Primary Cardiac Leiomyosarcoma: When Valvular Disease Becomes a Vascular Surgical Emergency
A 50-year-old previously healthy woman was referred to our department for suspected mitral valve endocarditis because of a 3-week history of fever, weight loss, and the echocardiographic discovery of a mitral valve mass. On admission, the patient was cachectic (body mass index of 18 kg/m 2 ) and subfebrile (37.5°C) with dyspnea at rest, jugular vein distension, and tender liver enlargement. Her heart examination showed regular tachycardia (100 bpm), apical systolic murmur (3/6 degree), and
... 6 degree), and diastolic rumble. The ECG showed sinus tachycardia (105 bpm) and signs of left atrial abnormality (wide, notched P wave measuring 140 ms; Figure 1 ). Chest x-ray revealed a cardiothoracic index of 0.6, with left atrial enlargement and interstitial edema ( Figure 2 ). Echocardiography showed an enlarged left atrium and a partially mobile mass on the mitral valve involving mainly the anterior leaflet and the anterolateral commissure ( Figure 3A and 3B and Movies I and II in the online-only Data Supplement). This caused severe obstruction of the left ventricular inflow tract, with a mean transmitral gradient of 12 mm Hg and a functional valve area of 1 cm 2 (Figure 4 ). Moderate mitral regurgitation was also noted. Transesophageal echocardiography confirmed the presence of a 22ϫ11-mm highly mobile mass on the mitral valve oscillating between the left atrium and the left ventricle during the cardiac cycle ( Figure 3C and 3D and Movie III in the online-only Data Supplement). No other intracardiac masses were detected. Shortly after admission, the patient presented a transient episode of left calf pain, paresthesia, and reduced power in the left lower limb that lasted for 30 minutes with complete recovery. Abdominal ultrasonography with color Doppler showed a filling defect at the level of aortic bifurcation and origin at the common iliac arteries ( Figure 5 ). The episode was interpreted as a possible peripheral embolic event. Coronary angiography showed normal coronary arteries, but injection into the abdominal aorta revealed a round filling defect at the level of the aortic bifurcation ( Figure 6 ). Immediately afterward, echocardiography was repeated and showed the disappearance of the mobile part of the mass (Movie IV in the online-only Data Supplement), with a decrease in the transmitral mean gradient (Figure 7) . Emergency surgical removal of both the intracardiac and intraaortic portions of the tumor was performed simultaneously Figure 1 . Twelve-lead ECG shows sinus rhythm, signs of left atrial abnormality (wide, notched P wave measuring 140 ms), and nonspecific ST-T changes.