|Year : 2014 | Volume
| Issue : 3 | Page : 215-219
Definitive treatment of lutembacher syndrome
Rajat Arora, Suresh V Patted, Prabhu C Halkati, Basavaprabhu Amarked, Ameet Sattur, Amit Joshi
Department of Cardiology, Jawaharlal Nehru Medical College, Karnataka Lingayat Education Society University, Belgaum, Karnataka, India
|Date of Web Publication||19-Sep-2014|
Department of Cardiology, JN Medical College KLES University, Nehru Nagar,Belgaum, Karnataka
Source of Support: None, Conflict of Interest: None
Atrial septal defect (ASD) with rheumatic mitral stenosis is popularly known as eponym of Lutembacher syndrome. Definitive treatment of Lutembacher syndrome has been under the purview of cardiothoracic surgeons but with changing times, transcatheter treatment has been possible. We present a case of 38-year-old female with Lutembacher syndrome treated transcatheterly. This case attests the feasibility of transcatheter treatment in selected group of patients of Lutembacher treatment.
Keywords: Atrial/therapy, balloon dilatation, heart catheterization, heart septal defects, Lutembacher syndrome/therapy, mitral valve stenosis/therapy
|How to cite this article:|
Arora R, Patted SV, Halkati PC, Amarked B, Sattur A, Joshi A. Definitive treatment of lutembacher syndrome. J Sci Soc 2014;41:215-9
| Introduction|| |
The association of atrial septal defect with mitral stenosis carries the eponym of Lutembacher's syndrome (1916), , though according to Soulie, Bouvrain, and Sibille (1954), it had been described a century earlier by Corvisart.
Until recently, surgery was the only definite curative treatment available and involved closure of the ASD and mitral commissurotomy or mitral valve replacement. 
With the advent of interventional cardiology, comprehensive treatment of Lutembacher syndrome has come under the purview of cardiologists.
We present a patient with Lutembacher syndrome, who underwent definitive percutaneous treatment by concurrent transcatheter device closure of a secundum ASD and balloon valvuloplasty of rheumatic mitral stenosis.
| Case report|| |
A 38-year-old female first noted dyspnea on exertion while doing her routine physical activity. Patient had been diagnosed to have rheumatic heart disease and was on penicillin prophylaxis for the same.
At the time of admission, patient was in New York Heart Association functional Class II. Clinical examination demonstrated loud first sound and second heart sound was widely split and "fixed" with a loud, palpable pulmonary component. A loud mid diastolic murmur from the left sternal border to the apex without presystolic accentuation was heard.
Chest X-ray showed gross cardiomegaly with pulmonary plethora [Figure 1]. Electrocardiogram revealed sinus tachycardia, right axis deviation with Rsr' pattern [Figure 2].
|Figure 1: Chest X-ray posterior-anterior view shows cardiomegaly and pulmonary plethora|
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|Figure 2: Electrocardiography shows sinus tachycardia, Rsr' a ern, and le atrial enlargement|
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The typical echocardiographic features of mitral stenosis in association with an enlarged right ventricle and abnormal septal motion are generally consistent with pulmonary hypertension and functional tricuspid regurgitation. In the absence of clinical and echocardiographic features of pulmonary hypertension, the combination of mitral stenosis and "volume overload" of the right ventricle should alert the clinician to the diagnosis of Lutembacher's syndrome. 
Transthoracic echocardiogram revealed dilated right atrium, right ventricle, and left atrium. Left ventricle appeared underfilled. There was severe rheumatic mitral stenosis with mitral valve orifice of 0.9 cm 2 . There was moderate subvalvular fusion without any valvular calcification with a calculated Wilkins Score of 7.  A large ostium secundum ASD measuring
2.23 cm was seen with good margins with left to right shunt [Figure 3], [Figure 4], [Figure 5].
|Figure 3: Trans thoracic echocardiography (TTE) in apical fourchamber view shows large ostium secundum ASD with good margins, with le to right shunt. Mitral valve is thickened|
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|Figure 4: TTE in short axis view shows mitral valve area of 0.9 cm2 by planimetry. Note-Thickened anterior mitral leafl et (AML) and posterior mitral leafl et (PML) with fusion of anterolateral and posteromedial commissures|
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|Figure 5: TTE in parasternal long-axis view shows thickened AML and PML with inverted hockey appearance of AML. Note-Minimal turbulence at mouth of mitral valve in presence of severe mitral stenosis that is characteristic in presence of associated ASD|
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With this underlying profile of severe mitral stenosis with large ostium secundum ASD suitable for percutaneous treatment, a definitive curative transcatheter treatment was planned.
After informed consent, mild sedation and antibiotic prophylaxis, right femoral artery and vein assess was secured and 2,500 units of heparin were administered.
