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==Overview==
==Overview==
'''Atrioventricular septal defect''' (AVSD), previously known as "common atrioventricular canal" (CAVC) or "[[endocardial cushion]] defect", is characterized by a deficiency of the atrioventricular septum of the [[heart]]. It is caused by an abnormal or inadequate fusion of the superior and inferior [[endocardial cushions]] with the mid portion of the [[atrial septum]] and the muscular portion of the [[ventricular septum]].  
'''Atrioventricular septal defect''' (AVSD), previously known as "common atrioventricular canal" (CAVC) or "[[endocardial cushion]] defect", is a congenital heart defect characterized by the deficiency in development of the atrioventricular septum of the [[heart]]. It is caused by an abnormal fusion of the superior and inferior [[endocardial cushions]] with the mid-portion of the [[atrial septum]] and the muscular portion of the [[ventricular septum]]. The malformation results in a hole in the heart between the [[mitral valve|mitral]] and [[triscuspid valve|triscupid valves]]. This lack of separation can cause hemodynamic complications.


A variety of different classifications have been used, but the defects are usefully divided into "partial" and "complete" forms. In the partial AVSD, there is a defect in the primum or inferior part of the atrial septum but no direct intraventricular communication (ostium primum defect). In the complete AVSD, there is a large ventricular component beneath either or both the superior or inferior bridging leaflets of the AV valve.
AVSD is classified as either complete, partial, or intermediate/transitional. Classification is based on the extent of loss of separation between the chambers of the heart. In partial AVSD, there is a defect in the primum or inferior part of the atrial septum but no direct intraventricular communication (ostium primum defect). In complete AVSD, there is a large ventricular component beneath either or both the superior or inferior bridging leaflets of the AV valve. In intermediate or transitional AVSD, the leaflets of the common AV valve are stuck to the ventricular septum causing a division of the two valves and resulting in a marginal sized hole between the ventricle. Transitional AVSDs are similar to partial AVSDs in behavior but visually appear more like a complete AVSD.  


If there is a defect in the septum, it is possible for blood to travel from the left side of the heart to the right side of the heart, or the other way around. Since the right side of the heart contains venous blood with a low oxygen content, and the left side of the heart contains arterial blood with a high oxygen content, it is beneficial to prevent any communication between the two sides of the heart and prevent the blood from the two sides of the heart from mixing with each other.
Most infants are largely asymptomatic and may only exhibit signs of failure to thrive. A clinical diagnosis often occurs based on the presence of a heart murmur and can be detected at any time, from fetal development to later in life. A fetal echocardiogram can detect the presence of a murmur during prenatal care. A cardiac catheterization can also used for diagnosis.  


AVSDs account for approximately 5% of all congenital heart diseases. It is most commonly associated with patients with [[Down syndrome]] (trisomy 21) or heterotaxy syndromes.<ref>Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65(suppl):441–444.</ref> Forty-five percent of children with Down syndrome have congenital heart disease. Of these, 35–40% have AV septal defects.<ref>Al-Hay AA et al: Complete atrioventricular septal defect, Down syndrome and surgical outcome: Risk factors. Ann Thorac Surg 2003;75:412.</ref>
AVSDs can result in significant changes in hemodynamics. A defect in the septum can result in blood freely traveling from the left side of the heart to the right side of the heart resulting in mixing of oxygenated and deoxygenated blood. This mixed blood is pumped back to the lungs and causes an increase in blood volume in the lungs resulting in [[hypertension]], [[cardiomegaly]], and damage to the blood vessels. A high pulmonary pressure can result in a [[pulmonary edema]]
 
Surgical therapy is the most reliable method of treatment. An open heart surgery will separate the [[mitral valve|mitral]] and [[triscuspid valve|triscuspid valve]] and close the atrial and ventricular septal defects. Surgical therapy is not advised until a child is old enough to undergo surgery. A pulmonary artery banding surgery may be used to delay the necessity for surgery.
 
AVSDs account for approximately 5% of all congenital heart diseases. It is most commonly associated with patients with [[Down syndrome]] (trisomy 21) or heterotaxy syndromes.<ref>Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65(suppl):441–444.</ref> 45% of children with Down syndrome have some form of congenital heart disease. Of these, 35–40% have AV septal defects.<ref>Al-Hay AA et al: Complete atrioventricular septal defect, Down syndrome and surgical outcome: Risk factors. Ann Thorac Surg 2003;75:412.</ref>


==References==
==References==

Revision as of 13:14, 8 July 2011

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Atrioventricular septal defect (AVSD), previously known as "common atrioventricular canal" (CAVC) or "endocardial cushion defect", is a congenital heart defect characterized by the deficiency in development of the atrioventricular septum of the heart. It is caused by an abnormal fusion of the superior and inferior endocardial cushions with the mid-portion of the atrial septum and the muscular portion of the ventricular septum. The malformation results in a hole in the heart between the mitral and triscupid valves. This lack of separation can cause hemodynamic complications.

AVSD is classified as either complete, partial, or intermediate/transitional. Classification is based on the extent of loss of separation between the chambers of the heart. In partial AVSD, there is a defect in the primum or inferior part of the atrial septum but no direct intraventricular communication (ostium primum defect). In complete AVSD, there is a large ventricular component beneath either or both the superior or inferior bridging leaflets of the AV valve. In intermediate or transitional AVSD, the leaflets of the common AV valve are stuck to the ventricular septum causing a division of the two valves and resulting in a marginal sized hole between the ventricle. Transitional AVSDs are similar to partial AVSDs in behavior but visually appear more like a complete AVSD.

Most infants are largely asymptomatic and may only exhibit signs of failure to thrive. A clinical diagnosis often occurs based on the presence of a heart murmur and can be detected at any time, from fetal development to later in life. A fetal echocardiogram can detect the presence of a murmur during prenatal care. A cardiac catheterization can also used for diagnosis.

AVSDs can result in significant changes in hemodynamics. A defect in the septum can result in blood freely traveling from the left side of the heart to the right side of the heart resulting in mixing of oxygenated and deoxygenated blood. This mixed blood is pumped back to the lungs and causes an increase in blood volume in the lungs resulting in hypertension, cardiomegaly, and damage to the blood vessels. A high pulmonary pressure can result in a pulmonary edema

Surgical therapy is the most reliable method of treatment. An open heart surgery will separate the mitral and triscuspid valve and close the atrial and ventricular septal defects. Surgical therapy is not advised until a child is old enough to undergo surgery. A pulmonary artery banding surgery may be used to delay the necessity for surgery.

AVSDs account for approximately 5% of all congenital heart diseases. It is most commonly associated with patients with Down syndrome (trisomy 21) or heterotaxy syndromes.[1] 45% of children with Down syndrome have some form of congenital heart disease. Of these, 35–40% have AV septal defects.[2]

References

  1. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65(suppl):441–444.
  2. Al-Hay AA et al: Complete atrioventricular septal defect, Down syndrome and surgical outcome: Risk factors. Ann Thorac Surg 2003;75:412.

de:Atrio-ventrikulärer Septumdefekt nn:Atrioventrikulær septumdefekt uk:Атріовентрикулярний септальний дефект

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