Publications by authors named "Shushui Wang"

20 Publications

  • Page 1 of 1

Clinical characteristics and survival of children with hypertrophic cardiomyopathy in China: A multicentre retrospective cohort study.

EClinicalMedicine 2022 Jul 27;49:101466. Epub 2022 May 27.

Department of Cardiology, Shanghai Children's Medical Centre, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.

Background: Few data on paediatric hypertrophic cardiomyopathy (HCM) are available in developing countries. A multicentre, retrospective, cohort study was conducted to profile the clinical characteristics and survival of children with HCM in China.

Methods: We collected longitudinal data on children with HCM aged 0-18 years at three participating institutions between January 1, 2010 and December 31, 2019. Patients were identified by searching for the diagnosis using ICD-10 codes from the electronic medical records database. HCM was diagnosed morphologically with echocardiography or cardiovascular magnetic resonance imaging. The exclusion criteria were secondary aetiologies of myocardial hypertrophy. The primary outcomes were all-cause death or heart transplantation. The Kaplan-Meier method was used to estimate the survival rate of different groups.

Findings: A total of 564 children were recruited, with a median age at diagnosis of 1.0 year (interquartile range, IQR: 0.4-8.0 years), followed for a median of 2.6 years (1977 patient-years, IQR:0.5, 5.9 years). The underlying aetiology was sarcomeric (382, 67.7%), inborn errors of metabolism (IEMs) (108, 19.2%), and RASopathies (74, 13.1%). A total of 149 patients (26.4%) died and no patients underwent heart transplantation during follow-up. The survival probability was 71.1% (95% confidence interval [CI], 66.3%-75.3%) at 5 years. Patients with IEMs or those diagnosed during infancy had the poorest outcomes, with an estimated 5-year survival rate of 16.9% (95% CI, 7.7%-29.1%) and 56.0% (95% CI, 48.8%-62.5%), respectively. Heart failure was the leading cause of death in the cohort (90/149, 60.4%), while sudden cardiac death was the leading cause in patients with sarcomeric HCM (32/66, 48.5%).

Interpretation: There is a high proportion of patients with IEM and a low proportion of patients with neuromuscular disease in children with HCM in China. Overall, mortality remains high in China, especially in patients with IEMs and those diagnosed during infancy.

Funding: National Natural Science Fund of China (81770380, 81974029), China Project of Shanghai Municipal Science and Technology Commission (20MC1920400, 21Y31900301).
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http://dx.doi.org/10.1016/j.eclinm.2022.101466DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9157015PMC
July 2022

Association between cumulative blood pressure in early adulthood and right ventricular structure and function in middle age: The CARDIA study.

Clin Cardiol 2022 Jan 3;45(1):83-90. Epub 2022 Jan 3.

Department of Cardiac Pediatrics, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital/Guangdong Academy of Medical Sciences, Guangzhou, China.

Objective: Cumulative blood pressure (BP) exposure is a known risk factor for cardiovascular disease. This study sought to investigate the association between cumulative BP from early adulthood to middle age and right ventricular (RV) structure and function in middle age.

Methods: We included 2844 participants from the CARDIA study (Coronary Artery Risk Development in Young Adults). Cumulative BP over the 30-years follow-up was defined as the sum of the product of mean BP for each pair of consecutive examinations and the time interval between these two consecutive examinations in years. RV structure and function were assessed by echocardiography. The main analyses utilized logistic and linear regression models.

Results: In fully adjusted models, higher cumulative systolic BP was independently associated with lower tricuspid annular plane systolic excursion (TAPSE), right ventricular peak systolic velocity (RVS'), right ventricular early diastolic velocity (RVe'), and higher pulmonary arterial systolic pressure. Higher cumulative diastolic BP was independently associated with smaller RV basal diameter, lower TAPSE, RVS', and RVe'. For categorical analyses of RV dysfunction, cumulative systolic BP was not related to systolic dysfunction. Per 1-SD increase in cumulative systolic BP was associated with a higher risk of diastolic dysfunction, while an increase in cumulative diastolic BP was associated with a higher risk of systolic dysfunction and diastolic dysfunction.

Conclusions: Cumulative exposure to increased BP from early adulthood to middle age was associated with incipient RV systolic and diastolic dysfunction in middle age. Exposure to higher diastolic BP levels from early adulthood to middle age was associated with a smaller RV basal diameter in middle age.
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http://dx.doi.org/10.1002/clc.23763DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8799061PMC
January 2022

Blood cyst of the pulmonary valve causing right ventricular outflow tract obstruction: A case report.

J Clin Ultrasound 2022 Mar 16;50(3):347-350. Epub 2021 Oct 16.

