Congenitally corrected transposition with atrioventricular septal defect

1998 ◽  
Vol 8 (4) ◽  
pp. 472-478 ◽  
Author(s):  
Fause Attie ◽  
Pedro Iturralde ◽  
Carlos Zabal ◽  
Maria Rijlaarsdam ◽  
Alfonso Buendia ◽  
...  
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Conduction ◽  
Atrioventricular Valve ◽  
His Bundle ◽  
Electrophysiological Studies ◽  
Congenitally Corrected Transposition ◽  
Atrial Stimulation ◽  
Common Atrioventricular Valve ◽  
Corrected Transposition

AbstractWe describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement. All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ven-triculography.In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the pos terior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulationThe clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventric ular valves.

Melody valve insertion for relief of “Tricuspid” stenosis in an unbalanced atrioventricular septal defect

2021 ◽  
pp. 1-3
Author(s):  
Samuel Menahem ◽  
Philip A. Roberts ◽  
David Tanous
Keyword(s):  
Right Ventricle ◽  
Young Woman ◽  
Right Atrium ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Valve ◽  
Melody Valve ◽  
Hypoplastic Right Ventricle ◽  
Common Atrioventricular Valve

Abstract A Melody valve was successfully placed across a very stenotic right-sided component of a common atrioventricular valve because of ongoing troublesome arrhythmias in a young woman with an unbalanced atrioventricular septal defect, a very dilated right atrium and a hypoplastic right ventricle. Four years later, she remains well.

Surgical repair of atrioventricular septal defect with common atrioventricular valve in early infancy

2006 ◽  
Vol 16 (S3) ◽  
pp. 52-58 ◽  
Author(s):  
François Lacour-Gayet ◽  
David N. Campbell ◽  
Max Mitchell ◽  
Sunil Malhotra ◽  
Robert H. Anderson
Keyword(s):  
Pulmonary Hypertension ◽  
Septal Defect ◽  
Surgical Repair ◽  
Surgical Techniques ◽  
Clinical Results ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Valve ◽  
The Past ◽  
Cardiac Surgeons ◽  
Common Atrioventricular Valve

The repair of atrioventricular septal defect with a common atrioventricular valve is reconstructive surgery at its best, and hence one of the favourite operations performed by paediatric cardiac surgeons. In the past, the post-operative course from such patients was dominated by the occurrence of pulmonary hypertension crises, which were responsible for significant morbidity and mortality. Nowadays, repair is generally undertaken early in infancy, and this approach has mitigated the problems emanating from pulmonary hypertension. Coupled with a better understanding of the anatomy, and adaptation of the surgical techniques, repair can now be achieved safely at around 2 to 4 months of life, without increasing the risk of postoperative regurgitation across the reconstructed left atrioventricular valve. In this review, we discuss the surgical techniques required for, and clinical results of, such early repair.

Prevalence at birth, “natural” risk and survival with atrioventricular septal defect

1991 ◽  
Vol 1 (4) ◽  
pp. 285-289 ◽  
Author(s):  
Milan Samaánek
Keyword(s):  
Septal Defect ◽  
Heart Defects ◽  
Atrioventricular Septal Defect ◽  
First Year ◽  
Atrioventricular Valve ◽  
Cardiac Malformation ◽  
Natural Risk ◽  
Live Births ◽  
Complete Form ◽  
Common Atrioventricular Valve

SummaryPrevalence of atrioventricular septal defect in liveborn infants at birth was calculated in Bohemia (population of 6.3 million). Of 664,208 liveborn infants, 4,409 had a congenital malformation of the heart and in 126 of them (0.19/1,000 live births, 2.9% of all heart defects) this was found to be an atrioventricular septal defect. The ratio between those with a common valvar orifice (complete form) versus those with separate right and left atrioventricular valvar orifices (incomplete form) was 1.14:1. The ratio of girls to boys was 1.17:1. Children born in March, July and October were more likely to have the defect (0.26/1,000 live births) than those born in May (0.10/ 1,000 live births). The defect was the eighth most frequent critical congenital cardiac malformation. Eight in each 100,000 liveborn children, and 3.56% of all patients with congenital cardiac disease, are at high risk of dying due to deficient atrioventricular septation, which in 82.5% resulted in the presence of a common atrioventricular valve (complete form). Unconditional death rate was studied in 1,008 liveborn children who died with a cardiac malformation before the age of 15 before the advent of cardiac surgery. Of these, 64 (6.35%) died with an atrioventricular septal defect which was complete in 5.95% and incomplete in 0.40%. All but one of 60 children with the complete form (common atrioventricular valve) died in the first year of life. The last child died between the age of 2 and 5 years. The estimate for natural survival was calculated using our own data on the prevalence and age distribution of mortality. Of all the liveborn children with atrioventricular septal defect, 91% are expected to survive the first month, 81% (78−85) the first trimester, 63% (56−69) six months, and 49% (35−54) the first year. The survival curve remains stable thereafter.

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