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Coronary Artery Complications after Right Ventricular Outflow Tract Reconstruction surgery

congenital Heart Disease 2022年第3期17卷 281-295页

作者： Hye Won Kwon Mi Kyoung Song Sang Yun Lee Gi Beom Kim Sungkyu Cho Jae Gun Kwak Woong-Han Kim Whal Lee Eun Jung Bae Department of Pediatrics Seoul National University HospitalSeoul National University College of MedicineSeoulSouth Korea Department of Thoracic and Cardiovascular surgery Seoul National University HospitalSeoul National University College of MedicineSeoulSouth Korea Department of Radiology Seoul National University HospitalSeoulSouth Korea

Background:Mechanisms and clinical manifestations of coronary artery complications after right ventricular outflow tract reconstruction surgery are not well ***:Patients who had coronary artery complications after pulmonary valve replacement or the Rastelli procedure at a single tertiary centre were retrospectively ***:Coronary artery complications were identified in 20 patients who underwent right ventricular outflow tract reconstruction *** median age at diagnosis of coronary artery complication was 21 years(interquartile range:13–25 years).Mechanisms of coronary artery complications were compression by adjacent materials in 12 patients,dynamic compression of intramural course of coronary artery in two patients,and intraoperative injury in six *** coronary artery anomalies were identified in 50%(10/20)of *** patients presented with early postoperative haemodynamic *** patients showed late onset symptoms or signs of coronary insufficiency,including chest pain,ventricular dysfunction,or ventricular *** artery stenosis was incidentally found on cardiac computed tomography angiography in two asymptomatic *** patients underwent surgical interventions,and one patient underwent percutaneous coronary intervention for coronary *** patient with recurrent ventricular tachycardia required an implantable *** were two deaths in patients with intraoperative coronary ***:Preoperative coronary evaluation and long-term follow-up for the development of coronary artery complications are required in patients undergoing right ventricular outflow tract reconstruction surgery to prevent ventricular dysfunction,arrhythmias,and death,especially among those with congenital coronary anomalies.

关键词： congenital heart disease right ventricular outflow tract reconstruction surgery coronary artery disease

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Randomized Study Comparing Pre-Operative Glycemic Profile in Pediatric cardiac Surgical Patients Administered Oral Carbohydrate Solution Preoperatively versus Those Kept Fasting

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World Journal of Cardiovascular Diseases 2018年第6期8卷 298-306页

作者： Uma Balasubramaniam Usha Kiran Suruchi Hasija V. Devagourou Department of Cardiothoracic and Vascular Anaesthesia AIIMS New Delhi India Department of Cardiothoracic and Vascular surgery AIIMS New Delhi India

Objectives: Hypoglycemia is a recognized danger in pediatric patients. Extended period of preoperative fasting in this subset of patients is not well tolerated with metabolic derangements. The oral carbohydrate loading preoperatively can ameliorate many adverse effects. The aim of this study was to compare the glycemic profile in pediatric cardiac surgical patients kept fasting preoperatively with those fed oral clear solutions of carbohydrate half hour prior to induction of anaesthesia. Also we tried to establish a correlation with other factors contributing to preoperative hypoglycemia. Methodology: We planned a randomized controlled study. Group A included patients who were kept fasting according to the ASA guidelines preoperatively and Group B included patients who received 2 ml per kg of body weight of 10% Dextrose water as oral feeds half hour before the expected time of start of anaesthesia. Results: The mean (SD) preoperative BG concentrations were higher in group B (102.5 ±16.97) as compared to group A (64.08 ± 25.37) (p value -0.86 and -0.67) (pvalue Conclusion: Preoperative oral carbohydrate preloading can develop as the easiest and cheapest path to better perioperative blood glucose concentration management in congenital cardiac disease children.

