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 AS-amyloidosis. Dual pathology or novel disease? A multimodality, multi-centre assessment across health and disease

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
K Patel ◽  
P Scully ◽  
C Nitsche ◽  
S Williams ◽  
T Tillin ◽  
...  
Keyword(s):  
Carpal Tunnel Syndrome ◽  
Old Age ◽  
Wall Thickness ◽  
Carpal Tunnel ◽  
Troponin T ◽  
Left Ventricular ◽  
Older Age ◽  
Cardiac Biomarkers ◽  
P Value ◽  
Tunnel Syndrome

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): British Heart Foundation onbehalf AS-Amyloidosis consortium Background The coexistence of severe aortic stenosis (lone AS) and transthyretin cardiac amyloidosis (lone amyloidosis) is common, but the resultant AS-amyloidosis phenotype is unclear. Purpose We characterised AS-amyloidosis, hypothesizing that the dual insult of AS-amyloidosis results is a severe phenotype. Methods We compared four cohorts with deep phenotyping: 81 older age controls, 359 lone AS, 36 AS-amyloidosis (Perugini grade 2 and 3) and 107 lone amyloidosis (Perugini grade 2 and 3). Results AS-amyloidosis was similar to lone AS with respect to left ventricular mass and LVEF (57 (45, 64)%). It was similar to lone amyloidosis with respect to lateral S" (0.04 (0.03, 0.06) m/s), NT-proBNP (4149 (1449, 6459) ng/L) and troponin T (56 (34, 100) ng/L). Whilst, prevalence of carpal tunnel syndrome (CTS) (17%) and diastolic function (E/A ratio 1.1 (0.8, 2.8)) were intermediate. Conclusion AS-amyloidosis is not a double insult from AS and amyloidosis, but a mixed phenotype with features similar to lone amyloidosis (cardiac biomarkers), lone AS (remodelling and LVEF) or intermediate (diastology and CTS). Characteristics across all 4 groups Variable Older age controls (n = 81) Lone AS (n = 359) AS-amyloidosis (n = 36) Lone amyloidosis (n = 107) P value Age (years) 82 (80, 84)*†‡ 85 (80, 88)§∞ 88 (85, 92)# 80 (75, 84) <0.005 Sex (% male) 69 *‡ 49 ∞ 61 # 94 <0.005 Carpal tunnel syndrome (%) 0 2 § 17 # 38 <0.005 Voltage/mass ratio 0.22 (0.14, 0.27)‡ 0.18 (0.13, 0.28)∞ 0.18 (0.09, 0.21)# 0.07 (0.05, 0.10) <0.005 NT-ProBNP (ng/L) 131 (66, 221)*†‡ 1629 (639, 3941)§∞ 4149 (1449, 6459) 2888 (1755, 5483) <0.005 hsTnT (ng/L) 12 (8, 17)*†‡ 24 (15, 40)§∞ 56 (34, 100) 62 (41, 82) <0.005 Inferolateral wall thickness (cm) 0.9 (0.8, 1.0)*†‡ 1.1 (0.9, 1.3)∞ 1.3 (1.1, 1.5)# 1.7 (1.6, 1.9) <0.005 Anteroseptal wall thickness (cm) 1.0 (0.9, 1.2)*†‡ 1.4 (1.2, 1.6)§∞ 1.5 (1.3, 1.8) 1.7 (1.6, 1.9) <0.005 Indexed LV mass (g/m2) 79 (66, 102)*†‡ 128 (99, 152)∞ 126 (116, 140)# 174 (159, 200) <0.005 LVEF (%) 59 (54, 63)‡ 59 (50, 65)∞ 57 (45, 64)# 39 (31, 48) <0.005 Lateral S" (m/s) 0.08 (0.07, 0.09)*†‡ 0.07 (0.05, 0.08)§∞ 0.05 (0.04, 0.07) 0.05 (0.04, 0.06) <0.005 Septal S" (m/s) 0.06 (0.06, 0.08)*†‡ 0.05 (0.04, 0.06)∞ 0.04 (0.03, 0.06) 0.04 (0.03, 0.05) <0.005 E/A 0.7 (0.6, 0.8)*†‡ 0.8 (0.7, 1.3)§∞ 1.1 (0.8, 2.8)# 2.4 (1.8, 3.3) <0.005 RV Wall thickness (cm) 0.4 (0.3, 0.4)*†‡ 0.4 (0.4, 0.6)∞ 0.6 (0.4, 0.7)# 0.8 (0.7, 1.0) <0.005 TAPSE (cm) 2.4 (2.0, 2.7)*†‡ 2.1 (1.6, 2.5)∞ 1.9 (1.5, 2.1)# 1.4 (1.2, 1.9) <0.005 Classical LFLG AS (%) 9 13 0.472 * p < 0.05, Old age control vs Lone AS † p < 0.05, Old age control vs AS-amyloidosis ‡ p < 0.05, Old age control vs Lone amyloidosis § p < 0.05, Lone AS vs AS-amyloidosis ∞ p < 0.05, Lone AS vs Lone amyloidosis # p < 0.05, AS-amyloidosis vs Lone amyloidosis Abstract Figure. AS-amyloidosis compared to other cohorts

