Approximately 20% of acute myocardial infarction (AMI) patients with multivessel disease experience adverse outcomes after complete revascularization. We aim to investigate the underlying metabolic mechanism of ischemia-reperfusion injury responsible for abnormal hemodynamic stresses in high-risk patients undergoing complete revascularization. Elevated preoperative serum levels of long-chain acylcarnitine (LCAC) 16:1 are associated with an increased risk of poor prognosis following complete revascularization. Multi-omics analyses reveal that reperfusion injury activates fatty acid degradation, and carnitine palmitoyltransferase 1A (CPT1A) is identified as a key regulator of LCACs in the interaction network in porcine models. In the early stages of reperfusion injury in non-culprit lesions, the release and prolonged elevation of circulating LCACs primarily depend on the activation of endothelial CPT1A through hemodynamic injury, which can be reduced using an inhibitor (etomoxir). Excess LCACs enter cardiomyocytes via the organic cation transporter 2, leading to imbalanced mitochondrial quality control and causing cardiomyocyte death.

ABSTRACT Adherence to antihypertensive medications is essential for blood pressure (BP) control, influencing long‐term outcomes in hypertensive patients. This study examines the association between visit‐to‐visit variability in BP and heart rate (HR) and its effect on adherence to beta‐blocker therapy among outpatients. Conducted across 160 hospitals in China from January 1, 2011, to December 31, 2011, this study included 9225 hypertensive outpatients prescribed metoprolol succinate. BP and HR variability were assessed over three visits (baseline, 1‐month, and 2‐month follow‐up) using standard deviation (SD) and mean‐independent parameters. Nonadherence was defined as medication discontinuation or treatment regimen changes by the 2‐month follow‐up. Among the 9037 patients analyzed, the mean age was 58.85 years (±12.54), and 52.9% were male. Visit‐to‐visit variability in the rate–pressure product (RPP; SBP×HR) was a significant predictor of nonadherence, with an odds ratio (OR) of 1.26 (95% confidence interval [CI]: 1.04–1.53, p < 0.05) for the top‐decile SD of RPP, independent of mean RPP. Variability in diastolic blood pressure (DBP) and pulse pressure (PP) were also associated with nonadherence, with ORs of 1.65 (95% CI: 1.35–2.00, p < 0.001) for DBP and 1.66 (95% CI: 1.39–1.99, p < 0.001) for PP, independent of their mean values. Patients with fluctuations in PP or HR had a higher risk of nonadherence compared to those with consistent reductions in these measures. Visit‐to‐visit variability in RPP, DBP, and PP is a significant predictor of nonadherence to beta‐blockers, regardless of mean levels. Addressing this variability is critical for improving adherence to antihypertensive treatments and optimizing patient outcomes.

This study analyzed Global Burden of Disease (GBD) 2019 data to assess cardiovascular disease (CVD) burden in women worldwide. Using annual incident cases, deaths, age-standardized incidence (ASIR), and mortality rates (ASMR) from 1990-2019, trends were evaluated via percentage changes and estimated annual percentage change (EAPC). Globally, incident CVD cases in women rose by 73.86% (15.90 to 27.64 million), while deaths increased by 47.45% (6.06 to 8.94 million). Despite this, ASIR (EAPC: -0.57%) and ASMR (EAPC: -1.63%) declined. Regional disparities emerged in ischemic heart disease (IHD): ASIR increased in low/middle socio-demographic index (SDI) regions (EAPC: +0.04% and + 0.08%) but decreased in high-middle/high SDI regions (EAPC: -1.09% and -2.24%). Findings highlight persistent IHD incidence growth in lower-resource settings despite global improvements in age-standardized rates.

