The management of acute chest syndrome (ACS) in sickle cell disease occurring concurrently with pulmonary embolism resulting from tricuspid valve endocarditis poses an atypical challenge. We present a case in which this complex interaction occurs and the prompt interventions that were utilized to give the best possible outcome.

Background:Since 2005, the cardioprotective effects of glucagon-like peptide 1 receptor agonists (GLP-1 RAs) have garnered attention. The cardioprotective effect could be an added benefit to the use of GLP-1 RA. This systematic review and meta-analysis aimed at summarizing observational studies that recruited type 2 diabetes individuals with fewer cardiovascular (CV) events before enrolling in the research. Methods:Systematically, the databases were searched for observational studies reporting compound CV events and deaths in type 2 diabetics without having the risk of cardiovascular diseases (CVDs) compared to other glucose-lowering agents. A meta-analysis was carried out using random effects model to estimate the overall hazard ratio (HR) with a 95% confidence interval (CI). Five studies were found eligible for the systematic review including a total of 64,452 patients receiving either liraglutide (three studies) or exenatide (two studies). Results:The pooled HR for major adverse cardiac event (MACE) and extended MACE was 0.72 (95% CI: 0.65 - 0.93, I2 = 68%) and 0.93 (95% CI: 0.89 - 0.98, I2 = 29%), respectively. The pooled HR for hospitalization due to heart failure (HHF) and occurrence of HF was 0.84 (95% CI: 0.77 - 0.91, I2 = 79%) and 0.83 (95% CI: 0.75 - 0.94, I2 = 95%), respectively. For stroke, GLP-1 RA was associated with a significant risk reduction of 0.86 (95% CI: 0.75 - 0.98, I2 = 81%). There was no significant myocardial infarction (MI) risk reduction with GLP-1 RA. As for all-cause mortality, the pooled HR for the occurrence of all-cause mortality was 0.82 (95% CI: 0.76 - 0.88, I2 = 0%). The pooled HR for the occurrence of CV death was 0.75 (95% CI: 0.65 - 0.85, I2 = 38%). GLP-1 RA therapy was associated with a significantly low risk of MACE, extended MACE, all-cause mortality, and CV mortality. Except for MACE, the heterogenicity among the studies was low. Conclusion:We conclude that GLP-1 RA is associated with a low risk of CV events composites and mortality. The findings support the cardioprotective effect of GLP-1 RA.

Introduction Acute decompensated heart failure is one of the leading and most costly causes for hospitalization in the United States. Therapeutic options for Heart Failure with preserved Ejection Fraction (HFpEF) remain significantly limited, when compared to heart failure with reduced ejection fraction (HFrEF). The concomitant presence and severity of aortic stenosis (AS) is associated with significant morbidity and mortality in heart failure patients, requiring early identification. Hypothesis The severity of AS is associated with unique characteristics, which present themselves in patients hospitalized with decompensated HFpEF. Methods We collected clinical, demographic, laboratory data and echocardiographic parameters of patients admitted with acute decompensated heart failure between August 2016 and August 2017. Patients with an echocardiogram report completed within three months of hospitalization, demonstrating an ejection fraction of at least 50% and confirmed AS were included in our analysis. We compared length of stay (LOS), all-cause readmission within 30 days and recurrent decompensated heart failure within 6 months. Results There were 79 subjects with AS, admitted with a primary diagnosis of HFpEF. Median age of the sample was 86 years (IQR: 80-92), with 68.4% female. There were 16 patients (20.5%) with severe AS. The proportion of those with severe AS admitted with HFpEF did not differ from those with mild to moderate AS (81.3% vs. 70%, p = 0.219). There were 24 deaths within 1 year of hospitalization, 15 which occurred on a subsequent hospitalization. There were no differences in LOS of patients with severe and mild to moderate AS (mean 7.1 days vs. 7.9 days, p = 0.657), 30-day readmission (18.8% vs. 20.6%, p = 0.869) or death within 1 year (37.5% vs. 28.6%, p = 0.967). Serum sodium was significantly lower in patients with severe AS compared to mild and moderate AS (136.3 vs. 139.6 mmol/L, p = 0.024). Conclusion In patients admitted with decompensated HFpEF and severe AS, serum sodium was significantly lower than patients with mild to moderate AS. Serum sodium may be a marker for severity of aortic stenosis.

