Lutembacher's syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-in-Chief: Ayokunle Olubaniyi, M.B,B.S

Overview

Lutembacher's syndrome is a rare form of congenital heart disease. It refers to a combination of congenital atrial septal defect complicated by an acquired mitral stenosis. The atrial septal defect is usually a specific type called a secundum atrial septal defect.

Epidemiology and Demographics

This is a very rare disease. The incidence is 0.001/1000000.

Complications and Prognosis

Complications are usually related to a late diagnosis. They include pulmonary hypertension and heart failure. Early diagnosis and surgical treatment has a good prognostic value.

Diagnosis

History and Symptoms

Symptoms depend on the size of ASD, extent of mitral stenosis and degree of changes in the pulmonary circulation. Presentation can be due to cardiac failure, atrial arrhythmias, dyspnoea, exercise intolerance, paradoxical emboli or other disease-related complications like pulmonary hypertension and infective endocarditis.

References

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