Cardiomyopathy in Celiac Disease: A Systematic Review.

(1) Background: Cardiomyopathy in celiac disease or celiac cardiomyopathy (CCM) is a serious and potentially life-threatening disease that can occur in both adults and children. However, data supporting the causal relationship between celiac disease (CD) and cardiomyopathy (CMP) are still inconsistent. The aim of this study was to review and synthesize data from the literature on this topic and potentially reveal a more evidence-based causal relationship. (2) Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to search Medline, Embase, and Scopus databases from database inception until September 2023. A total of 1187 original articles were identified. (3) Results: We identified 28 CCM patients (19 adult and 9 pediatric) with a mean age of 27.4 ± 18.01 years. Adult patients with CCM were predominantly male (84.2%) while pediatric patients were predominantly female (75%). The most common comorbidities associated with CCM were anemia (75%) and pulmonary hemosiderosis (20%). In 35% of patients, CCM occurred before the diagnosis of CD, while in 48% of patients, CCM and CD were diagnosed at the same time. Diagnosis of CD preceded diagnosis of CCM in only 18% of patients. Diagnosis of CCM is often delayed with an average, from the onset of symptoms to diagnosis, of 16 months. All patients were treated with a gluten-free diet in addition to guideline-directed medical therapy. At 11-month follow-up, cardiovascular improvement was seen in 60.7% of patients. Pediatric mortality was 33.3%, while adult mortality was 5.3%. (4) Conclusions: Clinicians should be aware of the possible association between CD and CMP, and we recommend CD work-up in all patients with CMP who have concomitant anemia. While we identified only 28 cases in the literature, many cases might go unreported due to a lack of awareness regarding CCM. A high degree of clinical suspicion and a prompt diagnosis of CCM are essential to minimizing the risks of morbidity and mortality, as the combination of a gluten-free diet and guideline-directed medical therapy can improve clinical outcomes.

Liver Dysfunction Associated With In-Hospital Mortality in Adult Extracorporeal Membrane Oxygenation Support.

Extracorporeal membrane oxygenator support is a powerful clinical tool that is currently enjoying a resurgence in popularity. Wider use of extracorporeal membrane oxygenator support is limited by its significant risk profile and extreme consumption of resources. This study examines the role of markers of liver dysfunction in predicting outcomes of adult patients requiring extracorporeal membrane oxygenator support. DESIGN: Retrospective review. SETTING: Large extracorporeal membrane oxygenator center, Chicago, IL. PATIENTS: This study reports a single institution experience examining all adult patients for whom extracorporeal membrane oxygenator support was used over an 8-year period. Data were collected regarding patient demographics, details of extracorporeal membrane oxygenator support provided, laboratory data, and outcomes. Trends in liver function were examined for their ability to predict survival. INTERVENTION: Extracorporeal membrane oxygenator support, critical care. MEASUREMENTS AND MAIN RESULTS: Mean age was 50 years (range, 19-82 yr). There were 86 male patients (56.6%) and 66 female patients (43.4%). Indications for initiation of extracorporeal membrane oxygenator support included cardiac 76 patients (50.0%), respiratory 48 patients (31.6%), extracorporeal cardiopulmonary resuscitation 21 patients (13.3%), and combined cardiac/respiratory seven patients (4.6%). Mean duration of extracorporeal membrane oxygenator support was 17 days (range 1-223 d) or median 8 days (interquartile range, 4-17 d). Overall, in-hospital mortality was 56% (86/152). Forty-five percent of adult patients (68/152) surpassed at least one of the following established liver dysfunction thresholds: total bilirubin greater than 15 mg/dL, aspartate aminotransferase greater than 20× upper limit of normal, and alanine aminotransferase greater than 20× upper limit of normal. The multivariable logistic analysis yielded three significant findings associated with in-hospital mortality: highest total bilirubin greater than 15 (adjusted odds ratio = 4.40; 95% CI, 1.19-21.87; p = 0.04), age (adjusted odds ratio = 1.03; 95% CI, 1.00-1.05; p = 0.04), and highest lactate (adjusted odds ratio = 1.15; 95% CI, 1.06-1.26; p = 0.002). CONCLUSIONS: Increases in age, highest total bilirubin, and lactate all correlated with in-hospital mortality in multivariable analysis of patients requiring extracorporeal membrane oxygenator support.

Extracorporeal Membrane Oxygenation Bridges Inoperable Patients to Definitive Cardiac Operation.

