Open Access

ARTICLE

Irene Martin de Miguel¹, C. Charles Jain¹, Alexander C. Egbe¹, Jason H. Anderson², Heidi M. Connolly¹, William R. Miranda^1,*
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 >

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REVIEW

Emma Payne^1,2, Thomas Wilson^2,3, Marjan Haghighi^1,4, Zoe McCallum^2,3, Yves d’Udekem⁵, Julian Ayer^1,4,*
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 >

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CASE REPORT

Igor Mokryk^1,2,*, Vitaly Demyanchuk^1,2, Volodymyr Vashkeba², Ilya Nechay¹, Borys Todurov^1,2
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 >

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CASE REPORT

Aya Miyazaki^1,2,*, 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 >

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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 Lee^1,6,*
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 >

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ARTICLE

Rui Jiang^1,3,*, 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 >

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ARTICLE

Yuan Hu¹, Xiaohui Yang², Jie Dong³, Peng Huang², Jinwen Luo², Guangxian Yang², James D. St. Louis⁴, Xicheng Deng^2,*
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 >

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ARTICLE

Huan Zhou^1,#, Jin Kang^2,#, Jun Gao^2,*, 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 >

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ARTICLE

Shuting Chang^1,2, Guanzhao Zhang^2,3, 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 Li^2,*
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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