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Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of pulmonary atresia by Dual-Source Computed Tomography

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Congenital Heart Disease 2023年第1期18卷 113-125页

作者： Wenlei Qian Xinzhu Zhou Ke Shi Li Jiang Xi Liu Liting Shen Zhigang Yang Department of Radiology West China HospitalSichuan UniversityChengduChina Department of Radiology Chengdu Women’s and Children’s Central HospitalSchool of MedicineUniversity of Electronic Science and Technology of ChinaChengduChina Department of Radiology Peking University Cancer Hospital and InstituteBeijingChina

Background:pulmonary atresia(PA)is a group of heterogeneous complex congenital heart *** one study modality might not get a correct *** study aims to investigate the diagnostic power of dualsource computed tomography(DSCT)for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography(TTE).Materials and Methods:This retrospective study enrolled 79 patients and divided them into three groups according to their main *** associated malformations and clinical information,including treatments,were recorded and compared among the three *** diagnostic power of DSCT and TTE on all associated malformations were *** surgical index(McGoon ratio,pulmonary arterials index(PAI),and total neopulmonary arterial index)and radiation dose were calculated on the basis of ***:Of the patients,32,30,and 17 were divided into the groups of PA with ventricular septal defect(VSD),PA with VSD and major aortopulmonary collateral arteries,and PA with other major malformations,***,182,162,and 13 intracardiac,extracardiac,and other major malformations were diagnosed,***,DSCT showed a better diagnostic performance in extracardiac deformities(154 vs.117,p<0.001),whereas TTE could diagnose intracardiac deformities better(159 vs.139,p=0.001).The McGoon ratio,PAI,and treatment methods were significantly different among the three groups(p=0.014,p=0.008,and p=0.018,respectively).Conclusion:More than one imaging modality should be used to make a correct diagnosis when clinically suspecting *** is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.

关键词： pulmonary atresia complex congenital heart diseases dual-source computed tomography transthoracic echocardiography

A Novel Perspective on Histopathology Provides Novel Insights into Surgical Effects in pulmonary atresia,Ventricular Septal Defect,and Major Aortopulmonary Collateral Arteries:A Case-Series Study

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Congenital Heart Disease 2021年第3期16卷 245-254页

作者： Jinyang Liu Xianchao Jiang Runsi Wang Li Li Ju Zhao Fuxia Yan Run Yuan Qiang Wang Center for Pediatric Cardiac Surgery Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina National Clinical Research Center for Cardiovascular Diseases Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Pathology Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Cardiopulmonary Bypass Fuwai HospitalNational Center of Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Anesthesiology Fuwai HospitalNational Center of Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Nutriology Fuwai HospitalNational Center of Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

Background:Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries(MAPCAs)and outcomes of selective unifocalization of pulmonary atresia with ventricular septal defects and ***:This is a case-series *** patients were *** assessment was conducted to determine whether collateral arteries should be unifocalized or treated with intraoperative *** were collected and stained by HE and ET+***:Twelve patients underwent one-stage unifocalization at a median age of 37 months(range:6–228 months)and a median weight of 14.0 kg(range:5.0–49.0 kg),which produced a favorable right ventricle to aortic systolic pressure ratio of no more than 0.5 except in one patient who *** were divided into three groups:Group 1(n=6),had no native pulmonary arteries,and collateral arteries supplied all pulmonary blood;Group 2(n=6)presented dysplastic native pulmonary arteries on one or both sides,and in some lung lobes or segments,blood was supplied only by collateral arteries;Group 3(n=1)had well-developed left and right pulmonary arteries,and collateral arteries,and pulmonary arteries provided blood flow to the same *** reports demonstrated two types of collateral arteries:Elastic arteries presented an arborization distribution similar to native pulmonary artery walls,while muscular arteries showed high resistance and *** selectively unifocalized single-supply collateral arteries with morphologic features based on the arborization ***:We found that there were two kinds of MAPCAs with different histology,and we performed selective UF for MAPCAs that might belong to the elastic *** unifocalization achieved a low right ventricle to aortic systolic pressure ratio and favorable surgical effects.

