scholarly journals 515 Exertional syncope and ventricular arrhythmia in a patient with cardiac amyloidosis

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Giuseppe Fede ◽  
Giuseppe Abate ◽  
Paolo Belluardo ◽  
Nicoletta Guccione ◽  
Giovanni Tasca ◽  
...  
Keyword(s):  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Troponin T ◽  
Global Longitudinal Strain ◽  
Lateral Wall ◽  
Wall Thickening ◽  
Multisystem Disorder ◽  
Reduced Ejection Fraction ◽  
Right Ventricle Free Wall ◽  
Poor Tolerance

Abstract Aims Systemic amyloidosis is a multisystem disorder caused by the misfolding, aggregation, and deposition of certain proteins in various organs and tissues. Cardiac involvement is common and worsens the prognosis. Atrial fibrillation is the most frequent arrhythmia in cardiac amiloidosis (CA), but many cases of ventricular arrhythmias (VA) and sudden cardiac death (SCD) have been described. Methods and results We present a case of a 64-year-old man admitted following exertional syncope occurred after climbing a flight of stairs. In the previous 2 weeks he had experienced fatigue and poor tolerance to exertion; moreover, 2 days before he had another episode of transient loss of consciousness after exertion. His medical history included left gonarthrosis and surgery for right carpal tunnel syndrome one year before. Physical examination did not revealed signs of heart failure. A 12-lead electrocardiogram (ECG) showed a pseudoinfarction pattern with QS waves in V2 and V3 leads and low voltage in all limb leads (Figure 1). Routine blood tests revealed N-terminal pro-bran natriuretic peptide (NT-ProBNP) of 3436 pg/ml (n.v. 0–125) and high-sensitive troponin T of 67 pg/ml (n.v. < 58). A trans-thoracic echocardiogram showed left ventricle (LV) concentric thickening wall with granular and sparkling pattern, mild reduced ejection fraction, reduced global longitudinal strain with apical sparing, grade 3 diastolic disfunction, biatrial enlargement, mild mitral regurgitation, right ventricle free wall thickening (8 mm), mild reduced TAPSE, and mild pericardial effusion. Cardiac magnetic resonance (CMR) confirmed ventricular wall thickening with evidence at T1 mapping of interstitial infiltration more evident in the septum and inferior-lateral wall with apical savings (Figure 2). A total body 99mTc-HDP scintigraphy showed cardiac uptake with intensity similar to bone signal (Perugini Score 2) suggesting ATTR cardiac amyloidosis (Figure 3). Both kappa and lambda concentrations were normal. The genetic testing did not reveled mutations in the TTR gene. We concluded for a diagnosis of ATTR wild-type CA. The Holter ECG monitoring registered numerous ventricular ectopic beats and an episode of non-sustained ventricular tachycardia. A coronary angiography ruled out coronary artery disease. In consideration of the clinical-instrumental picture an ICD was implanted. Conclusions In conclusion, CA is still underdiagnosed. VA and SCD may further complicate the prognosis. Early diagnosis and adequate stratification of the arrhythmic risk are essential to ensure patients the best therapeutic strategies.

scholarly journals Cardiac amyloidosis mimicking acute coronary syndrome: a case report and literature review

2020 ◽  
Author(s):  
Huan T Nguyen ◽  
Chuyen T H Nguyen
Keyword(s):  
Acute Coronary Syndrome ◽  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Troponin T ◽  
Elevated Serum ◽  
Clinical Signs ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Abdominal Skin ◽  
Coronary Syndrome

