scholarly journals Cardiac Arrhythmias in Muscular Dystrophies Associated with Emerinopathy and Laminopathy: A Cohort Study

2021 ◽  
Vol 10 (4) ◽  
pp. 732
Author(s):  
Michał Marchel ◽  
Agnieszka Madej-Pilarczyk ◽  
Agata Tymińska ◽  
Roman Steckiewicz ◽  
Ewa Ostrowska ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Cardiac Arrhythmias ◽  
Ventricular Arrhythmias ◽  
Cardiac Involvement ◽  
Electrocardiographic Monitoring ◽  
Life Threatening ◽  
Electrocardiogram Ecg ◽  
Surface Electrocardiogram ◽  
Implanted Device

Introduction: Cardiac involvement in patients with muscular dystrophy associated with Lamin A/C mutations (LMNA) is characterized by atrioventricular conduction abnormalities and life-threatening cardiac arrhythmias. Little is known about cardiac involvement in patients with emerin mutation (EMD). The aim of our study was to describe and compare the prevalence and time distribution of cardiac arrhythmias at extended follow-up. Patients and methods: 45 consecutive patients affected by muscular dystrophy associated to laminopathy or emerinopathy were examined. All patients underwent clinical evaluation, 12-lead surface electrocardiogram (ECG), 24 h electrocardiographic monitoring, and cardiac implanted device interrogation. Results: At the end of 11 (5.0–16.6) years of follow-up, 89% of the patients showed cardiac arrhythmias. The most prevalent was atrial standstill (AS) (31%), followed by atrial fibrillation/flutter (AF/Afl) (29%) and ventricular tachycardia (22%). EMD patients presented more frequently AF/AFl compared to LMNA (50% vs. 20%, p = 0.06). Half of the EMD patients presented with AS, whilst there was no occurrence of such in the LMNA (p = 0.001). Ventricular arrhythmias were found in 60% of patients with laminopathy compared to 3% in patients with emerinopathy (p < 0.001). The age of AVB occurrence was higher in the LMNA group (32.8 +/− 10.6 vs. 25.1 +/− 9.1, p = 0.03). Conclusions: Atrial arrhythmias are common findings in patients with muscular dystrophy associated with EMD/LMNA mutations; however, they occurred earlier in EMD patients. Ventricular arrhythmias were very common (60%) in LMNA and occurred definitely earlier compared to the EMD group.

scholarly journals Follow up study of cardiac involvement in Emery-Dreifuss muscular dystrophy.

1989 ◽  
Vol 64 (5) ◽  
pp. 713-715 ◽  
Author(s):  
M Yoshioka ◽  
K Saida ◽  
Y Itagaki ◽  
T Kamiya
Keyword(s):  
Muscular Dystrophy ◽  
Cardiac Involvement ◽  
Follow Up Study

1175Structural progression increases the risk of ventricular arrhythmias in patients with arrhythmogenic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Chivulescu ◽  
Ø.H Lie ◽  
H Skulstad ◽  
B A Popescu ◽  
R O Jurcut ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Cardiomyopathy ◽  
Cardioverter Defibrillator ◽  
Structural Progression ◽  
Life Threatening ◽  
Arrhythmic Event ◽  
History Of ◽  
Structural Disease

