Evaluation of Naples prognostic score to predict long-term mortality in patients with pulmonary embolism.

Background: The Naples prognostic score (NPS), which reflects the inflammatory and nutritional status of patients, is often used to determine prognosis in cancer patients. The aim of this study was to determine the long-term prognostic value of the NPS in acute pulmonary embolism (APE) patients. Methods: Two hundred thirty-nine patients diagnosed with APE were divided into two groups according to their NPS, and long-term mortality was compared. Results: The long-term mortality was observed in 38 patients out of 293 patients in the mean follow-up of 24 months. Multivariate analysis showed that NPS as a categorical parameter and NPS as a numeric parameter were independent predictors of long-term mortality. Conclusion: This study highlights that NPS may have the potential to predict long-term mortality in APE patients.

Successful Percutaneous Closure of an Aorto-right Ventricular Fistula After Sutureless Aortic Valve Replacement: A Case Report.

Rupture of a sinus of valsalva aneurysm (SVA) and the development of an aorto-right ventricular fistula (ARVF) is a rare condition, associated with high morbidity and mortality rates if left untreated. Opening of the SVA rupture into the right heart chambers may result in various morbidities, such as pulmonary hypertension. We present a case of a patient who developed ARVF following sutureless aortic valve replacement, and was subsequently treated successfully via a percutaneous approach.

The prognostic importance of the Naples prognostic score for in-hospital mortality in patients with ST-segment elevation myocardial infarction.

BACKGROUND: The Naples prognostic score (NPS) is an effective inflammatory and nutritional scoring system widely applied as a prognostic factor in various cancers. However, the prognostic significance of NPS is unknown in ST-segment elevation myocardial infarction (STEMI). We aimed to analyze the prognostic value of the NPS in-hospital mortality in patients with STEMI. METHODS: The study consisted of 3828 patients diagnosed with STEMI who underwent primer percutaneous coronary intervention. As the primary outcome, in-hospital mortality was defined as all-cause deaths during hospitalization. The included patients were categorized into three groups based on NPS (group 1:NPS = 0,1,2; group 2:NPS = 3; group 3:NPS = 4). RESULTS: Increased NPS was associated with higher in-hospital mortality rates( P  < 0.001). In the multivariable logistic regression analysis, the relationship between NPS and in-hospital mortality continued after adjustment for age, male sex, diabetes, hypertension, Killip score, SBP, heart rate, left ventricular ejection fraction, myocardial infarction type and postprocedural no-reflow. A strong positive association was found between in-hospital mortality and NPS by multivariable logistic regression analysis [NPS 0-1-2 as a reference, OR = 1.73 (95% CI, 1.04-2.90) for NPS 3, OR = 2.83 (95% CI, 1.76-4.54) for NPS 4]. CONCLUSION: The present study demonstrates that the NPS could independently predict in-hospital mortality in STEMI. Prospective studies will be necessary to confirm the performance, clinical applicability and practicality of the NPS for in-hospital mortality in STEMI.

Evaluation of the Relationship Between Diabetic Retinopathy and the Development of Left Atrial Stiffness in Patients with Diabetes Mellitus.

OBJECTIVE: Based on several studies, atrial remodeling results in an increase in left atrial (LA) stiffness, which is indicative of a worsened reservoir function. A typical microvascular consequence of diabetes mellitus (DM) is diabetic retinopathy. Therefore, the objective of this study was to assess the factors that might be related to LA stiffness in DM patients. MATERIALS AND METHODS: There were 200 DM patients in the study population. The LA stiffness value of 0.33 led to the division of the patients into 2 groups. According to these groups, the parameters to predict the development of the LA stiffness were investigated. RESULTS: The patient population's median age was 54.7 ± 9.4 years, and of them, 105 (52.5% of the population) were men. Retinopathy was substantially linked with LA stiffness. Interventricular septum thickness (B coefficient: 0.261, 95% CI 0.128; 0.394; P < .001), mitral annular plane systolic excursion (B coefficient: -0.350, 95% CI -0.489; -0.2212; P < 0.001), and retinopathy (B coefficient: 0.644, 95% CI 0.307; 0.983; P < .001) were identified as independent predictors of the progression of LA stiffness by the linear regression model. CONCLUSION: The results of the current investigation demonstrated a correlation between higher LA stiffness values and the presence of diabetic retinopathy in diabetic patients.

Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy and future perspectives.

BACKGROUND: The hypertrophic cardiomyopathy (HCM) risk- sudden cardiac death (SCD) model provides a convenient tool for determining the risk of SCD in patients with HCM even though some patients with low-risk scores still remain at risk of SCD. Hence, the aim of our study was to assess the performance of HCM Risk-SCD in a large series of consecutive patients with HCM who had been followed up in a tertiary center. METHODS: The study population consisted of 389 consecutive HCM patients who had been followed up between 2004 and 2021. Demographic and clinical characteristics, estimated 5-year risk using the HCM Risk-SCD model, were compiled, and survival data were collected during follow-up. Patients were divided into 2 groups according to their long-term survival, and HCM risk-SCD scores of these two groups were compared. RESULTS: The long-term mortality was observed in 47 patients out of 389 patients in the during a mean follow-up of 55.5 ± 12.7 months. The mean HCM Risk-SCD score of surviving patients was significantly lower than that of non-survivors (1.8% vs. 3.0%, p < .001). The HCM Risk-SCD score was above 6% in nine (2.6%) survivors and nine (19.1%) non-survivors (p < .001). The ROC curve based on the HCM Risk-SCD score had 61% sensitivity and 61% specificity for risk threshold of for 2.0%, 38% sensitivity and 99% specificity a threshold of ≥4%, 17% sensitivity, and 99% specificity for a threshold of ≥6%. CONCLUSION: A new risk algorithm with higher sensitivity is needed, although the HCM risk-SCD model is still quite useful in identifying patients at a high risk for SCD.

Effect of ivabradine on ventricular arrhythmias in heart failure patients with reduced ejection fraction.

BACKGROUND/INTRODUCTION: Heart failure patients with reduced ejection fraction are at high risk for ventricular arrhythmias and sudden cardiac death. Ivabradine, a specific inhibitor of the If current in the sinoatrial node, provides heart rate reduction in sinus rhythm and angina control in chronic coronary syndromes. OBJECTIVE: The effect of ivabradine on ventricular arrhythmias in heart failure patients with reduced ejection fraction patients has not been fully elucidated. The aim of this study was to investigate the effect of ivabradine use on life-threatening arrhythmias and long-term mortality in heart failure patients with reduced ejection fraction patients. METHODS: In this retrospective study, 1,639 patients with heart failure patients with reduced ejection fraction were included. Patients were divided into two groups: ivabradine users and nonusers. Patients presenting with ventricular tachycardia, the presence of ventricular extrasystole, and ventricular tachycardia in 24-h rhythm monitoring, appropriate implantable cardioverter-defibrillator shocks, and long-term mortality outcomes were evaluated according to ivabradine use. RESULTS: After adjustment for all possible variables, admission with ventricular tachycardia was three times higher in ivabradine nonusers (95% confidence interval 1.5-10.2). The presence of premature ventricular contractions and ventricular tachycardias in 24-h rhythm Holter monitoring was notably higher in ivabradine nonusers. According to the adjusted model for all variables, 4.1 times more appropriate implantable cardioverter-defibrillator shocks were observed in the ivabradine nonusers than the users (95%CI 1.8-9.6). Long-term mortality did not differ between these groups after adjustment for all covariates. CONCLUSION: The use of ivabradine reduced the appropriate implantable cardioverter-defibrillator discharge in heart failure patients with reduced ejection fraction patients. Ivabradine has potential in the treatment of ventricular arrhythmias in heart failure patients with reduced ejection fraction patients.

