Search results for "cardiomyopathy"
showing 10 items of 242 documents
Assessment of atrial diastolic function in patients with hypertrophic cardiomyopathy by cine magnetic resonance imaging
2015
Purpose: This study was conducted to assess the role of atrial function by cardiac magnetic resonance (CMR) for the evaluation of diastolic physiology in patients with hypertrophic cardiomyopathy (HCM) compared to healthy controls. Materials and methods: We enrolled 23 consecutive patients affected by HCM and 43 healthy subjects as age-matched control cases (CC). CMR was performed through acquisition of cine steady-state free precession sequences using a 1.5-T scanner. Image postprocessing was carried out using Tracking Tool software. Results: Atrial volumes were significantly higher in patients with HCM compared to CC: maximum atrial volume (p = 0.007) and minimum atrial volume (p = 0.01).…
Therapeutic management in Sicilian patients with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy and focus on the role of implanta…
2014
Arrhythmogenic Right Ventrticular Dysplasia/Cardiomyopathy(ARVD/C) is an inherited cardiomyopathy characterized by right ven-tricularmyocytelosswithfibrofattyreplacement,ahighriskofventric-ular arrhythmias (VA) and sudden cardiac death (SCD) [1]. Preventionof SCD represents the most important management strategy and theachievement of this target can be reached by different therapeuticstrategies including implantable cardioverter-defibrillator (ICD)implantation, pharmacologic therapy, catheter ablation of ventriculartachycardia (VT) and cardiac transplantation [2,3]. The aim of thisstudy is to examine the outcome of the different therapies adopted ina group of affected patients, focusing on th…
Nemaline myopathy and heart failure: role of ivabradine; a case report
2015
Background Nemaline myopathy (NM) is a rare congenital myopathy characterized by muscle weakness, hypotonia and the presence in muscle fibers of inclusions known as nemaline bodies and a wide spectrum of clinical phenotypes, ranging from severe forms with neonatal onset to asymptomatic forms. The adult-onset form is heterogeneous in terms of clinical presentation and disease progression. Cardiac involvement occurs in the minority of cases and little is known about medical management in this subgroup of NM patients. We report a rare case of heart failure (HF) in a patient with adult-onset NM in whom ivabradine proved to be able to dramatically improve the clinical picture. Case presentation …
Glucose insulin potassium infusion improves systolic function in patients with chronic ischemic cardiomyopathy
2002
Objective: We assessed the effects of glucose–insulin–potassium (GIK) by echocardiography in stable patients with ischemic dysfunction. Methods: Twelve male patients with stable coronary disease (SCD) and ejection fraction (EF) <45% were studied for systolic function. GIK (glucose 30%, 300 insulin units and KCl 6 g/l) was infused at 1 ml/kg per h over 20 min. Hemodynamic and echocardiographic measurements were recorded at rest (T0), at the end (20 min) of GIK infusion (T + 20), 20 and 40 min after the end of the infusion (T + 40 and T + 60). Results: At T + 20, a significant decrease in WMSI (wall motion score index) was observed compared with T0 (2.16±0.14 vs. 2.30±0.16: P<0.05). An increa…
Desmin-related myopathies
1997
Desmin-related myopathies are marked by accumulation of desmin, which is often familial and associated with cardiomyopathy. When multifocal this excess is characterized by inclusions such as cytoplasmic or spheroid bodies, when disseminated the excess is called granulofilamentous material. Excess of desmin might represent an abnormal type of protein metabolism.
Ventricular Tachycardia and Early Fibrillation in Patients With Brugada Syndrome and Ischemic Cardiomyopathy Show Predictable Frequency-Phase Propert…
2015
[EN] Background¿ Ventricular fibrillation (VF) has been proposed to be maintained by localized high-frequency sources. We tested whether spectral-phase analysis of the precordial ECG enabled identification of periodic activation patterns generated by such sources. Methods and Results¿Precordial ECGs were recorded from 15 ischemic cardiomyopathy and 15 Brugada syndrome (type 1 ECG) patients during induced VF and analyzed in the frequency-phase domain. Despite temporal variability, induced VF episodes lasting 19.6±7.9 s displayed distinctly high power at a common frequency (shared frequency, 5.7±1.1 Hz) in all leads about half of the time. In patients with Brugada syndrome, phase analysis of …
An unusual case of sudden cardiac death during sexual intercourse
2018
The most common cause of sudden death during sexual intercourse in adults is heart disease, and it is usually the male, whereas the death of the woman is unusual. Generally, in these cases, death occurs as a result of cardiovascular disease. The authors report an unusual case of the sudden death of a young woman during sexual intercourse. The post-mortem investigations (autopsy, cardiac nuclear magnetic resonance and cardiac histology) demonstrated a previously undiagnosed arrhythmogenic right ventricular cardiomyopathy. The terminal cause of death was a malignant arrhythmia from arrhythmogenic right ventricular cardiomyopathy. This is the first report of a case in which sexual activity ca…
Sudden Cardiac and Noncardiac Death in Sports: Epidemiology, Causes, Pathogenesis, and Prevention
2018
AbstractAlthough few doubts remain that physical exercise should be widely promoted for maintenance of health and fitness, the risk of adverse events such as sudden death (especially due to cardiac causes, i.e., sudden cardiac death [SCD]) during exercise remains tangible. The overall risk of sudden death in athletes is relatively low (i.e., usually comprised between 0.1 and 38/100,000 person-years), and globally comparable to that of the general population. However, up to 20% of all sudden death cases are still recorded while exercising. The most frequent underlying disorders encountered in SCD are hypertrophic cardiomyopathy and coronary artery disease (CAD), representing three quarters o…
Incidence and Clinical Impact of Right Ventricular Involvement (Biventricular Ballooning) in Takotsubo Syndrome: Results From the GEIST Registry
2021
Background: The short- and long-term prognosis of Takotsubo syndrome (TTS) presenting with right ventricular (RV) involvement remains poorly understood. Research Question: What is the incidence and clinical outcome of RV involvement in TTS? Study Design and Methods: This study analyzed 839 consecutive patients with TTS (758 female subjects and 81 male subjects) in a multicenter registry. RV involvement was defined as wall motion abnormality of the RV free wall, with or without apical involvement. The median long-term follow-up was 2.1 years (interquartile range, 0.3-4.5 years). The primary outcome was in-hospital and out-of-hospital all-cause mortality. The secondary end point was a composi…
A bi-allelic loss-of-function SARS1 variant in children with neurodevelopmental delay, deafness, cardiomyopathy, and decompensation during fever
2021
Aminoacyl-tRNA synthetases (aaRS) are ubiquitously expressed enzymes responsible for ligating amino acids to their cognate tRNA molecules through an aminoacylation reaction. The resulting aminoacyl-tRNA is delivered to ribosome elongation factors to participate in protein synthesis. Seryl-tRNA synthetase (SARS1) is one of the cytosolic aaRSs and catalyzes serine attachment to tRNASer . SARS1 deficiency has already been associated with moderate intellectual disability, ataxia, muscle weakness, and seizure in one family. We describe here a new clinical presentation including developmental delay, central deafness, cardiomyopathy, and metabolic decompensation during fever leading to death, in a…