Prevalence Of Cardiac Amyloidosis Research Articles

Prevalence of Cardiac Amyloidosis in Patients Undergoing Echocardiography at a Tertiary Center.

Prevalence of Cardiac Amyloidosis in Patients Undergoing Echocardiography at a Tertiary Center.

Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis

Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis

Prevalence of Cardiac Amyloidosis Among Elderly Patients With Recent-Onset Atrial Fibrillation: The PREVAL-ATTR Study

BackgroundTransthyretin cardiac amyloidosis (ATTR-CA) is increasingly recognized as a treatable form of heart failure. Atrial fibrillation (AF) is common in patients with ATTR-CA. Whether recent-onset AF can be used as an early marker to identify patients with ATTR-CA has not been elucidated. MethodsThis was a prospective study conducted at 3 Spanish centres. ATTR-CA noninvasive screening was offered to patients ≥ 65 years of age recently diagnosed (< 1 year) with nonvalvular AF and who had ≥ 1 echocardiographic, electrocardiographic, or clinical sign suggestive of ATTR-CA. ResultsA total of 121 patients were included (75% male, mean age 77 ± 7 years). Ten patients (8.3%; 95% confidence interval [CI],4-14.7%), were diagnosed with cardiac amyloidosis (CA): 5 with definite wild-type ATTR-CA (ATTRwt), 4 with likely ATTRwt, and 1 with undetermined CA. Compared with patients without CA, patients with CA were older (84 ± 4 vs 76 ± 7 years; P < 0.001), more frequently men (90% vs 59%; P = 0.047), presented higher median N-terminal pro-B-type natriuretic peptide (NTproBNP) (3800 pg/L, interquartile range [IQR]:1682-6101 vs 1048 pg/mL, IQR: 427-3154; P = 0.017) and higher left ventricular hypertrophy (LVH) (14 mm, IQR: 13-17 vs 12 mm, IQR: 12-13; P = 0.003). Patients with CA also showed higher rate of permanent AF (90% vs 49.5%; P = 0.018) and a greater need for pacemaker implantation during follow-up (30% vs 7.3%; P = 0.049). No differences in mortality were observed between patients with and without CA after a median follow-up of 13 months (IQR: 11-16 months). ConclusionsRoutine DPD scanning in elderly patients with recent-onset AF, LVH and an additional red flag may help to identify patients with ATTR-CA. However, larger studies evaluating this strategy in more diverse clinical settings would be required.

Prevalence of cardiac amyloidosis in atrial fibrillation: a CMR study prior to catheter ablation.

The frequency of cardiac amyloidosis potentially present in patients with atrial fibrillation (AF) remains unclear. The purpose of this study is to determine the frequency and clinical characteristics of cardiac amyloidosis latent in AF by performing cardiac magnetic resonance imaging (MRI) in patients scheduled for AF ablation. We retrospectively analyzed 193 consecutive patients who underwent CA and cardiac MRI for atrial fibrillation. The primary endpoint of the study was the frequency of histologically confirmed cardiac amyloidosis or suspected cardiac amyloidosis [positive imaging findings on cardiac MRI strongly suspecting cardiac amyloidosis (diffuse subendocardial late gadolinium enhancement or MRI-derived extracellular volume of > 0.40)]. Among the 193 patients, 8 were confirmed or suspected cases of cardiac amyloidosis, representing a frequency of 4% (8/193 patients). Multivariate analysis identified interventricular septal thickness at end-diastole (LVSd) as an independent and significant predictor of cardiac amyloidosis (OR: 1.72, 95% CI 1.12-2.87, p = 0.020).The optimal cut-off value for IVSd was determined to be > 12.9mm based on the Youden index. At this cut-off, the sensitivity was 75.0% (95% CI 34.9-96.8%) and the specificity was 92.3% (95% CI 87.4-95.7%), allowing for the identification of patients with definite or suspected cardiac amyloidosis. The frequency of confirmed and suspected cases of cardiac amyloidosis among patients with an IVSd > 12.9mm was 30% (6/20 patients). In addition, prevalence of biopsy-proven cardiac amyloidosis was 10% (2/20). The prevalence of cardiac amyloidosis in atrial fibrillation patients scheduled for ablation with cardiac hypertrophy is not negligible.

Clinical characteristics of patients with high extracellular volume fraction evaluated by cardiac computed tomography for coronary artery evaluation.

