A rare case report of iatrogenic Lutembacher’s syndrome with cyanosis and hemodynamic insights

Background: Iatrogenic Lutembacher’s syndrome is a rare condition defined by the presence of an acquired atrial septal defect (ASD) along with rheumatic mitral stenosis, frequently resulting from transseptal interventions like balloon mitral valvotomy (BMV).

Case Presentation: We report the case of a 39-year-old woman with rheumatic heart disease who presented with breathlessness, central cyanosis and peripheral oedema for 10 days. She had previously undergone BMV twice. Clinical evaluation indicated signs of right heart failure and cyanosis. An ECG revealed atrial fibrillation with a rapid ventricular response, and chest radiography showed cardiomegaly. Echocardiography indicated moderate mitral stenosis, significant tricuspid regurgitation, tricuspid stenosis, right ventricular dysfunction, and two residual iatrogenic ASDs with bidirectional shunting, predominantly from right to left. The patient's condition improved with conservative management. Although a closure procedure was initially planned, the heart team decided to postpone the intervention due to the severe right ventricular dysfunction. The patient has been under regular follow-up for the past one and a half years, during which her symptoms have been well-controlled.

Conclusions: Persistent iatrogenic ASDs can result in significant hemodynamic consequences, including hypoxemia, especially when there are elevated pressures on the right side of the heart. This case highlights the hemodynamic importance of persistent iatrogenic ASDs and underscores the need for individualized decision-making based on a collaborative heart team approach in their management.