Benign primary cardiac neoplasms are rare but may cause significant morbidity and mortality. However, they are usually treatable and can often be diagnosed with echocardiography, computed tomography (CT), or magnetic resonance (MR) imaging. Myxomas typically arise from the interatrial septum from a narrow base of attachment. Fibroelastomas are easily detected at echocardiography as small, mobile masses attached to valves by a short pedicle. Cardiac fibromas manifest as a large, noncontractile, solid mass in a ventricular wall at echocardiography and as a homogeneous mass with soft-tissue attenuation at CT. They are usually homogeneous and hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images. Paragangliomas usually appear as large, echogenic left atrial masses at echocardiography and as circumscribed, heterogeneous masses with low attenuation at CT. These tumors are usually markedly hyperintense on T2-weighted MR images and iso- or hypointense relative to myocardium on T1-weighted images. Cardiac lipomas manifest at CT as homogeneous, low-attenuation masses in a cardiac chamber or in the pericardial space and demonstrate homogeneous increased signal intensity that decreases with fat-saturated sequences at T1-weighted MR imaging. Cardiac lymphangiomas manifest as cystic masses at echocardiography and typically demonstrate increased signal intensity at T1- and T2-weighted MR imaging. Familiarity with these imaging features and with the relative effectiveness of these modalities is essential for prompt diagnosis and effective treatment.
Primary cardiac malignancies are rare tumors that are difficult to diagnose clinically. Different primary cardiac malignancies may have different clinical, morphologic, and radiologic features and intracardiac locations. Angiosarcoma is the most common primary cardiac malignancy. It tends to occur in the right atrium and involve the pericardium. Because of its tendency to hemorrhage, angiosarcoma often demonstrates areas of increased signal intensity with T1-weighted sequences. Undifferentiated sarcomas typically occur in the left atrium and have variable epidemiologic and radiologic features. Rhabdomyosarcoma is the most common primary cardiac malignancy in children and is more likely than other primary cardiac sarcomas to involve the valves. Primary cardiac osteogenic sarcoma almost always occurs in the left atrium and frequently demonstrates calcification. Certain features (eg, broad base of attachment, origin at a site other than the atrial septum) help differentiate this tumor from left atrial myxoma. Leiomyosarcoma favors the left atrium and tends to invade the pulmonary veins and mitral valve. Fibrosarcoma also tends to occur in the left atrium and is often necrotic. Liposarcoma is very rare and usually manifests as a large, infiltrating mass. Foci of macroscopic fat are occasionally seen. Primary cardiac lymphoma occurs more commonly in immunocompromised patients, frequently involves the pericardium, and, unlike other primary cardiac malignancies, may respond to chemotherapy. The advent of cross-sectional imaging has allowed earlier detection of primary cardiac malignancies as well as more accurate diagnosis and characterization.
Background-Although segmental or circumferential ablation is effective in eliminating pulmonary vein (PV)-mediated atrial fibrillation (AF), this procedure may be complicated by the occurrence of PV stenosis. Methods and Results-To establish the clinical presentation, diagnostic manifestations, and interventional management of PV stenosis, 23 patients with stenosis of 34 veins complicating ablation of AF were evaluated. Each patient became symptomatic 103Ϯ100 days after undergoing ablation. In 8 veins, the ablation producing the PV stenosis was a repeated procedure for continued AF. Nineteen patients presented with dyspnea on exertion, 7 with dyspnea at rest, 9 with cough, and 6 with chest pain. On multirow spiral computed tomography examination, the narrowest lumen of the affected PVs measured 3Ϯ2 mm compared with 13Ϯ3 mm at baseline (PՅ0.001). The relative perfusion of affected lung segments on isotope scans was reduced to 4Ϯ3% of total perfusion compared with 22Ϯ10% in unaffected segments. At percutaneous intervention, these veins showed 80Ϯ13% stenosis, with a mean gradient of 12Ϯ5 mm Hg. This was significantly reduced to a residual stenosis of 9Ϯ8% (PՅ0.001) and a residual gradient of 3Ϯ4 mm Hg (PՅ0.001).Twenty veins were treated with balloon dilatation alone, whereas 14 veins were stented with standard 10-mm-diameter bare-metal stents. Although the symptomatic response was nearly immediate and impressive, 14 patients developed in-stent or in-segment restenosis, requiring repeated interventions in 13. Conclusions-Percutaneous intervention produces rapid and dramatic symptom relief in patients with highly symptomatic PV stenosis after radiofrequency ablation for AF. Nevertheless, alternative treatment methods will be required to decrease recurrent in-stent or in-segment restenosis. (Circulation. 2005;111:546-554.)
Background-Electron-beam computed tomography (EBCT) is used to measure coronary calcification but not for aortic valve calcification (AVC). Its accuracy, association with aortic stenosis (AS) severity, and diagnostic and prognostic value with respect to AVC are unknown. Methods and Results-In 30 explanted aortic valves, the AVC score by EBCT (1125Ϯ1294 Agatston units [AU]) showed a strong linear correlation (rϭ0.96, PϽ0.0001) with valvular calcium weight (653Ϯ748 mg) by pathology that allowed estimation of calcium weight as AVC score/1.7, with a small standard error of the estimate (53 mg). In 100 consecutive clinical patients, we measured AVC by EBCT and AS severity by echocardiographic aortic valve area (AVA). The AVC score was 1316Ϯ1749 AU (range 0 to 7226 AU). Intraobserver and interobserver variabilities were excellent (4Ϯ4% and 4Ϯ10%, respectively). AVC and AVA were strongly associated (rϭ0.79, PϽ0.0001) but had a curvilinear relationship that suggested that AVC and AVA provide complementary information. AVC score Ն1100 AU provided 93% sensitivity and 82% specificity for diagnosis of severe AS (AVA Ͻ1 cm 2 ), with a receiver operator characteristic curve area of 0.89. AVC assessment by echocardiography was often more severe than by EBCT (PϽ0.0001). During follow-up, 22 patients either died, developed heart failure, or required surgery. With adjustment for age, sex, symptoms, ejection fraction, and AVA, the AVC score was independently predictive of event-free survival (risk ratio 1.06 per 100-AU increment [1.02 to 1.10], PϽ0.001), even after adjustment for echocardiographic calcifications. Conclusions-AVC is accurately and reproducibly measured by EBCT and shows a strong association and diagnostic value for severe AS. The curvilinear relationship between AVC and AVA suggests these measures are complementary, and indeed, AVC provides independent outcome information. Thus, AVC is an important measurement in the evaluation of patients with AS.
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