Background:Non-invasive computed tomography angiography (CTA)-based fractional flow reserve (CT-FFR) could become a gatekeeper to invasive coronary angiography. Deep learning (DL)-based CT-FFR has shown promise when compared to invasive FFR. To evaluate the performance of a DL-based CT-FFR technique, DeepVessel FFR (DVFFR). Methods:This retrospective study was designed for iScheMia Assessment based on a Retrospective, single-center Trial of CT-FFR (SMART). Patients suspected of stable coronary artery disease (CAD) and undergoing both CTA and invasive FFR examinations were consecutively selected from the Beijing Anzhen Hospital between January 1, 2016 to December 30, 2018. FFR obtained during invasive coronary angiography was used as the reference standard. DVFFR was calculated blindly using a DL-based CT-FFR approach that utilized the complete tree structure of the coronary arteries. Results:Three hundred and thirty nine patients (60.5 ±10.0 years and 209 men) and 414 vessels with direct invasive FFR were included in the analysis. At per-vessel level, sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) of DVFFR were 94.7%, 88.6%, 90.8%, 82.7%, and 96.7%, respectively. The area under the receiver operating characteristics curve (AUC) was 0.95 for DVFFR and 0.56 for CTA-based assessment with a significant difference (P < 0.0001). At patient level, sensitivity, specificity, accuracy, PPV and NPV of DVFFR were 93.8%, 88.0%, 90.3%, 83.0%, and 95.8%, respectively. The computation for DVFFR was fast with the average time of 22.5 ± 1.9 s. Conclusions:The results demonstrate that DVFFR was able to evaluate lesion hemodynamic significance accurately and effectively with improved diagnostic performance over CTA alone. Coronary artery disease (CAD) is a critical disease in which coronary artery luminal narrowing may result in myocardial ischemia. Early and effective assessment of myocardial ischemia is essential for optimal treatment planning so as to improve the quality of life and reduce medical costs.

Previous studies have provided relatively limited evidence in examining the impact of preoperative serum albumin levels on the length of hospital stay (LOS) in patients with hip fractures. This study aimed to elucidate the association between preoperative serum albumin levels and LOS in elderly patients with hip fractures. This retrospective cohort study included 1444 elderly patients undergoing surgical treatment for hip fractures at the Second People’s Hospital of Shenzhen from January 2012 to December 2021. The main exposure variable was serum albumin level, and the outcome variable was LOS. A generalized additive model was used to identify the nonlinear relationship between serum albumin levels and LOS, and saturation effects were calculated using a two-piecewise linear regression model. Subgroup analysis was conducted using stratified logistic regression. The median LOS was 10 (8–15) days among all included patients. After adjusting for potential confounding factors (age, sex, comorbidities, hypertension, coronary heart disease, stroke, diabetes, duration of surgery, surgical method, intraoperative blood loss, red cell distribution width, estimated glomerular filtration rate, blood urea nitrogen, postoperative pneumonia, and transfer to ICU), a nonlinear relationship was found between serum albumin levels and LOS, with a turning point at 3.06. On the left side of the turning point, the effect size was not statistically significant (β = 1.84, 95% CI −1.00 to 4.68, P = .2042), while on the right side of the turning point, for every 1 g/dL increase in albumin, LOS decreased by 1.05 days (β = −1.05, 95% CI −1.90 to −0.20, P = .0162). A nonlinear relationship was observed between preoperative serum albumin levels and LOS in elderly patients with hip fractures. When albumin levels were >3.06 g/dL, they were negatively correlated with LOS. This has important implications for clinicians in the development of nutritional improvement strategies.

