Lutembacher syndrome (LS) was first described in a letter by anatomist Johann Friedrich Meckel in 1750. .Corvisart who later described the association of atrial septal defect (ASD) and mitral stenosis (MS) in 1811. However, the first comprehensive account of these two defects was reported by a French physician Rene Lutembacher in 1916, after whom this syndrome was eventually named. He described his first case of this syndrome in a 61-year-old woman and attributed the mitral valvular lesion to congenital mitral stenosis (MS). The definition of LS has changed many times since then. Opinion differs regarding what lesions the syndrome should include. Although defined as MS in combination with ASD, some authors also classify ASD with mitral regurgitation (MR) as a part of the LS spectrum. However, the current consensus defines LS as any combination of ASD (congenital or iatrogenic) and MS (congenital or acquired). In a typical case with LS, the ASD is usually more than 15 mm in size. However, in the current era of percutaneous balloon mitral valvuloplasty (BMV) for acquired MS, residual iatrogenic ASD secondary to trans-septal puncture is more common than the congenital ASD. Physicians refer to this as iatrogenic LS.In a typical case with LS, the ASD is usually more than 15 mm in size.