Association of an atrial septal defect with congenital or acquired mitral valve stenosis. This disease is more common in less developed countries, where rheumatic fever is still common.
The hemodynamic effects depend on the interaction between the two malformations:
- tight mitral stenosis and large ASD: the ASD allows decompression of the dilated LA; pulmonary venous hypertension develops less rapidly, but there is an increase in pressures in the RA and RV, with a consequent increase in pulmonary flow and a risk of hyperkinetic pulmonary hypertension (reversible then irreversible increase in pulmonary artery muscularis)
- tight mitral stenosis and restrictive ASD: the evolution is that of classic mitral stenosis, with pulmonary venous hypertension, pulmonary arterial vasoconstriction and progressive obliteration of the pulmonary arterioles.
The risk of mitral valve calcification is lower than in isolated mitral stenosis. Risk of atrial fibrillation.
Treatment: either open-heart surgery or balloon dilatation (or commissurotomy) of the mitral valve and endovascular closure of the ASD.
Anesthetic implications:
preoperative cardiac echocardiography to determine the severity of the mitral stenosis and the importance of the interatrial shunt, as well as presence and extent of pulmonary hypertension; prevention of endocarditis. Management of mitral stenosis: fixed cardiac output, avoidance of volume overload, very cautious IV induction (very patient titration of low-dose hypnotics). Prevent any increase in pulmonary resistance.
References :
- Kulkarni SS, Sakaria AK, Mahajan SK, Shah KB.
Lutembacher’s syndrome.
J Cardiovasc Dis Res 2012;3:179-81
- Aminde LN, Dzudie A, Takah NF, et al.
Current diagnostic and treatment strategies for Lutembacher syndrome: the pivotal role of echocardiography.
Cardiovasc Diagn Ther 2015 ; 5:122-32.
- Turki S, Arya A, Kajal K, Gourav KP.
Neuraxial anesthesia in Lutembacher’s syndrome? Yes, we can!
Saudi J Anesth 2023 ;17 : 416-8.
Updated: January 2024