Department of Pediatric Cardiology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of Structural Heart Disease, Guangzhou, China.

Blood cysts are rare cardiac tumors, often involve the atrioventricular valves. Blood cysts are usually small and tend to be clinically silent. Here, we report a case of blood cyst adhered to the junction of the pulmonary valve and the right ventricular outflow, causing severe outflow obstruction. This case was initially misdiagnosed as pulmonary valve stenosis by transthoracic echocardiography (TTE) and even underwent percutaneous balloon pulmonary valvuloplasty (PBPV). As the right ventricle-to-pulmonary artery peak gradient did not decrease after PBPV procedure, the boy underwent TTE again and the diagnosis was corrected as blood cyst. The cystic mass was successfully resected subsequently, and the boy recovered well.
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http://dx.doi.org/10.1002/jcu.23086DOI Listing
March 2022

Transcatheter Closure of Perimembranous and Intracristal Ventricular Septal Defects Using Amplatzer Duct Occluder II in Children.

J Interv Cardiol 2021 11;2021:4091888. Epub 2021 Sep 11.

Department of Cardiac Pediatrics, Guangdong Cardiovascular Institute, Guangdong Academy of Medical Sciences, Guangdong Provincial People's Hospital, Guangzhou 510080, China.

Background: Transcatheter closure of aneurysmal perimembranous ventricular septal defect (pmVSD), pmVSD near the aortic valve, and intracristal VSD (icVSD) with symmetrical or asymmetrical ventricular septal defect occluders still presents significant challenges. We report our experience with transcatheter closure of pmVSD and icVSD using Amplatzer duct occluder II (ADO II) in children.

Method: We retrospectively analyzed all children, who presented to our hospital consecutively between March 2014 and June 2020 for attempted transcatheter closure of pmVSD or icVSD with the ADO II device. Standard safety and last-follow-up outcomes were assessed and compared.

Results: In total, 41 patients underwent transcatheter closure of VSD with the ADO II (28 in pmVSD and 13 in icVSD groups) with a median age of 3.5 years (total range: 0.9 to 12 years) and median weight of 15.0 kg (total range: 10.0 to 43.0 kg). Implantation was successful in 40/41 patients (97.5%, 27/28 in pmVSD group, 13/13 in icVSD group). One patient with mild aortic valve prolapse in pmVSD group developed new-onset moderate aortic regurgitation after a 4/4 mm ADO II was deployed; however, this resolved after the device was retrieved and successfully replaced with a 5 mm zero eccentric VSD occluder. There was no procedure-related mortality. After a median follow-up of six months (total range: 6 to 72 months), complete closure rates were 85.1% and 76.9% among pmVSD and icVSD groups, respectively. In the pmVSD group, one case of new-onset moderate tricuspid regurgitation was observed at six months, and there was one case of severe tricuspid regurgitation that had progressed from mild tricuspid regurgitation at 12 months. No serious complications were noted in the icVSD group.

Conclusion: ADO II provides a safe and reproducible alternative for the closure of perimembranous and intracristal ventricular septal defects with a diameter less than 5 mm in young children.
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http://dx.doi.org/10.1155/2021/4091888DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8452420PMC
November 2021

Initial Clinical Experience with the Biodegradable Absnow Device for Percutaneous Closure of Atrial Septal Defect: A 3-Year Follow-Up.

J Interv Cardiol 2021 30;2021:6369493. Epub 2021 Jul 30.

Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou 510100, China.

Objective: We reported the 3-year follow-up results of initial clinical experience with the Absnow device, a novel biodegradable occluder for percutaneous closure of atrial septal defect (ASD).

Background: The Absnow device is a total biodegradable septal occluder with double-disc poly-L-lactic acid (PLLA) framework and PLLA membranes intergraded into the device to ensure its biodegradability, clinical safety, and efficacy.

Methods: Five pediatric patients were enrolled from May to June 2018 in our institution and were followed up for 3 years. A clinical evaluation and transthoracic echocardiography were performed at 24 hr, 1 month, 3 months, 6 months, 12 months, and yearly after implantation. Primary endpoints were a composite clinical success, comprising of clinical closure success and safety at the 36-month follow-up evaluation. Secondary endpoints included technical success, procedure success, closure success, and safety at each of the follow-up visits.

Results: The median subject age was 3.6 years (range 3.1-6.5 years). The mean ASD diameter was (13.7 ± 2.9) mm. The median device size was 20 mm (range 14 to 24 mm). Technical and procedure success was achieved in 100% (5/5) of the patients. At 2-year follow-up, 3 of the 5 patients developed new-onset residual shunts and 2 of them reached a moderate degree. At 3-year follow-up, the residual shunt size increased over time in all the 3 patients, and 1 of them had right ventricular enlargement. All of the 5 patients were free from serious adverse events during the 3-year follow-up, with no device embolization, thromboembolization, or reintervention to the target defect.

