Subaortic Stenosis in Adult Patients with Atrioventricular Septal Defect
CONCLUSIONS: Surgical correction of subaortic obstruction in adult patients with AVSD can be accomplished with a low morbidity and mortality. Subaortic stenosis can appear late after the initial repair of AVSD, and they remain at risk for recurrence after resection.PMID:35987344 | DOI:10.1016/j.athoracsur.2022.08.011 (Source: The Annals of Thoracic Surgery)
Source: The Annals of Thoracic Surgery - August 20, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Yalile Perez Joseph A Dearani William R Miranda Elizabeth H Stephens Source Type: research

Subaortic Stenosis in Adult Patients with Atrioventricular Septal Defect
CONCLUSIONS: Surgical correction of subaortic obstruction in adult patients with AVSD can be accomplished with a low morbidity and mortality. Subaortic stenosis can appear late after the initial repair of AVSD, and they remain at risk for recurrence after resection.PMID:35987344 | DOI:10.1016/j.athoracsur.2022.08.011 (Source: The Annals of Thoracic Surgery)
Source: The Annals of Thoracic Surgery - August 20, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Yalile Perez Joseph A Dearani William R Miranda Elizabeth H Stephens Source Type: research

European epidemiological patterns of cannabis- and substance-related congenital cardiovascular anomalies: geospatiotemporal and causal inferential study
Environ Epigenet. 2022 Jul 5;8(1):dvac015. doi: 10.1093/eep/dvac015. eCollection 2022.ABSTRACTAs prenatal and community cannabis exposures have recently been linked with congenital heart disease (CHD), it was of interest to explore these associations in Europe in a causal framework and space-time context. Congenital anomaly data from Eurocat, drug-use data from the European Monitoring Centre for Drugs and Drug Addiction, and income from the World Bank. Countries with rising daily cannabis use had in general higher congenital anomaly rates over time than those without (time: status interaction: β-Est. = 0.0267, P = 0.0059)...
Source: Cancer Control - August 15, 2022 Category: Cancer & Oncology Authors: Albert Stuart Reece Gary Kenneth Hulse Source Type: research

Modified single-patch repair for atrioventricular septal defects results in good functional outcomes in the absence of deep ventricular septal defects
We compared two-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects (CAVSD) and evaluated their impact on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes. (Source: The Journal of Thoracic and Cardiovascular Surgery)
Source: The Journal of Thoracic and Cardiovascular Surgery - August 12, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Yasuyuki Kobayashi, Shingo Kasahara, Shunji Sano, Yasuhiro Kotani Source Type: research

High-risk genes involved in common septal defects of congenital heart disease
This study supports previously published studies that suggested GATA4 and MYH6 as the high-risk genes responsible for septal defects. Thus, this study contributes to a better understanding of the genes involved in heart development by identifying the high-risk genes and interacting proteins in the pathway.PMID:35863714 | DOI:10.1016/j.gene.2022.146745 (Source: Gene)
Source: Gene - July 21, 2022 Category: Genetics & Stem Cells Authors: S Chaithra Swati Agarwala N B Ramachandra Source Type: research

High-risk genes involved in common septal defects of congenital heart disease
This study supports previously published studies that suggested GATA4 and MYH6 as the high-risk genes responsible for septal defects. Thus, this study contributes to a better understanding of the genes involved in heart development by identifying the high-risk genes and interacting proteins in the pathway.PMID:35863714 | DOI:10.1016/j.gene.2022.146745 (Source: Gene)
Source: Gene - July 21, 2022 Category: Genetics & Stem Cells Authors: S Chaithra Swati Agarwala N B Ramachandra Source Type: research

Commentary: Scoops and Goose Necks: Long Term Challenges Following Atrioventricular Septal Defect Repair
In the present study, Ramgren and colleagues provide insights from their 25-year experience with reoperations following repair of atrioventricular septal defects (AVSD). Reoperations were required in approximately one-tenth of the study cohort, the majority of which involved the left atrioventricular valve (AVV), followed by the left ventricular outflow tract (LVOT). In-hospital mortality following reoperation was (Source: Seminars in Thoracic and Cardiovascular Surgery)
Source: Seminars in Thoracic and Cardiovascular Surgery - July 14, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Aditya Sengupta, Meena Nathan Tags: CONGENITAL – Commentary Source Type: research

Modified atrioventricular valve replacement in children
This report describes a technique that uses a trapezoid-shaped patch to separate the implanted valve suture-line from the AVN, which allows surgeons to implant larger valves while simultaneously preventing injury to the AVN. (Source: Journal of Cardiac Surgery)
Source: Journal of Cardiac Surgery - July 13, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Fumiya Yoneyama, Michiaki Imamura Tags: HOW DO I DO IT Source Type: research

