Lutembacher's syndrome
For patient information, click here.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S [2]
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-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, or even a patent foramen ovale (PFO) complicated by an acquired mitral stenosis.[1] The atrial septal defect is usually a specific type called a secundum atrial septal defect. Iatrogenic Lutembacher’s syndrome has also been reported.[2]
References
- ↑ Goldman 2011, pp. 400
- ↑ Sadaniantz A, Luttmann C, Shulman RS, Block PC, Schachne J, Thompson PD (1990). “Acquired Lutembacher syndrome or mitral stenosis and acquired atrial septal defect after transseptal mitral valvuloplasty”. Cathet Cardiovasc Diagn. 21 (1): 7–9. PMID 2208272.
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S
Historical Perspective
Lutembacher’s syndrome was named after René Lutembacher, a french cardiologist, who described the syndrome in 1916.[1]
References
- ↑ Shen XQ, Zhou SH, Zhou T, Qi SS, Fang ZF, Lv XL (2005). “Transcatheter treatment of Lutembacher syndrome”. Chin Med J (Engl). 118 (21): 1843–5. PMID 16336826.
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S
Overview
Pathophysiology
The presence of both ASD and mitral stenosis occuring together, usually modify the clinical and hemodynamic manifestation of each other. The presence of an ASD creates a second exit (left-to-right shunt) for the blood in the left atrium; consequently reducing the hemodynamic effects of a severe mitral stenosis. In the same fashion, the pressure in the left atrium, pulmonary veins and the pulmonary capillaries decrease if the ASD is large. Therefore, the typical presentation of mitral stenosis as a result of increased hydrostatic pressure such as orthopnea, paroxysmal nocturnal dyspnea, hemoptysis and pulmonary edema are attenuated or diminished, and are often substituted by symptoms of low volume output such as weakness and fatigue.[1]
References
- ↑ Olivares-Reyes A, Al-Kamme A (2005). “Lutembacher’s syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement”. J Am Soc Echocardiogr. 18 (10): 1105. doi:10.1016/j.echo.2005.01.017. PMID 16198889.
Epidemiology and Demographics
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S
Overview
Lutembacher’s syndrome is a rare disease with an incidence of approximately 0.001/1000000.
Epidemiology and Demographics
The incidence is approximately 0.001/1000000.[1] This syndrome is more frequently seen in adults because the mitral stenosis is usually an acquired valvulopathy of rheumatic origin. It is also more commonly observed in female patients because both ASD and MS are more prevalent in this gender.[2]
References
- ↑ Berry NS, Bauman JL, Gallastegui JL, Bauma W, Beckman KJ, Hariman RJ (1988). “Analysis of antiarrhythmic drug concentrations determined during electrophysiologic drug testing in patients with inducible tachycardias”. Am J Cardiol. 61 (11): 922–4. PMID 3354470.
- ↑ Olivares-Reyes A, Al-Kamme A (2005). “Lutembacher’s syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement”. J Am Soc Echocardiogr. 18 (10): 1105. doi:10.1016/j.echo.2005.01.017. PMID 16198889.
Natural History, Complications and Prognosis
Natural History | Complications | Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S
Overview
Natural History, Complications and Prognosis
Complications are usually related to a late diagnosis. They include pulmonary hypertension, heart failure, and infective endocarditis. Early diagnosis and surgical treatment has a good prognostic value.
References
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Electrocardiogram | X-Ray | Echocardiography
Looking for the patient version?
© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH