scholarly journals Rare Cause of Wide QRS Tachycardia

2015 ◽  
Vol 2015 ◽  
pp. 1-6
Author(s):  
Nikolay Yu. Mironov ◽  
Natalia A. Mironova ◽  
Marina A. Saidova ◽  
Olga V. Stukalova ◽  
Sergey P. Golitsyn

Cardiac involvement is a well-known feature of neuromuscular diseases. Most commonly cardiac manifestations occur later in the course of the disease. Occasionally severe cardiac disease, including conduction disturbances, life-threatening arrhythmias, and cardiomyopathy, with its impact on prognosis, may be dissociated from peripheral myopathy. We report a case of bundle branch reentrant ventricular tachycardia as primary manifestation of myotonic dystrophy and discuss associated diagnostic and treatment challenges.

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Panagiotis Korantzopoulos ◽  
Aris Bechlioulis ◽  
Lampros Lakkas ◽  
Katerina K Naka

Abstract Background  Cardiac electrical disturbances represent the most frequent cardiac manifestations of myotonic dystrophy Type 1 (MD1). Limited data suggest that the prevalence of Brugada syndrome in MD1 may be increased compared to the general population. Case summary  We report a case of a 22-year-old asymptomatic man with repolarization abnormalities in leads V1–V3 suggestive of Type III Brugada pattern. The patient had a family history of MD and incidents of sudden death in relatives. Drug-induced Brugada Type 1 syndrome was revealed after procainamide challenge. A ventricular stimulation study was positive since a polymorphic ventricular tachycardia was induced after two extrastimuli. The patient underwent implantation of a single chamber cardiac defibrillator (ICD). Eight months after the procedure he suffered an appropriate ICD shock due to rapid polymorphic ventricular tachycardia. Discussion  Brugada syndrome is linked with MD1. Potential life-threatening arrhythmias may develop in the adult life of MD1 patients. Electrocardiographic surveillance and tailored invasive treatment with ICDs can prevent sudden cardiac death in this setting.


1991 ◽  
Vol 14 (8) ◽  
pp. 696-698 ◽  
Author(s):  
Aviv Mager, ◽  
Boris Strasberg, ◽  
Bruria Zlotikamien, ◽  
Chaim Kaplinsky, ◽  
Samuel Sclarovsky,

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Paul Calis ◽  
Remco Berendsen ◽  
Angelique Logeman ◽  
Elise Sarton ◽  
Leon Aarts

Amiodarone-induced thryrotoxicosis (AIT) is a rare but serious complication of amiodarone use, especially in patients with severe cardiac disease. We present a patient who developed AIT, following administration of amiodarone for life-threatening ventricular arrhythmias. We discuss the medical management of AIT and anesthetic considerations for management of patients with thyrotoxicosis and severe cardiac disease who require surgery including thyroidectomy.


2010 ◽  
Vol 23 (2) ◽  
pp. 324-349 ◽  
Author(s):  
Alicia Hidron ◽  
Nicholas Vogenthaler ◽  
José I. Santos-Preciado ◽  
Alfonso J. Rodriguez-Morales ◽  
Carlos Franco-Paredes ◽  
...  

SUMMARY Parasitic infections previously seen only in developing tropical settings can be currently diagnosed worldwide due to travel and population migration. Some parasites may directly or indirectly affect various anatomical structures of the heart, with infections manifested as myocarditis, pericarditis, pancarditis, or pulmonary hypertension. Thus, it has become quite relevant for clinicians in developed settings to consider parasitic infections in the differential diagnosis of myocardial and pericardial disease anywhere around the globe. Chagas' disease is by far the most important parasitic infection of the heart and one that it is currently considered a global parasitic infection due to the growing migration of populations from areas where these infections are highly endemic to settings where they are not endemic. Current advances in the treatment of African trypanosomiasis offer hope to prevent not only the neurological complications but also the frequently identified cardiac manifestations of this life-threatening parasitic infection. The lack of effective vaccines, optimal chemoprophylaxis, or evidence-based pharmacological therapies to control many of the parasitic diseases of the heart, in particular Chagas' disease, makes this disease one of the most important public health challenges of our time.


