Lutembacher Syndrome

Lutembacher syndrome is a combination of mitral stenosis and a left-to-right atrial shunt, usually an ostium secundum atrial septal defect (ASD). Mitral stenosis is congenital or acquired (rheumatic heart disease). There is usually marked right ventricular hypertrophy and failure, and reduced blood flow to the left ventricle because blood flows back to the right atrium through the ASD.

Facts about Lutembacher Syndrome:

It is very rare.
Reported incidence is decreasing because of many fewer cases of rheumatic mitral valve disease.
Occurs predominantly in females.
Usually presents in young adults.

Presentation

Can present at any age.
Patients may remain asymptomatic for many years.
Symptoms are mainly due to the ASD. Signs and symptoms vary according to the size of the ASD.
Pulmonary congestion and symptoms due to right ventricular failure.
May have a history of rheumatic fever.
Predisposition to atrial arrhythmias (atrial fibrillation is very common).
Symptoms caused by mitral stenosis are seen less frequently in Lutembacher syndrome than in isolated mitral stenosis. They are more common in Lutembacher syndrome patients with a small ASD.

Surgery is now performed early rather than late because the rates of heart failure and cardiac arrhythmia increase with age. Patients with pulmonary hypertension and irreversibly increased pulmonary vascular resistance (Eisenmenger’s syndrome) invariably develop progressive right-sided heart failure after ASD closure and die.

Complications

Right heart failure
Atrial arrhythmias
Pulmonary congestion
Infective endocarditis
Eisenmenger’s syndrome (very uncommon with a large ASD and a high left atrial pressure due to the mitral stenosis)

Prognosis

Mortality and morbidity rates are related to the relative severity of the individual lesions.
Prognosis is generally good and patients have lived into their ninth decade without developing any cardiac symptoms.
Some women have had multiple pregnancies without complications.

Source: PatientPlus.com