Efficiency of MVP ECG Risk Score for Prediction of Long-Term Atrial Fibrillation in Patients With ICD for Heart Failure With Reduced Ejection Fraction.

BACKGROUND AND OBJECTIVES: The morphology-voltage-P-wave duration (MVP) electrocardiography (ECG) risk score is a newly defined scoring system that has recently been used for atrial fibrillation (AF) prediction. The aim of this study was to evaluate the ability of the MVP ECG risk score to predict AF in patients with an implantable cardioverter defibrillator (ICD) and heart failure with reduced ejection fraction in long-term follow-up. METHODS: The study used a single-center, and retrospective design. The study included 328 patients who underwent ICD implantation in our hospital between January 2010 and April 2021, diagnosed with heart failure. The patients were divided into low, intermediate and high-risk categories according to the MVP ECG risk scores. The long-term development of atrial fibrillation was compared among these 3 groups. RESULTS: The low-risk group included 191 patients, the intermediate-risk group 114 patients, and the high-risk group 23 patients. The long-term AF development rate was 12.0% in the low-risk group, 21.9% in the intermediate risk group, and 78.3% in the high-risk group. Patients in the high-risk group were found to have 5.2 times higher rates of long-term AF occurrence compared to low-risk group. CONCLUSIONS: The MVP ECG risk score, which is an inexpensive, simple and easily accessible tool, was found to be a significant predictor of the development of AF in the long-term follow-up of patients with an ICD with heart failure with reduced ejection fraction. This risk score may be used to identify patients who require close follow-up for development and management of AF.

The prognostic impact of inflammation in patients with decompensated acute heart failure, as assessed using the pan-immune inflammation value (PIV).

There is increasing evidence that composite scores based on blood counts, which are reflectors of uncontrolled inflammation in the development and progression of heart failure, can be used as prognostic biomarkers in heart failure patients. The prognostic effects of pan-immune inflammation (PIV) as an independent predictor of in-hospital mortality in patients with acute heart failure (AHF) were evaluated based on this evidence. The data of 640 consecutive patients hospitalized for New York Heart Association (NYHA) class 2-3-4 AHF with reduced ejection fraction were analyzed and 565 patients were included after exclusion. The primary outcome was in hospital all-cause death. Secondary outcomes were defined as the following in-hospital events: Acute kidney injury (AKI), malignant arrhythmias, acute renal failure (ARF) and stroke. The PIV was computed using hemogram parameters such as lymphocytes, neutrophils, monocytes and platelets. Patients were categorized as low or high PIV group according to the median value, which was 382.8. A total of 81 (14.3%) in-hospital deaths, 31 (5.4%) AKI, 34 (6%) malignant arrhythmias, 60 (10.6%) ARF and 11 (2%) strokes were reported. Patients with high PIV had a higher in-hospital mortality rate than patients with low PIV (OR: 1.51, 95% CI, 1.26-1.80, p < 0.001). Incorporating PIV into the full model significantly improved model performance (odds ratio X2, p < 0.001) compared to the baseline model constructed with other inflammatory markers. PIV is a potent predictor of prognosis with better performance than other well-known inflammatory markers for patients with AHF.

Left Ventricular Mass Index Predicts Pacemaker-Induced Cardiomyopathy in Patients with Dual Chamber Permanent Pacemakers Implanted Due to Complete Atrioventricular Block.

