Lethargy, Confusion, and a Mediastinal Abnormality in a 68-Year-Old Woman: Syndromes

Sinus of Valsalva aneurysms may also develop as acquired lesions that result from the valvular disruption that occurs in patients with bacterial endocarditis, syphilitic aortitis, or rheumatoid heart disease, and as a developmentally acquired defect in patients with Marfan’s and Ehlers-Danlos syndromes.
Sinus of Valsalva aneurysms may exist as asymptomatic lesions but pose a risk for sudden rupture into the right atrium or right ventricle. Patients may also present with aortic insufficiency, as in the present patient, or rarely, right-sided outflow obstruction from an enlarging aneurysm. Elective surgical repair of asymptomatic aneurysms is generally advised because of the unpredictable potential for rupture.

Rupture of a sinus of Valsalva aneurysm usually causes the gradual onset of dyspnea, fatigue, chest pain, and a rapid pulse, but may cause acute onset of symptoms, including cardiorespiratory failure. The outcome with nonoperative management is usually poor; excellent results have been obtained with operative management. The chest radiograph may suggest the diagnosis of a sinus of Valsalva aneurysm if attention is directed to the mediastinal contours. “Skiagraphy” is a method of interpreting chest roentgenograms based on the appearance of various “moguls” as one “skis” down each side of the “mediastinal mountain.” To our knowledge, the concept of skiagraphy was first introduced by Daves in 1970. Figure 2 shows a chest roentgenogram with the normal moguls outlined. On the left, the first normal mogul is found paratrache-ally above the carina, representing the aortic knob. canadian health & care mall

The second normal mogul is located just above the left mainstem bronchus and represents the main pulmonary artery. The fourth normal mogul along the left mediastinum sits on the hemidiaphragm and represents the cardiac apex. The only normal moguls found along the right mediastinum are the first, which corresponds to an anatomically variant right aortic arch (in which case the left first mogul is absent) and the gently sloping fourth mogul, which represents the right atrium.


Figure 2. Normal chest roentgenogram demonstrates normal “moguls” on left side of mediastinum. See text for description of what each mogul represents. Note that normally there is not a left third mogul.

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