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Background: There are few data on sex differences in suspected cardiac sarcoidosis. Methods: Consecutive patients with histologically proven sarcoidosis and suspected cardiac involvement were studied. We investigated sex differences in presenting features, cardiac involvement, and the long-term incidence of a primary composite end point of all-cause death or significant ventricular arrhythmia and secondary end points of all-cause death and significant ventricular arrhythmia. Results: Among 324 patients, 163 (50.3%) were female and 161 (49.7%) were male patients. Female patients had a greater prevalence of chest pain (37.4% versus 23.6%; P =0.010) and palpitations (39.3% versus 26.1%; P =0.016) than male patients but not dyspnea, presyncope, syncope, or arrhythmias at presentation. Female patients had a lower prevalence of late gadolinium enhancement on cardiovascular magnetic resonance imaging (20.2% versus 35.4%; P =0.003) and less often met criteria for a clinical diagnosis of cardiac sarcoidosis (Heart Rhythm Society consensus criteria, 22.7% versus 36.0%; P =0.012 and 2016 Japanese Circulation Society guideline criteria, 8.0% versus 19.3%; P =0.005), indicating lesser cardiac involvement. However, the long-term incidence of all-cause death or significant ventricular arrhythmia was not different between female and male patients (23.2% versus 23.2%; P =0.46). Among the secondary end points, the incidence of all-cause death was not different between female and male patients (20.7% versus 14.3%; P =0.51), while female patients had a lower incidence of significant ventricular arrhythmia compared with male patients (4.3% versus 13.0%; P =0.022). On multivariable analyses, sex was not associated with the primary end point (hazard ratio for female patients, 1.36 [95% CI, 0.77–2.43]; P =0.29). Conclusions: We observed distinct sex differences in patients with suspected cardiac sarcoidosis. A paradox was identified wherein female patients had a greater prevalence of chest pain and palpitations than male patients, but lesser cardiac involvement, and a similar long-term incidence of all-cause death or significant ventricular arrhythmia.

Patients with congenital heart disease have unique pacing challenges and often require epicardial systems. High pacing threshold is one of the major factors that decrease these systems' durability. Sometimes surgical reintervention or lead replacement is difficult or prohibitive in these patients due to repeated surgeries and extensive epicardial scar. We present a case report of a patient with Fontan physiology and chronically high epicardial lead pacing thresholds requiring frequent generator changes and a unique solution focusing on improving generator longevity.

Background: The association between ventricular arrhythmias (VAs) and mortality in patients supported by continuous flow left ventricular assist devices (LVAD) remains controversial. Objective: To evaluate the association between pre-implantation, early (≤30 day) post-implantation VAs and mortality in bridge to transplant (BTT) and destination therapy (DT) LVAD patients, separately. The risk factors for post LVAD VAs were also investigated.Methods: In this observational cohort study, we included 341 patients who received a first time, continuous flow LVAD between January 1st 2010 and July 30th 2018. We used Kaplan Meier curves and multivariable cox regression analyses to study the association between VAs and mortality in the BTT and DT populations.Results: The mean age of the cohort was 58 ± 14 years, with 82% males, 53% had ischemic cardiomyopathy, and 45% were DT. The mean follow-up was 2.2 ± 2.1 years. In both BTT and DT cohorts, pre LVAD VAs were not associated with mortality after LVAD implantation (log-rank p=0.95 and p=0.089, respectively). In the BTT population, early post-LVAD VAs were not statistically associated with increased mortality (log rank p=0.072). In the DT patients, early post LVAD VAs were associated with a 67 % increase in the hazards rate of mortality on LVAD support (HR 1.67 [1.05-2.65], p=0.029). The final model was adjusted for type of cardiomyopathy, INTERMACS profile, glomerular filtration rate, post LVAD atrial fibrillation, age and cerebrovascular events.Conclusion: Early post-LVAD VA is common after LVAD implantation and is an independent predictor of mortality in the DT LVAD population.

Introduction: Cardiac sarcoidosis is increasingly recognized as a cause of cardiomyopathy and mortality. However, there are no data on sex differences in patients with suspected cardiac sarcoidosis. Hypothesis: We hypothesized that sex differences exist in the clinical presentation, cardiac involvement, and long-term clinical outcomes of patients with suspected cardiac sarcoidosis. Methods: We performed a retrospective cohort study to examine sex differences in presenting features, CMR findings, and the long-term incidence of adverse clinical outcomes among consecutive patients with histologically proven sarcoidosis and suspected cardiac involvement investigated by cardiovascular magnetic resonance imaging (CMR). The primary composite clinical endpoint was all-cause mortality or significant ventricular arrhythmia. The secondary endpoints were all-cause mortality and significant ventricular arrhythmia. Results: Among 324 patients, 163 (50.3%) were women and 161 (49.7%) were men. Women reported a greater prevalence of chest pain and palpitations than men, but not dyspnea, presyncope, or syncope. Women were less likely to have LGE or meet the criteria for a clinical diagnosis of cardiac sarcoidosis, indicating lesser cardiac involvement. The long-term incidence of the primary composite endpoint (hazard ratio for women 1.36; 95% confidence interval 0.77-2.43; p = 0.29; Figure 1 ) or the cause of death between women and men (p = 0.62). However, women had a significantly lower cumulative incidence of significant ventricular arrhythmia compared with men (4.3% vs. 13.0%; log-rank p = 0.022). Conclusions: There were distinct sex differences in patients with histologically proven sarcoidosis and suspected cardiac involvement. A paradox was noted wherein women had a greater prevalence of chest pain and palpitations than men, but had lesser cardiac involvement, and a similar long-term incidence of all-cause death or significant ventricular arrhythmia.