Groin complications are considered to be the most common adverse events during cryoballon ablation. We aimed to compare the safety and cost-effectiveness of the LaPFOE technique (combined low approach femoral venous puncture, pre-dilation with a femoral artery sheath core, and figure-of-eight suture) with double-ProGlide technique and manual compression after cryoballoon ablation for atrial fibrillation with uninterrupted oral anticoagulants. Conventional manual compression, the double-ProGlide technique and the LaPFOE technique were applied in the first 100 patients, the following 92 patients, and the last 100 patients, respectively. The occurrence of groin complications was evaluated immediately and at the three-month follow-up. A total of 292 participants (71.23

BACKGROUND: This study was designed to evaluate the impact of blood eosinophil count (BEC) on adverse cardiovascular events (CVE) in patients with acute coronary syndrome (ACS) undergoing percutaneous coronary intervention (PCI), across varying different glucose metabolism statuses. METHOD: Patients were classified into three groups based on glucose metabolism status (diabetes mellitus [DM], prediabetes mellitus [preDM], and normal glucose regulation [NGR]). They were further divided into quintiles (Q1-Q5) based on BEC levels. The primary CVE endpoint comprised cardiac death, nonfatal stroke or myocardial infarction, and target vessel revascularization, monitored over a five-year follow-up period. RESULTS: During the follow-up, a total of 866 CVE were recorded. Patients in Q1 and Q5 demonstrated significantly higher CVE risk within the preDM and DM groups. Kaplan-Meier survival analysis revealed that Q3 exhibited the lowest incidence of CVE (p < 0.001). Incorporating BEC into the Cox regression model improved the C-statistics for CVE prediction in both preDM and DM groups. Restricted cubic spline analysis indicated a U-shaped relationship between BEC and CVE risk, with an identified inflection point at 0.11. CONCLUSION: These findings provided the first clinical evidence that BEC levels were associated with CVE risk in ACS patients with preDM or DM undergoing PCI.

Objective:This research aimed to address the critical need for effective prognostic tools in patients with acute coronary syndrome (ACS) and type 2 diabetes mellitus (T2DM) undergoing percutaneous coronary intervention (PCI) by exploring the potential significance of integrating estimated glucose disposal rate (eGDR) and neutrophil-to-lymphocyte ratio (NLR). Methods:Major adverse cardiovascular and cerebrovascular events (MACCE) were the primary endpoint. Log rank test was conducted to compare the Kaplan-Meier curves across the overall follow-up period, and multivariate Cox regression was used to investigate the association between the eGDR/NLR and MACCE. Results:One hundred fifty-four patients (9.5%) experienced MACCE including 15 cardiac deaths, 97 nonfatal MI, 120 TVR, and 10 strokes. Patients were distributed into low and high eGDR/NLR groups (lower eGDR [eGDR-L] group, higher eGDR [eGDR-H] group, lower NLR [NLR-L] group, and higher NLR [NLR-H] group) based on the median value of eGDR and NLR, further divided into four groups: eGDR-L + NLR-L, eGDR-H + NLR-L, eGDR-L + NLR-H, and eGDR-H + NLR-H. eGDR-L + NLR-H group exhibited significantly higher risks of MACCE (17.4%), compared to another three groups. An independent correlation between eGDR/NLR and MACCE was demonstrated by Cox regression analysis, establishing if the eGDR and NLR was treated as a continuous or categorical variable. Compared to eGDR-H + NLR-L group, patients in eGDR-L + NLR-H group had the uppermost MACCE risk (HR: 5.201; 95% CI 2.764-7.786; P < 0.001). A linear relationship between eGDR/ NLR and MACCE was showed by restricted cubic spline curves. Incorporating the eGDR and NLR toward the baseline risk model developed the precision of forecasting MACCE (baseline risk model-AUC: 0.611 vs baseline risk model + eGDR + NLR-AUC: 0.695, P < 0.001). Conclusion:Combining eGDR with NLR can be utilized to forecast long-term MACCE and substantially improve the accuracy of risk stratification in ACS patients with T2DM following PCI.

