Open Access
ARTICLE
Irene Martin de Miguel1, C. Charles Jain1, Alexander C. Egbe1, Jason H. Anderson2, Heidi M. Connolly1, William R. Miranda1,*
Congenital Heart Disease, Vol.17, No.6, pp. 605-615, 2022, DOI:10.32604/chd.2022.023969
Abstract Background: Coarctation of the aorta (CoA) is associated with a generalized arteriopathy and long-term complications despite repair. Data on invasive exercise hemodynamics in this population are lacking. Accordingly, we reviewed adults with CoA undergoing exercise catheterization to assess 1. hemodynamic profile; 2. feasibility for assessment of CoA severity. Methods: Twenty patients undergoing exercise cardiac catheterization (12 arm adduction and 8 supine cycle ergometry) at a quaternary care center between 2004 and 2021 were identified. Resting and exercise hemodynamic data were abstracted from the procedure logs. Results: Mean age was 43.6 ± 12.0 years. Eleven patients (55%) had resting pulmonary arterial wedge pressure… More >
Open Access
REVIEW
Emma Payne1,2, Thomas Wilson2,3, Marjan Haghighi1,4, Zoe McCallum2,3, Yves d’Udekem5, Julian Ayer1,4,*
Congenital Heart Disease, Vol.17, No.6, pp. 617-639, 2022, DOI:10.32604/chd.2022.024775
Abstract Background: Patients born with a single ventricle circulation commonly experience growth failure in early life, which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectory and clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, a systematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patients with clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluated by Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies. Results: Of 527 studies that underwent title… More >
Open Access
CASE REPORT
Igor Mokryk1,2,*, Vitaly Demyanchuk1,2, Volodymyr Vashkeba2, Ilya Nechay1, Borys Todurov1,2
Congenital Heart Disease, Vol.17, No.6, pp. 641-646, 2022, DOI:10.32604/chd.2022.025096
Abstract Congenital heart disease (CHD) is one of the risk factors for developing infective endocarditis (IE). Right-sided IE occurs in 5%–10% of endocarditis cases, and pulmonary valve (PV) is involved in less than 2% of such patients. Literature data are few, and optimal treatment methods, indications for surgery, and types of operative techniques are still under debate. We present an adult patient with a rare combination of the ventricular septal defect (VSD) and PV IE who underwent surgical treatment. Neocuspidization with autologous pericardium was utilized for the reconstruction of his PV. We discuss details of this novel surgical technique. More >
Graphic Abstract
Open Access
CASE REPORT
Aya Miyazaki1,2,*, Hideki Uemura2, Yasuyo Takeuchi3, Junya Tomida4, Yasuo Ono1, Yoshifumi Fujimoto1, Norie Mitsushita1, Akio Ikai1
Congenital Heart Disease, Vol.17, No.6, pp. 647-652, 2022, DOI:10.32604/chd.2022.021837
Abstract Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart disease with unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sinus node dysfunction suffered from heart failure symptoms with preserved left ventricular function. Simply changing the pacemaker’s lower rate from 60 to 75 bpm, New York Heart Association classification improved from III to II, and hemodynamic parameters drastically improved. We regarded this case as informative. Appropriate heart rate could be higher in congenital patients with failing right and non-failing left ventricles than in adults with malfunctioning LV. More >
Graphic Abstract
Open Access
ARTICLE
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.17, No.6, pp. 653-673, 2022, 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 >
Graphic Abstract
Open Access
ARTICLE
Rui Jiang1,3,*, Kaisheng Lai2, Jianping Xu1, Xiang Feng1, Shaoye Wang1, Xiaojian Wang3, Zhe Liu2
Congenital Heart Disease, Vol.17, No.6, pp. 675-686, 2022, DOI:10.32604/chd.2022.021626
Abstract Background: The etiology of pulmonary arterial hypertension associated with congenital heart disease (PAHCHD) is complicated and the phenotype is heterogeneous. Genetic defects of NOTCH3 were associated with
cerebral disease and pulmonary hypertension. However, the relationship between NOTCH3 mutations and the
clinical phenotype has not been reported in CHD-PAH. Methods: We eventually enrolled 142 PAH-CHD patients
from Fuwai Hospital. Whole exome sequencing (WES) was performed to screen the rare deleterious variants of
NOTCH3 gene. Results: This PAH-CHD cohort included 43 (30.3%) men and 99 (69.7%) women with the mean
age 29.8 ± 10.9 years old. The pathogenic or likely pathogenic mutations… More >
Open Access
ARTICLE
Yuan Hu1, Xiaohui Yang2, Jie Dong3, Peng Huang2, Jinwen Luo2, Guangxian Yang2, James D. St. Louis4, Xicheng Deng2,*
Congenital Heart Disease, Vol.17, No.6, pp. 687-695, 2022, DOI:10.32604/chd.2022.024333
Abstract Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The management varies 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 who underwent congenital heart surgery from January 2018 to December 2021 at our institution to identify potential interventricular septal hematoma cases. Relevant data of the patients identified were collected. Focus was put on the presentation, management, outcomes according to patent medical charts and serial echocardiographic report data. Results: In total, there were 5 patients included. The mean age and… More >
Graphic Abstract
Open Access
ARTICLE
Huan Zhou1,#, Jin Kang2,#, Jun Gao2,*, Xiaoyuan Feng1, Li Zhou2, Xia Xiao2, Zhengliang Meng2, Chengwen Guo2
Congenital Heart Disease, Vol.17, No.6, pp. 697-707, 2022, DOI:10.32604/chd.2022.022648
Abstract Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume and
ejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to use
RT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septal
defect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study
18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the same
age (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE.
The measurements included: left and right ventricular volume indexed to… More >
Graphic Abstract
Open Access
ARTICLE
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.17, No.6, pp. 709-716, 2022, 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 >
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