Types Of Cardiac Amyloidosis Research Articles

Histopathological and Immunohistochemical Characteristics of Different Types of Cardiac Amyloidosis.

Cardiac involvement is the most important factor determining prognosis in patients with systemic amyloidosis. This retrospective observational study of 98 patients with amyloidosis was undertaken to assess the amyloid types that are most likely to affect the heart, describe histopathological and clinical features of cardiac amyloidosis, and estimate the number of cases not diagnosed clinically prior to death. All cases were divided into two groups based on the method of examination. The first group included 46 patients with cardiac amyloidosis revealed via endomyocardial biopsies (EMBs), and the second group included 52 amyloidosis patients who did not undergo EMBs, in whom cardiac involvement was identified only at autopsy. The EMBs demonstrated that AL amyloidosis was detected in 21 (46%) specimens, ATTR amyloid in 24 cases (52%), and AA amyloid in 1 case (2%). The autopsy reports defined 15 (46%) cases of AL amyloidosis, 21 (40%) of ATTR and 16 (31%) of AA amyloidosis. It should be noted that a clinical diagnosis of ATTR amyloidosis was made only in 9.5% of patients from the autopsy group, suggesting that ATTR may be an underdiagnosed cause of heart failure in elderly patients. The most intense amyloid deposits were determined in biopsy and autopsy specimens of patients with AL kappa amyloidosis, underlying a poorer prognosis.

Prognostic value of soluble ST2 in AL and TTR cardiac amyloidosis: a multicenter study.

Both light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis are types of cardiac amyloidosis (CA) that require accurate prognostic stratification to plan therapeutic strategies and follow-ups. Cardiac biomarkers, e.g., N-terminal pro-B-type natriuretic peptide (NT-proBNP) and high-sensitivity cardiac troponin T (Hs-cTnT), remain the cornerstone of the prognostic assessment. An increased level of soluble suppression of tumorigenesis-2 (sST2) is predictive of adverse events [all-cause death and heart failure (HF) hospitalizations] in patients with HF. This study aimed to evaluate the prognostic value of circulating sST2 levels in AL-CA and ATTR-CA. We carried out a multicenter study including 133 patients with AL-CA and 152 patients with ATTR-CA. During an elective outpatient visit for the diagnosis of CA, Mayo Clinic staging [NT-proBNP, Hs-cTnT, differential of free light chains (DFLCs)] and sST2 were assessed for all AL patients. Gillmore staging [including estimated glomerular filtration rate (eGFR), NT-proBNP] and Grogan staging (including NT-proBNP and Hs-cTnT) were assessed for TTR-CA patients. The median age was 73 years [interquartile range (IQR) 61-81], and 53% were men. The endpoint was the composite of all-cause death or first HF-related hospitalization. The median follow-up was 20 months (IQR 3-34) in AL amyloidosis and 33 months (6-45) in TTR amyloidosis. The primary outcome occurred in 70 (53%) and 99 (65%) of AL and TTR patients, respectively. sST2 levels were higher in patients with AL-CA than in patients with ATTR-CA: 39 ng/L (26-80) vs. 32 ng/L (21-46), p < 0.001. In AL-CA, sST2 levels predicted the outcome regardless of the Mayo Clinic score (HR: 2.16, 95% CI: 1.17-3.99, p < 0.001). In TTR-CA, sST2 was not predictive of the outcome in multivariate models, including Gillmore staging and Grogan staging (HR: 1.17, CI: 95% 0.77-1.89, p = 0.55). sST2 level is a relevant predictor of death and HF hospitalization in AL cardiac amyloidosis and adds prognostic stratification on top of NT-proBNP, Hs cTnT, and DFLC.

Heart amyloidosis

The article highlights the latest scientific data on the clinic, diagnosis and modern treatment of cardiac amyloidosis. Particular attention is paid to the peculiarities of the clinical course and differences in the diagnosis and treatment of various types of cardiac amyloidosis, primarily AL-amyloidosis and transthyretin amyloidosis. The most up-to-date diagnostic tests that are used to detect the disease and the new treatments that can be used for different types of amyloidosis are highlighted.

