Progressive electrocardiographic changes in parallel with cardiac magnetic resonance findings in fabry disease

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Figliozzi ◽  
A Camporeale ◽  
M Pieroni ◽  
F Pieruzzi ◽  
M Namdar ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Fabry Disease ◽  
Cardiac Involvement ◽  
P Wave ◽  
Funding Source ◽  
Ecg Changes ◽  
Bundle Branch Blocks ◽  
Group A ◽  
Group B

Abstract Background Cardiac Magnetic Resonance (CMR) allows to detect progressive stages of cardiac involvement in Fabry Disease (FD). A systematic description of electrocardiographic (ECG) alterations occurring in FD is currently missing. Purpose To explore ECG changes in progressive stages of FD cardiomyopathy. Methods 71 FD patients and 17 healthy controls underwent CMR with T1 mapping and 12-lead ECG. ECG analysis included the duration of the P-wave and the interval between the end of P-wave and the beginning of QRS (PendQ). FD patients in the test cohort were divided into 3 groups with increasing severity of cardiac involvement: A) normal T1, no LVH; B) low T1, no LVH; C) low T1, LVH. Results An increase of Pwave/PendQ ratio was observed in Group A compared to Controls (1.08 vs. 0.75, p<0.0001). Higher Pwave/PendQ ratio (1.50 vs. 1.08, p<0.0001), shorter PQc interval (127.9 vs. 159.5, p=0.0007), increased Sokolow-Lyon Index (SLI) (3.2 vs. 2.4, p<0,001) and T wave amplitude (0.6 vs. 0.4 mV, p=0.002) characterized Group B in comparison with Group A. A higher prevalence of left bundle branch blocks (13.6% vs. 0%, p=0.03) and repolarization abnormalities (77.3% vs. 5.7%), wider QRS (120 vs 95 msec, p<0.0001) and QT (460 vs 400 msec, p=0.003) intervals were found in Group C compared to Group B. SLI (AUC 0.769), Pwave/PendQ (AUC 0.778), QRS (AUC 0.703) and QT (AUC 0.769) durations resulted to be independent predictors of low T1 values on CMR at stepwise multivariate analysis. Conclusion FD is characterized by progressive ECG changes. The identification of ECG parameters able to predict a lowering of myocardial T1 values on CMR may promote early detection of cardiac involvement, helping to target the therapeutic approach. Progressive ECG and CMR changes in FD Funding Acknowledgement Type of funding source: Other. Main funding source(s): This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

scholarly journals POS0886 COULD BE INTERSTITIAL MYOCARDITIS A FEATURE OF THE ANTISYNTHETASE SYNDROME?

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 699.1-699
Author(s):  
A. Gil-Vila ◽  
G. Burcet ◽  
A. Anton-Vicente ◽  
D. Gonzalez-Sans ◽  
A. Nuñez-Conde ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Late Gadolinium Enhancement ◽  
Magnetic Resonance ◽  
Interstitial Lung Disease ◽  
Cardiac Involvement ◽  
T2 Mapping ◽  
Extracellular Volume ◽  
Interstitial Tissue ◽  
Antisynthetase Syndrome ◽  
Gadolinium Enhancement