关键词： congenital cardiac Disease Hypoglycemia Hypocalcemia Oral Carbohydrate Solution Preoperative Cyanotics Hematocrit

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Surgical outcomes of functional single ventricle with total anomalous pulmonary venous connection

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South China Journal of Cardiology 2020年第1期21卷 38-46,52页

作者： OU Yan-qiu LIU Xiao-bing CEN Jian-zheng LIU Xiao-qing Department of Epidemiology Guangdong Cardiovascular InstituteWHO Collaborating Center for Research and Training in Cardiovascular DiseasesGuangdong Provincial Key Laboratory of South China Structural Heart DiseaseGuangdong Provincial People’s HospitalGuangdong Academy of Medical SciencesGuangzhou 510180GuangdongChina Department of cardiac surgery Guangdong Cardiovascular InstituteWHO Collaborating Center for Research and Training in Cardiovascular DiseasesGuangdong Provincial Key Laboratory of South China Structural Heart DiseaseGuangdong Provincial People’s HospitalGuangdong Academy of Medical SciencesGuangzhou 510180GuangdongChina

Background The surgical outcomes have been greatly improved over the past four decades for functional single ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connection(TAPVC),especially obstructed TAPVC,has remained a clinical challenge. Little is known about the results of surgical treatment for patients with FSV-TAPVC in China. Methods Forty consecutive patients with FSV-TAPVC undergoing initial surgical palliation(median age:16.8 months;body weight:8.25 kg)were retrospectively enrolled from 2006 to 2016 in a specialized cardiovascular institute. TAPVC was repaired in 30 patients. The mean follow-up period was 49 months. Results All of the 7 patients with preoperative pulmonary vein obstruction(PVO)underwent repair of TAPVC at the first operation and survived. When a systemic to pulmonary(S-P)shunt or pulmonary artery banding(PAB)was necessary,patients with concomitant TAPVC repair had lower mortality(30.0%)and higher total cavopulmonary connection completion rate(20.0%)than those without TAPVC repair[75.0% and 0%,respectively],although with no statistical significance(Fisher test,P=0.505 and P=0.245). The overall survival rates for the cohort at 1,3 and 5 years after the initial surgical intervention were 80.0%,77.1% and 77.1%,respectively. Multivariate COX regression analysis detected that SP shunt(adjusted odds ratio:6.51;95%CI:1.26-33.71,P=0.030)is the only risk factor for survival. The reintervention rate for postoperative PVO was higher in patients with preoperative PVO than those without(57.1%vs. 4.3%,Log Rank:P=0.006). Conclusion The mid-term results of surgical repair of FSV-TAPVC are still challenging. When PVO exists,surgical repair for TAPVC is suggested as soon as possible. Repair for TAPVC should also be considered when S-P shunt or PAB is necessary. Further study with larger population are warranted to support our finding.

关键词： congenital heart defects functional single ventricle total anomalous pulmonary venous connection sutureless technique

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The Genotype and Expression of TGFβ2 Gene in Children with congenital Conotruncal Defects

The Genotype and Expression of TGFβ2 Gene in Children with ...

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第十二次全国医学遗传学学术会议

作者： Yingying Meng Xiaojing Ma Jing Zhang Huijun Wang Duan Ma Guoying Huang cardiac Center Children's Hospital of Fudan University Key Laboratory of Molecular Medicine Ministry of EducationDepartment of Biochemistry and Molecular BiologyInstitute of Medical SciencesShanghai Medical CollegeFudan University

Objectives:Animal studies have shown that knockout of the transforming growth factor beta 2(TGFP2) gene results in diverse cardiovascular malformations and its dysregulated expression is involved in the pathogenesis of heart ***,little information is available on the genetic and expression alternations of TGFP2 gene in children with congenital heart disease. Herein,we investigated the genotype and expression of TGFP2 gene in children with congenital conotruncal defects(CTD).Methods:We sequenced the whole coding region of TGFP2 gene in 400 children with *** further analyzed the mRNA and protein expression of the TGFP2 gene in the myocardial tissues in 37 children with CTD and 5 age-matched healthy children with real-time PCR and ***:DNA sequencing showed no pathogenic mutations in the coding region of TGFp2 gene except for a silent mutation(1250T>C) in the exon 4 in one *** were no significant differences for the TGFP2 expression at either mRNA or protein level in the myocardial tissues between children with CTD and children without heart ***:The results indicate that germline mutation of the TGFp2 gene is not a common cause of CTD in human and the TGFP2 expression level may be less critical in humans than in animals for the pathogenesis of CTD.

