Echo-morphological correlates in patients with atrioventricular septal defect and common atrioventricular junction

2006 ◽  
Vol 16 (S3) ◽  
pp. 43-51 ◽  
Author(s):  
William T. Mahle ◽  
Girish S. Shirali ◽  
Robert H. Anderson
Keyword(s):  
Septal Defect ◽  
Clinical Presentation ◽  
Three Dimensional ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Canal ◽  
Atrioventricular Junction ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
The Common ◽  
The Right

It is now well recognized that patients fulfilling the diagnostic criterions for the group of hearts usually described as atrioventricular canal malformations, or atrioventricular septal defects, can present with shunting at atrial level, at both atrial and ventricular levels, and on occasion, with shunting only at ventricular level.1,2It is also well recognized that, in most instances, the patients with shunting exclusively at atrial level have separate atrioventricular valvar orifices for the right and left ventricles, this arrangement often described as the “ostium primum” variant of atrial septal defect.3Morphological and echocardiographic studies, however, have shown that, in this variant presumed to represent deficient atrial septation, it is the atrioventricular septal structures, rather than the atrial septum, which are deficient, the phenotypic feature being the presence of a common atrioventricular junction.4,5In this review, we will show how, using modern day echocardiographic techniques, particularly the newly developed potential for three-dimensional display, it is an easy matter to identify the presence or absence of the common atrioventricular junction, and then to demonstrate the various relationships between the valvar leaflets, the septal structures, and the common junction itself which determine the options for clinical presentation within the group.

Atrioventricular septal defect with an imperforate right-sided component of the common atrioventricular valve: anatomic and embryologic considerations

2013 ◽  
Vol 24 (3) ◽  
pp. 540-542
Author(s):  
Lazaro E. Hernandez ◽  
Lazaros K. Kochilas ◽  
Robert H. Anderson
Keyword(s):  
Septal Defect ◽  
Cardiac Development ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Valve ◽  
Atrioventricular Junction ◽  
Atypical Case ◽  
Hypoplastic Right Ventricle ◽  
The Common ◽  
The Right ◽  
Common Atrioventricular Valve

AbstractWe describe an atypical case of an atrioventricular septal defect with a common atrioventricular junction in which the right-sided component of the common atrioventricular valve was imperforate, producing tricuspid atresia with a severely hypoplastic right ventricle and an ostium primum defect. We discuss the implications of the anatomic findings with regard to concepts of cardiac development, drawing a comparison with similar cases previously reported.

The comparative relationships between locations of the papillary muscles and electrophysiologic QRS axis in patients with atrioventricular septal defect and common as opposed to separate orifices in the valve guarding the common atrioventricular junction

2016 ◽  
Vol 27 (2) ◽  
pp. 261-266 ◽  
Author(s):  
Liying Low ◽  
Salim F. Idriss ◽  
Robert H. Anderson ◽  
Charles Maynard ◽  
Galen Wagner ◽  
...  
Keyword(s):  
Septal Defect ◽  
Pressure Overload ◽  
Frontal Plane ◽  
Atrioventricular Septal Defect ◽  
Papillary Muscles ◽  
Atrioventricular Junction ◽  
Atrioventricular Septal Defects ◽  
Younger Age ◽  
The Common ◽  
Ventricular Shunting

AbstractBackgroundKnowledge regarding factors that influence deviation of the QRS axis is important when seeking to differentiate between physiological and pathological changes. We hypothesised that, in contrast to those patients with an atrioventricular septal defect and common atrioventricular junction permitting only atrial shunting, those associated with ventricular shunting would show no relationship between the positions of the papillary muscles and the degree of the leftward deviation of the QRS axis.MethodsWe compared the positions of endocardial origin of the papillary muscles, and the frontal plane QRS axis, in patients with atrioventricular septal defects and common atrioventricular junction permitting exclusively atrial as opposed to atrial and ventricular shunting.ResultsWe analysed 18 patients with atrial and ventricular shunting and 23 patients with exclusively atrial shunting. The correlation coefficient between the ratio of distances of the papillary muscles from the mid-septum and the amount of leftward deviation in the frontal plane QRS axis was 0.1 (p=0.4) in those with ventricular shunting and 0.26 (p=0.01) in those with exclusively atrial shunting.ConclusionsIn contrast to patients with the so-called primum form of atrioventricular septal defect, in whom the locations of the papillary muscles correlate with the degree of QRS axis, such relationships are lacking in patients with defects permitting both atrial and ventricular shunting. It may be, therefore, that the presence of ventricular shunting and/or their younger age causes pressure overload, which negates the leftward QRS forces caused by the abnormally positioned papillary muscles.