Conclusion: This 3-year follow-up result of initial experience with the biodegradable Absnow device has demonstrated acceptable safety with no procedural complications. Notably, the high rate of residual shunt significantly affected its efficacy. The long-term safety and efficacy of the device should be further evaluated in a large cohort of patients in future studies.
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http://dx.doi.org/10.1155/2021/6369493DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8349294PMC
October 2021

Biodegradable polymeric occluder for closure of atrial septal defect with interventional treatment of cardiovascular disease.

Biomaterials 2021 07 26;274:120851. Epub 2021 Apr 26.

State Key Laboratory of Molecular Engineering of Polymers, Department of Macromolecular Science, Fudan University, Shanghai, 200438, China. Electronic address:

The next-generation closure device for interventional treatment of congenital heart disease is regarded to be biodegradable, yet the corresponding biomaterial technique is still challenging. Herein, we report the first fully biodegradable atrial septal defect (ASD) occluder finally coming into clinical use, which is made of biodegradable poly(l-lactic acid) (PLLA). We characterized the physico-chemical properties of PLLA fibers as well as the raw polymer and the operability of the as-fabricated occluders. Cell behaviors on material were observed, and in vivo fiber degradation and inflammatory responses were examined. ASD models in piglets were created, and 44 PLLA ASD occluders were implanted via catheter successfully. After 36 months, the PLLA ASD occluders almost degraded without any complications. The mechanical properties and thickness between newborn and normal atrial septum showed no significant difference. We further accomplished the first clinical implantation of the PLLA ASD occluder in a four-year boy, and the two-year follow-up up to date preliminarily indicated safety and feasibility of such new-generation fully biodegradable occluder made of synthetic polymers.
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http://dx.doi.org/10.1016/j.biomaterials.2021.120851DOI Listing
July 2021

Safety and Efficacy of Transcatheter Occlusion of Perimembranous Ventricular Septal Defect with Aortic Valve Prolapse: A Six-Year Follow-Up Study.

J Interv Cardiol 2021 18;2021:6634667. Epub 2021 Mar 18.

Graduate School, The Second School of Clinical Medicine, Southern Medical University, Guangzhou, China.

Background: With the rapid development of transcatheter techniques and instruments, transcatheter occlusion for patients with perimembranous ventricular septal defect (pVSD) and aortic valve prolapse (AVP) was constantly being tried, while the efficacy and safety of pVSD with AVP remain controversial.

Objective: The aim of this study was to evaluate long-term efficacy and safety of transcatheter occlusion of pVSD with AVP.

Methods: We retrospectively analyzed 164 children with pVSD and AVP who underwent transcatheter occlusion between January 2013 and November 2014. AVP was divided into 3 degrees according to right coronary leaflet morphology at end-diastole during aortic root angiography. Patient demographic and clinical data were collected.

Results: There were 97 males and 67 females (median age, 40.0 (30.0-62.7) months; average weight, 16.94 ± 9.02 kg). Mild ( = 63), moderate ( = 89), and severe ( = 12) AVP success rates were 93.7%, 89.9%, and 58.3%, respectively. Immediately after procedure, there was no new-onset aortic regurgitation (AR) above trivial degree, residual shunt above mild degree, or complications requiring medication or operation, except for 1 patient who developed transient complete atrioventricular block. During follow-up, 1 mild AVP patient aggravated from mild to moderate AR and 1 moderate AVP patient aggravated from trivial to moderate AR. The new-onset AR in mild, moderate, and severe AVP was 2%, 1.8%, and 20%, respectively. AR disappeared in 17 patients. Residual shunt occurred in 9 patients after procedure, 4 of which disappeared during the follow-up period. No serious complications occurred in any patient during follow-up. Five-year cardiovascular event-free survival rates for mild, moderate, and severe AVP were 89.6%, 94.5%, and 80.0%, respectively.

Conclusion: Transcatheter occlusion of pVSD with mild and moderate AVP has a high success rate and few complications, which is safe and effective in long-term follow-up. Transcatheter occlusion of pVSD with severe AVP has low success rates and high AR incidence. Therefore, transcatheter occlusion of pVSD with AVP is recommended for mild to moderate, but not severe, AVP.
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http://dx.doi.org/10.1155/2021/6634667DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7997740PMC
June 2021

Transcatheter Closure of Perimembranous Ventricular Septal Defect with Aneurysm: Radiologic Characteristic and Interventional Strategy.

J Interv Cardiol 2020 24;2020:6646482. Epub 2020 Dec 24.

