IntroductionCardiac amyloidosis (CA) has been considered a rare disease. However, its prevalence is growing as healthcare practitioners have new, less invasive diagnostic tools that aid in the early detection of this disease. Bone scintigraphy with monoclonal protein testing is one such evidence-based, noninvasive screening option that can be offered in community healthcare facilities. This work aims to identify intervention strategies to improve the timely workup of transthyretin CA, including evidence-based practices such as bone scintigraphy and monoclonal protein testing.MethodsThe team used rapid qualitative content analysis to develop a system map of care for cardiac amyloidosis, guided by both patient and healthcare expert perspectives. To explore the map's utility and accuracy, the team conducted follow-up in-depth interviews with clinicians. They explored clinicians' perspectives on strategies to facilitate the early diagnosis of CA and explored barriers and implementation strategies that can influence the diagnostic work-up for CA.ResultsClinicians provided recommendations for how to expand the map, particularly in terms of including additional healthcare professionals, screening strategies, and barriers to care for patients. After expanding the preliminary system map of care, the team identified 23 distinct strategies to improve the early detection of CA. Clinicians recommended additional strategies, such as patient and provider education, patient take-back, the use of artificial intelligence with electronic health record data, and the formation of healthcare teams specializing in cardiac amyloidosis, to enhance early detection and treatment of this condition.DiscussionThis work can inform researchers and clinicians about selecting and implementing intervention and implementation strategies within health systems to enhance the early detection of cardiac amyloidosis.
Introduction Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed, progressive, and fatal disease of the heart. The Veterans Health Administration is America’s largest integrated health care system, providing care at 170 medical centers and 1193 outpatient sites of care, and serving 9.1 million enrolled veterans each year. Regional differences in the incidence of ATTR-CM in the Veterans Affairs (VA) population have not been reported and, if present, may offer opportunities to improve health equity. Hypothesis Geospatial healthcare differences in ATTR-CM incidence exist in the United States (US) VA population. Methods Cohort study using historical data from routine clinical care of veterans across all VA facilities in the US (Jan 2012 to Dec 2021), accessed from electronic records. ATTR-CM was defined using a combination of ICD-9/10 codes for transthyretin amyloidosis and heart failure. Incidence rates, defined as cases of ATTR-CM per 100,000 person-years (PY), were analyzed by state/territory for the time periods before disease-modifying therapy (DMT) availability (2016-2018) and after (2019-2021). Rates were standardized using the direct method to the overall VA population by age, sex, and race. Results A total of 2136 patients with ATTR-CM from the VA were included in this analysis. Before DMT availability, the median ATTR-CM incidence was 2.39 (range, 2.05-2.74) per 100,000 PY, which increased to 4.86 (range, 4.38-5.34) post-DMT availability. Overall, Puerto Rico (23.38), Utah (17.62), Rhode Island (17.58), and Oregon (15.31) were associated with the highest standardized incidence rates of ATTR-CM post-DMT availability (Figure 1A). Comparing the change in ATTR-CM incidence from pre- to post-DMT availability, many states/territories demonstrated an increase in incidence rates between these periods (Figure 1B). Utah, Puerto Rico, Rhode Island, South Dakota, Arizona, and Oregon were associated with the greatest increases in ATTR-CM incidence from pre- to post-DMT availability. The greatest decreases in ATTR-CM incidence from pre- to post-DMT availability were seen in New Mexico and Nevada. Conclusions The incidence of ATTR-CM in the VA population increased nationally in the setting of available treatment, possibly due to improved awareness in most states. However, geographic differences exist in the incidence of ATTR-CM at the state-level, with some high-incidence states continuing to improve and lagging states falling further behind. Further investigation is needed around factors, such as access to cardiovascular care and disease awareness, to understand the reasons for regional ATTR-CM detection differences and how to improve care across VA facilities.