Electronic cigarettes may increase the risk of long-term cardiovascular morbidity. To protect the heart, awareness should be raised of the risks and limits of E-cigarette aerosol exposure. Thus, this systematic review and meta-analysis assessed the cardiovascular risk of e-smoking. This systematic review was conducted by using the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) statement. We searched PubMed, Embase, Scopus, Web of Science, and Science Direct databases in December 2022 to identify studies investigating e-cigarettes' impact on the heart. The study was supported by meta-analysis and qualitative review. Out of the initial 493 papers, only 15 met the inclusion criteria and were included in the study. The cumulative number of participants in the myocardial infarction (MI) group was 85,420, and in the sympathetic groups in whom the systolic blood pressure (SBP), diastolic blood pressure (DBP), mean blood pressure (MBP), and heart rate (HR) were measured, were 332 cigarette smokers. The control group included the "never use," "non-smokers," and "never smoke." The pooled analysis showed a significant difference between the e-cigarette smokers and the control group regarding the risk of developing MI in former smokers (OR= 0.12; 95% CI: 0.01-1.72, P = 0.12) and never smoked (OR= 0.02; 95% CI: 0.00-0.44, P = 0.01) favoring the control group. The pooled analysis of the included studies showed a significant difference between the e-cigarette smokers with nicotine and the control group regarding the mean difference (MD) of the SBP (MD = 2.89; 95% CI: 1.94-3.84; P < 0.001), the DBP (MD = 3.10; 95% CI: 0.42-5.78; P = 0.02), the MBP (MD = 7.05; 95% CI: 2.70-1.40; P = 0.001), and HF (MD = 3.13; 95% CI: 0.96-5.29; P = 0.005) favoring the control group. We conclude that using e-cigarettes has a detrimental effect on cardiac health. The risk of severe cardiac conditions increases with e-cigarettes. Thus, vaping can do more harm than good. Consequently, the misleading notion that e-cigarettes are less harmful should be challenged.

Cardiac Amyloidosis (CA) is a manifestation of a systemic disorder resulting from the deposition of transthyretin (TTR) in the myocardium. This leads to a myriad of manifestations ranging from conduction defects to heart failure. Previously CA was considered a rare disease, but recent advances in diagnostics and therapeutics have revealed the prevalence to be higher than estimated. There are two major classes of treatments for TTR cardiac amyloidosis (ATTR-CA): TTR stabilizers, such as tafamidis and AG10, and RNA interference (siRNA), such as patisiran and vutrisiran. Clustered regularly interspaced short palindromic repeats of genetic information-Cas9 endonuclease (CRISPR-Cas9) utilizes an RNA-guided endonuclease to target specific locations in the genome. Until recently, CRISPR-Cas9 was studied in small animal models for its ability to decrease extracellular deposition and accumulation of amyloid in tissues. Gene editing has demonstrated some early clinical promise as an emerging therapeutic modality in the treatment of CA. In an introductory human trial involving 12 subjects with TTR amyloidosis and amyloid cardiomyopathy (ATTR-CM), CRISPR-Cas9 therapy has demonstrated a reduction in approximately 90% of serum TTR proteins after 28 days. In this article, the authors review the current literature on therapeutic gene editing as a prospective curative treatment modality for CA.

