Definitive Percutaneous Treatmentof Lutembacher’s Syndrome

George Joseph, *† MD, DM, K. Abhaichand Rajpal, MD, DM, and K.P. Suresh Kumar, MD, DM

Definitive percutaneous treatment of a patient with Lutembacher’s syndrome wassuccessfully accomplished using the Amplatzer septal occluder to close a secundumatrial septal defect and the Joseph mitral balloon catheter to dilate rheumatic mitral valvestenosis. Transcatheter therapy is an effective alternative to surgery in selected patientswith Lutembacher’s syndrome. Cathet. Cardiovasc. Intervent. 48:199–204, 1999.r 1999 Wiley-Liss, Inc.

Key words: atrial septal defect; mitral valve stenosis; balloon dilatation

INTRODUCTION

Lutembacher’s syndrome, a combination of congenitalatrial septal defect (ASD) and acquired mitral stenosis[1], has traditionally been treated by open heart surgery[2]. Percutaneous treatments of ASD, and that of mitralstenosis, have been separately described more than 15years ago [3,4]. Whereas transcatheter device closure ofASD has yet to gain wide acceptance [5–11], percutane-ous balloon valvuloplasty has become an establishedmethod of treatment of mitral stenosis [12–16]. Thesetwo interventions have been concurrently performed as apalliative rescue procedure, in a patient with Lutembach-er’s syndrome and severe pulmonary hypertension, toimprove risk prior to surgical therapy [17]. However,definitive percutaneous treatment of Lutembacher’s syn-drome has not been reported. We present a patient withLutembacher’s syndrome, in whom definitive percutane-ous treatment was successfully accomplished, by concur-rent transcatheter device closure of a secundum ASD andballoon valvuloplasty of rheumatic mitral stenosis.

CASE REPORT

A 23-year-old male presented with a 2-year history ofbreathlessness on exertion. At the time of admission tohospital, he was in New York Heart Association func-tional class 2. Heart disease had been detected severalyears earlier, and he had received rheumatic chemoprophy-laxis for the past 10 years. Physical examination revealedauscultatory features of mitral stenosis (loud first heartsound, opening snap, and mid-diastolic murmur at theapex). Additionally, there were features suggestive of

ASD (wide splitting of the second heart sound withsystolic ejection murmur at the pulmonary area). Theelectrocardiogram showed sinus rhythm, right axis devia-tion, and incomplete right bundle branch block. The chestX-ray revealed cardiomegaly and pulmonary plethora.Transthoracic and transesophageal echocardiography wasperformed (Fig. 1A). Both atria and the right ventriclewere enlarged. An 11-mm-diameter secundum ASD witha wide rim of atrial septum on all sides was present. Allpulmonary veins drained normally into the left atrium.The mitral valve was thickened and moderately stenosed,consistent with rheumatic heart disease. Two-dimen-sional planimetry was preferred over Doppler echocardi-ography in determining the mitral valve area (Table I)[18]. The mitral valve had preserved mobility, no calcifi-cation, and a morphologic score of 5 out of 16 [19]. Colorflow mapping revealed left-to-right shunt across the ASD(Fig. 1B), mild mitral regurgitation, mild aortic regurgita-tion, and moderate tricuspid regurgitation. As both theASD and the mitral stenosis appeared suitable for defini-

Department of Cardiology, Christian Medical College Hospital,Vellore, India

†In accordance with the policy of the Journal, the designated authordiscloses a financial or other interest in the subject discussed in thisarticle.

*Correspondence to: Dr. George Joseph, Department of Cardiology,Christian Medical College Hospital, Vellore 632 004, South India.E-mail: gjoseph59@hotmail.com

Received 18 January 1999; Revision accepted 31 March 1999

Catheterization and Cardiovascular Interventions 48:199–204 (1999)

r 1999 Wiley-Liss, Inc.

tive percutaneous treatment, concurrent transcathetertherapy was planned.