scholarly journals Complete Atrioventricular Septal Defect and Pulmonary Stenosis Diagnosed in a 49-Year-Old Woman after 10 Uneventful Births

2015 ◽  
Vol 42 (2) ◽  
pp. 166-168 ◽  
Author(s):  
Firat H. Altin ◽  
Okan Yildiz ◽  
Mehmet Karacalilar ◽  
Oyku Tosun ◽  
Ozgen Ilgaz Kocyigit ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Septal Defect ◽  
Medical Literature ◽  
Pulmonary Stenosis ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular ◽  
Spontaneous Delivery

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.

Imperforate Ebstein's malformation in atrioventricular septal defect

1991 ◽  
Vol 1 (2) ◽  
pp. 152-154 ◽  
Author(s):  
Jeong-Wook Seo ◽  
Woo Hee Jung ◽  
Yong Won Park
Keyword(s):  
Left Ventricle ◽  
Septal Defect ◽  
Lateral Wall ◽  
Atrioventricular Septal Defect ◽  
Left Hand ◽  
Left Lateral Wall ◽  
Right Isomerism ◽  
The Common ◽  
The Right ◽  
Double Inlet Left Ventricle

SummaryAnatomic data are presented of a female abortus with the unusual combination of Ebstein's malformation and atrioventricular septal defect with right isomerism of the atrial appendages, left-hand ventricular topology and discordant ventriculoarterial connection. The common atrioventricular orifice was guarded by an effectively common valve, the right half being guarded by a well-formed three-leaflet valve, but the posterior and left lateral wall of the orifice being deficient of valvar leaflet tissue. The right ventricular inlet was a blind chamber, an imperforate dimple being seen which was surrounded by valvar remnants. The apical trabecular and outlet components of the right ventricle communicated with the left ventricle through an outlet ventricular septal defect in front of the anterosuperior leaflet of the atrioventricular valve, giving the potential clinically for the lesion to be misdiagnosed as double inlet left ventricle.

scholarly journals Intracardiac Repair of Intermediate Atrioventricular Canal by Nunn’s Technique: A Video Presentation

2019 ◽  
pp. 64-68
Author(s):  
Ujjwal K. Chowdhury ◽  
Niwin George ◽  
Sukhjeet Singh ◽  
Lakshmi Kumari Sankhyan ◽  
Aandrei Jha ◽  
...  
Keyword(s):  
New England ◽  
Atrioventricular Septal Defect ◽  
Abnormal Development ◽  
Atrioventricular Canal ◽  
Endocardial Cushions ◽  
Atrioventricular Septal Defects ◽  
Intracardiac Repair ◽  
Common Etiology ◽  
Basal Portion ◽  
The Common

The atrioventricular septal defects encompass a spectrum of lesions in which the common etiology appears to be abnormal development of the superior and inferior endocardial cushions, resulting in a deficiency or absence of the atrioventricular septum [1-6]. This deficiency of the atrioventricular septum results in an ostium primum defect immediately above the atrioventricular valves and a scooped-out area in the inlet (basal) portion of the ventricular septum. Data from the New England Regional Infant Cardiac Program and the Baltimore-Washington infant study defined a prevalence of 0.118 and 0.352 per 1000 live births [7,8]. Approximately, 50-75% of these patients have trisomy 21 [7-9]. When viewed from a reverse perspective, approximately 35-40% of patients with Down’s syndrome have an atrioventricular septal defect [7-9].

scholarly journals Genetic testing for atrioventricular septal defect

2018 ◽  
Vol 2 (s1) ◽  
pp. 48-50
Author(s):  
Yeltay Rakhmanov ◽  
Paolo Enrico Maltese ◽  
Stefano Paolacci ◽  
Francesca Fanelli ◽  
Tommaso Beccari ◽  
...  
Keyword(s):  
Autosomal Recessive ◽  
Septal Defect ◽  
Autosomal Dominant ◽  
Autosomal Recessive Inheritance ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Canal ◽  
Atrioventricular Junction ◽  
Live Births ◽  
Gene Test ◽  
Atrioventricular Septation

