scholarly journals MELAS Syndrome with Cardiac Involvement: A Multimodality Imaging Approach

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Sara Seitun ◽  
Laura Massobrio ◽  
Anna Rubegni ◽  
Claudia Nesti ◽  
Margherita Castiglione Morelli ◽  
...  

A 49-year-old man presented with chest pain, dyspnea, and lactic acidosis. Left ventricular hypertrophy and myocardial fibrosis were detected. The sequencing of mitochondrial genome (mtDNA) revealed the presence of A to G mtDNA point mutation at position 3243 (m.3243A>G) in tRNALeu(UUR) gene. Diagnosis of cardiac involvement in a patient with Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes syndrome (MELAS) was made. Due to increased risk of sudden cardiac death, cardioverter defibrillator was implanted.

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 295
Author(s):  
Anna B. Reid ◽  
Luigi Venetucci ◽  
Matthias Schmitt ◽  
Gaetano Nucifora

The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is an uncommon cause of cardiac hypertrophy, fibrosis, and dysfunction. It shares similar features to numerous other causes of left ventricular hypertrophy, and therefore, because of its rarity, may not be immediately considered as a diagnosis. Prompt recognition of clinical and cardiac imaging features may expedite diagnosis and management. We report the case of a 38-year-old man admitted with neurological symptoms and in whom the diagnostic workup led to the diagnosis of MELAS syndrome with cardiac involvement.


2011 ◽  
pp. 119-125
Author(s):  
Thi Thuy Hang Nguyen

Objective: Prehypertensive individuals are at increased risk for developing hypertension and their complication. Many studies show that 2/3 prehypertensive individuals develop hypertension after 4 years. ECG and echocardiography are the routine tests used to assess LV mass. The objective of the research to determine the percentage of change in left ventricular morphology in the ECG, echocardiography, which explore the characteristics of left ventricular structural changes by echocardiography in pre-hypertensive subjects. Materials and method: We studied a total of 50 prehypertensive, 30 males (60%) and 20 females (40%), mean age 48.20±8.47years. 50 normotensive volunteers as control participants. These subjects were examined for ECG and echocardiography. Results: In prehypertensive group, with 18% of left ventricular hypertrophy on electrocardiogram, 12% of left ventricular hypertrophy on echocardiography; in the control group, we did not find any subjects with left ventricular hypertrophy. In the group with left ventricular hypertrophy, mostly eccentric left ventricular hypertrophy (83.33%), concentric left ventricular hypertrophy is 16.67%. Restructuring of left ventricular concentric for 15.9% of subjects without left ventricular hypertrophy on echocardiography. Conclusion: There have been changed in left ventricular morphology even in prehypertensive


2013 ◽  
Vol 2013 ◽  
pp. 1-8 ◽  
Author(s):  
Otelio Randall ◽  
John Kwagyan ◽  
Tamrat Retta ◽  
Kenneth Jamerson ◽  
Velvie Pogue ◽  
...  

Pulse pressure (PP), a marker of arterial system properties, has been linked to cardiovascular (CV) complications. We examined (a) association between unit changes of PP and (i) composite CV outcomes and (ii) development of left-ventricular hypertrophy (LVH) and (b) effect of mean arterial pressure (MAP) control on rate of change in PP. We studied 1094 nondiabetics with nephrosclerosis in the African American Study of Kidney Disease and Hypertension. Subjects were randomly assigned to usual MAP goal (102–107 mmHg) or a lower MAP goal (≤92 mmHg) and randomized to beta-blocker, angiotensin converting enzyme inhibitor, or calcium channel blocker. After covariate adjustment, a higher PP was associated with increased risk of CV outcome (RR = 1.28, CI = 1.11–1.47,P<0.01) and new LVH (RR = 1.26, CI = 1.04–1.54,P=0.02). PP increased at a greater rate in the usual than in lower MAP groups (slope ± SE: 1.08 ± 0.15 versus 0.42 ± 0.15 mmHg/year,P=0.002), but not by the antihypertensive treatment assignment. Observations indicate that control to a lower MAP slows the progression of PP, a correlate of cardiovascular remodeling and complications, and may be beneficial to CV health.


Author(s):  

Dilated cardiomyopathy (DCM) is a disease characterised as left ventricular (LV) or biventricular dilatation with impaired systolic function. Regardless of underlying cause patients with DCM have a propensity to ventricular arrhythmias and sudden cardiac death. Implantable Cardioverter Defibrillator (ICD) implantation for these patients results in significant reduction of sudden cardiac death [1-3]. ICD devices may be limited by right ventricle (RV) sensing dysfunction with low RV sensing amplitude. We present a clinical case of patient with DCM, implanted ICD and low R wave sensing on RV lead.


2021 ◽  
Vol 31 (1) ◽  
pp. 10-16
Author(s):  
Laura Tapoi ◽  
Alexandra Clement ◽  
Rodica Radu ◽  
Radu Sascau

Arrhythmogenic cardiomyopathy, as it has been recently redefi ned, is characterized by progressive myocyte loss with fibrosis and fat infiltration of the myocardium, which finally leads to a broad clinical spectrum ranging from heart failure symptoms to sudden cardiac death. The diagnosis of arrhythmogenic cardiomyopathy is challenging particularly because of its heterogeneity in presentation, which varies from focal right ventricular involvement to biventricular or prominent left ventricular phenotype. In the past decades, the development of new electrocardiographic and imaging diagnostic criteria for arrhythmogenic cardiomyopathy constituted an important area of research and resulted in the elaboration of the Padua criteria. However, even with the widespread availability of modern imaging techniques, there is still a lack of awareness in the health care community and this pathology persist in being under-or misdiagnosed. Given the limited indication of endomyocardial biopsy for the diagnosis of arrhythmogenic cardiomyopathy, one can conclude that the progress that has been made in the last few years in the multimodality imaging field is of utmost importance for the early detection and proper treatment of patients with arrhythmogenic cardiomyopathy, providing valuable prognostic information.