Author Reply to Commentary: Further elaboration of the “V-shaped double-layer patch technique”
I feel greatly honored to read the commentary by Dr Carl L. Backer.1 I think it is necessary to further elaborate on several situations mentioned by Dr Carl L. Backer. So far, there have been no accurate epidemiologic statistics of complete atrioventricular septal defect (CAVSD) in China. According to some published literature from other centers in China,2-4 surgical age in China is much greater than that in European and American countries, and there are fewer patients with Down syndrome, which may be related to our understanding and development of the treatment of CAVSD. (Source: The Journal of Thoracic and Cardiovascular Surgery)
Source: The Journal of Thoracic and Cardiovascular Surgery - July 5, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Shanquan Sun, Yangxue Sun Tags: Author Reply to Commentary Source Type: research

Reoperations after repair for atrioventricular septal defects: > 25 years experience at a single center
Our aim was to evaluate the total burden of reoperations after previous repair for atrioventricular septal defects, including long-term survival and identify risk factors for reoperation. All patients with surgical correction for AVSD 1993 – 2020 underwent a follow-up in October 2020. Clinical data were obtained by retrospective review and evaluated with Kaplan-Meier and competing risk analysis. Of 477 patients who underwent initial repair, 53 patients (11.1%) underwent a total of 82 reoperations. (Source: Seminars in Thoracic and Cardiovascular Surgery)
Source: Seminars in Thoracic and Cardiovascular Surgery - June 20, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Jens Johansson Ramgren, Shahab Nozohoor, Igor Zindovic, Ronny Gustafsson, Nina Hakacova, Johan Sj ögren Tags: CONGENITAL – Original Submission Source Type: research

Cohort study of intervened functionally univentricular heart in England and Wales (2000-2018)
Conclusion Although treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD. (Source: Heart)
Source: Heart - June 10, 2022 Category: Cardiology Authors: Hadjicosta, E., Franklin, R., Seale, A., Stumper, O., Tsang, V., Anderson, D. R., Pagel, C., Crowe, S., Espuny Pujol, F., Ridout, D., Brown, K. L. Tags: Open access Congenital heart disease Source Type: research

Repair of double orifice mitral valve with an atrioventricular septal defect in a girl with Ellis –Van Creveld syndrome
We describe the case of a 7-year-old girl with Ellis–van Creveld S yndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well. The correct diagnosis of this disorder in early life is essential in the overall prognosis of the syndrome. Clinical follow-up at regular intervals is very important in these patients to institute proper managements and prevent further complications. (Source: Clinical Case Reports)
Source: Clinical Case Reports - May 19, 2022 Category: General Medicine Authors: Alwaleed Al ‐Dairy, Samir Srour, Alaa Chaker, Fatimah Alzahraa Alsyed Hasan Tags: CASE REPORT Source Type: research

The V-shaped Double-layer Patch Technique for Complete Atrioventricular Septal Defect: A Novel Surgical Technique
Several surgical techniques for repair of a complete atrioventricular septal defect (CAVSD) have been developed. However, the postoperative complications with these methods may lead to reoperation during follow-up. The aim of this report is to share our experience with a modified surgical technique for CAVSD that has anatomic advantages postoperatively and could reduce the reoperation rate. (Source: The Journal of Thoracic and Cardiovascular Surgery)
Source: The Journal of Thoracic and Cardiovascular Surgery - May 17, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Shanquan Sun, Yangxue Sun, Jingsi Huang, Peng Zou, Jiao Rao, Weibin Xu, Qin Liu Source Type: research

Atrioventricular septal defect in Fontan circulation: right ventricular dominance, not valve surgery, adversely affects survival.
The impact of ventricular dominance and prior atrioventricular valve (AVV) surgery on patients outcomes after Fontan operation remains unclear. We sought to determine the impact of ventricular dominance and prior AVV surgery on transplantation-free survival and long-term AVV competency in patients with AVSD and Fontan circulation. (Source: The Journal of Thoracic and Cardiovascular Surgery)
Source: The Journal of Thoracic and Cardiovascular Surgery - April 20, 2022 Category: Cardiovascular & Thoracic Surgery Authors: Gregory King, Edward Buratto, Rachael Cordina, Ajay Iyengar, Leeanne Grigg, Andrew Kelly, Andrew Bullock, Julian Ayer, Nelson Alphonso, Yves d ’Udekem, Igor E. Konstantinov Source Type: research

Phenotypic overlap between cardioacrofacial dysplasia-2 and oral-facial-digital syndrome
Eur J Med Genet. 2022 Apr 16:104512. doi: 10.1016/j.ejmg.2022.104512. Online ahead of print.ABSTRACTOral-facial digital (OFD) syndrome is characterized by abnormalities of the face (hypertelorism and low set-ears), oral cavity (multiple frenula, lingual hamartoma, or lobulated tongue) and extremities (postaxial polydactyly). At least 19 genes have been implicated in the development of OFD syndrome. Herein, we report the case a 13-year-old patient with atrioventricular septal defect, moderate intellectual disability, epilepsy, and features of OFD, including multiple oral frenula, and postaxial polydactyly of the hands and f...
Source: European Journal of Medical Genetics - April 19, 2022 Category: Genetics & Stem Cells Authors: Mamiko Yamada Hisato Suzuki Hiroshi Futagawa Toshiki Takenouchi Fuyuki Miya Hiroshi Yoshihashi Kenjiro Kosaki Source Type: research