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Nonbacterial thrombotic endocarditis, a form of noninfectious thrombotic endocarditis, is mainly characterized by deposition of sterile platelet thrombi on heart valves. Usually, it is observed in advanced malignancy. Herein, we report a case of a previously healthy male with recent unprovoked deep vein thrombosis presented with acute ischemic stroke. The echocardiogram revealed aortic and mitral valve masses. Eventually, he was discovered to have advanced cholangiocarcinoma. The present case, apart from being the youngest reported case, is among the few reported cases which manifest the association between cholangiocarcinoma and nonbacterial thrombotic endocarditis.

Sick sinus syndrome (SSS) is a group of disorders characterized by an abnormal cardiac impulse formation or propagation from the sinoatrial node. Mutated SCN5A has been reported in SSS, however, homozygosity of SCN5A is exceedingly rare. Here, we report a consanguineous family with four affected children with SSS. Symptomatic bradycardia necessitated implanting a pacemaker in all of them. Sequencing SCN5A revealed a novel homozygous variant (p.Cys1850Arg), which was predicted to interfere with protein folding. Our report describes the phenotype of a novel homozygous SCN5A variant and contributes to the compendium of molecular pathology of inherited arrhythmias in consanguineous populations.

Sudden Cardiac Death (SCD) is a significant health problem worldwide. Multiple randomized controlled trials have shown that Implantable Cardioverter Defibrillators (ICDs) are effective life-saving management option for individuals at risk of SCD in both primary and secondary prevention. Although the conventional transvenous ICDs (TV-ICDs) are safe and effective, there are potential complications associated with its use, including localized pocket or wound infection or systematic infection, a vascular access related complication such as pneumothorax, and venous thrombosis, and lead related complications such as dislodgement, malfunction, and perforation. Furthermore, transvenous leads placement may not be feasible in certain patients like those with venous anomaly or occlusion, or with the presence of intracardiac shunts. Transvenous leads extraction, when needed, is associated with considerable morbidity & mortality and requires significant skills and costs. Totally subcutaneous ICD (S-ICD) is designed to afford the same life-saving benefit of the conventional TV-ICDs while avoiding the shortcomings of the TV-leads and to simplify the implant techniques and hence expand the use of ICDs in clinical practice. It becomes commercially available after receiving CE mark in 2009, and its use increased significantly after its FDA approval in 2012. This review aims to give an overview of the S-ICD system components, implantation procedure, clinical indications, safety, efficacy, and future directions.

Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiomyopathy characterized histologically by the replacement of ventricular myocardium with fibrous and fatty tissue, and clinically by ventricular tachycardia arrhythmias primarily of right ventricular (RV) origin. Implantable cardioverter defibrillator (ICD) is the only proven therapy to reduce mortality in ARVC/D patients. However, it has the risk of inappropriate anti-tachycardia pacing (ATP) or shocks. This study aimed to assess the occurrence of appropriate and inappropriate ICD therapies in ARVC/D patients who underwent ICD implantation in a single Cardiac Centre. Methods Retrospective analysis of the data of patients with the diagnosis of ARVC/D based on the 2010 revised Task Force Criteria, who underwent ICD implantation in the Heart Centre, at King Faisal Specialist Hospital and Research Center (KFSH&RC), Riyadh between January 1997 and May 2016. The clinical data and information about appropriate and inappropriate ICD therapies were obtained from medical records with the review of the available intra-cardiac electrograms (EGMs). Results Twenty-two ARVC/D patients with ICD implantation (20 males (91%), mean age at ICD implantation: 32 ± 14 years). ICD was implanted for secondary prevention of sudden cardiac death (SCD) in 15 patients (68.2%), and for primary prevention in 7 patients (31.8%). At mean follow-up of 9.4 ± 4.8 years, 11 patients (50%) had appropriate ICD therapies, and five patients (22.7%) had inappropriate ICD therapies. Out of 950 ICD therapies, 865 (91%) were appropriate (586 episodes of VT/VF treated with ATP (61.3%), and 279 episodes treated with shocks (29.37%)) and 85 (9.4%) were inappropriate (45 episodes treated with ATP (4.73%), and 40 treated with shocks (4.21%)). Conclusion ARVC/D patients are at risk of VT/VF arrhythmias. ICD therapy is the only proven life-saving therapy in those patients. Most of ICD therapies in our patient’s population are appropriate, and ATP therapy is effective in terminating most of VT episodes. Although we do not have any patient with subcutaneous ICD, the high success rate of ATP suggests that transvenous ICD would be more appropriate in ARVC/D patients.