Department of Pediatric Cardiology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangdong Cardiovascular Institute, Guangdong, China.

Objectives: We aimed to explore the radiologic characteristics and interventional strategies for perimembranous ventricular septal defect (pmVSD) with aneurysm.

Methods: 257 patients who underwent transcatheter closure of pmVSD with aneurysm were included in our study. We retrospectively reviewed the left ventricular opening of the aneurysm (a), diameter of the midsegment of the aneurysm (b), and diameter of the right ventricular opening of the aneurysm (c). If there were multiple defects within the aneurysm, the largest defect was denoted as and so forth. We developed a novel VSD classification method in which pmVSD with aneurysm was classified into three types (A, B, and C). When  > ≥ , it was classified as type A, when  >  ≥ , it was type B, and when  >  ≥ , it was type C; / described the relationship among defects.

Results: All of the 257 cases of pmVSD with aneurysm were defined using left ventriculography: type A, 60, type B, 58, and type C, 139. Transcatheter closure was attempted in 244 patients and succeeded in 227 cases (success rate was 93.0%; 227/244). Forty symmetric VSD occluders and 13 asymmetric VSD occluders were used for type A aneurysm occlusion; 31 symmetric VSD occluders, 19 asymmetric VSD occluders, and one Amplatzer duct occluder II (ADOII) were used for type B; 59 VSD symmetric occluders, 59 asymmetric VSD occluders, three eccentric VSD occluders, and two ADOII were used for type C. Within 24 hours after procedure, 2.2% patients had postprocedural residual shunt, and 2.2% experienced malignant arrhythmia (including type II second-degree AVB, cAVB, and CLBBB). Two hundred and twelve patients completed follow-up (93%, 212/227). No new severe complications were reported during follow-up, except in one patient who underwent surgery (removal of the device, VSD repair, and tricuspid valvuloplasty) due to severe postprocedural tricuspid regurgitation.

Conclusions: It is safe and effective to apply this method for the classification of pmVSD with aneurysm and its interventional strategy.
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http://dx.doi.org/10.1155/2020/6646482DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7775184PMC
May 2021

Congenital coronary artery fistula in pediatric patients: transcatheter versus surgical closure.

BMC Cardiovasc Disord 2020 11 16;20(1):484. Epub 2020 Nov 16.

Department of Cardiovascular Surgery, Guangdong Provincial Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, No. 106, Zhongshan 2nd Road, Yuexiu District, Guangzhou, 510000, China.

Objectives: Transcatheter closure (TCC) and surgical closure (SC) are the two main approaches for congenital coronary artery fistula (CCAF), but data on the comparisons of the efficacy and safety of these two approaches are limited.

Methods: We retrospectively reviewed pediatric patients with CCAF in Guangdong Cardiovascular Institute between January 2002 and December 2017. Patients who were qualified into our criteria were included into final analysis. The rate of successful closure and complications during hospitalization and at follow-up were compared between SC and TCC groups.

Results: In total, 121 pediatric patients (male, n = 69; female, n = 52) with CCAF were divided to TCC (n = 63) and SC groups (n = 58) according to the indications. The mean age was 5.3 ± 1.4 years. The baseline characteristics of these two groups were similar except for the fistula anatomic feature. After adjusted for the fistula anatomy, compared to SC, TCC was associated with higher risk of major complications (p = 0.013). Proportions of patients requiring blood transfusion and intra-operative blood loss were higher in SC versus TCC groups, as were longer duration of hospital and ICU stay during hospitalization. In contrast, myocardial ischemia (10.2% vs 0.0%, p = 0.028), residual shunts (16.9% vs 3.6%, p = 0.045) and new-onset moderate-to-severe valve regurgitation (11.9% vs 0.0%, p = 0.013) were higher in TCC group versus SC groups during follow-up.

Conclusions: TCC has less invasive and faster recovery. However, SC had a higher successful rate and lower risk of major complications in pediatric patients.
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http://dx.doi.org/10.1186/s12872-020-01769-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7670810PMC
November 2020

Feasibility of Transcatheter Closure of Secundum Atrial Septal Defect in Low Weight Infants Under 2-Year-Old from a 3-year Retrospective Cohort Study.

Am J Cardiol 2020 10 17;132:133-139. Epub 2020 Jun 17.