Abstract Background Cardiac amyloidosis, a progressive cardiac disease, results from the accumulation of undegraded proteinaceous substrates in the extracellular matrix of the heart. It may present as acute coronary syndrome (ACS); therefore, a clear distinction remains challenging in clinical practice. We describe a case of cardiac amyloidosis mimicking ACS. Case summary A 72-year-old man experienced chest discomfort for 2 days. He gradually developed dyspnoea during the preceding month. Electrocardiogram (ECG) showed sinus rhythm with right bundle branch block and low voltage. Echocardiography revealed concentric left ventricular thickening, biatrial dilation, and preserved ejection fraction with predominantly left ventricular basal hypokinesis. Serial testing of the cardiac biomarkers showed persistently increased high-sensitive cardiac troponin T levels and normal serum creatine kinase myocardial band levels. He was diagnosed with ACS with haemodynamic stability. However, coronary angiography demonstrated non-obstructive coronary arteries. Furthermore, significant macroglossia and periorbital purpura were noticed. Laboratory investigations revealed elevated serum immunoglobulin free light chain (FLC) kappa and lambda levels with an increased FLC ratio. Histological analysis of the biopsied abdominal skin confirmed amyloidosis. Discussion Cardiac amyloidosis often presents as restrictive cardiomyopathy. The usual symptoms include dyspnoea and peripheral oedema. Chest pain may manifest rarely, leading to misdiagnosis as coronary artery disease. Some findings suggestive of cardiac amyloidosis include clinical signs such as amyloid deposits, dyspnoea, low ECG voltage, and basal-predominant hypokinesis with relative apical sparing in echocardiography. Serum FLC test and abdominal skin biopsy can confirm the diagnosis of amyloidosis when a myocardial biopsy is not feasible.

scholarly journals Prediction of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging using electrocardiography findings in patients with confirmed cardiac amyloidosis

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
N Funabashi ◽  
Y Kobayashi
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
High Voltage ◽  
Cardiac Rhythm ◽  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Late Enhancement ◽  
Left Ventricular ◽  
Wall Thickening ◽  
Resonance Imaging

Abstract Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without high voltage R wave in V5 leads on ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invade the right ventricle (RV), and left (LA) and right (RA) atria to cause ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To predict sites of wall thickening and abnormal late enhancement (LE) on cardiac computed tomography (CT) and magnetic resonance imaging (MRI), suggesting amyloid invasion, using cardiac rhythm and other ECG findings in patients with cardiac amyloidosis confirmed by biopsy. Methods A total of 26 patients (11 females) with suspected cardiac amyloidosis, showing LV wall thickening by TTE without a high voltage R wave in V5 leads on ECG, underwent cardiac enhanced CT. LV wall thickening on CT in the early phase led to late phase acquisition to detect LE. Five patients (3 females, mean age 73 years) were diagnosed with cardiac amyloidosis: complicated multiple myeloma, 2; senile ATTR (transthyretin) amyloidosis, 1; immunoglobulin light chain (AL) amyloidosis 1; and transthyretin mutation, 1. Four patients underwent cardiac MRI. Results Two patients (cases 1 and 2) had SSS (junctional rhythm), one had atrial tachycardia, and the remaining two (cases 4 and 5) had a normal sinus rhythm. In case 1, ECG showed a left axis QRS wave deviation, no low voltage R wave in limb leads and a mild LA load. Wall thickening in the basal interventricular septum (IVS), LV inferior-posterior wall, LA on CT, LE in the endocardium in whole LV, RV, and RA on CT, and LE in the endocardium in whole LV, RV, LA, and IVS on MRI were observed. In case 2, ECG showed a normal QRS wave axis, no low voltage R wave in limb leads, no LA load, wall thickening in whole LV, RV, LA, and IVS on CT, and unclear (CT) or no (MRI) LE. In case 3, ECG showed a normal QRS wave axis, with low voltage R wave in limb leads, no LA load, wall thickening in LA and basal IVS on CT, LE in LA and basal IVS on CT, and LE in LA only on MRI. In case 4, ECG showed left axis QRS wave deviation, a low voltage R wave in limb leads, and no LA load, wall thickening in the LA and RV moderator band on CT, unclear LE on CT, and LE in whole LV, endocardium in the RV, and whole IVS on MRI. In case 5, ECG showed a right axis QRS wave deviation, low voltage R wave in limb leads, and a mild LA load, wall thickening in the IVS, LV lateral wall, LV anterior wall, RA, RV outflow tract, and RA appendage, and no LE on CT (MRI not performed). Conclusions This pilot study of a few patients with cardiac amyloidosis revealed few links between cardiac rhythm and other ECG findings with sites of wall thickening and abnormal LE. However, a longer-term study of more patients may lead to detecting an association between these variables with this methodology. FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals Cardiac Amyloidosis with Discordant QRS Voltage between Frontal and Precordial Leads

Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Low Voltage ◽  
Disease Process ◽  
Left Ventricular ◽  
Clinical Suspicion ◽  
Immunoglobulin Light Chain ◽  
Different Types

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.