Background: Pacing-induced cardiomyopathy (PICM) occurs as a result of high-burden right ventricular (RV) pacing, which usually develops in patients with complete atrioventricular (AV) block. There is a paucity of data on the association between PICM and pre-implantation left ventricular mass index (LVMI). Thus, the purpose of this study was to analyze the influence of LVMI on PICM in patients who had dual chamber permanent pacemakers (PPMs) implanted secondary to complete AV block. Methods: Overall, 577 patients with dual chamber permanent pacemakers (PPMs) were classified into three tertiles according to their pre- implantation LVMI. The average follow-up period was 57 ± 38 months. The baseline characteristics, laboratory and echocardiographic variables were compared between the tertiles. PICM was defined as a ≥ 10% drop in left ventricular ejection fraction (LVEF) from pre-implantation with a resultant LVEF < 50%. PICM occurred in 42 (7.2%) patients. The independent predictors of PICM development, as well as the impact of LVMI on PICM, were investigated. Results: After controlling for confounding baseline variables, the tertile with the greatest LVMI had a 1.8 times higher risk for the development of long-term PICM compared with the tertile with the lowest LVMI, which was accepted as the reference group. A receiver operating characteristic curve analysis revealed that the best LVMI cut- off value for predicting long-term PICM was 109.8 g/m2 with 71% sensitivity and 62% specificity (area under curve: 0.68; 95% confidence interval: 0.60-0.76; p < 0.001). Conclusions: This investigation revealed that pre-implantation LVMI had a prognostic role in predicting PICM in patients with an implanted dual chamber PPM due to complete AV block.

Primary Right Atrial Cardiac Angiosarcoma in Patient With Poland Syndrome: Case Report and Review of the Literature.

This article presents the case of a 24-year-old woman with Poland syndrome who developed primary right atrial cardiac angiosarcoma. The patient presented to the hospital with dyspnea and chest pain, and imaging studies revealed a large mass attached to the right atrium. Urgent surgery was performed to remove the tumor, and the patient underwent adjuvant chemotherapy afterward. Follow-up exams showed no signs of the tumor or any complications from treatment. Poland syndrome is a rare congenital disorder characterized by the absence of unilateral large pectoral muscle, ipsilateral symbrachydactyly, and other malformations of the anterior chest wall and breast. Although the condition does not predispose patients to malignancy, different pathologies can be seen in these patients due to the unknown etiology of the syndrome. Primary right atrial cardiac angiosarcoma is a rare malignancy, and its coexistence with Poland syndrome has not been well established in the literature. This case report highlights the need to consider cardiac angiosarcoma as a possible diagnosis in patients with Poland syndrome who present with cardiac symptoms.

Arrhythmias Beyond Atrial Fibrillation Detection Using Smartwatches: A Systematic Review.

BACKGROUND: Over the past few years, smartwatches have become increasingly popular in the monitoring of arrhythmias. Although the detection of atrial fibrillation with smartwatches has been the subject of various articles, there is no comprehensive research on the detection of arrhythmias other than atrial fibrillation. In this study, we included individual cases from the literature to identify the characteristics of patients with smartwatch-detected arrhythmias other than atrial fibrillation. METHODS: PubMed, Embase, and SCOPUS were searched for case reports, case series, or cohort studies that reported individual participant-level data, until January 6, 2022. The following search string was used for each databases: ('Smart Watch' OR 'Apple Watch' OR 'Samsung Gear') AND ('Supraventricular Tachycardia' OR 'Cardiac Arrhythmia' OR 'Ventricular Tachycardia' OR 'Atrioventricular Nodal Reentry Tachycardia' OR 'Atrioventricular Reentrant Tachycardia' OR 'Heart Block' OR 'Atrial Flutter' OR 'Ectopic Atrial Tachycardia' OR 'Bradyarrhythmia'). RESULTS: A total of 52 studies from PubMed, 20 studies from Embase, and 200 studies from SCOPUS were identified. After screening, 18 articles were included. A total of 22 patients were obtained from 14 case reports or case series. Four cohort studies evaluating various arrhythmias were included. Arrhythmias, including ventricular tachycardia, atrial fibrillation, atrial flutter, atrioventricular nodal reentry tachycardia, atrioventricular reentrant tachycardia, second- or third-degree atrioventricular block, and sinus bradycardia, were detected with smartwatches. CONCLUSIONS: Cardiac arrhythmias other than atrial fibrillation are also commonly detected with smartwatches. Smartwatches have an important potential besides traditional methods in the detection of arrhythmias and clinical practice.

