1.Application of bicuspid pulmonary valve sewn by 0.1 mm expanded polytetrafluoroethylene in right ventricle outflow tract reconstruction
Jianrui MA ; Tong TAN ; Miao TIAN ; Jiazichao TU ; Wen XIE ; Hailong QIU ; Shuai ZHANG ; Jian ZHUANG ; Jimei CHEN ; Jianzheng CEN ; Shusheng WEN ; Haiyun YUAN ; Xiaobing LIU
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2025;32(08):1127-1132
Objective To introduce a modified technique of right ventricular outflow tract (RVOT) reconstruction using a handmade bicuspid pulmonary valve crafted from expanded polytetrafluoroethylene (ePTFE) and to summarize the early single-center experience. Methods Patients with complex congenital heart diseases (CHD) who underwent RVOT reconstruction with a handmade ePTFE bicuspid pulmonary valve due to pulmonary regurgitation at Guangdong Provincial People’s Hospital from April 2021 to February 2022 were selected. Postoperative artificial valve function and right heart function indicators were evaluated. Results A total of 17 patients were included, comprising 10 males and 7 females, with a mean age of (18.18±12.14) years and a mean body weight of (40.94±19.45) kg. Sixteen patients underwent reconstruction with a handmade valved conduit, with conduit sizes ranging from 18 to 24 mm. No patients required mechanical circulatory support, and no in-hospital deaths occurred. During a mean follow-up period of 12.89 months, only one patient developed valve dysfunction, and no related complications or adverse events were observed. The degree of pulmonary regurgitation was significantly improved post-RVOT reconstruction and during follow-up compared to preoperative levels (P<0.001). Postoperative right atrial diameter, right ventricular diameter, and tricuspid regurgitation area were all significantly reduced compared to preoperative values (P<0.05). Conclusion The use of a 0.1 mm ePTFE handmade bicuspid pulmonary valve for RVOT reconstruction in complex CHD is a feasible, effective, and safe technique.
2.Exercise-induced Mitohormesis in Counteracting Age-related Sarcopenia
Zi-Yi ZHANG ; Mei MA ; Hai BO ; Tao LIU ; Yong ZHANG
Progress in Biochemistry and Biophysics 2025;52(6):1349-1361
Sarcopenia, an age-related degenerative skeletal muscle disorder characterized by progressive loss of muscle mass, diminished strength, and impaired physical function, poses substantial challenges to global healthy aging initiatives. The pathogenesis of this condition is fundamentally rooted in mitochondrial dysfunction, manifested through defective energy metabolism, disrupted redox equilibrium, imbalanced dynamics, and compromised organelle quality control. This comprehensive review elucidates the central role of exercise-induced mitochondrial hormesis as a critical adaptive mechanism counteracting sarcopenia. Mitohormesis represents an evolutionarily conserved stress response wherein sublethal mitochondrial perturbations, particularly transient low-dose reactive oxygen species (ROS) generated during muscle contraction, activate cytoprotective signaling cascades rather than inflicting macromolecular damage. The mechanistic foundation of this process involves ROS functioning as essential signaling molecules that activate the Keap1 nuclear factor erythroid 2 related factor 2 (Nrf2) antioxidant response element pathway. This activation drives transcriptional upregulation of phase II detoxifying enzymes including superoxide dismutase (SOD) and glutathione peroxidase (GPx), thereby enhancing cellular redox buffering capacity. Crucially, Nrf2 engages in bidirectional molecular crosstalk with peroxisome proliferator activated receptor gamma coactivator 1 alpha (PGC-1α), the principal regulator orchestrating mitochondrial biogenesis through coordinated induction of nuclear respiratory factors 1 and 2 (NRF1/2) along with mitochondrial transcription factor A (Tfam), collectively facilitating mitochondrial DNA replication and respiratory complex assembly. Concurrently, exercise-induced alterations in cellular energy status, specifically diminished ATP to AMP ratios, potently activate AMP activated protein kinase (AMPK). This energy-sensing kinase phosphorylates PGC-1α while concomitantly stimulating NAD dependent deacetylase sirtuin 1 (SIRT1) activity, which further potentiates PGC-1α function through post-translational deacetylation. The integrated AMPK/PGC-1α/SIRT1 axis coordinates mitochondrial biogenesis, optimizes network architecture through regulation