Supraventricular tachycardias, conduction disease, and cardiomyopathy in 3 families with the same rare variant in TNNI3K (p.Glu768Lys)

Rare genetic variants in TNNI3K encoding troponin-I interacting kinase have been linked to a distinct syndrome consisting primarily of supraventricular tachycardias and variably expressed conduction disturbance and dilated cardiomyopathy in 2 families.
Source: Heart Rhythm - Category: Cardiology Authors: Source Type: research

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This study compared the myocardial deformation patterns of ICM and NICM using 2-D speckle tracking echocardiography (2-D STE) and sought to find parameters valuable in the diagnosis and management of dilated cardiomyopathy. The study population comprised 84 consecutive patients with LV end-diastolic dimension>55 mm and ejection fraction (EF)
Source: Ultrasound in Medicine and Biology - Category: Radiology Authors: Tags: Original Contribution Source Type: research
We report a case of an 8-year-old girl who presented with syncopal attacks and a history of viral illness a month ago. On examination, she was conscious, oriented but had a heart rate of 42/min which was unresponsive to atropine. She was started on dobutamine and isoproterenol. Electrocardiography and echocardiography revealed complete heart block with moderate tricuspid regurgitation, dilated cardiomyopathy and low ejection fraction. Patient was planned for urgent permanent pacemaker insertion. General anaesthesia was administered with endotracheal tube and controlled ventilation using fentanyl, ketamine and pancuronium. ...
Source: Indian Journal of Anaesthesia - Category: Anesthesiology Authors: Source Type: research
Conclusions: In conclusion, non-expert providers can achieve a high level of proficiency for the categorization of heart failure using handheld TTE in low-resource settings and use of telemedicine and remote mentorship may improve performance and feasibility. The addition of TTE resulted in substantial improvement in etiological specificity. Further study is needed to understand implications of this strategy on healthcare utilization, long-term patient outcomes, and cost. PMID: 31694487 [PubMed - in process]
Source: Global Health Action - Category: International Medicine & Public Health Tags: Glob Health Action Source Type: research
AbstractThe enhanced activity of endothelial progenitor cells (EPCs) by AMP-activated protein kinase (AMPK) agonists might explain the reversal of chronic heart failure (CHF) –mediated endothelial dysfunction. We studied baseline circulating EPC numbers in patients with heart failure and clarified the effect of fenofibrate on both circulating angiogenic cell (CAC) and late EPC activity. The numbers of circulating EPCs in CHF patients were quantified by flow cytometry. Blood-derived mononuclear cells were cultured, and CAC and late EPC functions, including fibronectin adhesion, tube formation, and migration, were eval...
Source: Journal of Cardiovascular Translational Research - Category: Cardiology Source Type: research
AbstractCardiac involvement is a major cause of morbidity and mortality in TA (2 ‐4). Any structure of the heart can potentially be affected with predominant involvement of ascending aorta leading to aortic aneurysm with aortic regurgitation, coronary vasculitis and myocarditis leading to dilated cardiomyopathy. Nearly half of the patients have cardiac involvement at some poin t during the course of their disease (5‐7).
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Reply To Letter To The Editor Source Type: research
Abstract BACKGROUND: Atrial fibrillation (AF) is the most common arrhythmia in dilated cardiomyopathy (DCM). The epidemiology, clinical and prognostic significance of AF in DCM are poorly defined. AIMS: Analyse AF impact and prognostic role in DCM, explore the concept of AF-induced DCM. METHODS: Hospital records of 285 DCM patients from 2012-2018 were analysed. RESULTS: AF was present in 89 (31%) patients who were older, more frequently male, had higher BMI, NYHA class, heart rate (HR), creatinine, larger atria (all p
Source: Polish Heart Journal - Category: Cardiology Authors: Tags: Kardiol Pol Source Type: research
Abstract Sudden cardiac death represents a major public health issue, with up to 50% of the cardiovascular mortality. Coronary artery disease and dilated cardiomyopathy both represent almost 90% of sudden cardiac death burden. Primary prevention using implantable cardioverter defibrillator relies, in this population, on the left ventricle ejection fraction simple measurement. In this paper, we aim to discuss in which extent a better understanding of competing risk situation may help for a better patient selection and eventually for optimizing primary prevention using implantable cardioverter defibrillator. PM...
Source: Presse Medicale - Category: General Medicine Authors: Tags: Presse Med Source Type: research
AbstractWe aimed to detect residual cardiac dysfunction —if any—in children with recovered primary dilated cardiomyopathy (DCM) by using the left ventricular (LV) layer-specific myocardial strains. Fifty children with recovered primary DCM both clinically and echocardiographically were included as the patient group. Fifty healthy children of matched age and sex served as the control group. Echocardiographic evaluation was performed for all included children in the form of conventional echocardiography, tissue Doppler imaging (TDI), two-dimensional speckle tracking echocardiography (2D-STE), and LV layer-specifi...
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
CONCLUSION: Strict monitoring and control of hemodynamic parameters as well as meticulous fluid therapy is the cornerstone in improving outcome in patients with dilated cardiomyopathy undergoing renal transplant surgery. PMID: 31669040 [PubMed - as supplied by publisher]
Source: Revista Brasileira de Anestesiologia - Category: Anesthesiology Tags: Rev Bras Anestesiol Source Type: research
AbstractLeft ventricular assist device is a well-established therapy in heart failure adults, but less in children. A 13-year-old-boy with severe left ventricular dysfunction did not improve under medical treatment. A HeartMate 3 (HM3) was implanted as a bridge to transplantation. Despite the size limitation, the HM3 shows promising results and our case supports its feasibility in children.
Source: Mammalian Genome - Category: Genetics & Stem Cells Source Type: research
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