Novel Cause of Sudden Cardiac Death

Abstract

A 61-year-old black woman with hypertension, dyslipidemia, sickle cell trait, and α-thalassemia presented with a 3-week history of progressively altered mental status and seizures. Laboratory studies demonstrated multiple autoantibodies, including antinuclear antibody (speckled, titer 1:640) and anti-Smith and anti-ribonucleoprotein (both positive by enzyme immunoassay). Of note, her erythrocyte sedimentation rate was elevated at 86 mm/h (reference range 4–30 mm/h), her C-reactive protein was elevated at 4.2 mg/dL (0.0–0.5 mg/dL), and her serum IgG level was normal at 1510 mg/dL (751–1560 mg/dL). ECGs early in her hospitalization demonstrated normal sinus rhythm. Initial imaging studies including chest radiograph, echocardiogram, and thoracic computed tomography showed normal aortic root caliber with minimal arteriosclerosis and mild cardiomegaly. Two weeks after admission, follow-up imaging revealed slight dilation of the ascending aorta to 4 cm on chest computed tomography (Figure 1A) and minimal tortuosity of the descending aorta on chest radiograph, which was not explored further. Figure 1. A , Computed tomography of the chest with intravenous contrast 2 weeks after admission, demonstrating a slight (4 cm) dilation of the ascending aorta that was not noted previously. A indicates ascending aorta; D, …