Atrial Arrhythmias in Clinically Manifest Cardiac Sarcoidosis: Incidence, Burden, Predictors, and Outcomes.

Background Recent data have suggested a substantial incidence of atrial arrhythmias (AAs) in cardiac sarcoidosis (CS). Our study aims were to first assess how often AAs are the presenting feature of previously undiagnosed CS. Second, we used prospective follow-up data from implanted devices to investigate AA incidence, burden, predictors, and response to immunosuppression. Methods and Results This project is a substudy of the CHASM-CS (Cardiac Sarcoidosis Multicenter Prospective Cohort Study; NCT01477359). Inclusion criteria were presentation with clinically manifest cardiac sarcoidosis, treatment-naive status, and implanted with a device that reported accurate AA burden. Data were collected at each device interrogation visit for all patients and all potential episodes of AA were adjudicated. For each intervisit period, the total AA burden was obtained. A total of 33 patients met the inclusion criteria (aged 56.1±7.7 years, 45.5% women). Only 1 patient had important AAs as a part of the initial CS presentation. During a median follow-up of 49.1 months, 11 of 33 patients (33.3%) had device-detected AAs, and only 2 (6.1%) had a clinically significant AA burden. Both patients had reduced burden after CS was successfully treated and there was no residual fluorodeoxyglucose uptake on positron emission tomography scan. Conclusions First, we found that AAs are a rare presenting feature of clinically manifest cardiac sarcoidosis. Second, AAs occurred in a minority of patients at follow-up; the burden was very low in most patients. Only 2 patients had clinically significant AA burden, and both had a reduction after CS was treated. Registration URL: https://www.clini​caltr​ials.gov; unique identifier NCT01477359.

Sensitivity and specificity of chest imaging for sarcoidosis screening in patients with cardiac presentations.

BACKGROUND: Patients with sarcoidosis can present with cardiac symptoms as the first manifestation of disease in any organ. In these patients, the use of chest imaging modalities may serve as an initial screening tool towards the diagnosis of sarcoidosis through identification of pulmonary/mediastinal involvement; however, the use of chest imaging for this purpose has not been well studied. We assessed the utility of different chest imaging modalities for initial screening for cardiac sarcoidosis (CS). METHODS AND RESULTS: All patients were investigated with chest x-ray, chest computed tomography (CT) and/or cardiac/thorax magnetic resonance imaging (MRI). We then used the final diagnosis (CS versus no CS) and adjudicated imaging reports (normal versus abnormal) to calculate the sensitivity and specificity of individual and combinations of chest imaging modalities. We identified 44 patients (mean age 54 (±8) years, 35.4% female) and a diagnosis of CS was made in 18/44 patients (41%). The sensitivity and specificity for screening for sarcoidosis were 35% and 85% for chest x-ray, respectively (AUC 0.60; 95%CI 0.42-0.78; p value=0.27); 94% and 86% for chest CT (AUC 0.90; 95%CI 0.80-1.00; p value <0.001); 100% and 50% for cardiac/thorax MRI (AUC 0.75; 95%CI 0.56-0.94; p value=0.04). CONCLUSIONS: During the initial diagnostic workup of patients with suspected CS, chest x-ray was suboptimal as a screening test. In contrast CT chest and cardiac/thorax MRI had excellent sensitivity. Chest CT has the highest specificity among imaging modalities. Cardiac/thorax MRI or chest CT could be used as an initial screening test, depending on local availability.

Association of electrocardiographic morphology of exercise-induced ventricular arrhythmia with mortality.