Real world data on light chain cardiac amyloidosis: Still a delayed diagnosis.

Cardiac amyloidosis (CA) represents a myocardial disorder developed by fibril deposition of a heterogeneous group of misfolding proteins. Despite being rare, a high clinical index of suspicion and novel advanced diagnostic methods seem to facilitate its early recognition. Currently nine types of cardiac amyloidosis have been described with AL and ATTR being the most common. Light chain amyloidosis (AL) is a life-threatening disease, resulting from clonal plasma cells that produce amyloidogenic light chain fragments causing organ damage including the heart. Morbidity and mortality of these patients is strongly associated with the severity of cardiac involvement. Thus, early and precise diagnosis is crucial for prompt treatment initiation. In this study, we retrospectively analyzed data of 36 consecutive patients who were diagnosed with AL amyloidosis and treated in our center over the past 15 years. Heart involvement was present in 33 (92%) of them while 76% had severe cardiac disease as of stage IIIa and IIIb, according to the Mayo2004/European staging system. Almost one third of these patients experienced an early death occurring the first five months of diagnosis. To capture everyday clinical practice, we provide details on clinical presentation, diagnostic challenges, and outcome of these patients.

Utility and pitfalls of the electrocardiogram in the evaluation of cardiac amyloidosis

BackgroundCardiac amyloidosis is a protein misfolding disorder involving deposition of amyloid fibril proteins in the heart. The associated fibrosis of the conduction tissue results in conduction abnormalities and arrhythmias. “Classical” electrocardiogram (ECG) findings in cardiac amyloidosis include that of low voltage complexes with increased left ventricular wall thickness on echocardiography. However, this “classical” finding is neither sensitive nor specific. As cardiac amyloidosis is associated with a generally poor prognosis, the need for early recognition of this disease is important given the availability of new treatment options. In this review, we highlight 3 cases of patients with cardiac amyloidosis. Although presenting with typical clinical signs and symptoms, ECG for all 3 patients was not consistent with the classical findings described. They underwent further diagnostic tests which clinched the diagnosis of cardiac amyloidosis, allowing patients to receive targeted treatment. Through the review of the literature, we will highlight the different ECG patterns in patients with different types of cardiac amyloidosis and clinical scenarios, as well as the pitfalls of using ECG to identify the condition. Lastly, we also emphasize the current paradigms in diagnosing cardiac amyloidosis through the non‐invasive methods of echocardiography, cardiac magnetic resonance imaging, and nuclear technetium‐pyrophosphate imaging.ConclusionsElectrocardiogram is often the first investigation used in evaluating many cardiac disorders, including cardiac amyloidosis. However, classical features of cardiac amyloidosis on ECG are often not present. A keen understanding on the ECG features of cardiac amyloidosis and knowledge of the diagnostic workflow is important to diagnose this condition.

Sex-related differences in clinical presentation and all-cause mortality in patients with cardiac transthyretin amyloidosis and light chain amyloidosis

We aimed to ascertain whether sex-related differences are relevant to clinical presentation, cardiac phenotype and all-cause mortality in different types of cardiac amyloidosis, a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated.Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL).Women with wtATTR, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men, otherwise, bio-humoral parameters, NYHA class, and ECG characteristics were similar. Comparing females and male with wtATTR, hATTR and AL, no differences in Kaplan-Meier curves for all-cause mortality were observed with regard to sex, p-value >0.05.In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, all-cause mortality was not affected. Nevertheless, women with wtATTR had echocardiographic signs of more advanced disease and higher NAC score at diagnosis suggesting a possible later recognition of disease compared to men.

