Risk of Adverse Outcomes Associated With Cardiac Sarcoidosis Diagnostic Schemes.

BACKGROUND: Multiple cardiac sarcoidosis (CS) diagnostic schemes have been published. OBJECTIVES: This study aims to evaluate the association of different CS diagnostic schemes with adverse outcomes. The diagnostic schemes evaluated were 1993, 2006, and 2017 Japanese criteria and the 2014 Heart Rhythm Society criteria. METHODS: Data were collected from the Cardiac Sarcoidosis Consortium, an international registry of CS patients. Outcome events were any of the following: all-cause mortality, left ventricular assist device placement, heart transplantation, and appropriate implantable cardioverter-defibrillator therapy. Logistic regression analysis evaluated the association of outcomes with each CS diagnostic scheme. RESULTS: A total of 587 subjects met the following criteria: 1993 Japanese (n = 310, 52.8%), 2006 Japanese (n = 312, 53.2%), 2014 Heart Rhythm Society (n = 480, 81.8%), and 2017 Japanese (n = 112, 19.1%). Patients who met the 1993 criteria were more likely to experience an event than patients who did not (n = 109 of 310, 35.2% vs n = 59 of 277, 21.3%; OR: 2.00; 95% CI: 1.38-2.90; P < 0.001). Similarly, patients who met the 2006 criteria were more likely to have an event than patients who did not (n = 116 of 312, 37.2% vs n = 52 of 275, 18.9%; OR: 2.54; 95% CI: 1.74-3.71; P < 0.001). There was no statistically significant association between the occurrence of an event and whether a patient met the 2014 or the 2017 criteria (OR: 1.39; 95% CI: 0.85-2.27; P = 0.18 or OR: 1.51; 95% CI: 0.97-2.33; P = 0.067, respectively). CONCLUSIONS: CS patients who met the 1993 and the 2006 criteria had higher odds of adverse clinical outcomes. Future research is needed to prospectively evaluate existing diagnostic schemes and develop new risk models for this complex disease.

Outcomes of prednisone-tapering regimens for cardiac sarcoidosis: A retrospective analysis demonstrating a benefit of infliximab.

BACKGROUND: The optimal treatment strategy for cardiac sarcoidosis has not been standardized. We examined the effectiveness of three prednisone-tapering treatment regimens for cardiac sarcoidosis. METHODS: We retrospectively reviewed prednisone-tapering treatment regimens for cardiac sarcoidosis that contained prednisone alone (P), prednisone plus methotrexate (P-M), and prednisone plus infliximab containing regimens (P-I). We defined the success of each regimen as the ability to lower the daily prednisone dose to 7.5 mg or less for 6 or more months without developing an adverse cardiac event. We also examined the lowest effective daily prednisone dose achieved without developing an adverse cardiac event. RESULTS: We identified 61 treatment regimens in 33 cardiac sarcoidosis patients that were analyzed. The success rate of prednisone-tapering regimens was significantly different P: 8/30, 27%; P-M: 3/23, 13%; P-I: 6/8, 75%., p = 0.04. The lowest effective daily prednisone dose for the regimens was also significantly different: P: 14.1 ± 10.1 mg; P-M: 16.9 ± 9.4 mg; infliximab: 7.8 ± 4.9 mg, (p = 0.03); by both measures the success was greatest with the P-I regimen. CONCLUSIONS: For the treatment of cardiac sarcoidosis, prednisone-tapering regimens containing infliximab are superior to those containing prednisone alone or prednisone plus methotrexate in terms of reaching 7.5 mg/day of prednisone for more than 6 months and achieving the lowest effective prednisone.

Oral Anticoagulation for Atrial Fibrillation Thromboembolism Prophylaxis in the Chronic Kidney Disease Population: the State of the Art in 2019.

Atrial fibrillation (AF) is the most common cardiac rhythm disturbance and is associated with increased risk of thromboembolism. Oral anticoagulants are effective at reducing rates of thromboembolism in patients with AF in the general population. Patients with AF and concurrent chronic kidney disease (CKD) have higher risk of thromboembolism and bleeding compared with patients with normal renal function. Among moderate CKD and end-stage renal disease (ESRD) patients on chronic dialysis, the use of oral anticoagulants is controversial. Use of warfarin, while beneficial in non-CKD patients, raises a number of concerns such as increased bleeding risk, labile anticoagulant effect, and calciphylaxis, especially in the ESRD population. The newer direct oral anticoagulant (DOAC) agents have demonstrated comparable efficacy and improved safety profiles compared with coumadin but are not as well studied in the CKD population. This review highlights the efficacy and safety of coumadin and the DOACs for thromboembolism prophylaxis in non-valvular AF patients with CKD.