This study aims to evaluate the distribution of extracellular volume fraction detected via computed tomography, clinical characteristics of high extracellular volume fraction detected via computed tomography, and the rate of incidental detection of cardiac amyloidosis in patients undergoing cardiac computed tomography for coronary artery evaluation. This study included 874 consecutive patients (mean age, 74.4 ± 7.1 years; men, 65%), comprising men aged ≥60 years and women aged ≥70 years, who had undergone cardiac computed tomography between January 2020 and September 2022. The mean extracellular volume fraction detected via computed tomography was 29.7 ± 5.2%, and 108 patients (12.4%) had an extracellular volume fraction detected via computed tomography of ≥35%. Older age (75.9 ± 8.2 years vs. 74.2 ± 6.9 years; P = 0.042), male sex (75.9% vs. 63.0%; P = 0.007), impaired left ventricular ejection fraction, increased high-sensitivity cardiac troponin T and B-type natriuretic peptide levels, and increased left ventricular thickness showed significant associations with an extracellular volume fraction detected via computed tomography of ≥35%. Cardiac amyloidosis was diagnosed incidentally in 15 patients based on an increase in extracellular volume fraction detected via computed tomography. The prevalence of cardiac amyloidosis was 1.7% (15/874) and 14.3% (15/105) in the entire study population and in patients with an extracellular volume fraction detected via computed tomography of ≥35%, respectively. An increase in the extracellular volume fraction detected via computed tomography was suggestive of cardiac amyloidosis. Elevated extracellular volume fraction detected via computed tomography, associated with elevated cardiac biomarker levels and myocardial structural changes, may lead to the incidental diagnosis of cardiac amyloidosis.

Prevalence of cardiac amyloidosis in patients undergoing carpal tunnel release with amyloid deposition in removed tenosynovium

Abstract Background Carpal tunnel syndrome (CTS) is an early symptom and important clinical indicator for the diagnosis of cardiac amyloidosis. Amyloid deposition in removed tenosynovium during carpal tunnel release (CTR) is often confirmed; however, the prevalence of concomitant cardiac amyloidosis is not yet understood. Purpose This study aimed to evaluate the prevalence of amyloid deposition in removed tenosynovium in patients who underwent CTR and to clarify the prevalence of concomitant cardiac amyloidosis in patients who had amyloid deposition in removed tenosynovium. Methods Overall, 669 patients who underwent CTR were prospectively examined and evaluated for amyloid deposition after tenosynovium removal. Invitation and proposal letters for cardiac screening were sent to patients with amyloid deposition. Electrocardiography, echocardiography, and biomarkers were evaluated to assess the likelihood of cardiac amyloidosis. Results Amyloid deposition was observed in 261 (39%) patients, who were significantly older and predominantly male (p&amp;lt;0.05). Among them, 111 patients (mean age; 70.8 ± 7.3 years old; men: 55%) agreed to cardiac screening. 99mTc-labeled pyrophosphate (99mTc-PYP) scintigraphy was performed in 12 patients who met the following criteria: 1) interventricular septum diameter (IVSd) ≥14 mm or 2) 12 mm ≤ IVSd &amp;lt;14 mm with above-normal limits in high-sensitivity cardiac troponin T (hs-cTnT; &amp;gt;0.014 ng/mL). Six patients (50%) had positive findings on 99mTc-PYP scintigraphy and were diagnosed with wild-type transthyretin cardiac amyloidosis with Mayo prognostic stage I. The prevalence of concomitant cardiac amyloidosis was observed in 6/111 (5.4%) CTR patients with amyloid deposition and 50% (6/12) in patients with left ventricular hypertrophy (≥12 mm) with increased hs-cTnT levels. Conclusion Amyloid deposition in removed tenosynovium was frequently observed in elderly men with CTS. Cardiac screening may be useful for the early diagnosis of cardiac amyloidosis in patients undergoing CTR with amyloid deposition. This knowledge should be shared with orthopedic surgeons and cardiologists for the early diagnosis of cardiac amyloidosis.Flowchart of participant selectionGraphical Abstract

Abstract 13751: Clinical Characteristics With High Extracellular Volume Fraction in Patients Who Performed Cardiac Computed Tomography Angiography for the Purpose of Evaluating Coronary Arteries

