Abstract Objectives: This study aimed to diversify the spectrum of PRKAG2 variants and explore its clinical features in a Chinese Han population with hypertrophic cardiomyopathy (HCM). Methods: Whole-exome sequencing was performed on 200 patients diagnosed with HCM, and four causative PRKAG2 variants were identified in the probands and their relatives using Sanger sequencing. Their clinical manifestations, laboratory examinations, therapeutic methods, and outcomes were documented and analyzed. Results: Four variants were identified in six probands and seven of their relatives. Left ventricular hypertrophy was present in all probands. Five probands had sinus bradycardia, three had implanted pacemakers (PM), one developed heart failure, two had ventricular preexcitation, and one had atrial fibrillation. Conclusions: PRKAG2 cardiac syndrome (PCS) is a rare autosomal dominant disease characterized by ventricular hypertrophy, preexcitation, and progressive conduction defects, resulting in a high incidence of PM implantation. Genetic testing provides robust information for distinguishing PCS from sarcomeric HCM, which will be beneficial in guiding therapy and improving prognosis.

Abstract Sacubitril/valsartan (S/V) is a new drug which has been recently recommended by the international guidelines for the treatment of patients with chronic heart failure (HF) with reduced ejection fraction (HFrEF). Compared to angiotensin-converting enzyme inhibitors (ACEi) and angiotensin II receptor blockers (ARBs), S/V is associated with a better cardiovascular outcome, and to a greater beneficial effect on myocardial reverse remodeling. Recent evidence has shown that S/V is not only recommended in chronic patients but it is also approved in the acute setting; moreover, its safety and tolerability have been demonstrated also in the pediatric population. This review summarizes data on the effectiveness and tolerability of S/V in HFrEF patients and offers practical insights to manage this drug in every setting, providing an overview from randomized clinical trials' data to real-world evidence.

The four heart valves are arranged in different planes from each other. The aortic valve is most centrally located being related to all four cardiac chambers. Similar in construction to the pulmonary valve in possessing semilunar leaflets, neither valve has a ring-like annulus. Instead, the semilunar insertions of the leaflets form a crown-like arrangement. The semilunar valves are complexes comprising not only of leaflets but their attachments to arterial walls and adjoining ventricular structures at the ventriculo-arterial junction. The mitral and tricuspid valves guarding the atrioventricular junctions comprise of leaflets hinged to the junction, and have tendinous cords mainly anchored to papillary muscles that are inserted into ventricular walls to the support apparatus. A complete fibrous annulus is lacking, especially in the tricuspid valve and along the hinge line of the posterior mitral leaflet. This article also reviews the relationships of each valvar component and to adjacent cardiac structures.

Dysregulation of lipid metabolism is a major factor contributing to atherosclerotic cardiovascular disease (ACVD), which is the number one cause of death in western countries. The liver plays a central role in maintaining whole body cholesterol homeostasis via catabolism of cholesterol to bile acids, as well as biliary cholesterol excretion. The liver synthesizes lipoproteins that transport dietary cholesterol and fats to muscle and adipose tissue, directs reverse cholesterol transport of excess cholesterol from extrahepatic tissues and macrophages to the liver to convert to bile acids, and thus, protects against metabolism-related nonalcoholic fatty liver disease (NAFLD) and ACVD. Liver fibrosis/nonalcoholic steatohepatitis increases the risk and prevalence of cardiovascular disease morbidity and mortality. Bile acids are signaling molecules and metabolic regulators that activate farnesoid X receptor and G protein-coupled bile acid receptor-1 to regulate lipid, glucose, and energy metabolism. The bidirectional regulation of bile acids and the gut microbiota determine the rate of bile acid synthesis, the bile acid pool size, and the composition of the circulating bile acid pool. The liver-intestine-heart axis regulates lipid metabolism, inflammation, and the pathogenesis of metabolic diseases such as ACVD, NAFLD, diabetes, and obesity. This review focuses on the roles of liver-to-intestine, liver-to-heart and intestine-to-heart axes in cholesterol, lipoprotein, and bile acid metabolism; signaling in heart health and ACVD; and drug therapies for atherosclerosis.

Recently, the burden of coronary events in patients with acute coronary syndrome (ACS) has increased with a high recurrence rate and mortality. The management of blood lipid levels in patients with ACS is challenging under the current treatment mode. Based on practice experience of domestic specialists and the reference of international clinical practice, together with the evidence of lipid lowering in patients with ACS, this expert consensus proposes suggestions targeting lipid-lowering interventions, commonly used lipid-lowering drugs, and the clinical pathway of lipid management in patients with ACS. This work aims to standardize the lipid management and reduce the recurrence risk of cardiovascular events in patients with ACS.

