Publications by authors named "Yongtai Liu"

35 Publications

Using game theory to thwart multistage privacy intrusions when sharing data.

Sci Adv 2021 Dec 10;7(50):eabe9986. Epub 2021 Dec 10.

Department of Electrical Engineering and Computer Science, Vanderbilt University, Nashville, TN 37212, USA.

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http://dx.doi.org/10.1126/sciadv.abe9986DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8664254PMC
December 2021

Mitral valve regurgitation is associated with left atrial fibrosis in patients with atrial fibrillation.

J Electrocardiol 2022 Jan-Feb;70:24-29. Epub 2021 Nov 23.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Background: Low voltage zones (LVZ) are associated with poor outcomes in patients with atrial fibrillation (AF). The APPLE and DR-FLASH scores predict LVZ in patients undergoing catheter ablation. This study aimed to assess the relationship of mitral valve regurgitation (MR) and LVZ after adjusting for APPLE or DR-FLASH scores.

Methods: This was a retrospective study on patients with AF who underwent their first catheter ablation. All patients underwent a transthoracic echocardiographic examination before ablation. The APPLE and DR-FLASH scores were calculated at baseline. LVZ determined by high-density mapping was defined as bipolar voltage amplitude <0.5 mV. LVZ presence was defined as LVZ covering >5% of the left atrial surface area.

Results: Altogether, 152 patients (mean age 62.0 ± 10.8 years, 65.8% men, and 36.2% with persistent AF) were included. Of the 152 patients, 47 (30.9%) had LVZ. The patients with LVZ had more moderate-to-severe MR (17.0% vs. 3.8%, P = 0.014) and higher APPLE scores (1.7 ± 1.1 vs. 1.2 ± 1.1, P = 0.009) and DR-FLASH scores (3.0 ± 1.5 vs. 2.4 ± 1.4, P = 0.010). Using multivariate logistic regression analysis, we found moderate-to-severe MR was related to LVZ presence after adjusting for the APPLE (OR 4.040, P = 0.034) or DR-FLASH (OR 4.487, P = 0.020) scores. Furthermore, moderate-to-severe MR had an incremental predictive value for LVZ presence in addition to the APPLE (P = 0.03) or DR-FLASH (P = 0.02) scores.

Conclusion: In patients with AF, MR severity was related to LVZ after adjusting the APPLE score or DR-FLASH score.
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http://dx.doi.org/10.1016/j.jelectrocard.2021.11.031DOI Listing
November 2021

Response to ibutilide and the long-term outcome after catheter ablation for non-paroxysmal atrial fibrillation.

Cardiovasc J Afr 2021 Oct 15;32:1-6. Epub 2021 Oct 15.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Email:

Purpose: This study aimed to assess the relationship between the cardiac rhythm response to ibutilide infusion after pulmonary vein isolation and the recurrence of long-term atrial arrhythmias.

Methods: One hundred and thirty-eight patients with non-paroxysmal atrial fibrillation who had had their first catheter ablation were retrospectively included. All patients whose atrial fibrillation did not terminate after pulmonary vein isolation were administered intravenous ibutilide (1.0 mg). Those with termination of atrial fibrillation after ibutilide administration were defined as responders ( = 86); those without termination of atrial fibrillation, as non-responders ( = 52). The primary endpoint was any documented recurrence of atrial arrhythmia lasting more than 30 seconds after the initial catheter ablation.

Results: Conversion of atrial fibrillation to sinus rhythm, directly or via atrial flutter, with ibutilide administration was achieved in 62.3% of patients. A longer duration of atrial fibrillation was associated with failed termination of atrial fibrillation (odds ratio 1.009, 95% confidence interval 1.002-1.017, = 0.011). During a median follow-up period of 610 days (interquartile range 475-1 106) post ablation, non-responders ( = 24, 46.2%) had a higher recurrence rate of atrial arrhythmia than the responders ( = 26, 30.2%; log-rank, = 0.011) after the initial catheter ablation. Multivariate Cox regression analysis revealed that non-responders (hazard ratio 1.994, 95% confidence interval 1.117-3.561, = 0.020) was significantly correlated with recurrence of atrial arrhythmias.

Conclusions: In patients whose atrial fibrillation persisted after pulmonary vein isolation, the response to ibutilide administration could predict the recurrence of atrial arrhythmias after catheter ablation, which may be useful for risk stratification for recurrence of atrial fibrillation and individualised management of atrial fibrillation.
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http://dx.doi.org/10.5830/CVJA-2021-044DOI Listing
October 2021

Quality of life in ambulatory pulmonary arterial hypertension in connective tissue diseases and its relationship with risk stratification.

Pulm Circ 2021 Jul-Sep;11(3):20458940211029899. Epub 2021 Jul 11.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

The Pulmonary Arterial Hypertension Symptoms and Impact Questionnaire (PAH-SYMPACT) is a PAH-specific patient-reported outcome scale assessing patients' quality of life from four aspects: cardiopulmonary symptoms, cardiovascular symptoms, physical impacts and cognitive/emotional impacts. This study aimed to validate the Chinese version of PAH-SYMPACT and explore its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). In addition, 75 patients with CTD-PAH confirmed by right heart catheterization were invited to complete questionnaires including PAH-SYMPACT, the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The demographic, clinical, laboratory and treatment data were collected. The endpoint was treatment goal achievement status in 6-12 months after completing the questionnaires, defined as an integrated outcome. Participants' mean age was 36.4 ± 11.9 years and the mean pulmonary arterial pressure was 38.9 ± 13.67 mmHg. The reliability of the PAH-SYMPACT domains ranged from 0.83 to 0.88. Results of factor analysis basically conformed the original PAH-SYMPACT. The treatment goal achievement (TGA) status in 6-12 months was significantly associated with physical impacts scores (odds ratio: 0.180, 95% confidence interval: 0.036-0.908, P=0.038). The Chinese version of PAH-SYMPACT is a reliable measurement to evaluate quality of life in CTD-PAH patients and is also a potential predictor of patient's condition change in routine clinical practice.
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http://dx.doi.org/10.1177/20458940211029899DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8278470PMC
July 2021

Predicting the Risk of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: A Chinese Systemic Lupus Erythematosus Treatment and Research Group Cohort Study.

Arthritis Rheumatol 2021 10 12;73(10):1847-1855. Epub 2021 Aug 12.

Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Objective: Pulmonary arterial hypertension (PAH) is a life-threatening complication of systemic lupus erythematosus (SLE). However, there is no algorithm to identify those at high risk. This study was undertaken to develop a prediction model for PAH in patients with lupus that provides individualized risk estimates.