关键词： TGFβ2 gene mutation gene expression conotruncal defects

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Interventricular Septal Hematoma after congenital cardiac Defects Repair at a Single Institution

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congenital Heart Disease 2022年第6期17卷 687-695页

作者： Yuan Hu Xiaohui Yang Jie Dong Peng Huang Jinwen Luo Guangxian Yang James D.StLouis Xicheng Deng Department of Echocardiography and Ultrasound Hunan Children’s HospitalChangshaChina Heart Center Hunan Children’s HospitalChangshaChina Pediatrics Research Institute of Hunan Province Hunan Children’s HospitalChangshaChina Departments of surgery and Pediatrics Children’s Hospital of GeorgiaAugusta UniversityAugustaGeorgiaUSA

Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The managementvaries according to the literature. We present our experience with this rare complication. Methods:Echocardiography database were reviewed with the term ‘‘hematoma’’ or “hypoechoic mass” for patients whounderwent congenital heart surgery from January 2018 to December 2021 at our institution to identify potentialinterventricular septal hematoma cases. Relevant data of the patients identified were collected. Focus was put onthe presentation, management, outcomes according to patent medical charts and serial echocardiographic reportdata. Results: In total, there were 5 patients included. The mean age and weight at surgery were 5.5 ± 3.6 monthsand 5.5 ± 1.4 kg, respectively. Four patients were diagnosed with ventricular septal defect and the other one beingdouble outlet of the right ventricle. While all patients had intraoperative transesophageal echocardiography, 80%(4 of 5) of Interventricular septal hematoma were revealed intraoperatively. Only one patient received hematomadrainage intraoperatively while the other 3 identified in the operating room were only closely observed. One afterventricular septal defect repair presented continuous dysfunction of the left ventricle at the last follow-up, whilethe others were doing well. All hematomas resolved completely with a mean time to interventricular septal hematomaresolution of 35.8 ± 16.9 days. Conclusion: Infants seem to be at a higher risk for Interventricular septalhematoma following congenital heart surgery. While the majority of interventricular septal hematoma has abenign postoperative course, some may result in ventricular dysfunction. Management strategies may be chosenon a case-by-case basis.

关键词： Interventricular septal hematoma ventricular septal defect double outlet right ventricle transesophageal echocardiography high-pressure waterjet

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Etiopathogenesis research of tetralogy of Fallot

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South China Journal of Cardiology 2014年第1期15卷 84-92页

作者：齐霄陈寄梅庄建朱平 Department of Cardiovascular surgery Guangdong Cardiovascular InstituteGuangdong General HospitalGuangdong Academy of Medical Sciences Shantou University Medical College

Tetralogy of Fallot （TOF） is the most common form of cyanotic congenital cardiac disease in humans. With recent advances in corrective surgery early lethality from TOF is rare but long-term sequelae, including arrhythmia, ventricular dysfunction and often life-long disability. Now attention has shifted from immediate outcomes to understanding causation. This review summarizes current knowledge regarding the embryonic and molecular mechanisms involved in cardiac development, with particular attention paid to the role of transcription factors and to their potential usefulness in order to clarify the genetic basis of TOF.

关键词： congenital heart disease tetralogy-of-Fallot conotruncal defect transcription factor gene mutation.

