scholarly journals Súlyos szívelégtelenség hátterében álló transthyretin-amyloidosis elkésett diagnózisa

2021 ◽  
Vol 51 (2) ◽  
pp. 131-139
Author(s):  
Viktória Nagy ◽  
Gergely Rácz ◽  
Bence Radics ◽  
Lidia Hategan ◽  
Hedvig Takács ◽  
...  
Keyword(s):  
Heart Failure ◽  
Wall Thickness ◽  
Heart Muscle ◽  
Low Voltage ◽  
Troponin T ◽  
Diagnostic Delay ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Transthyretin Amyloidosis ◽  
Familial Form

Several heart muscle diseases can cause heart failure, the etiological diagnosis of which may serve the basis of targeted, tailored, effective personalized therapy. Such a heart muscle disease is transthyretin amyloidosis (ATTR) which is an infiltrative disorder affecting most frequently the heart and peripheral nerves, due to the accumulation of transthyretin protein. Deposition of mutant transthyretin, due to mutations in the TTR gene encoding for transthyretin, occurs in the familial form of the disease (hATTR), while in senile amyloidosis, wild-type transthyretin accumulates (wtATTR). We report on a 79-years-old male patient who presented 9 years before because of novel onset atrial fibrillation. Echocardiography at that time showed severe concentric left ventricular hypertrophy which progressed through the years. Laboratory values displayed increased liver enzymes and troponin T levels. He was hospitalized several times because of left- and later right-sided heart failure, with increasing tendency towards hypotension. At last admission he was admitted to us after suffering syncope at home necessitating cardiopulmonary resuscitation. Transthoracic echocardiography showed severe, concentric biventricular hypertrophy (LV wall thickness 22 mm, RV wall thickness 12 mm), with depressed LV and RV function, apical sparing, and low cardiac output. Taking low voltage, seen on ECG, and other anamnestic data into consideration, the suspicion of ATTR amyloidosis was raised. The patient died after a short observational period due to intractable heart failure. Autopsy and histology revealed systemic amyloidosis, affecting predominantly the heart which was interpreted as wtATTR according to immunohistochemistry, negative TTR gene sequencing and the lack of monoclonal gammopathy. Our case illustrates the importance of the diagnostic delay which is very frequently encountered in TTR amyloidosis. Features of ATTR which includes the multidisciplinary nature of assessment, specialized diagnostic modalities and unique therapy call for the need of specialized diagnostic centers.

scholarly journals P677 When to look for wtTTR amyloidosis in heart failure: increasing chances of positive gammagraphic study

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Ibero ◽  
A Riesgo ◽  
M Rodriguez ◽  
M Morales ◽  
J Muniz ◽  
...  
Keyword(s):  
Heart Failure ◽  
Wall Thickness ◽  
Troponin T ◽  
Interventricular Septum ◽  
Mean Value ◽  
Left Ventricular ◽  
P Value ◽  
Negative Results ◽  
Nyha Classification ◽  
Grade 3

