Solitary trunk from the right ventricle with a cleft mitral valve simulating the trifoliate left valve of an atrioventricular septal defect with common atrioventricular junction

2004 ◽  
Vol 14 (4) ◽  
pp. 444-446
Author(s):  
Geoffrey P. Sharratt ◽  
Robert H. Anderson
Keyword(s):  
Mitral Valve ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Left Ventricular ◽  
Papillary Muscles ◽  
Left Valve ◽  
Anterior Leaflet ◽  
Atrioventricular Junction ◽  
Arterial Trunk ◽  
The Right

We report a case of a true cleft in the anterior leaflet of the mitral valve. The cleft, however, is directed toward the ventricular septum, and the left ventricular papillary muscles have the same arrangement as seen in the setting of a common atrioventricular orifice. The atrioventricular septum is intact. This appearance reflects the presence, in this patient, of right ventricular origin of a solitary arterial trunk, so that there was no outflow tract within the left ventricle to interpose between the mitral valve and the septum.

A very unusual combination of straddling and overriding of the tricuspid valve associated with clefting of the mitral valve

2005 ◽  
Vol 15 (4) ◽  
pp. 422-424 ◽  
Author(s):  
Liane Eberhardt ◽  
Philippe Chetaille ◽  
Bernard Kreitmann
Keyword(s):  
Mitral Valve ◽  
Tricuspid Valve ◽  
Septal Defect ◽  
Surgical Repair ◽  
Atrioventricular Septal Defect ◽  
Left Ventricular ◽  
Atrioventricular Junction ◽  
Unusual Association ◽  
The Right ◽  
Double Orifice

We describe a patient in whom we found dual orifices in a straddling and overriding tricuspid valve, with two normally sized ventricles and a cleft in the mitral valve. The patient underwent successful surgical repair. We discuss the concept of “double-orifice right atrium”, as well as the need to differentiate the isolated cleft of the morphologically mitral valve from the zone of apposition between the left ventricular components of the bridging leaflets seen in the setting of atrioventricular septal defect with common atrioventricular junction. We emphasise the unusual association of these abnormalities of the right and left atrioventricular valves in patients with separate atrioventricular junctions.

scholarly journals Systematic cutting of selected secondary mitral valve chordae, in association with a shallow myectomy, in obstructive hypertrophic cardiomyopathy:impact on mitral valve function and patient management

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Margonato ◽  
R Abete ◽  
A Zyrianov ◽  
A Sorropago ◽  
M Chioffi ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Septal Defect ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Anterior Leaflet ◽  
Mitral Leaflet ◽  
Access To Treatment ◽  
Anterior Mitral Leaflet ◽  
Septal Myectomy ◽  
Number Of Patients

Abstract Introduction Few centers worldwide have large experience with performing an extended septal myectomy in patients with obstructive hypertrophic cardiomyopathy (HCM). Therefore, many HCM patients eligible for surgical relief of left ventricular (LV) outflow gradient do not have access to treatment. In a previous study, cutting fibrotic anterior mitral leaflet secondary chordae, in association with only a shallow myectomy, proved highly effective in moving the mitral valve (MV) apparatus away from the LV outflow tract and relieving the outflow gradient in our HCM patients with mild hypertrophy (<19 mm), a surgical approach that simplifies the operation. Purpose To assess whether chordal cutting is equally effective in improving MV geometry and relieving LV outflow gradient and heart failure symptoms in HCM patients with more marked hypertrophy. Methods Surgical outcome and MV geometry and function were assessed in 226 consecutive HCM patients who underwent systematic cutting of fibrotic anterior mitral leaflet secondary chordae, in association with a shallow myectomy and independently of magnitude of septal thickness, at our center from January 2015 to December 2018. Results Of 226 study patients, 1 (0.4%) died perioperatively. None had iatrogenic septal defect. Postoperatively, LV outflow gradient at rest decreased from 70±36 to 10±2 mmHg (P<0.001). In the 77 patients in whom data on the outflow gradient provoked with physiologic maneuvers after surgery were available, the provocable gradient was 16±10 mmHg. NYHA functional class improved significantly (P<0.001), with the number of patients in class III-IV decreasing from 178 (79%) to 2 (0.9%). No patient had residual severe MV regurgitation and only 4 (1.7%) had moderate-to-severe regurgitation. Quality of the echocardiogram allowed assessment of MV geometry in 212 (94%) patients. In the 62 patients with mild hypertrophy, anterior leaflet-annulus ratio increased 27% postoperatively, from 0.43+0.06 to 0.55+0.06 and MV tenting area decreased 34% from 2.9+0.6 to 1.9+0.4 cm2 (P<0.001), indicating repositioning of MV coaptation away from the outflow tract (with increased outflow tract dimension). Similarly, in 150 patients with marked hypertrophy, anterior leaflet-annulus ratio increased 27% from 0.43+0.05 to 0.55+0.06 and tenting area decreased 28% from 2.9+0.6 to 2.1+0.4 cm2 (P<0.001). Conclusions Our results show that cutting fibrotic anterior mitral leaflet secondary chordae, by moving the MV apparatus away from the LV outflow tract and independently of the magnitude of septal hypertrophy, contributes to improve the results of septal myectomy and reduces the need for a deep septal excision (and associated risk of iatrogenic septal defect) in patients with obstructive HCM. Therefore, chordal cutting could make the myectomy operation more accessible to surgeons, increasing the availability of surgical treatment for HCM patients eligible for invasive abolition of LV outflow obstruction. Funding Acknowledgement Type of funding source: None

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).

