Search results for "Cardiomyopathy"

Modelling cardiac mechanics of left ventricular noncompaction

2020

Left ventricular noncompaction (LVNC) can be defined as a cardiomyopathy characterised by a pattern of prominent trabecular structure and deep intertrabecular recesses, that is thought to be caused by an arrest of normal endomyocardial morphogenesis. Using patient-specific computational modelling, we assessed the cardiac mechanics of five patients with LVNC and compared myocardial stress and pump performance to those of healthy controls. Findings shown that patients with LVNC have impaired left ventricular (LV) function, making it possible that the lack of fibre shortening of noncompacted layer can determine poor heart function. Pronounced end-systolic wall stress on left ventricular wall o…

Primary desminopathies.

2007

•  Introduction •  Desmin is an essential component of the extrasarcomeric cytoskeleton in striated muscle cells •  Distal myopathy,cardiac arrhythmias,cardiomyopathy:classical criteria of primary desminopathies •  Sub-sarcolemmal and cytoplasmic desmin-positive protein aggregates:the morphological hallmark of primary and secondary desminopathies •  The spectrum of pathogenic desmin gene mutations •  The molecular pathogenesis of primary desminopathies: some answers gained,but even more questions raised •  Diagnostic work-up to distinguish primary from secondary desminopathies •  Treatment and clinical management of primary desminopathy patients Abstract Mutations of the human desmin gene o…

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…

Evidence of impaired coronary flow reserve and elevated microvascular resistances in a case of recurrent left apical ballooning.

2011

A 56-year old man was admitted for precordial pain associated with dyspnea and evidence of myocardial ischemia on ECG and cardiac ultrasound. His previous history included an episode of left apical ballooning five years before. At angiography, no evidence of significant epicardial coronary artery disease was manifest. Interestingly, both coronary flow reserve and the index of microvascular resistances were abnormal, showing evidence of impaired microvascular function. This is the first report providing direct evidence of microvascular dysfunction in a patient with (recurrent) apical ballooning.

Early-onset ataxia with cardiomyopathy and retained tendon reflexes maps to the friedreich's ataxia locus on chromosome 9q

1995

Absence of lower limb tendon reflexes has been considered an essential diagnostic criterion for Friedreich's ataxia (FA). However, preservation of knee and ankle jerks has been reported in a few patients. Linkage analysis to FA locus (FRDA) on chromosome 9q13-21.1 was performed in 11 patients from 6 families with FA phenotype, including cardiomyopathy, but retained reflexes (FARR). A maximal lod score of 3.38 at recombination fraction theta equal to 0.00 was obtained demonstrating that FARR maps to the FRDA locus. These results suggest that FARR is a variant phenotype of FA.

A case of arrhythmogenic right ventricular cardiomyopathy without ventricular arrhythmias

2014

Abstract We submit a case report of a 21-year-old man admitted to our emergency room for acute right heart failure. Arrhythmogenic right ventricular cardiomyopathy (ARVC) was diagnosed but ventricular arrhythmias have never been detected. On the basis of the diagnosis of ARVC and for the frequent episodes of symptomatic bradycardia, the patient underwent implantation of a biventricular defibrillator (CRT-D). In ARVC symptoms usually appear between the ages of 30–50. Especially in young patients the most common clinical presentation of ARVC are palpitations and syncope due to ventricular tachycardia with left bundle branch morphology. In cases of older patients in whom the disease has been d…

The electrocardiographic ‘triangular QRS-ST-T waveform’ pattern : a marker of severe haemodynamic compromise in Takotsubo syndrome : a case report

2020

Abstract Background Takotsubo cardiomyopathy is characterized by transient regional systolic dysfunction of the left ventricle, mimicking myocardial infarction. Although systolic left ventricular (LV) function normalizes in most cases, the outcome is not always favourable. Recently, a rare electrocardiogram (ECG) finding, lambda wave ST elevation or ‘triangular QRS-ST-T waveform’, was suggested as a possible marker of poor outcome in Takotsubo patients Case summary After a brief episode of chest pain and shortness of breath, a 67-year-old woman developed cardiogenic shock. Her resting ECG showed widespread ST elevations, which soon evolved into a pattern of triangular QRS-ST-T waveforms in …

Complications of Diabetes 2017

2018

Analysis on sarcoglycans expression as markers of septic cardiomyopathy in sepsis-related death

2018

The post-mortem assessment of sepsis-related death can be carry out by many methods recently suggested as microbiological and biochemical investigations. In these cases, the cause of death is a multiple organ dysfunction due to a dysregulated inflammatory response occurring after the failure of infection control process. It was highlighted also that the heart can be a target organ in sepsis which determines the so-called septic cardiomyopathy characterized by myocardial depression. Several mechanisms to explain the pathophysiology of septic cardiomyopathy were suggested, but very few studies about the structural alterations of cardiac cells responsible for myocardial depression were carried…

Impact of pericardial effusion on cardiac mechanics in patients with dilated cardiomyopathy

2013

Dilated cardiomyopathy (CDM) is a degenerative disease of the myocardium accompanied by left ventricular (LV) remodeling, resulting in an impaired pump performance. Differently, pericardial effusion(PE) is a liquid accumulation in the pericardial cavity, which may inhibit blood filling of heart chambers. Clinical evidence show that PE may improve pump performance in patients with CDM. Therefore, this study aims to assess wall stress and global function of patients with CDM, PE as compared to healthy patient. These findings suggests that CDM has an important implication in the mechanical changes of LV and right ventricle by increasing wall stress and reducing pump function. Conversely, PE de…