- Identification of clinically relevant phenotypes in patients with Ebstein anomaly. [Journal Article]
- CCClin Cardiol 2018 Mar 22
- CONCLUSIONS: This study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting.
- Four TRPM4 Cation Channel Mutations Found in Cardiac Conduction Diseases Lead to Altered Protein Stability. [Journal Article]
- FPFront Physiol 2018; 9:177
- Transient receptor potential melastatin member 4 (TRPM4), a non-selective cation channel, mediates cell membrane depolarization in immune response, insulin secretion, neurological disorders, and canc...
Transient receptor potential melastatin member 4 (TRPM4), a non-selective cation channel, mediates cell membrane depolarization in immune response, insulin secretion, neurological disorders, and cancer. Pathological variants inTRPM4gene have been linked to several cardiac phenotypes such as complete heart block (CHB), ventricular tachycardia, and Brugada syndrome (BrS). Despite recent findings regarding the functional implications of TRPM4 in cardiac diseases, the molecular and cellular mechanisms leading to altered conduction are poorly understood. In the present study, we identify and characterize four novelTRPM4variants found in patients with CHB or ventricular fibrillation. Three of them, p.A101T, p.S1044C and a double variant p.A101T/P1204L, led to a decreased expression and function of the channel. On the contrary, the variant p.Q854R showed an increase in TRPM4 current. Recent evidence indicates that altered degradation rate of mutant proteins represents a pathogenic mechanism underlying genetic diseases. In consequence, protein turnover of WT-TRPM4 and TRPM4 variants overexpressed in HEK293 cells was analyzed using cycloheximide, an inhibitor of protein biosynthesis. Upon addition of cycloheximide, WT-TRPM4 decayed with a half-life of ~20 h, while loss-of-expression variants showed a ~30% increase in degradation rate, with a half-life close to 12 h. Together, the gain-of-expression variant showed a higher stability and a doubled half-life compared to WT-TRPM4. In conclusion, decreased or increased protein expression of several TRPM4 variants linked to cardiac conduction disorders or ventricular arrhythmias were found to be caused by altered TRPM4 half-life compared to the WT form.
- The Clue Is in the U Wave: Torsades de Pointes Ventricular Tachycardia in a Hypokalemic Woman on Methadone. [Journal Article]
- AEAnn Emerg Med 2018; 71(4):473-476
- European Heart Rhythm Association (EHRA) position paper on arrhythmia management and device therapies in endocrine disorders, endorsed by Asia Pacific Heart Rhythm Society (APHRS) and Latin American Heart Rhythm Society (LAHRS). [Journal Article]
- EEuropace 2018 Mar 16
- Endocrine disorders are associated with various tachyarrhythmias, including atrial fibrillation (AF), ventricular tachycardia (VT), ventricular fibrillation (VF), and bradyarrhythmias. Along with und...
Endocrine disorders are associated with various tachyarrhythmias, including atrial fibrillation (AF), ventricular tachycardia (VT), ventricular fibrillation (VF), and bradyarrhythmias. Along with underlying arrhythmia substrate, electrolyte disturbances, glucose, and hormone levels, accompanying endocrine disorders contribute to development of arrhythmia. Arrhythmias may be life-threatening, facilitate cardiogenic shock development and increase mortality. The knowledge on the incidence of tachy- and bradyarrhythmias, clinical and prognostic significance as well as their management is limited; it is represented in observational studies and mostly in case reports on management of challenging cases. It should be also emphasized, that the topic is not covered in detail in current guidelines. Therefore, cardiologists and multidisciplinary teams participating in care of such patients do need the evidence-based, or in case of limited evidence expert-opinion based recommendations, how to treat arrhythmias using contemporary approaches, prevent their complications and recurrence in patients with endocrine disorders. In recognizing this close relationship between endocrine disorders and arrhythmias, the European Heart Rhythm Association (EHRA) convened a Task Force, with representation from Asia-Pacific Heart Rhythm Society (APHRS) and Sociedad Latinoamericana de Estimulación Cardíaca y Electrofisiología (SOLAECE), with the remit of comprehensively reviewing the available evidence and publishing a joint consensus document on endocrine disorders and cardiac arrhythmias, and providing up-to-date consensus recommendations for use in clinical practice.
- Management of sudden cardiac death in cardiac sarcoidosis using the wearable cardioverter defibrillator. [Journal Article]
- PlosPLoS One 2018; 13(3):e0194496
- CONCLUSIONS: Management of sudden cardiac death among cardiac sarcoidosis patients was aided by the wearable cardioverter defibrillator resulting in successful termination of ventricular tachycardia/fibrillation upon delivery of shock.
- Fatal Cardiac Hemochromatosis in a Patient with Hereditary Spherocytosis. [Journal Article]
- IHInt Heart J 2018 Mar 20
- A 31-year-old man was admitted to our hospital with atrial tachycardia and cardiogenic shock. He had been diagnosed with hereditary spherocytosis (HS) during childhood, but he never received any red ...
