Common Atrioventricular Canal Defects

Cohen, Meryl S.

doi:10.1002/9781444306309.ch15

Cited by 4 publications

(5 citation statements)

References 49 publications

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“…If there is associated significant AV valve regurgitation, ventricular imbalance or coarctation signs of cardiac failure will occur much earlier [16,20]. There is a small subgroup of patients with complete AVSD who do not develop signs of cardiac failure despite a significant ventricular component.…”

Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

Congenital Heart Disease in Down Syndrome

1993

Pediatrics in Review

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Down syndrome remains the most common chromosomal abnormality in live-born infants in the world today. The association between Down syndrome and congenital heart disease (CHD) is well known, and it is widely recognized that CHD contributes significantly to the morbidity of children with Down syndrome. The reported incidence of CHD in Down syndrome patients is between 40 and 60%. The most commonly described defect is complete atrioventricular septal defect (AVSD), which comprises 30-40% of all cardiac defects. Complex genetic factors are involved. Routine cardiac screening of all newborn babies with Down syndrome is recommended. Expert groups suggest that the cardiac status of all children with Down syndrome should be established by 6 weeks of age to permit appropriate and timely treatment avoiding the establishment of irreversible pulmonary vascular disease that would make corrective surgery impossible.

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Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

Congenital Heart Disease in Down Syndrome

1993

Pediatrics in Review

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“…In Rastelli type A, the superior bridging leaflet is divided at the level of the ventricular septum; in Rastelli type B, the division of the superior bridging leaflet occurs to a right ventricular papillary muscle; and in Rastelli type C, the superior bridging leaflet is undivided or free floating. Rastelli type C is the most common arrangement found in Down syndrome [20].…”

Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

“…Ventricular hypoplasia and atrial isomerism are also described although infrequently with Down syndrome. Tetralogy of Fallot is the most commonly observed association and is seen in up to 6.7% cases of AVSD [20]. There is a high incidence of associated other extra cardiac abnormalities.…”

Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

“…If the ventricular component is large, left to right shunting occurs after the first few weeks of life as the pulmonary vascular resistance falls, and the infant will develop signs of congestive heart failure. If there is associated significant AV valve regurgitation, ventricular imbalance or coarctation signs of cardiac failure will occur much earlier [16,20]. There is a small subgroup of patients with complete AVSD who do not develop signs of cardiac failure despite a significant ventricular component.…”

Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

“…There is a small subgroup of patients with complete AVSD who do not develop signs of cardiac failure despite a significant ventricular component. In these individuals there is persistent elevation of pulmonary vascular resistance [20].…”

Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

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Congenital Heart Disease in Down Syndrome

1993

Pediatrics in Review

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Trisomy 21, Down syndrome, is one of a number of chromosomal abnormalities associated with congenital heart disease. Recent studies indicate that approximately 5% of all congenital heart defects are associated with some form of chromosomal abnormality, the majority of which are Down syndrome. Reports of the incidence of congenital heart disease in patients who have Down syndrome have varied, but it is commonly accepted to be 50%. Endocardial cushion defect and ventricular septal defects both have been reported as the "most common," but the majority of investigators accept the endocardial cushion defect as being the more frequent. The association between endocardial cushion defects and Down syndrome is so striking that when an endocardial cushion defect is diagnosed in an infant, the possibility of Down syndrome always should be considered.

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