of fusion proteins mitofusin 1 (Mfn1), mitofusin 2 (Mfn2) and optic atrophy protein 1 (OPA1), and enhances clearance of damaged organelles via selective activation of mitophagy receptors BCL2 interacting protein 3 (Bnip1) and FUN14 domain containing 1 (FNDC1). Exercise further stimulates the mitochondrial unfolded protein response (UPRmt), increasing molecular chaperones such as heat shock protein 60 (HSP60) and HSP10 to preserve proteostasis. Within the mitochondrial matrix, SIRT3 fine-tunes metabolic flux through deacetylation of electron transport chain components, improving phosphorylation efficiency while attenuating pathological ROS emission. Distinct exercise modalities differentially engage these pathways. Aerobic endurance training primarily activates AMPK/PGC-1α signaling and UPRmt to expand mitochondrial volume and oxidative capacity. Resistance training exploits mechanical tension to acutely stimulate mechanistic target of rapamycin complex 1 (mTORC1) mediated protein synthesis while modulating dynamin related protein 1 (Drp1) phosphorylation dynamics to support mitochondrial network reorganization. High intensity interval training generates potent metabolic oscillations that rapidly amplify AMPK/PGC-1α and Nrf2 activation, demonstrating particular efficacy in insulin-resistant phenotypes. Strategically designed concurrent training regimens synergistically integrate these adaptations. Mitochondrial-nuclear communication through tricarboxylic acid cycle metabolites and mitochondrially derived peptides such as mitochondrial open reading frame of 12s rRNA-c (MOTS-c) coordinates systemic metabolic reprogramming, with exercise-responsive myokines including fibroblast growth factor 21 (FGF-21) mediating inter-tissue signaling to reduce inflammation and enhance insulin sensitivity. This integrated framework provides the scientific foundation for precision exercise interventions targeting mitochondrial pathophysiology in sarcopenia, incorporating biomarker monitoring and exploring pharmacological potentiators including nicotinamide riboside and MOTS-c mimetics. Future investigations should delineate temporal dynamics of mitohormesis signaling and epigenetic regulation to optimize therapeutic approaches for age-related muscle decline.
3.Single-center experience in the treatment of severe aortic stenosis with XcorTM transcatheter aortic valve replacement system: 1-year follow-up results.
Shengwen WANG ; Haozhong LIU ; Haijiang GUO ; Tong TAN ; Hanxiang XIE ; Xiang LIU ; Hailong QIU ; Jimei CHEN ; Huiming GUO ; Jian LIU
Journal of Zhejiang University. Medical sciences 2025;54(2):141-148
OBJECTIVES:
To analyze the early clinical outcomes of the XcorTM transcatheter aortic valve replacement (TAVR) system in treating severe aortic stenosis. This study has been registered at Chinese Clinical Trial Registry (ChiCTR2200065593).
METHODS:
This single-arm, prospective clinical trial enrolled patients with severe aortic stenosis treated with the XcorTM TAVR system at the Section of Heart Valve & Coronary Artery Surgery, Guangdong Provincial People's Hospital. Perioperative and follow-up parameters were compared to evaluate differences in hemodynamic outcomes. All-cause mortality, aortic regurgitation, paravalvular leakage, cerebrovascular events, and reoperation were analyzed.
RESULTS:
Thirty-two patients with severe aortic stenosis were included (20 males, 12 females), with (70.9±4.3) years old and a Society of Thoracic Surgeons (STS) score of 6.45% (6.07%, 7.28%). Notably, 87.5% of patients had New York Heart Association (NYHA) class≥Ⅲ. All patients underwent successful XcorTM bioprosthesis implantation, achieving an immediate technical success rate of 100.0% and device success rate of 96.9%. Mean aortic valve gradient decreased from (55.21±23.17) mmHg (1 mmHg=0.133 kPa) to (8.45±5.30) mmHg, peak aortic jet velocity decreased from (4.66±0.85) m/s to (1.99±0.48) m/s, aortic valve area increased from (0.66±0.21) cm² to (2.09±0.67) cm² (all P<0.01). Intraoperative ventricular fibrillation occurred in one patient, while one case exhibited moderate prosthetic valve regurgitation and paravalvular leakage post-procedure. At 12-month follow-up, sustained improvements were observed in cardiac function, left ventricular ejection fraction, hemodynamic parameters, and SF-12 quality-of-life scores (all P<0.01). All-cause mortality was 12.5% (4/32), with 13.8% (4/29) developing moderate paravalvular leakage.