Abstract Introduction Wild type transtirretin (wtATTR) amyloidosis has become increasingly recognized as a major cause for heart failure (HF). Diagnosis requires complex work up such as DPD scintigraphy (DPDs). Availability of DPDs is limited urging to identify factors to increase its diagnostic rentability. Methods Retrospective study of HF patients between 2013 and 2019 with suspected wtATTR and DPDs was performed. Baseline characteristics, biomarkers, EKG findings, TTE parameters (LVEF, LV diastolic function, TAPSE, interventricular septum (IVS), LV mass (LVM), relative LV wall thickness (RWT), indexed LVTD volume and diameter, indexed LA diameter (iAPLAD)) and DPDs results were studied. Two groups were created according to DPDs (SP those with positive results for amyloidosis and SN those with negative results). For statistical SPSS v.21 was used. Results 37 patients were studied. In our population 54% had positive DPDs for amyloidosis. Among SP patients 50% were classified as grade 2 of Perugini classification meanwhile 50% were grade 3; mean value of heart to contralateral ratio was 2,73 ± 0,8. There were no differences in NYHA classification. Differences among SP and SN patients are reflected in Table 1. Conclusions In our population SP was found to have higher biomarkers values and higher IVS, LVM, RWT and iAPLAD with poorer RV function. Further investigation is needed in order to confirm our result and identify prognostic factors. Table 1: Results Total (37) PS (20) PN (17) p value Age (Y) 78 ± 7,9 80 ± 6,5 75,29 ± 8,7 0,05 Gender (male) (%) 30 (8) 19 (95) 11(64) 0,11 Carpal tunnel sdr (%) 3(8) 3 (15) 0 (0) 0,09 Systolic blood pressure (x ± sd) 127 ± 21 118 ± 18 138,5 ± 19 <0,01 ProBNP (x ± sd) 3596 ± 4002 4615 ± 4538 1761 ± 1927 <0,05 Troponin T (x ± sd) 132 ± 360 66,4 ± 35 43 ± 39 0,09 Pseudoinfarction pattern (%) 26 (70) 17 (85) 9 (52) <0,05 IVS (mm) (x ± sd) 14,6 ± 4,5 16,6 ± 5,3 13 ± 3 <0,01 LVM (g/m2) (x ± sd) 142,5 ± 68 180 ± 80 111 ± 32 <0,01 RWT (x ± sd) 0,68 ± 0,42 0,8 ± 0,24 0,56 ± 0,5 <0,01 LVEF (%) (x ± sd) 57,4 ± 11 54 ± 12 60 ± 10 0,12 iAPLAD (mm/m2) (x ± sd) 25,4 ± 6 28,7 ± 6 22,7 ± 4,3 <0,01 TAPSE (mm) (x ± sd) 18,6 ± 5 16,1 ± 4 20,6 ± 5 <0,05 IVS = interventricular septum; LVM = left ventricular mass; RWT = relative wall thickness; iAPLAD= indexed anteroposterior left atrium diameter.

Combination of Huangqi Granule and Rosuvastatin Improves the Cardiac Function in Heart Failure

2020 ◽  
Vol 19 (2) ◽  
pp. 181-187
Author(s):  
Jing Li ◽  
Yun Zhang ◽  
Weizhong Huangfu ◽  
Yuhong Ma
Keyword(s):  
Heart Failure ◽  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Wall Thickness ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Model Group ◽  
Ventricular Ejection Fraction ◽  
Posterior Wall Thickness

Using rat models of heart failure, we evaluated the effects of rosuvastatin and Huangqi granule alone and in combination on left ventricular end-diastolic dimension, left ventricular end-systolic dimension, left ventricular ejection fraction, left ventricular posterior wall thickness at end-diastole, and left ventricular posterior wall thickness at end-systole. Results showed that left ventricular end-diastolic dimension, left ventricular end-systolic dimension in the rosuvastatin + Huangqi granule group were significantly decreased (P ‹ 0.01), while left ventricular ejection fraction, left ventricular posterior wall thickness at end-diastole and left ventricular posterior wall thickness at end-systole were significantly increased (P ‹ 0.05). The serum IL-2, IFN-β, and TNF-α in rosuvastatin + Huangqi granule group were significantly lower than those in model group (P ‹ 0.05). However, the levels of S-methylglutathione and superoxide dismutase in rosuvastatin + Huangqi granule group were significantly higher, while nitric oxide was significantly lower than that in the model group (P ‹ 0.05). Also, compared to the model group, the apoptosis rate, and the autophagy protein LC3-II in the cardiomyocytes of rosuvastatin + Huangqi granule group was significantly decreased (P ‹ 0.01), while the level of p62 protein was significantly increased (P ‹ 0.01). The levels of AMPK and p-AMPK in cardiomyocytes were significantly lower in rosuvastatin + Huangqi granule group; however, the levels of mTOR and p-mTOR showed an opposite trend (P ‹ 0.05). To sum up, rosuvastatin + Huangqi granule could improve the cardiac function, decrease the level of oxidative stress, and inflammatory cytokines in rats with HF. The possible underlying mechanism might be inhibition of autophagy and reduced apoptosis in cardiomyocytes by regulating AMPK-mTOR signaling pathway.