2009 ◽  
Vol 19 (2) ◽  
pp. 170-178 ◽  
Author(s):  
Vlasta Fesslová ◽  
Paola Corti ◽  
Giovanna Sersale ◽  
Attilio Rovelli ◽  
Pierluigi Russo ◽  
...  

AbstractObjectiveTo analyze cardiac involvement and its progression in mucopolysaccharidoses, and to assess the short term impact of new therapeutic strategies.Patients and methodsWe studied echocardiographically 57 patients with various types of mucopolysaccharidoses, specifically types I, II, III, IV and VI, with a median age at the diagnosis of cardiac involvement of 5 years, following them for a median of 4.6 years, with a range from 0.9 to 21.2 years. We used a scoring system, along with the so-called delta score, to quantify the severity of involvement at baseline and at last examination, and to chart their progression over time.ResultsCases with cardiac involvement increased from 59.6% to 87.3% at the last examination. The scores increased with age, and were significantly different according to the specific type of mucopolysaccharidosis. Involvement of the mitral valve was most common, often associated with an aortic valvar anomaly and/or left ventricular hypertrophy. Patients with the first and second types had more severe involvement than those with the third or fourth types. Patients undergoing transplantation of haematopoietic stem cells seem to stabilize after an initial worsening while, in contrast, we were unable to demonstrate an effect of enzyme replacement therapy on the progression of the cardiac disease, possibly because those receiving such treatment had a higher median age, more severe cardiac disease and shorter follow-up.ConclusionsCardiac involvement was present early in more than a half of the patients identified as having mucopolysaccharidosis, and generally progressed, being more frequent and severe in the first and second types of the disease. Longer follow-up is needed to demonstrate any significant improvement induced by new therapies.


ESC CardioMed ◽  
2018 ◽  
pp. 1530-1534
Author(s):  
Henning Bundgaard ◽  
Anna Axelsson ◽  
Alex Christensen ◽  
Helle Petri

Myotonic dystrophy type 1 (DM1) is a multisystemic disease, mainly involving skeletal muscles, the central nervous system, the gastrointestinal tract, and the heart. DM1 is inherited as an autosomal dominant trait. Patients with DM1 have a reduced life expectancy with a mean age at death of approximately 53 years. Fibrosis, fat infiltration, and degeneration are seen in both skeletal muscles and in the myocardium. Cardiac involvement in DM1 patients is a major concern as the cause of death is of cardiac origin in 30% of patients. The major cardiac manifestations, including conduction abnormalities, supraventricular and ventricular arrhythmias, and reduced left ventricular systolic function, may lead to sudden cardiac death (SCD) or death from progressive heart failure. The increased risk of SCD underscores the need for assessment of cardiac involvement in order to prevent SCD. Clinical evaluation at the time of diagnosis and life-long repeated follow-up should include clinical assessment, electrocardiogram, Holter monitoring, and echocardiography. Generally, cardiac manifestations in DM1 patients should be treated according to general guidelines. An important topic to be resolved is whether a subset of patients, not fulfilling traditional criteria for pacemaker implantation, but who are estimated to be at high risk of severe conduction abnormalities, benefit from pacemaker or defibrillator implantation on a primary prophylactic basis.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Jonathan Solaimanzadeh ◽  
Aaron Freilich ◽  
Michael R Sood

Abstract Background Coronavirus disease 2019 (COVID-19) has been shown to have extensive effects on the cardiovascular system. Its long-term cardiac manifestations, however, remain unclear. Case presentation We report the case of a Caucasian patient with a mild and self-limited presentation of COVID-19, with subsequent development, months later, of exertional dyspnea and non-sustained ventricular tachycardia, long after resolution of his illness and after returning to aerobic exercise. The patient had normal screening tests including electrocardiogram (ECG) and echocardiogram 4 months after his illness. Cardiac magnetic resonance imaging demonstrated epicardial and pericardial fibrosis of the right ventricle free wall and outflow tract and the pericardium over the anterior wall, 6 months following the initial infection. First abnormal ECG was recorded at month 7 following illness. Conclusions This case suggests an insidious and possible long-term cardiac involvement and reflects the challenges in traditional workups and screening modalities in identifying cardiac involvement in COVID-19.