Abstract Background Arrhythmogenic cardiomyopathy (AC) is an inheritable cardiomyopathy with incomplete penetrance, variable phenotype severity and poorly described disease progression. It is characterized by high risk of life-threatening ventricular arrhythmias and sudden cardiac death in young individuals. Risk stratification and selection of patients presenting without history of life-threatening arrhythmic events for cardioverter-defibrillator implantation in primary prevention remains challenging. Purpose We aimed to assess the impact of disease progression on arrhythmic outcomes in AC patients. Methods We included consecutive AC probands and mutation-positive family members with at least one complete follow-up evaluation. Echocardiographic and electrical parameters were defined according to the 2010 Revised Task Force criteria at inclusion and at last follow-up. Structural progression was defined as development of new echocardiographic diagnostic criteria. Electrical progression was defined as the development of new diagnostic depolarization, repolarization and/or premature ventricular complex count criteria during follow-up. Non-sustained ventricular tachycardia or ventricular tachycardia occurring during follow-up defined incident ventricular arrhythmic events. Results We included a total of 144 patients (48% female, 47% probands, 40±16 years old). At inclusion, 54 patients (37%) had a history of arrhythmic events, 30 patients (21%) had overt structural disease and 114 (79%) had no or minor structural disease. During 7.0 (IQR: 4.5 to 9.4) years of follow-up, 49 patients (43%) with no or minor structural disease at inclusion developed new structural criteria being defined as progressors. Among 80 participants with no or minor structural disease and no arrhythmic history at inclusion, a first arrhythmic event occurred in 14 (17%). The incidence of arrhythmic events was higher in progressors (11/27, 41%) than in non-progressors (3/53, 6%) (p<0.001) (Figure). Structural progression was associated with higher risk of first arrhythmic events during follow-up when adjusted for sex, age at inclusion and follow-up duration, independent of electrical progression (7.6, 95% CI [1.5, 37.2], P=0.01). Incident arrhythmic events distribution Conclusion Almost half of patients without overt structural cardiac disease at genetic diagnosis develop new structural criteria during 7 years follow-up and 17% experienced their first ventricular arrhythmic event. Structural progression was independently associated with ventricular arrhythmic events during follow-up. These findings highlight the increased risk of arrhythmias when structural abnormalities are detected. Their finding may initiate the evaluation for primary prevention cardioverter-defibrillator implantation.

scholarly journals Swallow-induced syncope in 5 patients

2017 ◽  
Vol 7 (4) ◽  
pp. 316-323 ◽  
Author(s):  
Ibrahim Aydogdu ◽  
Can Hasdemir ◽  
Ahmet Acarer ◽  
Sezin Alpaydin ◽  
Cumhur Ertekin
Keyword(s):  
Standing Position ◽  
Permanent Pacemaker ◽  
Diagnosis And Treatment ◽  
Afferent Pathways ◽  
Electrocardiographic Monitoring ◽  
Threatening Condition ◽  
Pacemaker Placement ◽  
Life Threatening

AbstractBackground:We sought to characterize a cohort of participants with swallow-induced syncope (SIS) with clinical and electrophysiologic evaluations.Methods:Using electrocardiographic monitoring and neurophysiologic methods of swallowing, we evaluated a cohort of 5 patients with SIS, 4 of whom had longitudinal follow-up.Results:We determined electrophysiologically that the duration between the onset of swallow and a bradyarrhythmia or asystole is extremely short (2–3 seconds) in SIS. Most participants with SIS do not have a neurologic or esophageal disorder. SIS can occur with different food types, in sitting or standing position, and has varying frequency in different participants. Permanent pacemaker placement is a curative measure in SIS.Conclusions:Our findings suggest that SIS is elicited by reflex afferent pathways originating in the oropharynx, rather than an esophageal origin, as previously proposed. Our longitudinally followed cohort with detailed clinical and electrophysiologic characterization should aid the clinician in the diagnosis and treatment of this potentially life-threatening condition.

Prognostic value of non-ischemic ring-like left ventricular scar pattern in patients with apparently idiopathic ventricular arrhythmias: a CMR imaging study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Nucifora ◽  
D Muser ◽  
S Castro ◽  
R Casado Arroyo ◽  
D Benhayon ◽  
...  
Keyword(s):  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Imaging Study ◽  
Left Ventricular ◽  
High Rate ◽  
Life Threatening ◽  
History Of ◽  
Group A ◽  
Group B

Abstract Background The presence of left ventricular (LV) late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) has been correlated to life-threatening arrhythmic events in patients with apparently idiopathic ventricular arrhythmias (VAs). Aim of the present study was to investigate the prognostic significance of a specific LV-LGE phenotype characterized by a subepicardial/midmyocardial “ring-like” pattern of fibrosis. Methods Out of a total of 518 consecutive patients with apparently idiopathic VAs who underwent CMR study, 79 (15%) had evidence of LV-LGE. Of these, 23 (4%) patients had LV LGE with ring-like pattern, defined as subepicardial or midmyocardial LGE involving at least 3 contiguous segments in the same slice (group A), while 56 (11%) patients had LV LGE with no ring-like pattern (group B). The remaining 439 patients had no LGE (group C). The end-point of the study was a composite SCD, resuscitated cardiac arrest and nonfatal episodes of ventricular fibrillation or documented sustained ventricular tachycardia. Results Group A patients were more frequently males compared to groups B and C (96% vs. 79% vs. 52%; p&lt;0.01) and had more frequently a family history of SCD and/or cardiomyopathy (30% vs. 11% vs. 5%; p&lt;0.01). All patients in Group A showed VAs with a predominant RBBB morphology vs. 38 (68%) patients in Group B and 65 (15%) in Group C (p&lt;0.01). During a follow-up of 63±39 months, the composite outcome occurred in 13 patients (57%) in Group A vs. 11 (20%) in Group B and 2 (1%) in Group C (p&lt;0.01). Conclusion In patients with apparently idiopathic VAs, a nonischemic LV-LGE with a ring-like pattern at CMR is associated with a high rate of malignant arrhythmic events during follow-up. Funding Acknowledgement Type of funding source: None

