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Congenital heart diseases trends in S?o Paulo State,Brazil:a national live birth data bank analysis

World Journal of Pediatrics 2022年第7期18卷 472-481页

作者： Paola Soares Fernandes Larissa Rosario Magalhaes Tainara Rita Pezzini Edige Felipe de Sousa Santos Mauricio Giusti Calderon Medical School Nine of July University(Uni9).Sao Bernardo do CampoSPBrazil Epidemiology Department Public Health SchoolSao Paulo University(USP)Sao PauloSPBrazil Pediatric Surgery Department Hospital Benficencia Portuguesa de Sao PauloRua Maestro Cardim768Sao PauloSP CEP 01323-011Brazil Pediatric Surgery Department Hospital Santa Catarina PaulistaAv.Paulista200.Sao PauloSP CEP:01310-000Brazil

Background Congenital heart diseases(CHD)are the most common type of birth defects,affecting millions of newborns every year;no prevalence data are available in S?o Paulo State,Brazil's most populous *** objective is to identify trends in prevalence and risk factors for CHD in Sao Paulo State,*** We developed a population-based study to estimate the CHD trend of prevalence in recent years,stratified by maternal age and geographical clusters,using all cases of CHD identified by the Live Births Information System(SINASC-Sistema de Informa??o Sobre Nascidos Vivos)from January 1,2005,to December 31,*** calculated the trends of prevalence,the regression coefficient(β),the annual percent change(APC),and 95%confidence interval using the Prais-Winsten regression model,with the Durbin-Watson *** We found 10,594 cases of CHD among 8,536,101 live births(LB),a prevalence of 12.4/10,000 *** was no difference in the sex distribution;they are primarily Caucasian(60.2%),75.2%born at term,and 74.4%weight>2500 g,66.9%of births were by cesarean section.S?o Paulo State presented an increasing CHD trend of prevalence(APC=18.9%).The highest CHD prevalence rate was in mothers aged≥35 years(22.2/10,000 LB).There were 12,271 specific congenital heart defects among 10,594 patients(1.16 CHD/patient).Atrial septal defect has the highest number of cases(3835),with a prevalence of 4.49/10,000 LB,corresponding to 31.3%of all *** CHD had an increasing prevalence trend in recent years,being highest in S?o Paulo City and≥35-year mothers.

关键词： Congenital abnormalities Epidemiology heart defects Interrupted time series PrevalencePublic health

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Implementation of a High-Risk Outpatient Clinic for Children with Complex Congenital heart Disease in a Reference Service in Brazil

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Congenital heart Disease 2023年第6期18卷 649-656页

作者： Gustavo Foronda Vanessa Ferreira Amorim de Melo Claudia Regina Pinheiro de Castro Grau Ingrid Magatti Piva Glaucia Maria Penha Tavares Ana Cristina Sayuri Tanaka Nana Miura Department of Pediatric Cardiology and Adult Congenital heart Diseases Heart Institute of the University of São PauloSão PauloBrazil Foronda Clinic São PauloBrazil Institute of Orthopedics and Traumatology University of São PauloSão PauloBrazil Department of Echocardiography Heart Institute of the University of São PauloSão PauloBrazil

Background:Children with congenital heart disease(CHD),even after surgical approaches,and especially those who undergo staged procedures in theﬁrst months of life,remain vulnerable to readmissions and complications,requiring very close monitoring and differentiated intervention ***:Descriptive and exploratory study,of the experience report type,which presents the process of building the high-risk outpatient clinic for complex congenital heart diseases(AAR)at the Instituto do Coração(InCor).Results:Report of the path taken to structure the AAR,demonstrating the organization,interface with the multidisciplinary team,admission and discharge criteria,training,and patient proﬁ*** theseﬁve years of care,275 patients were treated,59.65%with biventricular interstage physiology,followed by univentricular interstage physiology(34.55%),residual defects after surgical procedures(3.63%),tumors with risk of mechanical obstruction(1.45%)and patients with an intrauterine approach(0.72%).The signiﬁcant number of critical patients who were successfully discharged from hospital(44.72%),the low mortality rate due to sudden deaths at home and the high adherence to follow-up cor-roborate the impact of this specialized ***:Despite the limitations of the study,this experi-ence report showed that with few resources,there is the possibility of organizing an AAR with differentiated care,with the objective of early detection and treatment of residual injuries,identiﬁcation of early interventions,edu-cation of parents for follow-up of their children,resulting in individualized treatment,promoting a better quality of life for this population.