The association of QRS duration with atrial fibrillation in a heart failure with preserved ejection fraction population: a pilot study.

BACKGROUND: Heart failure is a significant cause of morbidity and mortality, yet patient risk stratification may be difficult. Prevention or treatment of atrial fibrillation (AF) may be an important strategy in these patients that could positively affect their outcome. It has been demonstrated that in patients with systolic dysfunction, prolonged QRS duration (QRSd), an easily measured electrocardiographic parameter, is associated with AF. HYPOTHESIS: Prolonged QRSd is associated with an increase in prevalence of AF in patients with heart failure with preserved ejection fraction(HFPEF). METHODS: Between February 2006 and February 2009, 718 patients were discharged with a diagnosis of HF from the Dartmouth-Hitchcock Medical Center. Of these, 206 had EF ≥50% by echocardiography performed within 72 hours of admission. After exclusions, 82 patients remained, of which 25 had AF and 57 had sinus rhythm. Characteristics of the AF and sinus-rhythm patients were compared in this pilot study. RESULTS: After adjustment for age, prior diagnosis of HF, and left atrial area, there was a nonsignificant trend (odds ratio: 2.2, 95% CI of 0.3-17.2) for a QRSd >120 ms to be associated with AF. CONCLUSIONS: Similar to results in patients with systolic dysfunction, patients with preserved EF may have an association between a prolonged QRSd and AF.

Impact of early initiation of corticosteroid therapy on cardiac function and rhythm in patients with cardiac sarcoidosis.

BACKGROUND: There is limited data on the effect of corticosteroid therapy in patients with cardiac sarcoidosis (CS). We sought to examine the impact of early initiation of corticosteroid therapy, within a month of CS diagnosis, on left ventricular ejection fraction (LVEF), ventricular arrhythmias (VAs), and atrioventricular (AV) block. METHODS: We retrospectively identified 30 CS patients from a large university sarcoidosis clinic. The effect of early initiation of corticosteroid therapy on LVEF was assessed by serial echocardiography, and on VAs and AV block was assessed by Holter monitoring and/or device interrogations. RESULTS: The median time from diagnosis of extra-cardiac sarcoidosis to CS was 40months. 90% (27/30) of the CS patients received corticosteroid therapy and 85% percent (23/27) had early initiation of corticosteroid therapy. Fourteen patients (47%) had reduced EF<50%. 9/14 patients who had early initiation of corticosteroid therapy had improvement in mean EF (25% to 46%, P<0.001); 5/14 patients who had a delay in initiation or who did not receive corticosteroids had no improvement in mean EF (41% to 37%, P=0.47). Fourteen patients (47%) had VAs and 5 patients (17%) had advanced AV block. Early initiation of corticosteroid therapy resulted in no VA recurrences in 8/11 patients (72%), and complete recovery of AV conduction in 2/3 patients (67%). Patients with VAs (n=3) or advanced AV block (n=2) who failed to receive early corticosteroid therapy did not show improvement. CONCLUSIONS: There is often a delay in manifestation of cardiac sarcoidosis for several years from the diagnosis of extra-cardiac sarcoidosis. Prompt initiation of corticosteroid therapy in CS patients may improve outcomes whereas delayed initiation of corticosteroids or failure to use corticosteroids may be associated with worse outcomes.

Non-sustained wide complex tachycardia: an underappreciated sign to aid in diagnosis.

AIMS: The electrocardiographic (ECG) signs used to differentiate ventricular tachycardia (VT) from supraventricular tachycardia (SVT) with aberrancy are specific but not highly sensitive. The purpose of this study was to define the utility of an underappreciated form of atrioventricular (AV) dissociation at the onset of tachycardia, a single dissociated P wave, in the differentiation of non-sustained monomorphic wide complex tachycardia (WCT) in hospitalized patients. METHODS AND RESULTS: We prospectively analysed tracings from 102 consecutive hospitalized patients who had an episode of non-sustained (≥5 beats, <30 s), monomorphic, WCT (≥100 b.p.m.) on telemetry. WCT was classified as VT, SVT with aberrancy, or undifferentiated WCT based on predefined criteria. Of 102 patients with WCT, 3 (3%) had SVT with aberrancy, 43 (42%) had an undifferentiated WCT, and 56 (55%) had VT. ECG evidence of a single dissociated P wave at the onset of tachycardia (i.e. AV dissociation at the onset) was identified in 29 patients (28%) compared with less frequent traditional signs of VT including second-degree ventriculoatrial (VA) block in 18 patients (18%), AV dissociation during tachycardia in 17 patients (17%), fusion beats in 10 patients (10%), and capture beats in 3 patients (3%). On multivariate analysis, only the prematurity index predicted the occurrence of AV dissociation at the onset of the tachycardia (odds ratio 1.239, 95% confidence interval 1.033-1.486, P = 0.021). CONCLUSION: When evaluating WCT in hospitalized patients, a single dissociated P wave at the onset of tachycardia is an easily recognizable diagnostic sign of VT, and is observed more frequently than the other accepted criteria for VT.

