Lutembacher Syndrome Associated With Pulmonary Hypertension: The Importance of Early Diagnosis for Enabling Surgical Treatment

Introduction

Lutembacher syndrome (LS) is defined by the simultaneous presence of an atrial septal defect (ASD) and mitral stenosis (MS). Although it is a rare condition, its clinical significance lies in the considerable hemodynamic impact and the potential for early progression to irreversible pulmonary hypertension (PH).

The exact prevalence of LS remains unknown. However, for estimation purposes, MS is considered the most frequent manifestation of rheumatic fever (RF) in young women, and the prevalence of RF in developing countries ranges from 5 to 10 cases per 1,000 children. Furthermore, the incidence of congenital ASD in patients with MS is estimated at 0.6% to 0.7%.

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