关键词： Congenital heart defects ambulatory care heart defects

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Dynamic Changes in Left and Right Cerebral Oxygen Saturation during Selective Cerebral Perfusion in Young Infants

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Congenital heart Disease 2023年第6期18卷 639-647页

作者： Hwa-Young Jang Sang-Jun Beon Sung-Hoon Kim In-Kyung Song Won-Jung Shin Department of Anesthesiology and Pain Medicine Laboratory for Cardiovascular DynamicsAsan Medical CenterUniversity of Ulsan College of MedicineSeoul05505Korea Department of Physician Education and Training Asan Medical CenterSeoul05505Korea

Objectives:We investigated whether the selective cerebral perfusion(SCP)technique causes differences in changes in cerebral perfusion between both hemispheres in young infants,using cerebral oxygen saturation(ScO2)as an ***,we determined the association between the discrepancy in ScO2 and cerebral perfu-sion pressure during ***:The difference in ScO2 between the left and right cerebral hemispheres(ΔScO2 Rt-Lt)was calculated during clamping of the innominate artery(IA)and during ***:In 25 infants(aged 2 to 78 days),the left and right ScO2 were well maintained(median 63.2%and 60.9%during IA clamping,respectively;64.0%and 65.6%during SCP,respectively).During IA clamping,right and left ScO2 decreased(med-ian-1.4%and-1.0%,respectively).During SCP,rightΔScO2 was higher compared to leftΔScO2(median 1.5%vs.0.6%;p<0.001).Eight patients had a higher rightΔScO2 than leftΔScO2 throughout *** had lowerΔScO2 Rt-Lt during IA clamping(median-3.2%vs.0.0%;p<0.001)and higherΔScO2 Rt-Lt during SCP than others(median 5.0%vs.-0.8%;p<0.001).During and after SCP,the correlation coefﬁcient between rightΔScO2 and change in the mean arterial pressure was higher in patients with a discrepancy than in others(r=0.731 vs.r=0.519;p<0.001).Conclusions:This study suggests that SCP permits adequate bilateral cerebral ***,the unilateral cerebral perfusion technique may cause a difference in cerebral perfusion between both hemispheres in young infants;this may depend on the perfusion pressure.

关键词： Cerebral circulation congenital heart defects monitoring near-infrared spectroscopy

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Characteristics of In-Hospital Patients with Congenital heart Disease Requiring Rapid Response System Activations: A Japanese Database Study

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Congenital heart Disease 2022年第1期17卷 31-43页

作者： Taiki Haga Tomoyuki Masuyama Yoshiro Hayashi Takahiro Atsumi Kenzo Ishii Shinsuke Fujiwara Department of Pediatric Critical Care Medicine Osaka City General HospitalOsaka534-0021Japan Department of Emergency and Critical Care Medicine Jichi Medical University Saitama Medical CenterSaitama330-8503Japan Department of Intensive Care Medicine Kameda Medical CenterChiba296-8602Japan Department of Emergency Medicine Seirei Hamamatsu General HospitalShizuoka430-8558Japan Department of Anesthesiology Intensive Care UnitFukuyama City HospitalHiroshima721-8511Japan Department of Emergency Medicine NHO Ureshino Medical CenterSaga843-0393Japan