scholarly journals Through thick and thin: Diagnosis of transthyretin cardiac amyloidosis through non-invasive multimodality imaging

2021 ◽  
pp. 201010582110061
Author(s):  
Raja Ezman Raja Shariff ◽  
Hafisyatul Aiza Zainal Abidin ◽  
Sazzli Kasim
Keyword(s):  
Cardiac Amyloidosis ◽  
Troponin T ◽  
Multimodality Imaging ◽  
High Sensitivity ◽  
Dark Blood ◽  
Non Invasive ◽  
Doppler Study ◽  
Alternative Means ◽  
High Sensitivity Troponin T

Cardiac amyloidosis is a severely underdiagnosed cause of heart failure with preserved ejection fraction. We report a case of highly probable transthyretin (ATTR) cardiac amyloidosis (ATTR-CA) diagnosed through the assistance of non-invasive multimodality imaging. An 81-year-old man presented with worsening dyspnoea, reduced effort tolerance and limb swelling. Examination and bedside investigations demonstrated congestive cardiac failure. On arrival, N-terminal-pro B-type natriuretic peptide was 2400 ng/L, and high-sensitivity troponin T was 78 mmol/L. Echocardiography showed severe left and right ventricular hypertrophy, and a Doppler study revealed diastolic dysfunction. Cardiac magnetic resonance imaging revealed on non-conventional dark blood sequence an abnormal inversion time for nulling myocardium suggestive of infiltrative disease, including amyloidosis. The patient was referred for nuclear-based studies involving technetium-99m pyrophosphate which demonstrated changes highly diagnostic of ATTR-CA. Early diagnosis of ATTR-CA remains paramount due to the increasing availability of disease-modifying therapies. Current guidelines recognise the role of multimodality imaging in confidently recognising the disease without the need for histological evidence in the appropriate context, providing an alternative means of diagnosis.

scholarly journals Relationship between left atrial strain and left atrial bipolar voltage in patients undergoing catheter ablation for atrial fibrillation

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
Y Takahashi ◽  
T Kitai ◽  
T Watanabe ◽  
T Fujita
Keyword(s):  
Atrial Fibrillation ◽  
Left Atrial ◽  
Speckle Tracking ◽  
Longitudinal Strain ◽  
Low Voltage ◽  
Pulmonary Veins ◽  
Global Longitudinal Strain ◽  
Inferior Wall ◽  
Bipolar Voltage ◽  
Global Mean

Abstract Background Low-voltage zone (LVZ) in the left atrium (LA) seems to represent fibrosis. LA longitudinal strain assessed by speckle tracking method is known to correlate with the extent of fibrosis in patients with mitral valve disease. Purpose We sought to identify the relationship between LA longitudinal strain and LA bipolar voltage in patients with atrial fibrillation (AF). We tested the hypothesis that LA strain can predict LA bipolar voltage. Methods A total of 96 consecutive patients undergoing initial AF ablation were analyzed. All patients underwent transthoracic echocardiography including 2D speckle tracking measurement on the day before ablation during sinus rhythm (SR group, N=54) or during AF (AF group, N=42). LA longitudinal strain was measured at basal, mid, and roof level of septal, lateral, anterior, and inferior wall in apical 4- and 2-chamber view. Global longitudinal strain (GLS) was defined as an average value of the 12 segments. LA voltage map was created using EnSite system, and global mean voltage was defined as a mean of bipolar voltage of the whole LA excluding pulmonary veins and left atrial appendage. LVZ was defined as less than 1.0 mV. Results There was a significantly positive correlation between GLS and global mean voltage (r=0.708, p<0.001). Multivariate regression analysis showed that GLS and age were independent predictors of global mean voltage. There was a significant negative correlation between global mean voltage and LVZ areas. Conclusions There was a strong correlation between LA longitudinal strain and LA mean voltage. GLS can independently predict LA mean voltage, subsequently LVZ areas in patients with AF. Funding Acknowledgement Type of funding source: None

scholarly journals The value of regional myocardial function assessment in patients with acute myocarditis at baseline and mid term follow-up

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Boidol ◽  
M Koziel ◽  
K Miszalski-Jamka ◽  
J Klys ◽  
Z Kalarus ◽  
...  
Keyword(s):  
Longitudinal Strain ◽  
Ventricular Remodeling ◽  
Clinical Course ◽  
Troponin T ◽  
Global Longitudinal Strain ◽  
Cardiac Resynchronization ◽  
Global Improvement ◽  
Myocardial Disease ◽  
Mechanical Dispersion

