Atrioventricular Septal Defect: What Is in a Name?

1988

International Journal of Cardiology, 1986

We examined the cross-sectional echocardiographic findings of 171 patients with atrioventricular septal defects. The echocardiographic findings were confirmed by angiography, surgery and/or autopsy. The echocardiographic findings determined whether the common atrioventricular junction was guarded by a common valve or separate right and left valves. In addition, we were able to judge whether the bridging leaflets were related to the septal structures so as to permit both interatrial and interventricular communications [ 127 cases1 or whether the interatrial communication ("ostium primum atrial septal defect") [43 cases] or an interventricular communication [l case] existed in isolation. Defects existing with a common atrioventricular valve could be further classified as having minimal bridging of the antero-superior leaflet (Rastelli Type A (113 cases]); intermediate bridging (Rastelli Type B 13 cases]); or extreme bridging (Rastelli Type C [ 11 cases]). Of the patients with Down's syndrome, 9 had separate right and left valves while 66 had a common valve, all the latter existing in the setting of minimal bridging of the antero-superior leaflet. In the overall group, there were 9 cases having an unbalanced ventricular mass, 5 with right ventricular dominance and 4 with left dominance. Other associated defects were common. The echocardiographic findings were supplemented by pulsed Doppler examination. Atrioventricular valve insufficiency, when mild, was frequently demonstrated only in the right atrium just above the leaflets of the atrioventricular valve.

Current Cardiology Reports, 2021

Atrioventricular septal defects (AVSD) represent a broad spectrum of congenital anomalies from simple to the most complex heart defects including some distinct types. Clinical presentation and timing of intervention differ by morphological subset and functional anatomy. Herein, we review morphological variations and characteristics that determine appropriate intervention and provide insights into functional anatomy based on detailed three-dimensional (3D) assessment of AVSDs. The understanding of functional morphology of AVSDs has improved significantly with detailed 3D echocardiographic evaluation of the atrioventricular junction and valve morphology. As prenatal detection of AVSDs has increased significantly, it has become the most common fetal cardiac diagnosis enabling antenatal counseling and delivery planning. Advances in diagnosis and perioperative care have resulted in optimal outcomes. The diagnosis and management of AVSDs have improved over the years with enhanced understa...

Pediatric Cardiology, 1982

A rare form of endocardial cushion defect is an atrioventrieular (AV) canal-type ventricular septal defect with cleft mitral valve in the absence of a primum atrial septal defect. In two infants with Down's syndrome, preoperative delineation of this entity was accomplished. Mmode echocardiographic features included presence of two distinct AV valves, attachment of the anterior portion of the auterier leaflet of the mitral valve to the interventricular septum, and normal septal motion. Two-dimensional echocardiography demonstrated the intact atrial septuna, ventricular septal defect, presence of two distinct AV valves, and the cleft mitral valve. Angled (axial) cineangiography was employed to delineate the AV valve morphological characteristics, position of ventricular septal defect, and absence of a primum atrial septal defect. Since the surgical approach to this lesion is different from other forms of endocardial cushion defects and specific problems related to AV valve morphology may be encountered, the preoperative diagnosis of this entity is important.

Heart, 1979

Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scooped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect. 'G. P. P. was a visiting fellow from Ospedale GM Lancisi, Ancona, Italy. 2F. J. M. and R. H. A. are supported by the British Heart Foundation together with the Vandervell Foundation and the Joseph Levy Foundation, respectively.

https://www.ijrrjournal.com/IJRR_Vol.7_Issue.11_Nov2020/Abstract_IJRR0026.html, 2020

Atrial septal defect (ASD) is one of the most common congenital cardiac lesions in adults. While the most common symptoms include fatigue, exertional dyspnea, and palpitations, the presentation of ASD can be protean. In this article, we describe three uncommon presentations of ostium secundum ASD. Patient 1 presented with pericardial tamponade. Patient 2 presented with ventricular tachycardia. Patient 3 had moderately severe mitral regurgitation. These patients had chest radiographs and electrocardiograms typical of secundum ASD, but their presentations were atypical.

