Catheterization and Cardiovascular Interventions 50:68 70 (2000)

Transcatheter Treatment of a Case of

Lutembacher Syndrome
Elaine M.C. Chau,* Chi-Hang Lee, and Wing-Hing Chow
Lutembacher syndrome refers to the rare combination of congenital atrial septal defect
and acquired mitral stenosis. This condition is usually treated by mitral valve operation
with concomitant closure of the atrial septal defect. We describe a case of Lutembacher
syndrome that was treated successfully with percutaneous transcatheter mitral commis-
surotomy using the Inoue balloon and closure of the atrial septal defect with the Am-
platzer atrial septal defect occluder. Cathet. Cardiovasc. Intervent. 50:68 70, 2000.
2000 Wiley-Liss, Inc.

Key words: Lutembacher syndrome; mitral stenosis; atrial septal defect

INTRODUCTION DISCUSSION

A 26-year-old Chinese female presented to her gen-
eral practitioner with increased dyspnea and was found
to have severe mitral stenosis and a secundum atrial
defect on echocardiogram. The mitral valve area cal-
culated from the continuity equation was 0.8 cm2. The
valve was pliable with little calcification and there was
no significant mitral regurgitation. The diameter of the
secundum atrial defect was estimated to be 1.06 cm
1.26 cm on transesophageal echocardiogram. After
informed consent was given, the patient underwent
cardiac catheterization. This showed the presence of a
left-to-right shunt at the atrial level with a ratio of
pulmonary flow to systemic flow of 4.5. The systolic
pulmonary artery pressure was 33 mm Hg. The mitral
valve area was calculated to be 0.61 cm2 by Gorlins
method. The mitral valve was crossed and dilated
twice with an Inoue balloon catheter set at 25 mm and
at 26 mm, respectively (Fig. 1A). There was good
hemodynamic improvement with reduction of mean
mitral valve gradient from 12 to 2 mm Hg. The stretch
diameter of the secundum atrial septal defect was
determined using a sizing catheter to be 18 mm. A
20-mm Amplatzer atrial septal defect occluder was
deployed under transthoracic echocardiography (Fig.
1B). Chest X-ray performed on the following day
showed a significant decrease in heart size (Fig. 2).
Transthoracic echocardiogram showed a mitral valve
area of 1.8 cm2 with a mean gradient of 4 mm Hg. The
atrial septal defect occluder was seen in situ with no
left-to-right shunt (Fig. 3). At 1-month follow-up after
the combined procedure, the patient was well with
good symptomatic improvement.
The combination of congenital atrial septal defect and
acquired mitral stenosis was described by Lutembacher
[1] in 1916. The hemodynamic effects of these two
coexisting conditions are interesting. Mitral stenosis aug-
ments the left-to-right shunt through the atrial septal
defect while the nonrestrictive atrial septal defect decom-
presses the left atrium, reducing the mitral valve gradi-
ent. In this condition, the mitral valve area should be
determined by the Doppler continuity equation or
planimetry because the Doppler pressure half-time
method is an inaccurate measure of mitral valve area [2].
Traditionally, this condition was corrected by surgical
treatment [3]. The use of percutaneous treatment of
Lutembacher syndrome was first described by Ruiz et al.
[4] in 1992. Combined umbrella closure of the atrial
septal defect with Locks clamshell occluder in conjunc-
tion with mitral and aortic balloon valvotomies was car-
ried out as a rescue procedure in a 43-year-old female
with recurrent atrial septal defect, severe aortic and mi-
tral stenosis, and pulmonary hypertension. Unfortu-
nately, that patient died suddenly at 8 weeks after the
procedure. In our case, the transcatheter treatment was
considered curative rather than palliative. The devices
employed were also different from those used by Ruiz et
al. [4]. Nowadays, the Inoue balloon is widely used for
percutaneous balloon mitral valvotomy. The Amplatzer
Department of Cardiology, Grantham Hospital, Hong Kong,
China
*Correspondence to: Elaine M.C. Chau, Grantham Hospital, 125
Wong Chuk Hang Road, Aberdeen, Hong Kong, China.
Received 18 August 1999; Revision accepted 21 October 1999

2000 Wiley-Liss, Inc.

 Transcatheter Treatment 69

Fig. 1. A: Dilatation of the mitral valve using the Inoue balloon.

B: Deployment of the Amplatzer septal occluder after percuta-
neous balloon mitral valvotomy.

atrial septal defect occluder, on the other hand, is a new

self-centering device with a high complete closure rate of
93%97% at 1 month of follow-up [5,6]. The advantages
of this device in comparison with other septal occluders Fig. 2. Chest radiographs before (A) and after (B) the com-
include simple delivery system using smaller-sized bined procedure showing a marked decrease in heart size and
sheaths, retrievability, and absence of sharp corners or disappearance of the pulmonary plethora.
spokes. The combined use of these two devices has
70 Chau et al.

quicker recovery from the procedure, and short hospital

stay.

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