scholarly journals Common Arterial Trunk Associated with Functionally Univentricular Heart: Anatomical Study and Review of the Literature

2021 ◽  
Vol 8 (12) ◽  
pp. 175
Author(s):  
Sami Chatila ◽  
Lucile Houyel ◽  
Manon Hily ◽  
Damien Bonnet
Keyword(s):  
Left Ventricle ◽  
Heart Defects ◽  
Rare Condition ◽  
Anatomical Study ◽  
Atrioventricular Septal Defect ◽  
Univentricular Heart ◽  
Review Of The Literature ◽  
Atrioventricular Canal ◽  
Common Arterial Trunk ◽  
Arterial Trunk

Common arterial trunk (CAT) is a rare congenital heart disease that is commonly included into the spectrum of conotruncal heart defects. CAT is rarely associated with functionally univentricular hearts, and only few cases have been described so far. Here, we describe the anatomical characteristics of CAT associated with a univentricular heart diagnosed in children and fetuses referred to our institution, and we completed the anatomical description of this rare condition through an extensive review of the literature. The complete cohort ultimately gathered 32 cases described in the literature completed by seven cases from our unit (seven fetuses and one child). Four types of univentricular hearts associated with CAT were observed: tricuspid atresia or hypoplastic right ventricle in 16 cases, mitral atresia or hypoplastic left ventricle in 12 cases, double-inlet left ventricle in 2 cases, and unbalanced atrioventricular septal defect in 9 cases. Our study questions the diagnosis of CAT as the exclusive consequence of an anomaly of the wedging process, following the convergence between the embryonic atrioventricular canal and the common outflow tract. We confirm that some forms of CAT can be considered to be due to an arrest of cardiac development at the stages preceding the convergence.

An anatomical study of 110 cases with deficiency of the aorticopulmonary septum with emphasis on the role of the arterial duct

1992 ◽  
Vol 2 (4) ◽  
pp. 342-352 ◽  
Author(s):  
Leon M. Gerlis ◽  
Candida C. d'A. MacGregor ◽  
Siew Yen Ho
Keyword(s):  
Aortic Arch ◽  
Anatomical Study ◽  
Left Pulmonary Artery ◽  
Clinical Situation ◽  
Interrupted Aortic Arch ◽  
Developmental Defect ◽  
Common Arterial Trunk ◽  
Arterial Duct ◽  
Arterial Trunk

AbstractTo assess the prevalence and role of the arterial duct in hearts with incomplete development of the aorticopulmonary septum, 110 autopsy specimens, comprising 100 examples of common arterial trunk and 10 cases with aorticopulmonary window, were studied. In addition to intracardiac malformations, these specimens were analyzed to determine the side of the aortic arch, the presence and location of any interruption of the arch, the presence of any anomaly of origin and course of the subclavian arteries, and the status of the arterial duct. The arterial duct was present in 33 cases, absent in 63 cases and undetermined in four cases with common arterial trunk. The duct provided an essential pathway for flow in the 20 cases with interrupted aortic arch, and in two cases with interruption of the proximal portion of the left pulmonary artery. The presence of the duct in 11 cases, in functional terms, was not essential. In the 10 hearts with aorticopulmonary window, the duct was present in eight. It was an obligatory part of the circulatory pathways in three cases where the aortic arch was interrupted. The prevalence of non-obligatory ducts was 71% in hearts with aorticopulmonary window compared to 15% in hearts with common arterial trunk. The prevalence of the duct in cases with aorticopulmonary window suggests this lesion is a later developmental defect. The functional role of an arterial duct in these hearts should be properly assessed in the clinical situation.

Common Arterial Trunk With Atrioventricular Septal Defect: New Observations Pertinent to Repair

2009 ◽  
Vol 87 (5) ◽  
pp. 1495-1499 ◽  
Author(s):  
Iki Adachi ◽  
Siew Yen Ho ◽  
Margot M. Bartelings ◽  
Karen P. McCarthy ◽  
Anna Seale ◽  
...  
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Common Arterial Trunk ◽  
Arterial Trunk

Surgical repair of atrioventricular septal defect with common atrioventricular junction when associated with tetralogy of Fallot or double outlet right ventricle

2006 ◽  
Vol 16 (S3) ◽  
pp. 59-64 ◽  
Author(s):  
Christo I. Tchervenkov ◽  
Samantha Hill ◽  
Danny Del Duca ◽  
Stephen Korkola
Keyword(s):  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Surgical Techniques ◽  
Atrioventricular Septal Defect ◽  
Double Outlet Right Ventricle ◽  
Biventricular Repair ◽  
Atrioventricular Junction ◽  
Common Arterial Trunk ◽  
Arterial Trunk

The association of atrioventricular septal defect with common atrioventricular junction and malformations of the ventricular outflow tracts presents a significant challenge for the surgeon. In the most common of these, the association with tetralogy of Fallot, several surgical techniques have been described, and shown to deliver excellent results.1–10On the other hand, in the setting of more extreme malformations, such as double-outlet right ventricle, discordant ventriculo-arterial connections, or common arterial trunk, albeit rare lesions, the combination presents a more formidable surgical challenge, as evidenced by the few reports of successful repair of these lesions. This challenge is both physiological, when dealing with a very sick neonate or infant, as well as anatomical in terms of the complexity of the malformation and the ability to achieve a successful biventricular repair. Our goal in this review is to discuss the surgical treatment in the setting of tetralogy of Fallot and double outlet right ventricle, with emphasis on biventricular repair.