Routine left and right heart catheterization was performed. This showed the presence of left to right shunt at atrial level with the pulmonary-to-systemic flow ratio (Qp/Qs) of 3.2. The mean pulmonary pressure was 35 mm Hg. The mean pressure of the left atrium was 8 mm Hg [Table 1].
The mitral valve was crossed and with an Inoue balloon. mitral valve was dilated with a balloon diameter of
24 mm [Figure 6].
A 0.035 × 260 cm J Tip Amplatz super Stiff wire (Amplatz Super Stiff™ Guidewire, Boston Scientific corporation, Miami, USA) was kept in the left upper pulmonary vein with the help of 7F GL catheter. Then device was placed across the ASD and the position of the device was confirmed both on the transthoracic echocardiography and fluoroscopy. A 28 mm Amplatzer ASD device (AMPLATZER™ Septal Occluder St. Jude Medical, Inc., One St. Jude Medical Drive,USA) was deployed first without success and then upsized to 32 mm with complete occlusion of the defect without any left to right shunt [Figure 7].
Postprocedure TTE demonstrated ASD device in situ with no residual shunt. Mitral valve was opening well with both anterolateral and posteromedial commissures well split and MVO of 1.84 cm 2 by planimetry [Figure 8] and [Figure 9].
|Figure 8: Postprocedure TTE demonstrates ASD deviCe in situ with no residual le to right shunt. Peak and mean gradient by continuous wave Doppler across mitral valve is seen|
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|Figure 9: Postprocedure TTE across mitral valve in short axis view demonstrates MVO of 1.84 cm2 by planimetry|
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There were no complications during the procedure and patient was discharged 2 days later.
| Discussion|| |
The original case of Lutembacher syndrome was a 61-year-old woman who had been pregnant 7 times.  The incidence of mitral stenosis (MS) in patients with ASD is 4%, and conversely, ASD in patients with MS is 0.6%-0.7%. 
Mitral stenosis, at the bedside, is recognized by the combination of a loud first sound, normally split second sound, an opening snap, and a loud mid diastolic murmur with presystolic accentuation. An associated atrial septal defect decompresses the left atrium, resulting in the absence of presystolic accentuation of the diastolic murmur. Then flow across the tricuspid valve, which is relatively large because of the mitral obstruction, results in a loud mid diastolic murmur. The mitral diastolic murmur becomes inseparable from the tricuspid flow murmur and both together are heard as a loud middiastolic murmur from the left sternal edge to the apex. 
In Lutembacher syndrome, MS augments the left-to-right shunt through the ASD, while the nonrestrictive ASD decompresses the LA, reducing the diastolic mitral pressure gradient. That means the Doppler pressure half-time method would underestimate the degree of MS; this occurred in our patient. The planimetry method yields accurate estimates of mitral valve area in Lutembacher syndrome. 
Joseph et al.,  reported the first case of definitive percutaneous treatment of Lutembacher's syndrome, by a combination of transcatheter device closure of a secundum ASD and balloon mitral valvuloplasty. 
There, the mitral valve was dilated using a25-mm-diameter cylindrical single balloon (Joseph mitral balloon catheter, (Jomiva,™ Numed, Hopkinton, New York) and the ASD was closed using a 16-mm Amplatzer septal occluder (AMPLATZER™ Septal Occluder St. Jude Medical, Inc., One St. Jude Medical Drive, USA)
Prior to them, Ruiz et al.,  have described concurrent transcatheter ASD closure using Lock's clamshell occluder and double-balloon mitral and aortic valvotomies, performed as a palliative rescue procedure, in a patient with Lutembacher's syndrome and severe pulmonary hypertension, to improve risk prior to surgical therapy.
There is currently no randomized or cross-sectional study, which investigates combined percutaneous treatment in patients with Lutembacher syndrome. However, the success rate of combined percutaneous treatment in case studies is very high and no short- or long-term complications have been reported. ,,,,,, On the contrary, the complete closure rate of ASD by the septal occluder device during combined percutaneous treatment was found to be 93%-97%. 
The most important complication of the procedure is embolization of the septal occluder device. The procedure of retrieval and reimplantation is implemented when there is embolization of small devices; however, surgical interventions are required for large devices. 
The success rate of the other percutaneous intervention with BMV by the transseptal Inoue balloon technique was 99%. 
The life-threatening complication rate (death, puncture in the left ventricle, and stroke) was very low (0%-0.5%) and the risk of developing important and severe complications (grade 3-4 mitral regurgitation, cardiac tamponade, and thromboembolism) was found to be lower when compared to the other procedures. 
The combined percutaneous treatment technique is reported to reduce the mortality and morbidity risk associated with cardiac surgery, to decrease physiologic trauma due to thoracotomy scar and to reduce the surgery-related length of stay in the hospital in patients with Lutembacher syndrome. 
