A Phenomenal Binomaly: 2 Rare Diseases Presenting With Electrical Storm.

A previously well 34-year-old man presents with electrical storm after performing headstands. A step-by-step review of clinical information and case development is presented with discussion. Ultimately, 2 rare diagnoses are discovered and their potential contribution to a cascade of complications resulting in ventricular arrhythmia are discussed. (Level of Difficulty: Intermediate.).

The Syncopal Sarcoma: A Rare Case of Primary Cardiac Synovial Sarcoma.

Primary cardiac synovial sarcomas are very rare, representing <1% of all primary cardiac tumors. We report the case of a 19-year-old man with syncope and dynamic obstructive shock caused by a large right-sided intracardiac tumor. (Level of Difficulty: Beginner .).

Comparison of Heart Team vs Interventional Cardiologist Recommendations for the Treatment of Patients With Multivessel Coronary Artery Disease.

Importance: Although the heart team approach is recommended in revascularization guidelines, the frequency with which heart team decisions differ from those of the original treating interventional cardiologist is unknown. Objective: To examine the difference in decisions between the heart team and the original treating interventional cardiologist for the treatment of patients with multivessel coronary artery disease. Design, Setting, and Participants: In this cross-sectional study, 245 consecutive patients with multivessel coronary artery disease were recruited from 1 high-volume tertiary care referral center (185 patients were enrolled through a screening process, and 60 patients were retrospectively enrolled from the center's database). A total of 237 patients were included in the final virtual heart team analysis. Treatment decisions (which comprised coronary artery bypass grafting, percutaneous coronary intervention, and medication therapy) were made by the original treating interventional cardiologists between March 15, 2012, and October 20, 2014. These decisions were then compared with pooled-majority treatment decisions made by 8 blinded heart teams using structured online case presentations between October 1, 2017, and October 15, 2018. The randomized members of the heart teams comprised experts from 3 domains, with each team containing 1 noninvasive cardiologist, 1 interventional cardiologist, and 1 cardiovascular surgeon. Cases in which all 3 of the heart team members disagreed and cases in which procedural discordance occurred (eg, 2 members chose coronary artery bypass grafting and 1 member chose percutaneous coronary intervention) were discussed in a face-to-face heart team review in October 2018 to obtain pooled-majority decisions. Data were analyzed from May 6, 2019, to April 22, 2020. Main Outcomes and Measures: The Cohen κ coefficient between the treatment recommendation from the heart team and the treatment recommendation from the original treating interventional cardiologist. Results: Among 234 of 237 patients (98.7%) in the analysis for whom complete data were available, the mean (SD) age was 67.8 (10.9) years; 176 patients (75.2%) were male, and 191 patients (81.4%) had stenosis in 3 epicardial coronary vessels. A total of 71 differences (30.3%; 95% CI, 24.5%-36.7%) in treatment decisions between the heart team and the original treating interventional cardiologist occurred, with a Cohen κ of 0.478 (95% CI, 0.336-0.540; P = .006). The heart team decision was more frequently unanimous when it was concordant with the decision of the original treating interventional cardiologist (109 of 163 cases [66.9%]) compared with when it was discordant (28 of 71 cases [39.4%]; P < .001). When the heart team agreed with the original treatment decision, there was more agreement between the heart team interventional cardiologist and the original treating interventional cardiologist (138 of 163 cases [84.7%]) compared with when the heart team disagreed with the original treatment decision (14 of 71 cases [19.7%]); P < .001). Those with an original treatment of coronary artery bypass grafting, percutaneous coronary intervention, and medication therapy, 32 of 148 patients [22.3%], 32 of 71 patients [45.1%], and 6 of 15 patients [40.0%], respectively, received a different treatment recommendation from the heart team than the original treating interventional cardiologist; the difference across the 3 groups was statistically significant (P = .002). Conclusions and Relevance: The heart team's recommended treatment for patients with multivessel coronary artery disease differed from that of the original treating interventional cardiologist in up to 30% of cases. This subset of cases was associated with a lower frequency of unanimous decisions within the heart team and less concordance between the interventional cardiologists; discordance was more frequent when percutaneous coronary intervention or medication therapy were considered. Further research is needed to evaluate whether heart team decisions are associated with improvements in outcomes and, if so, how to identify patients for whom the heart team approach would be beneficial.