110 Sex-related differences in clinical presentation and outcome of patients with cardiac transthyretin amyloidosis and light chain amyloidosis

Abstract Aims We aimed to ascertain whether sex-related differences are relevant to the pathophysiology, presentation, and outcomes in different types of cardiac amyloidosis—a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated. Methods and results Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL). Wt-ATTR women, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men. Otherwise, bio-humoural parameters, NYHA class, and ECG characteristics were similar. No differences in outcome were observed with regard to sex. Conclusions In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, outcome was not affected. Nevertheless, women with wtATTR showed a worse profile at diagnosis and evidence suggesting a later recognition of disease compared to men, highlighting the need for a higher index of suspicion in female patients.

The utility of positron emission tomography in cardiac amyloidosis.

Cardiac amyloidosis, characterized by progressive restrictive cardiomyopathy, presents unusual diagnostic challenges. Conventional cardiac scintigraphy has shown limited utility in the quantification of disease burden and serial follow-up of cardiac amyloidosis. The advent of specialized positron emission tomography with specific amyloid-binding radiotracers has the potential to change currently employed diagnostic algorithms for the imaging of cardiac amyloidosis. This review aims to discuss the diagnostic utility of amyloid-binding radiotracers, including Pittsburg compound B, florbetapir, florbetapan, and sodium fluoride. These tracers have promising potential for the early detection of the particular type of cardiac amyloidosis, pursuing relevant medical intervention, assessing amyloid burden, monitoring treatment response, and overall prognostication.

Transthyretin cardiac amyloidosis: a review of the nuclear imaging findings with emphasis on the radiotracers mechanisms.

Cardiac amyloidosis is a protein deposition disease characterized by the infiltration of the myocardium and coronary arteries resulting in a progressive thickening of both ventricles, interatrial septum and atrioventricular valves, eventually leading to organ failure. It is a disease hard to diagnose, due to the lack of diagnostic investigations. However, development of new and more accurate examinations is undergoing. Endomyocardial biopsy is the gold standard investigation for this disease, but it has its limitations (invasive and not widely available). Other investigations may be able to detect the presence of cardiac amyloidosis but cannot specify the type involved. To that end, nuclear medicine through bone scanning offers a simple, non-invasive solution to detect, differentiate and diagnose transthyretin cardiac amyloidosis (ATTR) from other types of cardiac amyloidosis. In order to demonstrate the importance of bone scanning we will present a few methods of image processing based on literature and a personalized method, followed by a few important examples of positive cases. The aim of this review was to present the current methods of ATTR detection with emphasis on nuclear medicine bone scanning and its important place in the decision algorithm of the cardiologist for a personalized approach to this pathology.

Cardiac manifestations in Finnish gelsolin amyloidosis patients

Introduction Finnish gelsolin amyloidosis (AGel amyloidosis) is an inherited systemic amyloidosis with well-known ophthalmological, neurological and cutaneous symptoms. Additionally, cardiomyopathies, conduction disorders and need of cardiac pacemakers occur in some patients. This study focuses on electrocardiographic (ECG) findings in AGel amyloidosis and their relation to cardiac magnetic resonance (CMR) changes. We also assessed whether ECG abnormalities were associated with pacemaker implantation and mortality. Materials and methods In this cohort study, 51 genetically verified AGel amyloidosis patients (mean age 66 years) without cardiac pacemakers underwent 12-lead ECG and CMR imaging with contrast agent in 2017. Patients were followed-up for 3 years. Results Conduction disturbances were found in 22 patients (43%). Nine (18%) presented with first-degree atrioventricular block, six (12%) with left anterior hemiblock, seven (14%) with left or right bundle branch block and two (4%) with non-specific intraventricular conduction delay. Low QRS voltage was present in two (4%) patients. Late gadolinium enhancement (LGE) concentrating on the interventricular septum and inferior parts of the heart was present in 19 (86%) patients with conduction abnormalities. During the follow-up, only one patient received a pacemaker, and one patient died. Discussion Conduction disorders and septal LGE are common in AGel amyloidosis, whereas other ECG and CMR findings typically observed in most common cardiac amyloidosis types were rare. Septal pathology seen in CMR may interfere with the cardiac conduction system in AGel amyloidosis, explaining conduction disorders, although pacemaker therapy is rarely required.