A 31-year-old man was admitted to our hospital with atrial tachycardia and cardiogenic shock. He had been diagnosed with hereditary spherocytosis (HS) during childhood, but he never received any red blood cell transfusions. Right ventricular endomyocardial biopsy revealed multiple myocardial hemosiderin deposits, and he was diagnosed with cardiac hemochromatosis. In addition to the iron deposition in the heart, the loss of myocyte and severe interstitial fibrosis were present. His cardiac function did not improve even after the cardioversion for atrial tachycardia, and he suffered from recurrent heart failure. Despite intensive medical treatment for heart failure and arrhythmias in combination with iron chelation therapy, he eventually died of progressive and refractory heart failure. Hemochromatosis is a systemic disorder characterized by the excessive deposition of iron in multiple organs. The occurrence of hemochromatosis in HS is extremely rare, and previous reports have shown that the coexistence of heterozygosity for the HFE gene mutation in HS patients causes excess iron storage. The prognosis is poor due to progressive congestive heart failure and refractory arrhythmias. Here we report a rare case of fatal cardiac hemochromatosis associated with HS. The possibility of cardiac hemochromatosis needs to be considered in cases of heart failure or arrhythmia in patients with HS.
- [Initial experience of catheter ablation of ventricular tachycardia originate from endocardium via direct ventricle puncture access in patients underwent mechanical valve implantation]. [Journal Article]
- ZXZhonghua Xin Xue Guan Bing Za Zhi 2018 Mar 24; 46(3):213-217
- Objective: To evaluate the results of catheter ablation of ventricular tachycardia (VT) via direct ventricle puncture access in patients without traditional approach.Methods:Two i...
Objective: To evaluate the results of catheter ablation of ventricular tachycardia (VT) via direct ventricle puncture access in patients without traditional approach.Methods:Two idiopathic left fasicular VT patients with mechanical aortic and mitrial valve repalcement and 1 patient with right ventricular originated VT post mechanical tricuspid valve repalcement from March 2010 to July 2012 in Fuwai hospital were enrolled in this study. For left fasicular VT patients, catheter ablation was performed using transapical left ventricular access via minithoracotomy. For the patient with right ventricular originated VT, catheter ablation was performed via percutaneous right ventricle puncture at xiphoid. Abaltion was guided under EnSite NavX mapping system. The feasibility of VT ablation via direct ventricle puncture access and long-term VT recurrence were investigated.Results:Catheter ablation was successful in all patients, and all clinical VTs were eliminated. The procedure time was 53, 62 and 74 minutes respectively with radiation time 11, 16 and 20 minutes. The ablation time was 130, 170 and 240 seconds individually. No procedure related complication occurred. After a follow-up time of 76, 55 and 82 months respectively, no VT recurrence was found in patients with left fasicular VT. New-onset VT with different morphology with previous VT was recorded in the patient with right ventricular originated VT, subcutaneous implantable defibrillator was implanted finally in this patient.Conclusions:For patients with endocardial origined ventricular arrhythmias which could not be ablated via traditional approaches, direct ventricle puncture access with hybrid techniques provides a new approach foreliminating VTs in these patients.
- Ventricular Tachycardia and Cardiovascular Collapse following Flexible Bronchoscopy: Lidocaine Cardiotoxicity. [Journal Article]
- JBJ Bronchology Interv Pulmonol 2018; 25(2):e24-e26
- Antiarrhythmic drug therapy during cardiopulmonary resuscitation: should we use it? [Journal Article]
- COCurr Opin Crit Care 2018 Mar 20
- CONCLUSIONS: The benefit of antiarrhythmic drugs appears to be for those patients in whom initial early CPR and defibrillation attempts fail and the antiarrhythmic drug is given early. There does not appear to be any clear survival benefit for any one particular drug and other factors such as availability and cost should be considered when deciding which drug to use. Furthermore, other interventions (e.g. percutaneous coronary intervention and extra-corporeal CPR) may provide additional survival benefit when defibrillation attempts and antiarrhythmic drugs are not effective.
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- Symptoms of massive cardiac tamponade during support of biventricular assist device. [Journal Article]
- IJInt J Artif Organs 2018 Mar 01; :391398818762351
- Typical symptoms of cardiac tamponade are jugular venous distension, pulsus paradoxus, hypotension, and rest dyspnea. However, these clinical symptoms can be masked in patients with ventricular assis...
Typical symptoms of cardiac tamponade are jugular venous distension, pulsus paradoxus, hypotension, and rest dyspnea. However, these clinical symptoms can be masked in patients with ventricular assist device and even more in patients supported with a biventricular-HeartWare ventricular assist device. Hereby, we report the case of a 30-year-old man supported with a biventricular-HeartWare ventricular assist device, who underwent a computed tomography scan due to suspect of ventricular assist device thrombosis. In the first scan, no pericardial effusion could be detected; however, a flow-limiting formation suggestive of a thrombus was localized in the outflow graft of the right ventricular assist device immediately before the anastomosis with pulmonary artery. Lysis therapy was initiated. On the same day, two episodes of sustained ventricular tachycardia and ventricular fibrillation without hemodynamic instability had to be promptly treated with antiarrhythmic drugs and electrical defibrillations. On the same day, a second computed tomography scan showed a massive pericardial effusion that required an emergency resternotomy.