Related Experiment Video
Updated: Jun 23, 2026

Chronic Ovine Model of Right Ventricular Failure and Functional Tricuspid Regurgitation
Published on: March 17, 2023
Progressive Right-Sided Heart Failure and Secondary Tricuspid Valve Disease in Pediatric Lutembacher Syndrome: A Case
Christopher Collin Hayes1, Kelly Chodos2, Bryan P Stefek1
1Department of Pediatric Cardiology, Pennsylvania State University Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA.
Background:
Lutembacher syndrome (LS) is a rare condition characterized by the coexistence of mitral stenosis (MS) and an atrial septal defect (ASD). Clinical manifestations vary according to the severity of MS and the size of the ASD.
Case Presentation:
We report a 13-year-old female with congenital MS and a secundum ASD who presented with decompensated right heart failure after being lost to follow-up for 10 years. Progressive worsening of MS and enlargement of the ASD resulted in significant left-to-right shunting, severe right heart volume overload, pulmonary hypertension, tricuspid annular dilation, and severe tricuspid regurgitation. Cardiac catheterization demonstrated low cardiac output, pulmonary overcirculation, and postcapillary pulmonary hypertension. The patient underwent mechanical mitral valve replacement, ASD closure, and tricuspid annuloplasty.
Outcome:
Postoperatively, the patient experienced transient left ventricular dysfunction that gradually improved with medical therapy. At 2-year follow-up, she demonstrated normal biventricular systolic function and complete resolution of heart failure symptoms.
Conclusion:
This case highlights how a large ASD may initially mask symptoms of severe MS while simultaneously promoting progressive right-sided heart failure and secondary tricuspid valve disease. Early surveillance and timely intervention are essential to prevent irreversible cardiac remodeling in patients with LS.
Related Concept Videos
Mitral Regurgitation I: Introduction
Mitral Stenosis I: Introduction
Mitral Stenosis II: Clinical features and Diagnostic Tests
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
Aortic Regurgitation I: Introduction
Cardiomyopathy II: Dilated Cardiomyopathy