Background: Cardiac computed tomography angiography (CCTA) is useful for the evaluation of anatomical coronary artery lesions. Recently, extracellular volume fraction by computed tomography (ECV CT ) have become possible by imaging the delay phase after coronary CT angiography. ECV CT has been reported to be useful for detailed examination and prognosis prediction after therapeutic intervention of cardiomyopathy. Cardiac amyloidosis is known as a cardiomyopathy with noticeable increased ECV, and cardiac amyloidosis should be suspected in patients with high ECV. The aim of this study was to evaluate clinical characteristics of high ECV CT and incidental detection of cardiac amyloidosis in patients who underwent CCTA for the purpose of evaluation of coronary arteries. Methods and Results: We examined consecutive 875 patients (mean age; 74.3 ± 7.1 years, men; 64%) who underwent CCCA in men aged ≥ 60 years and women aged ≥ 70 years. ECV CT quantification was performed with pre-contrast and post-contrast delayed phase CT images with the subtraction-derived method. Mean ECV CT was 29.7 ± 5.2%, and a total of 109 patients (12.3%) had ECV CT ≥ 35%. The patients with ECV CT ≥ 35% were significantly older (75.8±8.1 years vs. 74.1±6.9 years; P=0.038) and male dominant 75.4% vs 63.1%; P=0.007). Increased of high-sensitivity cardiac troponin T and B-type natriuretic peptide levels, left ventricular diastolic diameter, left ventricular thickness, impaired left ventricular ejection fraction, and E/e’ were significantly associated with ECV CT ≥ 35%. The prevalence of cardiac amyloidosis was estimated to be at least 1.7% (15/875) in entire study patients and 14.2% (15/106) in patients with ECV CT ≥ 35%. In these cases, increased ECV CT was a clue of the diagnosis of cardiac amyloidosis. Conclusion: CCTA was useful not only for evaluation of coronary arteries but also for evaluation of myocardial properties by ECV CT . Elevated ECV CT was associated with elevated cardiac biomarkers and structural changes of myocardium. There is an incidental diagnosis of cardiac amyloidosis in approximately 1.7% of cases underwent CCTA for the purpose of coronary artery evaluation detected by increased ECV CT .

Prevalence of Cardiac Amyloidosis in Patients Undergoing Carpal Tunnel Release With Amyloid Deposition

Carpal tunnel syndrome (CTS) is considered an early sign of cardiac amyloidosis (CA) because amyloid deposition is often confirmed in the tenosynovium removed during carpal tunnel release (CTR); however, the prevalence of concomitant CA is unclear.Methods and Results: We prospectively examined 700 patients who underwent CTR and evaluated amyloid deposition after tenosynovium removal. Amyloid deposition was observed in 261 (37%) patients, who were significantly older and predominantly male (P<0.05). Of them, 120 agreed to cardiac screening. We performed 99 mTc-labeled pyrophosphate (99 mTc-PYP) scintigraphy in 12 patients who met either of the following criteria: (1) interventricular septal diameter (IVSd) ≥14 mm or (2) 12 mm ≤ IVSd < 14 mm with above-normal limits in high-sensitivity cardiac troponin T (hs-cTnT). Six patients (50%) had positive findings on 99 mTc-PYP scintigraphy and were diagnosed with wild-type transthyretin CA. Concomitant CA was observed in 6/120 (5%) CTR patients with amyloid deposition and 50% (6/12) in patients with left ventricular hypertrophy (≥12 mm) with increased hs-cTnT levels. Amyloid deposition was frequently observed in the removed tenosynovium of elderly men with CTS. Cardiac screening may be useful for early diagnosis of CA in patients undergoing CTR with amyloid deposition.