Lyme Carditis in the Fast Lane: From Alternating Bundle Branch Block to Asystole in 12 Hours.

Lyme borreliosis is a multisystem infectious disease with well-known cardiac involvement, including potential carditis as well as conduction abnormalities. We report a case of Lyme disease in a previously healthy 24-year-old male presenting with alternating right- and left-bundle branch block, indicating infra-Hisian atrioventricular (infra-His) block with an accelerated fascicular escape rhythm. Inless than 12 hours, the conduction abnormalities progressed to asystole requiring the urgent placement of a temporary transvenous pacemaker. Subsequently, with appropriate antibiotic treatment, the patient's conduction abnormalities resolved in a week without the need for a permanent pacemaker.

Pathophysiology and clinical management of cardiac sarcoidosis.

Cardiac sarcoidosis is a potentially life-threatening condition characterized by formation of granulomas in the heart, resulting in conduction disturbances, atrial and ventricular arrhythmias, and ventricular dysfunction. The presentation of cardiac sarcoidosis ranges from asymptomatic with an abnormal imaging scan, to palpitations, syncope, symptoms of congestive heart failure, and sudden cardiac death. Screening for cardiac sarcoidosis has not been standardized, but the presence of cardiac symptoms on medical history and physical examination, and an abnormal electrocardiogram (ECG), Holter monitoring, or echocardiogram has been shown to be highly sensitive for detecting cardiac sarcoidosis. A signal-averaged ECG might also have a role in screening for cardiac sarcoidosis in asymptomatic patients. Although endomyocardial biopsies are highly specific for the diagnosis of cardiac sarcoidosis, procedural yield is very low and appropriate findings on cardiac MRI or PET are, therefore, often used as diagnostic surrogates. Treatment for cardiac sarcoidosis usually involves immunosuppressive therapy, particularly corticosteroids. Additional therapy might be required, depending on the clinical presentation, including implantation of an internal defibrillator, antiarrhythmic agents, and catheter ablation.

In-hospital complications associated with reoperations of implantable cardioverter defibrillators.

Repeat implantable cardioverter defibrillator (ICD) procedures are increasing and may be associated with higher risks for complications. To provide more information for clinical decision making, especially in light of recent defibrillator advisories, we examined a large national cohort to characterize repeat ICD procedural outcomes. Using data from the National Cardiovascular Data Registry (ICD Registry), we compared patient characteristics, reasons for ICD implantation, and associated in-hospital adverse events among 92,751 patients receiving their first device and 81,748 patients who underwent repeat procedures with (n = 31,057) and without (n = 50,691) lead involvement. Hierarchical multivariable logistic regression was used to determine the predictors of in-hospital complications. Complication rates were higher in those who underwent repeat ICD procedures with lead involvement (lead implantation or revision), compared with patients who underwent initial implants (3.2% vs 2.6%, p <0.001) or versus those with pocket-only (e.g., generator change only) procedures (3.2% vs 0.6%, p <0.001). There were significantly more in-hospital deaths, lead dislodgements, and infections requiring antibiotics in the lead involvement cohort. Compared with those who had a pocket-only procedure, the multivariable adjusted odds ratio of any complication were increased at 4.20 (95% confidence interval: 3.66 to 4.82, p <0.001) in patients who underwent repeat procedures with lead involvement excluding lead extraction or 7.11 (95% confidence interval: 5.96 to 8.48, p <0.001) in procedures involving lead extractions. In conclusion, repeat ICD procedures, when involving the addition or revision of a lead with or without concurrent lead extraction, are associated with higher complication rates compared with initial implants and with those who underwent pocket-only procedures.

Utility of cardiac magnetic resonance imaging to differentiate cardiac sarcoidosis from arrhythmogenic right ventricular cardiomyopathy.