Abstract Background The clinical course and ventricular remodeling in inflammatory myocardial disease could be unpredictable. No single functional parameter has been confirmed as a powerful predictor of clinical course and functional recovery assessment in patients with acute inflammatory myocardial disease. Purpose The aim of the study was to assess the mechanical properties of the myocardium in patients with active myocarditis at baseline and follow-up. Methods Database from a high volume, tertiary cardiology center was analysed to identify patients with active myocarditis, based on clinical presentation and ≥1 diagnostic criteria from different categories (including electrocardiography/holter, elevated troponin T/I levels, functional or structural abnormalities on cardiac imaging or tissue characterization by cardiac magnetic resonance) between 2016 and 2019. Conventional and speckle tracking echocardiography including global longitudinal strain (GLS) mechanical dispersion (MD) was completed at baseline and at 17±13 months follow-up. MD was calculated as a standard deviation of time to peak longitudinal strain derived from all left ventricle segments in 3 apical views. Results 61 consecutive patients [50 M, 11F, end-diastolic volume 212±84 ml, end-systolic volume 130±90ml, ejection fraction (EF) 42±16%] were enrolled. During the entire follow-up 1 patient died at early observation. Implantable cardioverter-defibrillator was implanted in 5 patients (primary prevention 4, secondary 1), cardiac resynchronization therapy pacemaker in 1 patient. Despite of significant global improvement (EF 42±16% vs 52±10%, p<0.001) the limited regional improvement was noticed (GLS 14±6% vs 15±4%, p = NS; MD 47±18 ms vs 45±20 ms, p=NS) in all patients at 17±13 months follow-up. There was a strong negative association between GLS and MD at baseline (Figure 1), and slightly weaker at follow-up (R=0.47, Pearson's correlation). Moreover, the GLS correlated well the change of MD in each individual patient. Conclusions Mechanical dispersion and global longitudinal strain may serve as an additional markers of myocardial damage and potential predictive markers in non ischemic cardiomyopathy patients with proven inflammatory origin. Funding Acknowledgement Type of funding source: None

Abstract 14432: Myocardial Work in Stage I and II Hypertensive Patients

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Akshar Jaglan ◽  
Sarah Roemer ◽  
Ana C Perez Moreno ◽  
Bijoy K Khandheria
Keyword(s):  
Systolic Function ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Deformation Analysis ◽  
Hypertensive Patients ◽  
Reduced Ejection Fraction ◽  
Work Index ◽  
Significant Difference ◽  
Global Work ◽  
Lv Systolic Function

Introduction: Myocardial work is a novel parameter that can be used in a clinic setting to assess left ventricular (LV) pressures and deformation. This study sought to distinguish patterns of global myocardial work index in hypertensive vs. non-hypertensive patients. Methods: Fifty (25 male, mean age 60±14 years) hypertensive patients and 15 (7 male, mean age 38±12 years) control patients underwent transthoracic echocardiography at rest. Hypertensive patients were divided into stage 1 (26 patients) and stage 2 (24 patients) based on the 2017 American College of Cardiology guidelines. We excluded patients with suboptimal image quality for myocardial deformation analysis, reduced ejection fraction (EF), valvular heart disease, and arrhythmia. Global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency (GWE) were estimated from LV pressure strain loops utilizing proprietary software from speckle-tracking echocardiography. LV systolic and diastolic pressures were estimated using a noninvasive brachial artery cuff. Results: Global longitudinal strain (GLS) and EF were preserved between the two groups with no statistically significant difference whereas there was a statistically significant difference in the GWI (p<0.01), GCW (p=0.03), GWW (p<0.01), and GWE (p=0.03) (Figure and Table). Conclusions: Myocardial work gives us a closer look at the relationship between LV pressure and contractility in settings of increased load dependency whereas LVEF and GLS cannot. We show how myocardial work is an advanced assessment of LV systolic function in hypertensive patients.

