Epidemiology and risk of psychiatric disorders among patients with celiac disease: A population-based national study.

BACKGROUND AND AIM: Celiac disease (CD) is a chronic disorder resulting from an immune reaction to gluten in genetically predisposed individuals. Although several studies have linked CD to psychiatric diseases, there are limited data on this topic. Using a large database, we sought to describe the epidemiology of several psychiatric disorders in CD. METHODS: We queried a multicenter database (Explorys Inc), an aggregate of electronic health record data from 26 major integrated healthcare systems from 2016 to 2020 consisting of 360 hospitals in the USA. A cohort of patients with a Systematized Nomenclature Of Medicine - Clinical Terms diagnosis of CD was identified. Multivariate analysis was performed using Statistical Package for Social Sciences version 25. RESULTS: Of the 37 465 810 patients in the database between 2016 and 2020, there were 112 340 (0.30%) individuals with CD. When compared with patients with no history of CD, patients with CD were more likely to have a history of anxiety (odds ratio [OR]: 1.385; 95% confidence interval [CI]: 1.364-1.407), depression (OR: 1.918; 95% CI: 1.888-1.947), bipolar (OR: 1.321; 95% CI: 1.289-1.354), attention-deficit hyperactivity disorder (OR: 1.753; 95% CI: 1.714-1.792), eating disorder (OR: 15.84; 95% CI: 15.533-16.154), and childhood autistic disorder (OR: 4.858; 95% CI: 3.626-6.508). Patients with CD and psychiatric conditions were more likely to be smokers, with history of alcohol and substance abuse as well as a history of personality disorder. CONCLUSIONS: In this large database, patients with CD are at increased risk of having multiple psychiatric diseases including anxiety, depression, bipolar, attention-deficit hyperactivity disorder, eating disorder, and childhood autism. Individual care and referral to psychiatry when appropriate are warranted while taking care of this group of patients.

SERUM LEVELS OF CARCINOEMBRYONIC ANTIGEN IN PATIENTS WITH TYPE 2 DIABETES.

OBJECTIVE: To investigate whether serum carcinoembryonic antigen (CEA) levels are associated with type 2 diabetes mellitus (T2DM) and glycated hemoglobin (HbA1c). METHODS: A comparative, cross-sectional, observational study was conducted at Jordan University Hospital, Amman, Jordan, on 282 adult subjects from March 2012 to June 2015. Subjects were classified into 2 groups: T2DM subjects (n = 168) and a healthy comparison group (n = 114). Subjects with any condition known to be associated with elevated CEA levels were excluded. HbA1c and serum CEA levels were measured, and body mass index (BMI) was determined. RESULTS: Subjects with T2DM had significantly higher mean serum CEA than controls (2.4 ± 1.5 vs. 1.5 ± 1.2 ng/mL, P<.0001). Sex did not correlate with CEA levels, while age (Spearman's rho [ρ] = 0.18, P = .002) and HbA1c (ρ = 0.56, P<.0001) did; however, age no longer correlated after correcting for diabetic status. HbA1c was the only variable shown to correlate with CEA in a stepwise linear regression (r = 0 .37, P<.001). CONCLUSION: We observed a statistically significant association between elevated CEA and T2DM, despite average CEA values for both groups being within the reference range. In addition, serum CEA levels correlated positively with HbA1c values. ABBREVIATIONS: ADA = American Diabetes Association BMI = body mass index CA 19-9 = carbohydrate antigen 19-9 CEA = carcinoembryonic antigen CRP = C-reactive protein DM = diabetes mellitus HbA1c = glycated hemoglobin JUH = Jordan University Hospital T2DM = type 2 diabetes mellitus ρ = Spearman's correlation coefficient.

Cancer and Cardiovascular Disease: The Conjoined Twins.