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.

Surgical Correction of a Partial Atrioventricular Septal Defect With a Ventricular Septal Defect in a Dog

2005 ◽  
Vol 41 (2) ◽  
pp. 137-143 ◽  
Author(s):  
Midori Akiyama ◽  
Ryou Tanaka ◽  
Kohji Maruo ◽  
Yoshihisa Yamane
Keyword(s):  
Mitral Valve ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Atrioventricular Septal Defect ◽  
Left Ventricular ◽  
Surgical Correction ◽  
Cardiac Murmur ◽  
Left Ventricular Outflow

Echocardiography of a dog with a cardiac murmur revealed an ostium primum septal defect, a ventricular septal defect, and mitral valve malformation with regurgitation. The mitral valve and tricuspid valve were separated and displaced at the same level as the ventricular septum. The mitral valve had a cleft in the septal cusp. Cardiac catheterization and angiocardiography showed a left-to-right shunt and a "goose-neck sign," which indicated an elongated left ventricular outflow tract. The diagnosis of a partial atrioventricular septal defect with ventricular septal defect was made. Surgical correction was successfully performed under extracorpo-real circulation using a cardiopulmonary bypass system.

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.

scholarly journals Atrioventricular Septal Defect: What Is in a Name?

2021 ◽  
Vol 8 (2) ◽  
pp. 19
Author(s):  
Michael Rigby
Keyword(s):  
Mitral Valve ◽  
Septal Defect ◽  
Adult Congenital Heart Disease ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Canal ◽  
Aortic Leaflet ◽  
Adult Congenital ◽  
The Right ◽  
Cardiac Surgeons ◽  
Almost All

Robert Anderson has made a huge contribution to almost all aspects of morphology and understanding of congenital cardiac malformations, none more so than the group of anomalies that many of those in the practice of paediatric cardiology and adult congenital heart disease now call ‘Atrioventricular Septal Defect’ (AVSD). In 1982, with Anton Becker working in Amsterdam, their hallmark ‘What’s in a name?’ editorial was published in the Journal of Thoracic and Cardiovascular Surgery. At that time most described the group of lesions as ‘atrioventricular canal malformation’ or ‘endocardial cushion defect’. Perhaps more significantly, the so-called ostium primum defect was thought to represent a partial variant. It was also universally thought, at that time, that the left atrioventricular valve was no more than a mitral valve with a cleft in the aortic leaflet. In addition to this, lesions such as isolated cleft of the mitral valve, large ventricular septal defects opening to the inlet of the right and hearts with straddling or overriding tricuspid valve were variations of the atrioventricular canal malformation. Anderson and Becker emphasised the differences between the atrioventricular junction in the normal heart and those with a common junction for which they recommended the generic name, ‘atrioventricular septal defect’. As I will discuss, over many years, they continued to work with clinical cardiologists and cardiac surgeons to refine diagnostic criteria and transform the classification and understanding of this complex group of anomalies. Their emphasis was always on accurate diagnosis and communication, which is conveyed in this review.

Morphology of atrioventricular septal defect and fetal hydrops in the fetal rat

1993 ◽  
Vol 3 (1) ◽  
pp. 13-19 ◽  
Author(s):  
Kazuo Momma ◽  
Masahiko Ando
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Right Atrial ◽  
Whole Body ◽  
Fetal Hydrops ◽  
Cross Sectional ◽  
Arterial Trunk ◽  
Fetal Rat ◽  
Subcutaneous Edema ◽  
The Right

AbstractWe studied 28 fetal rats with atrioventricular septal defect with a common orifice produced from 300 fetuses treated with bis-diamine (200mg) on the ninth and tenth day of pregnancy. After undergoing rapid whole- body freezing on the 21st day, they were studied by means of serial cross-sectional photographs of the frozen thorax. Forty-one fetuses with normal hearts subsequent to treatment with bis-diamine served as controls. Atrioventricular septal defect was isolated in three, or was associated with either tetralogy of Fallot (n=14) or common arterial trunk (n=11). Fetal hydrops was associated with evidence of regurgitation of the atrioventricular valves. In a subgroup with larger right atrial volumes (increased by more than two standard deviation over the mean in the controls), the following parameters were significantly increased: subcutaneous edema, pericardial effusion, thickening of the left atrioventricular valve, cardiac chamber volumes and masses. The superior and inferior caval veins, and the descending aorta were significantly smaller in fetuses with atrioventricular septal defect than in controls. Fetal atrioventricular septal defect, therefore, was associated frequently with hydrops and increased volumes of the right atrium and both ventricles. Hydropic changes included cutaneous edema and increased pericardial, pleural, and peritoneal fluid.

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.

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