Background Despite heightened awareness of cardiac amyloidosis (CA), the care of patients with CA remains challenging, particularly at the Veterans Affairs (VA), where populations are predominantly older and male. Objectives This study aimed to characterize the current landscape of CA care across the VA. Methods As a quality improvement study, a survey instrument was developed and disseminated to all 127 VA cardiology programs from March to May 2025. The survey captured data on diagnostic modality availability, therapeutic resources, multidisciplinary care team composition, and self-designated amyloidosis center designation. Results Complete responses were received from 54 cardiology programs (42.5%). There was substantial variability in program-level experience, clinician comfort, access to diagnostics, and therapeutics regardless of clinical complexity index designation. Thirty-one survey respondents (57.4%) reported feeling “very comfortable” treating CA, although 7 (13.0%) reported being “hesitant” to provide care for Veterans with CA. Twelve programs (22.2%) reported having only one cardiologist managing CA patients, and 7 (13.0%) indicated that no cardiologists at their center treated Veterans with CA. Only 4 programs (7.4%) reported having a formalized multidisciplinary CA team. Lastly, facility complexity did not have an obvious relationship to size, scope, or readiness to care for Veterans with CA. Conclusions The VA appears strongest at the initial diagnostic stages, with high availability to initiate a workup for transthyretin CA, with considerable latent capacity to support comprehensive CA care including a high-risk patient population, widespread diagnostic capabilities, national access to therapies, and robust telehealth infrastructure. Targeted implementation to standardize CA care delivery, while leveraging existing strengths, could meaningfully improve care for Veterans with CA.
BACKGROUND:Heart failure is a clinical syndrome resulting from numerous pathological conditions. One cause of heart failure, transthyretin cardiac amyloidosis, presents insidiously with common and seemingly unrelated symptoms. New treatments for cardiac amyloidosis are available that extend and improve life. However, providers are not testing patients for transthyretin cardiac amyloidosis. We took a systems science approach to explore the system of care for transthyretin cardiac amyloidosis testing by depicting the healthcare system from patient presentation to treatment. Our goal was to define an ideal healthcare system to improve the uptake of testing protocols and enhance patient outcomes. METHODS:We assembled clinicians, researchers, and patients to participate in a co-design workshop using the first step of System Evaluation Theory to define an ideal testing and diagnostic protocol using transthyretin cardiac amyloidosis as a case study. We tasked workshop attendees with defining the patient and clinician journey from symptom presentation to diagnosis. We generated a system map using a qualitative matrix analysis of a transcript of the workshop discussion. RESULTS:The matrix analysis organized input from all stakeholders, allowing for the creation of a system map that reveals the complexity of the transthyretin cardiac amyloidosis testing process and potential implementation strategies to improve the efficiency and effectiveness of the system. This methodology successfully yielded generalizable elements of a testing protocol and testable strategies to facilitate the implementation of a protocol adapted to fit local site needs. CONCLUSIONS:The substeps outlined within System Evaluation Theory Step 1 helped identify an ideal system for testing and diagnosing transthyretin cardiac amyloidosis care that could be applied to specific settings to identify, improve, and implement protocols for other complex diseases.
BACKGROUND:Cognitive impairment (CI) is increasingly recognised as a common comorbidity among older adults with heart failure (HF) and is associated with reduced self-care, poor medication adherence, and worse clinical outcomes. Reported prevalence varies widely across studies due to differences in populations, cognitive assessment tools, and geographic regions. This systematic review and meta-analysis aimed to estimate the global prevalence of CI among older adults with HF and examine sources of variability across studies. METHODS:This systematic review and meta-analysis was conducted in accordance with PRISMA guidelines. PubMed and Scopus were searched from database inception to October 2024, with Google Scholar used as a supplementary source. Observational studies including adults aged ≥65 years with clinically diagnosed HF and validated cognitive assessments were included. Pooled prevalence estimates were calculated using a random-effects model with double arcsine (Freeman-Tukey) transformation. Heterogeneity was assessed using the I2 statistic. Prespecified subgroup analyses were performed by age, geographic region, and study design. Sensitivity analyses were conducted using a leave-one-out approach. RESULTS:Twenty-five studies comprising 12,112 older adults with HF (mean age 80.0±4.9 years; 52.6% female) were included. Overall, 3,669 patients had CI, yielding a pooled prevalence of 43.5% (95%-confidence interval: 35.2%-51.9%) with substantial heterogeneity (I2=98.6%). CI prevalence was highest in the United States (50.9%), followed by Asia (46.5%) and Europe (35.0%). Patients aged ≥80 years demonstrated higher prevalence (48.6%) compared with those aged 70-79 years (38.9%). Prospective studies reported higher prevalence than retrospective designs. Sensitivity analyses confirmed the robustness of the findings. CONCLUSIONS:Approximately 44% of older adults with HF have CI, representing a substantial global burden. These findings support routine cognitive screening in older HF populations to guide clinical decision-making and improve outcomes. Future studies should evaluate longitudinal cognitive trajectories and interventions aimed at improving both cognitive and cardiovascular outcomes.
