Lutembacher syndrome (LS) was first described in a letter by anatomist Johann Friedrich Meckel in Corvisart who later described the. Lutembacher’s syndrome is the combination of mitral valve stenosis and a secundum-type atrial septal defect. These can be congenital or the mitral stenosis can. Lutembacher’s syndrome, on the other hand, is characterised by decompression of the LA through the atrial septal defect (ASD). As a result, the.

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Thus, there is notable cardiomegaly with prominent pulmonary artery pulsations and systolic thrill at the upper sternal border [23,27]. Occurrence of Lutembacher syndrome in a rural regional hospital: To treat ASD a device closure can be used. In addition to the ASD, MS can either be acquired present either from an episode of rheumatic fever or the mother has or had rheumatic fever during the pregnancy or congenital the child being born with the disorder.

The non-restrictive ASD allows the LA to decompress, thereby relieving the pulmonary venous hypertension.

Juvenile mitral stenosis and Lutembacher’s syndrome

Open in a separate window. Traditionally the gold-standard treatment has been open heart surgery.

Percutaneous balloon mitral valvuloplasty by the Inoue balloon technique: Don’t miss out Read your latest personalised notifications Ok, got it. There occurs a reciprocal decrease in the left ventricular filling and stroke volume. Mitral stenosis and left to right shunt at the atrial level.

Tex Heart Inst J. During the more common form of Lutembacher’s syndrome, ASD Ostium secundum, a hole will form in the flap of tissue septum primium that should close between the two atria after birth. The hospital stay for each group also varied, the surgical group was 3. The medical management of juvenile MS is similar to that of adults in the form lutembacehr diuretics and rate-controlling drugs.


Arch Mal Coeur ;9: Balloon mitral commissurotomy in juvenile rheumatic mitral stenosis: Mortality rates as low as 1.

The size of the ASD is calculated in the usual manner. The authors have no conflicts of interest to declare.

However, the calcification of mitral valve is less common in LS, as there occurs decompression of LA, resulting in less turbulent flow across the mitral valve. To treat Lutembacher’s syndrome, the underlying causes of the disorder must first be treated: Atrial septal defect ASD with rheumatic mitral stenosis is popularly known as eponym of Lutembacher syndrome. Opinion differs regarding what lesions the syndrome should include.

However, LS patients are often critically ill with deranged hepatic and renal parameters, making them high risk for open surgical repair. No MV calcification is seen.

Lutembacher’s syndrome

It needs to be emphasised that PTMC in the presence of a large ASD is challenging due to the lack of anchoring support of the interatrial septum IAS syndrlme makes the catheter unstable [33].

The incidence of this condition is very rare. A paediatric transseptal needle set is preferable. Moderate to severe pulmonary arterial hypertension is common and a majority will show hilar congestion. The electrocardiogram shows sinus rhythm in most patients [].


Her investigation reports were as follows: The hemodynamic features and natural history of the patients depend upon the size of the ASD, severity of MS, compliance of the right ventricle and the degree of pulmonary vascular resistance. J Comp Eff Res ;4: As a result of blood flow being stopped or slowed by the faulty valve, pressure begins to build in the heart. Atrial septal defect with mitral stenosis Lutembacher’s syndrome in a woman of A Transseptal puncture using a paediatric transseptal needle.

Planimetry is the more reliable method to assess the severity of MS in patients with LS. J Am Coll Cardiol ; Lutembacher’s syndrome is diagnosis primarily by physical examinations for heart sounds, electrocardiograms, chest radiogram, transthoracic or transesophageal echocardiography, color flow mapping, and Doppler imaging. Am Heart J ; Chest x-rays reveal enlarged RA, RV and main pulmonary artery with increased end-on pulmonary vascular markings suggesting increased left to right shunt.

Mahajanand Kuldeep B.

Juvenile mitral stenosis and Lutembacher’s syndrome

OMV will need a repeat procedure in the future for restenosis. Tricuspid leaflets are seen coapting surface to surface Click here to view.

The ASD may be congenital ostium secundum or sinus venosus type or acquired e. Two-dimensional echocardiography of a year-old boy with severe mitral stenosis.

Shigenobu M, Sano S.