scholarly journals Hereditary transthyretin amyloidosis: predictors of conduction disease

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.F Dias De Frias ◽  
P Rodrigues ◽  
M Trepa ◽  
M Fontes-Oliveira ◽  
R Costa ◽  
...  
Keyword(s):  
Troponin T ◽  
Pacemaker Implantation ◽  
Outcome Data ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
P Value ◽  
Neurologic Manifestations ◽  
Ventricular Pacing ◽  
Transthyretin Amyloidosis ◽  
Formal Indication

Abstract Introduction Pacemakers are frequently needed due to a high prevalence of conduction disease in mutated ATTR amyloidosis (mATTR). We aimed to identify the variables associated with the need of pacemaker implantation in this population. Methods We retrospectively studied 255 patients with suspicion of heart involvement of mATTR observed at our cardiology clinic during the last year. Clinical and outcome data were retrieved by chart review. We have defined the need for pacemaker implantation as: 1) the formal guidelines indications or 2) Ventricular pacing >10% in patients who had prophylactic pacemaker implantation prior to liver transplantation (LT). This way, we have defined 3 different groups: group 1: patients with no evidence of conduction disease; group 2: patients with conduction disease, but no formal indication for pacemaker implantation; and group 3: patients with formal indication for pacemaker implantation or ventricular pacing >10% in patients who had prophylactic pacemaker implantation prior to hepatic transplantation. Results We included 255 patients (50±14 years, 53% male, 52.5% treated with tafamidis and 27% had prior LT, and 10% with atrial fibrillation), 43.3% with no evidence of conduction disease, 32.3% with conduction disease, but no formal indication for pacemaker implantation and 24.4% with formal indication for pacemaker implantation. Patients with formal indication for pacemaker implantation were older, with longer duration of neurologic manifestations, with higher concentration of both Troponin T and NT-proBNP and with higher number of organs affected. In multivariate analysis, longer duration of neurologic manifestations (OR 1.090 – 95% IC: 1.036–1.145, p-value 0.001), Left ventricular (LV) maximal wall thickness (OR 1.230 – 95% CI: 1.070–1.414, p-value 0.004), neurologic staging (OR 3.420 – 95% CI: 1.443–8.104, p-value 0.005) and higher number of organs affected (OR 1.719 – 95% CI: 1.218–2.424, p-value 0.002) all showed to be independent predictors of the need for pacemaker implantation, in contrast to LV ejection fraction and serum concentration of Troponin T and NT-proBNP. We've also found a statistical significant association between conduction disease and ophthalmic manifestations. Conclusions Our findings suggest that the need for pacemaker implantation in patients with mATTR is closer linked to the duration, severity and affected number of organs than to cardiac biomarkers or echocardiographic findings. Funding Acknowledgement Type of funding source: None

scholarly journals Prognostic Value of Combination of Cardiac Troponin T and B-Type Natriuretic Peptide after Initiation of Treatment in Patients with Chronic Heart Failure

2003 ◽  
Vol 49 (12) ◽  
pp. 2020-2026 ◽  
Author(s):  
Junnichi Ishii ◽  
Wei Cui ◽  
Fumihiko Kitagawa ◽  
Takahiro Kuno ◽  
Yuu Nakamura ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chronic Heart Failure ◽  
Cardiac Troponin ◽  
Troponin T ◽  
Myocardial Damage ◽  
Left Ventricular ◽  
Functional Class ◽  
Cardiac Troponin T ◽  
Cardiac Events ◽  
Nyha Functional Class