Methods: A multicenter, longitudinal cohort study was undertaken from January 2003 to January 2020. The study collected data on 3,624 consecutively evaluated patients diagnosed as having SLE. The diagnosis of PAH was confirmed by right-sided heart catheterization. Cox proportional hazards regression and least absolute shrinkage and selection operator were used to fit the model. Model discrimination, calibration, and decision curve analysis were performed for validation.

Results: Ninety-two lupus patients (2.54%) developed PAH during a median follow-up of 4.84 years (interquartile range 2.42-8.84). The final prediction model included 5 clinical variables (acute/subacute cutaneous lupus, arthritis, renal disorder, thrombocytopenia, and interstitial lung disease) and 3 autoantibodies (anti-RNP, anti-Ro/SSA and anti-La/SSB). A 10-year PAH probability-predictive nomogram was established. The model was internally validated by Harrell's concordance index (0.78), the Brier score (0.03), and a satisfactory calibration curve. According to the net benefit and predicted probability thresholds, we recommend annual screening in high-risk (>4.62%) lupus patients.

Conclusion: We developed a risk stratification model using routine clinical assessments. This new tool may effectively predict the future risk of PAH in patients with SLE.
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http://dx.doi.org/10.1002/art.41740DOI Listing
October 2021

Interventricular systolic asynchrony predicts prognosis in patients with systemic sclerosis-associated pulmonary arterial hypertension.

Rheumatology (Oxford) 2021 May 31. Epub 2021 May 31.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, China.

Objective: Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc) with high mortality. Interventricular systolic asynchrony (IVSA) is observed in PAH patients, but the effect of IVSA and its association with long-term mortality and clinical events in SSc-associated PAH are unclear. This study aimed to investigate the impact of IVSA on the prognosis of SSc-associated PAH.

Methods: Between March 2010 and July 2018, a total of 60 consecutive patients with SSc-associated PAH were enrolled. The end point was a composite of all-cause mortality and clinical worsening. Asynchrony was assessed by colour-coded tissue Doppler imaging (TDI) echocardiography. The myocardial sustained systole curves (Sm) of the basal portion of the right ventricular (RV) free wall and left ventricular (LV) lateral wall were obtained. IVSA was defined as the time difference from the onset of the QRS complex to the end of Sm between LV and RV.

Results: Patients with greater IVSA time differences presented with advanced pulmonary vascular resistance (PVR). The IVSA time difference was an independent predictive factor (HR = 1.018, 95% CI 1.005-1.031, p = 0.005) for the composite end point and was significantly associated with PVR (r = 0.399, R2=0.092, p = 0.002). Kaplan-Meier survival curves showed that patients with greater IVSA had worse prognoses (log-rank p = 0.001).

Conclusion: In conclusion, IVSA analyzed by colour-coded TDI echocardiography provided added value as a noninvasive, easy-to-use approach for assessing the prognosis of patients with SSc-associated PAH. A significant IVSA time difference identifies the subgroup of patients at high risk of a poor prognosis.
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http://dx.doi.org/10.1093/rheumatology/keab465DOI Listing
May 2021

Validation of the REVEAL Prognostic Models in Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension.

Front Med (Lausanne) 2021 4;8:618486. Epub 2021 Mar 4.

Department of Epidemiology and Bio-Statistics, Institute of Basic Medical Sciences, China Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

No previous studies have investigated the predictive performance of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) prognostic equation and simplified risk score calculator in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). We aimed to validate these prediction tools in an external cohort of patients with SLE-PAH. In this study, the validation cohort consisted of patients with SLE-PAH registered in a prospective, multicenter, nationwide database between November 2006 and May2016. The follow-up of patients was censored at 1 year. Discrimination, calibration, model fit, and risk stratification of the REVEAL prognostic equation and simplified risk score calculator were validated. As a result, a total of 306 patients with SLE-PAH were included. The 1-year overall survival rate was 91.5%. The C-index of the prognostic equation was 0.736, demonstrating reasonably good discrimination, and it was greater than that for the simplified risk score calculator (0.710). The overall calibration slope was 0.83, and the Brier score was 0.079. The risk of renal insufficiency and World Health Organization Functional Class III (WHO FC III) were underestimated, and the risk assigned to a heart rate >92 bpm in the REVEAL prognostic models was not observed in our validation cohort. Both model discrimination and calibration were poor in the very high-risk group. In conclusion, the REVEAL models exhibit good discriminatory ability when predicting 1-year overall survival in patients with SLE-PAH. Findings from both models should be interpreted with caution in cases of renal insufficiency, WHO FC III, and heart rate >92 bpm.
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http://dx.doi.org/10.3389/fmed.2021.618486DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7969505PMC
March 2021

Contribution of Free-Text Comments to the Burden of Documentation: Assessment and Analysis of Vital Sign Comments in Flowsheets.

J Med Internet Res 2021 03 4;23(3):e22806. Epub 2021 Mar 4.

School of Nursing, Vanderbilt University, Nashville, TN, United States.

Background: Documentation burden is a common problem with modern electronic health record (EHR) systems. To reduce this burden, various recording methods (eg, voice recorders or motion sensors) have been proposed. However, these solutions are in an early prototype phase and are unlikely to transition into practice in the near future. A more pragmatic alternative is to directly modify the implementation of the existing functionalities of an EHR system.

Objective: This study aims to assess the nature of free-text comments entered into EHR flowsheets that supplement quantitative vital sign values and examine opportunities to simplify functionality and reduce documentation burden.

Methods: We evaluated 209,055 vital sign comments in flowsheets that were generated in the Epic EHR system at the Vanderbilt University Medical Center in 2018. We applied topic modeling, as well as the natural language processing Clinical Language Annotation, Modeling, and Processing software system, to extract generally discussed topics and detailed medical terms (expressed as probability distribution) to investigate the stories communicated in these comments.

Results: Our analysis showed that 63.33% (6053/9557) of the users who entered vital signs made at least one free-text comment in vital sign flowsheet entries. The user roles that were most likely to compose comments were registered nurse, technician, and licensed nurse. The most frequently identified topics were the notification of a result to health care providers (0.347), the context of a measurement (0.307), and an inability to obtain a vital sign (0.224). There were 4187 unique medical terms that were extracted from 46,029 (0.220) comments, including many symptom-related terms such as "pain," "upset," "dizziness," "coughing," "anxiety," "distress," and "fever" and drug-related terms such as "tylenol," "anesthesia," "cannula," "oxygen," "motrin," "rituxan," and "labetalol."