2023 Expert Consensus of the Taiwan Society of Cardiology on the Diagnosis and Treatment of Cardiac Amyloidosis.

Cardiac amyloidosis is one form of systemic amyloidosis caused by abnormal amyloid fibrils deposited in the extracellular space of the myocardium causing heart failure because of restrictive cardiomyopathy and conduction disturbances. The incidence and prevalence of cardiac amyloidosis are higher than previously noted, particularly among special populations. The most common forms of cardiac amyloidosis are light chain and transthyretin amyloid cardiomyopathy. Even though more than 70% of patients with systemic amyloidosis have cardiac amyloidosis, the diagnosis is often delayed, suggesting significant gaps in the knowledge of cardiac amyloidosis and a lack of multidisciplinary teamwork in our daily practice. The Taiwan Society of Cardiology Heart Failure Committee organized experts to draft the "Expert Consensus on the diagnosis and treatment of cardiac amyloidosis." This statement aims to help clinicians and healthcare professionals improve early diagnosis and management of cardiac amyloidosis in Taiwan. The expert panel met virtually to review the data and discuss the consensus statements. Our review provided practical information about diagnostic methods and algorithms, clinical clues and red-flag signs, cardiac amyloidosis per se and its comorbidities treatment modalities, and follow-up plans for asymptomatic transthyretin gene carriers. We especially innovate two acronyms, "HFpEF MUTED CALL" and "HFmrEF MUST COUNT", to help in the early diagnosis and screening of transthyretin amyloid cardiomyopathy as shown in the Central Illustration.

Prevalence of cardiac amyloidosis among cardiac- and non-cardiac patients undergoing echocardiography at a large university echo lab in Germany: a cohort study

Abstract Background Cardiac amyloidosis (CA) is a progressive infiltrative cardiomyopathy that leads to severe heart failure and, once clinically relevant, poses a high mortality risk. Epidemiological data for CA are still sparse and heterogeneous, but urgently needed since novel therapies against ATTR-CA are now available. Aim We aimed to assess minimum prevalence rates of CA among cardiac and non-cardiac patients undergoing routine echocardiography in an echo lab of a large university clinic serving all medical specialities in central Germany. Methods We established a retro- and prospective database based on suspicious findings in echocardiography for all echocardiographic exams over an 8.5 year period (03/2013 and 09/2021). During this time, 41,375 patients underwent at least one echo exam. Indication for echos were mixed, and consisted of non-cardiac and cardiac patients with acute and chronic illnesses as well as screening exams (e.g. before major surgery or chemotherapy). Suspicious echocardiography were retrospectively analysed whether they underwent further diagnostic or if it remained an unproven suspicion (figure). All echos of suspicious cases were comprehensively re-analysed regarding novel surrogate parameters and indices for CA. Various clinical and laboratory parameters were collected. Results The mean age of the cohort of 41,375 patients was 65.1±15.8 years for the first or only echo examination with a slightly unbalanced gender distribution (45% female). The age distribution is shown in the table. While 128 patients were suspected, the diagnosis was falsified in 16 patients and proven in 47 patients (light chain n=16, ATTR n=31; figure). A substantial proportion of patients with suspicious echo received no further diagnostic work-up (N=65). Over the given time frame of 101 months, the prevalence of suspicious echocardiograms was 1 per 323 patients (or 31 per 10,000). The proven cases had a prevalence of 1 per 880 patients (or 11.3 per 10,000). Since ATTR-CA is a disease of the elderly, we saw a vanishingly small number of cases in persons younger than 70 years (table), whereas 90% of all CA patients were older than 70 years. At timing of diagnosis, AL-CA patients were almost 10 years younger compared to diagnosis of ATTR-CA (69.1±8.4 years vs. 77.4±8.5 years). In patients older than 80 years, the prevalence rate of ATTR-CA peaked with 22.6 cases per 10.000 patients (0.23%). The prevalence rate of ATTR-CA was roughly twice as high compared to AL-CA. Conclusion For the first time, an estimation of minimum prevalence rates for CA for a defined volume of echocardiograms in an all-comers population is provided. In a mixed population of a hospital of maximum care CA prevalence rate is much higher than expected. Awareness for this “rare” disease is needed, since routine echo exams can prompt suspicion in clinically silent or so far unrecognized CA cases. Funding Acknowledgement Type of funding sources: None.

Prevalence of cardiac amyloidosis in Spanish patients with heart failure: The PREVAMIC study design

Prevalence of cardiac amyloidosis in Spanish patients with heart failure: The PREVAMIC study design

Prevalence and Outcomes of Cardiac Amyloidosis in All-Comer Referrals for Bone Scintigraphy.