Remnant cholesterol (RC) exert a significant influence on atherosclerotic cardiovascular disease development. However, the prognostic implications of RC in menopausal women received percutaneous coronary intervention (PCI) who experiencing acute coronary syndrome (ACS) remain uncertain. RC was derived by subtracting the sum of high-density lipoprotein cholesterol and low-density lipoprotein cholesterol from the total cholesterol. Kaplan-Meier survival and Cox regression analysis were employed for assessing the correlation between continuous RC levels and composite and individual adverse events in Q1-Q4 quartiles. Receiver operator characteristic (ROC) curves, derived from Cox regression, were employed for analyzing the relationship between RC and both composite and individual adverse events. 1505 consecutive menopausal women who underwent PCI and diagnosed with ACS were included. Kaplan-Meier survival analysis demonstrated a progressive reduction in composite adverse event survival rates across the four groups, observed in both the general population and among diabetic individuals, as RC values increased (Log-rank P < 0.001). The analysis of multivariate Cox regression indicated RC remained independently associated with both composite and individual adverse events. ROC analysis showed that RC enhanced the area under the curve both in total and diabetic populations for composite adverse events. Among menopausal women diagnosed with ACS who underwent PCI, heightened levels of RC were found to be independently correlated with an increased occurrence of adverse events.

Glycated Albumin (GA) and atherogenic index of plasma (AIP) are two important biomarkers that respectively reflect lipid and glucose levels. Previous research has revealed their roles in cardiovascular diseases (CVD) and diabetes. However, their combined predictive ability in forecasting cardiovascular events (CVE) after percutaneous coronary intervention (PCI) among postmenopausal acute coronary syndrome (ACS) patients remains insufficiently studied. Based on the levels of AIP (AIP-L and AIP-H) and GA (GA-L and GA-H), four groups were used to categorize the patients. The CVE assessed included cardiac death, nonfatal myocardial infarction (MI) and nonfatal stroke. To evaluate the relationship between AIP, GA, and CVE, multivariate Cox regression analyses were performed. 98 patients (7.5

Background Metabolic abnormalities are associated with the occurrence, severity, and poor prognosis of coronary artery disease (CAD), some of which affect the antiplatelet efficacy of clopidogrel. Free fatty acids (FFAs) is a biomarker for metabolic abnormalities, and elevated FFAs is observed among CAD patients. Whether FFAs enhances residual platelet reactivity induced by adenosine diphosphate (ADP) while using clopidogrel was unknown. The purpose of our study is exploring the issue. Method Current study included 1,277 CAD patients using clopidogrel and used logistic regression to detect whether the higher level of FFAs is associated with high residual platelet reactivity (HRPR). We additionally performed subgroup and sensitivity analyses to evaluate the stability of the results. We defined HRPR as ADP-induced platelet inhibition rate (ADP i ) < 50% plus ADP-induced maximum amplitude (MA ADP ) > 47 mm. Results 486 patients (38.1%) showed HRPR. The proportion of HRPR among patients with higher FFAs (>0.445 mmol/L) is greater than among patients with lower FFAs (46.4% vs. 32.6%, P < 0.001). Multivariate logistic regression demonstrated that higher FFAs (>0.445 mmol/L) is independently associated with HRPR (adjusted OR = 1.745, 95% CI, 1.352–2.254). After subgroup and sensitivity analyses, the results remained robust. Conclusion The higher level of FFAs enhances residual platelet reactivity induced by ADP and is independently associated with clopidogrel HRPR.

