Lutembacher's syndrome

Lutembacher's syndrome is a form of congenital heart disease. Lutembacher's syndrome was first described by a French cardiologist by the name of Rene' Lutembacher (1884–1968) of Paris, France in 1916. Lutembacher syndrome is a rare disease that affects one of the chambers of the heart as well as a valve of the heart. Lutembacher's syndrome is known to affect females more often than males. Lutembacher is an extremely rare disease. Lutembacher's can affect children or adults; the person can either be born with the disorder or develop it later in life.Lutembacher affects more specifically the atria of the heart and the mitral or biscupid valve. The disorder itself is known more specifically 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 through the left atrium into the left ventricle. There are several types of septal defects that may occur with Lutembacher's syndrome: ASD Ostium Secundum or ASD (Primium); Ostium Secundum is the most prevalent.Lutembacher is caused indirectly as the result of heart damage or disorders and not something that is necessarily infectious. Lutembacher's 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 Lutembacher's 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 formaen 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 primium closes the foramen ovale or hole between the two atria and becomes part of the atrial wall. The failure of the hole between the two atria to close after birth leads to a disorder called ASD primium. The most common problems with an opening found in the heart with Lutembacher's syndrome is Ostium Secundum. Ostium Secundum is a hole that is found within the flap of tissue (septum primium) that will eventually close the hole between the two atria after birth. With either type of ASD, ASD will usually cause the blood flow from the right atrium to skip going to the right ventricle and instead flow to the left atrium. If mitral stenosis (the hardening of flap of tissue known as a valve which opens and closes between the left atrium and ventricle to control blood flow) is also present, blood will flow into the right atrium through the hole between the atria wall instead of flowing into the left ventricle and systemic circulation. Eventually this leads to other problems such as the right ventricle failing and a reduced blood flow to the left ventricle.In addition to the ASD, acquired MS can be present either from an episode of rheumatic fever (the mother has or had rheumatic fever during the pregnancy) or the child being born with the disorder (congenital MS). With the combination of both ASD and MS, the heart can be under severe strain as it tries to move blood throughout the heart and lungs. To correct Lutembacher's syndrome, surgery is often done. There are several types of surgeries depending on the cause of Lutembacher's syndrome(ASD Primium 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 Percutaneous transcatheter therapy Transcatheter therapy of balloon valvuloplasty to correct MS↑ ↑ 2.0 2.1 2.2 2.3 2.4 ↑ 3.0 3.1 3.2 3.3 3.4 ↑ ↑ ↑ 6.0 6.1 6.2 6.3 ↑