The prevalence of cardiac amyloidosis (CA) in the general population and associated prognostic implications remain poorly understood. We aimed to identify CA prevalence and outcomes in bone scintigraphy referrals. Methods: Consecutive all-comers undergoing 99mTc-3,3-diphosphono-1,2-propanodicarboxylic-acid (99mTc-DPD) bone scintigraphy between 2010 and 2020 were included. Perugini grade 1 was defined as low-grade uptake and grade 2 or 3 as confirmed CA. All-cause mortality, cardiovascular death, and heart failure hospitalization (HHF) served as endpoints. Results: In total, 17,387 scans from 11,527 subjects (age, 61 ± 16 y; 63.0% women, 73.6% cancer) were analyzed. Prevalence of 99mTc-DPD positivity was 3.3% (n = 376/11,527; grade 1: 1.8%, grade 2 or 3: 1.5%), and was higher among cardiac than noncardiac referrals (18.2% vs. 1.7%). In individuals with more than 1 scan, progression from grade 1 to grade 2 or 3 was observed. Among patients with biopsy-proven CA, the portion of light-chain (AL)-CA was significantly higher in grade 1 than grade 2 or 3 (73.3% vs. 15.4%). After a median of 6 y, clinical event rates were: 29.4% mortality, 2.6% cardiovascular death, and 1.5% HHF, all independently predicted by positive 99mTc-DPD. Overall, adverse outcomes were driven by confirmed CA (vs. grade 0, mortality: adjusted hazard ratio [AHR] 1.46 [95% CI 1.12-1.90]; cardiovascular death: AHR 2.34 [95% CI 1.49-3.68]; HHF: AHR 2.25 [95% CI 1.51-3.37]). One-year mortality was substantially higher in cancer than noncancer patients. Among noncancer patients, also grade 1 had worse outcomes than grade 0 (HHF/death: AHR 1.45 [95% CI 1.01-2.09]), presumably because of longer observation and higher prognostic impact of early infiltration. Conclusion: Positive 99mTc-DPD was identified in a substantial number of consecutive 99mTc-DPD referrals and associated with adverse outcomes.

Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination.

Background: The prevalence of cardiac amyloidosis (CA) is unknown.Aims and Methods: We sought to (a) determine the prevalence of CA in unselected patients ≥75 years undergoing autopsy, (b) characterize cardiological profiles of CA and non-CA patients by providing clinical-histological correlations, and (c) compare their cardiological profiles. After dedicated staining, the localization (interstitial or vascular) and the distribution (non-diffuse or diffuse) of amyloid deposition were analyzed. Cardiological data at last evaluation were retrospectively assessed for the presence of CA red-flags.Results: CA (50% light chains, 50% transthyretin) was found in 43% (n = 24/56) of the autopsied hearts. Atria were involved in 96% of cases. Amyloid localized both at the perivascular and interstitial levels (95.5 and 85%, respectively) with a slightly predominant non-diffuse distribution (58% of cases). Compared to the other patients, CA patients had a more frequent history of heart failure (HF) (79 vs. 47%, p = 0.014), advanced NYHA functional class (III-IV 25 vs. 6%, p = 0.047), atrial fibrillation (68 vs. 36%, p = 0.019), discrepancy between QRS voltage and left ventricular (LV) thickness (70 vs. 12%, p < 0.001), thicker LV walls (15 vs. 11 mm, p < 0.001), enlarged left atrium (49 vs. 42 mm, p = 0.019) and restrictive filling pattern (56 vs. 19%, p = 0.020). The presence of right ventricular amyloidosis seemed to identify hearts with a higher amyloid burden. Among the CA patients, >30% had ≥3 echocardiographic red-flags of disease.Conclusion: CA can be found in 43% of autopsied hearts from patients ≥75 years old, especially in patients with HF, LV hypertrophy and atrial fibrillation.

Abstract 10988: Prevalence and Outcomes of Cardiac Amyloidosis in All-Comer Referrals for Bone Scintigraphy