CONCLUSIONS
The XcorTM TAVR system demonstrated favorable early outcomes in severe aortic stenosis patients, significantly improving symptoms and hemodynamics while exhibiting excellent performance in preventing malignant arrhythmias and coronary obstruction.
Humans
;
Male
;
Female
;
Aortic Valve Stenosis/surgery*
;
Transcatheter Aortic Valve Replacement/methods*
;
Aged
;
Follow-Up Studies
;
Prospective Studies
;
Treatment Outcome
;
Aged, 80 and over
;
Heart Valve Prosthesis
;
Middle Aged
4.Screening and characterization of camelid-derived nanobodies against hemoglobin.
Ning ZHONG ; Wenhui LEI ; Zuying LIU ; Xiaoxiao XIE ; Lingjing ZHANG ; Tengchuan JIN ; Minjie CAO ; Yulei CHEN
Chinese Journal of Biotechnology 2025;41(4):1515-1534
Hemoglobin, the principal protein in red blood cells, is crucial for oxygen transport in the bloodstream. The quantification of hemoglobin concentration is indispensable in medical diagnostics and health management, which encompass the diagnosis of anemia and the screening of various blood disorders. Immunological methods, based on antigen-antibody interactions, are distinguished by their high sensitivity and accuracy. Consequently, it is necessary to develop hemoglobin-specific antibodies characterized by high specificity and affinity to enhance detection accuracy. In this study, we immunized a Bactrian camel (Camelus bactrianus) with human hemoglobin and subsequently constructed a nanobody library. Utilizing a solid-phase screening method, we selected nanobodies and evaluated the binding activity of the screened nanobodies to hemoglobin. Initially, human hemoglobin was used to immunize a Bactrian camel. Following four immunization sessions, blood was withdrawn from the jugular vein, and a nanobody library with a capacity of 2.85×108 colony forming units (CFU) was generated. Subsequently, ten hemoglobin-specific nanobody sequences were identified through three rounds of adsorption-elution-enrichment assays, and these nanobodies were subjected to eukaryotic expression. Finally, enzyme-linked immunosorbent assay and biolayer interferometry were employed to evaluate the stability, binding activity, and specificity of these nanobodies. The results demonstrated that the nanobodies maintained robust binding activity within the temperature range of 20-40 ℃ and exhibited the highest binding activity at pH 7.0. Furthermore, the nanobodies were capable of tolerating a 10% methanol solution. Notably, among the nanobodies tested, VHH-12 displayed the highest binding activity to hemoglobin, with a half maximal effective concentration (EC50) of 10.63 nmol/L and a equilibrium dissociation constant (KD) of 2.94×10-7 mol/L. VHH-12 exhibited no cross-reactivity with a panel of eight proteins, such as ovalbumin and bovine serum albumin, while demonstrating partial cross-reactivity with hemoglobin derived from porcine, goat, rabbit, and bovine sources. In this study, a hemoglobin-specific high-affinity nanobody was successfully isolated, demonstrating potential applications in disease diagnosis and health monitoring.
