A 36-year-old male, was admitted to our Heart department: He experienced progressive dyspnea on effort and at rest. During the physical examination, he was found to have low blood pressure (90/60 mmHg), sinusal tachycardia and gallop rhythm. An olosistolic murmur was heard in the mitral area. Rales were available on pulmonary auscultation. The electrocardiogram (ECG) revealed sinus rhythm and left ventricular hypertrophy (Fig. 1).
Chest x-ray showed left atrial and ventricular enlargement; alveolar edema in the hilar regions of both lung fields.
The echocardiogram (TTE) showed left ventricular enlargment, atrial dilatation, moderate/severe mitral regurgitation and moderate aortic regurgitation; mitral valve masses were suspected.
Based on this diagnosis, the patient underwent a transesophageal-echo (TEE).
TEE showed, on apical 4-chamber section, an anulare structure attached from a membrane to the atrial wall anterior mitral valve leaflet and just proximal to the posterior mitral leaflet (Fig. 2, 3).
This supravalvular ring was proximal to the left atrial appendage, in contradistinction to "cor triatriatum"; it restricted the leaflets movement and impaired their opening. A severe mitral regurgitation (IV grade PISA) and mild diastolic gradient (6 mm/Hg DP mean) was detected by the Doppler-echocardiography (see additional file 1).
Additional File 1: TEE showed, on apical 4-chamber section, an anulare structure attached like a membrane to the atrial wall anterior mitral valve leaflet and just proximal to the posterior mitral leaflet. This supravalvular ring was proximal to the left atrial appendage; a severe mitral regurgitation (IV grade PISA) and a mild diastolic gradient (6 mm/Hg DP mean) was detected by the Doppler-echocardiography. A moderate/severe aortic regurgitation was seen. (MPG 2 MB)
A moderate/severe aortic regurgitation was seen. Mitral vegetations were not identified. Coronary angiography was normal; left cineventriculography showed a severe aortic and mitral regurgitation, but a mitral supravalvular structure was not noticed.
Based on TEE diagnosis, the patient underwent surgery in November 2002.
A left atriotomy enabled the identification of a membrane right above the mitral valve with 2 small openings that allowed blood to flow from the atrium to the left ventricle. The membrane was excised and the mitral and aortic valves were replaced. Follow up: after 6 month, the patient was in I NYHA class. The transtoracic echo showed a normal function of prostesis.