Teratology, 2001

Background: Recent advances in clinical, pathological, and genetic aspects of atrioventricular septal defects (AVSD) have set the stage for epidemiologic investigations into possible risk factors. Previous analyses of the total case group of AVSD included complete and partial subtypes without analysis of the subsets. Methods: To address the question of possible morphogenetic heterogeneity of AVSD, the Baltimore-Washington Infant Study data on live-born cases and controls (1981-1989) was reanalyzed for potential environmental and genetic risk-factor associations in complete AVSD (n ϭ 213), with separate comparisons to the atrial (n ϭ 75) and the ventricular (n ϭ 32) forms of partial AVSD. Results: Complete and ventricular forms of AVSD had a similar proportion of isolated cases (12.2% and 15.6%, respectively, without associated extracardiac anomalies) and high rates of Down syndrome, whereas the atrial form of partial AVSD included 55% isolated cases. Trisomy 18 occurred in 22% of infants with the ventricular form, compared with Ͻ2% in the other AVSD groups. Analysis of potential risk factors revealed further distinctions. Complete AVSD as an isolated cardiac defect was strongly associated with maternal diabetes (odds ratio [OR] ϭ 20.6; 95% confidence interval [CI] ϭ5.6-76.4) and also with antitussive use (OR ϭ 8.8; CI ϭ 1.2-48.2); there were no strong associations other than maternal age among Down syndrome infants with this type of heart defect. Isolated cases with the atrial type of partial AVSD were associated with a family history of heart defects (OR ϭ 6.2; CI ϭ 1.4-24.4) and with paternal occupational exposures to ionizing radiation (OR ϭ 5.1; CI ϭ 1.4-27.4), but no risk factors were associated with Down syndrome. There were no significant associations of any risk factors in the numerically small subsets of isolated and Down syndrome cases with the ventricular form of partial AVSD. Conclusions: These results indicate a similar risk profile of complete AVSD and the ventricular type of partial AVSD, with a possible subset of the latter due to trisomy 18. Maternal diabetes constituted a potentially preventable risk factor for the most severe, complete form of AVSD.

World Journal of Cardiovascular Surgery, 2020

In atrioventricular septal defect (AVSD), anatomical lesions usually are an ostium primum atrio-septal defect; a common atrioventricular valve, an inlet ventricular septal defect (VSD). It is most often associated with major chromosomal abnormalities. It is the most common congenital disease in Down syndrome. Our case was an infant with a Down syndrome who was admitted for a dyspnea (stage 3 in New York Heart Association classification) and tachycardia. The diagnosis was made by a transthoracic echocardiography. It showed a rare variety of atrioventricular septal defect without an atrial septal defect. The child was treated successfully with one patch technic and no recurrence was noted.

European Journal of Cardio-Thoracic Surgery, 2009

A wealth of experience has been gained in the management of atrioventricular septal defect (AVSD) since the first complete correction of this malformation in 1955. The success of surgical therapy followed an enhanced understanding of morphology and physiology as well as major improvements in imaging of this congenital heart defect. Therapeutic success in the management of patients with AVSD has been extended to include those with associated lesions such as tetralogy of Fallot, double outlet right ventricle and relative degrees of ventricular hypoplasia. Although operative mortality is low and long-term survival is relatively good, important detrimental residual or AVSD-related complications such as left atrioventricular valve regurgitation, left ventricular outflow tract obstruction still carry significant late morbidity in a proportion of patients. This article reviews our current understanding of the morphology of this defect, aspects of diagnosis and surgical treatment options. #

Biomedical Journal of Scientific & Technical Research, 2018

It is a crescent shaped defect of the atrial septum and is also named as an ostium primum atrial septal defect (ASD). Anatomically, bridging leaflets attach to the ventricular septum with leaving only an interatrial connection. Despite the presence of a single valve annulus, there are two valvular orifices. In partial AV