Chinese Medical Journal 2006; 119(4):351-352
Letter
Lutembacher syndrome: an ideal combination for
percutaneous treatment
T o the Editor : I read with interest the case report
by Shen et al1 of transcatheter treatment of
Lutembacher syndrome. Lutembacher syndrome
refers to a congenital atrial septal defect (ASD)
complicated by acquired mitral stenosis (MS).2 The
incidence of MS in patients with ASD is 4%, the
incidence of ASD in patients with MS is 0.6% －
0.7%.2 Lutembacher syndrome in the past has been
overdiagnosed or misdiagnosed because of the
presence of a mid-diastolic murmur in patients with
ASD, until it was realized that the mid-diastolic
murmur was due to excessive flow through the
tricuspid valve from the left-to-right interatrial shunt.
In patients with MS who had undergone
percutaneous balloon mitral valvuloplasty (PBMV)
through the transseptal approach, the latter
procedure created an ASD during the procedure:
the iatrogenic Lutembacher syndrome. The
incidence of atrial left-to-right shunt following PBMV
is 11% － 12%.3 In most of these patients, the
magnitude of the shunt was small and the defect
usually diminished in size or closed over the
ensuing months. In rare instances, the iatrogenic
ASD might be hemodynamically significant enough
to warrant surgical intervention.
Mitral stenosis serves to augment the left-to-right
atrial shunt through the ASD, but decompression of
the obstructed left atrium by the ASD attenuates the
symptoms of MS. When the ASD is sizable, it has
an ameriorating effect on the clinical manifestations
of MS. The ameriorating role of the ASD in MS was
evident in Lutembacher’s original report of 1916; the
patient was a 61-year-old woman who had been
pregnant seven times.4 An earlier case report in the
literature in 1880 (and referred to by Perloff 2) was of
a 74-year-old woman who had endured 11
pregnancies. Survival to advanced age has also
been reported;2 in one instance an 81-year-old
woman experienced no symptoms related to her
heart disease until she reached 75 years of age.5
351
These favorable reports, however, should not
obscure the fact that the long-term natural history of
ASD is unfavorably influenced by MS, which
augments the left-to-right shunt and predisposes to
atrial fibrillation and right ventricular failure.6 The
presence of MS, especially when accompanied by
mitral regurgitation, increases susceptibility to
infective endocarditis, in contrast to the low
incidence of infective endocarditis in uncomplicated
ASD.2 Therefore, these patients in the past usually
underwent corrective surgery as soon as the
diagnosis was established.
With the advent of percutaneous technique of
transcatheter closure of ASD and PBMV, Lutem-
bacher syndrome can now be successfully treated
nonsurgically.6 Because the Inoue technique has
been used in the largest number of patients around
the world7 with excellent long-term results,8 it has
become the procedure of choice for PBMV.9 The
Amplatzer septal occluder appears to be the device
of choice for transcatheter closure of ASD.10 By
combining these two techniques in the same patient
at the same cardiac catheterization, percutaneous
management of Lutembacher syndrome can obviate
the morbidity and mortality associated with cardiac
surgery, the psychological trauma of a thoracotomy
scar, the prolonged hospital stay followed by
another prolonged period of home convalescence
and the possibility of repeat thoracotomy for mitral
restenosis.6 In the latter situation, PBMV may be
repeated.8 Of course, the presence of the Amplatzer
septal occluder makes it undesirable to use the
transseptal approach. An alternative approach
would be transarterial retrograde nontransseptal
technique in which entry into the left atrium is
achieved retrogradely via the left ventricle.6
Tsung O. Cheng
Professor of Medicine
George Washington University Medical Center
Washington, D.C. 20037, U.S.A.
Email: tcheng@mfa.gwu.edu
352 Chin Med J 2006; 119(4):351-352

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Transcatheter treatment of Lutembacher syndrome. Am Heart J 1995; 129:1197-1203.
Chin Med J 2005;118:1843-1845. 8. Chen CR, Cheng TO, Chen JY, Huang YG, Huang T,
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York/Oxford: Igaku-Shoin; 1992: 269. 1998; 43:132-139.
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communication interauriculaire. Arch Mal Coeur 1916; valvuloplasty by the Inoue balloon technique: the
9:237-260. procedure of choice for treatment of mitral stenosis. Am
5. Rosenthal L. Atrial septal defect with mitral stenosis J Cardiol 1998; 81:624-628.
(Lutembacher’s syndrome) in a woman of 81. Br Med J 10. Omeish A, Hijazi ZM. Transcatheter closure of atrial
1956; 2:1351. septal defects in children and adults using the
6. Cheng TO. Coexistent atrial septal defect and mitral Amplatzer Septal Occluder. J Intervent Cardiol 2001;
stenosis (Lutembacher syndrome): an ideal 14:37-44.
combination for percutaneous treatment. Cathet (Received January 9, 2006)
Cardiovasc Intervent 1999; 48:205-206. Edited by WANG Mou-yue