Lutembacher's syndrome
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Lutembacher's syndrome is a very rare[1] 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).[2] 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.[3] 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.[4] With the valve being so small, blood has difficulty passing from the left atrium into the left ventricle. 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 | |
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This condition affects the atrium | |
Specialty | Medical genetics |
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. The syndrome was first described by René Lutembacher (1884–1968)[5] of Paris in 1916.[2]
To correct Lutembacher's syndrome, surgery is often done. There are several types of surgeries depending on the cause of Lutembacher's 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. Amplatzer umbrella or CardioSEAL to seal the hole)[2][citation needed]
- Percutaneous transcatheter therapy[2]
- Transcatheter therapy of balloon valvuloplasty to correct MS[2]