Conclusions: Considering that flowsheet comments are generally not displayed or automatically pulled into any clinical notes, our findings suggest that the flowsheet comment functionality can be simplified (eg, via structured response fields instead of a text input dialog) to reduce health care provider effort. Moreover, rich and clinically important medical terms such as medications and symptoms should be explicitly recorded in clinical notes for better visibility.
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http://dx.doi.org/10.2196/22806DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7974764PMC
March 2021

Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors.

Ther Adv Chronic Dis 2021 22;12:2040622320987051. Epub 2021 Jan 22.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing 100730, China.

Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes.

Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated Kaplan-Meier curves.

Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% 20%,  = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029-1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 10/L [area under the curve (AUC) = 0.692,  = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% 21.4%,  = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% 4.3%,  = 0.035) than the control group. Outcomes ( = 0.131) and survival ( = 0.1972) were not significantly different between the groups.

Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.
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http://dx.doi.org/10.1177/2040622320987051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7841702PMC
January 2021

Prognostic Value of Circulating sST2 for the Prediction of Mortality in Patients With Cardiac Light-Chain Amyloidosis.

Front Cardiovasc Med 2020 20;7:597472. Epub 2021 Jan 20.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Systemic light-chain (AL) amyloidosis is a multisystemic disorder leading to multiple organ dysfunction and mortality that is often caused by cardiac involvement. Soluble suppression of tumorigenicity 2 (sST2) is a novel biomarker identified for risk stratification of heart disease. The aim of this study was to investigate the value of circulating sST2 levels in prognosis and mortality risk assessments for the AL amyloidosis population. A total of 56 patients diagnosed with AL amyloidosis were enrolled in Peking Union Medical College Hospital (PUMCH) from January 2015 to May 2018. The relationships between the clinical parameters and overall survival (OS) and risk factors for disease progression were assessed. Additionally, receiver operating characteristic (ROC) curves, Kaplan-Meier analysis, and Cox hazard models were performed to explore the predictive value of sST2 in mortality rates. We found that the median OS of all patients was 7.3 [interquartile range (IQR) 4.4, 15.9] months. The median baseline sST2 level was 12.2 (IQR 5.1, 31.1) ng/ml, and the sST2 high group had more severe patients with a higher Mayo stage. In the ROC analysis, the area under the curve (AUC) was 0.728 [95% confidence interval (CI) 0.603-0.853] for sST2 to predict the outcomes of AL amyloidosis patients, and the optimal cutoff value was 12.34 ng/ml (sensitivity 80.2%, specificity 61.1%). Moreover, in multivariate Cox proportional hazards regression analysis, sST2 acted as an independent predictor of poor functional outcome in patients with AL amyloidosis. In AL amyloidosis patients, sST2 was a strong and independent prognostic biomarker for all-cause mortality, providing complementary prognostic information of a novel scoring system for risk stratification.
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http://dx.doi.org/10.3389/fcvm.2020.597472DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7855859PMC
January 2021

Enabling realistic health data re-identification risk assessment through adversarial modeling.

J Am Med Inform Assoc 2021 03;28(4):744-752

Department of Biomedical Informatics, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Objective: Re-identification risk methods for biomedical data often assume a worst case, in which attackers know all identifiable features (eg, age and race) about a subject. Yet, worst-case adversarial modeling can overestimate risk and induce heavy editing of shared data. The objective of this study is to introduce a framework for assessing the risk considering the attacker's resources and capabilities.

Materials And Methods: We integrate 3 established risk measures (ie, prosecutor, journalist, and marketer risks) and compute re-identification probabilities for data subjects. This probability is dependent on an attacker's capabilities (eg, ability to obtain external identified resources) and the subject's decision on whether to reveal their participation in a dataset. We illustrate the framework through case studies using data from over 1 000 000 patients from Vanderbilt University Medical Center and show how re-identification risk changes when attackers are pragmatic and use 2 known resources for attack: (1) voter registration lists and (2) social media posts.

Results: Our framework illustrates that the risk is substantially smaller in the pragmatic scenarios than in the worst case. Our experiments yield a median worst-case risk of 0.987 (where 0 is least risky and 1 is most risky); however, the median reduction in risk was 90.1% in the voter registration scenario and 100% in the social media posts scenario. Notably, these observations hold true for a wide range of adversarial capabilities.

Conclusions: This research illustrates that re-identification risk is situationally dependent and that appropriate adversarial modeling may permit biomedical data sharing on a wider scale than is currently the case.
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http://dx.doi.org/10.1093/jamia/ocaa327DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8711654PMC
March 2021

Predictive Value of Pulmonary Arterial Compliance in Systemic Lupus Erythematosus Patients With Pulmonary Arterial Hypertension.

Hypertension 2020 10 10;76(4):1161-1168. Epub 2020 Aug 10.

Department of Rheumatology (J.Z., M.L., X.Z.), Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.

Pulmonary arterial hypertension is a serious complication of systemic lupus erythematosus. It is characterized by increased right ventricular afterload which mainly comprises pulmonary arterial compliance (PAC) and pulmonary vascular resistance. The role of PAC in predicting the outcome of systemic lupus erythematosus-associated pulmonary arterial hypertension has not been investigated yet. Between February 2012 to December 2016, 120 consecutive patients diagnosed with systemic lupus erythematosus-associated pulmonary arterial hypertension based on right heart catheterization were enrolled, prospectively. Baseline clinical characteristics and hemodynamic assessment were analyzed. Baseline right ventricular afterload was stratified according to the PAC and pulmonary vascular resistance. The end point was a composite of all-cause mortality and clinical worsening. Among them, end points occurred in 49 (41%) patients after 15 months (interquartile range, 8.5-24.0). Patients with a PAC <1.39 mL/mm Hg had a 3.09-fold higher risk (95% CI, 1.54-6.20, =0.001) of the end point events than the patients with a PAC ≥1.39 mL/mm Hg. Multivariable Cox regression analysis showed that stratified right ventricular afterload was the only independent predictor for the end point (hazard ratio, 2.009 [95% CI, 1.390-2.904], <0.001). A 3-group prediction risk was created. The patients with the highest right ventricular afterload (PAC <1.39 mL/mm Hg and pulmonary vascular resistance ≥10.3Wood Unit) had the highest risk (χ, 6.10; <0.014) of experiencing the end point. Our results suggest that PAC is a good predictor of mortality and clinical worsening in systemic lupus erythematosus-associated pulmonary arterial hypertension. PAC, in addition to pulmonary vascular resistance, may be an attractive tool for screening high-risk populations in these patients.
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http://dx.doi.org/10.1161/HYPERTENSIONAHA.120.15682DOI Listing
October 2020

Biomedical Research Cohort Membership Disclosure on Social Media.