Tricuspid valve dysfunction and in particular tricuspid stenosis has recently been described secondary to cardiac implantable electronic devices. The valve is subjected to different mechanisms of injury related to the endocardial lead passing through its plane. The lead can form a loop or perforate one of the leaflets and initiate inflammatory response and fibrotic changes. Multimodality cardiac imaging is required to diagnose this clinical entity and decide on the best treatment plan. Here we present a case of a young female who developed tricuspid stenosis secondary to permanent pacemaker lead that was implanted 24 years before. We performed a review for all cases reported in the literature with a similar condition and various treatment approaches. .

BACKGROUND: Normal single-photon emission computed tomography (SPECT) myocardial perfusion imaging (MPI) has a high negative predictive value for ischemic heart disease. Thus, the presence of subclinical coronary atherosclerosis detected by coronary artery calcification (CAC) score in patients who have undergone SPECT MPI is unknown. OBJECTIVES: Determine the prevalence of coronary artery calcification (CAC) in patients with normal SPECT MPI and examine the association of CAC with conventional coronary artery disease (CAD) risk factors. DESIGN: Cross-sectional analytical study using medical records from February 2010 to April 2016. SETTINGS: Single tertiary-care center. PATIENTS AND METHODS: We studied patients referred from the outpatient clinical services for clinically indicated noninvasive CAD diagnosis with MPI SPECT. CAC scoring was subsequently performed within 3 months after a normal MPI. We excluded patients with chest pain or decompensated heart failure or patients with a history of CAD. The study population was divided into three groups: patients with a CAC score of 0, a CAC score from 1 to 300, and a CAC score more than 300. The groups were analyzed by age and other demographic and clinical characteristics. MAIN OUTCOME MEASURE(S): Prevalence of CAC in patients with normal MPI. RESULTS: The prevalence of CAC was 55% (n=114) in 207 patients with a mean (SD) age of 57.1 (10.4) years. Twelve percent had severe coronary atherosclerosis (CAC score >300). All patients had a normal MPI SPECT. CAC scores were 0 for 93 patients (45%), 1 to 300 for 89 (43%), and more than 300 for 24 (12%). There was a strong association between CAC score and age (P

Gitelman syndrome (GS) is an autosomal recessive tubulopathy recently implicated in cases with ventricular arrhythmias (VAs), the latter being considered linked to electrolytes’ imbalance. However, a direct causal relationship is considered to be an oversimplification for a complex molecular dysfunction. Recent work has suggested a degree of microvascular dysfunction in patients with GS that might be attributed as a mechanism of arrhythmia. We report a case of GS presenting with VAs complicated by cardiomyopathy. The high load of premature ventricular contractions that were attributed to the hypokalaemia has masked the presence of the left ventricular (LV) outflow tract tachycardia. Her LV systolic function recovered after successful electrophysiology ablation procedure. Atrioventricular nodal re-entry tachycardia was discovered incidentally during the study and was ablated successfully.

Infections after placement of cardiac implantable electronic devices (CIEDs) are associated with substantial morbidity and mortality. There is limited evidence on prophylactic strategies, other than the use of preoperative antibiotics, to prevent such infections.We conducted a randomized, controlled clinical trial to assess the safety and efficacy of an absorbable, antibiotic-eluting envelope in reducing the incidence of infection associated with CIED implantations. Patients who were undergoing a CIED pocket revision, generator replacement, or system upgrade or an initial implantation of a cardiac resynchronization therapy defibrillator were randomly assigned, in a 1:1 ratio, to receive the envelope or not. Standard-of-care strategies to prevent infection were used in all patients. The primary end point was infection resulting in system extraction or revision, long-term antibiotic therapy with infection recurrence, or death, within 12 months after the CIED implantation procedure. The secondary end point for safety was procedure-related or system-related complications within 12 months.A total of 6983 patients underwent randomization: 3495 to the envelope group and 3488 to the control group. The primary end point occurred in 25 patients in the envelope group and 42 patients in the control group (12-month Kaplan-Meier estimated event rate, 0.7% and 1.2%, respectively; hazard ratio, 0.60; 95% confidence interval [CI], 0.36 to 0.98; P = 0.04). The safety end point occurred in 201 patients in the envelope group and 236 patients in the control group (12-month Kaplan-Meier estimated event rate, 6.0% and 6.9%, respectively; hazard ratio, 0.87; 95% CI, 0.72 to 1.06; P0.001 for noninferiority). The mean (±SD) duration of follow-up was 20.7±8.5 months. Major CIED-related infections through the entire follow-up period occurred in 32 patients in the envelope group and 51 patients in the control group (hazard ratio, 0.63; 95% CI, 0.40 to 0.98).Adjunctive use of an antibacterial envelope resulted in a significantly lower incidence of major CIED infections than standard-of-care infection-prevention strategies alone, without a higher incidence of complications. (Funded by Medtronic; WRAP-IT ClinicalTrials.gov number, NCT02277990.).

