Mehta, Davendra

Cardiac sarcoidosis is a component of an often multiorgan granulomatous disease of still uncertain cause. It is being recognized with increasing frequency, mainly as the result of heightened awareness and new diagnostic tests, specifically cardiac magnetic resonance imaging and 18 F-fluorodeoxyglucose positron emission tomography scans. The purpose of this case-based review is to highlight the potentially life-saving importance of making the early diagnosis of cardiac sarcoidosis using these new tools and to provide a framework for the optimal care of patients with this disease. We will review disease mechanisms as currently understood, associated arrhythmias including conduction abnormalities, and atrial and ventricular tachyarrhythmias, guideline-directed diagnostic criteria, screening of patients with extracardiac sarcoidosis, and the use of pacemakers and defibrillators in this setting. Treatment options, including those related to heart failure, and those which may help clarify disease mechanisms are included.

Abstract Background Cardiac amyloidosis (CA) remains an underdiagnosed entity. Atrial fibrillation (AF) is common in patients with CA, likely owing to direct amyloid deposition in the left atrium. However, the prevalence of AF in CA and its association with in-hospital outcomes has not been studied in large populations. Purpose Our aim was to study the trends, baseline characteristics, and clinical impact of AF in patients with CA in the United States using the Healthcare Cost and Utilization Project (HCUP) National) Inpatient Sample (NIS) from 1999 to 2014. Methods We queried the NIS and identified CA using ICD-9-CM codes 277.39 and 425.7. AF in CA patients was identified using the ICD-9-CM code of 427.31. Statistical Analysis System (SAS) 9.4 was used for analysis of data. Results There were a total of 145,920 CA hospitalizations between 1999 and 2014 in the United States, of which 37,070 (25.4%) had AF. The prevalence of AF remained consistent from 27.5% in 1999 to 27.4% in 2014. The mean age of patients with AF was 72.9±28.2 years and for patients without AF was 67±31.9 years (p<0.0001). The majority of the patients with AF were male (60.3%) and the study group was predominantly white (62.8% in patients with AF and 56.4% in patients without AF). CA patients with AF suffered more from thyroid-related disease (22.5% vs 16.1%), heart failure (62.9% vs 36.5%) and renal failure (34.7% vs 30.5%) and less from hypertension (29.3% vs 34.0%) and diabetes mellitus (23.2% vs 25.2%) (p<0.0001). There was no significant difference in the Charlson comorbidity score between the groups. Inpatient mortality was significantly higher in CA patients with AF (10.4% vs 6.5%, p<0.001). However, in-patient mortality has been decreasing over the years from 10.3% in 1999 to 7.6% in 2014. Furthermore, cardiogenic shock was significantly higher in CA patients with AF (2.1% vs 1.2%, p<0.001), yet the use of mechanical circulatory support was not significantly different between the groups (0.42% vs 0.35%, p=0.375). Pacemaker implantation was also noted to be higher in CA patients with AF compared to patients without AF (2.8% vs 1.2%, p<0.0001). There was no significant difference between CA patients with and without AF in mean cost of hospitalization ($58222±10752 vs $57695±545, p=0.081) or length of stay (7.9±0.1 vs 7.7±0.1 days, p=0.7089). Conclusion CA with atrial fibrillation is a well-recognized entity, and our large scale retrospective analysis found significant association with worse in-hospital outcomes and cardiogenic shock. Interestingly, trend of in-patient mortality in CA has been decreasing over the years, likely owing to improved imaging modalities for diagnosis. Optimal management of AF in CA is imperative to improve outcomes in this population. Funding Acknowledgement Type of funding source: None