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The inotropic score as a predictor of mortality in neonates after complex cardiac surgery

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South China Journal of Cardiology 2021年第2期22卷 96-102,F0003页

作者： ZHOU Hui-jun DU Yang ZHANG Chong-jian Department of Cardiovascular surgery Guangdong Provincial People's HospitalGuangdong Academy of Medical SciencesGuangzhou 510180GuangdongChina.

Background Neonates undergoing cardiac surgery are at high risk of postoperative *** is necessary to identify clinical factors that are indicative of in-hospital *** study investigated the predictive value of inotropic score(IS)and vasoactive-inotropic score(VIS)for mortality in neonates after complex cardiac *** This was a retrospective analysis of the 214 neonates who underwent complex cardiac surgery for congenital heart disease at a tertiary pediatric cardiac surgery intensive care unit from January 2015 to November *** demographics,intraoperative and postoperative variables were *** logistic regression and the receiver operating characteristic(ROC)curve were performed to evaluate the relationship between IS/VIS and in-hospital *** The neonates were divided into two groups according to *** in the non-surviving group(n=16)had higher IS and VIS than those in surviving group(n=198).On multivariable regression analysis,after adjustment for potential confounders,IS but not VIS remained a strong predictor of in-hospital mortality,[initial IS:odds ratio(per 5-score increase):1.68,95%confidence interval:1.16-2.44,P=0.007;maximum IS:odds ratio(per 5-score increase):1.62,95%confidence interval:1.10-2.37,P=0.014].Conclusions In newborns with congenital heart disease,IS but not VIS within 24 hours is helpful for prediction of mortality at the early postoperative period after neonatal complex cardiac surgery.

关键词： neonates cardiac surgery inotrope score vasoactive inotrope score

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Hemodynamic Changes and Clinical Outcomes after the Intra/Extracardiac Fenestrated Fontan Procedure

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World Journal of Cardiovascular surgery 2018年第2期8卷 29-39页

作者： Naveen Menon Chihyang Lin Cesar I. Mesia Achintya Moulick Randy Stevens Vicki Mahan Drexel University College of Medicine and Department of Pediatric Cardiothoracic surgery St. Christopher’s Hospital for Children Philadelphia PA USA

Background: The primary concern of the Fontan procedure has been atrial arrhythmias, but little attention has been given to hemodynamic changes. This study’s purpose was to examine the hemodynamics and arrhythmias of the Intra/extracardiac fenestrated Fontan, and determine any advantages/disadvantages of this newer Fontan procedure. Methods: Patients undergoing the intra/ extracardiac fenestrated Fontan procedure at this institute between December 2009 and June 2017 were included in this retrospective evaluation of preoperative and postoperative hemodynamics during cardiac catheterization. End Diastolic Pressure (EDP), Pulmonary Artery Pressure (PAP), Left Atrial Pressure (LAP), and Transpulmonary Gradient (TPG) were the hemodynamic markers of interest. Occurrence of arrhythmias was also examined preoperatively, less than 2 weeks postoperatively, and greater than 2 weeks postoperatively. Morbidities and mortalities were also evaluated. Results: Fourteen patients underwent the procedure between December 2009 and June 2017. Preoperative data was available in all of these patients. Postoperative data was partially incomplete for atrial arrhythmias and hemodynamic data. EDP rose from 9.29 ± 3.50 mmHg to 10.33 ± 3.12 mmHg. PAP rose from 12.57 ± 2.50 mmHg to 14.27 mmHg. TPG dropped from 5.00 ± 2.29 mmHg to 3.25 ± 1.67 mmHg. LAP rose from 7.57 ± 2.87 mmHg to 10.30 ± 1.95 mmHg. Atrioventricular valve (AVV) regurgitation remained the same pre and postoperatively. 8 of 13 patients developed arrhythmias at less than 2 weeks postoperatively, and 5 of 12 patients developed arrhythmias at greater than 2 weeks postoperatively. There were no morbidities or mortalities. Conclusions: We found a statistically significant change in EDP, PAP, LAP, and TPG levels. It is unclear whether this is an advantage or disadvantage. The significance of these changes is unclear in this small population of patients. Further evaluation of hemodynamics, arrhythmias, morbidities and mortalities associa