Extracorporeal membrane oxygenation (ECMO) offers an opportunity for patient recovery through complete cardiopulmonary support but is associated with complications that limit duration and overall utility. We examine the role of ECMO as a potential bridge to high-risk cardiac surgery in otherwise inoperable cases. This study reports a retrospective, multi-institution experience examining all patients for whom ECMO was used preoperatively as a bridge to definitive cardiac surgery without exception. A consecutive patient database (December 2011 through August 2017) was utilized. European System for Cardiac Risk Evaluation (EuroSCORE) 2 was calculated as a metric of patient acuity and risk assessment. Observed and expected mortality were compared. Twelve adult patients fit inclusion criteria and were supported with ECMO during the study period. There were five males and seven females. Average age was 56 (39-77) years. All 12 patients were supported with venoarterial ECMO for cardiogenic shock. This was done in preparation for corrective conventional cardiac surgery. Definitive cardiac surgical procedures included complex valve (n = 5), left ventricular assist device (n = 3), coronary artery bypass grafting (CABG; n = 2), CABG/ventricular septal defect repair (n = 1), and mitral valve replacement/CABG (n = 1). Average time of ECMO support was 200 (range 113-379) hours. Three patients were decannulated from ECMO at the conclusion of definitive cardiac surgery. Risk assessed by Logistic EuroSCORE 2 ranged from 64% to 89%. Average EuroSCORE 2-predicted mortality representing all 12 patients was 77%. Thirty day mortality was 25% (3/12), and hospital mortality was 33% (4/12). Seven patients are still alive today, with a mean survival of 37 (range 2-64) months. Two deaths were associated with gastrointestinal bleeding and two with evolving liver failure. Mean difference between the EuroSCORE 2 prediction model and actual observed 30 day mortality rate was 42.33 (95% CI 36.86-47.98) with a two-tailed, one-sample t test value of p < 0.001. ECMO can successfully be utilized as a bridge to conventional cardiac surgical procedures in critically ill patients, with a historically high mortality.

Postoperative takotsubo cardiomyopathy: an illustration of the electrocardiographic features that raise suspicion for takotsubo.

Takotsubo cardiomyopathy is an increasingly recognized clinical disorder mimicking acute coronary syndrome. It is usually preceded by physical or emotional stress and recovery of the left ventricular systolic function occurs in most cases within 1-4 weeks. Takotsubo cardiomypathy can masquerade as ST-segment elevation myocardial infarction when chest pain, ST-segment elevation, and high cardiac biomarkers coexist. ST-segment elevation is encountered in approximately half of the cases of takotsubo cardiomyopathy and its pattern is indistinguishable at times from ST-segment elevation myocardial infarction. However, several electrocardiographic criteria have been shown to characterize takotsubo cardiomyopathy. Awareness of these electrocardiographic features has several diagnostic and therapeutic implications. Nevertheless, these electrocardiographic criteria alone cannot reliably differentiate between both entities, and the diagnosis of takotsubo cardiomyopathy is only established after coronary angiography confirms the absence of occlusive coronary artery disease and the characteristic apical ballooning is evident on left ventriculogram (in the case of the apical form). Herein, we present a case of postoperative takotsubo cardiomyopathy and discuss the various electrocardiographic features that raise suspicion for this transient cardiac syndrome.

Pre-transplant depression as a predictor of adherence and morbidities after orthotopic heart transplantation.

BACKGROUND: Psychosocial factors are useful predictors of adverse outcomes after solid organ transplantation. Although depression is a known predictor of poor outcomes in patients who undergo orthotopic heart transplantation (OHT) and is actively screened for during pre-transplant evaluation, the effects of early identification of this entity on post-transplant outcomes are not clearly understood. The purpose of this study was to evaluate the impact of pre-transplant depression on outcomes after OHT. METHOD: In this retrospective study, 51 patients that underwent psychosocial evaluation performed by a social worker prior to the transplant and followed up in our center post-transplant were enrolled. Patients were stratified by the presence/absence of depression during the initial encounter. Primary end-points were overall survival, 1st-year hospitalizations, overall hospitalizations, rejections, and compliance with medications and outpatient appointments. RESULTS: Depressed patients were 3.5 times more likely to be non-compliant with medications; RR = 3.5, 95% CI (1.2,10.2), p = 0.046 and had higher incidence of first year hospitalizations (4.7 ± 3.1 vs. 2.2 ± 1.9, p = 0.046), shorter time to first hospitalization 25 days (IQR 17-39) vs. 100 days (IQR 37-229), p = 0.001. Patients with depression also had higher overall hospitalizations (8.3 ± 4.4 vs. 4.6 ± 4.2, p = 0.025,) and higher number of admissions for infections (2.8 ± 1.3 vs. 1.5 ± 1.4, p = 0.018) compared to patients without depression. There were no statistically significant differences in total number of rejections or compliance with outpatient appointments. Kaplan-Meier survival analysis did not reveal differences between the two groups (mean 3705 vs. 3764 days, log-rank p = 0.52). CONCLUSION: Depression was a strong predictor of poor medication compliance and higher rates of hospitalization in transplant recipients. No difference in survival between depressed and non-depressed patients after OHT was noted.

Delayed Retrograde Ascending Aortic Dissection After Endovascular Repair of Descending Dissection.

We report the case of a 34-year-old woman who developed delayed retrograde ascending aortic dissection after previous endovascular repair of her descending thoracic aorta. Preoperative computed tomography imaging specifically highlights the interaction of endograft springs with the dissection flap and surrounding aortic tissues. Intraoperative findings are presented for comparison and further discussion.