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Review
. 2015 Apr;5(2):122-32.
doi: 10.3978/j.issn.2223-3652.2015.03.07.

Current diagnostic and treatment strategies for Lutembacher syndrome: the pivotal role of echocardiography

Leopold Ndemnge Aminde  1 , Anastase Dzudie  1 , Noah Fongwen Takah  1 , Kathleen Blackett Ngu  1 , Karen Sliwa  1 , Andre Pascal Kengne  1
Affiliations
Review

Current diagnostic and treatment strategies for Lutembacher syndrome: the pivotal role of echocardiography

Leopold Ndemnge Aminde et al. Cardiovasc Diagn Ther. 2015 Apr.

Abstract

Lutembacher syndrome (LS) is a rare cardiac abnormality characterized by any combination of a congenital or iatrogenic atrial septal defect (ASD) and a congenital or acquired mitral stenosis (MS). Clinical features and hemodynamic effects of LS depend on the balance of effects of the MS and the ASD. Prognosis is influenced by several factors [pulmonary vascular resistance, right ventricle (RV) compliance, size of ASD and MS severity] but the occurrence of secondary pulmonary hypertension and congestive heart failure is commonly associated with poor outcome. Echocardiography remains the gold standard for diagnosis and evaluation of LS. Timely diagnosis is critical for modifying the natural course, by allowing patients to benefit from currently available percutaneous trans-catheter therapies with favorable effects on the outcomes. This article is a review of published literature on the current diagnostic and therapeutic modalities for LS, focusing on the pivotal role of echocardiography as the key diagnostic tool. Clinical suspicion of LS should prompt extensive investigation with non-invasive and where possible, invasive technics. Multicenter registers have a potential to assist the evaluation of long term outcomes of percutaneous trans-catheter therapies in patients with LS.

Keywords: Lutembacher syndrome (LS); atrial septal defect (ASD) diagnosis; echocardiography; mitral stenosis (MS); trans-catheter therapy.

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Figures

Figure 1
Figure 1
2D echocardiographic short axis view showing severe MS. Adapted with permission from Arora et al. (16). 2D, two-dimension; MS, mitral stenosis.
Figure 2
Figure 2
(A) 2D echocardiographic apical view showing a large ASD; and (B) a colour flow mapping demonstrating shunt across. Adapted with permission from Arora et al. (16). 2D, two-dimension; ASD, atrial septal defect.
Figure 3
Figure 3
3D echocardiogram showing mitral valve and sub-valvular apparatus. Adapted with permission from Tezcan et al. (35). 3D, three dimension.
Figure 4
Figure 4
Inoue balloon for mitral valve dilation. Adapted with permission from Arora et al. (16).
Figure 5
Figure 5
Amplatzer septal occluder for ASD. Adapted with permission from Arora et al. (16). ASD, atrial septal defect.
See this image and copyright information in PMC

References

    1. Kengne AP, Mayosi BM. Readiness of the primary care system for non-communicable diseases in sub-Saharan Africa. Lancet Glob Health 2014;2:e247-8. - PubMed
    1. Kumar S, Walters TE, Halloran K, et al. Ten-year trends in the use of catheter ablation for treatment of atrial fibrillation vs. the use of coronary intervention for the treatment of ischaemic heart disease in Australia. Europace 2013;15:1702-9. - PubMed
    1. Mocumbi AO, Ferreira MB. Neglected cardiovascular diseases in Africa: challenges and opportunities. J Am Coll Cardiol 2010;55:680-7. - PubMed
    1. Nagamani AC, Nagesh CM. Lutembacher Syndrome (Ch. 64). In: Vijayalakshmi IB, Syamasundar Rao P, Chugh R. eds. A Comprehensive Approach to Congenital Heart Diseases. India: Jaypee Brothers Medical Publisher, 2013:908-16.
    1. Bashi VV, Ravikumar E, Jairaj PS, et al. Coexistent mitral valve disease with left-to-right shunt at the atrial level: clinical profile, hemodynamics, and surgical considerations in 67 consecutive patients. Am Heart J 1987;114:1406-14. - PubMed