Abstract 20041: Single Ventricle Outcome Following Neonatal Palliation of Severe Ebstein’s Anomaly With Modified Starnes Procedure

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
S. Ram Kumar ◽  
Nathan Noh ◽  
Novell Castillo ◽  
Brian Fagan ◽  
Grace Kung ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Nyha Class ◽  
Pacemaker Implantation ◽  
Outcome Data ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

Abstract 2832: Which Cardiac Marker is Most Useful to Predict Final Infarct Size and Cardiac Function Following Primary Percutaneous Coronary Intervention For Acute Myocardial Infarction?

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Stanley Chia ◽  
O. Christopher Raffel ◽  
Faisal Merchant ◽  
Frans J Wackers ◽  
Fred Senatore ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Acute Myocardial Infarction ◽  
Infarct Size ◽  
Troponin I ◽  
Troponin T ◽  
Coronary Intervention ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Primary Pci ◽  
Percutaneous Coronary

Background: Assessment of cardiac biomarker release has been traditionally used to estimate the size of myocardial damage after acute myocardial infarction (AMI). However, the significance of cardiac biomarkers in the setting of primary percutaneous coronary intervention (PCI) has not been systematically studied in a large patient cohort. We evaluated the usefulness of serial and single time-point measures of various cardiac biomarkers (creatine kinase (CK), CK-MB, troponin T and I) in predicting infarct size and left ventricular ejection fraction (LVEF) after primary PCI. Methods: EVOLVE (Evaluation of MCC-135 for Left Ventricular Salvage in AMI) was a randomized double-blind, placebo-controlled trial comparing the efficacy of intracellular calcium modulator as an adjunct to primary PCI in patients with first large AMI. Levels of cardiac biomarkers (CK, CK-MB mass, troponin T and I) were determined in 375 patients at baseline before PCI and 2, 4, 12, 24, 48 and 72 hours thereafter. Single photon emission computed tomography imaging was performed to measure infarct size and LVEF on day 5. Results: Area under curve and peak concentrations of all cardiac markers: CK, CK-MB mass, troponin T and troponin I were significantly correlated with myocardial infarct size and LVEF determined on day 5 (Spearman correlation, all P< 0.001; Table ). Troponin I, however provided the best predictor and a single measure at 72 hr was a strong indicator of both infarct size and LVEF. Using receiver operator characteristics curve, troponin I cutoff value of >55 pg/mL at 72 hr has 90% sensitivity and 70% specificity for detection of large infarct size≥10% ( c =0.88; P< 0.001). Conclusions: Plasma levels of CK, CK-MB, troponin T and troponin I remain useful predictors of infarct size and cardiac function in the era of primary PCI for AMI. A single measurement of circulating troponin I at 72 hours can provide an effective and convenient indicator of infarct size and LVEF in clinical practice. Correlation of cardiac biomarkers with Day 5 SPECT determined infarct size and LVEF

scholarly journals Use of Cardiac Biomarkers for Monitoring Improvement of Left Ventricular Function by Immunoadsorption Treatment in Dilated Cardiomyopathy