Contrast-induced encephalopathy (CIE) is considered as an uncommon complication following cardiac catheterization. Due to the varied manifestations, CIE has no formal diagnostic criteria. In fact, the incidence of CIE may be greatly underestimated because of the difficulty in its differential diagnosis with other cerebrovascular complications. Thus, making a flow diagram according to patients’ clinical symptoms and examinations after cardiac catheterization to help clinicians diagnose CIE is important and needed. In this report, we describe a case of probable CIE in a 66-year-old Chinese man with hypertension who underwent cardiac catheterization with stents placement in the bifurcation lesion, during which 80 ml iopromide contrast was used. About 2 h following the procedure, the patient lost his consciousness suddenly and suffered from a status epilepticus. Malignant arrhythmias were not found through continuous electrocardiogram monitoring, but mild ST-segment elevation was displayed in leads I and aVL. The echocardiography, plasma glucose and electrolyte levels were normal. Emergency re-angiography with percutaneous transluminal coronary angioplasty was performed in the culprit lesion, which involved 60 ml iopromide contrast. However, the patient remained unconsciousness and epilepticus. Non-contrast computed tomography (CT) of the head showed cortical and subarachnoid enhancement as well as prolonged retention of contrast media in the middle cerebral artery. With supportive treatment of intravenous hydration, sedative and dehydrant, the patient recovered 3 h later and finally discharged without any neurological deficits. CIE is an acute reversible encephalopathy induced by contrast media. It is exceptionally challenging to make the diagnosis of CIE following cardiac catheterization since there is a lack of consensus on the definition of CIE. Via this case we reviewed the related literatures, through which a flow diagram of the differential diagnosis and clinical decision making was given, which could help to differentiate CIE from other neurological complications following cardiac catheterization.

[This corrects the article DOI: 10.3389/fcvm.2023.1115142.].

目的 探讨复心合剂联合琥珀酸美托洛尔缓释片治疗冠状动脉肌桥心绞痛患者的临床疗效.方法 选择2020年2月—2022年4月于山东中医药大学附属医院、中国中医科学院广安门医院及首都医科大学附属北京安贞医院心内科就诊的冠状动脉肌桥心绞痛患者92例,根据随机数字表法分为对照组与观察组各46例,对照组给予琥珀酸美托洛尔缓释片治疗,观察组在对照组基础上联合复心合剂治疗,观察2组临床疗效,治疗前后中医证候评分、平板运动心电图指标(运动心绞痛时间、运动ST段下移0.1 mV时间)、西雅图心绞痛量表(SAQ)评分、血管内皮功能[血清内皮素(ET-1)、一氧化氮(NO)、血管内皮功能指数(RHI)].结果 治疗2个月后观察组总有效率高于对照组(89.13％vs.76.09％,χ2/P=7.568/0.023);治疗2个月后2组中医证候评分中胸闷或胸痛、气短乏力、心慌失眠、食欲不振等评分均较治疗前降低,且观察组低于对照组(t/P=3.318/0.001、4.511/<0.001、2.209/0.030、2.142/0.035).治疗2个月后2组运动心绞痛时间、运动ST段下移0.1 mV时间、SAQ评分(躯体活动受限程度、心绞痛稳定状态、心绞痛发作情况、治疗满意程度、疾病认知程度)均较治疗前升高,且观察组高于对照组(t/P=4.715/<0.001、6.736/<0.001、2.844/0.006、2.293/0.024、3.190/0.002、6.084/<0.001、2.229/0.028).治疗2个月后2组ET-1水平均较治疗前降低,NO水平及RHI指数均较治疗前升高,且观察组优于对照组(t/P=8.213/<0.001、3.073/0.003、2.472/0.015).结论 复心合剂联合琥珀酸美托洛尔缓释片治疗冠状动脉肌桥心绞痛患者疗效显著.