Cancer and cardiovascular disease are the two most common causes of death worldwide. As the fields of cardiovascular medicine and oncology continue to expand, the area of overlap is becoming more prominent demanding dedicated attention and individualized patient care. We have come to realize that both fields are inextricably intertwined in several aspects, so much so that the mere presence of one, with its resultant downstream implications, has an impact on the other. Nonetheless, cardiovascular disease and cancer are generally approached independently. The focus that is granted to the predominant pathological entity (either cardiovascular disease or cancer), does not allow for optimal medical care for the other. As a result, ample opportunities for improvement in overall health care are being overlooked. Herein, we hope to shed light on the interconnected relationship between cardiovascular disease and cancer and uncover some of the unintentionally neglected intricacies of common cardiovascular therapeutics from an oncologic standpoint.

Immune checkpoint inhibitor induced myocarditis, myasthenia gravis, and myositis: A single-center case series.

BACKGROUND: Immune checkpoint inhibitors can result in overlap syndrome comprised of myasthenia gravis, myositis and myocarditis. However, the mortality predictors have not been clearly delineated. METHODS: We examined the characteristics of 11 patients diagnosed with overlap syndrome at Cleveland Clinic. All the available clinical, diagnostic, biochemical and disease specific factors were examined. Clinical predictors of increased mortality were using student t-test for parametric data and Wilcoxon-signed rank testing for nonparametric data. RESULTS: Seven patients out of eleven patients were alive during the analysis. Our study did confirm that troponins were indicator of early demise. However, study showed that elevated creatinine, BUN, and decreased hemoglobin were also observed in patients who met early demise. Unlike previously published studies, elevated NT Pro-BNP and reduced left ventricular ejection fraction were not a seen in this study. However, there were higher incidence of electrical abnormalities in deceased patients when compared to alive. CONCLUSION: Our study is first to examine various clinical parameters of overlap syndrome that might be predictive of mortality. This study confirms troponin as possible predictor and adds elevated creatinine, BUN and reduced hemoglobin as possible early biomarkers in deceased patients. The analysis showed that reduced LVEF was not a seen in deceased patients.

Spontaneous Coronary Artery Dissection Case Series: Clinical Presentation, Angiographic Imaging, Management, and Outcomes.

Spontaneous coronary artery dissection (SCAD) is a rare but more frequently recognized cause of acute coronary syndrome. Due to the low incidence rate of this condition, there are no consensus guidelines to treat this clinical entity. Most of the information on the clinical presentation and management of SCAD originates from case reports and series; however, there has not been yet a prospective randomized study to guide treatment of SCAD. It is mostly seen in women and particularly those of younger ages. Most cases have an underlying etiology although the underlying pathophysiology is not fully understood. Patients might present in various ways including chest pain, ST- elevation acute coronary syndrome, ventricular arrhythmia, and sudden cardiac death. Although conservative management is mostly recommended with better outcomes, revascularization should be considered in certain circumstances. In this article, we describe a case series of five patients with SCAD. Additionally, this article reviews clinical presentation, incidence, diagnostic modalities, management and risk of recurrences in patients diagnosed with SCAD.

Ischemic or Coronary Evaluations in Patients With Monomorphic VT Electrical Storm Undergoing VT Ablation.

BACKGROUND: Monomorphic ventricular tachycardia (VT) electrical storm (ES) in patients with coronary artery disease is dependent on scarred myocardium. The role of routine ischemic or coronary evaluations before ablation in patients presenting with monomorphic VT storm, without acute coronary syndrome (ACS), remains unknown. OBJECTIVES: This study sought to assess the impact of ischemic or coronary evaluations on procedural outcomes and post-ablation mortality in monomorphic VT storm patients. METHODS: All patients undergoing VT ablation at the Cleveland Clinic from 2014 to 2020 after presenting with monomorphic VT storm were enrolled in a prospectively maintained registry. The associations among ischemic or coronary evaluations and short-term procedural efficacy, acute outcomes, and mortality during follow-up were assessed. RESULTS: A total of 97 consecutive patients with monomorphic VT storm in the absence of ACS underwent VT ablations. This cohort was characterized by severe LV systolic dysfunction (mean left ventricular ejection fraction 30.3%, 67% with known ischemic cardiomyopathy) with moderately severe heart failure (median NYHA functional class II); 45% of patients underwent ischemic or coronary evaluations via coronary angiography (10%), noninvasive myocardial perfusion (26%), or both (9%). The yield of these evaluations was low: No acute coronary occlusions were identified. There was no association between ischemic evaluation and acute ablation outcomes or mortality during follow-up. Similarly, in a secondary analysis, the yield of ischemic or coronary evaluations in patients with monomorphic VT storm and known coronary disease (regardless of ablation status) was found to be low. CONCLUSIONS: Ischemic evaluations in patients with monomorphic VT storm without ACS may not improve procedural outcomes or mortality after ablation.