关键词： Fontan Functionally Univentricular Heart Circulatory Hemodynamics Hypoplastic Left Heart Syndrome congenital Heart Disease (CHD)

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Coronary Artery Anomalies Detected by Multi-Detector Computed Tomography: A Single Centre Experience

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World Journal of Cardiovascular Diseases 2023年第6期13卷 261-274页

作者： Basma Hammad Eman El-sharkawy Yasser Morsi Noha Shabaan Salah Eltahan Mohamed Elshafi Shady Abohashem Tanveer Mir Mostafa Elwany Cardiology Department Alexandria University Alexandria Egypt International cardiac Center-Scan Alexandria Egypt Radiology Department Alexandria University Alexandria Egypt Massachusettes General Hospital Boston Massachusetts USA Detroit Medical Center Wayne State University Detroit Michigan USA

Background: Coronary artery anomalies (CAA) prevalence represents less than 1% of congenital heart diseases. It includes anomalies in origin, course, and termination. Its detection has been easier with advances in imaging techniques using multi-detector computed tomography (MDCT). MDCT helps not only detection of the anomalous origin, but it allows delineation of the course and termination of the arteries, differentiation between benign and malignant courses, and guiding therapeutic interventions. Results: There were consecutive patients with a low-to-intermediate probability of coronary artery disease scanned with 128 MDCT. Each patient underwent a non-contrast prospective gating acquisition for coronary calcium scoring followed by contrast-enhanced helical retrospective gated scans for the detection of coronary artery origin, course, termination, and detection of concomitant atherosclerosis. We scanned 1000 patients with a mean age of 57.5 ± 8.3, and 68% were males. Thirty-two anomalies were noted (3.2%) including;nineteen (1.9%) anomalous origin from the opposite sinus, three (0.3%) anomalous left coronary arteries from the pulmonary artery (ALCAPA), one (0.1%) super dominant left anterior descending artery (LAD) giving origin to the posterior descending artery (PDA), three single coronary arteries (0.3%)in which the left main (LM) and right coronary arteries were originating with a common stem from the right coronary sinus (RCS)and the LM took a pre-pulmonic course. Along with six (0.6%) dual LAD including five (0.5%) patients with type I (short LAD and long diagonal), there was one (0.1%) type 4 with an extra LAD originating from the RCS with a pre-pulmonic course. Conclusions: MDCT allows easy detection of coronary anomalies with high spatial resolution and overcomes limitations in conventional invasive coronary angiography. Based on our study we recommend the use of MDCT as an efficient and feasible modality for the diagnosis of coronary anomalies once this p

关键词： Coronary Artery Anomalies Spatial Resolution Multi-Detector Computed Tomography

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先天性袋状结肠被纵向剪开可用于阴道重建

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世界核心医学期刊文摘（儿科学分册） 2006年第8期2卷 46-46页

作者： Wester T. Lckgren G. Christofferson R. Rintala R.J. 朱新菊 Department of paediatric surgery University Children's HospitalSE-751 85 UppsalaSweden

congenital pouch colon(CPC)is a rare malformation in which the distal part o f a shortened colon forms a dilated *** is associated with an anorectal ma *** report 2 patients with CPC,one with a cloaca and one with vestib ular fistula and vaginal *** is the first description of CPC,vestibular fistula,and vaginal *** purpose of this report was to demonstrate tha t the pouch can be split longitudinally-in analogy with Bianchi’s intestinal l engthening procedure [Bianchi *** loop lengthening:a technique for in creasing small intestinal length.J Pediatr Surg 1980;15:145-51]-to create a v agina and to reconstruct the anorectum with preserved blood supply.

关键词：阴道重建先天性结肠肛门直肠畸形阴道闭锁血液供应直肠肛门 CPC 前庭瘘延长术

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