Background:Antisynthetase syndrome (ASS) is characterized by inflammatory myopathy, interstitial lung disease, arthritis, mechanical hands and Raynaud phenomenon, among other features. Recent studies have shown that idiopathic inflammatory myopathies (IIM) may develop cardiac involvement, either ischemic (coronary artery disease) or inflammatory (myocarditis). We wonder if characteristic lung interstitial involvement (interstitial lung disease) that appears in patients with the ASS may also affect the myocardial interstitial tissue. New magnetic resonance mapping techniques could detect subclinical myocardial involvement, mainly as edema (increase extracellular volume in interstitium and extracellular matrix), even in the absence of visible late Gadolinium enhancement (LGE).Objectives:Our aim was to describe the presence of interstitial myocarditis in a group of patients with ASS.Methods:Cross-sectional, observational study performed in a tertiary care center. We included 13 patients diagnosed with ASS (7 male, 53%, mean (SD) age at diagnosis 56,8 years (±11,8)). The patients were consecutively selected from our outpatient myositis clinic. Myositis specific and associated antibodies were performed by means of line immunoblot (EUROIMMUN©). Cardiac magnetic resonance (CMR) was performed on all patients. The study protocol includes functional cine magnetic resonance and standard late gadolinium enhancement (LGE), as well as novel parametric T1 and T2 mapping sequences (modified look locker inversion recovery sequences - MOLLI) with extracellular volume (ECV) calculation 20 minutes after the injection of a gadolinium-based contrast material.Results:CMR could not be performed in one patient due to anxiety. All patients studied (12) had a normal biventricular function, without alteration of segmental contraction. A third (4 out of 12, 33%) of the studied patients showed elevated T2 myocardial values without focal LGE, half of them (2/4) with an elevated ECV, consistent with myocardial edema. Two patients with normal T2 values showed unspecific LGE focal patterns, one in the right ventricle union points and another with mild interventricular septum enhancement (Figure 1). None of the patients studied refer any cardiac symptomatology. All the four patients with T2 mapping alterations (100%) had interstitial lung involvement, but only 4 out of 8 (50%) of the rest ASS patients without T2 mapping positivity. The autoimmune profile was as follows: 10 anti-Jo1/Ro52, 1 anti-EJ/Ro52, 2 anti-PL12.Conclusion:Myocarditis, although subclinical, appears to be a feature in ASS patients. T1 and T2 mapping sequences might be valuable to detect and monitor subclinical cardiac involvement in these patients. The possibility that the same etiopathogenic mechanism may be involved in the interstitial tissue in lung and myocardium is raised. More studies must be done in order to assert the prevalence of myocarditis in ASS.References:[1]Dieval C et al. Myocarditis in Patients With Antisynthetase Syndrome: Prevalence, Presentation, and Outcomes. Medicine (Baltimore). 2015 Jul;94(26):e798.[2]Myhr KA, Pecini R. Management of Myocarditis in Myositis: Diagnosis and Treatment. Curr Rheumatol Rep. 2020 Jul 22; 22:49.[3]Sharma K, Orbai AM, Desai D, Cingolani OH, Halushka MK, Christopher-Stine L, Mammen AL, Wu KC, Zakaria S. Brief report: antisynthetase syndrome-associated myocarditis. J Card Fail. 2014 Dec;20(12):939-45.Figure 1.Cardiac magnetic resonance images from ASS patients.Disclosure of Interests:None declared

scholarly journals Proteoglycan remodeling is accelerated in women with angina pectoris and diffuse myocardial fibrosis: the iPOWER Study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N.D Mygind ◽  
S Holm Nielsen ◽  
M Mide Michelsen ◽  
A Pena ◽  
D Bechsgaard Frestad ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Angina Pectoris ◽  
Myocardial Fibrosis ◽  
T1 Mapping ◽  
Obstructive Coronary Artery Disease ◽  
Extracellular Volume ◽  
Diffuse Myocardial Fibrosis ◽  
Public Institution ◽  
Funding Source

Abstract Background Women with angina and no obstructive coronary artery disease (CAD) have an unfavourable prognosis, possibly due to coronary microvascular disease and diffuse myocardial fibrosis (DMF). In DMF myocardial extracellular matrix (ECM) proteins are actively remodeled by matrix metalloproteinase (MMP). Purpose We investigated MMP-mediated degradation of the protegoglycans biglycan and versican in women with angina pectoris and possible DMF assessed by cardiac magnetic resonance T1 mapping. Methods Seventy-one women with angina pectoris and no obstructive CAD were included. Asymptomatic age-matched women served as controls (n=32). Versican and biglycan were measured in serum by specific competitive enzyme-linked immunosorbent assays. T1 mapping was performed by cardiac magnetic resonance with gadolinium measuring T1 and extracellular volume (ECV). Results Both biglycan and versican levels were higher in symptomatic women compared with controls; 31.4 ng/mL vs. 16.4 ng/mL (p<0.001) and 2.1 ng/mL vs. 1.8 ng/mL (p<0.001), respectively (Figure 1) and were moderately correlated to global ECV (r2=0.38, p<0.001 and r2=0.26, p=0.015 respectively). Conclusion Turnover of biglycan and versican was increased in symptomatic compared to asymptomatic women and associated to ECV, supporting a link between angina with no obstructive CAD and fibrotic cardiac remodeling. The examined biomarkers may prove to be suitable for monitoring active ECM remodeling. Figure 1. Levels of BGM and VCANM Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): This work was supported by The Danish Heart Foundation, the Danish Research Fund (Den Danske Forskningsfond) and by University of Copenhagen.