Background: Subcutaneous implantable cardioverter defibrillator (S-ICD) system has been proven to be an effective therapy for prevention of sudden cardiac death (SCD) in selected patients. Although the Shockless IMPLant Evaluation (SIMPLE) trial has shown that defibrillation threshold (DFT) testing is not necessary for transvenous ICD (TV-ICD) systems, it is still recommended for S-ICD systems. We aimed to study the efficacy and safety of S-ICD implantation without DFT in our Heart Center with the comparison of S-ICD patients’ outcome to those with a single chamber TV-ICD without DFT in the same period. Methods: A retrospective analysis of patients underwent S-ICD without DFT from December 2014 to May 2016 with the comparison to single chamber TV-ICD patients implanted during the same period. Results: Thirty consecutive patients (23 males (76.7%); mean age 41 ± 13 years; mean left ventricular ejection fraction 30±12%) received a S-ICD for primary (25 patients, 83.3%) or secondary prevention (five patients, 16.7%) of SCD. During a mean follow-up of 710.6 ± 190 days, three patients received 38 appropriate ICD shocks (90.5%), and two patients received four inappropriate shocks (9.5%). There were two mortalities (6.7%): one cardiac and one non-cardiac. When compared to 30 consecutive who received a single chamber TV-ICD during the same period, there was no significant difference in mortality. Conclusions: Implantation of S-ICD using intermuscular approach without DFT seems to be safe and effective. Data from large S-ICD registries with long-term follow-up, and preferably randomized controlled studies, are needed to confirm this finding. Cardiol Res. 2017;8(6):319-326 doi: https://doi.org/10.14740/cr638w

Patients with complex congenital heart disease (CHD) and low left ventricular ejection fraction are at an increased risk of sudden cardiac death (SCD). Prevention of SCD by subcutaneous implantable cardioverter defibrillator (S-ICD) implantation may represent a valuable option in certain CHD patients. Patients with CHD and dextrocardia pose a challenge in S-ICD system implantation, and nonstandard device placement may be required. Furthermore, electrocardiogram (ECG) screening prior to S-ICD implantation in CHD patients has significant limitations. This case represents the placement of a S-ICD system on the right side of the chest in a 26-year-old male with severe biventricular failure and nonsustained ventricular tachycardia following multiple corrective surgeries of situs inversus totalis, double-outlet right ventricle with a ventricular septal defect, and pulmonary atresia. The use of S-ICDs in a CHD population and in particular CHD patients with dextrocardia and right-sided S-ICD implantation is briefly discussed.

Background: Permanent pacemaker (PPM) implantation after cardiac surgery is required in 0.4-6% of patients depending on cardiac surgery type. PPM implantation in the early postoperative period may reduce morbidity and postoperative hospital stay. We performed a retrospective review of electronic medical records of adult patients with coronary artery bypass grafting (CABG), valve surgery, or both, over a 3-year period. Our aim was to identify predictors of PPM requirements and PPM dependency on follow-up in the current surgical era. Methods: After exclusion of patients with congenital heart disease, patients who already had a PPM or implantable cardioverter defibrillator (ICD), and patients with an indication for PPM or ICD before surgery, we identified 1,234 adult patients who underwent cardiac surgery between January 2007 and December 2009. A retrospective review of electronic medical records and pacemaker clinic data was performed. Results: Patients’ mean age was 46.65 ± 16 years, and 59% were males. CABG was performed in 575 (46.6%) cases, aortic valve replacement in 263 (21.3%), mitral valve replacement in 333 (27%), and tricuspid valve replacement in 76 patients (6.2%). Twenty patients (1.6%) required implantation of a PPM postoperatively. Indications for PPM implantation included complete atrioventricular (AV) block in 13 (65%), sick sinus syndrome in three (15%), and atrial fibrillation (AF) with a slow ventricular rate in four (20%). Predictors for PPM requirement by multivariate analysis were the presence of pulmonary hypertension (P-HTN), reoperation, and left bundle branch block (LBBB) (P < 0.05). Late follow-up was available in 18 patients, at 84.5 ± 30 months. Eleven patients (61%) were PPM dependent on long-term follow-up. Conclusions: Patients at high risk for PPM implantation after cardiac surgery include those with P-HTN, reoperation, and pre-existing LBBB. Of those receiving a PPM, about one-third will recover at least partially at long-term follow-up. We recommend preoperative assessment for risk of requiring postoperative PPM, to counsel patients about this risk and early PPM implantation in high-risk patients who are PPM dependent after surgery. Cardiol Res. 2016;7(4):123-129 doi: http://dx.doi.org/10.14740/cr480w