关键词： Histopathology selective unifocalization pulmonary atresia major aortopulmonary collateral arteries

Management of ductal spasm in a neonate with pulmonary atresia and an intact ventricular septum during cardiac catheterization:A case report

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World Journal of Clinical Cases 2022年第35期10卷 13015-13021页

作者： Xu Zhang Ning Zhang Hai-Cheng Song Yue-Yi Ren Department of Heart Center Qingdao Women's and Children's Hospital Affiliated to Qingdao UniversityQingdao 266034Shandong ProvinceChina Department of Medical Technology Qingdao Women's and Children's Hospital Affiliated to Qingdao UniversityQingdao 266034Shandong ProvinceChina

BACKGROUND Ductal spasm is a rare but life-threatening complication of cardiac catheterization in neonates with pulmonary atresia and an intact ventricular *** patients with ductal-dependent pulmonary blood flow,ductal spasm may lead to refractory hypoxemia and severe hemodynamic instability,which need to be treated in perfect *** SUMMARY We present a male infant with a gestational age of 39 wk,and his fetal echocardiography showed pulmonary *** 28 d of age,transcatheter pulmonary valvuloplasty with balloon dilatation was *** hours after the operation,the patient's pulse oxygen saturation continued to *** patient was then transferred to receive cardiac *** catheterization,the invasive blood pressure and pulse oxygen saturation suddenly decreased,and repeated aortography revealed partial occlusion of the ductus *** no longer changed when pulse oxygen saturation rose to 51%after approximately 20 min of maintenance ***,a ductal stent was used for *** and hypoxemia were *** We should know that ductal spasm may occur during pulmonary atresia and intact ventricular septum cardiac *** the pathophysiology of ductal-dependent pulmonary blood flow and make comprehensive perioperative preparations essential to deal with hemodynamic disorders caused by ductal spasm.

关键词： Cardiac catheterization Ductus arteriosus Ductal stent Ductal spasm Intact ventricular septum pulmonary atresia Case report

Original article：A novel two-stage complete repair method for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

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Chinese Medical Journal 2010年第3期 259-264页

作者： MEI Ju DING Fang-bao ZHU Jia-quan BAO Chun-rong XIE Xiao ZHANG Yun-jiao Department of Cardiothoracic Surgery Xinhua Hospital Shanghai Jiao Tong University School of Medicine Shanghai 200092 China

Background pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs） remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs. Methods Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 （17.9±1.6）. There were a total of 43 MAPCAs in all the patients （3-5 （3.9~0.7） MAPCAs per patient）. The accumulated Nakata index was （222.9＋_29.9） mm2/m2 （ranged from 182 to 272）. All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline sternotomy. Results All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95%-100%, which was significantly higher than pre-operation （P 〈0.01）. During the follow-up period of 3-51 （25.4±15.2） months, there were no late death and no need for reintervention. All the patients enjoyed their lives with good conditions. Conclusions This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative surgical a

关键词： pulmonary atresia major aortopulmonary collateral arteries unifocalization surgery congenital heart disease

A novel two-stage complete repair method for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

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中华医学杂志(英文版) 2010年第3期123卷 259-264页

Background pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries(MAPCAs) remains a challenging complex congenital heart disease *** the present study,we aimed to developa two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and *** Between December 2003 and December 2008,7 female and 4 male patients between the age of 5 and 10years who were suffering from PA-VSD and MAPCAs were selected and *** native pulmonary artery trunkswere absent in all patients;the lungs were solely supplied by major aortopulmonary collaterals,and the numbers ofsupplied lung segments ranged from 15 to 20 (17.9±1.6).There were a total of 43 MAPCAs in all the patients (3-5(3.9±0.7) MAPCAs per patient).The accumulated Nakata index was (222.9±29.9) mm~2/m~2 (ranged from 182 to 272).Allthe patients underwent two sequential *** one included left major aortopulmonary collateral unifocalizationand modified Blalock-Taussig shunt from left posterior lateral thoracotomy;stage two comprised right unifocalization,ligation of the shunt,followed by ventricular septal defect closure and right ventricular outflow tract reconstructionassisted with cardiopulmonary bypass from midline *** All the patients survived the initial surgery,but one of them died of low cardiac output syndrome on the thirdday after the second *** complications included pneumonia in one case and capillary leaksyndrome in *** oxygen saturation maintained about 95%-100%,which was significantly higher thanpre-operation (P<0.01).During the follow-up period of 3-51 (25.4±15.2) months,there were no late death and no needfor *** the patients enjoyed their lives with good *** This two-stage complete repair strategy was well-tolerated and effective with good outcome,thus offeringan alternative surgical approach in the treatment of PA-VSD an

关键词： pulmonary atresia major aortopulmonary collateral arteries unifocalization surgery congenital heart disease

Impact of right ventricle to pulmonary artery connection and systemic-to-pulmonary artery shunt surgery on promoting the development of pulmonary vasculature in patients with pulmonary atresia with ventricular septal defect

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South China Journal of Cardiology 2022年第1期23卷 33-38页

作者： MA Jian-rui ZHANG Shuai QIU Hai-long ZHENG Hai-yun CEN Jian-zheng Department of Cardiovascular Surgery Guangdong Cardiovascular InstituteGuangdong Provincial People's HospitalGuangdong Academy of Medical SciencesGuangzhou 510080China

