Case Report Fatal Aortic Dissection in a Patient with Giant Cell Arteritis: A Case Report and Review of the Literature

Abstract

Giant cell arteritis may lead to catastrophic, large-vessel complications from chronic vascular wall inflammation without prompt diagnosis and treatment. We describe a rare case of acute aortic dissection without preceding aneurysm secondary to histologically confirmed giant cell arteritis (GCA) in an 85-year-old female with a four-year history of polymyalgia rheumatica and temporal arteritis diagnosed per biopsy six months prior to presentation. The literature is reviewed and the clinical implications of this case are discussed. Case Report An 85-year-old woman with a four-year history of polymyalgia rheumatica and temporal arteritis diagnosed per biopsy six months prior to presentation with an acute sensation of neck tightness with radiation to her bilateral shoulders and the epigastrium. Past medical history was significant for atrial fibrillation status following ablation, coronary artery disease status following remote myocardial infarction medically managed, hyperlipidemia, and hypertension. Her home medications included atenolol, coumadin, and prednisone 20 mg PO twice daily. She had intermittently received several rapidly tapered oral steroid courses over the preceding two years for polymyalgia rheumatica and temporal arteritis flares with resolution of clinical symptoms and normalization of acute inflammatory markers. At admission, her vital signs and cardiopulmonary exam were within normal limits. An electrocardiogram showed sinus rhythm with first-degree atrioventricular block. Cardiac biomarkers and a chest radiograph were unremarkable. Labs were significant for a stable known normochromic normocytic anemia of chronic disease (hemoglobin 10.3 g/dL), indolent chronic lymphocytic leukemia (white blood cell count of 11,400), an international normalized ratio of 2.0, and an erythrocyte sedimentation rate (ESR) of 49 mm/hr. A transthoracic echocardiogram eight months prior to admission demonstrated hyperdynamic left ventricular function with ejection fraction of 65%-70%, mild aortic insufficiency, and mild concentric left ventricular hypertrophy. Within several hours of admission, the patient was found to be unresponsive and progressively bradycardic with a depressed left ventricular function with ejection fraction of 45%. She later developed a wide complex tachycardia before terminating in pulseless electrical activity. Subsequent autopsy showed extensive granulomatous inflammation with lymphocytes, giant cells, and elastic membrane destruction in the aorta and vertebrobasilar and coronary arteries. There was evidence of early aortic root and proximal segment dissection without aneurysmal dilatation, associated with a mild pericardial effusion and a left hemothorax. Despite mild, nonobstructive atherosclerosis, there was no evidence of coronary artery occlusion or myocardial focal ischemic changes. Additionally, no large artery stenosis was appreciated