Abstract Background Orthotopic heart transplantation is the most effective long-term therapy for end-stage heart disease. Denervation of transplanted heart with the loss of autonomic modulation, vasculopathy, utilization of immunosuppressant drugs, and risk of allograft rejection may result in change in the prevalence of arrhythmias in transplanted hearts. Purpose To describe the trends, distribution and the clinical impact of arrhythmias in transplanted hearts in a large nationwide population. Methods We queried the National Inpatient Sample with administrative codes. Cardiac transplant patients were identified using procedure ICD-9-CM codes 37.5 and 33.6. Common arrhythmias were extracted using appropriate validated ICD-9-CM codes. Statistical Analysis System (SAS) version 9.4 was used for analysis of data. Results There was a total of 30,020 hospitalizations of heart transplant recipients between 1999 and 2014 in the United States and 16342 (54.4%) of these had arrhythmias. The prevalence of total arrhythmias increased from 53.6% (n=1,158) in 1999 to 67.3% (n=1,575) in 2014. The most common arrhythmia was atrial fibrillation (26.83%) followed by ventricular tachycardia (22.86%) and the prevalence of individual arrhythmias is as shown in Figure 1. Cardiogenic shock was higher in transplanted hearts with arrhythmias when compared with patients without arrhythmias (25.96% vs 18.18%; p<0.001). Transplant recipients with arrhythmias were also associated with an increased use of mechanical circulatory device (18.22% vs 12.67%, p<0.001). The use of implantable cardiac defibrillators and permanent pacemaker was also higher in the arrhythmia group (2.19% vs 0.63% and 40.43% vs 30.24% respectively, p<0.0001). However, there was no significant difference in inpatient mortality between transplant recipients with arrhythmias and without arrhythmias (7.72% vs 6.90%, p=0.225). Further, there was no significant difference in frequency of strokes between the groups (4.98% vs 5.08%; p=0.857). The total hospital cost when adjusted for inflation was significantly higher in the arrhythmic patients, with an average cost of about $570,415±9,590 vs $439,707±8362 in patients without arrhythmias (p<0.0001). The mean length of hospitalization was 44.2±0.8 days in patients with arrhythmias compared to 33.9±0.8 days in patients without arrhythmias (p<0.0001). Conclusion A significant proportion of patients with heart transplant have cardiac arrhythmias and are associated with worse in-hospital outcomes of cardiogenic shock, increased length of stay, and cost of hospitalization. However, they are not associated with worse inpatient mortality. Funding Acknowledgement Type of funding source: None

AbstractBackgroundPatients with cardiac amyloidosis (CA) have increased mortality, which can be explained in part by an increased risk of arrhythmias. The burden of arrhythmias in CA, their predictors, and impact on in‐hospital outcomes remains unclear. The role of implantable cardioverter‐defibrillators (ICD) in this population is also uncertain.MethodsWe queried the National Inpatient Sample (NIS) using ICD‐9‐CM codes 277.39 and 425.7 to identify CA. Twelve common arrhythmias were extracted using appropriate, validated ICD‐9‐CM codes. ICD implantation was identified using procedure ICD‐9 codes 37.94 to 37.98, 00.51 and 00.54.ResultsThere were a total of 145,920 CA hospitalizations between 1999 and 2014 in the United States and 56,199 (38.5%) of them were associated with arrhythmias. The prevalence of arrhythmias remained relatively constant from 41.5% in 1999 to 40.2% in 2014. The most common arrhythmia was atrial fibrillation (25.4%). In‐patient mortality was significantly higher in CA patients with arrhythmias (10.4% vs 6.5%, P < .001). ICD implantation was performed in 1,381 (0.94%) patients with CA and analysis revealed an incremental trend in implantation over the study period (0.48% in 1999 to 0.65% in 2014). In‐hospital mortality was significantly lower in patients who underwent ICD implantation (3.7% vs 8%; P = .0078). CA patients with arrhythmias also had an increased cost of hospitalization and length of stay ($65,046 ± 1,079 vs $53,322 ± 687 and 8.3 ± 0.1 vs 7.4 ± 0.1 days, respectively; P < .0001).ConclusionCardiac arrhythmias are common in patients with CA and are associated with worse in‐hospital outcomes, increased length of stay, and cost of hospitalization.