Non-bacterial thrombotic endocarditis: A state-of-the-art contemporary review.

Non-bacterial thrombotic endocarditis (NBTE), also known as Libman-Sacks, marantic, thrombotic, or verrucous endocarditis, is a form of non-infective endocarditis that affects cardiac structures in patients who have predisposing underlying conditions.1 As it is rarely encountered in routine clinical practice, the condition may be overlooked or misdiagnosed. On the other hand, other similar clinical entities might be erroneously labeled as NBTE. Notwithstanding its ostensibly uncommon appearance in clinical practice, our understanding of NBTE has been expanding, especially with the advent of modern and advanced diagnostic tools that facilitate the evaluation process. Herein, we provide a comprehensive review of NBTE, with a focus on the contemporary diagnostic evaluation and management.

Takotsubo cardiomyopathy in orthotopic liver transplant recipients: A cohort study using multi-center pooled electronic health record data.

BACKGROUND: Takotsubo cardiomyopathy (TCM), or stress-induced cardiomyopathy, is associated with adverse prognosis. Limited data suggest that TCM occurring in orthotopic liver transplant (OLT) recipients is associated with elevated peri-operative risk. AIM: To characterize the predictors of TCM in OLT recipients, using a large, multi-center pooled electronic health database. METHODS: A multi-institutional database (Explorys Inc, Cleveland, OH, USA), an aggregate of de-identified electronic health record data from 26 United States healthcare systems was surveyed. A cohort of patients with a Systematized Nomenclature of Medicine-Clinical Terms of "liver transplant" between 09/2015 and 09/2020 was identified. Subsequently, individuals who developed a new diagnosis of TCM following OLT were identified. Furthermore, the risk associations with TCM among this patient population were characterized using linear regression. RESULTS: Between 09/2015 and 09/2020, of 37718540 patients in the database, 38740 (0.10%) had a history of OLT (60.6% had an age between 18-65 years, 58.1% female). A new diagnosis of TCM was identified in 0.3% of OLT recipients (45.5% had an age between 18-65 years, 72.7% female), compared to 0.04% in non-OLT patients [odds ratio (OR): 7.98, 95% confidence intervals: 6.62-9.63, (P < 0.0001)]. OLT recipients who developed TCM, compared to those who did not, were more likely to be greater than 65 years of age, Caucasian, and female (P < 0.05). There was also a significant association with cardiac arrhythmias, especially ventricular arrhythmias (P < 0.0001). CONCLUSION: TCM was significantly more likely to occur in LT recipients vs non-recipients. Older age, Caucasian ethnicity, female gender, and presence of arrhythmias were significantly associated with TCM in LT recipients.

Patients With Inflammatory Bowel Disease on Treatment Have Lower Rates of Celiac Disease.