Withholding versus Continuing Angiotensin-converting Enzyme Inhibitors or Angiotensin II Receptor Blockers before Noncardiac Surgery: An Analysis of the Vascular events In noncardiac Surgery patIents cOhort evaluatioN Prospective Cohort.

BACKGROUND: The effect on cardiovascular outcomes of withholding angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers in chronic users before noncardiac surgery is unknown. METHODS: In this international prospective cohort study, the authors analyzed data from 14,687 patients (including 4,802 angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker users) at least 45 yr old who had in-patient noncardiac surgery from 2007 to 2011. Using multivariable regression models, the authors studied the relationship between withholding angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers and a primary composite outcome of all-cause death, stroke, or myocardial injury after noncardiac surgery at 30 days, with intraoperative and postoperative clinically important hypotension as secondary outcomes. RESULTS: Compared to patients who continued their angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers, the 1,245 (26%) angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker users who withheld their angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers in the 24 h before surgery were less likely to suffer the primary composite outcome of all-cause death, stroke, or myocardial injury (150/1,245 [12.0%] vs. 459/3,557 [12.9%]; adjusted relative risk, 0.82; 95% CI, 0.70 to 0.96; P = 0.01) and intraoperative hypotension (adjusted relative risk, 0.80; 95% CI, 0.72 to 0.93; P < 0.001). The risk of postoperative hypotension was similar between the two groups (adjusted relative risk, 0.92; 95% CI, 0.77 to 1.10; P = 0.36). Results were consistent across the range of preoperative blood pressures. The practice of withholding angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers was only modestly correlated with patient characteristics and the type and timing of surgery. CONCLUSIONS: Withholding angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers before major noncardiac surgery was associated with a lower risk of death and postoperative vascular events. A large randomized trial is needed to confirm this finding. In the interim, clinicians should consider recommending that patients withhold angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers 24 h before surgery.

Transesophageal echocardiography in patients with cryptogenic ischemic stroke: a systematic review.

BACKGROUND: The clinical utility of routine transesophageal echocardiography (TEE) for patients with unexplained ischemic stroke is controversial. We performed a systematic review to determine the frequency of detection of new cardiac findings in patients with cryptogenic ischemic stroke (IS) undergoing transesophageal echocardiography (TEE). METHODS: Systematic review and meta-analysis of cohort studies of consecutive patients with "cryptogenic" IS undergoing TEE after routine etiologic workup. Patients were categorized into 2 groups: A (< 55 years) and B (≥ 55 years). Outcomes included proportion of patients with new TEE-detected cardiac findings and proportion of patients commenced on oral anticoagulation after TEE. RESULTS: Twenty-seven studies were included (n = 5,653). We identified significant heterogeneity among studies and report a range of prevalence rates and I2 statistic as our primary analysis. Prevalence of individual cardiac findings on TEE varied significantly among studies; patent foramen ovale (A: 12.0%-57.8%, I2 = 89.9%; B: 3.9%-43.5%, I2 = 86.7%), atrial septal aneurysm (A: 0-48.9%, I2 = 91.9%; B: 3.5%-25.0%, I2 = 84.5%), left atrial thrombus (A: 0-10.9%, I2 = 61.1%; B: 0-21.2%, I2 = 91.7%), spontaneous echo contrast (A: 0-11.9%, I2 = 57.2%; B: 0-21.3%, I2 = 89.8%), and aortic atheroma (A: 0-9.6%, I2 = 53.8%; B: 2.8%-44.4%, I2 = 89.7%). Definitions of common findings were not provided for many studies. Five studies (n = 591) reported on the proportion of patients who were commenced on anticoagulant therapy after TEE (range 0-30.7%). CONCLUSIONS: Routine TEE in patients with cryptogenic IS identifies cardiac findings in a large proportion. However, there is marked interstudy variation in the definition and prevalence of common findings. Transesophageal echocardiography-detected findings prompted the introduction of anticoagulant therapy in up to one-third of patients. However, these were mostly not for established guideline-based indications based on randomized controlled trial evidence. It is unclear if routine use of TEE in patients with cryptogenic IS is indicated.