Objectives:This study aimed to study the characteristics of in-hospital deterioration in patients with congenital heart disease who required rapid response system activation and identify risk factors associated with 1-month ***:We retrospectively analysed data from a Japanese rapid response system registry with 35 participating *** included consecutive patients with congenital heart disease who required rapid response system activation between January 2014 and March *** regression analyses were performed to examine the associations between 1-month mortality and other patient-specific ***:Among 9,607 patients for whom the rapid response system was activated,only 82(0.9%)had congenital heart *** few patients with congenital heart disease were being treated at the cardiology and cardiovascular surgery departments(12.3%and 9.9%,respectively).Moreover,the incidences of rapid-response events after intensive care unit discharge or surgery were low(6.8% and 12.2%,respectively).The most common reason for rapid response system activation was respiratory dysfunction(desaturation:35.4%,tachypnoea:25.6%,and new dyspnoea:19.5%).Rapid response system interventions and intensive care unit transfers were required for 65.9% and 20.7%of patients,*** mortality rate was 1.2% at the end of the rapid response system intervention and 11.0% after 1 ***,decreased respiratory rate and decreased heart rate at rapid response system activation were associated with increased 1-month *** adjusted odds ratio was 1.10(95% confidence interval 1.02–1.19)and 1.02(95% confidence interval,1.00–1.04) for respiratory rate and heart rate,***:Rapid response systems were rarely activated after cardiac surgery and intensive care unit discharge,which were situations with a high risk of sudden deterioration in patients with congenital heart ***,encouraging the use of the rapid response sys

关键词： heart defects congenital emergencies clinical deterioration hospital rapid response team critical care This

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Factors Affecting the Genetic Diagnostic Rate in Congenital heart Disease

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Congenital heart Disease 2022年第6期17卷 653-673页

作者： Jun Sung Park Go Hun Seo Yunha Choi Soojin Hwang Minji Kang Hyo-Sang Do Young-Hwue Kim Jeong Jin Yu Ellen Ai-Rhan Kim Euiseok Jung Byong Sop Lee Jae Suk Baek Beom Hee Lee Department of Pediatrics Asan Medical CenterUniversity of Ulsan College of MedicineSeoulKorea 3 Billion Inc.SeoulKorea Genome Research Center for Birth defects and Genetic Diseases Asan Institute for Life SciencesAsan Medical CenterSeoulKorea Division of Cardiology Department of PediatricsAsan Medical CenterUniversity of Ulsan College of MedicineSeoulKorea Division of Neonatology Department of PediatricsAsan Medical CenterUniversity of Ulsan College of MedicineSeoulKorea Medical Genetics Center Asan Medical Center Children’s HospitalUniversity of Ulsan College of MedicineSeoulKorea

Background: Over 400 genes contribute to the development of congenital heart disease (CHD). Additionally,multisystemic manifestations accompanying syndromic CHD pose a higher risk of genetic diseases. This studyinvestigated the diagnostic yield of whole-exome sequencing (WES) in patients with sporadic syndromic CHDand the phenotypic factors affecting the genetic diagnostic rate. Methods: Sixty-four patients with sporadic syndromicCHD aged 0.05), betweenthe groups with and without a diagnostic variant. However, patients with ≥3 extracardiac phenotypes had a significantlyhigher likelihood of having a diagnostic variant than those with ≤2 (38.3% vs. 5.9%, odds ratio = 9.93,95% confidence interval = 1.21–81.44, P = 0.013). Conclusions: The number of extracardiac phenotypes is importantin predicting the possibility of genetic diagnosis. Physicians will be able to select patients with a high probabilityof genetic diagnosis and provide appropriate genetic counseling based on the results of this study.