2021 ◽  
Vol 19 ◽  
Author(s):  
Jean-Jacques Monsuez ◽  
Marilucy Lopez-Sublet

: Persons living with HIV infection (PLWH) have been recognized to have an increased risk of sudden cardiac death (SCD). Prevention of this risk should theoretically be included in their long-term management. However, only a few approaches have been proposed to optimize such interventions. Targeting detection of the commonly associated conditions such as coronary artery disease, left ventricular dysfunction, heart failure, QT interval prolongation and ventricular arrhythmias is the first step of this prevention. However, although detection of the risk of SCD is a suitable challenge in PLWH, it remains uncertain whether optimized treatment of the identified risks would unequivocally translate into a decrease in SCD rates.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Tuomas Kenttä ◽  
Bruce D Nearing ◽  
Kimmo Porthan ◽  
Jani T Tikkanen ◽  
Matti Viitasalo ◽  
...  

Introduction: Noninvasive identification of patients at risk for sudden cardiac death (SCD) remains a major clinical challenge. Abnormal ventricular repolarization is associated with increased risk of lethal ventricular arrhythmias and SCD. Hypothesis: We investigated the hypothesis that spatial repolarization heterogeneity can identify patients at risk for SCD in general population. Methods: Spatial R-, J- and T-wave heterogeneities (RWH, JWH and TWH, respectively) were automatically analyzed with second central moment technique from standard digital 12-lead ECGs in 5618 adults (46% men; age 50.9±12.5 yrs.) who took part in Health 2000 Study, an epidemiological survey representative of the entire Finnish adult population. During average follow-up of 7.7±1.4 years, a total of 72 SCDs occurred. Thresholds of RWH, JWH and TWH were based on optimal cutoff points from ROC curves. Results: Increased RWH, JWH and TWH (Fig.1) in left precordial leads (V4-V6) were univariately associated with SCD (P<0.001, each). When adjusted with clinical risk markers (age, gender, BMI, systolic blood pressure, cholesterol, heart rate, left ventricular hypertrophy, QRS duration, arterial hypertension, diabetes, coronary heart disease and previous myocardial infarction) JWH and TWH remained as independent predictors of SCD. Increased TWH (≥102μV) was associated with a 1.9-fold adjusted relative risk (95% confidence interval [CI]: 1.2 - 3.1; P=0.011) and increased JWH (≥123μV) with a 2.0-fold adjusted relative risk for SCD (95% CI: 1.2 - 3.3; P=0.004). When both TWH and JWH were above threshold, the adjusted relative risk for SCD was 3.2-fold (95% CI: 1.7 - 6.2; P<0.001). When all heterogeneity measures (RWH, JWH and TWH) were above threshold, the risk for SCD was 3.7-fold (95% CI: 1.6 - 8.6; P=0.003). Conclusions: Automated measurement of spatial J- and T-wave heterogeneity enables analysis of high patient volumes and is able to stratify SCD risk in general population.


2020 ◽  
Vol 4 (FI1) ◽  
pp. 1-6 ◽  
Author(s):  
Gianfranco Mitacchione ◽  
Marco Schiavone ◽  
Alessio Gasperetti ◽  
Giovanni B Forleo

Abstract Background Coronavirus disease 2019 (COVID-19) has been associated with myocardial involvement. Among cardiovascular manifestations, cardiac arrhythmias seem to be fairly common, although no specifics are reported in the literature. An increased risk of malignant ventricular arrhythmias and electrical storm (ES) has to be considered. Case summary We describe a 68-year-old patient with a previous history of coronary artery disease and severe left ventricular systolic disfunction, who presented to our emergency department describing cough, dizziness, fever, and shortness of breath. She was diagnosed with COVID-19 pneumonia, confirmed after three nasopharyngeal swabs. Ventricular tachycardia (VT) storm with multiple implantable cardioverter defibrillator (ICD) shocks was the presenting manifestation of cardiac involvement during the COVID-19 clinical course. A substrate-based VT catheter ablation procedure was successfully accomplished using a remote navigation system. The patient recovered from COVID-19 and did not experience further ICD interventions. Discussion To date, COVID-19 pneumonia associated with a VT storm as the main manifestation of cardiac involvement has never been reported. This case highlights the role of COVID-19 in precipitating ventricular arrhythmias in patients with ischaemic cardiomyopathy who were previously stable.


2020 ◽  
Vol 90 (1) ◽  
Author(s):  
Antonio Landi ◽  
Anto Luigi Andres ◽  
Massimo Napodano

Left ventricular pseudoaneurysms (LVP) are rare but may arise after myocardial infarction, trauma or cardiac surgery, tending to expand and rupture over the time. We show the case of a 75-year-old patient with a recurrent giant ventricular pseudoaneurysm, who presented to the emergency department with sustained ventricular tachycardia. Pseudoaneurysmatic lesion was investigated through echocardiography, angiography and Cardiac Computed Tomography, in order to evaluate the size and spatial orientation of the pseudoaneurysm and to set a tailored treatment. At emergency department, sustained ventricular tachycardia may be the first and unique clinical presentation of ventricular pseudoaneurysm late recurrence, whose management requires a multimodality imaging approach to guide surgical correction.


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