Background Transthyretin amyloid cardiomyopathy (ATTR‐CM) is a progressive, fatal disease associated with a substantial health care system burden. The aim of this study was to compare health care resource utilization and associated costs among individuals with ATTR‐CM, with non‐amyloid heart failure (HF), and without HF in the United States. Methods Optum's de‐identified Clinformatics Data Mart Database (January 2016 to September 2023) was used to identify patients with ATTR‐CM based on HF and/or cardiomyopathy claims within 2 years of the first ATTR diagnosis. Patients with ATTR‐CM were matched to patients with non‐amyloid HF (HF/cardiomyopathy without ATTR) and non‐HF controls using 1:1 propensity score matching. All‐cause and cardiovascular‐related hospitalizations and costs were compared during follow‐up. Results Each matched cohort included 4571 patients (mean age, 75.3–75.5 years; 56.0%–56.6% male; mean follow‐up: 2.9–3.2 years). The ATTR‐CM, non‐amyloid HF, and non‐HF cohorts had 3.3, 2.7, and 1.5 all‐cause hospitalizations per patient during follow‐up, respectively. Compared with non‐amyloid HF, patients with ATTR‐CM had a longer mean length of stay per hospitalization (all‐cause and cardiovascular‐related: 8.0 versus 7.5 days; P <0.001), more mean days hospitalized annually per patient (all‐cause: 10.6 versus 8.4; cardiovascular‐related: 10.5 versus 8.3 [both P <0.001]), and higher mean annual inpatient hospitalization costs per patient (all‐cause: $42 868 versus $36 061; cardiovascular‐related: $42 841 versus $36 027 [both P <0.001]). In ATTR‐CM subgroups, mean cardiovascular‐related length of stay and days hospitalized were higher among patients aged 46 to 64 years and 65 to 80 years (versus >80 years), Black patients (versus White patients), and patients in the Northeast or South US regions (versus West regions). Conclusions ATTR‐CM is associated with a higher health care resource utilization/cost burden than non‐amyloid HF and the general population.
Objectives:Despite the rapid development of AI in clinical medicine, reproducibility and methodological limitations hinder its clinical utility. In response, MINimum Information for Medical AI Reporting (MINIMAR) standards were introduced to enhance publication standards and reduce bias, but their application remains unexplored. In this review, we sought to assesses the quality of reporting in AI/ML studies of cardiac amyloidosis (CA) an increasingly important cause of heart failure. Materials and Methods:Using PRISMA-ScR guidelines, we performed a scoping review of English-language articles published through May 2023 which applied AI/ML techniques to diagnose or predict CA. Non-CA studies and those with selective feature sets were excluded. Two researchers independently screened and extracted data. In all, 20 studies met criteria and were assessed for adherence to MINIMAR standards. Results:The studies showed variable compliance with MINIMAR. Most reported participant age (90%) and gender (85%), but only 25% included ethnic or racial data, and none provided socioeconomic details. The majority (95%) developed diagnostic models, yet only 85% clearly described training features, and 20% addressed missing data. Model evaluation revealed gaps; 80% reported internal validation, but only 20% conducted external validation. Discussion and Conclusion:This study, one of the first to apply MINIMAR criteria to ML research in CA, reveals significant variability and deficiencies in reporting, particularly in patient demographics, model architecture, and evaluation. These findings underscore the need for stricter adherence to standardized reporting guidelines to enhance the reliability, generalizability, and clinical applicability of ML/AI models in CA.