Introduction: The prevalence of cardiac amyloidosis (CA) in the general population as well as prognostic implications remain poorly understood. Hypothesis: We aimed to identify CA prevalence and outcomes in bone scintigraphy referrals. Methods: Between 2010 and 2020, consecutive all-comers undergoing 99mtechnetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy were included retrospectively. CA was defined as positive cardiac tracer uptake (Perugini grade-1: subclinical CA; grade-2/3: clinical CA). All-cause mortality, cardiovascular (CV) death and hospitalization for heart failure (HHF) served as endpoints. Results: 17387 scans from 11527 subjects (61±16 y/o, 63.0% female, 73.6% cancer) were analyzed. Prevalence of CA was 3.3% (n=376/11527; grade-1: 1.8%, grade-2/3: 1.5%), and was higher among cardiac vs. non-cardiac referrals (18.2% vs. 1.7%). In 4 individuals with &gt;1 scan, progression from subclinical to clinical CA was observed. Cardiac biopsy revealed underlying transthyretin CA in 3 grade-1 patients. After a median of 6 years, clinical event rates were: 29.4% mortality, 2.6% CV death, and 1.5% HHF, all independently predicted by CA. Overall, adverse outcomes were driven by clinical CA (clinical vs. no CA, mortality: adjusted hazard ratio [AHR] 1.46 [95% confidence interval 1.12-1.90]; CV death: AHR 2.34 [1.49-3.68]; HHF: AHR 2.25 [1.51-3.37]). In non-cancer patients, subclinical CA had equally increased mortality to clinical CA (log-rank, p &gt;0.05), presumably due to longer observation/life expectancy. Conclusions: CA was identified in a substantial number of consecutive DPD referrals and associated with adverse outcomes. Subclinical (grade-1) progressed to clinical (grade-2/3) CA at follow-up and both had similarly increased mortality among non-cancer patients. Thus, in grade-1, clarification of the underlying pathology using myocardial biopsy is indicated to enable timely targeted therapy.

Prevalence of cardiac amyloidosis in patients undergoing transcatheter edge-to edge mitral valve repair

Abstract Introduction Cardiac amyloidosis (CA) is associated with severe aortic stenosis, however, its prevalence in patients with severe mitral regurgitation in elderly patients is unknown. Methods Patients scheduled for transcatheter edge-to edge mitral valve repair (TMVR) were prospectively screened for CA using 99m technetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy and subsequent serum as well as urine free light-chain quantification in case of a positive DPD scan, defined as visual cardiac update based on the Perugini grading scale. Results Out of 100 patients undergoing TMVR, 28 patients (28.0%) had a positive DPD-scan (DPD+). 14 patients (14.0%) showed Perugini grade I enhancement, 9 patients (9.0%) grade II enhancement, and in 5 patients (5.0%), grade III enhancement was present. 28 patients suffered from TTR and two from AL- amyloidosis (one patient had a combination of TTR and AL-amyloidosis). When compared to patients with a negative scan (DPD-), DPD+ patients presented with similar baseline characteristics such as age (DPD- vs DPD+ 76y/o vs 77y/o, p=0.44), gender (female; 62.7% vs 50.0%, p=0.25), coronary artery disease (59.7% vs 42.9%, p=0.13), previous valve surgery (25.4% vs 14.3%, p=0.24) and atrial fibrillation (68.7% vs 78.6%, p=0.33). Also, NYHA functional class and EuroScore II were similar (NYHA ≥ III; 85.1% vs 82.1%, p=0.72, and EuroScore II 9.9±9.8% vs 7.0±4.8%, p=0.21, respectively). On echocardiography, DPD+ patients presented with more pronounced left and right ventricular hypertrophy (interventricular septum: 15mm vs 13mm, p&amp;lt;0.01) but similar left ventricular ejection fraction (44.9% vs 42.3%, p=0.34). At 3-months after TMVR, DPD+ patients showed significant improvement in BNP serum levels when compared to DPD- patients (DPD+ vs DPD-: +315±2569pg/ml vs −2404±8696pg/ml, p=0.03), while NYHA functional class remained unchanged (NYHA improvement ≥1 class: 57.6% vs 50.0%, p=0.52) Conclusions In this single centre experience, CA was highly prevalent among elderly patients with severe mitral regurgitation scheduled for TMVR. TMVR in CA patients resulted in significant improvement of NT-pro BNP levels. Future studies need to clarify the prognostic relevance of CA in this specific patient population. Funding Acknowledgement Type of funding sources: None.

The Incidence and Prevalence of Cardiac Amyloidosis in a Large Community-Based Cohort in Alberta, Canada

BackgroundDespite 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 ResultsIn 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 < .0001). Patients with probable cardiac amyloidosis were more likely to be male, have a higher comorbidity burden, greater health care use, and poorer outcomes as compared with patients with HF without amyloidosis. A much larger group of patients was identified as possible cardiac amyloidosis (n = 46,255), with similar increase in prevalence from 2004 to 2018 (from 416 to 850 per 100,000 person-years). ConclusionsThe 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.