scholarly journals RECURRENT SYNCOPE AS A SYMPTOM OF ELECTRICAL STORM – CASE PRESENTATION

2021 ◽  
Vol 8 (4) ◽  
pp. 271-273
Author(s):  
Przemysław Skoczyński ◽  
Paweł Pochciał
Keyword(s):  
Ventricular Arrhythmias ◽  
Electrical Storm ◽  
Monitoring Equipment ◽  
Case Presentation ◽  
Diffi Cult ◽  
Patient Reported ◽  
Cardiology Clinic ◽  
Life Threatening ◽  
History Of

Dangerous ventricular arrhythmias leading to sudden cardiac death (SCD) are some of the most diffi-cult diagnostic challenges. They are often mildly symptomatic. Their often self-limiting nature means that they are difficult to capture on ECG. A 75-year old woman with chronic heart failure due to nonis-chemic cardiomyopathy reported to the cardiology clinic for a scheduled routine follow-up of the ICD, implanted three years prior as primary prevention of SCD. The patient reported recent episodes of sud-den weakness and described the episodes as hypotension. The patient associated it with too aggressive treatment of arterial hypertension. During the visit the patient experienced one of these episodes that she had previously described. The monitoring equipment in the clinic revealed ventricular tachycardia (VT). The history of the implanted ICD revealed many similar previous episodes including 5 episodes in the last 24 hours which led to a diagnosis of electrical storm. Dangerous ventricular arrhythmias may be mildly symptomatic and they are often underestimated by the patient. Fainting, especially in situa-tions unusual for the vasovagal reflex or orthostatic hypotension, should always arouse vigilance to-wards life-threatening ventricular arrhythmia.

scholarly journals Reversible intraventricular conduction defect in aconite poisoning: A Case report

2017 ◽  
Vol 13 (1) ◽  
pp. 108-111
Author(s):  
Ajay Kumar Yadav ◽  
S Chaudhuri ◽  
PP Gupta ◽  
R Chaudhary ◽  
BD Aryal ◽  
...  
Keyword(s):  
Cardiac Arrhythmias ◽  
Ventricular Arrhythmias ◽  
Therapeutic Index ◽  
Wild Plant ◽  
Accidental Ingestion ◽  
Narrow Therapeutic Index ◽  
Intraventricular Conduction ◽  
Life Threatening ◽  
Conduction Defect ◽  
Clinical Problems

Aconitine and related alkaloids found in the Aconitum species are highly cardiotoxic and neurotoxic. The wild plant (especially the roots and root tubers) is extremely toxic. Severe aconite poisoning can occur after accidental ingestion of the wild plant or consumption of an herbal made from aconite roots. In traditional Chinese medicine, aconite roots are used only after processing to reduce the toxic alkaloid content. Soaking and boiling during processing will hydrolyze aconite alkaloids into less toxic and non-toxic derivatives. However, the use of a larger than recommended dose and inadequate processing increases the risk of poisoning. The aconite herb is one of the commonly-prescribed ingredients for various clinical problems. However, due to its narrow therapeutic index, toxicities are not uncommonly encountered, including life-threatening cardiac arrhythmias like ventricular arrhythmias Health Renaissance 2015; 13 (1): 108-111

scholarly journals Frequency of ventricular tachyarrhythmias in patients with different risk scores for sudden cardiac death in hypertrophic cardiomyopathy and cardioverter defibrilators implanted in primary prevention

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
N Brankovic ◽  
NN Radovanovic ◽  
N Vujadinovic ◽  
B Kircanski ◽  
V Sajic ◽  
...  
Keyword(s):  
Primary Prevention ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Risk Scores ◽  
Icd Implantation ◽  
Life Threatening ◽  
Group A ◽  
Group B