Department of Cardiac Pediatrics, Guangdong Cardiovascular Institute, Guangdong Academy of Medical Sciences/Guangdong General Hospital, Guangzhou, China. Electronic address:

We aimed to evaluate the feasibility of interventional treatment of atrial septal defect (ASD) in low weight infants under 2-year-old. Seven hundred and ninety-three secundum ASD patients were divided into 2 groups: 665 were above 2-year-old and 128 were under 2-year-old. The basic conditions before the operation, postoperative complications within 24 hours, and adverse outcomes during a three-year follow-up were compared between the 2 groups using multivariate analysis. There were significant differences in age, weight, and the diameter of the ASD between the 2 groups (p <0.001). The immediate success rate of the procedure was 96.7%. There were no significant differences in the success rate of the procedure, the incidence of residual shunt, arrhythmia, procedure-related arrhythmia, and occluder shedding between 2 groups (p >0.05). Similarly, we found no association between age ≤2-year-old and any adverse outcomes postprocedure within 24 hours, including procedure failure (OR = 0.35; 95%CI: 0.04 to 2.93), residual shunt (OR = 1.07; 95%CI: 0.54 to 2.14), arrhythmia (OR = 0.68; 95%CI: 0.32 to 1.43), or procedure-related arrhythmia (OR = 0.34; 95%CI: 0.04 to 2.87). In the follow-up data, we found no association between age ≤2-year-old and arrhythmia (HR = 0.95; 95%CI: 0.50 to 1.80) and procedure-related arrhythmia (HR = 0.96;95%CI:0.25 to 3.64). Kaplan-Meier survival curves indicated no significant difference in the occurrence of arrhythmia between the 2 groups (log-rank test: p = 0.776). In conclusion, percutaneous ASD closure in young and low weight infants has a high success and low complication rate, along with reliable effects.
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http://dx.doi.org/10.1016/j.amjcard.2020.06.011DOI Listing
October 2020

Berberine protects Kawasaki disease-induced human coronary artery endothelial cells dysfunction by inhibiting of oxidative and endoplasmic reticulum stress.

Vascul Pharmacol 2020 04 15;127:106660. Epub 2020 Feb 15.

The Department of Cardiology, Shenzhen Children's Hospital, Shenzhen, China. Electronic address:

Kawasaki disease (KD) is an acute febrile illness characterized by systemic vasculitis especially in coronary arteries. Berberine (BBR) shows several beneficial effects on cardiovascular system. The present study is to investigate whether BBR exerts protective effect against KD-induced damage of human coronary artery endothelial cell (HCAECs) and the underlying mechanisms. HCAECs exposed to medium with 15% serum from KD patients or healthy volunteers for 24 h. Stimulated HCAECs were treated with vehicle (without BBR) and BBR (20 μM) for 24 h, the cell apoptosis, cell cycle, induction of intracellular reactive oxygen species (ROS) and protein expression were examined by flow cytometry and western blot. The KD-induced differentially expressed proteins in HCAECs were determined by quantitative proteomics. BBR inhibited HCAECs from apoptosis and arrested cell cycle at G0/G1 stage. BBR protected HCAECs from injury by inhibiting expression of THBD, vWF and EDN1. Bioinformatics analysis suggested that the oxidative and ER stress were involved in KD-induced damage in HCAECs. ROS production and the protein expression of ATF4, p-EIF2α, p-PERK, XBP1, p-IRE1, HSP90B1, HSPG2, DNAJC3, P4HB and VCP were increased by serum from KD patients and decreased by BBR treatment. BBR exerts its protective effects on KD-induced damage of HCAECs through its inhibitory effects on oxidative and ER stress indicating BBR as a therapeutic candidate for KD.
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http://dx.doi.org/10.1016/j.vph.2020.106660DOI Listing
April 2020

Catheter Ablation of Ventricular Arrhythmias Originating From the Pulmonary Sinus Cusp in Pediatric Patients: A Single-Center Retrospective Study.

Front Pediatr 2019 9;7:280. Epub 2019 Jul 9.

Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.

There are few reports of ventricular arrhythmias (VAs) originating from the pulmonary sinus cusp (PSC) in pediatric patients. Thus, we investigated the ablation of PSC-VAs in pediatric patients. Clinical, echocardiographic, and ablation data were reviewed in 10 consecutive symptomatic children who underwent successful ablation of VAs of PSC origin at our center between March 2014 and June 2018. The 10 patients' weights ranged from 29 to 63.5 kg, and all had structurally normal hearts and VAs with left bundle branch block (LBBB) morphologies and inferior axes. The initial ablation was performed in the right ventricular outflow tract (RVOT) or the aortic sinus cusp, which failed to terminate the VAs in nine patients. The successful ablation site was in the right cusp (RC) in seven patients, the anterior cusp in two patients, and the left cusp (LC) in one patient. The earliest potential recorded at the PSC ablation site preceded the onset of the QRS complex during VAs by 29.4 ± 4.9 ms. VAs with a LBBB morphologies and inferior axes may originate within the PSC of children. Ablation was effective and safe for the eradication of VAs originating from the PSCs in children. Due to the particularity of ablations in pediatric patients, mapping of PSCs should be considered when ablation fails in the RVOT.
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http://dx.doi.org/10.3389/fped.2019.00280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6629776PMC
July 2019

Feasibility, Safety and Long-Term Follow-Up of Transcatheter Closure of Secundum Atrial Septal Defects with Deficient Rims.