After informed consent, mild sedation and antibioticprophylaxis, right femoral arterial and venous access wasobtained, and 5,000 units of heparin was administered.Right and left heart catheterization, oximetry run, and leftventricular angiography were performed (Table I). Themean left atrial pressure and transmitral gradients wererelatively low, in spite of significant mitral stenosis,probably because of left atrial decompression via theASD (Fig. 2A). The stretched diameter of the ASD,

determined using a sizing balloon (Meditech, BostonScientific, Watertown, MA), was found to be 16 mm. Themitral valve was crossed using a balloon floatationcatheter, and an appropriately shaped 0.035’’ valvulo-plasty backup wire (Schneider, Minneapolis, MN) waspositioned with its tip at the left ventricular apex. Themitral valve was dilated using a 25-mm-diameter cylindri-cal single balloon (Joseph mitral balloon catheter, Numed,Ontario, Canada; Fig. 3), taking care to ensure that no partof the balloon was across the atrial septum. This abol-ished the transmitral pressure gradient (Fig. 2B), and

Fig. 1. A: Two-dimensional echocardiogram in apical four-chamber view showing stenoticmitral valve (vertical arrow) and atrial septal defect (horizontal arrow). B: Color flow imaging inthe same view showing left-to-right shunt through the atrial septal defect.

200 Joseph et al.

echocardiography now showed good opening of themitral valve. Repeat left ventricular angiogram showedno increase in mitral regurgitation. Next, the ASD wasclosed using a 16-mm Amplatzer septal occluder (AGAMedical, Golden Valley, MN), deployed using an 8 Frdelivery system, under transthoracic echocardiographicand fluoroscopic guidance. Subsequently, both echocardio-graphic color flow imaging (Fig. 4) and levophasepulmonary angiography (Fig. 5) showed complete clo-sure of the ASD. Right heart catheterization and oximetryrun were repeated. There were no complications during orafter the procedure. Echocardiography repeated 48 hrlater showed stable device position, absence of flowacross the atrial septum, and a mitral valve area of 2.2cm2. The patient was asymptomatic at the time ofdischarge from hospital.

DISCUSSION

Interest in transcatheter closure of ASD has increasedwith the arrival of the Amplatzer septal occluder [9–11].The reasons for the enthusiasm to use this device are thatits deployment is simple, quick, and can be done throughsmall (6 to 8 Fr) introducers; its self-centering abilityrenders higher complete closure rates compared to otherdevices; and device retrieval or repositioning prior torelease is easy [20,21]. Large multicenter trials arerequired to determine the true status of the Amplatzerseptal occluder vis-a-vis the other ASD closure devices.Though it will be a while before such information isavailable, and current use of this device remains investi-gational, the promising initial results with the Amplatzerseptal occluder has prompted some investigators to usethis device in more complex situations. Hakim et al. [21]

used the Amplatzer septal occluder to close an ASD in apatient with dextrocardia. Fontes et al. [22] reportedconcurrent closure of two ASDs and a patent ductusarteriosus in a child, using two Amplatzer septal occlud-ers and a Gianturco coil respectively. Yip et al. [23]described simultaneous transcatheter valvuloplasty forcongenital pulmonary valvar stenosis and Amplatzerdevice occlusion of a secundum atrial septal defect in onepatient.