Abstract 15293: Increased Level of Cardiac Troponin T is a Clue of Cardiac Amyloidosis in Patients With Myocardial Hypertrophy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Seiji Takashio ◽  
Megumi Yamamuro ◽  
Toshihisa Anzai ◽  
Hisao Ogawa
Keyword(s):  
Endomyocardial Biopsy ◽  
Cardiac Troponin ◽  
Cardiac Amyloidosis ◽  
Myocardial Hypertrophy ◽  
Troponin T ◽  
Area Under The Curve ◽  
Al Amyloidosis ◽  
Cardiac Troponin T ◽  
Characteristic Analysis ◽  
Precise Diagnosis

Background: Cardiac amyloidosis (CA) is an important differential diagnosis in patients with myocardial hypertrophy. The precise diagnosis of CA requires endomyocardial biopsy to demonstrate amyloid deposition, but this procedure is relatively invasive and cannot be performed routinely. Therefore, it is important to increase pretest probability of CA in patients with myocardial hypertrophy by noninvasive modalities. Because it is well known that cardiac troponin level is elevated in patients with CA, we hypothesized that increased level of cardiac troponin T contributes to diagnosis of CA in patients with myocardial hypertrophy using high sensitive assay (hs-TnT). Methods and Results: Among nonischemic patients with myocardial hypertrophy (interventricular septal thickness≧ 12 mm), hs-TnT level was measured in 28 CA patients (senile systemic amyloidosis: 13, AL amyloidosis: 14, familial amyloidosis: 1) proven amyloidosis pathologically and 29 non-CA patients proven by endomyocardial biopsy. It was significantly higher in CA patients than non-CA patients (0.075 [0.047-0.116] ng/ml vs. 0.013 [0.009-0.019] ng/ml; p<0.001: Figure). Receiver operating characteristic analysis selected 0.030 ng/ml as the best cutoff value of diagnosis for CA, with a sensitivity and specificity of 96% and 93%, respectively and area under the curve of 0.98 (95% confidence interval 0.94 to 1.00, p<0.001). Conclusions: Increased level of hs-TnT (>0.030 ng/ml) is highly suggestive of CA in patients with myocardial hypertrophy. These patients need additional diagnostic approach for CA.

scholarly journals Can myocardial work indices contribute to the exploration of patients with cardiac amyloidosis?

Open Heart ◽  
2020 ◽  
Vol 7 (2) ◽  
pp. e001346
Author(s):  
Aénora Roger-Rollé ◽  
Eve Cariou ◽  
Khailène Rguez ◽  
Pauline Fournier ◽  
Yoan Lavie-Badie ◽  
...  
Keyword(s):  
Cardiac Amyloidosis ◽  
Characteristic Curve ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Peak Oxygen Consumption ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Echocardiographic Parameters ◽  
Global Work ◽  
Better Than

BackgroundCardiac amyloidosis (CA) is a life-threatening restrictive cardiomyopathy. Identifying patients with a poor prognosis is essential to ensure appropriate care. The aim of this study was to compare myocardial work (MW) indices with standard echocardiographic parameters in predicting mortality among patients with CA.MethodsClinical, biological and transthoracic echocardiographic parameters were retrospectively compared among 118 patients with CA. Global work index (GWI) was calculated as the area of left ventricular pressure–strain loop. Global work efficiency (GWE) was defined as percentage ratio of constructive work to sum of constructive and wasted works. Sixty-one (52%) patients performed a cardiopulmonary exercise.ResultsGWI, GWE, global longitudinal strain (GLS), left ventricular ejection fraction (LVEF) and myocardial contraction fraction (MCF) were correlated with N-terminal prohormone brain natriuretic peptide (R=−0.518, R=−0.383, R=−0.553, R=−0.382 and R=−0.336, respectively; p<0.001). GWI and GLS were correlated with peak oxygen consumption (R=0.359 and R=0.313, respectively; p<0.05). Twenty-eight (24%) patients died during a median follow-up of 11 (4–19) months. The best cut-off values to predict all-cause mortality for GWI, GWE, GLS, LVEF and MCF were 937 mm Hg/%, 89%, 10%, 52% and 15%, respectively. The area under the receiver operator characteristic curve of GWE, GLS, GWI, LVEF and MCF were 0.689, 0.631, 0.626, 0.511 and 0.504, respectively.ConclusionIn CA population, MW indices are well correlated with known prognosis markers and are better than LVEF and MCF in predicting mortality. However, MW does not perform better than GLS.

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