OBJECTIVE:To access the efficacy and safety of the double-ProGlide technique for the femoral vein access-site closure in cryoballoon ablation with uninterrupted oral anticoagulants (OAC), and its impact on the electrophysiology laboratory time as well as hospital stay after the procedure in this observational study. METHODS:Patients with atrial fibrillation undergoing cryoballoon ablation with uninterrupted OAC at Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China from May 2019 to May 2021 were enrolled in this study. From October 2020, double-ProGlide technique was consistently used for hemostasis (ProGlide group), and before that conventional manual compression was utilized (manual compression group). The occurrence of vascular and groin complications was accessed during the hospital stay and until the three-month follow-up. RESULTS:A total of 140 participants (69.30% of male, mean age: 59.21 ± 10.29 years) were evaluated, 70 participants being in each group. Immediate hemostasis was achieved in all the patients with ProGlide closure. No major vascular complications were found in the ProGlide group while two major vascular complications were occurred in the manual compression group. The incidence of any groin complication was obviously higher in subjects with manual compression than patients with ProGlide devices (15.71% vs. 2.86%, P = 0.009). In addition, compared with the manual compression group, the ProGlide group was associated with significantly shorter total time in the electrophysiology laboratory [112.0 (93.3-128.8) min vs. 123.5 (107.3-158.3) min, P = 0.006], time from sheath removal until venous site hemostasis [3.8 (3.4-4.2) min vs. 8.0 (7.6-8.5) min, P < 0.001], bed rest time [8.0 (7.6-8.0) h vs. 14.1 (12.0-17.6) h, P < 0.001] and hospital stay after the procedure [13.8 (12.5-17.8) h vs. 38.0 (21.5-41.0) h, P < 0.001]. CONCLUSIONS:Utilization of the double-ProGlide technique for hemostasis after cryoballoon ablation with uninterrupted OAC is feasible and safe, which has the clinical benefit in reducing the total electrophysiology laboratory time and the hospital stay length after the procedure.

BACKGROUND:Systemic immune-inflammation index (SII) is a novel inflammatory marker based on neutrophils, platelets and lymphocytes counts, which has potential prognostic value among coronary artery disease (CAD) patients as described by some observational studies. We aimed to provide higher-certainty evidence to verify the association of SII with poor outcomes of CAD patients.METHODS:PubMed, Web of Science, Embase, Ovid and Scopus were searched to find relevant literature exploring the prognostic value of SII among CAD patients. Hazard ratios (HRs) with 95% confidence intervals (CIs) extracted from the literature included were pooled with the fixed-effect or random-effect model. Sensitivity analyses and subgroup analyses were conducted to detect the source of heterogeneity and evaluate the stability of results.RESULTS:A total of nine studies with 15,832 participants were included. The quantitative synthesis including eight studies with 15,657 participants showed that the high SII was related to the major adverse cardiovascular event in CAD patients (HR with 95% CI: 2.36 [1.67, 3.33]). After eliminating heterogeneity and adjusting for publication bias, the above result was still robust (HR with 95% CI: 1.67 [1.32, 2.12]). Additionally, we also demonstrated the prognostic values of SII for all-cause death, cardiovascular death, myocardial infarction and stroke.CONCLUSION:Higher SII has prognostic values for adverse outcomes in CAD patients.

BACKGROUND:High levels of lipoprotein(a) [Lp(a)] are linked to adverse cardiovascular events. The significance of Lp(a) for the survival of octogenarians with coronary artery disease (CAD) after drug-eluting stent (DES) insertion is, however, not known. The purpose of the study is to investigated the connection between Lp(a) and outcome in octogenarians with CAD after DES implantation. METHODS:We retrospectively enrolled a total of 506 consecutive octogenarians with CAD and DES implantation in our institution between January 2015 to August 2018. Two patient groups were established: a low group with plasma Lp(a) lower than 50 mg/dL (n = 408) and a high group with values above 50 mg/dL (n = 98). RESULTS:After following up for a median of 31.53 ± 8.22 months, Kaplan-Meier curves indicated that poorer outcome censored for major cardiovascular events (MACE), myocardial infarction (MI), and target vessel revascularization (TVR) in the high group relative to the low group (log-rank test p = 0.001, p = 0.008, and p < 0.001, respectively). High Lp(a) independently predicted MACE (hazard ratio (HR) 1.90; 95% confidence interval (CI) 1.28-2.84; p = 0.002), MI (HR 2.74; 95% CI 1.23-6.11; p = 0.014), and TVR (HR 3.65; 95% CI 1.99-6.69; p < 0.001) after covariate adjustment. CONCLUSIONS:High Lp(a) was also significantly related to poor long-term outcome in octogenarians with CAD after DES implantation.

