A Rare Cause of “Functional” Mitral Stenosis

A 78-year-old woman was urgently admitted to our hospital with a diagnosis of heart failure. Her electrocardiogram was in sinus rhythm, and chest X-ray showed bilateral pleural effusion (Picture 1). Computed tomography showed a tumor filling the left atrium (Picture 2, red arrows), but transthoracic echocardiography failed to detect a tumor in the left atrium due to poor observation (Picture 1). Preoperative coronary angiography revealed multiple nutrient vessels from both coronary arteries (Picture 2, white arrowheads). Emergent surgery was performed, and intraoperative transesophageal echocardiography showed a large tumor originating from the atrial septum that fit into the mitral valve during diastole and caused “functional” mitral stenosis (Picture 3). Intraoperative and macroscopic gross images and a pathological examination indicated the diagnosis of intimal sarcoma (Picture 4). Pre- and postoperative right heart catheterization findings showed marked improvement in hemodynamic parameters (pulmonary artery wedge pressure 34→13 mmHg, pulmonary artery pressure 56/32/40→23/12/16 mmHg, central venous pressure 12→7 mmHg, cardiac index 1.2→2.2 L/min/m²). Cardiac tumors are rare, especially intimal sarcoma, which is the least-reported primary tumor of the heart. Intimal sarcoma generally arises from great vessels and has a poor prognosis (1). When intimal sarcoma becomes a large tumor filling the left atrium, it can be a rare cause of “functional” mitral stenosis resulting in heart failure (2).

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The authors state that they have no Conflict of Interest (COI).