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  • Open Access

    ARTICLE

    Factors Affecting the Genetic Diagnostic Rate in Congenital Heart Disease

    Jun Sung Park1, Go Hun Seo2, Yunha Choi1, Soojin Hwang1, Minji Kang3, Hyo-Sang Do3, Young-Hwue Kim4, Jeong Jin Yu4, Ellen Ai-Rhan Kim5, Euiseok Jung5, Byong Sop Lee5, Jae Suk Baek4, Beom Hee Lee1,6,*

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.021580

    Abstract 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 study investigated the diagnostic yield of whole-exome sequencing (WES) in patients with sporadic syndromic CHD and the phenotypic factors affecting the genetic diagnostic rate. Methods: Sixty-four patients with sporadic syndromic CHD aged <18 years underwent WES between May 2018 and December 2020 in a single tertiary center, and the association between genetic testing data and extracardiac phenotypes was analyzed. Results: Extracardiac phenotypes were measured as 3.66 ± 3.05 (standard deviation, interquartile range: 2–5)… More >

  • Open Access

    ARTICLE

    Preoperative Feeding in Single Ventricle Neonates is Predictive of Shorter Time to Goal Feed

    Alyssia Venna1, Kathleen Reid2, Sarah Davis2, Jiaxiang Gai3, Yves d’Udekem1, Sarah Clauss2,*

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.021571

    Abstract Background: Patients with single ventricle anatomy are at increased risk of growth failure and malnutrition. Amongst cardiac centers, there is little standardization of feeding practices in this complex population. We hypothesized that initiation of our center’s preoperative feeding protocol would result in decreased gastrostomy tube (G-tube) use, decreased length of stay and would not result in increased Necrotizing Enterocolitis (NEC) rates. Methods: A single institution review of 52 patients who had undergone stage I single ventricle palliative repair was performed. Patient diagnoses were hypoplastic left heart syndrome (39%), atrioventricular canal (15%), and other (46%). Postoperative parameters such as time to… More >

  • Open Access

    ARTICLE

    Minimally Invasive Surgical Technique in Double Aortic Arch with Distal Atretic Left-Side in Infant: From a Single-Surgeon Clinical Experience

    Qi Liu, Shoujun Li, Zhongdong Hua*

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.020496

    Abstract Background: Double aortic arch (DAA) with distal left-sided aortic arch atresia (LAAA) can form complete vascular ring by ligamentum connection. We aimed to introduce an uncommon DAA-LAAA diagnosis and treatment by the minimally invasive surgical technique (MIST). Methods: We retrospectively reviewed 7 cases of DAA-LAAA that were treated from January 2017 to July 2021. All infant patients underwent surgical repair by minimally invasive surgical technique. Mean follow-up was 14.43 months (range, 5–21 months). Results: There were seven patients with DAA-LAAA, including six males and one female. Median age was 19.29 months (range, 9.0–29.0 months). Median weight was 11.30 kg (range,… More >

  • Open Access

    ARTICLE

    20 Year Follow-Up after the Fontan Operation in a Population with Hypoplastic Left Heart Syndrome

    Eleni P. Asimacopoulos*, Steven J. Staffa, Peter C. Laussen, Kirsten C. Odegard

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.020334

    Abstract Background: Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population. We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome (HLHS) from Stage I through Fontan completion. In this report, we examine their clinical status, anticoagulation and incidence of thromboembolic events up to 20 years post Fontan. Methods: A retrospective chart review was conducted for twenty (20) surviving patients, from 1998 through December 2020. Patients who underwent orthotopic heart transplantation (OTx) were followed until their transplant. Patients who were found in the original study to have a factor… More >

  • Open Access

    ARTICLE

    Prevalence of Congenital Heart Disease at Extremely High Altitudes in Tibet and the Establishment of a New Free Treatment Model

    Shuting Chang1,2, Guanzhao Zhang2,3, Nengjun Sun2, Xinpeng Yuan4, Qingting Wang5, Lin Zhu6, Feiyue Zhang7, Yu Xiu4, Yang Dong8, Yonghong Chen9, Zhanpeng Zhao10, Xiao Liu4, Qiang Shao11, Xiaofeng Xu12, Anshun Wang13, Mengjiao Li14, Bo Li2,*

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.018657

    Abstract Background: This study aimed to illustrate the prevalence of CHD by screening children in extremely high-altitude areas (over 4000 m to even 5000 m above sea level) and explore an aid model for early diagnosis and treatment for the Tibetan population. Methods: A total of 2242 students from different schools in Ngamring County, Xigaze city, Tibet from September 2019 to September 2020 were selected for screening. The students were examined through the inquiry of their current medical history and family history, cardiac auscultation and a physical examination, in order to screen out the suspected cases of CHD, and then the… More >

  • Open Access

    CASE REPORT

    A Rare Case of Concordant Atrioventricular Connection to L-Looped Ventricles in Situs Solitus: 4-Dimensional Magnetic Resonance Imaging and 3D Printing

