63-year old female with past medical history significant for rheumatoid arthritis presented to the emergency department with an edematous tongue with purple discoloration. The patient stated that for the past several days her tongue had become progressively more swollen with an increasing purple-blue discoloration. The patient denied any recent upper respiratory infection, changes in medication and diet, or travel.  On examination, the patient’s vital signs were stable and she was afebrile. Initial laboratory tests were unremarkable, except for a leukocytosis of 13.6 and an elevated alkaline phosphatase of 146.
On physical examination, it was noted that the patient’s tongue was erythematous with a dark purple color (Figure 1). No visible lesions were noted within the oral cavity. Lymphadenopathy was not present. A CT scan of the patient’s neck showed a very large and edematous tongue with induration of the anterior aspect of the neck, but no evidence of abscess or neck cellulitis. During the rest of the examination, it was noted that the patient had a purplish echymosis on her upper chest (Figure 2).

Upon further questioning, the patient stated that she has recently noticed that she does bruise easily, especially after playing with her dogs. She denied any past work-up for easy bruising or a family history with similar complaints. Further laboratory evaluation showed normalization of the patient’s white blood count and continued elevation of alkaline phosphatase at 146. Because of the patient’s history of easy bruising, it was decided to perform a work-up for a possible coagulation disorder. Additional laboratory results showed an elevated aPTT at 43.5 and decreased factor VIII assay of 27% (normal, 50-150%). The patient was subsequently diagnosed with an acquired autoimmune factor VIII inhibitor, also known as acquired hemophilia A. She was started on factor eight inhibitor bypass activity (FEIBA) treatment and prednisone for the acute episode. The tongue swelling decreased significantly and the ecchymosis on her chest improved. The patient was given one dose of rituximab in the hospital and, upon discharge, was started on cyclophosphamide for the treatment of antibodies to factor VIII. Follow-up one week after admission to the hospital showed continued improvement in the patient’s condition.

A 62 year old white male presented to the outpatient echocardiography laboratory for evaluation of shortness of breath. Five years prior, he had mechanical aortic valve replacement for a bicuspid aortic valve. His history is also significant for hypertension, tobacco abuse, and moderate alcohol use. He has had no evaluation for his mechanical aortic valve since surgery.

Resting blood pressure was noted to be 170/100 mmHg. Immediately upon parasternal long imaging, it was noted the patient had a large ascending aortic aneurysm of nearly 8 cm. There was no evidence of proximal aortic dissection, aortic regurgitation, or pericardial effusion. Left ventricular function was normal.

 The patient was immediately taken to the emergency room and started on an Esmolol drip for blood pressure control. A CT scan of the chest and abdomen confirmed a large 8.7 cm ascending aortic aneurysm with a Type A aortic dissection (Image 1). The patient was transferred to an academic tertiary care center. There it was felt the dissection was chronic and surgery was not emergent. He underwent left heart catheterization that showed no critical coronary disease. After complete dental extraction, he underwent successful aortic root and arch replacement. Prior to replacement, the aortic aneurysm and dissection had increased to nearly 12 cm (Image 2).

For patients who undergo isolated aortic valve replacement for a bicuspid aortic valve, close post-operative monitoring of the ascending and thoracic aorta for aneurysm must take place. In up to 10% of patients post aortic valve replacement, ascending aortic aneurysms that require repeat surgery can develop.

RGalani@jaxheart.com

Back to www.dcmsonline.org