Abstract Atrioventricular septal defect (AVSD) is a congenital heart defect characterized by a shared atrioventricular junction coexisting with deficient atrioventricular septation. The main morphological characteristic of AVSD is a common atrioventricular canal. The prevalence of AVSD is estimated at 0.31/1000 live births and is higher among subjects with PTPN11 mutations. ASD may have autosomal dominant or autosomal recessive inheritance. This Utility Gene Test was prepared on the basis of an analysis of the literature and existing diagnostic protocols. It is useful for confirming diagnosis, as well as for differential diagnosis, couple risk assessment and access to clinical trials.

Management of the left atrioventricular valve in 95 patients with atrioventricular septal defects and a common atrioventricular orifice—a ten year review

1991 ◽  
Vol 1 (4) ◽  
pp. 367-373 ◽  
Author(s):  
François Lacour-Gayet ◽  
Juan Comas ◽  
Jacqueline Bruniaux ◽  
Alain Serraf ◽  
Jean Losay ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Septal Defect ◽  
Primary Repair ◽  
Ventricular Outflow Tract ◽  
Atrioventricular Septal Defect ◽  
Left Ventricular ◽  
Atrioventricular Valve ◽  
Septal Defects ◽  
Left Ventricular Outflow ◽  
The Right

SummaryDuring a 10 year interval, between January 1, 1981 and January 1, 1991, primary repair of atrioventricular septal defect with a common atrioventricular orifice was performed in 95 patients younger than 1 year. Patients with atrioventricular septal defect having two atrioventricular orifices and a small ventricular septal defect, and those with severe hypoplasia of the left ventricle, were not included in the present study. According to the presence of associated anomalies, patients were divided into those with simple forms (72%) and those with complex forms (28%). These included parachute deformity of the left atrioventricular valve (10 cases), multiple muscular ventricular septal defects (5 cases), low-lying infundibular stenosis of the right ventricle (4 cases), obstruction of the left ventricular outflow tract (3 cases), tetralogy ofFallot (2 cases) and hypoplasia of the left ventricle (2 cases).

Atrioventricular septal defect in fetal life—a clinicopathological correlation

1991 ◽  
Vol 1 (4) ◽  
pp. 334-343 ◽  
Author(s):  
Andrew C. Cook ◽  
Lindsey D. Allan ◽  
Robert H. Anderson ◽  
Gurleen Sharland ◽  
Nuala L. K. Fagg
Keyword(s):  
Septal Defect ◽  
Fetal Echocardiography ◽  
Morphological Characteristics ◽  
Atrioventricular Septal Defect ◽  
Postnatal Life ◽  
Fetal Life ◽  
Clinicopathological Correlation ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
High Incidence

SummaryIn a series of over 7,000 pregnancies referred for fetal echocardiography, atrioventricular septal defects were detected in 103 fetuses, (17% of 594 anomalies). A large proportion (42%) also had isomerism of the atrial appendages, abnormalities in arrangement and morphology of the thoracoabdominal organs, and a high incidence of associated cardiac malformations. When this was associated with abnormalities of rhythm, the fetus often showed signs of intrauterine congestive heart failure; hydrops, pleural effusion and ascites. Those with trisomic karyotypes (predominantly trisomy 21) were associated with usual atrial arrangement and concordant connections of the cardiac segments. Of the 103 fetuses, 53 were examined at autopsy. Extracardiac abnormalities were documented, and detailed morphologic examination and measurements of the heart were made. The ratios of inlet to outlet septal disproportion were not significantly different from those found in postnatal hearts. Again, as in postnatal life, those hearts with common atrioventricular orifice had a greater degree of ‘scooping’ of the ventricular septum compared to those with separate right and left atrioventricular valves. There were no significant differences in these findings in relation to the karyotype of the fetus. There was an increased incidence of ventricular dominance, and also of coarctation or hypoplasia of the aortic arch, in fetal hearts when compared to autopsied hearts obtained in postnatal life. In summary, the distinguishing morphological characteristics of atrioventricular septal defect are present from 16 weeks of gestation.

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