BACKGROUND:Cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure (HF) that disproportionately impacts men of African descent. Without a standardized method of screening and scattered patient health information, clinicians must integrate data that spans multiple disease systems and is stored across the electronic health record. OBJECTIVES:The aim of this project was to create a dashboard to facilitate identification of high-risk African American (AA) veterans who would benefit from CA screening tests. This paper described the development of the dashboard and identified barriers and opportunities in dashboard development. METHODS:Three Veterans Affairs (VA) health systems participated in this project. Microsoft Structured Query Language (SQL) Report Builder was utilized to create an interactive dashboard that refreshes daily through stored procedures using SQL Server Integration Services and the SQL Server Job Agent. Inclusion criteria included AA patients less than 90 years old with a history of HF. The 2023 American College of Cardiology/American Heart Association consensus statement on diagnosis and treatment of transthyretin CA was the source of evidence in creating the inclusion criteria and parameters of interest. RESULTS:The final dashboard contained 1,732 HF patients who met inclusion criteria, of which 949 (55%) were identified as high risk. We faced several challenges in this project, including time required for dashboard development, limited team experience in specifying dashboard requirements, identifying informatics counterparts at all sites, and standardizing data across three VA hospitals. CONCLUSION:In this clinical improvement project, we created a dashboard that identifies AA veterans with HF at risk for CA and that can help to mitigate the impact of CA on this population.
Introduction: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive, often fatal disease. Recognition and diagnosis of ATTR-CM have increased due to greater awareness, improved diagnostics, and introduction of disease-modifying treatments (DMT). Hypothesis: Geographic disparities in ATTR-CM prevalence exist due to healthcare system and societal factors that impact the Veterans Affairs (VA) population. Methods: This study included a retrospective analysis of electronic health records of veterans with ATTR-CM (01/2012-12/2021) across all VA facilities in the United States (US). The primary outcome of this analysis was ATTR-CM prevalence, defined as cases per 100,000 person-years (PY). Prevalence was analyzed by state after the approval of DMT (2019). The distribution of US amyloidosis centers, classified by the International Society of Amyloidosis, Amyloidosis Foundation, and/or Amyloid Research Consortium, was also assessed. Results: A total of 2433 patients with ATTR-CM were included. The majority (85.7%) were aged ≥65 years, with 20.8% aged ≥85 years. Most (86.8%) patients were male; 51.5% were White and 41.3% were Black/African American. ATTR-CM prevalence in veterans was 6.1 per 100,000 PY in 2012 and increased to 16.8 in 2021 ( Figure 1 ). After 2019, the prevalence in veterans was high in Oregon (50.9), Utah (39.5), and South Dakota (35.4), and in many states in the Northeast (Massachusetts, 43.2; Rhode Island, 43.0; Vermont, 33.6; Connecticut, 29.3) ( Figure 2 ). Prevalence appeared to correlate with the distribution of amyloidosis centers in many regions, though prevalence was high in New Mexico and South Dakota despite the lack of dedicated amyloidosis centers. Conclusion: The documented prevalence of ATTR-CM increased over time in US veterans, though geographic disparities exist at the state level that appear to correlate with access to amyloidosis centers. For regions with lower-than-expected prevalence, strategies are needed to address regional disparities in disease awareness, diagnosis, and access to care.