Introduction Atrial Fibrillation is the most common dysrhythmia observed worldwide. It is commonly observed in heart failure, with a greater prevalence in Heart Failure with Preserved Ejection Fraction (HFpEF) as opposed to Heart Failure with Reduced Ejection Fraction. Atrial Fibrillation with rapid ventricular response (RVR) may result in increased morbidity and mortality, resulting in worsening heart failure, stroke or death. The clinical characteristics and outcomes of those who present with RVR on presentation in acute decompensated heart failure is not well known. Hypothesis The clinical characteristics and outcomes of patients who present with RVR on admission in decompensated HFpEF will differ significantly from those with rate-controlled atrial fibrillation (RCAF). Methods We collected clinical, demographic and admission laboratory data of patients who were admitted with acute decompensated HFpEF and a history of atrial fibrillation between August 2016 and August 2017. Patients with RVR and HFpEF were defined as having a heart rate of at least 100 on initial vitals, were admitted with a primary diagnosis of heart failure and was found to have an echocardiogram within 3 months of hospitalization with an ejection fraction of at least 40%. Length of stay, 30 day all-cause readmission rates and mortality at 1 year were assessed. Results A total of 181 subjects with a history of atrial fibrillation and HFpEF were admitted with acute decompensated heart failure during the study period. The median age was 82.0 years (IQR 74-89), with 59.7% female. There were 28 patients (15.3%) who presented with RVR on admission. The average length of stay (5.86 days vs. 6.92 days, p = 0.114) and 30-day readmission rates (21.4% vs. 26.8%, p = 0.540) did not significantly differ between the RVR and RCAF groups. Patients with RVR were significantly less likely to have reported a prior history of myocardial infarction (7.1% vs. 24.2%, p = 0.007) or coronary artery disease (52.3% vs. 17.9%, p < 0.001). Borderline significance was observed in patients presenting with RVR, who were less likely taking a mineralocorticoid receptor antagonist (3.6% vs. 12.5%, p = 0.050), a statin (46.4% vs. 65.4%, p = 0.075) or a loop diuretic (50% vs. 68.2%, p = 0.087) prior to admission. Conclusion In the subset of patients with decompensated HFpEF and atrial fibrillation, those who presented with RVR were significantly less likely to have had a history of coronary artery disease, with a trend towards significance observed in lower rates of mineralocorticoid receptor antagonists, statin use and loop diuretics. These findings suggest a unique pathophysiology among those with RVR from those with rate-controlled atrial fibrillation.

Introduction: Acute Decompensated Heart Failure is one of the most common reasons for hospitalization in the United States. Heart failure with preserved ejection fraction (HFpEF) accounts for nearly half of all heart failure admissions, significant contribution owing to various co-morbidities. The role of kidney impairment on admission and prognostic association with early HFpEF readmission is unclear. Methods: We conducted a retrospective analysis of patients admitted with decompensated HFpEF at a hospital in New York City. Clinical demographics & admission laboratory values collected to classify kidney function, according to the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula to calculate glomerular filtration rate (GFR). Severe renal impairment (SRI), defined as a GFR less than 15 mL/min/1.73 m 2 . Patients followed for one-year to assess all-cause & cardiac-specific readmission within 30 days & all-cause mortality within one year. Results: A total of 358 patients with HFpEF were studied, 218 (60.8%) were female, 196 (54.6%) with diabetes. There were 82 readmissions (22.9%) within 30 days, 37 (10.3%) due to cardiac-specific causes, 69 (19.3%) who died within one year of hospitalization. There were 47 (13.1%) who met criteria of SRI on admission. No difference in one-year mortality with SRI, compare to normal renal function (38.2% vs. 23.1%, p = 0.416). Patients with SRI were more likely to be readmitted within 30-days for cardiac specific reasons than normal renal function (27.6% vs. 8.5%, p = 0.034), while a borderline significant increase in all-cause readmission (38.2% vs. 23.1%, p = 0.066). Conclusions: Patients with HFpEF & SRI, were more likely to experience readmission within 30 days due to cardiac-specific reasons than patients with normal renal function. These findings may assist in enhancing cardiac-specific prevention strategies in discharge planning.

Introduction: Myocardial i nfiltration with amyloid fibrils increases myocardial echogenicity on echocardiography (echo). Increased echogenicity has been challenging to quantify. Radiomics methods may better detect changes in echogenicity than the human eye. Our objectives were (1) to detect an altered myocardial echogenicity in transthyretin cardiac amyloidosis (ATTR-CM) using radiomics analysis and (2) to compare myocardial echogenicity by radiomics to human interpretation. Methods: We evaluated echo images of 515 patients with suspected ATTR-CM referred for an 99m-technetium pyrophosphate scan at 3 major amyloidosis centers. Myocardial echogenicity was assessed at the basal interventricular septum using an open-source radiomics platform (LIFEx) to extract 41 texture-based features. The texture features were processed using a cloud platform (BigML), which performed automated model selection and hyperparameter tuning (Figure). The studies were randomly split into training (80%) and evaluation (20%) sets. Two echo and amyloidosis experts visually evaluated myocardial echogencity on 80 random studies and classified them into ATTR-CM or non- ATTR-CM. Results: In this cohort, 57% of patients were diagnosed with ATTR-CM. The best radiomics model was a decision forest with bootstrapping, with an AUC of 0.79 (95% CI: 0.70-0.88) for diagnosing ATTR-CM. By contrast, visual classification of echo texture as ATTR-CM or not was poor; the interobserver agreement for assessing a septal ROI for altered myocardial echogenicity consistent with ATTR-CM was a kappa of 0.29 and using the entire apical 4 chamber view 0.44. Given the poor agreement an AUC could not be calculated. Conclusions: We have validated the use of a radiomics approach to evaluate myocardial echogenicity in ATTR-CMP. This work lays the foundation to evaluate changes in myocardial texture using echo to raise the suspicion of cardiac amyloidosis and assess changes following targeted TTR therapies.

