LGE is common in CA and detects interstitial expansion from amyloid deposition. Global transmural or subendocardial LGE is most common, but suboptimal myocardial nulling and focal patchy LGE are also observed. LGE-CMR may detect early cardiac abnormalities in patients with amyloidosis with normal left ventricular thickness. The presence and pattern of LGE is strongly associated with clinical, morphologic, functional, and biochemical markers of prognosis.

Background-Primary amyloidosis has a poor prognosis as a result of frequent cardiac involvement. We recently reported a high prevalence of intracardiac thrombus in cardiac amyloid patients at autopsy. However, neither the prevalence nor the effect of anticoagulation on intracardiac thrombus has been evaluated antemortem. Methods and Results-We studied all transthoracic and transesophageal echocardiograms of cardiac amyloid patients at the Mayo Clinic. The prevalence of intracardiac thrombosis, clinical and transthoracic/transesophageal echocardiographic risks for intracardiac thrombosis, and effect of anticoagulation were investigated. We identified 156 patients with cardiac amyloidosis who underwent transesophageal echocardiograms. Amyloidosis was the primary type (AL) in 80; other types occurred in 76 patients, including 56 with the wild transthyretin type, 17 with the mutant transthyretin type, and 3 with the secondary type. Fifth-eight intracardiac thrombi were identified in 42 patients (27%). AL amyloid had more frequent intracardiac thrombus than the other types (35% versus 18%; Pϭ0.02), although the AL patients were younger and had less atrial fibrillation. Multivariate analysis showed that atrial fibrillation, poor left ventricular diastolic function, and lower left atrial appendage emptying velocity were independently associated with increased risk for intracardiac thrombosis, whereas anticoagulation was associated with a significantly decreased risk (odds ratio, 0.09; 95% CI, 0.01 to 0.51; PϽ0.006). Conclusions-Intracardiac thrombosis occurs frequently in cardiac amyloid patients, especially in the AL type and in those with atrial fibrillation. Risk for thrombosis increased if left ventricular diastolic dysfunction and atrial mechanical dysfunction were present. Anticoagulation therapy appears protective. Timely screening in high-risk patients may allow early detection of intracardiac thrombus. Anticoagulation should be carefully considered. (Circulation.

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Aneurysms of the Valsalva sinus (aortic sinus) can be congenital or acquired and are rare. They are more common among men than women and among Asians than other ethnic groups. Nonruptured aneurysms may be asymptomatic and incidentally discovered, or they may be symptomatic and manifest acutely with mass effect on adjacent cardiac structures. Ruptured Valsalva sinus aneurysms result in an aortocardiac shunt and may manifest as insidiously progressive congestive heart failure, severe acute chest pain with dyspnea, or, in extreme cases, cardiac arrest. Although both ruptured and nonruptured Valsalva sinus aneurysms may have potentially fatal complications, after treatment the prognosis is excellent. Thus, prompt and accurate diagnosis is critical. Most Valsalva sinus aneurysms are diagnosed on the basis of echocardiography, with or without angiography. However, both electrocardiographically gated computed tomography and magnetic resonance (MR) imaging can provide excellent anatomic depiction, and MR imaging can provide valuable functional information.

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