Cardiology 2016 4;134(2):118-26. Epub 2016 Mar 4.

Southern Medical University, Guangzhou, PR China.

Objective: The aim of this work was to evaluate the feasibility and safety of transcatheter closure procedures for the treatment of atrial septal defects (ASDs) with insufficient rims.

Methods: A total of 507 secondary ASDs were divided into two groups based on whether they had deficient rims or not (152 vs. 355 cases, respectively). Any complications, including residual shunt, heart arrhythmia, occluder translocation, etc., were followed up for 1-3 years.

Results: There were no differences in gender, weight, exposure time, ECG states, pulmonary pressure, the intervention success rate, occurrence of residual shunt, the operation time and occurrence of residual shunt during follow-up between the two groups (p > 0.05). However, the occurrence of rhythm disorders was significantly different between the two groups; ASDs with deficient rims were at an elevated risk (p < 0.05). Specifically, there was a significantly higher incidence in the occurrence of arrhythmia in the deficient rims group at 24 h postoperation, but no differences in arrhythmia incidence at any of the other follow-up time points (1, 3, 6, 12 and 36 months; p > 0.05).

Conclusions: Patients with deficient rims experience a high success rate of ASD intervention and low rate of complications when the procedures are performed by experienced operators.
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http://dx.doi.org/10.1159/000443265DOI Listing
December 2016

Endoscopic closure of acquired oesophagorespiratory fistulas with cardiac septal defect occluders or vascular plugs.

Respir Med 2015 Aug 5;109(8):1069-78. Epub 2015 May 5.

Dept. of Pediatric Cardiology, Guangdong Cardiovascular Institute and Guangdong General Hospital, Guangdong, 510080, China. Electronic address:

Objectives: To report an endoscopic treatment for inoperable oesophagorespiratory fistulas (ORFs).

Patients And Methods: Six patients with inoperable acquired tracheobronchial-oesophageal fistulas (four males and two females; mean age, 70.2 ± 10.28 years) were included. Cardiac septal defect occluders or vascular plugs were implanted through a flexible bronchoscope to close the ORFs. Monthly follow-ups were done for 16 months.

Results: All fistulas were successfully closed immediately after the procedure. The severe aspirated pneumonia was controlled in 7-10 days. The two mechanically ventilated patients were weaned successfully from the ventilator, and the general condition of patients improved rapidly. However, the fistulas recanalised in four patients because of the cutting effect of the edge of the occluders 1-12 months after the procedure. Other reasons, such as compression of the tracheal intubation balloon and repeated inflammation of the oesophageal diverticulum, may also have contributed to the recanalisation.

Conclusions: Endoscopic closure of acquired ORFs with cardiac septal defect occluders or vascular plugs improve patients' general condition immediately after the procedure, but may result in recanalisation longterm. The occlusion might be considered an abridgement to surgery.
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http://dx.doi.org/10.1016/j.rmed.2015.04.014DOI Listing
August 2015

[Prevalence and risk factors of arrhythmias after transcatheter closure of ventricular septal defect in children].

Zhonghua Xin Xue Guan Bing Za Zhi 2014 Oct;42(10):840-5

Department of Pediatric Cardiovascular, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangzhou 510080, China.

Objective: To evaluate the prevalence and risk factors of arrhythmia after transcatheter closure of ventricular septal defect (VSD) in children.

Methods: A total 1 069 children (583 males, mean age (7.7 ± 3.6) years) underwent transcatheter closure of VSD from January 2002 to December 2010 in our hospital were enrolled and retrospectively analyzed.VSD diameters were (4.0 ± 1.8)mm, 336 cases accompanied membranous aneurysm. Electrocardiogram were performed at 1, 3 days after the procedure.Once arrhythmias recorded, electrocardiogram was performed daily till discharge. All cases were followed up by ECG at 1, 3, 6, 12 months after the procedure in outpatient department and then in a year interval. The risk factors were identified by multivariable logistical analysis.