Pathophysiology and Therapeutic Approaches to Cardiac Amyloidosis.

Often considered a rare disease, cardiac amyloidosis is increasingly recognized by practicing clinicians. The increased rate of diagnosis is in part due the aging of the population and increasing incidence and prevalence of cardiac amyloidosis with advancing age, as well as the advent of noninvasive methods using nuclear scintigraphy to diagnose transthyretin cardiac amyloidosis due to either variant or wild type transthyretin without a biopsy. Perhaps the most important driver of the increased awareness is the elucidation of the biologic mechanisms underlying the pathogenesis of cardiac amyloidosis which have led to the development of several effective therapies with differing mechanisms of actions. In this review, the mechanisms underlying the pathogenesis of cardiac amyloidosis due to light chain (AL) or transthyretin (ATTR) amyloidosis are delineated as well as the rapidly evolving therapeutic landscape that has emerged from a better pathophysiologic understanding of disease development.

Abstract 16240: Knowledge of Clinical Presentation and Diagnostic Pathways for Cardiac Amyloidosis Among Physicians in the Middle East-Gulf Region: on Behalf of the Gulf Cardiac Amyloidosis Working Group

Background: There is a growing interest in raising awareness about amyloidosis as an under-recognized cause of heart failure and preserved ejection fraction (HFpEF). Recently, the prevalence of cardiac amyloidosis in the United States has increased, which may partly be attributed to initiatives from major professional societies aimed to improve patient identification and disease detection. Whether this has also impacted physicians’ knowledge about cardiac amyloidosis in the Middle East-Gulf region is unknown but critical to assess, as it would identify a need for dedicated regional educational activities. Methods: Physicians practicing in 5 Gulf countries (UAE, Bahrain, Qatar, Oman and Kuwait) were invited to participate in this anonymous, online survey by receiving a unique survey link by email. We assessed awareness of cardiac amyloidosis, knowledge of disease manifestations and approach to diagnosis. Responses to the survey were recorded using a 4- or 5-point Likert scale. Results: A total of 272 physicians participated in the survey. Most participating physicians were men (82%) and have been practicing cardiology (71%) for &gt;10 years (65%). Whereas 83% of responders considered themselves to be somewhat or extremely familiar with signs and symptoms of cardiac amyloidosis, only 63% would consider cardiac amyloidosis as a cause of HFpEF, 59% would consider it in patients with heart failure and orthostatic hypotension while only 39% consider cardiac amyloidosis in patients with low-flow, low-gradient severe aortic stenosis. Furthermore, cardiac MRI was found to be useful for diagnosis of cardiac amyloidosis by 92% of responders, while echocardiography, cardiac scintigraphy with bone-seeking radiotracers and biomarkers were felt to be useful by only 81%, 60% and 31% of survey participants, respectively. Conclusion: Despite perceived familiarity with cardiac amyloidosis among a group of mid-career cardiologists, there is need to raise awareness about the heterogenous manifestations of the disease and about the respective roles of testing modalities in making this diagnosis.

Estimated population prevalence of cardiac transthyretin amyloidosis in elderly men derived from incidental cardiac uptake from routine bone scans – “we're going to need a bigger boat”