Machine Learning Approach on High Risk Treadmill Exercise Test to Predict Obstructive Coronary Artery Disease by using P, QRS, and T waves' Features.

Treadmill Exercise Test (TET) results and patients' clinical symptoms influence cardiologists' decision to perform Coronary Angiography (CAG) which is an invasive procedure. Since TET has high false positive rates, it can cause an unnecessary invasive CAG. Our primary objective was to develop a machine learning model capable of optimizing TET performance based on electrocardiography (ECG) waves characteristics and signals. TET reports from 294 patients who underwent CAG following high risk TET were collected and categorized into those with critical CAD and others. The signal was converted to time series format. A dataset containing the P, QRS, and T wave times and amplitudes was created. Using this dataset, 5 machine learning algorithms were trained with 5-fold cross validation. All these models were then compared to the performance of cardiologists on V5 signal. The results from 5 machine learning models were clearly superior to the cardiologists' V5 signal performance (P < 0.0001). In addition, the XGBoost model, with an accuracy of 80.92±6.42% and an area under the curve (AUC) of 0.78±0.06, was the most successful model. Machine learning models can produce high-performance diagnoses using the V5 signal markers only as it does not require any clinical markers obtained from TET reports. This can lead to significant contributions to improving clinical prediction in non-invasive methods.

Effect of ivabradine on ventricular arrhythmias in heart failure patients with reduced ejection fraction

SUMMARY BACKGROUND/INTRODUCTION: Heart failure patients with reduced ejection fraction are at high risk for ventricular arrhythmias and sudden cardiac death. Ivabradine, a specific inhibitor of the If current in the sinoatrial node, provides heart rate reduction in sinus rhythm and angina control in chronic coronary syndromes. OBJECTIVE: The effect of ivabradine on ventricular arrhythmias in heart failure patients with reduced ejection fraction patients has not been fully elucidated. The aim of this study was to investigate the effect of ivabradine use on life-threatening arrhythmias and long-term mortality in heart failure patients with reduced ejection fraction patients. METHODS: In this retrospective study, 1,639 patients with heart failure patients with reduced ejection fraction were included. Patients were divided into two groups: ivabradine users and nonusers. Patients presenting with ventricular tachycardia, the presence of ventricular extrasystole, and ventricular tachycardia in 24-h rhythm monitoring, appropriate implantable cardioverter-defibrillator shocks, and long-term mortality outcomes were evaluated according to ivabradine use. RESULTS: After adjustment for all possible variables, admission with ventricular tachycardia was three times higher in ivabradine nonusers (95% confidence interval 1.5-10.2). The presence of premature ventricular contractions and ventricular tachycardias in 24-h rhythm Holter monitoring was notably higher in ivabradine nonusers. According to the adjusted model for all variables, 4.1 times more appropriate implantable cardioverter-defibrillator shocks were observed in the ivabradine nonusers than the users (95%CI 1.8-9.6). Long-term mortality did not differ between these groups after adjustment for all covariates. CONCLUSION: The use of ivabradine reduced the appropriate implantable cardioverter-defibrillator discharge in heart failure patients with reduced ejection fraction patients. Ivabradine has potential in the treatment of ventricular arrhythmias in heart failure patients with reduced ejection fraction patients.

Long-term follow-up of patients with drug-related atrioventricular block without a need of permanent pacemaker during index hospitalization.