Background and aims: although an association between metabolic syndrome (MS) and cardiovascular disease risk has been documented, the relationship in patients with complex calcified coronary lesions undergoing rotational atherectomy (RA) and drug-eluting stent(DES) insertion remains controversial. Here, the influence of MS on outcomes was assessed. Methods and results: we retrospectively included 398 patients who underwent RA and DES insertion for complex calcified coronary lesions in our institution between June 2015 and January 2019. The modified Adult Treatment Plan III was used to diagnose MS. The endpoint was major adverse cardiovascular events (MACEs), comprising mortality from all causes, myocardial infarction, and target vessel revascularization (TVR). In all, 173 (43.5%) patients had MS. MS was significantly associated with MACE over the 28.32 ± 6.79-month follow-up period (HR 1.783, 95% CI from 1.122 to 2.833) even after adjustment for other possible confounders. Conclusion: MS was frequently observed in patients treated with RA with DES insertion for complex calcified coronary lesions. MS independently predicted MACE in these patients.

目的：越清高水平脂蛋白a[Lp（a）]与心血管疾病的不良预后密切相关，故本研究评估Lp（a）对行冠状动脉旋磨术患者全因死亡率的影响。方法：分析自2015年1月至2018年5月，在首都医科大学附属北京安贞医院心内科成功行冠状动脉旋磨术的309例冠心病患者，根据Lp（a）水平，将患者分为高Lp（a）组[n=55,Lp（a）＞50g/L]和低Lp（a）组[n=254,Lp（a）≤50g/L]，采用Kaplan-Meier生存曲线和Cox比例危险模型分析两组患者的全因死亡率。结果：高Lp（a）组血脂异常明显高于低Lp（a）组（P＜0.001）。平均随访（30.77±6.70）个月，Kaplan-Meier生存曲线显示,高Lp（a）组患者的全因死亡率明显高于低Lp（a）组（P=0.016），有统计学意义。调整混杂因素后，高Lp（a）出现全因死亡的（HR=2.107,95%CI:1.074～4.131,P=0.030）。结论：高Lp（a）明显增加行冠状动脉旋磨术冠心病患者的的全因死亡率。

BACKGROUND:There is a lack of large-scale data on the clinical and genotype characteristics of homozygous familial hypercholesterolemia (HoFH) patients in Asia. OBJECTIVE:To define the characteristics of phenotypic and genetic HoFH probands from mainland China. METHODS:We collected data from patients with suspected HoFH from ten clinical hospitals across mainland China from 2003 to 2019. Clinical data and DNA testing were obtained in all patients. The Kaplan-Meier method was used to generate survival curves, and the groups were compared with the log-rank test. RESULTS:A total of 108 unrelated probands with suspected HoFH (mean age 14.9 years) were included. The three most common variants were W483X (c.1448 G>A), A627T (c.1879 G>A), H583Y (c.1747 C>T). The majority (64.8%) were compound heterozygotes (n = 70), 23 (21.3%) were true HoFH patients. True HoFH showed higher LDL-C levels compared to compound HoFH (16.8±3.6 mmol/L vs. 15.0±3.1 mmol/L, P = 0.022). During follow-up, only 21.2% patients exhibited an LDL-C reduction of more than 50%. Kaplan-Meier analysis showed that the true HoFH probands had significantly worse survival rates compared to other genotype probands (13-year survival; 20.3% vs. 76.7%, respectively; P = 0.016). In addition, true HoFH shows that 2.8-fold (P = 0.022) increase any death and 3.0-fold (P = 0.023) increase cardiovascular death risk in relative to other FH. CONCLUSIONS:This report shows that HoFH has devastating consequences, and that patients are often only diagnosed after they have been exposed to severely elevated LDL-C for years. Systematic screening and early intensive treatment are an absolute requirement for these young individuals with HoFH.