Diagnosis and treatment of cardiac amyloidosis: an interdisciplinary consensus statement.

SummaryThe prevalence and significance of cardiac amyloidosis have been considerably underestimated in the past; however, the number of patients diagnosed with cardiac amyloidosis has increased significantly recently due to growing awareness of the disease, improved diagnostic capabilities and demographic trends. Specific therapies that improve patient prognosis have become available for certain types of cardiac amyloidosis. Thus, the earliest possible referral of patients with suspicion of cardiac amyloidosis to an experienced center is crucial to ensure rapid diagnosis, early initiation of treatment, and structured patient care. This requires intensive collaboration across several disciplines, and between resident physicians and specialized centers. The aim of this consensus statement is to provide guidance for the rapid and efficient diagnosis and treatment of light-chain amyloidosis and transthyretin amyloidosis, which are the most common forms of cardiac amyloidosis.

Comparison of lambda and kappa light-chain cardiac amyloidosis in Polish patients diagnosed in cardiology department

Abstract Background Lambda (λ) and kappa (κ) types of light-chain amyloidosis (AL amyloidosis) are believed to have a similar prognosis. Data on the comparison of these two types of cardiac amyloidosis is scanty. Objectives The aim of the study was to investigate wether lambda light-chain cardiac amyloidosis indicates worse prognosis than the kappa variety. Methods The initial analysis covered all consecutive pts with cardiac AL amyloidosis diagnosed in the cardiology department from August 2011 to August 2019. Diagnosis was confirmed by increased serum free light-chains and positive tissue biopsy. Amyloid type was identified using immunohistochemical reactions. Blood pressure and heart rate (HR) were measured on admission. The difference between involved and uninvolved serum free light chains (dFLC), NT-proBNP, high-sensitivity troponin T (hs-TnT), creatinine, potassium, albumin and total protein were measured. During echocardiography, tissue Doppler imaging was used to assess early lateral (e' lat) and septal mitral annulus velocities and longitudinal myocardial velocities of ventricles. Standard parameters were measured. The presence of pleural effusion was assessed in chest X-ray. Results Sixty-four pts were diagnosed with AL amyloidosis. Four pts were excluded from the final analysis due to ambiguous amyloid typing. Median (interquartile range, IQR) age was 61 (52–67) yrs. Median (IQR) dFLC was 19.9 (5.5–50.6) mg/dL. Median NT-proBNP and hs-TnT concentrations were 4948 (2251–10206) pg/ml and 77 (39–139) ng/l, respectively. Forty-four pts had a λ type AL amyloidosis (73.3%). There were significant differences (p&amp;lt;0.05) between the λ and the κ groups in regard to: HR (80 vs. 73.5 BPM), systolic blood pressure (102 vs. 117 mmHg), serum creatinine (88 vs. 116 umol/L) and potassium (4.4 vs. 4.9 mmol/L), e' lat (5 vs. 7.5 cm/sec), left ventricular end-diastolic diameter (LVEDD, 42 vs. 46 mm), right ventricular end-diastolic diameter (RVEDD, 35 vs. 40 mm) and right ventricular wall thickness (8 vs. 6 mm). Pleural and pericardial effusions were more frequent in the λ group (59% vs. 19% and 80% vs. 44%, respectively) according to Fisher's exact test (p&amp;lt;0.05). Median (IQR) survivals for the λ and the κ groups were 3 (2–9) and 16 (5.5–22) months, respectively (p=0.03). The Kaplan-Meier curves analysis showed a trend towards worse survival of the λ group (Log rank test, p=0.08). Conclusions Cardiologists should be aware that lambda light-chain cardiac amyloidosis may indicate shorter survival than the kappa variety, although kappa AL amyloidosis may be associated with worse kidney function. Further research would be worth considering. Kaplan-Meier curves, Time (months) Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Institute of Cardiology

Amyloid cardiomyopathy in the elderly: clinical options, difficulties in diagnosis and treatment

This article analyzes the frequency and features of cardiac amyloidosis in the elderly. The morphology and pathogenesis of amyloid cardiomyopathy are presented. The features of the clinical picture of the heart and aortic lesions in systemic and local variants of amyloidosis are analyzed. Polymorbidity in older people, which complicates the diagnosis of cardiac amyloidosis and leads to a delay in treatment, is reviewed. The diagnostic methods of various types of cardiac amyloidosis are evaluated. Modern trends in the treatment of amyloidosis are presented.

