Lutembacher's syndrome refers to the presence of both an atrial septal defect and mitral valve stenosis.
Features:
(1) mitral valve stenosis, most often secondary to rheumatic fever
(2) atrial septal defect, which may be congenital or acquired
Factors affecting clinical findings:
(1) severity of the mitral stenosis and subsequently the left atrial pressure
(2) size of the atrial septal defect, which impacts the left-to-right shunt
(3) compliance of the right ventricle
(4) pulmonary vascular resistance
The size of the atrial septal defect depends on the cause. A typical congenital ASD has a diameter of 15 mm. An iatrogenic ASD (from transseptal catheterization) may be small.
Complications may include:
(1) pulmonary arterial hypertension
(2) infective endocarditis
(3) atrial fibrillation
(4) left and/or right heart failure
(5) biatrial enlargement
(6) Eisenmenger syndrome