In recent years, transcatheter treatment of mitral regurgitation (MR) has been extensively explored and has shown promising results. After transcatheter mitral valve repair (TMVr) with MitraClip has become a routine strategy in patients with symptomatic degenerate MR at prohibitive surgical risk, the benefits of TMVr are now being examined in patients with functional MR (FMR). However, the contradictory observations in COAPT and MITRA-FR clinical trials so far have suggested that only patients with severe FMR disproportionate to left ventricular disorder may benefit from this interventional therapy. Nevertheless, more evidence is required to support this hypothesis. In addition, various new devices have been developed, including two original designs from China Mainland, which have displayed excellent performance. Several percutaneous (femoral vein.transseptal) valves used in transcatheter mitral valve replacement have been developed and have also shown good feasibility. Furthermore, hybrid techniques in MR therapies have been emerging, which may reduce residual MR and improve the treatment effects.

Objectives: This study aimed to investigate the association between the metabolic syndrome (MetS) components and chronic kidney disease (CKD) in hypertension of different low-density lipoprotein cholesterol (LDL-C) levels. Methods: This national cross-sectional study was conducted in hospitals in 24 cities of China and comprised of 4792 eligible hypertensive participants with a recorded creatinine level between 2017 and 2018. All participants underwent a clinical survey and clinical assessment and were required to provide biomedical reports within 1 year from their outpatient visit. Student's t-test, log-rank, and Chi-square tests and receiver operating characteristic curve (ROC) analysis were used in data analysis. Results: Participants' average age was 65.82 ± 12.74 years; 54.65% were male. Overall, 52.73%, 63.29%, 26.84%, and 77.27% of participants had a high waist circumference, elevated triglyceride (TG) level, low high-density lipoprotein cholesterol level, and impaired fasting glucose/diabetes, respectively. The adjusted odds ratio (OR) for CKD with MetS was 1.67 (95% confidence interval [CI] 1.32–2.10, P

Transcatheter aortic valve replacement (TAVR) has developed rapidly in recent years as an alternative to surgical aortic valve replacement for patients with aortic valve diseases. As a minimally invasive approach, procedure success and good outcomes for patients largely depend on the procedure planning before TAVR. Moreover, imaging plays a vital role in contemporary TAVR planning. Among different types of imaging modalities, computed tomography (CT) is central. This article provides a systematic review of the role of CT in TAVR planning, including anatomic measurement and TAVR risk evaluation.

Background: Various comorbidities contribute to structural and functional changes in congestive heart failure (CHF). Echocardiography is a first-line diagnostic tool for screening and monitoring CHF patients. Hence, it is important to study the association between echocardiographic findings and comorbidities in CHF. Methodology: A retrospective study was conducted using data from CHF patients evaluated at the Madhavbaug clinics between August 2018 and January 2019. Patients were classified based on normal (>55%) and compromised (

Objective: The objective of the study is to construct a regional ST-segment elevation myocardial infarction (STEMI) treatment network system using the WeChat platform as a carrier and to explore the effect of this system on the time to reperfusion treatment and efficacy of treatment for patients with acute STEMI. Materials and Methods: The Department of Cardiology at our hospital constructed a STEMI rescue WeChat group including 120 emergency departments and acute care hospitals in the district. For all suspected STEMIs, relevant medical history data were uploaded to the WeChat group, a physician from the Department of Cardiology was consulted electronically, and this cardiologist was able to inform the local physician of a reperfusion treatment plan. One hundred and seventy-seven STEMI patients were transferred to our hospital within 12 h of presentation, of which 81 cases were transferred before the establishment of the WeChat group (the control group), and 96 cases were transferred using the WeChat system (WeChat group). The time to reperfusion, O-to-FMC time, FMC-to-B time, D-to-B time, average hospitalization stay, and mortality during hospitalization were observed and compared between the two groups. Results: The WeChat group had a lower O-to-FMC time compared with the control group, but this difference was not statistically significant. The FMC-to-B time, the time of D-to-B, and the average days of hospitalization in the WeChat group were all lower compared with those in the control group. The incidence of cardiovascular events and the heart function ratio of the patients after 6 months were compared, and the WeChat group was statistically superior to the control group in relation to these outcomes. Conclusion: The establishment of a STEMI regional treatment network based on the WeChat platform can shorten the time to reperfusion treatment, reduce postreperfusion cardiovascular events, shorten the days of hospitalization, and improve the heart function of patients.