Proposed stages of Fabry disease: insights from multiparametric cardiac MRI and advanced ECG

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
JA Bicho Augusto ◽  
N Johner ◽  
D Shah ◽  
S Nordin ◽  
K Knott ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Cardiac Mri ◽  
Cardiac Involvement ◽  
T2 Mapping ◽  
Left Ventricular ◽  
P Wave ◽  
Stress Perfusion ◽  
Healthy Controls ◽  
T Wave ◽  
Advanced Analysis

Abstract Funding Acknowledgements Type of funding sources: None. Background Staging of Fabry disease (FD) cardiomyopathy uses multiparametric cardiac MRI. Advanced disease is characterized by left ventricular hypertrophy (LVH), myocardial inflammation/oedema (high native T2 mapping) and/or fibrosis (late gadolinium enhancement, LGE). Pre-LVH involvement has been described and includes myocardial sphingolipid storage (low native T1 mapping), impaired LV global longitudinal strain (GLS) and microvascular disease/dysfunction (low stress myocardial blood flow, MBF, in perfusion mapping). We aimed to define (1) the early myocardial phenotype prior to T1 lowering/pre-storage and (2) the stages of cardiac involvement in FD.   Methods FD patients and age, sex and heart rate matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, GLS, pre-contrast T1 and T2 mapping, adenosine stress perfusion mapping [for MBF] and LGE). Results 114 Fabry patients (46 ± 13 years, 61% female, 37% [n = 72] had LVH) and 76 controls (49 ± 15 years, 50% female) were included. FD with vs without LVH in brief and as expected, FD with LVH had significantly (p < 0.05) lower MBF, GLS and T1, and higher T2 and %LGE. FD pre-LVH low T1 vs pre-LVH normal T1: low T1 patients (32/72, 44%) had higher LV mass index (67 ± 14 vs 59 ± 10g/m2, P = 0.011), maximum Q wave amplitude (2[1-2] vs 1[1-2]mm, P < 0.001), Sokolow-Lyon index (22[16-28] vs 17[13-23]mm, P = 0.031) and more fractionated QRS complexes (44 vs 18%, P = 0.020). FD pre-LVH normal T1 vs healthy controls: normal T1 pre-LVH Fabry patients (40/72, 56%) had reduced GLS (-18 ± 2 vs -20 ± 2%, P < 0.001), microvascular impairment (lower MBF 2.5 ± 0.7 vs 3.0 ± 0.8mL/g/min, P = 0.028), subtle T2 elevation (50 ± 4 vs 48 ± 2ms, p = 0.027) and limited LGE (%LGE 0.3 ± 1.1 vs 0%, P = 0.004) when compared to healthy controls; ECG abnormalities included shorter P wave duration (88 ± 12 vs 94 ± 15ms, P = 0.010) and T wave peak time (Tonset–Tpeak; 104 ± 28 vs 115 ± 20ms, P = 0.015), resulting in a more symmetric T wave with lower T wave time ratio (Tonset–Tpeak)/(Tpeak–Tend) (1.5 ± 0.4 vs 1.8 ± 0.4, P < 0.001) compared to controls. Conclusion Prior staging of Fabry cardiomyopathy included a pre-LVH stage (accumulation/storage) and two LVH stages (hypertrophy and inflammation; fibrosis and impairment). Here we define an even earlier stage, pre-LVH pre-detectable storage, defined by microvascular dysfunction, impaired GLS and altered atrial depolarization and ventricular repolarization intervals (see Figure). Abstract Figure. Proposed stages of cardiac involvement

Cardiac magnetic resonance in patients with muscular dystrophies

2020 ◽  
pp. 204748732092305 ◽  
Author(s):  
Chrysanthos Grigoratos ◽  
Alberto Aimo ◽  
Andrea Barison ◽  
Vincenzo Castiglione ◽  
Giancarlo Todiere ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
Cardiac Involvement ◽  
Prognostic Significance ◽  
Standard Technique ◽  
Muscle Disease ◽  
Added Value ◽  
Muscular Dystrophies ◽  
Inherited Disorders