Quadricuspid aortic valve (QAV) is a congenital heart anomaly in which the aortic valve has four cusps of various size possibilities, as opposed to the three symmetrical cusps generally observed. This cardiac valvular abnormality is rarely identified, with an estimated incidence rate of 0.013% to 0.043%, although recent technological advancements in diagnostics have contributed to an increase in detection. Historically, it had been typically encountered during open heart surgery or postmortem; however, it is presently diagnosed primarily via ultrasound echocardiography, and could go undetected unless specifically considered. It was first reported by Babington in 1847, and since then approximately 300 cases have been published. This condition is sporadically associated with additional congenital cardiovascular defects, with coronary artery irregularities being the most common. In more than half of published QAV incidences it has led to the progressive development of aortic regurgitation (AR) usually sans aortic stenosis, particularly amongst elderly patients, often requiring surgical intervention after 50 years of age. A fifth of total instances, but two-thirds of instances with AR, warrant surgery seldom amidst complications, with reconstructive tricuspidization preferred over valve replacement.

Background: Echocardiographic deformation-based ratios and novel multi-parametric scores have been suggested to discriminate transthyretin cardiac amyloidosis (ATTR-CM) from other causes of increased left ventricular wall thickness among patients referred for ATTR-CM evaluation. Their relative predictive accuracy has not been well studied. We sought to (1) identify echocardiographic parameters predictive of ATTR-CM and (2) compare the diagnostic accuracy of these parameters in patients with suspected ATTR-CM referred for technetium-99m-pyrophosphate scintigraphy. Methods: Echocardiograms from 598 patients referred to 3 major amyloidosis centers for technetium-99m-pyrophosphate to detect ATTR-CM were analyzed, including longitudinal strain (LS) analysis. Deformation ratios (septal apex to base ratio, relative apical sparing, ejection fraction to global LS), a multi-center European increased wall thickness score, and Mayo Clinic derived ATTR score (transthyretin cardiac amyloidosis score) were calculated. A logistic regression model was used to identify the parameters that best associated with a diagnosis of ATTR-CM. Comparison of the diagnostic capacity of the parameters was performed by receiver operating characteristic curves and the area under the curve (AUC). Results: Over half of the subjects (54.2%) were diagnosed with ATTR-CM (78% were men, median age of 76 years). Age, inferolateral wall thickness, and basal LS were the strongest predictors of ATTR-CM, AUC of 0.87 (95% CI: 0.83, 0.90), superior to the increased wall thickness score AUC of 0.78 (95% CI: 0.73, 0.83; P =0.004). An inferolateral wall thickness of ≥14 mm (AUC: 0.73) was as accurate as the published cut-offs for transthyretin cardiac amyloidosis score and septal apex to base (AUC: 0.72 and 0.69, P =0.8 and P =0.1, respectively), and was superior to ejection fraction to global LS and relative apical sparing (AUC: 0.64 and 0.53, P <0.001, respectively). A cut-off of ≥−8% for average basal LS (AUC: 0.76, CI: 0.72–0.79) had a similar area under the curve to transthyretin cardiac amyloidosis score (TCAS) ( P =0.2); outperforming the other indices ( P <0.01). Conclusion: Inferolateral wall thickness and average basal LS performed as well as or better than more complex echo ratios and multiparametric scores to predict ATTR-CM.