BACKGROUND: Most of the patients hospitalized due to drug-related atrioventricular (AV) block do not require permanent pacemaker implantation (PPI) since AV block regresses following cessation of the responsible drug. However, AV block requiring PPI may relapse in long-term follow-up. In this study, we retrospectively evaluated the factors predicting the need for a PPI in the long-term follow-up in patients admitted to our hospital with drug-related AV block but did not require PPI in index hospitalization. RESULTS: We evaluated 177 patients who had been hospitalized with drug-related AV block between January 2012 and July 2020 and who had not required PPI during hospital follow-up. The patients were divided into two groups according to whether PPI was performed or not. The independent predictors of long-term PPI were evaluated and the effect of glomerular filtration rate (GFR) of the patients during the index hospitalization on the long-term outcome was compared. A GFR above 60 ml/min is an independent significant risk factor in predicting long-term permanent pacemaker implantation in drug-related AV blocks. It is found that the need for PPI was 2.64 times higher without adjusted and 1.9 times higher with adjusted for all covariates in patients with GFR above 60 ml/min during hospitalization compared to those with GFR below 30 ml/min. CONCLUSIONS: GFR may be considered as an indicator of the PPI need in patients with drug-related atrioventricular AV block.

The relation between average 1-year home blood pressure and the change in pro-BNP and left ventricle mass index.

BACKGROUND: The purpose of this investigation was to examine the association between average 1-year home blood pressure and the change in left ventricular mass index (LVMI) and pro-brain natriuretic peptide (BNP) levels. METHODS: This prospective study was a subgroup analysis of lifestyle intervention using mobile technology in patients with high cardiovascular risk: a pragmatic randomized clinical Trial (LIGHT). In total, 242 patients were stratified into tertiles according to their average 1-year home blood pressure. RESULTS: Patients grouped into the tertile 3 (T3) had a lower 1-year mean, SBP and DBP. The T3 group had a 2.1 times higher rate of decrease in pro-BNP and a 1.6 times higher rate of decrease in LVMI compared with T1, compared with the reference group. The area under curve (AUC) value of average 1-year home blood pressure was higher than that of mean SBP or DBP. (AUC, 0.75 vs. AUC, 0.70 vs. AUC, 0.69, respectively). Spearman rank correlation demonstrated that average 1-year home blood pressure had a correlation with Δpro-BNP and ΔLVMI. CONCLUSION: The present study showed that average 1-year home blood pressure may have a significant association with a decrease in LVMI and pro-BNP. Our study appears to be the first to evaluate the association between average 1-year home blood pressure and the change in LVMI and pro-BNP.

Cardiac variables associated with atrial fibrillation occurrence and mortality in octogenarians implanted with dual chamber permanent pacemakers.

BACKGROUND: There is a dearth of data on the predictors of atrial fibrillation (AF) and the association between AF and long-term mortality in octogenarians with dual-chamber permanent pacemakers (PPM). We investigate the occurrence of AF and whether it is associated with overall mortality among octogenarians with dual-chamber PPM implants. METHODS: Three hundred and fifty-four patients with PPM implants were divided into two groups based on their long-term survival status. Baseline characteristics, laboratory variables, and echocardiographic variables were then compared between the groups, and independent predictors of the long-term incidence of AF and mortality were determined. RESULTS: Multivariable Cox regression analysis performed after adjusting for the parameters in univariable analysis revealed that diabetes, urea levels, albumin levels, paced QRS duration, and the frequency of atrial high-rate episodes (AHREs) were independently associated with a long-term risk of AF in octogenarians after having dual chamber PPMs implanted. The left ventricular (LV) ejection fraction, left atrial (LA) anteroposterior diameter, and AHRE + AF (HR 1.498, 95%CI 1.003-2.237, p = 0.048) were independent risk factors for the long-term mortality in octogenarians receiving dual-chamber PPMs implants. CONCLUSION: The occurrence of AF following dual-chamber PPM implantation is a significant prognostic factor in octogenarian patients.

COVID-19 vaccination and carditis in children and adolescents: a systematic review and meta-analysis.

