Abstract

Atrial septal defect (ASD) coexisting with mitral valve regurgitation has been described in literature with various aetiologies and pathophysiologic mechanisms. Mitral valve lesions coexisting with an ASD could either be congenital or acquired. The most prominent congenital mitral valve pathology is a cleft in the anterior leaflet of the mitral valve, as seen in patients with partial atrio-ventricular septal defects. Acquired mitral valve lesions include, hemodynamic induced annular dilatation resulting in significant mitral regurgitation, iatrogenic leaflet perforation and coexisting diseases such as infective endocarditis or rheumatic valve disease. However, the aetiology of the mitral valve regurgitation being due to rheumatic heart disease is uncommon. We report the case of a 12-year-old female who presented with easy fatigability, palpitation and a precordial bulge dating about 2 years. Examination revealed cardiac murmurs in the apex and pulmonary areas and transthoracic echocardiography confirmed the presence of an Ostium Secundum ASD and features of severe rheumatic mitral valve regurgitation. Child was initially placed on medications which included diuretics and an Angiotensin converting enzyme inhibitor ACEi, before she eventually had surgical closure of ASD and mitral valve replacement. The purpose of this case report is to highlight the symptomatology, diagnosis and treatment of this rare association, particularly in our environment.

Highlights

  • Atrial septal defect (ASD) with rheumatic mitral stenosis is popularly known as Lutembacher syndrome; the association of ASD and rheumatic mitral regurgitation has been described, but remain unnamed

  • Atrial septal defect (ASD) coexisting with mitral valve regurgitation has been described in literature with the incidence rate of 4 to 9.1% [2]

  • Congenital lesions of the mitral valve, as seen in partial atrioventricular septal defects (AVSD) is frequently a cleft in the mitral valve leaflet and other congenital abnormalities of the mitral valve resulting in insufficiency have been described [3]

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Summary

Introduction

Atrial septal defect (ASD) with rheumatic mitral stenosis is popularly known as Lutembacher syndrome; the association of ASD and rheumatic mitral regurgitation has been described, but remain unnamed. A recent report from India proposed that ASD with rheumatic mitral regurgitation should be included in the definition of Lutembacher syndrome [1]. Atrial septal defect (ASD) coexisting with mitral valve regurgitation has been described in literature with the incidence rate of 4 to 9.1% [2]. The rheumatic aetiology of the mitral regurgitation (MR) remains an important and distinct entity, which needs to be recognized in our environment. Olugbenga Olalekan Ojo et al.: Atrial Septal Defect with Rheumatic Mitral Regurgitation:

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