and 7 test characteristics were used to define the independent prognostic significance of MBFR on all-cause mortality. Results: Of 1255 patients followed for a mean of 3.2 years, 454 (36.2%) died. After adjusting for clinical variables, the magnitude of fixed and reversible perfusion defects was prognostic of death ( P =0.02 and 0.01, respectively), while the rest LVEF was not ( P =0.18). The addition of LVEF reserve did not add any incremental value, while the addition of MBFR revealed incremental prognostic value (hazard ratio per 0.1 unit decrease in MBFR=1.08 [95% CI, 1.05–1.11], P P =0.07 and 0.29, respectively). There was no interaction between MBFR and cause of cardiomyopathy (ischemic versus nonischemic). Conclusions: In patients with a known cardiomyopathy who did not require early revascularization, reduced MBFR as obtained by positron emission tomography myocardial perfusion imaging is associated with all-cause mortality while other positron emission tomography myocardial perfusion imaging measures were not.

Amyloidosis is a multisystem disease which continues to present in later stages due to delayed diagnosis. Once the disease is identified, the coordination of ongoing care and treatment becomes complex and often involves multiple specialists. As knowledge of the disease grows, healthcare providers within institutions have organized to create comprehensive amyloidosis programs to better serve patients in the region. In this review, we present considerations in starting a cardiac amyloidosis program from two institutions that have recently started such programs. Identification of multidisciplinary stakeholders, development of overarching program goals, creation of institutional buy-in, and emphasis on program growth and development are tenets of a successful program. The creation and growth of an amyloidosis program has the potential to raise awareness for the disease and benefit patients and institutions alike.

Cardiac amyloidosis is often preceded by orthopedic manifestations such as carpal tunnel syndrome, and 10% of patients who underwent idiopathic carpal tunnel release surgery will have biopsy-confirmed amyloid deposits in the tenosynovial sheath. Trigger finger is also commonly reported in patients with amyloidosis and involves the same tendon sheath as carpal tunnel syndrome, but the prevalence of amyloid deposition is unclear. This prospective cross-sectional study enrolled 100 patients aged ≥50 years at the time of surgery for idiopathic trigger finger. Patients underwent release surgery, and a sample of the tenosynovium of the affected finger was excised, stained with Congo red, and subtyped with mass spectrometry if amyloid was demonstrated. Further cardiac evaluation was performed in patients with amyloid deposition. Of the 100 patients (mean age 65.5 ± 8.1 years) enrolled, only 2 demonstrated amyloid deposits on Congo red staining. One patient with previous proteinuric kidney disease had fibrinogen A α-chain amyloidosis, and the other patient had untyped amyloidosis. Neither patient had cardiac involvement. A total of 13 of the 100 patients underwent concomitant carpal tunnel release surgery, and 2 of these patients had amyloid deposits in the carpal tunnel with “false-negative” samples from the trigger finger tenosynovium. In conclusion, biopsy during trigger finger release surgery demonstrated a 2% yield for amyloidosis, which is significantly lower than the previously published yield of 10% during carpal tunnel release surgery. This observation has important implications for the development of diagnostic algorithms to screen patients for amyloidosis during orthopedic operations.

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.

Carpal tunnel syndrome (CTS) can be caused by the deposition and accumulation of misfolded proteins called amyloid and is often an early manifestation of systemic amyloidosis. In patients undergoing surgery for idiopathic CTS, a recent study identified amyloidosis by tenosynovial biopsy in 10.2% of men older than 50 years and women older than 60 years; all positive patients had bilateral symptoms. These findings have led to a renewed interest in amyloidosis as an etiology of CTS. The 2 most common systemic amyloidoses, immunoglobulin light chain and transthyretin amyloidosis, affect the heart, nerves, and other organ systems throughout the body including the soft tissues. Patients with cardiac involvement of amyloidosis have an especially poor prognosis if the disease remains unrecognized and untreated. Early diagnosis is paramount, and patients classically present with cardiac disease several years after being operated on by a hand surgeon for carpal tunnel release. Herein, we present a review of amyloidosis as it pertains to CTS and an algorithm for the detection of amyloidosis in patients undergoing carpal tunnel release. Implementation of this straightforward algorithm will allow for early diagnosis of amyloidosis, a group of progressive and lethal diseases.

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.

Background Gene expression profiling (GEP) and donor-derived cell-free DNA (dd-cfDNA) are useful in acute rejection (AR) surveillance in orthotopic heart transplant (OHT) patients. We report a single-center experience of combined GEP and dd-cfDNA testing for AR surveillance. Methods GEP and dd-cfDNA are tested together starting at 2 months post-OHT. After 6 months, combined testing was obtained before scheduled endomyocardial biopsy (EMB), and EMB was canceled with a negative dd-cfDNA. This approach was compared to using a GEP-only approach, where EMB was canceled with a negative GEP. We evaluated for frequency of EMB cancellation with dd-cfDNA usage. Results A total of 153 OHT patients over a 13-month period underwent 495 combined GEP/dd-cfDNA tests. 82.2% of dd-cfDNA tests were below threshold. Above threshold results identified high-risk patients who developed AR. 378 combined tests ≥6 months post-OHT resulted in cancellation of 83.9% EMBs as opposed to 71.2% with GEP surveillance alone. There were 2 acute cellular and 2 antibody-mediated rejection episodes, and no significant AR ≥6 months. Conclusion Routine dd-cfDNA testing alongside GEP testing yielded a significant reduction in EMB volume by re-classifying GEP (+) patients into a lower risk group, without reduction in AR detection. The addition of dd-cfDNA identified patients at higher risk for AR.

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.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.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.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).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.

The SARS‐CoV‐2 pandemic poses unique and unprecedented challenges to patients, providers, and healthcare systems. Coronavirus Disease 2019 (COVID‐19) appears to be high‐risk in patients with cardiovascular disease as well as those on immunosuppressive agents in the setting of organ transplantation. Patients with COVID‐19 have been shown to develop acute myocarditis, characterized by either viral or immune‐mediated cardiac injury.