BACKGROUND: Coronavirus Disease-2019 (COVID-19) vaccination has been associated with the development of carditis, especially in children and adolescent males. However, the rates of these events in the global setting have not been explored in a systematic manner. The aim of this systematic review and meta-analysis is to investigate the rates of carditis in children and adolescents receiving COVID-19 vaccines. METHODS: PubMed, Embase and several Latin American databases were searched for studies. The number of events, and where available, at-risk populations were extracted. Rate ratios were calculated and expressed as a rate per million doses received. Subgroup analysis based on the dose administered was performed. Subjects ≤ 19 years old who developed pericarditis or myocarditis following COVID-19 vaccination were included. RESULTS: A total of 369 entries were retrieved. After screening, 39 articles were included. Our meta-analysis found that 343 patients developed carditis after the administration of 12,602,625 COVID-19 vaccination doses (pooled rate per million: 37.76; 95% confidence interval [CI] 23.57, 59.19). The rate of carditis was higher amongst male patients (pooled rate ratio: 5.04; 95% CI 1.40, 18.19) and after the second vaccination dose (pooled rate ratio: 5.60; 95% CI 1.97, 15.89). In 301 cases of carditis (281 male; mean age: 15.90 (standard deviation [SD] 1.52) years old) reported amongst the case series/reports, 261 patients were reported to have received treatment. 97.34% of the patients presented with chest pain. The common findings include ST elevation and T wave abnormalities on electrocardiography. Oedema and late gadolinium enhancement in the myocardium were frequently observed in cardiac magnetic resonance imaging (CMR). The mean length of hospital stay was 3.91 days (SD 1.75). In 298 out of 299 patients (99.67%) the carditis resolved with or without treatment. CONCLUSIONS: Carditis is a rare complication after COVID-19 vaccination across the globe, but the vast majority of episodes are self-limiting with rapid resolution of symptoms within days. Central illustration. Balancing the benefits of vaccines on COVID-19-caused carditis and post-vaccination carditis.

Clinical Characteristics, Genetic Findings and Arrhythmic Outcomes of Patients with Catecholaminergic Polymorphic Ventricular Tachycardia from China: A Systematic Review.

INTRODUCTION: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited cardiac ion channelopathy. The present study aims to examine the clinical characteristics, genetic basis, and arrhythmic outcomes of CPVT patients from China to elucidate the difference between CPVT patients in Asia and Western countries. METHODS: PubMed and Embase were systematically searched for case reports or series reporting on CPVT patients from China until 19 February 2022 using the keyword: "Catecholaminergic Polymorphic Ventricular Tachycardia" or "CPVT", with the location limited to: "China" or "Hong Kong" or "Macau" in Embase, with no language or publication-type restriction. Articles that did not state a definite diagnosis of CPVT and articles with duplicate cases found in larger cohorts were excluded. All the included publications in this review were critically appraised based on the Joanna Briggs Institute Critical Appraisal Checklist. Clinical characteristics, genetic findings, and the primary outcome of spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF) were analyzed. RESULTS: A total of 58 unique cases from 15 studies (median presentation age: 8 (5.0-11.8) years old) were included. All patients, except one, presented at or before 19 years of age. There were 56 patients (96.6%) who were initially symptomatic. Premature ventricular complexes (PVCs) were present in 44 out of 51 patients (86.3%) and VT in 52 out of 58 patients (89.7%). Genetic tests were performed on 54 patients (93.1%) with a yield of 87%. RyR2, CASQ2, TERCL, and SCN10A mutations were found in 35 (71.4%), 12 (24.5%), 1 (0.02%) patient, and 1 patient (0.02%), respectively. There were 54 patients who were treated with beta-blockers, 8 received flecainide, 5 received amiodarone, 2 received verapamil and 2 received propafenone. Sympathectomy (n = 10), implantable cardioverter-defibrillator implantation (n = 8) and ablation (n = 1) were performed. On follow-up, 13 patients developed VT/VF. CONCLUSION: This was the first systematic review of CPVT patients from China. Most patients had symptoms on initial presentation, with syncope as the presenting complaint. RyR2 mutation accounts for more than half of the CPVT cases, followed by CASQ2, TERCL and SCN10A mutations.