Background. Reduced heart rate variability (HRV) indicates dominance of the sympathetic system and a state of “physiologic stress.” We postulated that, in patients with critical illness, increases in HRV might signal successful resuscitation and improved prognosis. Methods. We carried out a prospective observational study of HRV on all patients referred to the rapid response team (RRT) and correlated with serial vital signs, lactate clearance, ICU admission, and mortality. Results. Ninety-one patients were studied. Significantly higher HRV was observed in patients who achieved physiological stability and did not need ICU admission: ASDNN 19 versus 34.5, p=0.032; rMSSD 13.5 versus 25, p=0.046; mean VLF 9.4 versus 17, p=0.021; mean LF 5.8 versus 12.4, p=0.018; and mean HF 4.7 versus 10.5, p=0.017. ROC curves confirmed the change in very low frequencies at 2 hours as a strong predictor for ICU admission with an AUC of 0.772 (95% CI 0.633, 0.911, p=0.001) and a cutoff value of −0.65 associated with a sensitivity of 78.6% and a specificity of 61%. Conclusions. Reduced HRV, specifically VLF, appears closely related to greater severity of critical illness, identifies unsuccessful resuscitation, and can be used to identify consultations that need early ICU admission.

This study was conducted to investigate the hypothesis that patients using β-blockers will develop hearing loss.A cross-sectional study.A total of 125 patients completed the study. A total of 63 patients were on β-blockers and 62 were not on β-blockers.Carvedilol was significantly associated with hearing loss. Other beta-blockers including metoprolol and atenolol showed no association with hearing loss. Linear multiple regression analysis was run including variables of gender, age, ischaemic heart disease, cardiac failure/dilated cardiomyopathy, frusemide and carvedilol use as predictors for total hearing loss severity at all frequencies. Age and gender, as well as carvedilol, were found to be the only statistically significant predictors for hearing loss severity.Chronic use of carvedilol was associated with significant hearing loss. This may need to be taken into account when prescribing the drug. Further randomised controlled studies with baseline audiometric hearing tests before starting treatment, and periodic follow-up tests, would provide a better assessment of the effect of carvedilol on hearing.

Key Teaching Points • Heart failure is a common health care problem. • Patients with heart failure and narrow QRS complexes who are refractory to medical therapy represent a management challenge, as they are not candidates for cardiac resynchronization therapy. • Cardiac contractility modulation (CCM) therapy represents a promising therapeutic modality for these patients. • CCM is associated with increased maximal oxygen consumption and improved quality of life in patients with heart failure. • CCM may lead to left ventricular reverse remodeling, and it is expected to increase left ventricular ejection fraction (LVEF) by ~5%. • Patients with heart failure New York Heart Association functional class III and an LVEF of ≥25% respond exceptionally well to CCM therapy as suggested by subgroup analysis of large studies in this field.

Background Infection of cardiac implantable electronic devices is a serious cardiovascular disease and it is associated with a high mortality. Mycobacterium species may rarely cause cardiac implantable electronic devices infection. Case presentation We are reporting a case of miliary tuberculosis in an Arab patient with dilated cardiomyopathy and a cardiac resynchronization therapy-defibrillator device that was complicated with infection of his cardiac resynchronization therapy-defibrillator device. To our knowledge, this is the third case in the literature with such a presentation and all patients died during the course of treatment. This underscores the importance of early diagnosis and management. We also performed a literature review of reported cases of cardiac implantable electronic devices infection related to Mycobacterium species. Conclusions Cardiac implantable electronic devices infection due to Mycobacterium species is an uncommon but a well-known entity. Early diagnosis and prompt management may result in a better outcome.