This study aimed to determine the prevalence on cardiac magnetic resonance (CMR) of right ventricular (RV) systolic dysfunction and RV late gadolinium enhancement (LGE), their determinants, and their influences on long-term adverse outcomes in patients with sarcoidosis.In patients with sarcoidosis, RV abnormalities have been described on many imaging modalities. On CMR, RV abnormalities include RV systolic dysfunction quantified as an abnormal right ventricular ejection fraction (RVEF), and RV LGE.Consecutive patients with biopsy-proven sarcoidosis who underwent CMR for suspected cardiac involvement were studied. They were followed for 2 endpoints: all-cause death, and a composite arrhythmic endpoint of sudden cardiac death or significant ventricular arrhythmia.Among 290 patients, RV systolic dysfunction (RVEF 40% in men and 45% in women) and RV LGE were present in 35 (12.1%) and 16 (5.5%), respectively. The median follow-up time was 3.2 years (interquartile range [IQR]: 1.6 to 5.7 years) for all-cause death and 3.0 years (IQR: 1.4 to 5.5 years) for the arrhythmic endpoint. On Cox proportional hazards regression multivariable analyses, only RVEF was independently associated with all-cause death (hazard ratio [HR]: 1.05 for every 1% decrease; 95% confidence interval [CI]: 1.01 to 1.09; p = 0.022) after adjustment for left ventricular EF, left ventricular LGE extent, and the presence of RV LGE. RVEF was not associated with the arrhythmic endpoint (HR: 1.01; 95% CI: 0.96 to 1.06; p = 0.67). Conversely, RV LGE was not associated with all-cause death (HR: 2.78; 95% CI: 0.36 to 21.66; p = 0.33), while it was independently associated with the arrhythmic endpoint (HR: 5.43; 95% CI: 1.25 to 23.47; p = 0.024).In this study of patients with sarcoidosis, RV systolic dysfunction and RV LGE had distinct prognostic associations; RV systolic dysfunction but not RV LGE was independently associated with all-cause death, whereas RV LGE but not RV systolic dysfunction was independently associated with sudden cardiac death or significant ventricular arrhythmia. These findings may indicate distinct implications for the management of RV abnormalities in sarcoidosis.

Background: Implantable cardioverter-defibrillators are used to prevent sudden cardiac death in patients with cardiac sarcoidosis. The most recent recommendations for implantable cardioverter-defibrillator implantation in these patients are in the 2017 American Heart Association/American College of Cardiology/Heart Rhythm Society Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death. These recommendations, based on observational studies or expert opinion, have not been assessed. We aimed to assess them. Methods: We performed a large retrospective cohort study of patients with biopsy-proven sarcoidosis and known or suspected cardiac sarcoidosis that underwent cardiovascular magnetic resonance imaging. Patients were followed for a composite end point of significant ventricular arrhythmia or sudden cardiac death. The discriminatory performance of the Guideline recommendations was tested using time-dependent receiver operating characteristic analyses. The optimal cutoff for the extent of late gadolinium enhancement predictive of the composite end point was determined using the Youden index. Results: In 290 patients, the class I and IIa recommendations identified all patients who experienced the composite end point during a median follow-up of 3.0 years. Patients meeting class I recommendations had a significantly higher incidence of the composite end point than those meeting class IIa recommendations. Left ventricular ejection fraction (LVEF) >35% with >5.7% late gadolinium enhancement on cardiovascular magnetic resonance imaging was as sensitive as and significantly more specific than LVEF >35% with any late gadolinium enhancement. Patients meeting 2 class IIa recommendations, LVEF >35% with the need for a permanent pacemaker and LVEF >35% with late gadolinium enhancement >5.7%, had high annualized event rates. Excluding 2 class IIa recommendations, LVEF >35% with syncope and LVEF >35% with inducible ventricular arrhythmia, resulted in improved discrimination for the composite end point. Conclusions: We assessed the Guideline recommendations for implantable cardioverter-defibrillator implantation in patients with known or suspected cardiac sarcoidosis and identified topics for future research.

A 65 year-old man with a history of type 1 diabetes previously treated with an auto islet cell transplant presented with acute kidney injury (creatinine 1.5 mg/dl) and asymptomatic hyperkalemia (K+, 6.8 mEq/l). The electrocardiogram revealed >2 mm coved ST-segment elevation with T-wave inversion in