AMIA Annu Symp Proc 2019 4;2019:607-616. Epub 2020 Mar 4.

Vanderbilt University, Nashville, TN.

To accelerate medical knowledge discovery, an increasing number of research programs are gathering and sharing data on a large number of participants. Due to the privacy concerns and legal restrictions on data sharing, these programs apply various strategies to mitigate privacy risk. However, the activities of participants and research program sponsors, particularly on social media, might reveal an individual's membership in a study, making it easier to recognize participants' records and uncover the information they have yet to disclose. This behavior can jeopardize the privacy of the participants themselves, the reputation of the projects, sponsors, and the research enterprise. To investigate the dangers of self-disclosure behavior, we gathered and analyzed 4,020 tweets, and uncovered over 100 tweets disclosing the individuals' memberships in over 15 programs. Our investigation showed that self-disclosure on social media can reveal participants' membership in research cohorts, and such activity might lead to the leakage of a person's identity, genomic, and other sensitive health information.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7153128PMC
June 2020

Predictive value of non-invasive right ventricle to pulmonary circulation coupling in systemic lupus erythematosus patients with pulmonary arterial hypertension.

Eur Heart J Cardiovasc Imaging 2021 01;22(1):111-118

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, No 1. Shuaifuyuan, Dongcheng District, Beijing 100730, China.

Aims: Pulmonary arterial hypertension (PAH) is a serious and devastating complication of systemic lupus erythematosus (SLE), especially when the right ventricle (RV) fails. Whether the ratio between tricuspid annular plane systolic excursion (TAPSE) and pulmonary artery systolic pressure (PASP) measured by echocardiography as a simple surrogate of RV to pulmonary circulation (PC) coupling predicts the outcome of SLE-associated PAH has not been investigated.

Methods And Results: Between February 2010 and August 2015, 112 consecutive patients with a diagnosis of SLE-associated PAH confirmed by right heart catheterization were enrolled prospectively. The endpoint was a composite of all-cause mortality and clinical worsening. Baseline clinical characteristics and echocardiographic assessment were analysed. Among all the patients, 47 (42%) patients experienced the endpoint (mean follow-up period 18.1 ± 12.0 months), including 20 patients who died during a median follow-up period of 48.5 months. Multivariable Cox regression analysis showed that TAPSE/PASP ratio [hazard ratio (HR) 0.004, P = 0.017] and 6-min walk distance (6MWD) (HR 0.997, P = 0.036) were the independent predictors for the endpoint. A three-group prediction risk was created based on combined assessment of the TAPSE/PASP ratio and 6MWD relative to their cut-off values. The patients with the worse RV-PC coupling (TAPSE/PASP <0.184 mm/mmHg) and the lower 6MWD (<395 m) had the highest risk (HR 4.62, confidence interval 2.27-9.41, P < 0.001) of experiencing the endpoint.

Conclusion: The TAPSE/PASP ratio, combined with 6MWD, provides clinical and prognostic insights into patients with SLE-associated PAH. A low TAPSE/PASP and low 6MWD identifies the subgroup of patients with high risk of poor prognosis.
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http://dx.doi.org/10.1093/ehjci/jez311DOI Listing
January 2021

Effect of different ranges of systolic blood pressure on left ventricular structure and diastolic function in a Chinese population: a cross-sectional population-based Shunyi study.

BMJ Open 2019 09 3;9(8):e028398. Epub 2019 Sep 3.

Cardiology, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China

Objectives: To evaluate the effect of different ranges of systolic blood pressure (SBP) on left ventricular (LV) geometry and diastolic function in Chinese population.

Design: Cross-sectional study.

Setting: Peking Union Medical College Hospital in Beijing, China.

Participants: All inhabitants aged 35 years or older, living in five villages of Shunyi were invited. Exclusion criteria included individuals who declined participation, presence of moderate to severe valvular heart disease, persistent atrial fibrillation and suboptimal echocardiograms.

Interventions: The baseline data of 1051 participants were analysed. The relationship between SBP and LV geometric and diastolic function assessed by echocardiography was analysed after adjusting for conventional cardiac risk factors.

Results: The adjusted value of SBP was independently associated with LV hypertrophy (LVH) and LV diastolic dysfunction (LVDDF) (all p<0.01). Setting individuals with SBP <120 mm Hg as the reference group (group 1), those with SBP between 120 mm Hg and 140 mm Hg (group 2) had higher risk odds of LVH and those with SBP ≥140 mm Hg (group 3) had higher risk odds of LVH and LVDDF (all p<0.01). With the increase of SBP, LV mass index (LVMI) and E/e' stepwise increased and e' stepwise decreased significantly from group 1 to 3 (all p<0.05). In the whole population, SBP was independently correlated with LVMI, LVEDD, Left Atrial Volume Index, e', and E/e' (all p<0.01).

Conclusions: SBP was independently related to LVH and LVDDF, SBP between 120 and 140 mm Hg was independently related to worse LV remodelling and diastolic function, these findings indicated the potential benefit of intensive SBP control.
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http://dx.doi.org/10.1136/bmjopen-2018-028398DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6731946PMC
September 2019

Effect of metformin on all-cause and cardiovascular mortality in patients with coronary artery diseases: a systematic review and an updated meta-analysis.

Cardiovasc Diabetol 2019 07 30;18(1):96. Epub 2019 Jul 30.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.

Background: Metformin is the most widely prescribed drug to lower glucose and has a definitive effect on the cardiovascular system. The goal of this systematic review and meta-analysis is to assess the effects of metformin on mortality and cardiac function among patients with coronary artery disease (CAD).

Methods: Relevant studies reported before October 2018 was retrieved from databases including PubMed, EMBASE, Cochrane Library and Web of Science. Hazard ratio (HR) was calculated to evaluate the all-cause mortality, cardiovascular mortality and incidence of cardiovascular events (CV events), to figure out the level of left ventricular ejection fraction (LVEF), creatine kinase MB (CK-MB), type B natriuretic peptide (BNP) and to compare the average level of low density lipoprotein (LDL).

