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Lutembacher's syndrome

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A very rare form of angiocardiopathy. Heart disease in which atrial septal defect is combined with mitral stenosis. There is usually marked dilatation and hypertrophy of the right side of the heart. The dilated pulmonary artery not infrequently exceeds the size of the aorta. Predominantly in women; usually seen in young adults but may occur in elderly patients. The syndrome is usually congenital but may also be acquired due to rheumatic fever. A familial occurrence in two sisters has been reported.

The condition was first described in 1750 by Johann Friedrich Meckel, Senior (1724-1774), then in 1865 by Thomas Bevill Peacock (1812-1882), and in 1866 by Louis Martineau (1836-1888).

Bibliography

  • T. B. Peacock:
    On some of the causes and effects of valvular disease of the heart.
    Article. 1865.
  • L. Martineau:
    Des endocardites. Paris, 1866.
  • R. Lutembacher:
    De la sténose mitrale avec communication interauriculaire.
    Archives des maladies du coeur et des vaisseaux, Paris, 1916, 9: 237-260.

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