Lutembachers syndrome is a very rare form of congenital heart disease that affects one of the chambers of the heart (commonly the atria) as well as a valve (commonly the mitral valve). It is commonly known as both congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). Congenital (at birth) atrial septal defect refers to a hole being in the septum or wall that separates the two atria; this condition is usually seen in fetuses and infants. Mitral stenosis refers to mitral valve leaflets (or valve flaps) sticking to each other making the opening for blood to pass from the atrium to the ventricles very small. With the valve being so small, blood has difficulty passing from the left atrium into the left ventricle. Septal defects that may occur with Lutembachers syndrome include: Ostium primum atrial septal defect or ostium secundum which is more prevalent.Lutembachers syndrome affects females more often than males. It can affect children or adults; the person can either be born with the disorder or develop it later in life. The syndrome was first described by René Lutembacher (1884–1968) of Paris in 1916.To correct Lutembachers syndrome, surgery is often done. There are several types of surgeries depending on the cause of Lutembachers syndrome (ASD Primum or ASD Ostium Secundum with Mitral Stenosis):

Suturing (stitching) or placing a patch of tissue (similar to skin grafting) over the hole to completely close the opening
Reconstructing of the mitral and tricuspid valve while patching any holes in the heart
Device closure of ASD (e.g.
Same symptoms and causes as weight, gain, ankle edema, and right upper quadrant pain. Less common symptoms
Paroxysmal nocturnal dyspnea, orthopnea, and hemoptysis (sign of pulmonary venous congestion): this symptoms are less frequent in Lutembachers syndrome and are more associated with MS and small ASD or patients who develop reverse Lutembachers syndrome. This symptom is caused by mitral stenosis. Related disorders
Eisenmenger syndrome
Ventricular Septal Defect
Mitral regurgitation
Ebstein disease

Causes
Lutembacher is caused indirectly by heart damage or disorders. Lutembachers syndrome is caused by either birth defects where the heart fails to close all holes in the walls between the atria or from an episode of rheumatic fever where damage is done to the heart valves such as the mitral valve and resultant in an opening of heart wall between atria. With Lutembachers syndrome, a fetus or infant is usually seen to have a hole in their heart wall (interatrial) separating their right and left atria. Normally during fetal development, blood bypasses the lungs and is oxygenated from the placenta. Blood passes from the umbilical cord and flows into the left atrium through an opening called the foramen ovale; the foramen ovale is a hole between the two atria. Once a baby is born and the lungs begin to fill with air and the blood flow of the heart changes, a tissue flap (somewhat like a trap door) called the septum primum closes the foramen ovale or hole between the two atria and becomes part of the atrial wall.