Mitral stenosis and hockey stick, do not always mean rheumatic valve disease

Excerpt

A 21-year-old woman comes to the office for six-month of progressive dyspnea. Relevant history of heart murmur and intellectual disability. Transthoracic echocardiography (TTE) showed a left ventricle with preserved dimensions and contractility, the left atrium was severely dilated (area of 37.3 cm²). The anterior leaflet of the mitral valve was arranged as a “dome” or “hockey stick” (Fig. 1, Panel A), while the posterior one exhibited restricted movement. Likewise, the apical 4-chamber view showed 4-chamber sclerosis of the leaflets and thickening of the subvalvular apparatus (Panel B). Parasternal short-axis view presented suboptimal acoustic window. Doppler assessment showed moderate mitral regurgitation (2 jets) with severe stenosis (mean gradient of 30 mmHg) and moderate probability of pulmonary hypertension (pulmonary artery systolic pressure of 41.4 mmHg). These morphological and functional characteristics suggested mitral rheumatic disease. 2D-3D transesophageal approach (TEE) surprisingly revealed redundant anterior mitral leaflet, circular mitral stenosis (basal papillary muscles joined by a fibrous bridge (Panel C) and a rudimentary tendinous chord with the opening area by 3D planimetry: 0.5cm² (Panel D). Conclusive findings in hammock mitral valve.

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