Animals
;
Camelus/immunology*
;
Single-Domain Antibodies/immunology*
;
Hemoglobins/immunology*
;
Humans
;
Peptide Library
5.Diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery
Hujun CUI ; Jimei CHEN ; Shushui WANG ; Jianzheng CEN ; Gang XU ; Shusheng WEN ; Xiaobing LIU ; Jian ZHUANG
Chinese Journal of Surgery 2024;62(3):242-247
Objective:To analyze the diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery (AAOCA).Methods:This is a retrospective case series study. From January 2016 to July 2023, 24 cases of high-risk AAOCA underwent surgical treatment in Department of Cardiac Surgery, Guangdong Provincial People's Hospital. There were 18 males and 6 females, operatively aged ( M (IQR)) 13 (26) years (range: 0.3 to 57.0 years). They were confirmed by cardiac ultrasound and cardiac CT, all of which had anomalous coronary running between the aorta and the pulmonary artery. There were 15 cases of the right coronary artery from the left aortic sinus of Valsalva, 6 cases of left coronary artery from the right aortic sinus of Valsalva, 3 cases of the sigle coronary artery. Only 3 patients had no obvious related symptoms (2 cases were complicated with a positive exercise stress test and 1 case with other intracardiac malformations), 21 cases had a history of chest tightness, chest pain, or syncope after exercise. Three patients suffered syncope after exercise and underwent cardiopulmonary resuscitation (2 cases were treated with an extracorporeal membrane oxygenerator (ECMO)). The gap from the first symptom to the diagnosis was 4.0 (11.5) months (range: 0.2 to 84.0 months). The detection rate of coronary artery abnormalities suggested by the first cardiac ultrasound was only 37.5% (9/24). Seven patients were complicated with other cardiac diseases (4 cases with congenital heart defects, 2 cases with coronary atherosclerotic heart disease, 1 case with mitral valve disease). Results:All 24 patients underwent surgical treatment (23 cases underwent abnormal coronary artery unroofing, 1 case underwent coronary artery bypass grafting), and 5 patients underwent other intracardiac malformation correction at the same time. There were no death or surgery related complications in the hospital for 30 days after the operation. A patient with preoperative extracorporeal cardiopulmonary resuscitation was continuously assisted by ECMO after emergency AAOCA correction and had complications such as limb ischemia necrosis and renal dysfunction after the operation. During the follow-up of 2.2 (3.3) years (range: 1 month to 7.2 years), one patient who previously underwent percutaneous transluminal coronary angioplasty with a stent implant experienced significant postoperative symptomatic relief, and the other discharged patients had no related symptoms.Conclusions:The accurate rate of initial diagnosis for high-risk AAOCA is still low, but the risk of cardiovascular accidents is high. For sports-related chest pain and other symptoms, more attention should be paid to the detection of AAOCA, especially for adolescents. Exercise stress testing can be helpful in evaluating the cardiovascular risk of asymptomatic AAOCA. Instant surgical treatment can achieve satisfactory curative effects.
6.Diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery
Hujun CUI ; Jimei CHEN ; Shushui WANG ; Jianzheng CEN ; Gang XU ; Shusheng WEN ; Xiaobing LIU ; Jian ZHUANG
Chinese Journal of Surgery 2024;62(3):242-247
Objective:To analyze the diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery (AAOCA).Methods:This is a retrospective case series study. From January 2016 to July 2023, 24 cases of high-risk AAOCA underwent surgical treatment in Department of Cardiac Surgery, Guangdong Provincial People's Hospital. There were 18 males and 6 females, operatively aged ( M (IQR)) 13 (26) years (range: 0.3 to 57.0 years). They were confirmed by cardiac ultrasound and cardiac CT, all of which had anomalous coronary running between the aorta and the pulmonary artery. There were 15 cases of the right coronary artery from the left aortic sinus of Valsalva, 6 cases of left coronary artery from the right aortic sinus of Valsalva, 3 cases of the sigle coronary artery. Only 3 patients had no obvious related symptoms (2 cases were complicated with a positive exercise stress test and 1 case with other intracardiac malformations), 21 cases had a history of chest tightness, chest pain, or syncope after exercise. Three patients suffered syncope after exercise and underwent cardiopulmonary resuscitation (2 cases were treated with an extracorporeal membrane oxygenerator (ECMO)). The gap from the first symptom to the diagnosis was 4.0 (11.5) months (range: 0.2 to 84.0 months). The detection rate of coronary artery abnormalities suggested by the first cardiac ultrasound was only 37.5% (9/24). Seven patients were complicated with other cardiac diseases (4 cases with congenital heart defects, 2 cases with coronary atherosclerotic heart disease, 1 case with mitral valve disease). Results:All 24 patients underwent surgical treatment (23 cases underwent abnormal coronary artery unroofing, 1 case underwent coronary artery bypass grafting), and 5 patients underwent other intracardiac malformation correction at the same time. There were no death or surgery related complications in the hospital for 30 days after the operation. A patient with preoperative extracorporeal cardiopulmonary resuscitation was continuously assisted by ECMO after emergency AAOCA correction and had complications such as limb ischemia necrosis and renal dysfunction after the operation. During the follow-up of 2.2 (3.3) years (range: 1 month to 7.2 years), one patient who previously underwent percutaneous transluminal coronary angioplasty with a stent implant experienced significant postoperative symptomatic relief, and the other discharged patients had no related symptoms.Conclusions:The accurate rate of initial diagnosis for high-risk AAOCA is still low, but the risk of cardiovascular accidents is high. For sports-related chest pain and other symptoms, more attention should be paid to the detection of AAOCA, especially for adolescents. Exercise stress testing can be helpful in evaluating the cardiovascular risk of asymptomatic AAOCA. Instant surgical treatment can achieve satisfactory curative effects.