Role of tafamidis in reducing the effect and progression of transthyretin amyloid cardiomyopathy.

e15612 Background: Transthyretin Amyloid Cardiomyopathy (ATTR-CM) is a rare, life-threatening, progressive and underdiagnosed cause of restrictive cardiomyopathy and heart failure. ATTR-CM is a type of cardiac amyloidosis caused due to misfolded and destabilized transthyretin amyloid protein deposits in the myocardium. Tafamidis is a transthyretin (TTR) modifying targeted drug that stabilizes and prevents tetramer dissociation and amyloid deposits. Our aim is to conduct a review of literature to determine the effect of tafamidis on the structural progression of transthyretin cardiomyopathy while also looking into all-cause mortality outcomes. Methods: A systematic search was performed in PubMed, Embase, and Cochrane using the MESH terms "Tafamidis" and “Transthyretin Amyloid cardiomyopathy.” Two authors independently reviewed article titles and abstracts. Disagreements were resolved by consensus and then adjudicated by a third author. All articles published in English up to the year 2019 were eligible for inclusion. Data was tabulated and analyzed. Studies measuring mortality outcomes and echocardiogram findings with the use tafamidis in ATTR-CM were included. Studies that did not measure mortality outcomes were excluded as well as case reports and animal studies. Results: Out of the studies identified through databases (n = 57), only 6 studies met the inclusion criteria as earlier studies delved strictly into the tolerability and safety of the drug. The studies that were included in this analysis are heterogeneous, because ATTR-CM is phenotypically heterogeneous (wild type-ATTR-CM or mutant ATTR-CM) and has varying degrees of amyloid deposits and cardiac symptoms. The role of tafamidis in reducing the effect of cardiomyopathy in transthyretin amyloid cardiomyopathy is unclear. Effects on the structural changes of the myocardium including the ejection fraction remain inconsistent. A recent multicenter, international, phase 3 randomized controlled trial shows reduction in all-cause mortality comparing tafamidis vs placebo. (29.5% vs 42.9%). Conclusions: With the recent approval of tafamidis, it is vital to review and analyze the available data to study its effects on transthyretin amyloid cardiomyopathy. While structural improvements with tafamidis therapy are inconsistent, reduction in all-cause mortality with this novel drug is promising.

An Analysis of Our Ten-Year Cohort of Patients with Cardiac Amyloidosis Supported by the Continuous-Flow Left Ventricular Assist Device

Limited data exists on the use continuous-flow left ventricular assist device (CF-LVAD) in patients with cardiac amyloidosis (CA). The largest single center analysis on CF-LVAD support in patients with CA reports a 50% mortality at 6-months. Our primary aim was to examine the survival and morbidity of our patients with diagnosed CA and supported by CF-LVAD. Data on demographics, type of cardiac amyloidosis, pre-implant echocardiographic features, and post-implant survival and morbidity outcomes was retrospectively extracted on patients with CA and supported by CF-LVAD at our institution from January 2009-October 2019. Descriptive statistics are reported. Six patients with diagnosed CA (mean age 64 ± 9 years) were supported by CF-LVAD (3 centrifugal, 3 axial) for a median 32.3 months (range 8.4, 48.3). Table 1 depicts pertinent patient demographics, survival and morbidity. Four patients had ATTR amyloid, and 2 had AL amyloidosis. LV ejection fraction (10-45%) and end-diastolic dimension (4.4-6.0 cm) varied pre-implant. All 6 patients were alive at 6-months on CF-LVAD support. Overall, 2 patients died, 2 were bridged to heart transplant, and 2 remain on destination therapy. One patient required a right ventricular assist device and dialysis immediately post-implant, and two patients had hospital re-admission for right heart failure. Various etiologies contributed to re-hospitalizations post-implant (data not shown). With CF-LVAD support, the patients with AL amyloid survived for a median 47.8 months. Our limited experience in patients with cardiac amyloidosis highlights the feasibility of supporting these patients with CF-LVAD as bridge to transplant or destination therapy. Future analyses are warranted to refine careful patient selection to improve outcomes, with particular consideration to the interplay between right ventricular hemodynamics and renal function.