Measures of cardiovascular risk and subclinical atherosclerosis in a cohort of women with a remote history of preeclampsia.

OBJECTIVE: We assessed for subclinical atherosclerosis using carotid intima-media thickness (CIMT) among women with and without a remote history of preeclampsia. Secondarily, we contrasted cardiovascular risk factors and electrocardiography between both groups. Women with a history of preeclampsia are at higher risk of future cardiovascular disease (CVD). The degree to which this is mediated by atherosclerosis is less understood, especially after several decades. METHODS: We performed a nested cohort study comprising 109 women with a remote history of preeclampsia 1:2 matched to 218 women with an uncomplicated pregnancy. After a median of 20 years since the index pregnancy, we measured blood pressure, height, weight, waist and hip circumference, and performed an oral 75 g glucose tolerance test (OGTT), fasting lipids, electrocardiography, albumin:creatinine ratio (ACR) and CIMT among all participants. RESULTS: While women with and without preeclampsia had similar family histories of CVD, those with preeclampsia had a higher rate of chronic hypertension (32% versus 10%, p < 0.0001), greater waist (p = 0.008) and hip circumferences (p = 0.001). No differences were seen on in OGTT, lipid or ACR measures. Average maximum CIMT was similar among those with versus without preeclampsia (0.831 mm versus 0.817, p = 0.38), and preeclampsia was not a significant predictor of CIMT in a multiple linear regression model (p = 0.63), despite more electrocardiograms compatible with coronary disease. CONCLUSION: Two decades after delivery, women with a remote history of preeclampsia had more CVD risk factors than women with unaffected pregnancies, but this was not reflected in a difference in CIMT.

Sclareol reduces CD4+ CD25+ FoxP3+ Treg cells in a breast cancer model in vivo.

BACKGROUND: Sclareol is a phytochemical used in people's diet in Southeast Asia. OBJECTIVE: To investigate the immunotherapeutic effectiveness of Sclareol against breast cancer by direct intraperitoneal injection. METHODS: Sclareol was isolated and purified from Salvia sclarea. Effect of Sclareol on cell growth inhibition was evaluated by MTT assay. Intraperitoneally injected Sclareol effects on reducing the tumor volume and shifting the cytokine profile were investigated. We also assessed if intraperitoneally injected Sclareol could improve the outcome of cancer therapy through suppressing the regulatory T cells. RESULTS: The results confirmed a significant decrease in the tumor size. Furthermore, a significant decrease in the level of IL-4 and an increase in the level of IFN-γ were noticed in the intraperitoneally injected Sclareol group (p<0.05). It was also observed that the splenocytes of treated animals significantly increase in cell proliferation assay. Moreover, measurements of splenic T regulatory cell indicated that intraperitoneally injected Sclareol significantly decreased the number of splenic T regulatory cell. CONCLUSION: Our results suggest that Sclareol, by reducing Treg cells frequency and also tumor size can enhance the effect of cancer therapy as an immuno-stimulant.

The ace of spades: reverse takotsubo cardiomyopathy in the context of angiographic embolization of recurrent metastatic serotonin-positive neuroendocrine tumour of the pancreas.

A 62-year-old woman undergoing embolization of recurrent neuroendocrine tumor, positive for serotonin, developed chest pain and bradycardia with lateral ST-segment depression. Cardiac biomarkers were elevated, and echocardiography revealed akinesis of all basal segments with a normally contracting apex. The absence of flow-limiting coronary disease on angiography confirmed the presence of reverse Takotsubo cardiomyopathy. After optimal medical therapy for six weeks, left ventricular function returned to normal. Takotsubo cardiomyopathy has been described across a wide variety of hyperadrenergic states; the description of the reverse-type Takotsubo cardiomyopathy in the setting of embolization of recurrent neuroendocrine with serotonergic positivity tumour is novel.

Cardiac outcomes in adults with supravalvar aortic stenosis.

AIMS: Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. METHODS AND RESULTS: This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams-Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams-Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions (P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams-Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams-Beuren syndrome (P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare. CONCLUSION: Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthood.