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Background Despite the improved awareness of cardiac amyloidosis among clinicians, its incidence and prevalence is not well-described in a community setting. We sought to investigate the incidence and prevalence of cardiac amyloidosis in the community. Methods and Results In the adult population of Alberta, we examined 3 cohorts: (1) probable cases of cardiac amyloidosis: the presence of physician-assigned diagnosis of amyloidosis (International Classification of Diseases [ICD]-10 code E85; ICD-9 277.3) and 1 or more health care encounter for heart failure (HF) (ICD-10 I50; ICD-9 428); (2) possible cardiac amyloidosis: the presence of clinical phenotypes suggestive of amyloidosis; and (3) a comparator HF cohort without amyloidosis. Between 2004 and 2018, 982 of the 145,329 patients with HF were identified as probable cardiac amyloidosis. During the same period, the incidence rates of probable cardiac amyloidosis increased from 1.38 to 3.69 per 100,000 person-years and the prevalence rates increased from 3.42 to 14.85 per 100,000 person-years (Ptrend Conclusions The incidence and prevalence of cardiac amyloidosis has increased over the last decade. Given the advent of new therapies for cardiac amyloidosis and considering their high cost, it is imperative to devise strategies to screen, identify, and track patients with cardiac amyloidosis from administrative databases.

Background Surveillance and outcome studies for heart failure (HF) require accurate identification of patients with HF. Algorithms based on International Classification of Diseases (ICD) codes to identify HF from administrative data are inadequate owing to their relatively low sensitivity. Detailed clinical information from electronic medical records (EMRs) is potentially useful for improving ICD algorithms. This study aimed to enhance the ICD algorithm for HF definition by incorporating comprehensive information from EMRs. Methods The study included 2106 inpatients in Calgary, Alberta, Canada. Medical chart review was used as the reference gold standard for evaluating developed algorithms. The commonly used ICD codes for defining HF were used (namely, the ICD algorithm). The performance of different algorithms using the free text discharge summaries from a population-based EMR were compared with the ICD algorithm. These algorithms included a keyword search algorithm looking for HF-specific terms, a machine learning–based HF concept (HFC) algorithm, an EMR structured data based algorithm, and combined algorithms (the ICD and HFC combined algorithm). Results Of 2106 patients, 296 (14.1%) were patients with HF as determined by chart review. The ICD algorithm had 92.4% positive predictive value (PPV) but low sensitivity (57.4%). The EMR keyword search algorithm achieved a higher sensitivity (65.5%) than the ICD algorithm, but with a lower PPV (77.6%). The HFC algorithm achieved a better sensitivity (80.0%) and maintained a reasonable PPV (88.9%) compared with the ICD algorithm and the keyword algorithm. An even higher sensitivity (83.3%) was reached by combining the HFC and ICD algorithms, with a lower PPV (83.3%). The structured EMR data algorithm reached a sensitivity of 78% and a PPV of 54.2%. The combined EMR structured data and ICD algorithm had a higher sensitivity (82.4%), but the PPV remained low at 54.8%. All algorithms had a specificity ranging from 87.5% to 99.2%. Conclusions Applying natural language processing and machine learning on the discharge summaries of inpatient EMR data can improve the capture of cases of HF compared with the widely used ICD algorithm. The utility of the HFC algorithm is straightforward, making it easily applied for HF case identification.

Introduction Hereditary transthyretin amyloidosis (ATTRv) is often associated with progressive infiltrative cardiac involvement, with cardiac symptoms occurring sometimes years prior to diagnosis. Hypothesis ATTRv patients demonstrate significant cardiovascular symptom manifestations and healthcare utilization prior to diagnosis. Methods Newly diagnosed ATTRv patients in IBM® MarketScan® Commercial and Medicare Supplemental data (US) were identified using a claims-based algorithm. Diagnosis required ≥1 claim with relevant amyloidosis diagnosis code (ICD-10-CM: E85.0-.4, E85.89, E85.9) during identification (ID) period (1/2016-12/2017), and the occurrence of ≥1 qualifying criteria during 2011-2017: ≥15 days diflunisal use, liver transplant, or claim with code E85.1 (neuropathic heredofamilial amyloidosis) or E85.2 (heredofamilial amyloidosis unspecified). Index date was defined as date of first claim with amyloidosis code in ID period, and the pre-index period as the 5 years prior to index date. Patients with an ICD-9/10 amyloidosis code during this period were excluded to focus on the newly diagnosed. Control subjects without ATTRv were identified and matched (age, gender, region) to ATTRv patients (ratio 3:1); meeting the same index and enrollment requirements. Frequency of selected cardiovascular (CV) conditions and healthcare use were measured in the pre-index period; demographics and Charlson comorbidity index (CCI) were measured 1 year pre-index. Results Among 141 patients with ATTRv and 423 matched controls, mean (SD) age was 62.5 (14.2) years and 53.9% were female. Mean CCI for ATTRv patients was 2.7 (3.0) vs 1.1 (1.9) for controls. In the pre-index period, CV conditions were more common among ATTRv patients relative to controls: chest pain (51.8 vs. 28.1%), dyspnea (49.6 vs 25.8%), bleeding (41.8 vs 21.3%), edema (27.0 vs 12.8%), heart failure (23.4 vs 5.9%), ventricular hypertrophy (19.9 vs 5.7%), hypotension (18.4 vs 6.1%), atrial fibrillation/flutter (18.0 vs 8.9%), stroke (12.8 vs 6.1%), and aortic stenosis (10.6 vs 4.5%); conditions were relatively higher in each pre-index year. Hospitalization (47.5 vs. 24.3%), emergency department visits (60.3 vs. 47.0%) and cardiac testing (81.6 vs. 65.7%) were also more frequent among ATTRv patients. Median time (months) to ATTRv diagnosis from initial symptom manifestation were largest for chest pain (43.0), dyspnea (41.1), aortic stenosis (39.2), and atrial fibrillation/flutter (34.3). Conclusions ATTRv patients have considerable CV disease burden in the 5 years preceding diagnosis. Increased awareness of characteristic CV manifestations may increase clinical suspicion, leading to early diagnosis and prompt intervention.

Introduction In the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT), tafamidis significantly reduced all-cause mortality and cardiovascular-related hospitalizations in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). Tafamidis inhibits formation of amyloid and may result in improvement of additional endpoints associated with disease stage. Hypothesis Patients with ATTR-CM treated with tafamidis are more likely to show improvement in functional capacity, health related quality-of-life and cardiac biomarkers compared with patients treated with placebo. Methods In ATTR-ACT, 441 adult patients with ATTR-CM (variant or wild-type) were randomized (2:1:2) to tafamidis 80 mg, tafamidis 20 mg, or placebo for 30 months, with pooled tafamidis (80mg and 20mg; n = 264) compared with placebo (n = 177). In this post-hoc analysis, change from baseline to Month 30 was assessed to determine the proportion of patients who improved in: 6-minute walk test distance (6MWT); Kansas City Cardiomyopathy Questionnaire-Overall Summary (KCCQ-OS) score; Patient Global Assessment (PGA); NYHA class; and NT-proBNP concentration. Results In total, 33.5% of tafamidis-treated patients had an improved 6MWT distance, compared with 12.9% of placebo-treated patients. KCCQ-OS score improved in 41.8% of patients treated with tafamidis compared with 21.4% with placebo. As reported on the PGA, a greater proportion of patients were ‘very much improved’, ‘much improved’, or ‘minimally improved’ with tafamidis (42.3%) compared with placebo (23.8%). For patients with baseline NYHA Class II, 8.2% of tafamidis-treated patients and 3.7% of placebo-treated patients improved to Class I. There was a greater proportion of patients whose NT-proBNP concentration decreased with tafamidis (37.6%) compared with placebo (12.5%). Conclusions ATTR-CM is a progressive disease for which either stabilization or improvement indicates an efficacious treatment. In ATTR-ACT over one-third of patients at Month 30 had improvements in measures of functional capacity, health-related quality of life, and cardiac biomarkers. These data provide evidence of clinical improvement for patients with ATTR-CM treated with tafamidis, in addition to the benefits in survival and reduced cardiovascular hospitalization.