scholarly journals The transcription factor Sox7 modulates endocardiac cushion formation contributed to atrioventricular septal defect through Wnt4/Bmp2 signaling

2021 ◽  
Vol 12 (4) ◽  
Author(s):  
Nanchao Hong ◽  
Erge Zhang ◽  
Huilin Xie ◽  
Lihui Jin ◽  
Qi Zhang ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Septal Defect ◽  
De Novo ◽  
Heart Defects ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Canal ◽  
Mesenchymal Transition ◽  
Atrioventricular Cushions

AbstractCardiac septum malformations account for the largest proportion in congenital heart defects. The transcription factor Sox7 has critical functions in the vascular development and angiogenesis. It is unclear whether Sox7 also contributes to cardiac septation development. We identified a de novo 8p23.1 deletion with Sox7 haploinsufficiency in an atrioventricular septal defect (AVSD) patient using whole exome sequencing in 100 AVSD patients. Then, multiple Sox7 conditional loss-of-function mice models were generated to explore the role of Sox7 in atrioventricular cushion development. Sox7 deficiency mice embryos exhibited partial AVSD and impaired endothelial to mesenchymal transition (EndMT). Transcriptome analysis revealed BMP signaling pathway was significantly downregulated in Sox7 deficiency atrioventricular cushions. Mechanistically, Sox7 deficiency reduced the expressions of Bmp2 in atrioventricular canal myocardium and Wnt4 in endocardium, and Sox7 binds to Wnt4 and Bmp2 directly. Furthermore, WNT4 or BMP2 protein could partially rescue the impaired EndMT process caused by Sox7 deficiency, and inhibition of BMP2 by Noggin could attenuate the effect of WNT4 protein. In summary, our findings identify Sox7 as a novel AVSD pathogenic candidate gene, and it can regulate the EndMT involved in atrioventricular cushion morphogenesis through Wnt4–Bmp2 signaling. This study contributes new strategies to the diagnosis and treatment of congenital heart defects.

Common arterial trunk associated with atrioventricular septal defect

1999 ◽  
Vol 9 (6) ◽  
pp. 617-620 ◽  
Author(s):  
Edmar Atik ◽  
Andressa Mussi Soares ◽  
Vera Demarchi Aiello
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Unusual Feature ◽  
Cross Sectional ◽  
Common Arterial Trunk ◽  
Arterial Trunk ◽  
Angiographic Findings

AbstractDescribed are the clinical, cross-sectional echocardiographic, electrocardiographic and angiographic findings, together with the results at autopsy, in a 5-month old infant with a common arterial trunk associated with an atrioventricular septal defect. As far as is known, this is only the 13th such case to be reported in the literature. A particularly unusual feature of this case was an intact atrial component of the atrioventricular septal defect.

The evolution of a hypoplastic left ventricle in an infant with an atrioventricular septal defect detected antenatally

1995 ◽  
Vol 5 (4) ◽  
pp. 360-362
Author(s):  
Robert William Michael Yates ◽  
Gurleen Kaur Sharland ◽  
Shakeel Ahmed Qureshi
Keyword(s):  
Left Ventricle ◽  
Left Atrial ◽  
Congenital Heart ◽  
Septal Defect ◽  
Fetal Echocardiography ◽  
Heart Defects ◽  
Atrioventricular Septal Defect ◽  
Cardiac Anatomy ◽  
Hypoplastic Left Ventricle

SummaryComplex congenital heart defects are accurately detectable antenatally using fetal echocardiography. In continuing pregnancies with fetal cardiac abnormalities, regular follow-up with advancing gestation is essential. This report documents the changes that occurred in the fetal cardiac anatomy in a fetus detected during antenatal scanning to have an atrioventricular septal defect with isomerism of the left atrial appendages.

The developmental components of the ventricles: their significance in congenital cardiac malformations

1991 ◽  
Vol 1 (2) ◽  
pp. 123-128 ◽  
Author(s):  
Maria V. de la Cruz ◽  
Concepción Sánchez Gómez ◽  
Raul Cayre
Keyword(s):  
Atrioventricular Septal Defect ◽  
Straight Tube ◽  
Common Arterial Trunk ◽  
New Information ◽  
Arterial Trunk ◽  
In Vivo Labeling ◽  
Chick Embryo Heart ◽  
Embryo Heart ◽  
The Right

SummaryOur experiments using in vivo labeling techniques in the chick embryo heart have demonstrated that the straight tube heart is constituted exclusively by the primordium of the trabeculated portion of the right and left ventricles. There are, therefore, no primitive cardiac cavities. Furthermore, in the stage of looping, two new ventricular components appear, namely, the inlet and the outlet. This new information on the normal development of the heart is important in order to understand both the anatomy of the normal heart and the development of congenital malformations. Due to the fact that the straight tube heart is constituted exclusively by the primordiums of the ventricular trabeculated portions the anatomic identification of the ventricles should be made on the basis of their apical trabeculated regions. The appearance of the components of the inlet and the outlet during the process of looping is the reason for the similarity in the congenital pathology of both regions, namely, a common arterial trunk and atrioventricular septal defect with common orifice. Because the trabeculated portions of the ventricles are the oldest developmental components, they form the basis on which malformations of the inlet or the outlet, or both, are superimposed.

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