Some patients diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) are eventually found to have cardiac sarcoidosis (CS). Accurate differentiation between these 2 conditions has implications for immunosuppressive therapy and familial screening. We sought to determine whether cardiac magnetic resonance imaging (MRI) could be used to identify the characteristic findings to accurately differentiate between CS and ARVC. Consecutive patients with a diagnostic MRI scan indicating CS and/or ARVC constituted the cohort. All patients diagnosed with CS had histologic confirmation of sarcoidosis, and all patients with ARVC met the diagnostic task force criteria. The cardiac MRI data were retrospectively analyzed to identify possible differentiating characteristics. Of the patients, 40 had CS and 21 had ARVC. Those with CS were older and had more left ventricular scar. The presence of mediastinal lymphadenopathy or left ventricular septal involvement was seen exclusively in the patients with CS (p <0.001). A family history of sudden cardiac death was seen only in the ARVC group (p = 0.012). The right ventricular ejection fraction and ventricular volumes were also significantly different between the 2 groups. In conclusion, patients with CS have significantly different cardiac MRI characteristics than patients with ARVC. The cardiac volume, in addition to the degree and location of cardiac involvement, can be used to distinguish between these 2 disease entities. The presence of mediastinal lymphadenopathy and left ventricular septal scar favors a diagnosis of CS and not ARVC. Consideration of CS should be given if these MRI findings are observed during the evaluation for possible ARVC.

Normal intima-media thickness on carotid ultrasound reliably excludes an ischemic cause of cardiomyopathy.

BACKGROUND: Coronary artery disease (CAD) is the most common cause of left ventricular systolic dysfunction (LVSD). Patients with ischemia as the cause of LVSD may warrant revascularization. Angiography is the most accurate method of CAD diagnosis but is invasive, expensive, and associated with some risk. Noninvasive imaging for CAD often involves expensive equipment, radiation exposure, medication, and/or contrast administration. Carotid ultrasound with measurement of intima-media thickness (IMT) is safe and inexpensive. Carotid IMT is well correlated with the presence of CAD. We assessed the accuracy of carotid ultrasound for identification of CAD as a potential etiology of LVSD. METHODS: Patients with LVSD (ejection fraction < or =40%) of uncertain etiology referred for angiography underwent carotid ultrasound. Patients with history of myocardial infarction were excluded. Two experienced cardiologists blinded to CAD status determined common carotid artery (CCA) IMT and plaque. Significant CAD was defined as > or =50% stenosis of any major artery. Ischemic LVSD was defined as (1) left main and/or proximal left anterior descending coronary artery > or =75% or (2) > or =2 major arteries with > or =75% stenosis. RESULTS: Mean ejection fraction was 27% +/- 10% in 150 patients. Significant CAD was found in 64 (42.7%) and ischemic LVSD in 40 (26.7%). Carotid plaque was seen in 95 (63.3%). Mean CCA IMT was > or =0.9 mm in 69 (46.0%). The combination of mean CCA IMT <0.9 mm and no plaque had negative predictive value for ischemic LVSD of 98%. CONCLUSIONS: Carotid ultrasound with IMT measurement is a valuable screening tool for excluding an ischemic etiology of LVSD when CAD is suspected.

Severe hypophosphatemia associated with gallstone pancreatitis: a case report and review of the literature.

Severe hypophosphatemia (<1.0 mg/dl) is a rarely reported complication of acute pancreatitis; when it does occur, it is typically attributed to alcohol abuse rather than the pancreatitis itself (1-5). In the literature, pancreatitis is not cited as a cause of hypophosphatemia (5, 6-16). Both pancreatitis and hypophosphatemia have widespread ramifications on human physiology, affecting hematologic, neural, hepatic, endocrine, respiratory, and renal systems. Given the possible synergistic consequences of pancreatitis and low serum phosphate, we emphasize the importance of recognizing hypophosphatemia as a complication of pancreatic inflammation.Herein, we report a case of acute pancreatitis unrelated to alcohol abuse associated with severe hypophosphatemia and review the pathophysiology.

Genetics of vascular anomalies: an update.

Vascular anomalies arise from genetic, environmental, mechanical, and/or hormonal factors. Some are inherited in a Mendelian fashion whereas others result from abnormal chromosomal segregation during gametogenesis or appear sporadically during various stages of life. Understanding the molecular basis of vascular development and vascular anomalies provides potential tools for diagnosis and treatment of the diseases.