Muscular dystrophies are inherited disorders sharing similar clinical features and dystrophic changes on muscle biopsy. Duchenne muscular dystrophy is the most common inherited muscle disease of childhood, and Becker muscular dystrophy is a milder allelic variant with a slightly lower prevalence. Myotonic dystrophy is the most frequent form in adults. Cardiac magnetic resonance is the gold standard technique for the quantification of cardiac chamber volumes and function, and also enables a characterisation of myocardial tissue. Most cardiac magnetic resonance studies in the setting of muscular dystrophy were carried out at single centres, evaluated small numbers of patients and used widely heterogeneous protocols. Even more importantly, those studies analysed more or less extensively the patterns of cardiac involvement, but usually did not try to establish the added value of cardiac magnetic resonance to standard echocardiography, the evolution of cardiac disease over time and the prognostic significance of cardiac magnetic resonance findings. As a result, the large and heterogeneous amount of information on cardiac involvement in muscular dystrophies cannot easily be translated into recommendations on the optimal use of cardiac magnetic resonance. In this review, whose targets are cardiologists and neurologists who manage patients with muscular dystrophy, we try to summarise cardiac magnetic resonance findings in patients with muscular dystrophy, and the results of studies evaluating the role of cardiac magnetic resonance as a tool for diagnosis, risk stratification and follow-up. Finally, we provide some practical recommendations about the need and timing of cardiac magnetic resonance examination for the management of patients with muscular dystrophy.

scholarly journals Prolonged Sinus Pauses upon Termination of Paroxysmal Atrial Fibrillation: Abnormal Right Atrial Electrophysiologic and Electroanatomic Findings

2017 ◽  
Vol 44 (2) ◽  
pp. 107-114 ◽  
Author(s):  
Zhengyu Bao ◽  
Hongwu Chen ◽  
Bing Yang ◽  
Michael Shehata ◽  
Weizhu Ju ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Sinus Node ◽  
P Wave ◽  
Right Atrial ◽  
Control Group ◽  
Paroxysmal Supraventricular Tachycardia ◽  
Group A ◽  
Group B ◽  
Activation Times ◽  
The Right

The efficacy of pulmonary vein antral isolation for patients with prolonged sinus pauses (PSP) on termination of atrial fibrillation has been reported. We studied the right atrial (RA) electrophysiologic and electroanatomic characteristics in such patients. Forty patients underwent electroanatomic mapping of the RA: 13 had PSP (group A), 13 had no PSP (group B), and 14 had paroxysmal supraventricular tachycardia (control group C). Group A had longer P-wave durations in lead II than did groups B and C (115.5 ± 15.4 vs 99.5 ± 10.9 vs 96.5 ± 10.4 ms; P=0.001), and RA activation times (106.8 ± 13.8 vs 99 ± 8.7 vs 94.5 ± 9.1 s; P=0.02). Group A's PP intervals were longer during adenosine triphosphate testing before ablation (4.6 ± 2.3 vs 1.7 ± 0.6 vs 1.5 ± 1 s; P <0.001) and after ablation (4.7 ± 2.5 vs 2.2 ± 1.4 vs 1.6 ± 0.8 s; P <0.001), and group A had more complex electrograms (11.4% ± 5.4% vs 9.3% ± 1.6% vs 5.8% ± 1.6%; P <0.001). Compared with group C, group A had significantly longer corrected sinus node recovery times at a 400-ms pacing cycle length after ablation, larger RA volumes (100.1 ± 23.1 vs 83 ± 22.1 mL; P=0.04), and lower conduction velocities in the high posterior (0.87 ± 0.13 vs 1.02 ± 0.21 mm/ms; P=0.02) and high lateral RA (0.89 ± 0.2 vs 1.1 ± 0.35 mm/ms; P=0.04). We found that patients with PSP upon termination of atrial fibrillation have RA electrophysiologic and electroanatomic abnormalities that warrant post-ablation monitoring.

scholarly journals Rare Cardiac Mass Assessed by Cardiac Magnetic Resonance

2019 ◽  
Vol 16 (6) ◽  
pp. 77-79
Author(s):  
Sebastian Militaru ◽  
Bernhard Gerber
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Cardiac Involvement ◽  
Specific Treatment ◽  
Cardiac Mass ◽  
Cardiac Complications ◽  
Erdheim Chester Disease ◽  
The Right ◽  
Erdheim Chester ◽  
Chester Disease