7.Early clinical outcomes of the domestic KokaclipTM transcatheter edge-to-edge mitral valve repair system for severe degenerative mitral regurgitation: A single-arm, prospective, single-group target value clinical trial
Tong TAN ; Bingqi FU ; Peijian WEI ; Nianjin XIE ; Haozhong LIU ; Xiaoyi LI ; Shengwen WANG ; Haijiang GUO ; Jian LIU ; Jimei CHEN ; Jian ZHUANG ; Huiming GUO
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2024;31(02):269-275
Objective To summarize and analyze the preliminary clinical outcomes of the KokaclipTM transcatheter edge-to-edge mitral valve repair system for severe degenerative mitral regurgitation (DMR). Methods This study was a single-arm, prospective, single-group target value clinical trial that enrolled patients who underwent the KokaclipTM transcatheter edge-to-edge repair (TEER) system for DMR in the Department of Heart Surgery of Guangdong Provincial People's Hospital, Guangdong Cardiovascular Institute from June 2022 to January 2023. Differences in the grade of mitral regurgitation (MR) during the perioperative and follow-up periods were compared, and the incidences of adverse events such as all-cause death, thoracotomy conversion, reoperation, and severe recurrence of MR during the study period were investigated. Results The enrolled patient population consisted of 14 (50.0%) females with a mean age of 70.9±5.4 years. Twenty-eight (100.0%) patients were preoperatively diagnosed with typeⅡ DMR, with a prolapse width of 12.5 (11.0, 16.1) mm, a degree of regurgitation 4+ leading to pulmonary venous reflux, and a New York Heart Association cardiac function class≥Ⅲ. All patients completed the TEER procedure successfully, with immediate postoperative improvement of MR to 0, 1+, and 2+ grade in 2 (7.1%), 21 (75.0%), and 5 (17.9%) patients, respectively. Mitral valve gradient was 2.5 (2.0, 3.0) mm Hg. Deaths, thoracotomy conversion, or device complications such as unileaflet clamping, clip dislodgement, or leaflet injury were negative. Twenty-eight (100.0%) patients completed at least 3-month postoperative follow-up with a median follow-up time of 5.9 (3.6, 6.8) months, during which patients had a mean MR grade of 1.0+ (1.0+, 2.0+) grade and a significant improvement from preoperative values (P<0.001). There was no recurrence of ≥3+ regurgitation, pulmonary venous reflux, reoperation, new-onset mitral stenosis, or major adverse cardiovascular events. Twenty-two (78.6%) patients’ cardiac function improved to classⅠorⅡ. Conclusion The domestic KokaclipTM TEER system has shown excellent preliminary clinical results in selected DMR patients with a high safety profile and significant improvement in MR. Additional large sample volume, prospective, multicenter studies, and long-term follow-up are expected to validate the effectiveness of this system in the future.
