Reverse Lutembacher syndrome

Reverse Lutembacher syndrome

Reverse Lutembacher syndrome is the term used to describe right to left interatrial shunt in the presence of severe tricuspid stenosis. Burke MA and Mikati IA described documented the combination of the lesions in a patient who presented with features of platypnea orthodeoxia syndrome [1]. This forty three year old lady had bioprosthetic tricuspid valve stenosis. Right to left shunt occurred across a patent foramen ovale (PFO). She also suffered from stroke due to paradoxical embolism across the PFO. Successful surgical treatment with replacement of the stenotic bioprosthetic valve with a new one and closure of the patient foramen ovale was achieved.

In another report by Essop MR and associates [2] described a patient who had undergone balloon dilatation of rheumatic mitral and aortic stenosis and later developed cyanosis and clubbing due to right to left shunt across the septal puncture defect. This was due to the development of severe tricuspid stenosis which occurred over a four year period. This case was dealt with by balloon dilatation of the stenosed tricuspid valve which lead to re establishment of left to right shunt across the atrial septal defect. Thus reverse Lutembacher syndrome was converted back to Lutembacher syndrome!

References

  1. Burke MA and Mikati IA. Platypnea-Orthodeoxia Syndrome Associated with Bioprosthetic Tricuspid Valve Stenosis and Reverse Lutembacher Syndrome. Circulation. 2011; 123: e222-e224.
  2. M R Essop, A R Essop, S Bedhesi, P E Sareli. Cyanosis and clubbing in a patient with iatrogenic Lutembacher syndrome. Eur Heart J. 1995;16:421-3.