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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.
As Lutembacher's syndrome is known for ASD and MS, most of the symptoms experienced will be associated with ASD and MS. For most people, they will remain asymptomatic (experience no symptoms) but when symptoms are shown, they are due mainly to ASD and will vary depending on the size of the hole in the atria.
Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale ; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO).
Atrioventricular septal defect (AVSD) or atrioventricular canal defect (AVCD), also known as "common atrioventricular canal" or "endocardial cushion defect" (ECD), is characterized by a deficiency of the atrioventricular septum of the heart that creates connections between all four of its chambers.
The gap below it is known as the ostium primum (from Latin 'first opening'), and becomes increasingly small. The septum primum eventually fuses with the endocardial cushion, closing the ostium primum off completely. Meanwhile, perforations appear in the superior part of the septum primum, forming the ostium secundum (from Latin 'second opening').
The septum primum, a septum which grows down to separate the primitive atrium into the left atrium and right atrium, grows in size over the course of heart development. The primary interatrial foramen is the gap between the septum primum and the septum intermedium , which gets progressively smaller until it closes.
An Atrial septal defect is a relatively common heart malformation that occurs when the interatrial septum fails to develop properly. Persistence of the ostium secundum is the most common atrial septal defect. [3] Additionally, in a subset of the population, the foramen ovale is not overtly patent but the two septa have not fused.
The septum primum is on the left side of the heart in the left atrium while the septum secundum is much thicker and is located on the right side, in the right atrium. During development, blood shunts from the floor of the right atrium through the foramen ovale in the septum secundum then up through the foramen secundum in the septum primum. [2]