Biomolecules ◽  
2019 ◽  
Vol 9 (11) ◽  
pp. 654
Author(s):  
Weinmann ◽  
Werner ◽  
Koenig ◽  
Rottbauer ◽  
Walcher ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Troponin I ◽  
Troponin T ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Lv Function ◽  
Long Term Follow Up ◽  
Acute Changes

Immunoadsorption and subsequent administration of intravenous immunoglobulin (IVIG) have shown beneficial effects on cardiac function and symptoms in patients with dilated cardiomyopathy. Biomarkers play an emerging role in disease monitoring and outcome prediction of heart failure (HF) patients. We aimed to analyze cardiac biomarkers as predictor for improvement of left ventricular (LV) function after immunoadsorption treatment in dilated cardiomyopathy (DCM). Thirty-one patients with dilated cardiomyopathy on optimized HF pharmacotherapy received a single cycle of immunoadsorption for five days followed by IVIG administration. Left ventricular ejection fraction (LVEF) and heart failure biomarkers (hs troponin T, hs troponin I, NT-proBNP and sST2) were evaluated before treatment, after the last cycle of immunoadsorption and during a median follow-up of 30.5 months. We correlated HF biomarkers before immunoadsorption and acute changes of HF biomarkers by immunoadsorption with LV improvement during the long-term follow-up. LV function improved significantly after immunoadsorption from 28.0 to 42.0% during the long-term follow-up (p < 0.0001). Evaluation of biomarker levels showed a significant decrease for hs troponin I (from 9.2 to 5.5 ng/L, p < 0.05) and NT-proBNP (from 789.6 to 281.2 pg/mL, p < 0.005). Correlation of biomarker levels before immunoadsorption and LVEF at the long-term follow-up show good results for hs troponin T (r = −0.40, r2 = 0.16, p < 0.05), hs troponin I (r = −0.41, r2 = 0.17, p < 0.05) and sST2 (r = −0.46, r2 = 0.19, p < 0.05). Correlation of biomarker levels before immunoadsorption and the individual increase in LV function was significant for hs troponin T (r = −0.52, r2 = 0.27, p < 0.005) and hs troponin I (r = −0.53, r2 = 0.29, p < 0.005). To imply a tool for monitoring outcome immediately after immunoadsorption treatment, we investigated the correlation of acute changes of biomarker levels by immunoadsorption treatment and individual increase in LV function. A drop in hs troponin T (r = −0.41, r2 = 0.17, p < 0.05) and hs troponin I (r = −0.53, r2 = 0.28, p < 0.005) levels demonstrate a good correlation to improvement in LVEF during the long-term follow-up. Conclusion: Hs troponin T and I levels correlate with LV function improvement during long-term follow-up. Acute decrease of troponins by immunoadsorption treatment is paralleled by individual improvement of LVEF at the long-term follow-up. Thus, troponins could serve as a monitoring tool for the improvement of LV function after immunoadsorption treatment in dilated cardiomyopathy.

scholarly journals Changes in Cardiac Biomarkers During Doxorubicin Treatment of Pediatric Patients With High-Risk Acute Lymphoblastic Leukemia: Associations With Long-Term Echocardiographic Outcomes

2012 ◽  
Vol 30 (10) ◽  
pp. 1042-1049 ◽  
Author(s):  
Steven E. Lipshultz ◽  
Tracie L. Miller ◽  
Rebecca E. Scully ◽  
Stuart R. Lipsitz ◽  
Nader Rifai ◽  
...  
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
High Risk ◽  
Troponin T ◽  
Lymphoblastic Leukemia ◽  
Cardiac Injury ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Inflammatory Biomarker ◽  
Doxorubicin Group