AbstractA 43 year old male was referred to our center for assessment of the cardiac involvement in Erdheim-Chester disease (EHD) by cardiac magnetic resonance (CMR). The patient presented with a history of bone involvement as well as retroperitoneal mass, demonstrated to consist fibrosis as well as histiocyte infiltration.The CMR examination included cine (SSFP – steady state free procession), T1 weighted (T1w) and T2 weighted (T2w) sequences, as well as late enhancement images 10 minutes after gadolinium based contrast injection (0.2 mmol/kg). The acquired images showed normal dimensions and function for both right and left cardiac chambers. However, a cardiac mass was revealed in the free wall of the right atrium and the junction with the right ventricle, with clear borders and a diameter of 2.5 cm (Image 1). The tissue was best viewed on axial sequences and was isointense on cine, T1w and T2w images and was mildly enhanced on LGE images. Consequently, the diagnosis of cardiac involvement in EHD was confirmed. The patient was started on specific treatment for EHD and 3-year CMR follow-up showed regression of cardiac involvement.Erdheim-Chester disease is a rare disorder most frequently characterized by non-Langerhans histiocytic multifocal osteosclerotic lesions, with multisystemic granulomatosis and widespread manifestations, as well as highly variable severity(1). ECD affects the cardiovascular system in 75% of patients with infiltration of the pericardium and the right atrioventricular septum being the most common presentation. Typically the mass appears isointense on T1 and T2 weighted images and has low contrast enhancement, as was the case in our patient. In approximately 60% of cases death occurs because of cardiac complications, like pericardial tamponade, myocardial infarction, cardiomyopathy or arrhythmias(2). Patients may sometimes be successfully treated with biologic therapy, interferon alpha or radiotherapy.

scholarly journals Incidence of post-operative cardiac complications in cardiac patients undergoing urological procedures

2015 ◽  
Vol 1 (2) ◽  
pp. 80
Author(s):  
Mastan Saheb Shaik ◽  
Venkata Rama Rao M ◽  
Sailaja K
Keyword(s):  
General Anaesthesia ◽  
Regional Anaesthesia ◽  
Cardiac Complications ◽  
Anesthetic Techniques ◽  
Ecg Changes ◽  
Arterial Blood ◽  
Group A ◽  
Urological Procedures ◽  
The Mean ◽  
Group B

Context: There are limited studies indicating the incideance of post operative morbidity and mortality in high risk patients. Urological procedures constitute a major portion of geriatric surgeries where a cardiac obligation is almost inevitable and selected for this study purpose.Aims: To compare the incidence of post operative cardiovascular complications during regional and general anesthetic techniques in patients with known cardiac risk undergoing urological surgeriesSettings and Design: Prospective randomized double blind study.Methods and Material: 40 patients aged above 50 years posted for elective urological surgeries were enrolled in the study after obtaining approval from hospital ethics committee and written informed consent from the patients. Patients were divided into two groups A& B. Group A (n=20) received general and group B (n=20) received regional anaesthesia (spinal/ Epidural). All the patients received standard premedication and their basal vitals( BP, HR, ECG pattern) were recorded. The same parameters were monitored in the post operative period at regular intervals.Statistical analysis used: Student T test is used to test the significance of stastical difference in the variables between the two groups.Results: The mean heart rate and the mean arterial blood pressure were increased (P<0.01) in the general anaesthesia group. Group A showed 10% incidence of ECG changes and group B showed 35% incidence of ECG changes. But the changes in the hemodynamics were not significant.Conclusions: There is no difference between regional anaesthesia and general anaesthesia regarding the post operative outcome after urological procedures with respective hemodynamic changes.

scholarly journals Cardiac involvement in hypereosinophilic syndromes detected by cardiac magnetic resonance imaging

2013 ◽  
Vol 34 (suppl 1) ◽  
pp. 3501-3501
Author(s):  
C. Merten ◽  
H. W. Beurich ◽  
D. Zachow ◽  
M. Abdel-Wahab ◽  
F. Arndt ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Cardiac Magnetic Resonance Imaging ◽  
Cardiac Involvement ◽  
Resonance Imaging
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