|Year : 2014 | Volume
| Issue : 3 | Page : 180-182
Percutaneous transcatheter treatment of Lutembacher syndrome
Mannuva Boochi Babu, Durgaprasad Rajasekhar, Velam Vanajakshamma
Department of Cardiology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
|Date of Web Publication||17-Sep-2014|
Mannuva Boochi Babu
Department of Cardiology, Sri Venkateswara Institute of Medical Sciences, Tirupati - 517 507, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Lutembacher syndrome is a rare combination of congenital ostium secundum atrial septal defect (ASD) and acquired rheumatic mitral stenosis. The gold standard treatment for this condition is the surgical closure of ASD and mitral valve replacement. Here, we report a case of Lutembacher syndrome treated percutaneously with a combined ASD closure with Cocoon septal occluder and balloon mitral valvotomy.
Keywords: Balloon mitral valvotomy, cocoon septal occluder, Lutembacher
|How to cite this article:|
Babu MB, Rajasekhar D, Vanajakshamma V. Percutaneous transcatheter treatment of Lutembacher syndrome. J NTR Univ Health Sci 2014;3:180-2
| Introduction|| |
Lutembacher syndrome, first described by Lutembacher, a French physician in 1916 is a combination of a congenital ostium secundum atrial septal defect (ASD) and acquired mitral stenosis (MS).  It is more common in females. Traditionally the condition is treated surgically.  With the introduction of percutaneous balloon mitral valvuloplasty (PBMV) and percutaneous transcathter closure of ASD nowadays, both diseases can be treated with percutaneous transcathter therapy in suitable patients and in those who are reluctant to the surgery.
| Case report|| |
A 42-year-old female patient presented to our outpatient department in 2009 with a history of dyspnea on exertion (from New York Heart Association class III), palpitations, and decreased exercise tolerance of 2 years duration. Physical examination of precordium showed tapping apical impulse, loud first heart sound, fixed splitting of the second heart sound, loud P2 component of second heart sound, opening snap, grade II/IV mid-diastolic rumbling murmur at the apex, grade 3/6 ejection systolic murmur at the pulmonary area and mid-diastolic murmur (MDM) at the tricuspid area. Twelve lead electrocardiogram showed sinus rhythm, incomplete right bundle branch block and biatrial enlargement. Transthoracic echocardiogram (TTE) showed enlargement of both atria and the right ventricle, severe MS with a mean diastolic gradient of 18 mmHg and maximum diastolic gradient of 28 mmHg across the mitral valve and valve area of 0.7 cm 2 calculated by planimetry. The Wilkins echo score was 6. Mitral regurgitation (MR) was trivial, tricuspid regurgitation was mild and the right ventricular systolic pressure was 46 mmHg. TTE also showed 12 mm ostium secundum ASD with left to right shunt. Transesophageal echocardiogram (TEE) demonstrated enlargement of both atria and the right ventricle, severe MS with mean diastolic gradient of 14 mmHg and maximum diastolic gradient of 27 mmHg, trivial MR without any clot in the left atrium and atrial appendage and 11 mm ostium secundum ASD with left to right shunt with adequate rims. Her coronary angiography was found to be normal. After explaining the treatment options, patient was taken up for transcatheter treatment. Through right femoral vein approach mitral valve was crossed and dilated with Accura PBMV balloon catheter (Vascular Concepts Limited, UK) up to 24 mm [Figure 1]a] after administering heparin (5000 U) under TTE guidance. The mean gradient fell from 18 mmHg to 4 mmHg after mitral balloon valvotomy. The mitral valve area increased to 2.0 cm 2 and a mild MR was observed. The stretched diameter of the ostium secundum ASD using a sizing balloon catheter was found to be 12 mm. Under TEE and fluoroscopy guidance a 14 mm Cocoon septal occluder (Vascular Innovations Co. Ltd., Thailand) was deployed across the ASD without any residual left-to-right shunt [Figure 1]b]. At 10 th day follow-up, the patient had good symptomatic improvement. After that, the patient was at regular 3-month follow-up and remained asymptomatic. At 5 years follow-up, TTE showed mitral valve area of 1.59 cm 2 by planimetry [Figure 2]a], mild MR and without left-to-right shunt across interatrial septum [Figure 2]b].