Molecular Imaging of Cardiac Amyloidosis.

The aim of this review is to give an update on the molecular imaging tools currently available as well as to discuss the potential roles and limitations of molecular imaging in cardiac amyloidosis. Molecular imaging plays a central role in the evaluation of patients with suspected cardiac amyloidosis. It can be used to diagnose and distinguish between the different types of cardiac amyloidosis. The diagnostic properties of bone scintigraphy are such that it allows reliable diagnosis of transthyretin cardiac amyloidosis without the need of endomyocardial biopsy in a significant proportion of patients. Furthermore, molecular tracers assessing amyloid plaque burden and sympathetic innervation may be useful for the non-invasive evaluation diagnosis and risk stratification of patients with suspected cardiac amyloidosis. Cardiac amyloidosis is an under-recognized cause of left ventricular hypertrophy and heart failure in the elderly. The role of molecular imaging in cardiac amyloidosis is expected to grow considering the arrival of new therapies and molecular imaging probes.

Incidence of cardiovascular events in patients implanted with pacemakers or cardiac defibrillators in cardiac amyloidosis

Cardiac amyloidosis is a fatal disease. It may represent 10% of non-ischemic heart diseases. Conduction and rhythm disorders are common causes of death. There are no real guidelines about PM or ICD indications in cardiac amyloidosis. Cardiac devices (PM/ICD) prolong survival by decreasing MACE (cardiovascular death; sudden cardiac death; delivered therapies by ICD; cardiac decompensation; heart transplant) by treating rhythmic and conductive events and improving cardiac hemodynamics. The aim was to demonstrate the benefit in terms of cardiovascular mortality or morbidity of implantation of cardiac devices (PM/DAI) in patients with different types of cardiac amyloidosis. A total of 1053 patients were screened for cardiac amyloidosis between 10/2010 and 12/2016 within Réseau Amylose Mondo r (CHU Henri-Mondor–Créteil). A total of 186 AL amyloidosis (AL), 225 hereditary amyloidosis (hTTR) and 168 senile amyloidosis (wtTTR) were included in our study and followed from inclusion in the database until their death or until 12/2016. Overall a total of 70/186 PM/ICD were implanted in AL amyloidosis (38%), 99/225 in hTTR (44%) and 96/168 in wtTTR (55%). Patients implanted with a PM/ICD had a 24-month risk of developing MACE almost twice as large as non-implanted patients, for any type of amyloidosis (HR 1.73 [1.28; 2.34]; P < 0,001). A propensity score was created to identify factors associated with prosthesis implantation. In multivariate analysis, incorporating the propensity score, the best statistical model for identifying the risk factors for MACE occurrence showed that the presence of a PM/ICD was not associated with the occurrence of MACE at 24 months (C-stat 0.75) ( Fig. 1 ). This study demonstrates that PM/ICD are not associated with an overdose of major cardiovascular complications, as suggested in some studies. Our data suggest that these prostheses have a place in this disease, provided to find the good criteria of selections of the patients to implant.