BACKGROUND: The association between celiac disease and inflammatory bowel disease (IBD) has been studied; however, the impact of IBD therapy on celiac disease is not known. Using a large database, we sought to describe the association of celiac disease and IBD and the impact of IBD treatment. METHODS: We queried a large multicenter database (Explorys Inc.), an electronic health record data aggregate from 26 American health care systems. We identified a cohort of patients with celiac disease and IBD between 1999 and 2020 and conducted a statistical analysis using a multivariate model. RESULTS: Of the 72,965,940 individuals in the database, 133,400 had celiac disease (0.18%), 191,570 (0.26%) had ulcerative colitis (UC), and 230,670 (0.32%) had Crohn disease (CD). Patients with IBD were more likely to have a diagnosis of celiac disease (odds ratio [OR], 13.680), with a greater association with CD. Treated patients with UC and with CD, respectively, had a lower risk association with celiac disease compared to those not undergoing IBD treatment, specifically corticosteroids (OR, 0.407 and 0.585), 5-aminosalicylates (OR, 0.124 and 0.127), immunomodulators (OR, 0.385 and 0.425), and anti-tumor necrosis factor drugs (OR, 0.215 and 0.242). There was no lower risk association in the vedolizumab group, but there was a higher risk association among the ustekinumab group. CONCLUSIONS: In this large dataset, we showed a bidirectional association between celiac disease and IBD that was stronger with CD. Patients with IBD treated using corticosteroids, 5-aminosalicylates, immunomodulators, or anti-tumor necrosis factor drugs had a lower association with celiac disease. Additional studies are required to determine the underlying mechanisms for IBD therapy-related modification of celiac disease incidence.

Multimodality imaging in the diagnosis and management of prosthetic valve endocarditis: A contemporary narrative review.

Infective endocarditis is one of the leading life-threatening infections around the world. With the exponential growth in the field of transcatheter interventions and advances in specialized surgical techniques, the number of prosthetic valves and cardiac implantable devices has significantly increased. This has led to a steep rise in the number of cases of prosthetic valve endocarditis (PVE) comprising up to 30% of all cases. Clinical guidelines rely on the use of the modified Duke criteria; however, the diagnostic sensitivity of the modified Duke criteria is reduced in the context of PVE. This is in part attributed to prosthesis related artifact which greatly affects the ability of echocardiography to detect early infective changes related to PVE in certain cases. There has been increasing recognition of the roles of complementary imaging modalities and updates in international society recommendations. Prompt diagnosis and treatment can prevent the devastating consequences of this condition. Imaging modalities such as cardiac computed tomography and 18-fluorodeoxyglucose positron emission tomography/computed tomography are diagnostic tools that provide a complementary role to echocardiography in aiding diagnosis, pre-operative planning, and treatment decision-making process in these challenging cases. Understanding the strengths and limitations of these adjuvant imaging modalities is crucial for the implementation of appropriate imaging modalities in clinical practice.

Post-Myocardial Infarction Ventricular Septal Rupture Bridged to Heartmate 3 with an Impella 5.5.

Optimal timing of surgical repair for patients diagnosed with a post-myocardial infarction ventricular septal rupture is controversial. Urgent surgical intervention to prevent hemodynamic decompensation must be balanced against delayed repair to allow for tissue stabilization and increased likelihood of a successful outcome. We report the use of an axillary Impella 5.5 (Abiomed Inc, Danvers, MA) temporary left ventricular assist device to aid in hemodynamic stabilization, shunt fraction reduction, and tissue maturation with eventual definitive surgical repair in a patient who presented with a post-myocardial infarction ventricular septal rupture and cardiogenic shock.

A Contemporary 20-Year Cleveland Clinic Experience of Nonbacterial Thrombotic Endocarditis: Etiology, Echocardiographic Imaging, Management, and Outcomes.