Abstract Background/Introduction Transthyretin amyloidosis (TTR) is a cause of restrictive cardiomyopathy and heart failure predominantly in elderly men. Two main factors have moved TTR amyloidosis from super-specialist centres into mainstream cardiology: We aimed to determine the potential magnitude of referrals to our embryonic cardiac TTR service from patients having routine bone scans for non-cardiac reasons. We planned to estimate the prevalence of cardiac TTR in our local over 65 male population to plan service provision. Methods All HDP bone scans performed at a teaching and research hospital in the UK from the 2017/18 financial year were reviewed (n=1530). Our hospital is the only provider of these scans locally. Of these, 1399 were for oncological and musculoskeletal (oncology/MSK) indications and 37 were referred to specifically “exclude amyloidosis”. We excluded paediatric and duplicate follow-up imaging. There are approximately 140,000 people over aged 65 living within our catchment region. We have assumed approximately 50% are male. Results Myocardial uptake was present in 7/1399 of the oncology/MSK group and 3/7 (43%) of these already had features of heart failure. In these 7 patients bone scans were performed to investigate bony metastases in 6 (1 oesophageal cancer and 5 for prostate cancer) and 1 following an orthopaedic procedure. Cardiac uptake was present in 10/37 of the “exclude amyloid” group. In those with cardiac uptake across both groups (17) 94% were male with a mean age of 83 (sd ±6.59) and 41% were from the oncology/musculoskeletal group. Incidental cardiac uptake was seen in 1:200 routine HDP scans. When looking at males &amp;gt;65 specifically the uptake rate increases to (6 out of 701 scans) i.e. 1:117. Assuming there is no increased risk of TTR in patients with prostate or oesophageal cancer, then an estimate of cardiac TTR in the 75,000 males over the age of 65 locally would be approximately 640 men. Conclusions Bone scans account for 41% of all HDP scans with incidental cardiac uptake and therefore represents a significant potential referral source for a cardiac amyloid service. Our data suggests a potential prevalence of cardiac amyloidosis in 1:117 men over 65 with 43% already having heart failure symptoms and signs. Our age and sex specific prevalence suggests cardiac TTR is neither a rare nor unusual diagnosis. We will use our prevalence estimate to ensure our cardiac TTR service is resourced appropriately. We suggest that cardiac amyloid and cardio-oncology services should include pathways incorporating rapid access routes for suitable patients with incidental cardiac uptake on bone scans performed by non-cardiologists. Funding Acknowledgement Type of funding source: None

Prognostic impact of suspected cardiac amyloidosis in aortic stenosis patients referred for transcatheter aortic valve implantation

Abstract Background The prevalence of cardiac amyloidosis (CA) and aortic stenosis (AS) both increase with age. Transcatheter aortic valve implantation (TAVI) expands the number of patients (P) eligible for treatment of AS, emphasizing the need to understand the prevalence of CA in AS and its prognostic associations. Echocardiography with speckle tracking has emerged as a useful method to enhance the clinical suspicion and to provide prognostic information. Purpose To estimate the prevalence of CA in P with severe AS referred for TAVI and to evaluate the impact of concomitant CA in prognosis. Methods 94 consecutive AS P who underwent TAVI with maximum left ventricular wall thickness (LVWT)&amp;gt;12 mm were retrospectively identified. Clinical data, pre TAVI echocardiographic parameters and follow up (FU) data regarding all-cause mortality and MACE (including all-cause mortality, admission for heart failure, pacemaker implantation and stroke) were analysed. We registered apical sparing pattern in bull's eye plots (ASPB), calculated relative apical longitudinal strain formula (RALS) [average apical LS/(average basal LS + mid-LS)] and ejection fraction/global longitudinal strain (EF/GLS) ratio. Results Mean age was 82.2±5.8 years (Y), with 43 men (45.7%). 27.7% were in NYHA functional class II, 64.9% in functional class III and 7.4% in functional class IV. Median EF was 57±15% and 26.6% presented EF&amp;lt;50%. Suspected CA evaluated by ASPB was found in 39 P (41.5%) and RALS &amp;gt;1 was identified in 22 P (23.4%). An EF/GLS ratio &amp;gt;4.1 was obtained in 53 P (56.4%). Over a median follow-up of 13.4±25.8 months, 28 deaths (29.8%) and 31 MACEs (33.0%) occurred. The presence of ASPB was associated with increased all-cause mortality (33.3% vs. 5.6%, p=0.002) and MACE (48.7% vs 22.2%, p=0.01). RALS&amp;gt;1 correlated also with all-cause mortality (31.8% vs. 12.5%, p=0.04) and with new bundle branch block and indication for pacemaker implantation (46.2% vs 37.0%, p=0.05). P with GLS&amp;gt;−14.8% and ASPB had significantly worse prognosis regarding all-cause mortality (p=0.003) and MACE (p=0.007). Kaplan–Meier survival analysis showed that survival was significantly worse for P with ASPB (log-rank 0.002). With multivariate Cox regression analysis, ASPB was independently associated with all-cause mortality (HR=4.49, p=0.039). Conclusions Suspected CA appears prevalent among patients with AS and associates with all-cause mortality. The importance of screening for CA in older AS patients and optimal treatment strategies in those with CA warrant further investigation, especially in the era of transcatheter aortic valve implantation. Funding Acknowledgement Type of funding source: None