Results: In this meta-analysis were included 40 studies comprising 1,066,408 patients. The cardiovascular mortality, all-cause mortality and incidence of CV events were lowered to adjusted HR (aHR) = 0.81, aHR = 0.67 and aHR = 0. 83 respectively after the patients with CAD were given metformin. Subgroup analysis showed that metformin reduced all-cause mortality in myocardial infarction (MI) (aHR = 0.79) and heart failure (HF) patients (aHR = 0.84), the incidence of CV events in HF (aHR = 0.83) and type II diabetes mellitus (T2DM) patients (aHR = 0.83), but had no significant effect on MI (aHR = 0.87) and non-T2DM patients (aHR = 0.92). Metformin is superior to sulphonylurea (aHR = 0.81) in effects on lowering the incidence of CV events and in effects on patients who don't use medication. The CK-MB level in the metformin group was lower than that in the control group standard mean difference (SMD) = - 0.11). There was no significant evidence that metformin altered LVEF (MD = 2.91), BNP (MD = - 0.02) and LDL (MD = - 0.08).

Conclusion: Metformin reduces cardiovascular mortality, all-cause mortality and CV events in CAD patients. For MI patients and CAD patients without T2DM, metformin has no significant effect of reducing the incidence of CV events. Metformin has a better effect of reducing the incidence of CV events than sulfonylureas.
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http://dx.doi.org/10.1186/s12933-019-0900-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6668189PMC
July 2019

Detecting the Presence of an Individual in Phenotypic Summary Data.

AMIA Annu Symp Proc 2018 5;2018:760-769. Epub 2018 Dec 5.

Vanderbilt University, Nashville, Tennessee, USA.

As the quantity and detail of association studies between clinical phenotypes and genotypes grows, there is a push to make summary statistics widely available. Genome wide summary statistics have been shown to be vulnerable to the inference of a targeted individual's presence. In this paper, we show that presence attacks are feasible with phenome wide summary statistics as well. We use data from three healthcare organizations and an online resource that publishes summary statistics. We introduce a novel attack that achieves over 80% recall and precision within a population of 16,346, where 8,173 individuals are targets. However, the feasibility of the attack is dependent on the attacker's knowledge about 1) the targeted individual and 2) the reference dataset. Within a population of over 2 million, where 8,173 individuals are targets, our attack achieves 31% recall and 17% precision. As a result, it is plausible that sharing of phenomic summary statistics may be accomplished with an acceptable level of privacy risk.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6371366PMC
December 2019

Right Ventricular Function is Associated With Quality of Life in Patients With Systemic Lupus Erythematosus Associated Pulmonary Arterial Hypertension.

Heart Lung Circ 2019 Nov 27;28(11):1655-1663. Epub 2018 Sep 27.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Background: Right ventricular (RV) function has been identified as an important determinant of outcome in patients with pulmonary hypertension. We aimed to investigate the relationship between echocardiographic-derived RV function and health-related quality of life (HRQOL) in patients with systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-APAH), and to identify the best echocardiographic parameter for evaluating RV function in these patients.

Methods: Sixty (60) consecutive patients with SLE-APAH (all female, mean age 33.6±8.2years) were recruited from May 2013 to November 2014. Echocardiograph, right heart catheterisation, SLE disease activity index (SLEDAI), and functional status and SF-36 generic questionnaire were assessed.

Results: Echocardiograph-derived RV systolic function was significantly correlated with haemodynamics (p<0.05), with tricuspid annular plane systolic excursion (TAPSE) showing the strongest correlation with pulmonary vascular resistance (R=0.278, p<0.001) and cardiac index (R=0.215, p<0.001). Patients with a TAPSE<17mm had a shorter 6-minute-walk-distance (6MWD), lower mixed venous oxygen saturation, and higher plasma N-terminal pro-brain natriuretic peptide (p<0.05). Patients with TAPSE <17mm had lower physical component summary (PCS) and mental component summary (MCS) scores than those with TAPSE ≥17mm (35.5±13.2 vs. 55.0±15.5; 46.3±15.3 vs. 64.8±18.8, respectively, all p<0.05). On multiple regression analysis, a TAPSE <17mm was independently related to lower PCS (β -15.797, 95% confidence interval [CI] -24.746 to -6.848, p=0.001) and lower MCS (β -12.887, 95% CI -24.018 to -1.755, p=0.024).

Conclusions: TAPSE is a useful index for RV function assessment, and is associated with HRQOL in patients with SLE-APAH.
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http://dx.doi.org/10.1016/j.hlc.2018.09.002DOI Listing
November 2019

Molecular analysis of inherited cardiomyopathy using next generation semiconductor sequencing technologies.

J Transl Med 2018 08 30;16(1):241. Epub 2018 Aug 30.

McKusick-Zhang Center for Genetic Medicine, State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Science & Peking Union Medical College, 5 Dong Dan San Tiao, Beijing, 100005, China.

Background: Cardiomyopathies are the most common clinical and genetic heterogeneity cardiac diseases, and genetic contribution in particular plays a major role in patients with primary cardiomyopathies. The aim of this study is to investigate cases of inherited cardiomyopathy (IC) for potential disease-causing mutations in 64 genes reported to be associated with IC.

Methods: A total of 110 independent cases or families diagnosed with various primary cardiomyopathies, including hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction, and undefined cardiomyopathy, were collected after informed consent. A custom designed panel, including 64 genes, was screened using next generation sequencing on the Ion Torrent PGM platform. The best candidate disease-causing variants were verified by Sanger sequencing.

Results: A total of 78 variants in 73 patients were identified. After excluding the variants predicted to be benign and VUS, 26 pathogenic or likely pathogenic variants were verified in 26 probands (23.6%), including a homozygous variant in the SLC25A4 gene. Of these variants, 15 have been reported in the Human Gene Mutation Database or ClinVar database, while 11 are novel. The majority of variants were observed in the MYH7 (8/26) and MYBPC3 (6/26) gene. Titin (TTN) truncating mutations account for 13% in our dilated cardiomyopathy cases (3/23).

Conclusions: This study provides an overview of the genetic aberrations in this cohort of Chinese IC patients and demonstrates the power of next generation sequencing in IC. Genetic results can provide precise clinical diagnosis and guidance regarding medical care for some individuals.
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http://dx.doi.org/10.1186/s12967-018-1605-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117967PMC
August 2018

Is it possible to apply the treat-to-target strategy in primary Sjögren's syndrome-associated pulmonary arterial hypertension?

Clin Rheumatol 2018 Nov 24;37(11):2989-2998. Epub 2018 Jul 24.

Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College and Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, Beijing, 100730, China.