Purpose Doxorubicin causes cardiac injury and cardiomyopathy in children with acute lymphoblastic leukemia (ALL). Measuring biomarkers during therapy might help individualize treatment by immediately identifying cardiac injury and cardiomyopathy. Patients and Methods Children with high-risk ALL were randomly assigned to receive doxorubicin alone (n = 100; 75 analyzed) or doxorubicin with dexrazoxane (n = 105; 81 analyzed). Echocardiograms and serial serum measurements of cardiac troponin T (cTnT; cardiac injury biomarker), N-terminal pro-brain natriuretic peptide (NT-proBNP; cardiomyopathy biomarker), and high-sensitivity C-reactive protein (hsCRP; inflammatory biomarker) were obtained before, during, and after treatment. Results cTnT levels were increased in 12% of children in the doxorubicin group and in 13% of the doxorubicin-dexrazoxane group before treatment but in 47% and 13%, respectively, after treatment (P = .005). NT-proBNP levels were increased in 89% of children in the doxorubicin group and in 92% of children in the doxorubicin-dexrazoxane group before treatment but in only 48% and 20%, respectively, after treatment (P = .07). The percentage of children with increased hsCRP levels did not differ between groups at any time. In the first 90 days of treatment, detectable increases in cTnT were associated with abnormally reduced left ventricular (LV) mass and LV end-diastolic posterior wall thickness 4 years later (P < .01); increases in NT-proBNP were related to an abnormal LV thickness-to-dimension ratio, suggesting LV remodeling, 4 years later (P = .01). Increases in hsCRP were not associated with any echocardiographic variables. Conclusion cTnT and NT-proBNP may hold promise as biomarkers of cardiotoxicity in children with high-risk ALL. Definitive validation studies are required to fully establish their range of clinical utility.

A correlative study of cardiac biomarkers and left ventricular ejection fraction (LVEF) from N9831, a phase III randomized trial of chemotherapy and trastuzumab as adjuvant therapy for HER2-positive breast cancer

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 579-579 ◽  
Author(s):  
L. A. Kutteh ◽  
T. Hobday ◽  
A. Jaffe ◽  
B. LaPlant ◽  
D. Hillman ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Troponin T ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Phase Iii ◽  
Left Ventricular Ejection ◽  
Tnf Alpha ◽  
Ventricular Ejection Fraction ◽  
Pre Treatment ◽  
Positive Breast Cancer

579 Background: N9831 and other clinical trials have demonstrated the additive survival benefit of trastuzumab (H) therapy with chemotherapy for patients with resected HER-2 positive breast cancer. Cardiac toxicity (CTox) is increased by this strategy. Prediction of or early detection of CTox is important. Methods: 95 patients provided informed consent. Brain natriuretic peptide (BNP), C-reactive protein (CRP), troponin T (TnT) and I (TnI), TNF-alpha, IL-1b, and IL-6 were to be drawn at baseline, after initiation of doxorubicin/cyclophosphamide (AC), after initiation of paclitaxel (T), and after the initiation of H (may have been given concurrently with T). 67 patients with at least a baseline sample, a post-treatment sample, and a baseline and subsequent evaluation of LVEF were studied. No patient in this group had a grade 3 decline in LVEF. Only 3 of these patients did not receive H. Values above the 99th percentile were considered abnormal for troponin. 40 ng/ml was considered elevated for BNP. Results: Values of CRP, TNF-alpha, IL- 6 and IL1b changed in very few patients and without a discernible pattern. For further evaluation of BNP, TnI, and TnT, 2 definitions of CTox were tested: 1. a >10% drop in LVEF from baseline (n=27) and 2. a drop in LVEF of 10–15% to less than the institutional lower limit of normal (LLN) OR a >15% decline in LVEF (n=14). No pre-treatment marker appeared to predict a > 10% decline in LVEF (definition 1). For patients fulfilling definition 2, pre-treatment BNP > 40 was seen in 21% of patients vs 8% of normals (nls) (p= 0.18) and an abnormal TnI (>0.04) was seen in 21% vs 6% of nls (p=0.10). Only doubling of BNP appeared promising as a serial marker of CTox using definition 2 (27% vs 7% for nls (p = 0.09). Conclusions: Baseline BNP and TnI and serial measures of BNP may be promising for further investigation for the prediction and detection of treatment-related CTox. Due to limitations of the study including the small numbers of patients analyzed, the difficulty in defining CTox, and the variation in the timing of sample procurement and assessment, these data are considered exploratory. Supported by CA-25224, CA-2115, CA 38926, CA31946. No significant financial relationships to disclose.

