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 > Graphic Abstract

Vitaliy Suvorov^1,*, Vladimir Zaitcev¹, Karolina Andrzejczyk²

Congenital Heart Disease, Vol.17, No.3, pp. 365-374, 2022, DOI:10.32604/chd.2022.019126

Abstract Background: Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome (HLHS) and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period, especially in severely unstable patients. In our study we compared different surgical techniques of bilateral pulmonary artery banding (PAB) in respect to their success in balancing systemic and pulmonary blood flow. Methods: We included 44 neonates with a HLHS and congenital heart diseases (CHD) with a functional single ventricle underwent a hybrid operation: bilateral PAB and patent ductus arteriosus stenting. The hybrid… More >

Sanam Safi^1,2, Takato Yamasaki^1,3, David J. Glidden⁴, Stephen P. Sanders^1,5, Chrystalle Katte Carreon^1,6,*

Congenital Heart Disease, Vol.17, No.2, pp. 141-146, 2022, DOI:10.32604/chd.2022.019743

Abstract A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome. More >

Taiki Haga^1,*, Tomoyuki Masuyama², Yoshiro Hayashi³, Takahiro Atsumi⁴, Kenzo Ishii⁵, Shinsuke Fujiwara⁶

Congenital Heart Disease, Vol.17, No.1, pp. 31-43, 2022, DOI:10.32604/CHD.2022.017407

Abstract 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 mortality. Methods: We retrospectively analysed data from a Japanese rapid response system registry with 35 participating hospitals. We included consecutive patients with congenital heart disease who required rapid response system activation between January 2014 and March 2018. Logistic regression analyses were performed to examine the associations between 1-month mortality and other patient-specific variables. Results: Among 9,607 patients for whom the rapid response system was activated, only 82 (0.9%) had congenital… More >

Mohammadrafie Khorgami¹, Ali Sadeghpour Tabaei^2,*, Elio Caruso^3,*, Silvia Farruggio³, Negar Omidi⁴, Maryam Moradian¹, Behzad Mohammadpour Ahranjani⁵, Zahra Khajali6 and Rahele Zamani¹

Congenital Heart Disease, Vol.16, No.6, pp. 573-584, 2021, DOI:10.32604/CHD.2021.016271

Abstract Background: Most children in need of cardiac pacemakers remain dependent on the function of the permanent from childhood to adulthood. We sought to evaluate and compare the function between epicardial and endocardial pacemakers in pediatric groups with different conditions. Methods: Between 2012 and 2018, this single-canter study evaluated 44 pediatric patients with indications for epicardial or endocardial pacemakers. Results: The 2 groups, at a median age of 5 (0.1–16) years, were compared concerning the characteristics of the leads used (n = 80: bipolar, unipolar, steroid-eluting, and non–steroid-eluting), survival data, and complications. The reason for pacemaker implantation was congenital complete heart… More >

Jacqueline S. Lee^1,2, Angelica Blais^1,2, Julia Jackson¹, Bhavika J. Patel¹, Lillian Lai⁴, Gary Goldfield^1,3, Renee Sananes⁵, Patricia E. Longmuir^1,2,3,*

Congenital Heart Disease, Vol.16, No.3, pp. 255-267, 2021, DOI:10.32604/CHD.2021.014628

Abstract Background: Pediatric cardiology patients often experience decreased quality of life (QoL) and higher rates of mental illness, particularly with severe disease, but the relationship between them and comparisons across diagnostic groups are limited. This mixed-methods cross-sectional study assessed the association between QoL anxiety and behavior problems among children with structural heart disease, arrhythmia, or other cardiac diagnoses. Methods: Children (6–14 years, n = 76, 50% female) and their parents completed measures of QoL (PedsQL), behavior (BASC-2, subset of 19 children) and anxiety (MASC-2, children 8+ years). Pearson correlations/regression models examined associations between QoL, behavior and anxiety, controlling for age, sex,… More >

Aparna Kulkarni^1,*, Richard Neugebauer², Shelby Kutty³

Congenital Heart Disease, Vol.16, No.3, pp. 299-307, 2021, DOI:10.32604/CHD.2021.014495

Abstract Objective: The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease (AdoCHD) from the Pediatric Health Information System (PHIS) database. Methods: The PHIS database was queried for all AdoCHD admissions aged 12–18 years (1/1/2004–12/31/2013). Major forms of CHD were identified by their International Classification of Diseases, ninth revision codes, further verified based on their secondary diagnosis and/or procedure codes. Patient characteristics, diagnoses, procedures and vital status were assessed. Results: In total, there were 4,267 adolescents admitted to 42 Children’s Hospitals, 58.3% were males, 24.6% single ventricle (SV) patients, 64.1% bi-ventricle… More >

Sotiria C. Apostolopoulou^1,*, Stella Brili², Eftihia Sbarouni³, Dimitris Tousoulis², Konstantinos Toutouzas²

Congenital Heart Disease, Vol.15, No.5, pp. 369-375, 2020, DOI:10.32604/CHD.2020.012092

Abstract Objective: Acquired coronary artery disease, initially thought to rarely affect survivors of congenital heart disease, is increasingly recognized in this population, as these patients grow in age and numbers in the recent era. This study reports our experience with coronary artery disease in adults with congenital heart disease and discusses treatment issues and the existing literature. Methods: Retrospective review of all charts of adults with congenital heart disease and acquired coronary artery disease was performed. Patients’ clinical characteristics, diagnosis, risk factors, noninvasive and invasive imaging and management data were recorded. Results: Coronary artery disease was diagnosed at 35–70 of age… More >

Or Bercovich¹, Tal Tirosh-Wagner², Lior Goldberg¹, Amir Vardi³, David Mishali⁴, Gideon Paret^1,#, Yael Nevo-Caspi^1,*,#

Congenital Heart Disease, Vol.15, No.4, pp. 239-249, 2020, DOI:10.32604/CHD.2020.011576

Abstract Objective: To test the hypothesis that circulating miRNAs-146a, -146b, -155, and -21 reflect the inflammatory state of children following heart surgery, and that they may, therefore, correlate with postoperative parameters. We aimed to quantify miRNAs in blood samples from pediatric patients before and 6, 12, and 24 hours after surgery and to evaluate correlations between the miRNA levels and the postoperative course. Setting: PICU. Patients: Forty-two pediatric patients with CHD who underwent cardiac surgery at Safra Children’s Hospital between 2012–2016. Interventions: none. Outcome Measures: The primary outcomes were the postoperative cardiac complications and the secondary outcomes were the length of… More >

Coralie Katharina Dicks¹, Gerhard-Paul Diller¹, Kristina Wasmer², Paul C. Helm^3,4, Ulrike M. M. Bauer^3,4, Helmut Baumgartner¹, Stefan Orwat^1,#, Alicia Jeanette Fischer^1,#,*

Congenital Heart Disease, Vol.15, No.2, pp. 117-125, 2020, DOI:10.32604/CHD.2020.011520

Abstract Background: Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts. Methods and results: Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75–46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to… More >