The treat-to-target (T2T) strategy improved long-term survival of patients with pulmonary arterial hypertension (PAH). Little was known about applying the T2T strategy in primary Sjogren's syndrome-associated PAH (pSS-PAH). We investigated how to identify patients who are more likely to reach the treatment goals in a cohort of pSS-PAH. In this way, we explored the possibility of implementing T2T in pSS-PAH. Data were retrospectively collected from patients visiting our center between 2007 and 2017. PAH was confirmed by right heart catheterization (RHC). Patients were treated following the T2T strategy. PAH treatment goals were defined by the 5th World Symposium on Pulmonary Hypertension. The primary end point was reaching the PAH treatment goals. Of the 62 patients enrolled, 98.4% were female, with a mean age of 38.9 ± 9.1 years at the diagnostic RHC. The median disease duration of pSS was 46 months (0-365), while the median disease duration of PAH was 12 months (0-149). Following the T2T strategy, 32 (50%) patients achieved the treatment goals. The 1-, 3-, and 5- year cumulative rates of reaching the goals were 40.6, 67.4, and 73.9%, respectively. Predictive factors included using immunosuppressants (HR 4.715, 95% CI 1.101-20.192, p = 0.037) and right ventricular anterior-posterior diameter (RV-APD) > 30 mm at baseline (HR 0.426, 95% CI 0.188-0.968, p = 0.042). The results provide strong evidence that patients who received immunosuppressants are more likely to reach the treatment goals. In contrast, impaired RV structure correlates to worse treatment response. The T2T strategy is effective in pSS-PAH.
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http://dx.doi.org/10.1007/s10067-018-4184-1DOI Listing
November 2018

Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome.

Int J Rheum Dis 2018 May 6;21(5):1068-1075. Epub 2018 Apr 6.

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.

Aim: To describe baseline characteristics of patients with primary Sjögren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH) and explore risk factors for PAH in pSS.

Methods: This case-control study included consecutive patients hospitalized with pSS-PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American-European Consensus Group classification criteria for pSS-PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were analyzed by univariate binary logistic regression to identify possible risk factors for PAH.

Results: Twenty-nine patients with RHC-confirmed pSS-PAH were included (mean age at onset, 38.4 ± 8.3 years; mean pSS duration, 54.6 months). PAH was the initial manifestation of pSS in 12 patients (41.4%), and shortness of breath was the most common symptom (29/29, 100%). Mean pulmonary arterial pressure was 51.8 ± 10.0 mmHg, mean cardiac index was 2.3 ± 0.8 L/min/m , and mean pulmonary vascular resistance was 13.0 ± 6.0 Wood units in this group. Treatments included immunosuppressive agents (93.1%) and PAH-targeted therapies (86.2%). We identified four independent risk factors for PAH in pSS: Raynaud's phenomenon (odds ratio [OR] = 9.660, P = 0.000), rheumatoid factor ≥ 200 U/mL (OR = 6.691, P = 0.001), hepatic injury (OR = 3.284, P = 0.008) and pericardial effusion (OR = 3.279, P = 0.016).

Conclusions: PAH can be the first manifestation of pSS. The pSS patients with Raynaud's phenomenon, high-titer rheumatoid factor, hepatic injury or pericardial effusion should be screened for PAH.
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http://dx.doi.org/10.1111/1756-185X.13290DOI Listing
May 2018

Red blood cell distribution width as a related factor of pulmonary arterial hypertension in patients with systemic sclerosis.

Clin Rheumatol 2018 Apr 21;37(4):979-985. Epub 2017 Nov 21.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, No.1 Shuaifuyuan, Beijing, 100730, China.

The aim of this study was to investigate the utility of red blood cell distribution width (RDW) as a simple and readily available marker of occurrence of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). One hundred and forty-five consecutive patients with SSc were recruited to the single-center cross-sectional study. Demographic characteristics, hematological parameters, Modified Rodnan Skin Score, and World Health Organization functional classification were determined. Diagnosis of PAH was based on screening by echocardiography and was confirmed by right heart catheterization. Interstitial lung disease (ILD) was diagnosed based on chest high-resolution computed tomography findings. There were no significant differences in gender, age, or disease duration between limited and diffused SSc groups. PAH was detected in 28 of lcSSc (33.3%) and 14 of dcSSc (23.0%) subjects. Patients with higher RDW values were more likely to be men with high anti-u1RNP titers and PAH. A significant correlation was found between RDW and high-sensitivity C-reactive protein (p = 0.375, p < 0.01) and the diffusing capacity of the lungs for carbon monoxide (ρ = - 0.396, p < 0.01). The SSc-PAH group had significantly higher RDW values compared to the SSc group without pulmonary disease (15.7 ± 2.2 and 13.7 ± 1.0, p < 0.001). The mean RDW in the SSc-PAH-ILD group was significantly higher than that in the SSc-ILD group (16.3 ± 2.2% and 14.0 ± 1.5%, p < 0.001). Besides the recognized risk factors, high RDW was an independent predictor of PAH in patients with SSc (OR = 3.314 [95%CI 1.038-10.580], p < 0.05). RDW may be a related factor for identifying the pulmonary arterial hypertension in SSc patients.
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http://dx.doi.org/10.1007/s10067-017-3918-9DOI Listing
April 2018

It's all in the timing: calibrating temporal penalties for biomedical data sharing.

J Am Med Inform Assoc 2018 01;25(1):25-31

Department of Biomedical Informatics, Vanderbilt University Medical Center, Nashville, TN, USA.

Objective: Biomedical science is driven by datasets that are being accumulated at an unprecedented rate, with ever-growing volume and richness. There are various initiatives to make these datasets more widely available to recipients who sign Data Use Certificate agreements, whereby penalties are levied for violations. A particularly popular penalty is the temporary revocation, often for several months, of the recipient's data usage rights. This policy is based on the assumption that the value of biomedical research data depreciates significantly over time; however, no studies have been performed to substantiate this belief. This study investigates whether this assumption holds true and the data science policy implications.

Methods: This study tests the hypothesis that the value of data for scientific investigators, in terms of the impact of the publications based on the data, decreases over time. The hypothesis is tested formally through a mixed linear effects model using approximately 1200 publications between 2007 and 2013 that used datasets from the Database of Genotypes and Phenotypes, a data-sharing initiative of the National Institutes of Health.

Results: The analysis shows that the impact factors for publications based on Database of Genotypes and Phenotypes datasets depreciate in a statistically significant manner. However, we further discover that the depreciation rate is slow, only ∼10% per year, on average.