The single-balloon mitral valvuloplasty technique us-ing the Joseph mitral balloon catheter (Numed) [24],which was utilized in the present case, is considerablysimpler and quicker than the conventional double-balloontechnique. This over-the-wire cylindrical balloon, which

TABLE I. Echocardiographic and Hemodynamic Data a

Variable Before After

EchocardiographyMitral valve area by

planimetry 1.1 cm2 2.2 cm2

Mitral regurgitation mild mildAngiography

Mitral regurgitation Grade 1/4 Grade 1/4Oximetry

Pulmonary artery saturation 83.4% 70.7%Oxygen step-up 21.9% 3.2%Qp/Qs 2.47:1 1.06:1

Pressure dataPulmonary artery pressure

[S/D (M)] 29/11 (18) mm Hg 18/8 (12) mm HgLeft atrial mean pressure 9 mm Hg 4 mm HgMean transmitral pressure

gradient 4 mm Hg 0.6 mm HgaBefore and after refer to data acquired pre- and postmitral valvuloplastyand atrial septal defect closure; Qp/Qs, pulmonary/systemic blood flowratio; S/D (M), systolic/diastolic (mean) pressures.

Fig. 2. Left atrial and left ventricular pressure tracings. Shadedarea represents diastolic transmitral pressure gradient. A: Be-fore mitral valvuloplasty. B: After mitral valvuloplasty.

Lutembacher’s Syndrome 201

expands to a fixed size, has several advantages. The thin,tough thermoplastic balloon material gives large bal-loons, up to 30 mm in diameter, a low crossing profile(septal dilatation using a 14 Fr dilator is sufficient, thoughnot required in this case because of the ASD). The short,blunt tip of the catheter prevents left ventricular perfora-tion and allows satisfactory positioning of balloon acrossthe mitral valve even if there is marked subvalvarcrowding or if the left ventricle is small. The 11 Frcatheter shaft with unique coaxial design allows veryrapid balloon inflation and deflation. Procedure costs arereduced considerably because a single balloon and wireare used (as compared to two wires and balloons in the

conventional double-balloon technique), the balloon it-self is cheap, and an additional balloon is not required forseptal dilatation. When compared to the Inoue balloontechnique, the present technique has comparable efficacy,safety, and simplicity [24]. However, the cost of theJoseph balloon catheter is less than half that of the Inoueballoon catheter, making the procedure cheaper even if asecond balloon of larger diameter is required for furtherdilatation. As in the present case where only one balloonwas used, a second balloon of larger diameter is notrequired in most cases.

To our knowledge, this is the first report of definitivepercutaneous treatment of Lutembacher’s syndrome, by acombination of transcatheter device closure of a secun-dum ASD and balloon mitral valvuloplasty. Prior to us,Ruiz et al. [17] have described concurrent transcatheterASD closure using Lock’s clamshell occluder [25] anddouble-balloon mitral and aortic valvotomies, performedas a palliative rescue procedure, in a patient with Lutem-bacher’s syndrome and severe pulmonary hypertension,to improve risk prior to surgical therapy. The patient,however, died before surgical repair could be undertaken.In the present case, a good split of the mitral valve andcomplete closure of the ASD were obtained withoutcomplications, and the patient was rendered asymptom-atic at the time of discharge from hospital. The procedurecan therefore be considered as definitive therapy, eventhough follow-up has not been carried out. The likelihoodof mitral restenosis occurring is small [26], but in caserepeat mitral valvuloplasty is required in this patientsome years later, the presence of the Amplatzer septaloccluder may make it undesirable to use the transseptal

Fig. 3. Mitral valve dilatation using the Joseph mitral balloon. Awaist is seen on the balloon, produced by the stenotic mitralvalve.

Fig. 4. Postprocedure echocardiography in apical four-chamber view showing good opening ofthe mitral valve (vertical arrow) and Amplatzer septal occluder in place across the atrial septum(horizontal arrow). Absence of flow across the atrial septum is evident.

202 Joseph et al.

approach. The retrograde nontransseptal approach [27]may be a better option in this situation.

In conclusion, definitive percutaneous treatment ofLutembacher’s syndrome is feasible in selected patientswith suitable septal anatomy and mitral valve morphol-ogy. With the presently available transcatheter tech-niques, this procedure can be safely and effectivelycarried out and should be considered an alternative tosurgical therapy.

ACKNOWLEDGMENT

We thank V. Sitapathy for photographic assistance.

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