P24-6 - Reduced Right Ventricular Peak Circumferential Strain Measured by MRI Predicts Wild-Type Transthyretin Amyloidosis in Patients With Ventricular Hypertrophy

Background: Wild-type transthyretin amyloidosis (ATTRwt) is often overlooked in elderly patients with left ventricular hypertrophy (LVH). Cardiac magnetic resonance (CMR)-tagging is established technique to evaluate LV local intra-myocardial motion, however usefulness to assess RV function by CMR is unknown. We assessed the hypothesis that the peak RV strain by CMR can distinguish ATTRwt. Methods: We analyzed 11 ATTRwt and 7 non-ATTRwt consecutive patients. We excluded patients younger than 50 years old, less than left ventricular ejectionfraction (LVEF) 50%, without LVH, old myocardial infarction, and with other types ofcardiac amyloidosis. To evaluate circumferential strain (CS), CMR-tagging images were analyzed on a post-processing workstation. The peak CS (%) and peak CS time (msec) of each segment were recorded. Results: LVEF was significantly lower in ATTRwt than non-ATTRwt (59.1 ± 4.0 vs66.2 ± 5.0, P = .004). The average of RV peak CS was significantly lower in ATTRwt than non-ATTRwt (−8.46 ± 2.32 vs −11.7 ± 2.86, P = .017). Univariate logistic regression analysis identified LVEF (odds ratio [OR]: 0.67, 95% confidence interval [95%CI]: 0.47–0.97, P = .49) and average of RV peak CS (OR: 1.74, 95%CI: 1.00–3.02, P = .49) were correlated with ATTRwt. In receiver operating characteristic analysis, the area under the curve for average of RV peak CS in discrimination between ATTRwt and non-ATTRwt were 0.83 (P = .02). Conclusion: Reduced RV peak CS predicts the presence of ATTRwt in elderly patients with LVH.

MISDIAGNOSIS OF CARDIAC AMYLOIDOSIS TYPE BY STANDARD IMMUNOHISTOCHEMISTRY

Background: Light chain (AL) & transthyretin (TTR) are the most common types of cardiac amyloidosis. AL is caused by a monoclonal gammopathy, but monoclonal gammopathy of unknown significance (MGUS) can also be seen in TTR amyloidosis & may confuse the clinical picture. It is essential to correctly

Reduced trans-mitral A-wave velocity predicts the presence of wild-type transthyretin amyloidosis in elderly patients with left ventricular hypertrophy.

Wild-type transthyretin amyloidosis (ATTRwt) is often overlooked in elderly patients with left ventricular hypertrophy (LVH). Impaired atrial function, in addition to ventricular diastolic dysfunction, is one of the hallmarks of cardiac amyloidosis. Here, we assessed the hypothesis that atrial function evaluated by A-velocity in pulse Doppler echocardiography is useful to differentiate ATTRwt in elderly patients with LVH. We analyzed 133 consecutive patients who underwent tissue biopsy to rule out infiltrative cardiomyopathy in our institute. We excluded patients younger than 50years, without LVH (LV thickness was less than 12mm), with other types of cardiac amyloidosis and patients with chronic atrial fibrillation, and analyzed remaining 51 patients (ATTRwt: 16, non-ATTRwt: 35). ATTRwt patients were significantly older and had advanced heart failure compared with non-ATTRwt group. In echocardiography, E/A, E/e', and relative wall thickness was significantly higher in ATTRwt group than non-ATTRwt group. A-velocity was significantly decreased in ATTRWT group compared with non-ATTRwt group (40.8±20.8 vs. 78.7±28.2cm/s, p=0.0001). Multivariate logistic analysis using eight forced inclusion models identified trans-mitral Doppler A-wave velocity was more significant factor of cardiac amyloidosis in ATTRwt. In receiver operating characteristic (ROC) analysis, the area under the curve (AUC) for A-wave velocity in discrimination between ATTRwt and non-ATTRwt were 0.86 (CI 0.76-0.96, p<0.001). The cut-off value was 62.5cm/s, and it yielded the best combination of sensitivity (69.7%) and specificity (87.5%) for prediction of amyloidosis. We concluded that reduced A-velocity predicts the presence of ATTRwt in elderly patients with LVH in sinus rhythm.