Hereditary angioedema is a genetic disorder typically related to insufficient or dysfunctional C1-esterase inhibitor. Patients present with episodic swelling of various body parts, such as the face, neck, bowel, genitals, and extremities. Acute or severe symptoms can lead to patients presenting to the emergency room, particularly when the neck and abdominopelvic regions are affected, which is often accompanied by radiologic imaging evaluation. Patients with hereditary angioedema can pose a diagnostic challenge for emergency department physicians and radiologists at initial presentation, and the correct diagnosis may be missed or delayed, due to lack of clinical awareness of the disease or lack of its consideration in the radiologic differential diagnosis. Timely diagnosis of hereditary angioedema and rapid initiation of appropriate therapy can avoid potentially life-threatening complications. This article focuses on the spectrum of common and characteristic acute imaging manifestations of hereditary angioedema and provides an update on important recent developments in its clinical management and treatment.
Suspected prosthetic valve dysfunction is a difficult clinical problem, because of the high risk of repeat valvular surgery. Echocardiographic measurements of prosthetic valvular dysfunction can be misleading, especially with bileaflet valves. Direct measurement of trans-valvular gradients is problematic because of potentially serious catheter entrapment issues. We report a case in which a high-fidelity pressure sensor angioplasty guidewire was used to cross prosthetic mitral and aortic valves in a patient, with hemodynamic and echocardiographic assessment. This technique was safe and effective, refuting the inaccurate non-invasive tests that over-estimated the aortic valvular gradient.
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