Results: All VSDs were closed successfully and the diameters of occluder was (7.2 ± 2.1)mm. The median follow-up time was 2.2 (1.0-4.2) years. Mortality was zero during follow up.Incidence of early ( < 1 month) post-procedure arrhythmias was 24.6 % (263 cases), and severe arrhythmias were recorded in 50 cases (4.7%). There were 43 late ( ≥ 1 month) post-procedure arrhythmias (4.0%) including 4 (0.4%) complete atrioventricular block. Multivariable logistic analysis revealed that VSD treated with thin-waist-big-side occluder (OR = 2.426, 95%CI:1.835-3.208, P < 0.001) , male gender (OR = 1.267, 95%CI:1.055-1.523, P = 0.011) were the risk factors while higher body weight (OR = 0.838, 95%CI:0.737-0.951, P = 0.006) was protective factor for early onset arrhythmia. Placement of asymmetrical occluder (OR = 4.777, 95%CI:2.079-10.978, P < 0.001) , longer procedure time (OR = 1.011, 95%CI:1.002-1.020, P = 0.012) , occluder from foreign countries (OR = 2.621, 95%CI:1.143-6.014, P = 0.021) were the risks factors for early onset severe conduct block. Treatment with thin-waist-big-side occluder (OR = 2.654, 95%CI: 1.042-6.760, P = 0.041) was the risk factor while higher body weight (OR = 0.373, 95%CI:0.159-0.875, P = 0.023) was a protective factor for late onset conduct block.

Conclusions: Arrhythmia after transcatheter closure of VSD is common in children, and late onset severe conduct block is rare. The weight of patients should not too light and symmetrical occluder should be chosen if possible in the transcatheter closure VSD procedure to minimize the risk of late onset conduct block.
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October 2014

[Anomalous origin of the left coronary artery from the pulmonary artery in infants: clinical features and the perioperative treatment strategies].

Zhonghua Er Ke Za Zhi 2014 Oct;52(10):777-82

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Objective: To investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).

Method: Data of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed. The patients' cardiac function was evaluated with echocardiography during follow-up.

Result: Most patients presented with symptoms of heart failure, such as tachypnea, diaphoresis, poor feeding, failure to thrive etc. Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients, endocardial hyperplasia in 5 patients, dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients. The left ventricular ejection fraction (LVEF) was (45.5±13.9)% (25%-77%). The left ventricular fractional shortening (LVFS) was (22.0±7.3)% (12%-38%). Twenty one patients underwent cardiovascular CT scan. Left coronary artery originated from left posterior sinus in 9 patients, from right posterior sinus in 1 patient, from lower main pulmonary artery in 5 patients, from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital, only 1 case who underwent angiography was diagnosed correctly. Two patients were misdiagnosed in 8 patients first-presented in our hospital. Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan. The preoperative therapies included using inotropic agents, diuretics and vasodilators according to cardiac function. Two patients underwent left coronary artery orifice ligation. Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system. Patients of NYHA IV with moderate mitral regurgitation (MR) and NYHA III with severe MR underwent mitral annuloplasty. If LVEF was less than 30% after weaning from cardiopulmonary bypass, blood pressure could not be maintained with medication, or lactates increased progressively, extracorporeal membrane oxygenation (ECMO) was demanded. Two patients showed low cardiac output syndrome immediately after surgical procedures, died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries, duration of ventilation was 7-500 hours, 11 of them were supported with ventilator for less than 60 hours. The mean length of hospital stay was (23.4±13.9) d (8-65 d). The follow-up duration ranged from 1-91 months (median 28.5 months). One case was lost to follow up. The patient died from infection 3 months after discharge. The cardiac functions of the remaining 22 patients were improved. The size of left ventricle of 14 patients recovered to normal. LVEF increased to the normal level in 20 cases. No patient underwent redo procedure.

Conclusion: The accurate diagnosis can be made based on history, electrocardiogram, echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants. Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.
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October 2014

[Efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty].

Zhonghua Er Ke Za Zhi 2014 Sep;52(9):703-5

Guangdong Province People's Hospital, Guangdong Academy of Medical Science, Guangzhou 510100, China.

Objective: To evaluate the efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty (RVP-PBAV) for congenital aortic stenosis (AS).

Method: A total of sixteen children with AS accepted the treatment with RRVP-PBAV. The patients were at ages 6 months to 15 years, their median age was 5.4 years. Their body weight was between 8.5 and 59.0 kg, average (22.3 ± 16.5) kg. The gradient pressure across the aortic valve was measured for all the patients and aortic regurgitation was observed. The follow-up time ranged from 1 month to 5.5 years.

Result: All patients underwent RVP-PBAV successfully. The ratios of balloon/valve were 0.86 to 1.12. The gradient pressure varied from preoperative Δp = (96 ± 32) mmHg (1 mmHg = 0.133 kPa) to the immediate postoperative ΔP = (41 ± 26) mmHg, (P < 0.05). One case had postoperative restenosis, and 3 cases were complicated with bicuspid aortic valve deformity.