Background : Addressing the global underutilization of essential heart failure (HF) medications like sodium-glucose co-transporter inhibitors (SGLT2i) and mineralocorticoid receptor antagonists (MRAs) is crucial to bridging treatment gaps. This study introduces the use of nudge interventions for clinicians to enhance medication use, with the idea that these interventions can be crucial to improving care for our heart failure patients. Methods : Our innovative approach tackles SGLT2i and MRA underutilization through a multicomponent nudge strategy, utilizing cognitive biases and nudge design within the VA healthcare system. This study focuses on clinician behavior change, aiming to promote appropriate prescriptions in a randomized controlled trial involving cardiology and primary care clinicians. Data collection includes demographics, vitals, comorbidity index, and implementation measures, contributing to closing the HF treatment gap, enhancing healthcare quality, and improving population health. Results : Anticipated outcomes involve evaluating the effectiveness of a multicomponent nudge strategy in increasing SGLT2i and MRA prescriptions by studying whether clinicians improve habits with the use of an informational-based alert or a peer-comparison alert. We aim to measure prescription rates within 30 days of appointments and assess safety, reach, adoption, and implementation. The study explores intervention adoption patterns and their impact on medication prescriptions within HFrEF and HFpEF subgroups, offering insights into potential clinician behavior change and healthcare system optimization. Conclusion : This study protocol introduces an innovative approach to address underutilized HF medications in a healthcare system. By advancing behavior change interventions, it aims to close the HF treatment gap and improve patient outcomes. By emphasizing an implementation science approach, this study highlights the importance of understanding clinician behaviors' role and its effects on patient care.
Background Shared medical appointments (SMAs) in heart failure (HF) are medical visits where several patients with HF meet with multidisciplinary providers at the same time for efficient and comprehensive care. It is unknown whether HF‐SMAs can improve overall and cardiac health status for high‐risk patients with HF discharged from acute care. Methods and Results A 3‐site, open‐label, randomized‐controlled‐trial was conducted. Participants within 12 weeks of HF acute care (emergency‐room/hospitalization) requiring intravenous diuretic therapy were randomized to receive either HF‐SMA or usual HF clinical care (usual‐care) on a 1:1 ratio. The HF‐SMA team, which consisted of a nurse, nutritionist, psychologist, nurse practitioner and/or a clinical pharmacist, provided four 2‐hour session HF‐SMAs that met every other week for 8 weeks. Primary outcomes were the overall health status measured by European Quality of Life Visual Analog Scale and cardiac health status by Kansas City Cardiomyopathy Questionnaire, 180 days postrandomization. Of the 242 patients enrolled (HF‐SMA n=117, usual‐care n=125, mean age 69.3±9.4 years, 71.5% White patients, 94.6% male), 84% of participants completed the study (n=8 HF‐SMA and n=9 usual‐care patients died). After 180 days, both HF‐SMA and usual‐care participants had significant improvements from baseline in Kansas City Cardiomyopathy Questionnaire that were not statistically different. Only HF‐SMA participants had significant improvements in European Quality of Life Visual Analog Scale (mean change = 7.2±15.8 in HF‐SMA versus −0.4±19.0 points in usual‐care, P < 0.001). Conclusions Both HF‐SMA and usual‐care in participants with HF achieved significant improvements in cardiac health status, but only a team approach through HF‐SMA achieved significant improvements in overall health status. Future larger studies are needed to evaluate hospitalization and death outcomes. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT02481921.