    Gregory Perens1,*, Takegawa Yoshida2, J. Paul Finn2

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.021233

    Abstract An infant male presented with the rare anatomy consisting of situs solitus, concordant atrioventricular connections to L-looped ventricles, double outlet right ventricle (DORV), and hypoplastic aortic arch. 6 months after neonatal aortic arch repair, the morphologic right ventricle function deteriorated, and surgical evaluation was undertaken to determine if either biventricular repair with a systemic morphologic left ventricle or right ventricular exclusion was possible. After initial echocardiography, magnetic resonance imaging (MRI) was used to create detailed axial and 4-dimensional (4D) images and 3-dimensional (3D) printed models. The detailed anatomy of this rare, complex case and its use in pre-surgical planning is… More >

  • Open Access

    ARTICLE

    Incidence and Related Risk Factors of Junctional Ectopic Tachycardia in Infants after Cardiac Surgery for Congenital Heart Disease

    Jae Hee Seol1,4,#, Se Yong Jung1,#, Jae Young Choi1, Han Ki Park2, Young Hwan Park2, Nam Kyun Kim1,3,*

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.018436

    Abstract Objective: Junctional ectopic tachycardia is common after cardiac surgery for congenital heart disease. However, its incidence and related risk factors in infants after cardiac surgery are not well known. The objective of this study was to determine the overall incidence and related risk factors for junctional ectopic tachycardia in neonates and infants. Methods: We enrolled a total of 271 patients aged <1 year who underwent open cardiac surgery at Severance Cardiovascular Hospital from January 2018 to December 2020. Exclusion criteria were immediate postoperative mortality, other arrhythmias detected in the perioperative period, and prematurity. Result: The overall incidence of junctional ectopic… More >

  • Open Access

    ARTICLE

    Comparison of Intracardiac and Extracardiac Malformations Associated with Single Atrium, Single Ventricle and Single Atrium-Single Ventricle Using DualSource Computed Tomography

    Tong Pang#, Li Jiang#, Yi Zhang, Mengxi Yang, Jin Wang, Yuan Li*, Zhigang Yang*

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.020401

    Abstract Background: To evaluate the qualitative and quantitative differences between intracardiac and extracardiac vascular malformations in patients with a single atrium (SA), single ventricle (SV) and single atrium-single ventricle (SA-SV) using dual-source CT (DSCT), and to compare the diagnostic performances of DSCT and transthoracic echocardiography (TTE). Methods: This retrospective study included 24 SA, 75 SV and 24 SA-SV patients who underwent both DSCT and TTE before surgery. The diagnostic values of DSCT and TTE for intracardiac and extracardiac malformations were compared according to the surgical results. The diameters of the major artery and vein were measured and calculated based on DSCT… More >

  • Open Access

    ARTICLE

    Perpulmonary Device Closure of Patent Ductus Arteriosus with Minimum Diameter More Than 4 mm in Infants

    Shibin Sun1,#, Geoffrey J. Changwe1,2,#, Zeeshan Farhaj1, Hongxin Li1,*, Yuekun Sun1, Zhongzheng Kong1

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.019943

    Abstract Background: Closure of large patent ductus arteriosus (PDA) in older children has been accomplished using surgical and percutaneous techniques with remarkable outcomes. However, outcomes amongst infants have been variable with several drawbacks. Here we describe a novel minimally invasive technique, a product of mini-thoracotomy and traditional percutaneous technique skills, accomplished exclusively under echocardiography guidance. Methods: Symptomatic infants with a significant left-to-right shunt from PDA measuring more than 4 mm were selected. The symptoms were varying degrees of tachypnea, tachycardia, heart failure, failure to thrive, recurrent respiratory tract infections, or intensive care unit treatment for a longer duration. Through a left… More >

  • Open Access

    ARTICLE

    Adults with Congenital Heart Disease during the COVID-19 Era: One-Year Tertiary Center Experience

    Fatma A. Taha1,2,*, Osama Amoudi1, Fareed Alnozha1, Reda Abuelatta1

    Congenital Heart Disease, Vol., , DOI:10.32604/chd.2022.020174

    Abstract Background: Adult patients with congenital heart disease (ACHD) might be at high risk of Coronavirus disease- 2019 (COVID-19). This study aimed to report on a one-year tertiary center experience regards COVID-19 infection in ACHD patients. Methods: This is a one-year (March-2020 to March-2021) tertiary-center retrospective study that enrolled all ACHD patients; COVID-19 positive patients’ medical records, and management were reported. Results: We recorded 542 patients, 205 (37.8%) COVID-19-positive, and 337 (62.2%) COVID-19-negative patients. Palliated single ventricle and Eisenmenger syndrome patients were more vulnerable to COVID-19 infection (P < 0.05*). Cardiovascular COVID-19 complications were arrhythmias in 47 (22.9%) patients, heart failure… More >

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