INTRODUCTION

Lutembacher syndrome (LS) was first described by Johann Friedrich Meckel in 1750. In 1811, Corvisart described the association of atrial septal defect (ASD) with mitral stenosis. However, the first comprehensive report of these two defects was made by Rene Lutembacher in 1916, and the syndrome was named after him.

LS is a condition in which there is a combination of mitral valve stenosis and ASD^[1]. LS with a sinus venosus-type interatrial communication (IAC) in association with anomalous pulmonary vein drainage is very rare.

A 42-year-old female patient presented to a cardiology outpatient clinic due to dyspnea on light exertion, associated with palpitations, during which she had nausea and chest discomfort. She had suffered with plegia and muscle atrophy in her right upper limb since she was three years old, which was possibly attributed to poliomyelitis sequel. She reported no cough, orthopnoea, or paroxysmal nocturnal dyspnea.

A physical examination revealed an irregular heart rhythm, jugular turgor, a diastolic murmur in the mitral area (2+/6+), and a systolic murmur in the tricuspid area (3+/6+). The electrocardiogram showed atrial fibrillation with an axis deviation to the right (Figure 1).

The patient also had a transthoracic echocardiogram showing an opening in the dome of the mitral valve with significant mitral stenosis, biatrial and right ventricular enlargements, moderate/significant tricuspid insufficiency, mild pulmonary arterial hypertension, and pericardial effusion with no hemodynamic repercussions (Figure 2).

A coronary angiotomography was requested to investigate the possibility of associated coronary artery disease, which demonstrated pericardial effusion, a discrete myocardial bridge in the middle third of the anterior descending artery with no luminal reduction, thickened mitral valve leaflets and reduced opening (estimated valve area at 0.6 cm2), and the presence of ground-glass images in both lung fields (Figure 3).

During surgical exploration, the mitral valve presented with very thick leaflets and a commissural fusion promoting severe stenosis. The subvalvular apparatus was fused with intense fibrosis, with no technical conditions for the preservation of the native valve.

In the surgical procedure for valve replacement with a 27-mm mechanical prosthesis and tricuspid commissuroplasty using the Kay technique to correct tricuspid insufficiency, superior sinus venosus-type IAC was identified with superior pulmonary vein return to the superior vena cava. The IAC was closed, and the pulmonary vein was diverted to the left atrium (Figure 4).

QUESTIONS

BRIEF CONSIDERATION OF THE CASE REPORTED

LS is a condition in which there is an association of mitral valve stenosis with ASD. LS with sinus venosus-type IAC associated with anomalous pulmonary vein return is very rare. During cardiac surgery for mitral valve replacement with a mechanical prosthesis and tricuspid commissuroplasty, upper sinus venosus-type IAC was identified with a right superior pulmonary vein return into the superior vena cava.

A surgical approach is the traditional therapy for the treatment of LS^[9]. In this case, the patient was referred for surgery given her condition of significant pericardial effusion and severe mitral stenosis, presenting with good clinical development after the procedure.

LEARNING POINTS