8.Prenatal ultrasound diagnosis of isolated persistent vitelline artery: a case report and literature review
Fengqin LIU ; Liuying ZHOU ; Na SHU ; Nan YE ; Jimei XI
Chinese Journal of Perinatal Medicine 2024;27(3):209-214
Objective:To summarize the ultrasonographic features and prognosis of fetal persistent vitelline artery.Methods:The prenatal ultrasound features, genetic testing results, and prognosis of a fetus with an isolated persistent vitelline artery that was diagnosed in our hospital in December 2021 were retrospectively analyzed. Relevant articles were retrieved from CNKI, VIP, Wanfang, Yiigle, PubMed, Embase, and UpToDate databases using the terms "persistent vitelline artery", "type Ⅱ single umbilical artery", and "prenatal ultrasound" in both Chinese and English. Prenatal ultrasound features and prognosis of the persistent vitelline artery in fetuses were summarized using descriptive statistical analysis.Results:(1) Case report: In this case, ultrasound at 23 gestational weeks showed that an abnormally large blood vessel deriving from the celiac artery near the superior mesenteric artery entered the placenta through the umbilical opening in parallel with the umbilical vein. Color Doppler showed a blood flow spectrum like that in the umbilical artery. The transverse section image showed that bilateral umbilical arteries were not observed in the bladder and the free segment of the umbilical cord was in the shape of the Chinese character "Lyu". No obvious other structural abnormalities and a negative result of genetic testing were observed in the fetus. Followed up to one year old, the patient showed normal growth and development. (2) Literature review: A total of five articles involving four cases were retrieved (three in English and two in Chinese). Among the five cases, including the present case, one was terminated due to left renal agenesis and abnormal heart arteries ratio revealed by prenatal ultrasound, and the remaining four cases without obvious structural abnormalities in the prenatal ultrasound were born and developed well. Histopathological examination of the umbilical cord was performed in three cases, of which two with persistent vitelline artery had a distinct internal elastic lamina, and one with remained vitelline duct.Conclusions:The prenatal ultrasound of fetal persistent vitelline artery is typically characterized by an abnormal vessel that is derived from the abdominal aorta or superior mesenteric artery and plays the function of the umbilical artery. The prognosis of the isolated persistent vitelline artery is good, but a better understanding of such embryonic abnormalities is needed as there are few relevant reports at home and abroad.
9.Surgical prognosis for infracardiac total anomalous pulmonary venous connection: experience in a single institution
Miaoyun CHEN ; Furong LIU ; Xiaobing LIU ; Jimei CHEN ; Shusheng WEN ; Haiyun YUAN ; Jianzheng CEN ; Jian ZHUANG
Chinese Journal of Thoracic and Cardiovascular Surgery 2024;40(1):28-33
Objective:This study aimed at reviewing surgical experiences and exploring risk factors for mortality and postoperative complications in patients with infracardiac total anomalous pulmonary venous connection (TAPVC).Methods:This retrospective study included 74 infants who underwent conventional repair (28 cases) and sutureless repair (46 cases) in one hospital from February 2009 to December 2022. Clinical data were reviewed to assess risk factors for mortality and postoperative pulmonary venous obstruction (PVO). Kaplan- Meier curves and cox regressions were applied to analyze the overall survival. Cumulative incidence curve and sub-distribution hazard models were used to evaluate postoperative PVO. Results:There were 4 early deaths and 3 late deaths, and the overall survival rate was 90.5%. A total of 12 patients complicated postoperative PVO. The median follow-up was 39.4 months ( IQR: 13.3 to 73.7 months). The overall survival rate was higher in the sutureless group than the conventional group ( P=0.003). The incidence rate of postoperative PVO in the conventional group was higher than that in the sutureless group ( P= 0.008). Risk factors for recurrent PVO included longer cardiopulmonary bypass time, the increase of direct bilirubin level and international normalized ratio level before surgical repair. Conclusion:Both sutureless and conventional repairs for patients with infracardiac TAPVC can achieve acceptable postoperative outcomes. Sutureless repair has a higher survival rate and a lower incidence of re-stenosis in pulmonary veins and anastomosis.
10.Outcomes of total cavopulmonary connection in the treatment of functional single ventricle with heterotaxy syndrome: A propensity score matching study
Linjiang HAN ; Xiang LIU ; Jianrui MA ; Ziqin ZHOU ; Jiazichao TU ; Ruyue ZHANG ; Miao TIAN ; Ying LI ; Haiyun YUAN ; Shusheng WEN ; Jimei CHEN
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2024;31(04):510-518
Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). Methods A retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). Conclusion Compared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.

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