Abstract Funding Acknowledgements Type of funding sources: None. Background The European Society of Cardiology’s (ESC) risk calculator for sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathies (HCM) is currently a tool that facilitates the decision to implant a cardioverter defibrillator (ICD) in primary prevention, but doubts still exist in everyday clinical practice. Purpose We aimed to determine the incidence of life-threatening ventricular arrhythmias during long-term follow-up in patients with different ESC HCM risk scores for SCD calculated on ICD implantation in primary prevention. Methods This retrospective, observational study was conducted in a tertiary center among adult patients with HCM and ICD devices (ICD-VR, ICD-DR and CRT-ICD) implanted in primary prevention from January 2008 to December 2019. The patients followed up for less than one year were excluded from the analysis. Study group was divided into three subgroups, according to the ESC 5 years risk score of SCD and based on it the estimated need for ICD implantation: group A with risk &lt; 4%, where ICD is generally not indicated; group B with risk 4-6%, where ICD may be considered; group C with risk ≥6%, where ICD should be considered. Regularly, ICD was interrogated twice a year, while emergency controls were performed after delivering of ICD therapy. Follow-up data including information about sustained ventricular tachicardia (VT) and ventricular fibrillation (VF) episodes were collected from patients medical records. Results In the observed period, ICD devices were implanted in 60 adult patients with HCM. Ten of them had history of sustained ventricular arrhythmias and 3 were lost to follow-up. Therefore, we analysed 47 patients (55.3% male, mean age 51.0 ± 15.7 years), 13 from group A, 12 from group B and 22 from group C. Mean follow-up period was 80.6 ± 37.9 months. During the follow-up period ventricular tachyarrhythmias (VT and VF, either in monitoring or therapy zone) were recorded in one patient from group A (7.7%), in two patients from group B (16.7%) and in 7 patients from group C (31.8%). Using Kruskal-Wallis analysis of variance, we did not find a statistically significant difference in the occurrence of ventricular tachyarrhythmias among the observed groups (p = 0.225). Conclusions Patients with HCM and a 5-year risk of SCD ≥ 6% calculated on ICD implantation had more frequent life-threatening ventricular arrhythmias during long-term follow-up, but not statistically significant. There should be an individual approach when deciding on the need for device implantation in all patients with HCM.

scholarly journals Matrix metalloproteinases in serum of Emery-Dreifuss muscular dystrophy patients.

2009 ◽  
Vol 56 (4) ◽  
Author(s):  
Irena Niebroj-Dobosz ◽  
Agnieszka Madej-Pilarczyk ◽  
Michał Marchel ◽  
Beata Sokołowska ◽  
Irena Hausmanowa-Petrusewicz
Keyword(s):  
Muscular Dystrophy ◽  
Matrix Metalloproteinases ◽  
Cardiac Involvement ◽  
Autosomal Dominant ◽  
Serum Levels ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Cardiac Symptoms

In the pathogenesis of dilated cardiomyopathy (DCM) in Emery-Dreifuss muscular dystrophy (EDMD) matrix metalloproteinases (MMPs) are supposed to be involved and may have diagnostic/prognostic value. Serum levels of MT1-MMP, MMP-2 and MMP-9 were quantified by ELISA and zymography in 22 EDMD patients and 15 age-matched controls. In the autosomal-dominant EDMD MMP-2 and MT1-MMP were increased in all cases, and MMP-9 was increased in two of the eight examined patients. In the X-linked EDMD MMP-2 expression was increased in all the cases, MMP-9 level was elevated in 3 of the 14 cases, and MT1-MMP was decreased in eight of these patients. There was no evident correlation between the MMPs level and the different cardiac parameters including left-ventricular end-diastolic diameter, left atrial diameter and left ventricular ejection fraction in either form of EDMD. The presented results indicate that a changed level of matrix metalloproteinases, especially that of MMP-2 in serum, may be of value for detection of cardiac involvement in EDMD patients, especially in those patients with no evident subjective cardiac symptoms. Further follow-up studies of MMPs are needed to check if their determination is of value for monitoring of the progression of atrial/ventricular dilatation. MMPs determinations may also be useful for monitoring DCM treatment by synthetic MMPs inhibitors.

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