Conclusion: The enduring value of data for subsequent studies implies that revoking usage for short periods of time may not sufficiently deter those who would violate Data Use Certificate agreements and that alternative penalty mechanisms may need to be invoked.
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http://dx.doi.org/10.1093/jamia/ocx101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6080807PMC
January 2018

Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China.

Int J Cardiol 2017 Jun 18;236:432-437. Epub 2017 Jan 18.

Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College & Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, Beijing 100730, China. Electronic address:

Objective: Pulmonary arterial hypertension (PAH) is a major cause of death in connective tissue disease patients. This study investigated the clinical characteristics and survival of CTD-PAH in Chinese patients.

Methods: This cohort study enrolled 190 consecutive PAH patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or primary Sjögren's syndrome (pSS) who visited our referral center between May 2006 and December 2014. Baseline demographics, clinical features, laboratory results, and hemodynamic assessments were analyzed. Cox proportional hazards regression analysis was used to identify independent factors associated with increased risk of mortality.

Results: The PAH patients were more likely to have SLE (58.4%) as the underlying CTD than SSc (26.3%) or pSS (15.3%). Mean age was 37.8±10.4years, and patients with SLE were youngest at the time of PAH diagnosis. The most prevalent autoantibody was anti-U1RNP antibody (55.8%). The three groups did not differ significantly regarding World Health Organization functional class or hemodynamic results. The overall 1-, 3-, and 5-year survival rates were 87.1%, 79.1%, and 62.9%, respectively. The 3-year survival rate of 81.3% for those with SLE-PAH was significantly better than that for patients with SSc-PAH (63.6%, P<0.05). Independent predictors of mortality were 6-minute walk distance (6MWD) ≤380m (HR 3.222, 95% CI 1.485-6.987, P=0.003) and underlying CTD (HR 1.684; 95CI% 1.082-2.622, P=0.021).

Conclusion: Independent predictors of mortality for CTD-PAH were 6MWD <380m and SSc as the underlying CTD. Increased awareness of pSS-PAH is needed because of its worse prognosis compared to SLE-PAH.
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http://dx.doi.org/10.1016/j.ijcard.2017.01.097DOI Listing
June 2017

Preoperative immunosuppressive therapy reduces paravalvular leakage after aortic valve surgery in patients with aortic regurgitation attributable to Behçet's disease.

Clin Exp Rheumatol 2016 Sep-Oct;34(6 Suppl 102):S26-S33. Epub 2015 May 25.

Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.

Objectives: Severe aortic regurgitation (AR) attributable to Behçet's disease (BD) is a rare but challenging problem in light of the surgical treatment notoriously complicated with paravalvular leakage (PVL) and high mortality. This study aims to test whether immunosuppressive therapy is effective in reducing the complication of the operation and improve the prognosis.

Methods: In a retrospective cohort of 644 hospitalised BD patients who were diagnosed and followed up between January 1998 and September 2014, 18 patients (2.8%) with severe AR due to BD were identified and their medical records were analysed.

Results: Among the 18 patients, 15 patients underwent the valve surgery and PVL developed in 7 (47%) at a median interval of 3.5 months. In the median follow-up of 32.5 months from first operations, patients with PVL had a significantly higher incidence of repeat surgery and death (71% vs. 0%, p=0.007). They less likely received preoperative immunotherapy (14% vs. 100%, p=0.001), had a lower cumulative dosage of cyclophosphamide before first operations (2.1±5.7g vs. 13.0±6.4g, p=0.004) and a higher preoperative erythrocyte sedimentation rate (44.4±20.7mm/first hour vs. 25.0±12.1mm/first hour, p=0.04) compared with those without developing PVL. Multivariate analysis showed preoperative immuno-suppressive therapy (hazard ratio 18.58; 95% confidence interval, 2.134-161.81; p=0.008) was an independent factor associated with the absence of PVL. The 5-year PVL-free survival rates were significantly higher in patients receiving preoperative immunotherapy (p=0.0004).

Conclusions: In patients with severe AR due to BD, preoperative immunosuppressive therapy, especially cyclophosphamide in conjunction with glucocorticoid, could reduce PVL after the corrective surgery and improve the outcomes.
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February 2017

A Case of Novel Lamin A/C Mutation Manifesting as Atypical Progeroid Syndrome and Cardiomyopathy.

Can J Cardiol 2016 09 19;32(9):1166.e29-31. Epub 2015 Nov 19.

Department of Cardiology, Peking Union Medical College Hospital, Beijing, China. Electronic address:

Mutations in the gene LMNA cause a wide spectrum of diseases that selectively affect different tissues and organ systems. The clinical features of these disorders can overlap but be generally categorized into 2 groups: cardiomyopathy and neuromuscular disorders; premature aging and lipodystrophy disorders. It is significant for a single patient who harbours the 2 sets of diseases simultaneously. We present a female patient with a unique phenotype including rare atypical progeroid syndrome and dilated cardiomyopathy. Genetic mutation detection in the gene LMNA revealed a novel heterozygous de novo mutation p.Leu59Val located in the first exon of gene LMNA c.175C>CG.
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http://dx.doi.org/10.1016/j.cjca.2015.11.011DOI Listing
September 2016

Serum IgE levels are associated with coronary artery disease severity.

Atherosclerosis 2016 08 12;251:355-360. Epub 2016 May 12.

Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China. Electronic address:

Background And Aims: Immunoglobulin E (IgE), a key element of allergic reactions, was considered to be involved in the development of atherosclerosis and the pathogenesis of myocardial ischemia. This study was designed to test whether total serum IgE levels were associated with the atherosclerosis severity of coronary artery disease (CAD).

Methods: Total serum IgE concentrations were measured in 708 consecutive patients who were presented to our center for coronary angiography. Atherosclerosis severity of CAD was assessed by the number of diseased vessels showing ≥50% diameter stenosis and quantified by Gensini score.

Results: Patients with CAD (N = 562) had higher serum IgE levels than those without CAD (N = 146) [55.90 (19.10-156.00) vs. 26.90 (11.80-62.10) KU/L, p = 0.003]. Furthermore, the serum IgE levels were significantly increased in patients with multivessel disease (MVD) compared to those with single-vessel disease [61.80 (23.20-159.00) vs. 32.45(14.15-94.38) KU/L, p = 0.003]. After adjustment for traditional cardiovascular risk factors, a high serum IgE level was an independent predictor for an increased risk of MVD (OR 1.003; 95% CI 1.001-1.004; p = 0.041). Receiver-operating characteristic curve analysis demonstrated that serum IgE levels improved the predictive capability of traditional risk factors for MVD (area under the curve with and without IgE: 0.734 and 0.713, respectively, p < 0.001). Meanwhile, there was a significant linear relationship between Gensini score and the serum IgE level quartiles (p for linear trend <0.001).