Background pulmonary atresia with ventricular septal defect(PA/VSD)is a rare and complex congenital heart disease(CHD).The optimal palliative surgical strategy for pulmonary atresia with ventricular septal defect(PA/VSD)in neonates and young infants is *** mainly includes the following two options,right ventricle to pulmonary artery connection(RV-PA)and systemic-to-pulmonary artery shunt surgery(SPS).Objectives:To determine the impact of the right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy on promoting the development of pulmonary vasculature in patients with pulmonary atresia and ventricular septal defect(PA/VSD).Methods:From January 2010 to December 2019,104 patients with PA/VSD in Guangdong Cardiovascular Institute who underwent right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy to promoting the development of pulmonary vasculature were identified and enrolled in this retrospective *** cohort was divided into two groups:group-Right Ventricle to pulmonary Artery Connection(RV-PA),who underwent initial palliation with staged repair(n=51),and group-Systemic-to-pulmonary Artery Shunt(SPS)(n=53).Preoperative and postoperative the development of pulmonary vasculature data have been collected and ***:Before the surgery,the Mc Goon Ratio of the RV-PA group significantly high than and the SPS group(Ppulmonary artery shunt,right ventricle to pulmonary artery connection is more e

关键词： pulmonary atresia Ventricular septal defect Congenital heart disease

The Clinical Application Value of Selective Unifocalization in the Treatment of Severe pulmonary Artery atresia with Ventricular Septal Defect

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Congenital Heart Disease 2021年第1期16卷 65-71页

作者： Jinyang Liu Bo Peng Xianchao Jiang Simeng Zhang Jiachen Li Lizhi Lv Qiang Wang Xiang Li Pediatric Cardiac Surgery Center National Center for Cardiovascular Disease and Fuwai HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijingChina Department of Cardiac Surgery Yunnan Fuwai Cardiovascular HospitalKunmingChina Medical Nutrition Center National Center for Cardiovascular Disease and Fuwai HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijingChina

Background:This study aims to explore the efficacy of selective unifocalization(UF)for major aortopulmonary collateral arteries(MAPCAs)unifocalization in children with pulmonary atresia with ventricular septal defect(PA/VSD).Methods:A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December ***,age,preoperative cardiovascular angiography test results and McGoon ratio were *** properties of the collateral arteries were evaluated by angiography,and selective UF for the“dendritic”MAPCAs and ligation of MAPCAs demonstrating distortion and ***:A total of 13 severe patients underwent one-stage repair,of which 1 case underwent ventricular septal fenestration and died after 2 weeks of ECMO *** median age was 37 months,and a median weight was 14.0 kg.A right ventricular to aortic systolic pressure ratio(pRV/pAo)of no more than 0.5 was achieved in 12 living ***:Selective unifocalization based on MAPCAs morphology can achieved a good outcome at the early *** surgical concept might be provided a novel insight into treatment for some of the subgroups presenting with this complex form of PA/VSD/MAPCAs.

关键词： pulmonary atresia major aortopulmonary collateral arteries selective unifocalization

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

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Chinese Medical Journal 2024年第12期137卷 1431-1436页

作者： Liqing Zhao Lei Wang Hongping Xia Yurong Wu Xianting Jiao Hong Zhu Sun Chen Kun Sun Department of Pediatric Cardiology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Diagnosis and Treatment Center for in utero Pediatric Disease Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Neonatology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few *** study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary ***:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May *** success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new ***-up clinical records and echocardiography were obtained during the prenatal and postnatal ***:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 *** median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful *** patient had pulmonary valve perforation without balloon *** procedure-related deaths or significant complications ***,one neonatal death occurred due to heart and renal *** median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up ***:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

关键词： Fetal cardiac intervention Valvuloplasty pulmonary atresia Critical aortic stenosis Congenital heart disease

Management of“Wall to Wall Heart”in a Transient Neonatal Tricuspid Regurgitation

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Congenital Heart Disease 2021年第3期16卷 205-210页

作者： Elio Caruso Silvia Farruggio Davide Calvaruso David Fabio Petruccelli David Angel Ortiz Ruiz Corrado Di Mambro Salvatore Agati Mediterranean Pediatric Cardiology Center“Bambino Gesù” San Vincenzo HospitalTaorminaItaly

We present a case of a one-day-old newborn,without prenatal diagnosis,referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation of 80%.Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation,functional pulmonary valve atresia with intact ventricular septum and reversal flow in the large patent ductus arteriosus(PDA).Chest X-ray showed severe cardiomegaly and wall to wall *** E1 infusion was started once after *** few days,clinical conditions progressively worsened because of right heart failure;a first pharmacological approach to close PDA failed and surgery ligation of PDA was necessary to restore anterograde pulmonary flow and heart size.

关键词： Wall to wall heart tricuspid regurgitation pulmonary atresia arterial duct