Echocardiographic assessment of Lutembacher syndrome

Abstract

A 59-year-old woman was a candidate for lumbar disc hernia surgery. She was complaining of shortness of breath on mild exertion. Preoperative cardiac assessment was performed. On physical examination, her blood pressure was 110/60 mm Hg and heart rate was 75 bpm. A grade II/VI holosystolic murmur and a diastolic rumble were heard at the cardiac apex. Electrocardiography (ECG) showed normal sinus rhythm with normal axis (Fig. 1A). Chest X-ray showed prominent hilar vasculature (Fig. 1B -red arrow), right atrial enlargement ( Fig. 1B -black arrow), and absence of left atrial enlargement (Fig. 1B). Evaluation by two-dimensional (2D) transthoracic echocardiography (TTE) revealed coexistence of ostium secundum atrial septal defect (ASD) and rheumatic mitral stenosis. Mitral valve leaflets appeared thickened and restricted in motion but calcification and subvalvular involvement could not be defined exactly (Figs. 2A, B). Planimetric mitral valve area (MVA) was 2.1 cm 2 , maximum diastolic gradient was 9 mm Hg, and mean gradient was 3 mm Hg. Also there was a mild-to-moderate mitral regurgitation. The diameter of ASD was estimated to be 2.2 cm 2 and colour flow mapping revealed left-to-right shunt across the defect (Figs. 2C, D). Subsequently, 3D TTE study was performed in order to achieve more accurate assessment of mitral valve apparatus. By 3D echocardiography (Figs. 2E, F), planimetric MVA was 1.7 cm 2 and subvalvular thickening including calcification was seen, indicating that limitations of 2D echocardiography should be recognised and determination of planimetric MVA should be performed accurately by 3D echocardiography before treatment. Soon after, the patient was referred for open-heart surgery. Lutembacher syndrome is a rare combination of congenital ASD and acquired mitral stenosis. Due to the limitations of 2D echocardiography, particularly for distinguishing calcification and subvalvular involvement and for accurate determination of planimetric MVA, mitral valve apparatus should be assessed by 3D echocardiography before treatment.

Lutembacher’s Syndrome in a young female treated Surgically: A Case Report

“…Patient with restrictive ASDs and moderate to severe MS, present much earlier and usually with features of pulmonary congestion from MS. 12 Two-dimensional echocardiography with color flow doppler is the diagnostic modality of choice in patients with Lutembacher's Syndrome (LS). The type and size of ASD and severity of MS are accurately estimated by this technique 13 .…”

Surgical Treatment of Lutembacher Syndrome with a Huge Right Atrium: A Case Report

“…Echocardiography is the gold standard for diagnosing LS. Given the limitations of 2-dimensional echocardiography, especially the identification of calcification and subvalvular involvement and accurate determination of the area of the MV orifice, 3-dimensional echocardiography should be used to evaluate the MV device before treatment [Tezcan 2014].…”