Conclusions: Increased total serum IgE levels are associated with MVD and contribute to discriminating CAD severity independently of traditional cardiovascular risk factors.
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http://dx.doi.org/10.1016/j.atherosclerosis.2016.05.020DOI Listing
August 2016

Baseline Characteristics and Risk Factors of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus Patients.

Medicine (Baltimore) 2016 Mar;95(10):e2761

From the Department of Rheumatology (CH, ML, QW, JZ, YZ, XZ), Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education and Department of Cardiology (YL, XG, JL, ZT), Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.

Peking Union Medical College Hospital (PUMCH) has started a single-center right heart catheterization (RHC)-based pulmonary arterial hypertension (PAH) study in systemic lupus erythematosus (SLE) since 2006. The baseline characteristics of these patients were described and the risk factor for PAH in lupus was identified.The demographic, clinical, laboratory, and treatment characteristics of SLE patients with PAH when they were registered were collected as the baseline data. A case-control study was conducted by taking the admitted SLE-non-PAH patients adjusted for age and gender in a 4:1 ratio during the same period as the controls. The associated variables were examined by binary multivariate logistic regression analysis to identify possible risk factors. A total of 111 RHC-confirmed SLE-PAH patients were enrolled, with the onset age of 34.6 ± 8.6 years old and the average SLE duration of 5 years. RHC revealed mPAP as 46.4 ± 11.4 mm Hg, CI as 2.7 ± 0.8 L/min × m, and PVR as 10.5 ± 4.8 WU. 46% of patients were WHO Fc I-II. All patients were treated with immunosuppressive agents and 65% patients had PAH-targeted therapy. The case-control study had confirmed 2 independent risk factors previously published: pericardial effusion (OR = 21.290, P < 0.001) and anti-RNP antibody (OR = 12.399, P < 0.001). Meanwhile, 6 independent variables were discovered: baseline SLE duration (OR = 1.118, P = 0.007), interstitial lung disease (OR = 17.027, P < 0.001=, without acute rash (OR = 3.258, P = 0.019), anti-SSA antibody (OR = 4.836, P = 0.004), SLEDAI≤9 (OR = 26.426, P < 0.001), ESR≤20 mm/h (OR = 12.068, P < 0.001), and uric acid > 357 μmol/L (OR = 9.666, P < 0.001) to be associated with PAH in SLE patients.The PUMCH study has shown that SLE patients complicated with PAH are usually earlier diagnosed and have less disease severity than patients without PAH. The immunosuppressive therapy rate and the PAH target therapy rate were high, which is consistent with reports from Western countries. This study has confirmed that pericardial effusion and positive anti-RNP antibody are risk factors for SLE-associated PAH. Long SLE disease duration, the presence of interstitial lung disease, without acute skin rash, positive anti-SSA antibody, low SLEDAI and ESR, and high uric acid levels are also associated with PAH in SLE patients.
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http://dx.doi.org/10.1097/MD.0000000000002761DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4998855PMC
March 2016

Predictors of health-related quality of life in patients with systemic lupus erythematosus associated pulmonary arterial hypertension.

Clin Exp Rheumatol 2016 Mar-Apr;34(2):291-5. Epub 2016 Mar 3.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Objectives: Understanding health-related quality of life (HRQoL) is important in the management of patients with systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-APAH), however, little is known about HRQoL and its determinants in these patients.

Methods: A total of 60 female SLE-APAH patients with mean age of 33.5 years were prospectively recruited from May 2013 to November 2014. Right heart catheter, SF-36 generic questionnaire, disease activity and functional status were assessed in all patients.

Results: The median duration of SLE was 5 years. Thirty-five participants were with low disease activity (SLEDAI: 0-4). Patients with SLE-APAH reported significant impairment in HRQoL. The mean physical component summary (PCS) and mental component summary (MCS) scores were 46.4 and 56.9, respectively. Among haemodynamic measurements, higher pulmonary vascular resistance and lower cardiac output (CO) were associated with worse HRQoL. Lower body mass index (BMI), lower mean blood pressure and higher disease activity were also associated with poor HRQoL. Multivariate analysis revealed that lower SLEDAI and higher mean blood pressure were predictors for better PCS. However, higher CO (CO≥4L/min) was the only parameter independently associated with both better PCS and MCS.

Conclusions: Self-reported HRQoL was impaired in patients with SLE-APAH. Higher CO was the most important predictor for better HRQoL in these patients.
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July 2016

[The clinical characteristics and prognosis of patients with peripartum cardiomyopathy].

Zhonghua Nei Ke Za Zhi 2016 Feb;55(2):127-30

Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

Objective: To explore the clinical characteristics and risk factors in patients with peripartum cardiomyopathy (PPCM).

Methods: A total of 35 patients admitted in Peking Union Medical College Hospital and diagnosed with PPCM between January 1995 and December 2014 was included and analyzed in this study. The subjects were divided into two groups: the early recovery and delayed recovery. Early recovery was defined as normalization of left ventricle ejection fraction (LVEF) ≥50% before 6 months post-diagnosis. Delayed recovery was defined if the length of time required for recovery of LVEF was longer than 6 months or death was reported during follow-up. Risk factors for delayed recovery were assessed.

Results: The incidence of PPCM was 1 per 1 067 deliveries between the study periods. The age of the 35 patients was (28.9 ± 5.6) years old. Among them, 20 (57.1%) patients were not in the first pregnancy, 13 (37.1%) had delivered before, and 5 (14.3%) patients had twin pregnancies. The LVEF at diagnosis was (34.1 ± 8.0) %. 62.9% (22 cases) of the subjects were in the early recovery, while 37.1% (13 cases) of the subjects were in the delayed recovery group, 2 of whom suffered death. Multivariate logistic regression indicated that LVEF(OR=1.339, 95%CI 1.063-1.688, P=0.013)and left ventricle end-dilated diameter(OR 0.763, 95%CI 0.607-0.960, P=0.021)were independent risk factors for delayed recovery.

Conclusions: PPCM is a rare but life-threatening complication of pregnancy. LVEF and left ventricle end-dilated diameter at diagnosis were two independent factors associated with the prognosis of PPCM.
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http://dx.doi.org/10.3760/cma.j.issn.0578-1426.2016.02.012DOI Listing
February 2016
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