We report a successful definitive transcatheter treatment of Lutembacher syndrome in a symptomatic young lady.
In conclusion, transcatheter treatment of Lutembacher syndrome can be the procedure of choice in selected group of patients with mitral valve stenosis and ostium secundum ASD suitable for percutaneous treatment with good, short, and long-term results.
| References|| |
|1.||Lutembacher R. De la stenosemitrale avec communication interauriculaire. Arch Mal Coeur 1916;9:237-60. |
|2.||Tandon R, Manchanda SC, Roy SB. Mitral stenosis with left-to-right shunt at atrial level. A diagnostic challenge. Br Heart J I971;33:773-81. |
|3.||Shigenobu M, Sano S. Surgical indications and treatment of mitral valve disease associated with secundum atrial septal defect with special reference to left ventricular geometry and function. J Cardiovasc Surg (Torino) 1994;35:469-74. |
|4.||Forman HR, Kotler MN, Segal BL, Parry WR. Lutembacher's syndrome: Recognition by echocardiography. J Clin Ultrasound 1979;7:53-6. |
|5.||Wilkins GT, Weyman AE, Abascal VM, Block PC, Palacios IF. Percutaneous balloon dilatation of the mitral valve: An analysis of echocardiographic variables related to outcome and the mechanism of dilatation. Br Heart J 1988;60:299-308. |
|6.||Perloff JK. The Clinical Recognition of Congenital Heart Disease, 4th ed. Philadelphia: Saunders; 1994. p. 323-8. |
|7.||Vasan RS, Shrivastava S, Kumar MV. Value and limitations of Doppler echocardiographic determination of mitral valve area in Lutembacher syndrome. J Am Coll Cardiol 1992;20:1362-70. |
|8.||Joseph G, Abhaichand Rajpal K, Kumar KP. Definitive percutaneous treatment of Lutembacher's syndrome. Catheter Cardiovasc Interv 1999;48:199-204. |
|9.||Ruiz CE, Gamra H, Mahrer P, Allen JW, O'Laughlin MP, Lau FY. Percutaneous closure of a secundum atrial septal defect and double balloon valvotomies of a severe mitral and aortic valve stenosis in a patient with Lutembacher's syndrome and severe pulmonary hypertension. Cathet Cardiovasc Diagn 1992;25:309-12. |
|10.||Wiedemann HR. Earliest description by Johann Friedrich Meckel, Senior (1750) of what is known today as Lutembacher syndrome (1916). Am J Med Genet 1994;53:59-64. |
|11.||Chau EM, Lee CH, Chow WH. Transcatheter treatment of a case of Lutembacher syndrome. Catheter Cardiovasc Interv 2000;50:68-70. |
|12.||Ledesma M, Martinez P, Cazares MA, Feldman T. Transcatheter treatment of Lutembacher syndrome: Combined balloon mitral valvuloplasty and percutaneous atrial septal defect closure. J Invasive Cardiol 2004;16:678-9. |
|13.||Shabbir M, Ahmed W, Akhtar K. Transcatheter Treatment of Lutembacher's syndrome. J Coll Physicians Surg Pak 2008;18:105-6. |
|14.||Aroney C, Lapanun W, Scalia G, Parsonage W. Transcatheter treatment of Lutembacher syndrome. Intern Med J 2003;33:259-60. |
|15.||Shen XQ, Zhou SH, Zhou T, Qi SS, Fang ZF, Lv XL. Transcatheter treatment of Lutembacher syndrome. Chin Med J (Engl) 2005;118:1843-5. |
|16.||Cheng TO. Coexistent atrial septal defect and mitral stenosis (Lutembacher syndrome): An ideal combination for percutaneous treatment. Catheter Cardiovasc Interv 1999;48:205-6. |
|17.||Cheng TO, Holmes DR Jr. Percutaneous balloon mitral valvuloplasty by the Inoue balloon technique: The procedure of choice for treatment of mitral stenosis. Am J Cardiol 1998;81:624-8. |
|18.||Thanopoulos BD, Laskari CV, Tsaousis GS, Zarayelyan A, Vekiou A, Papadopoulos GS. Closure of atrial septal defects with the Amplatzer occlusion device: Preliminary results. J Am Coll Cardiol 1998; 31:1110-6. |
|19.||Wilson NJ, Smith J, Prommete B, O'Donnell C, Gentles TL, Ruygrok PN. Transcatheter closure of secundum atrial septal defects with the Amplatzer septal occluder in adults and children-follow-up closure rates, degree of mitral regurgitation and evolution of arrhythmias. Heart Lung Circ 2008;17:318-24. |
|20.||Chen CR, Cheng TO. Percutaneous balloon mitral valvuloplasty by the Inoue technique: A multicenter study of 4832 patients in China. Am Heart J 1995;129:1197-203. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]