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The ostium primum atrial septal defect is a defect in the atrial septum at the level of the tricuspid and mitral valves. This is sometimes known as an endocardial cushion defect because it often involves the endocardial cushion, which is the portion of the heart where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid valve.
A defect in the ostium primum is occasionally classified as an atrial septal defect, [17] but it is more commonly classified as an atrioventricular septal defect. [18] [19] Ostium primum defects are less common than ostium secundum defects. [20] This type of defect is usually associated with Down syndrome. [21]
Fewer ASD cases are due to the ostium primum, where the “first opening” or ostium primum, doesn’t make it all the way down, again leaving an opening between right and left atria. This primum type of congenital defect is found in around 25% of patients with Down syndrome.
Failure of the septum primum to fuse with the endocardial cushion can lead to an ostium primum atrial septal defect. [1] This is the second most common type of atrial septal defect [2] and is commonly seen in Down syndrome. Typically, this defect will cause a shunt to occur from the left atrium to the right atrium.
Septal defects that may occur with Lutembacher's syndrome include: Ostium primum atrial septal defect or ostium secundum which is more prevalent. [1] Lutembacher's syndrome affects females more often than males. [1] It can affect children or adults; the person can either be born with the disorder or develop it later in life.
Failure of the septum primum to fuse with the endocardial cushion can lead to an ostium primum atrial septal defect. [1] This is the second most common type of atrial septal defect [2] and is commonly seen in Down syndrome. Typically this defect will cause a shunt to occur from the left atrium to the right atrium.
Surgical mortality for uncomplicated ostium primum defects in experienced centers is 2%; for uncomplicated cases of complete atrioventricular canal defect, 4% or less. Certain complications such as tetralogy of Fallot or highly unbalanced flow across the common AV valve can increase risk significantly. [13] [14]
Failure of the septum primum to fuse with the endocardial cushion can lead to an ostium primum atrial septal defect. [4] This is the second most common type of atrial septal defect [3] and is commonly seen in Down syndrome. Typically, this defect will cause a shunt to occur from the left atrium to the right atrium.