BACKGROUND: Nonbacterial thrombotic endocarditis, or marantic endocarditis, is rare. Contemporary data on the etiology, echocardiographic evaluation, and management of nonbacterial thrombotic endocarditis are limited. METHODS: A single-center retrospective cohort study was performed. Electronic medical records and echocardiographic records were searched for patients ages ≥18 years with a confirmed diagnosis of nonbacterial thrombotic endocarditis between January 1999 and November 2019. Demographic, echocardiographic, and management data were collected. RESULTS: Of 600,577 transthoracic echocardiograms (TTEs) and 89,264 transesophageal echocardiograms (TEEs), 42 patients had nonbacterial thrombotic endocarditis (mean age: 54 ± 14.5 years; 66.7% were female). The median duration of follow-up was 8.2 (interquartile range 3.3-24.4) months. Seventeen patients (40.5%) had malignancy, 33.3% had systemic lupus erythematosus, and 35.7% had antiphospholipid antibody syndrome. Stroke was the most common presentation (59.5%). TTE enabled the diagnosis in 19 cases (45.2%), compared with TEE, which identified the condition in 33 of 34 (97.1%) cases in which it was utilized. Three-dimensional echocardiography was performed in 17 TEEs. The most common valves involved were mitral (61.9%), and aortic (23.8%) valves. Thirty-two patients were managed with anticoagulation. Ten patients underwent surgery. Sixteen (38.1%) patients died, most of whom had a diagnosis of advanced malignancy. CONCLUSION: In a contemporary 20-year cohort, TTE and TEE played important roles in diagnosis, with superior diagnostic performance of TEE for nonbacterial thrombotic endocarditis. Mortality was high, and advanced malignancy portended a worse prognosis. Management in most cases was therapeutic anticoagulation. In select cases, surgery provided favorable outcomes.

Epidemiology of neuroendocrine tumors of the appendix in the USA: a population-based national study (2014-2019).

BACKGROUND: The appendix is the third most common place for neuroendocrine tumors (NETs) along the digestive tract and NETs are the most common neoplasms of the appendix. However, there are limited population-based data on the epidemiology of this disease. Using a large database, we sought to describe the epidemiology and risk association of NETs of the appendix. METHOD: We queried a multi-institutional database (Explorys Inc., Cleveland, OH, USA), comprising 360 hospitals in the United States (US), for patients with a diagnosis of NETs of the appendix from 2014-2019. RESULTS: Of the 30,324,050 individuals in the database, 2020 patients had an appendiceal NET diagnosis (0.007%). The most common presenting symptoms included abdominal pain, nausea, vomiting and diarrhea. Patients with appendiceal NETs were more likely to be female (odds ratio [OR] 1.36, 95% confidence interval [CI] 1.24-1.49), Caucasian (OR 2.71, 95%CI 2.40-3.07), with a history of smoking (OR 1.82, 95%CI 1.65-2.01), family history of primary gastrointestinal malignancy (OR 7.26, 95%CI 6.31-8.33), diagnosis of multiple endocrine tumor type 1 (OR 52.31, 95%CI 23.15-118.23), or neurofibromatosis type 1 (OR 16.37, 95%CI 7.24-37.01). CONCLUSIONS: In a population-based study in the US, using the Explorys database, we found the overall prevalence of NETs of the appendix to be 7 per 100,000 persons. The incidence in the year January 2019-January 2020 was 0.4 per 100,000 individuals. These rates are higher than previously reported and may be more accurate, given the more comprehensive nature of the Explorys database.

Super and Nonresponders to Catheter Ablation for Atrial Fibrillation: A Quality-of-Life Assessment Using Patient Reported Outcomes.

[Figure: see text].

Successful liver transplantation for acute sickle cell intrahepatic cholestasis: A case report and review of the literature.

BACKGROUND: Sickle cell hepatopathy (SCH) is an inclusive term referring to any liver dysfunction among patients with sickle cell disease. Acute sickle cell intrahepatic cholestasis is one of the rarest and most fatal presentations of SCH. We present the 23rd reported case of liver transplantation (LT) for SCH; a rare case of acute sickle cell intrahepatic cholestasis managed with LT from a hepatitis C virus (HCV) nucleic acid amplification test positive donor. CASE SUMMARY: A 29-year-old male with a past medical history of sickle cell disease presented with vaso-occlusive pain crisis. On examination, he had jaundice and a soft, non-tender abdomen. Initially he was alert and fully oriented; within 24 h he developed new-onset confusion. Laboratory evaluation was notable for hyperbilirubinemia, leukocytosis, anemia, thrombocytopenia, acute kidney injury and elevated international normalized ratio (INR). Imaging by ultrasound and computed tomography scan suggested a cirrhotic liver morphology with no evidence of biliary ductal dilatation. The patient was diagnosed with acute sickle cell intrahepatic cholestasis after excluding competing etiologies of acute liver injury. He underwent LT from an HCV nucleic acid amplification test positive donor 9 d after initial presentation. The liver explant was notable for widespread sinusoidal dilatation with innumerable clusters of sickled red blood cells and cholestasis. On postoperative day 3, HCV RNA was detectable in the patient's peripheral blood and anti-HCV therapy with glecaprevir/pibrentasvir was initiated on postoperative day 23. He subsequently achieved sustained virologic response after completing 3 mo of therapy and has been followed clinically for 12 mo post-transplant. CONCLUSION: This case highlights the utility of LT as a viable treatment option for acute sickle cell intrahepatic cholestasis.