BACKGROUND: The prognostic importance of exercise-induced ventricular arrhythmia (EIVA) may be confounded by the presence of lower-risk idiopathic right ventricular outflow tract arrhythmias with left bundle-branch block (LBBB) morphology. OBJECTIVE: To determine whether right bundle-branch block (RBBB)-morphology EIVA was associated with increased mortality. DESIGN: Retrospective cohort. SETTING: Academic medical center. PATIENTS: 585 unique patients with EIVA and 2340 patients without EIVA, matched by age, sex, and risk factor, who were referred for exercise testing in an academic medical center. MEASUREMENTS: Deaths and ischemia and infarction found on perfusion scan. RESULTS: During a mean follow-up of 24 months (SD, 13), 31 deaths occurred in the EIVA group compared with 43 deaths in the group without EIVA (5.3% vs. 1.8%; P < 0.001). Worse survival in patients with RBBB-morphology or multiple-morphology EIVA (6.9%) than in patients without EIVA caused this difference. Patients with LBBB-morphology EIVAs had a mortality rate (2.5%) similar to that of patients without EIVA (P = 0.93, log-rank test). Among patients without known atherosclerotic coronary artery disease, any RBBB-morphology EIVA was associated with death (hazard ratio, 2.73 [95% CI, 1.78 to 4.13]; P < 0.001), but LBBB-morphology EIVA was not (hazard ratio, 0.82 [CI, 0.18 to 2.04]; P = 0.72). LIMITATIONS: Not all LBBB-morphology EIVA can be dismissed, and not all RBBB-morphology EIVA is high risk. Further evaluation of patients for structural heart disease was clinically driven, not protocol-driven. CONCLUSION: Right bundle-branch block- or multiple-morphology EIVA is associated with increased mortality. Inclusion of patients with isolated LBBB-morphology EIVA, which often is idiopathic, may contribute to differences in the prognostic importance of EIVA in previous studies.

Sustained ventricular tachycardia associated with corrective valve surgery.

BACKGROUND: The causes of sustained monomorphic ventricular tachycardia (VT) after cardiac valve surgeries have not been studied extensively, although bundle-branch reentry has been reported. METHODS AND RESULTS: Records of 496 patients referred for electrophysiology study and catheter ablation of recurrent VT were reviewed. Twenty patients (4%) had VT after aortic or mitral valve surgery in the absence of known myocardial infarction. The median age was 53 years, and the median ejection fraction was 45%. In 4 patients, VT occurred early after surgery, and electrophysiology study was performed 3 to 10 days later. In the remaining patients, electrophysiology study was performed a median of 12 years (interquartile range 5 to 15 years) after surgery. Sustained VT was inducible in 17 patients. VT was attributed to scar-related reentry in 14 patients (70%) and to bundle-branch reentry in 2 (10%). Multiple VTs were present in 9 of 14 patients with scar-related reentry. A total of 42 induced VTs were targeted for ablation. Of the 14 patients with scar-related reentry, 9 (64%) had periannular scar, and 10 (71%) had an identifiable endocardial circuit isthmus. Ablation abolished 41 (98%) of the 42 targeted VTs. At a median follow-up of 2.1 years, 3 deaths occurred 8 to 14 months after ablation. One patient with incessant VT early after valve surgery suffered a stroke with residual hemianopsia. Of the 20 patients, 3 required repeat ablation after recurrence, and 2 of these who were not inducible during electrophysiology study had clinical recurrence that necessitated ablation. CONCLUSIONS: Sustained VT after valve surgery appears to be bimodal in presentation, occurring either early after surgery or years later. In this referral population, reentry in a region of scar is more common than bundle-branch reentry. Catheter ablation can be successful.

Myopotentials leading to ventricular fibrillation detection after advisory defibrillator generator replacement.

We present an unusual source of oversensing following an internal cardioverter-defibrillator generator change. The early appearance of reproducible myopotentials in the defibrillator sensing channel is usually due to a technical complication at the time of device implantation. Clues such as abrupt impedance change or reproduction with mechanical stimulation can help to localize a problem. Frequently the complication requires reoperation to examine the system. What do you do when everything seems to be working fine?

Pulmonary arterial embolization of pacemaker lead electrode tip.

Complications with extraction of abundant endovascular systems increase with time since implantation. As the number of implanted devices increases, successful management of complications needs to be disseminated. We present a 46-year-old woman with endovascular leads placed 15 years previously requiring extraction. Using laser-assistance the leads were removed, although the passive lead tips were unable to be extracted, and were retained in the superior vena cava. One lead tip embolized to the distal pulmonary bed within 24 hours of her operative procedure. Computed tomography and pulmonary arteriography suggested a near immediate thrombogenic process. A multidisciplinary approach was utilized to identify management strategies that allowed for a satisfactory patient outcome.