Lutembacher's syndrome: A rare combination of congenital and acquired heart disease – A case report and review of literature

Abstract

Lutembacher's syndrome is defined as the rare combination of congenital atrial septal defect and acquired mitral stenosis. The haemodynamic effects of this syndrome are a result of the interplay between the relative effects of the atrial septal defect and mitral stenosis. Mitral stenosis augments the left to right shunt through the atrial septal defect. The definition of Lutembacher's syndrome has undergone many changes. The earliest description in medical literature was found in a letter written by anatomist Johann Friedrich Meckel to Albrecht von Haller in 1750.In 1916, Lutembacher described his first case of this syndrome, involving a 61-year-old woman, and he attributed the mitral valvular lesion to congenital mitral stenosis. Because the mitral stenosis was, in fact, rheumatic in aetiology, the syndrome was defined eventually as a combination of congenital atrial septal defect and acquired, almost always rheumatic, mitral stenosis.

Keywords:Lutembacher's syndrome, congenital heart disease, valvular heart disease,  atrial septal defect, mitral stenosis