Wearable Cardioverter-Defibrillator Therapy for the Prevention of Sudden Cardiac Death: A Systematic Review and Meta-Analysis.

OBJECTIVES: This study sought to synthesize the available evidence on the use of the wearable cardioverter-defibrillator (WCD). BACKGROUND: Observational WCD studies for the prevention of sudden cardiac death have provided conflicting data. The VEST (Vest Prevention of Early Sudden Death) trial was the first randomized controlled trial (RCT) showing no reduction in sudden cardiac death as compared to medical therapy only. METHODS: We searched PubMed, EMBASE, and Google Scholar for studies reporting on the outcomes of patients wearing WCDs from January 1, 2001, through March 20, 2018. Rates of appropriate and inappropriate WCD therapies were pooled. Estimates were derived using DerSimonian and Laird's method. RESULTS: Twenty-eight studies were included (N = 33,242; 27 observational, 1 RCT-WCD arm). The incidence of appropriate WCD therapy was 5 per 100 persons over 3 months (95% confidence interval [CI]: 3.0 to 6.0, I2 = 93%). In studies on ischemic cardiomyopathy, the appropriate WCD therapy incidence was lower in the VEST trial (1 per 100 persons over 3 months; 95% CI: 1.0 to 2.0) as compared with observational studies (11 per 100 persons over 3 months; 95% CI: 11.0 to 20.0; I2 = 93%). The incidence of inappropriate therapy was 2 per 100 persons over 3 months (95% CI: 1.0 to 3.0; I2 = 93%). Mortality while wearing WCD was rare at 0.7 per 100 persons over 3 months (95% CI: 0.3 to 1.7; I2 = 94%). CONCLUSIONS: The rate of appropriately treated WCD patients over 3 months of follow-up was substantial; higher in-observational studies as compared with the VEST trial. There was significant heterogeneity. More RCTs are needed to justify continued use of WCD in primary prevention.

Transvenous Lead Extraction in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy.

OBJECTIVES: This study aimed to evaluate the effectiveness and safety of transvenous lead extraction (TLE) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND: ARVC is an increasingly diagnosed cardiomyopathy that often requires device implantation to prevent sudden death. Little is known about TLE in this setting, which has potential challenges and risks, primarily due to right ventricular (RV) wall thinning and fragility. METHODS: All consecutive patients with ARVC who underwent TLE at our institution between 1996 and 2016 were included. When extraction tools were used, sheaths were advanced to the RV with countertraction at the lead tip. Success and complications were defined in concordance with Heart Rhythm Society guidelines. RESULTS: Twenty-two TLE procedures in patients with ARVC involved extraction of 27 leads (22 defibrillators and 5 pacemakers). TLEs were performed due to evidence of lead malfunction (n = 17; 77%) or device infection (n = 5; 23%). Twenty-four leads (89%) were RV, and 3 leads (11%) were right atrial. The median age of the oldest extracted lead was 1,691 days (interquartile range [IQR]: 1,168 to 2,726 days). Specialized extraction tools were required in 20 procedures (91%). None required the use of a snare or a femoral workstation. The median procedural and fluoroscopic times were 152 min (IQR: 129 to 185 min) and 11 min (IQR: 6 to 18 min), respectively. Complete procedural success with removal of all leads was achieved in all cases. There were no major complications. CONCLUSIONS: In a high-volume center, TLE in patients with ARVC was associated with a high success rate and a low rate of complications when guideline-established techniques and tools were used.