|Figure 1: Fluoroscopic images showing (a) dilatation of mitral valve; (b) deployment of cocoon septal occluder across the interatrial septum|
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|Figure 2: Two-dimensional transthoracic echocardiographic images showing (a) mitral valve area of 1.59 cm2 by planimetry; (b) color Doppler showing no fl ow across the atrial septal occluder|
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| Discussion|| |
Lutembacher syndrome refers to a rare combination of ASD and MS. The incidence of ASD in patients with MS is 0.6-0.7% and the incidence of MS in patients with ASD is 4%.  In the past Lutembacher syndrome has been misdiagnosed or over diagnosed because of the presence of a mid diastolic murmur (MDM) in patients with ASD, until it was recognized that the MDM was due to excessive flow through the tricuspid valve from the left-to-right shunt across the interatrial septum.
The incidence of atrial left-to-right shunt following PBMV (iatrogenic Lutembacher syndrome) is 11-12%.  In most of these patients, the magnitude of the shunt was small, and the defect usually decreased in size or even closed over the ensuing months.
The hemodynamic effects of these two conditions are worth mentioning. In patients with Lutembacher syndrome, the natural history of MS is favorably influenced by ASD, which decompresses the left atrium, reduces the mitral gradient, and improves symptoms associated with MS. Moreover, in this condition, the mitral valve measurements should be determined by planimetry or a continuity equation because the Doppler pressure half-time method overestimates the mitral valve area.  In the presence of ASD, the transmitral pressure gradient is generally lower than expected for the degree of the MS, thereby falsely underestimating the pressure half-time and overestimating the mitral valve area. The natural history of ASD is unfavorably influenced by the presence of MS, which augments the left-to-right shunt caused by ASD and predisposes atrial fibrillation and right ventricular failure. 
Traditionally the condition is treated surgically.  Nowadays however; both the conditions are amenable to percutaneous transcatheter therapy. Percutaneous treatment of Lutembacher syndrome can overcome the morbidity and mortality associated with cardiac surgery, the psychological trauma of a thoracotomy scar and the possibility of repeat thoracotomy for mitral restenosis.  Indications for percutaneous intervention in these patients include:
- ASD with a Qp/Qs ratio of >1.5 with adequate rims,
- Symptomatic moderate to severe MS with valve morphology favorable for PBMV,
- Any degree of pulmonary hypertension, except in individuals with irreversible pulmonary hypertension (Eisenmenger syndrome). Contraindications to percutaneous therapy include:
- Left atrial blood clot,
- Grade 3 or higher MR,
- Bicommissural calcification,
- Lack of sufficient rims around the septal defect and presence of anomalous pulmonary venous drainage,
- Lack of expertise.
1992, Ruiz et al. first described the percutaneous treatment of the Lutembacher syndrome.  Later in 1999, Joseph et al. described the procedure of percutaneous treatment of Lutembacher syndrome with a 16-mm Amplatzer septal occluder and the Joseph mitral balloon catheter for MS.  Since then, around twelve cases have been reported all over the world. ,,,,,,,,,,,
This is a rare condition, and few case reports of percutaneous treatment of Lutembacher syndrome are available. Almost all reported immediate successful results. Because there is no long-term follow-up data available, we could not comment on the long-term prognosis of this therapy. Repeat PBMV can be performed in these patients if there is a recurrence of severe MS. However, since the trans-septal approach cannot be used due to the Cocoon septal occluder, PBMV can be done using the retrograde nontrans-septal approach in these patients.  To the best of our knowledge, this is the fifth reported case of Lutembacher syndrome in India to undergo a combined percutaneous treatment intervention. Our case shows the successful and sustained benefit of percutaneous treatment of Lutembacher syndrome even after 5 years.
| Conclusion|| |
Percutaneous transcatheter closure of ASD and mitral valvotomy is the treatment of choice for Lutembacher syndrome until and unless the lesions are incongruous for the procedures.
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[Figure 1], [Figure 2]