关键词： heart defects congenital whole-exome sequencing genetic testing phenotype

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Diagnosis of isolated dextrocardia using angiocardiography or surgery

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Chinese Medical Journal 2004年第11期117卷 1655-1658页

作者：马宁蒋世良黄连军赵世华徐仲英凌坚郑宏 Department of Radiology Cardiovascular Institute Fuwai Hospital Peking Union Medical College Chinese Academy of Medical Sciences Beijing 100037 China

Background Isolated dextrocardia is a rare phenomenon a nd usually associated with multiple cardiac anomalies. This study was to evaluat e the accuracy of diagnosis of isolated dextrocardia by using angiocardiography and to compare it with the results of *** The clinical data of 27 cases of congenital isolated dextrocardia were collected to understand the diagnostic approaches to the major cardiac anomalies. All cases underwent angiocardiography followed by palliative or curative surgery. The diagnosis was compared by angiocardiography relying on segmental analysis with the pathological features observed in *** The results of angiocardiography of 22 patients were the same as the pathological features observed during surgery, including one case with congenital left ventricular diverticulum was inadvertently omitted in angiocardiograhy. There were significantly dissimilar diagnoses between angiocardiograhy and post-operation in 5 patients, including anatomical corrected transposition of great arteries misinterpreted as corrected transposition of the great arteries in 1, complete transposition of great arteries misinterpreted as corrected transposition of the great arteries in 1, single ventricle misinterpreted as double-outlet right ventricle in 1, and anatomical double-outlet left ventricle misinterpreted as corrected transposition of the great arteries in 2. Misdiagnostic rate of angiocardiograhy was almost 20%.Conclusions Angiocardiography is of great significance in the diagnosis and classification of isolated dextrocardia. However, because of the intricacy of cardiac anomalies of isolated dextrocardia, atrial angiography and double oblique projection are needed to improve the accuracy of diagnosis to support surgical treatments.

关键词： heart defects congenital dextrocardia angiocardiography surgery

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Unicuspid Aortic Valve, an Extremely Rare Congenital Anomaly in Adults: A Systemic Review

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World Journal of Cardiovascular Diseases 2023年第6期13卷 283-288页

作者： Dilesha Dilangi Kumanayaka Alex Otto Mit Chauhan Vadim Belinschi Noreen Mirza Mohammad Nabil Rayad Addi Suleiman Department of Internal Medicine New York Medical College at Saint Michael’s Medical Center Newark NJ USA Department of Medical Education University of New England College of Osteopathic Medicine Portland Maine USA

The most common congenital heart defect is the bicuspid aortic valve (BAV) occurring with fusion of one of the three-valve commissures. A rarer valvular phenomenon is the unicuspid aortic valve (UAV) which has an estimated prevalence of 0.02% in the adult population. Two morphologic subtypes of UAV exist: the earlier presenting, acommissural UAV and the later appearing unicommissural. To better characterize and manage patients with UAVs, a systematic review of this rare phenomenon was performed. The objective of our study was to conduct a systematic review of adequate case studies involving UAV patients to describe patient characteristics, clinical presentation, management, and common sequela featured there within. The mean age of presentation in this review was 35.6 years. When diagnosing UAV, TEE was the most utilized diagnostic modality (65%) followed by intraoperative diagnosis (17%) discovered with valve repair or incidentally during another procedure. TTE was utilized to diagnose only 7% of the cases reviewed likely owing to the difficulty and inaccuracies in identifying UAV with this method that were previously established by multiple series. Interventional options for UAV are balloon versus surgical valvotomy, aortic valve replacement, Ross procedure and aortic root replacement. They are performed on an individual basis and all cases ultimately require aortic valve replacement (AVR) or Ross procedure (to avoid anticoagulation). Aortic root replacement is additionally required if aortic root/ascending aorta diameter exceeds 4.5 cm. In this review, Aortic aneurysm (16%) cases resulted in surgical interventions. There may be a need to implant permanent pacemaker (in 3% - 8%) during AVR when calcified UAV has calcification extending into interventricular septum.

关键词： Unicuspid Aortic Valve Bicuspid Aortic Valve heart defects Unicommisural Valve

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Adult Congenital heart Disease

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1998年

作者： Gary D., M.D. Webb,Michael S. Connelly,Jane Somerville,Gary D. Webb

来源：畅想之星图书光盘版（南... 评论

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