Effectiveness of using cardiac biomarkers to detect late anthracycline cardiotoxicity in childhood leukemia survivors.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 9592-9592
Author(s):  
Beata Mladosievicova ◽  
Dagmar Urbanova ◽  
Eva Radvanska ◽  
Eva Mikuskova ◽  
Iveta Simkova
Keyword(s):  
Childhood Leukemia ◽  
Troponin T ◽  
Combined Therapy ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Left Ventricular Ejection ◽  
Control Group ◽  
Anthracycline Cardiotoxicity ◽  
Ventricular Ejection Fraction ◽  
Group I

9592 Background: Cardiotoxicity is usually detected only when clinical symptoms or progressive cardiac dysfunction has already occurred. Cardiac biomarkers (troponin T and N-terminal fragment brain natriuretic peptide precursor) have been hypothesized to reflect subclinical anthracycline cardiotoxicity earlier than echocardiography. This study aims to assess the effectiveness of using cTNT and NTproBNP in asymptomatic long-term survivors of childhood leukemia treated with and without antracyclines (ANT). Methods: Sixty-nine childhood leukemia survivors 5 - 25 years after completion of therapy were evaluated with immunochemical analysis of cTnT and NT-proBNP and echocardiography. Patients from group I (n = 36) received combined therapy with anthracyclines (ANT) with total cumulative dose 95-600 (median 221) mg/m2, patients from group II (n = 33) received therapy without anthracyclines (nonANT). Control group consisted from 44 age- and gender-matched apparently healthy subjects. Results: Levels of NTproBNP were significantly higher in ANT group than in controls (median 51,52 vs 17,37 pg/ml; p=0.0026). Patients treated with ANT had significantly increased median values of NTproBNP compared with patients in non ANT group (51,52 vs 12,24 pg/ml; p=0.0002). CTnT levels remained below the diagnostic cut-off levels in all groups. No patient had echocardiographic abnormalities, but significant differences were found in mean values of left ventricular ejection fraction and deceleration time between patients treated with and without ANT. Conclusions: Assessment of plasma NTproBNP concentrations may be an effective tool for detection of late subclinical cardiac damage in survivors of childhood leukemia previously treated with low ANT doses. Higher NTproBNP levels in patients after ANT therapy might reflect an initial stage of cardiotoxicity before the development of echocardiographic abnormalities. This study was supported by a grant from Ministry of Health 2007/42-UK-18, Slovakia.

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 Understanding Pacing-Induced Cardiomyopathy: a mini review

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Sidhi Laksono Purwowiyoto ◽  
Reynaldo Halomoan Siregar ◽  
Steven Philip Surya
Keyword(s):  
Cardiac Function ◽  
Right Ventricular ◽  
Sinus Node ◽  
Pacemaker Implantation ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Preventive Strategy ◽  
Ventricular Pacing ◽  
Ventricular Ejection Fraction ◽  
Right Ventricular Pacing

Patients with total atrioventricular block or sinus node dysfunction will need pacemaker implantation to improve the physiologic function of the heart.  It is known that chronic pacing such as right ventricular pacing could deteriorate the cardiac function (decreased left ventricular ejection fraction) due to dyssynchrony. This condition is knows as pacing-induced cardiomyopathy (PICM). The incidence of PICM could reach 19.5% during 3 years follow-up. The right ventricle is one of the locations for implantation. Chronic right ventricular pacing may cause interventricular dyssynchrony and disrupt the contraction mechanism in the heart. These will lead to cardiac remodeling and eventually impair the left ventricular function. Therapy is needed in patients with PICM to improve the symptoms and maintain the cardiac function. This article will further highlight the definition, mechanism, risk factor, treatment and preventive strategy for patients with PICM.

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