Background: Shared medical appointments (SMAs) in heart failure (HF) are medical visits where several patients with HF meet with multidisciplinary providers at the same time for efficient and comprehensive care. It is unknown whether HF-SMAs can improve overall and cardiac health status for high-risk patients discharged with HF. Methods: A 3-site, open-label, randomized-controlled-trial was conducted. Participants within 12 weeks of HF hospitalization were randomized to receive either HF-SMA or usual HF clinical care (usual-care) on a 1:1 ratio. The HF-SMA team, which consisted of a nurse, nutritionist, psychologist, nurse practitioner and/or a clinical pharmacist, provided four 2-hour session HF-SMAs that met every other week for 8 weeks. Primary outcomes were the overall health status measured by EQ5D-VAS and cardiac health status by KCCQ, 180 days post-randomization. Results: Of the 242 patients enrolled (HF-SMA n=117, usual-care n=125, mean age 69.3±9.4 years, 71.5% white, 94.6% male), 84% of participants completed the study (n=8 HF-SMA and n=9 usual-care patients died). After 180 days, both HF-SMA and usual-care participants had similar and significant improvements from baseline in KCCQ, but only HF-SMA participants had significant improvements in EQ5D-VAS (mean change = 7.2 +/- 15.8 in HF-SMA versus -0.4 +/- 19.0 points in usual-care, p<0.001). Conclusion: Both HF-SMA and usual care in HF participants achieved significant improvements in cardiac health status, but only a team approach through HF-SMA achieved significant improvements in overall health status. A larger population and a longer follow-up time are needed in future studies to evaluate re-hospitalization and death outcomes.### Competing Interest StatementThe authors have declared no competing interest.### Clinical TrialClinicalTrials.gov ID: [NCT02481921][1]### Funding StatementThis research was supported by a VA HSRD Merit Review Grant IIR 14-293.### Author DeclarationsI confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained.YesThe details of the IRB/oversight body that provided approval or exemption for the research described are given below:The Institutional Review Board and Research and Development Committees at the Providence, Phoenix, and Cleveland Veterans Affairs Medical Centers approved the protocol.I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals.YesI understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance).YesI have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable.YesThe data that support the findings of this study are available from the corresponding author upon reasonable request. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT02481921&atom=%2Fmedrxiv%2Fearly%2F2024%2F03%2F07%2F2024.03.04.24303754.atom
BackgroundIn 2020, the Veterans Affairs (VA) health care system deployed a heart failure (HF) dashboard for use nationally. The initial version was notably imprecise and unreliable for the identification of HF subtypes. We describe the development and subsequent optimization of the VA national HF dashboard.Materials and MethodsThis study describes the stepwise process for improving the accuracy of the VA national HF dashboard, including defining the initial dashboard, improving case definitions, using natural language processing for patient identification, and incorporating an imaging-quality hierarchy model. Optimization further included evaluating whether to require concurrent ICD-codes for inclusion in the dashboard and assessing various imaging modalities for patient characterization.ResultsThrough multiple rounds of optimization, the dashboard accuracy (defined as the proportion of true results to the total population) was improved from 54.1% to 89.2% for the identification of HF with reduced ejection fraction (HFrEF) and from 53.9% to 88.0% for the identification of HF with preserved ejection fraction (HFpEF). To align with current guidelines, HF with mildly reduced ejection fraction (HFmrEF) was added to the dashboard output with 88.0% accuracy.ConclusionsThe inclusion of an imaging-quality hierarchy model and natural-language processing algorithm improved the accuracy of the VA national HF dashboard. The revised dashboard informatics algorithm has higher use rates and improved reliability for the health management of the population.
Background: Prior studies suggest variability in cardiac amyloidosis (CA) detection across the U.S. with underdiagnosis in some regions. Research Question: It is unknown whether geographic variation exists in the occurrence of CA amongst U.S. Veterans in a contemporary cohort. Method: This is a retrospective observational cohort study. CA was defined as ≥ 1 heart failure (ICD9/10) and ≥ 1 amyloidosis diagnosis. For geographic mapping the location was the state of the diagnosing Veterans Affairs (VA) facility. The population at risk was patients in each state with ≥1 VA encounter during the year. The outcomes were incidence and prevalence rates of CA at the state and U.S. Census regional level (per 100,000 patients). The data source was the VA Corporate Data Warehouse (MDClone ADAMS platform). Results: The incidence and prevalence rates of CA by region are shown in the Table, and by state in the Figure. There was an increase in incidence and prevalence in all regions in 2012 and 2021. In both years, incidence and prevalence were highest in the Northeast and lowest in the South. Conclusion: Despite increasing recognition of CA in the VA Health System, there is regional variation in incidence and prevalence in 2012 and 2021. VA facilities in the Midwest, West and especially the South may have differences in CA detection relative to those in the Northeast region. Further research into health system, provider, and patient factors leading to this variation is needed to ensure health equity and quality of care for patients with CA.