Diagnostics ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 989
Author(s):  
Cecilia Lazea ◽  
Simona Bucerzan ◽  
Camelia Al-Khzouz ◽  
Anca Zimmermann ◽  
Ștefan Cristian Vesa ◽  
...  

Gaucher disease (GD), one of the most common lysosomal disorders, is characterised by clinical heterogeneity. Cardiac involvement is rare and refers to pulmonary hypertension (PH), valvular abnormalities and myocardial infiltrative damage. The aim of this study was to evaluate cardiac involvement in a group of Romanian GD patients. Phenotypic and genotypic characterisation was carried out in 69 patients with GD type 1. Annual echocardiography and electrocardiography were performed to assess pulmonary pressure, morphology and function of the valves and electrocardiographic changes. Nine patients (13%) exhibited baseline echocardiographic signs suggesting PH. Mitral regurgitation was present in 33 patients (48%) and aortic regurgitation in 11 patients (16%). One patient presented aortic stenosis. Significant valvular dysfunction was diagnosed in 10% of patients. PH was associated with greater age (p < 0.001), longer time since splenectomy (p = 0.045) and longer time between clinical onset and the start of enzyme replacing therapy (p < 0.001). Electrocardiographic changes were present in five patients (7%).


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
R Zanoni ◽  
V Ferrara ◽  
G Lanati ◽  
G Vitale ◽  
F Di Nicola ◽  
...  

Abstract Background Anderson Fabry (AF) disease is a X-linked lysosomal storage disorder with multiorgan involvement. Cardiac disease, mainly represented by left ventricular hypertrophy (LVH) and arrhythmias, is the most frequent cause of premature death. It is well know that specific therapy is less effective after the development of LVH and myocardial fibrosis, therefore early cardiac detection (before LVH) is important. New cardiac magnetic resonance (CMR) parametric imaging techniques (T1 and T2 maps) enable myocardial tissue changes associated with AF disease. Purpose To evaluate the relationship between CMR tissue characterization and clinical and instrumental manifestations of AF disease to find early markers of cardiac involvement. Methods 31 AF patients (9 males, mean age 49±16 years) underwent ECG, echocardiogram and contrast CMR. TnI, BNP, pro-BNP and serum lyso-Gb3 were dosed. T1 mapping was performed in a pre-contrast acquisition with the modified Look-Locker inversion recovery (MOLLI) sequences. CMR results were compared with those of 43 healthy age and gender-matched controls. Results In AF patients native septal T1 values were significantly lower compared to healthy controls (median 949 vs 991 msec, p=0.0137) and were inversely related to Lyso-Gb3 serum levels (p=0.003). Patients with LVH had lower T1 septal values in comparison with patients without LVH (892 vs 981 msec; p=0.0012). Patients with classic form had abnormal low T1 values more frequently than pts with late onset variant (78 vs 23%; p=0.038). In AF patients native septal T2 values were significantly higher compared to the control group (53 vs 49 msec; p=0.0004) and correlated with troponin I (p=0.008) and NT-pro BNP (p=0.006) serum levels. No difference was found between pts with and without LVH (53.5 vs 52.5 ms; p=0.797) and the prevalence of abnormal high T2 values was similar between patients with late onset AF and pts with classical form (53% vs 50%; p=1.000). All patients with late onset AF and high T2 values were females. Conclusions CMR T1 (low values) and T2 (high values) mapping are useful tools to detect early cardiac involvement before LVH and to better understand the pathophysiology of cardiac disease in AF patients. Subclinical tissue inflammation, detectable through T2 maps, seems to be an additional pathogenetic mechanism related to the Gb3 storage that contributes to organ damage and precedes LVH, particularly in females patients with late onset phenotype. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Sant'Orsola-Malpighi Hospital


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