Abstract Background: Recent studies have suggested that cardiac troponin T (cTnT) and troponin I may detect ongoing myocardial damage involved in the progression of chronic heart failure (CHF). This study was prospectively designed to examine whether the combination of cTnT, a marker for ongoing myocardial damage, and B-type natriuretic peptide (BNP), a marker for left ventricular overload, would effectively stratify patients with CHF after initiation of treatment. Methods: We measured serum cTnT, plasma BNP, and left ventricular ejection fraction (LVEF) on admission for worsening CHF [New York Heart Association (NYHA) functional class III to IV] and 2 months after initiation of treatment to stabilize CHF (n = 100; mean age, 68 years). Results: Mean (SD) concentrations of cTnT [0.023 (0.066) vs 0.063 (0.20) μg/L] and BNP [249 (276) vs 753 (598) ng/L], percentage increased cTnT (>0.01 μg/L; 35% vs 60%), NYHA functional class [2.5 (0.6) vs 3.5 (5)], and LVEF [43 (13)% vs 36 (12)%] were significantly (P <0.01) improved 2 months after treatment compared with admission. During a mean follow-up of 391 days, there were 44 cardiac events, including 12 cardiac deaths and 32 readmissions for worsening CHF. On a stepwise Cox regression analysis, increased cTnT and BNP were independent predictors of cardiac events (P <0.001). cTnT >0.01 μg/L and/or BNP >160 ng/L 2 months after initiation of treatment were associated with increased cardiac mortality and morbidity rates. Conclusion: The combination of cTnT and BNP measurements after initiation of treatment may be highly effective for risk stratification in patients with CHF.

scholarly journals Modification of a Volume-Overload Heart Failure Model to Track Myocardial Remodeling and Device-Related Reverse Remodeling

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Egemen Tuzun ◽  
Roger Bick ◽  
Cihan Kadipasaoglu ◽  
Jeffrey L. Conger ◽  
Brian J. Poindexter ◽  
...  
Keyword(s):  
Heart Failure ◽  
Wall Thickness ◽  
Ventricular Remodeling ◽  
Diastolic Pressure ◽  
Volume Overload ◽  
Left Ventricular ◽  
Reverse Remodeling ◽  
Sheep Model ◽  
Dystrophin Expression ◽  
Cellular Markers

Purpose. To provide an ovine model of ventricular remodeling and reverse remodeling by creating congestive heart failure (CHF) and then treating it by implanting a left ventricular assist device (LVAD). Methods. We induced volume-overload heart failure in 2 sheep; 20 weeks later, we implanted an LVAD and assessed recovery 11 weeks thereafter. We examined changes in histologic and hemodynamic data and levels of cellular markers of CHF. Results. After CHF induction, we found increases in LV end-diastolic pressure, LV systolic and diastolic dimensions, wall thickness, left atrial diameter, and atrial natriuretic protein (ANP) and endothelin-1 (ET-1) levels; β-adrenergic receptor (BAR) and dystrophin expression decreased markedly. Biopsies confirmed LV remodeling. After LVAD support, LV systolic and diastolic dimensions, wall thickness, and mass, and ANP and ET-1 levels decreased. Histopathologic and hemodynamic markers improved, and BAR and dystrophin expression normalized. Conclusions. We describe a successful sheep model for ventricular and reverse remodeling.

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 Retrospective evaluation of echocardiographic variables for prediction of heart failure hospitalization in heart failure with preserved versus reduced ejection fraction: A single center experience

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0244379
Author(s):  
Michael M. Hammond ◽  
Changyu Shen ◽  
Stephanie Li ◽  
Dhruv S. Kazi ◽  
Marwa A. Sabe ◽  
...  
Keyword(s):  
Heart Failure ◽  
Wall Thickness ◽  
Systolic Function ◽  
Medical Center ◽  
Left Ventricular ◽  
Single Center ◽  
Reduced Ejection Fraction ◽  
Medicare Claims ◽  
Structural Variables ◽  
Eccentric Hypertrophy