Conclusion: The treatment with RVP-PBAV for congenital aortic stenosis is safe and reliable. Rapid ventricular pacing is a safe procedure to stabilize the balloon during balloon aortic valvuloplasty and may decrease the incidence of aortic insufficiency.
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September 2014

[Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases].

Zhonghua Er Ke Za Zhi 2014 Jul;52(7):544-7

Department of Pediatrics, Guangdong General Hospital (Guangdong Academy of Medical Sciences), Guangdong Cardiovascular Institute, Guangzhou 510080, China.

Objective: To investigate the mutation and background of SLC22A5 in 6 patients with primary carnitine deficiency (PCD) who only presented as cardiomyopathy.

Method: Genomic DNA were abstracted from the blood of the patients and their parents. Using high-throughput sequencing to determine the mutation site.Using Sanger method to confirm the mutated alleles in PCD patients and detect the corresponding sequences in their patients. Using SIFT and PolyPhen to predict the function of protein for detected missense mutations.

Result: Three different mutations were identified, including 2 nonsense mutations (R254X and R289X), 1 missense mutation (C113Y), R254X was the most frequently seen mutation. Four patients had compound heterozygous mutations and 2 patients had homozygous mutations. Their parents were found to have heterozygous mutations in corresponding alleles.

Conclusion: R254X, R289X and C113Y might be associated with primary carnitine deficiency.
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July 2014

Potential biomarkers predicting risk of pulmonary hypertension in congenital heart disease: the role of homocysteine and hydrogen sulfide.

Chin Med J (Engl) 2014 ;127(5):893-9

Department of Cardiac Pediatrics, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong 510080, China. Email:

Background: Pulmonary hypertension (PH) is a common complication of congenital heart disease (CHD). Although risk stratification is vital for prognosis and therapeutic guidance, the need for understanding the role of novel biomarkers cannot be overlooked. The aim of the present study was to investigate the changes of homocysteine and hydrogen sulfide levels and find potential biomarkers for early detection and treatment.

Methods: Between September 2012 and April 2013, we prospectively collected data on 158 pediatric patients with left to right shunt CHD at our institution. Standard right heart catheterizations were performed in all cases. Seventy-seven cases were associated with PH. The levels of homocysteine and hydrogen sulfide were detected with fluorescence polarization immunoassay and a sensitive silver-sulphur electrode, respectively. Enzyme-linked immunosorbent assay was used to determine the expression of methylenetetrahydrofolate reductase (MTHFR), cystathionine β-synthase (CBS), and cystathionine gamma-lyase (CSE). Radioimmunoassays were used to obtain folic acid and vitamin B12 levels.

Results: The difference in the levels of homocysteine, folic acid, vitamin B12, hydrogen sulfide, as well as the MTHFR and CSE expression between patients with PH and without PH were statistically significant (all P < 0.05). Homocysteine had the best sensitivity and specificity to predict PH (P < 0.001). Subgroup analysis showed that the levels of homocysteine and hydrogen sulfide, and the expression of CSE and MTHFR between patients with dynamic and obstructive PH were significantly different (all P < 0.05). Based on the ROC curve, homocysteine had the best sensitivity and specificity to predict obstructive PH (P = 0.032), while CSE had the most significant sensitivity and specificity to predict the dynamic PH (P = 0.008).

Conclusions: Increased levels of homocysteine and decreased levels of hydrogen sulfide were significantly negatively correlated in PH associated with CHD. The underlying mechanism involved the decreased expression of MTHFR and CSE along with vitamin B12 deficiency. Homocysteine and hydrogen sulfide are potential biomarkers to predict PH.
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February 2015

Acute myocardial infarction and ascending aortic aneurysm in a child with Behçet's disease.

Turk J Pediatr 2008 Jan-Feb;50(1):81-5

Department of Pediatric Cardiology, Guangdong Provincial Cardiovascular Institute, Guangzhou, China.

A 12-year-old boy with a history of recurrent aphthous ulcerations and cutaneous erythema nodosum suddenly experienced chest pain. Together, the symptoms and a positive skin pathergy test established a diagnosis of Behçet's disease. An acute extensive anterior myocardial infarction was based on electrocardiography and a cardiac computed tomography confirmed an ascending aortic aneurysm. Coronary angiography showed mural irregularity and a 50% constriction in the first diagonal artery. Following treatment with urokinase, corticosteroids, colchicine, and aspirin, most symptoms gradually improved. There were no complications noted at the one-year follow-up evaluation. Myocardial infarction is a rare event in children with Behçet's disease; treatment with corticosteroids and colchicine can result in regression of concomitant aneurysm.
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May 2008
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