Background Limited data exist on the differential ability of variables on transthoracic echocardiogram (TTE) to predict heart failure (HF) readmission across the spectrum of left ventricular (LV) systolic function. Methods We linked 15 years of TTE report data (1/6/2003-5/3/2018) at Beth Israel Deaconess Medical Center to complete Medicare claims. In those with recent HF, we evaluated the relationship between variables on baseline TTE and HF readmission, stratified by LVEF. Results After excluding TTEs with uninterpretable diastology, 5,900 individuals (mean age: 76.9 years; 49.1% female) were included, of which 2545 individuals (41.6%) were admitted for HF. Diastolic variables augmented prediction compared to demographics, comorbidities, and echocardiographic structural variables (p < 0.001), though discrimination was modest (c-statistic = 0.63). LV dimensions and eccentric hypertrophy predicted HF in HF with reduced (HFrEF) but not preserved (HFpEF) systolic function, whereas LV wall thickness, NT-proBNP, pulmonary vein D- and Ar-wave velocities, and atrial dimensions predicted HF in HFpEF but not HFrEF (all interaction p < 0.10). Prediction of HF readmission was not different in HFpEF and HFrEF (p = 0.93). Conclusions In this single-center echocardiographic study linked to Medicare claims, left ventricular dimensions and eccentric hypertrophy predicted HF readmission in HFrEF but not HFpEF and left ventricular wall thickness predicted HF readmission in HFpEF but not HFrEF. Regardless of LVEF, diastolic variables augmented prediction of HF readmission compared to echocardiographic structural variables, demographics, and comorbidities alone. The additional role of medication adherence, readmission history, and functional status in differential prediction of HF readmission by LVEF category should be considered for future study.

scholarly journals Az N-terminális pro-B natriureticus peptid mérésének korai ismétlése akut szívelégtelenség miatt hospitalizált betegeken

2018 ◽  
Vol 159 (25) ◽  
pp. 1009-1012
Author(s):  
János Tomcsányi ◽  
Miklós Somlói ◽  
Béla Bózsik ◽  
Tamás Frész ◽  
Erzsébet Nagy
Keyword(s):  
Heart Failure ◽  
Acute Heart Failure ◽  
Natriuretic Peptide ◽  
Troponin T ◽  
Left Ventricular Systolic Dysfunction ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Response To Therapy ◽  
Prospective Data

Abstract: Introduction: The determination of natriuretic peptide levels in patients hospitalized for suspected acute heart failure is important for the confirmation of the diagnosis and for the prognosis. Changes in natriuretic peptide levels in response to therapy have a strong prognostic value. Aim: To decide whether repeated natriuretic peptide measurements for acute heart failure show changes that could influence the diagnosis and/or the prognosis. Method: Prospective data collection was carried out of N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels on admission and within 12 hours in patients hospitalized for acute heart failure. Only the data of those patients were analyzed whose symptoms started within 24 hours prior to admission and were due to acute heart failure. Results: The 23 patients whose data we analyzed had an average age of 77.9 ± 8.3 years. Most of them had left ventricular systolic dysfunction with an average ejection fraction of 34.1 ± 3.9%. The time between the start of symptoms and the first measurement was 6.7 ± 2.2 hours, while the time until the repeated determination was 6.5 ± 2.2 hours after the first measurement. The median value of the NT-proBNP levels in the 6 hours control showed an increase from 5064 pg/mL to 8847 pg/mL (p<0.0005), which amounts to a 75 percent increase – mean hs-troponin T showed an increase from 46 ± 25 ng/L to 78 ± 51 ng/L (p<0.002). Conclusions: A significant increase in NT-proBNP levels is to be expected in early repeated measurement after hospital admission. This fact could have diagnostic and prognostic consequences if validated in a larger patient population. Orv Hetil. 2018; 159(25): 1009–1012.

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