Asian Cardiovasc Thorac Ann 2007;15:252-254
© 2007 Asia Publishing EXchange Ltd
High-Risk Repair of Ascending Aortic Aneurysm Due to Giant Cell Aortitis
Pavan Atluri, MD,
Y Joseph Woo, MD
Division of Cardiothoracic Surgery, Department of Surgery, University of Pennsylvania School of Medicine, Philadelphia, USA
For reprint information contact: Y Joseph Woo, MD Tel: 1 215 662 2956 Fax: 1 215 349 5798 Email: wooy{at}uphs.upenn.edu, Division of Cardiothoracic Surgery, Department of Surgery, University of Pennsylvania, Silverstein 4, 3400 Spruce Street, Philadelphia, PA 19104, USA.
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ABSTRACT
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Giant cell arteritis increases the risk of developing a thoracic aortic aneurysm. Thoracic aortic aneurysm repair in octogenarians carries a profound increase in postoperative morbidity and mortality. We report the successful repair of an ascending aortic aneurysm in an 83-year-old woman with a history of treatment for temporal arteritis and pathologic evidence of giant cell aortitis.
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INTRODUCTION
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Autoimmune vasculitides increase the risk of thoracic aortic aneurysmal disease. Giant cell arteritis (GCA), also referred to as temporal arteritis, is associated with a 17.3-fold increased risk of aortic degeneration.1,2 We report the successful repair of a GCA-mediated aortic root aneurysm in an octogenarian previously treated for temporal arteritis.
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CASE REPORT
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An 83-year-old woman presented with shortness of breath. Transthoracic echocardiography revealed severe aortic insufficiency (4+), mild mitral regurgitation (2+), and a 5.4 cm aortic root aneurysm at the level of the sinotubular junction. There was moderate left ventricular dysfunction with global hypokinesis and an ejection fraction of 45%. Preoperative cardiac catheterization showed non-critical coronary artery disease with luminal irregularities. A computed tomographic scan of the thorax confirmed an isolated 6 cm ascending aortic aneurysm. The patient’s medical history included temporal arteritis treated with high-dose steroids 6 years previously, with resolution of the symptoms, hypertension, and paroxysmal atrial fibrillation. Preoperative medications included aspirin, beta blocker, angiotensin-converting enzyme inhibitor, and thiazide diuretic. The patient was neurologically intact, with clear lung fields, and a loud diastolic murmur. After extensive discussion, she agreed to proceed with replacement of the aortic root, ascending aorta, and aortic valve.
Prior to cardiopulmonary bypass, a transesophageal echocardiogram was performed to confirm the structural abnormalities (Figure 1
). Cardiopulmonary bypass was carried out with distal arch cannulation, right atrial cannulation, and a retrograde coronary sinus catheter. An uncomplicated aortic root replacement was completed with a no. 21 porcine xenograft and a no. 24 Dacron interposition graft extension between the porcine root and aortic arch. Total cardiopulmonary bypass time was 228 min and aortic cross clamp time was 173 min. Core body temperature was maintained at 30°C for the duration of the procedure. The patient was weaned from cardiopulmonary bypass without difficulty. A postoperative transesophageal echocardiogram revealed preserved left ventricular function (ejection fraction, 50%–55%) and trivial (1+) aortic and mitral insufficiency. The patient was hemodynamically stable on arrival in the intensive care unit with minimal inotropic support (low-dose epinephrine, 2 µg·min–1). She was extubated on postoperative day 1, transferred to the cardiothoracic surgical ward for blood pressure management and physical rehabilitation on postoperative day 2, and discharged 8 days later to an inpatient rehabilitation facility, in a stable condition. At her 1-month follow-up, she reported resolution of her symptoms. Gross pathologic evaluation of her native ascending aorta revealed an aneurysmal aorta devoid of dissection with minimal calcification of the native aorta and aortic valve. Microscopy revealed degeneration of the elastic fibers of the ascending aorta and giant cells within the arterial wall (Figure 2). Giant cell arteritis was the cause of the aneurysmal degeneration.
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Figure 1. Intraoperative transesophageal echocardiographic images demonstrating (A) severe aortic insufficiency and (B) an aneurysm of the ascending aorta at the level of the sinotubular junction.
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Figure 2. Light microscopy showing inflammatory cells and giant cells (white arrow) within the media of the diseased native ascending aorta, indicative of giant cell aortitis. Hematoxylin and eosin stain, magnification x 20.
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DISCUSSION
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Giant cell arteritis is a chronic inflammatory disorder affecting medium and large arteries, predominantly in people older than 50 years of age.3,4 Symptoms of GCA include fatigue, weight loss, anorexia, low-grade fever, anemia, visual loss, jaw claudication, and headaches. An elevated erythrocyte sedimentation rate is almost universally present. Diagnosis is made with temporal artery biopsy demonstration of mononuclear infiltrates, granuloma formation, or giant cells.5 Treatment of temporal arteritis includes a limited course of high-dose steroid therapy for intracranial symptoms. This is not adequate for extracranial disease, as seen by involvement of the aorta, subclavian, axillary, brachial, iliac, or cervical arteries. The inflammatory changes in GCA affect the structural integrity of the intimal and medial layers of medium and large arteries. The inflammatory insult that ensues is mediated by T-lymphocytes, macrophages, and multinucleated giant cells. Clonal proliferation of activated T-cells extends the immune insult and releases inflammatory mediators such as interleukin-2 and interferon-
. Treatment does not eliminate vascular T-cell-mediated immune activity.6 Macrophages within the large arterial wall release metalloproteinases, pro-inflammatory cytokines, and oxidative radicals that result in fragmentation of the internal elastic lamina, medial smooth muscle loss, medial scarring, and reduced intimal and medial structural integrity.
A population-based study reported 18% of patients with GCA manifested aortic aneurysm and/or dissection, with 11% presenting with thoracic aortic aneurysm.2 A significant proportion of thoracic aneurysms in the older population, attributed to atherosclerotic disease are a manifestation of GCA. There are limited reports of repair of thoracic aortic aneurysms due to GCA, suggesting limited knowledge of this disease. A large proportion of patients remain asymptomatic until presenting emergently with aortic dissection or rupture. Early symptoms can include aortic regurgitation, heart failure, cerebrovascular events, or upper extremity claudication from great vessel involvement. When coupled with the predominant presentation of this disease in the elderly, therapeutic intervention becomes a challenge. There is a very high mortality (31%) associated with emergency aortic surgical intervention in these patients.3 A retrospective review analyzing complications in patients with GCA and thoracic aortic involvement noted a very high rate of progression of aortic disease to rupture, congestive heart failure (78%), and aortic dissection (39%),7 with 50% mortality.8 This supports early elective surgical treatment of GCA-associated thoracic aortic disease.
Current treatment modalities are unable to eliminate the disease process that reduces the integrity of the aortic wall, necessitating close follow-up of this patient population. A high index of suspicion for aortic disease is necessary, given the grave consequences. Asymptomatic patients require close monitoring of the thoracic and abdominal aorta by echocardiography, ultrasound, or computed tomographic angiography. It was believed that this patient was adequately treated for temporal arteritis. Continued inflammation, as manifested by giant cells within the ascending aorta, resulted in aortic root dilatation, aortic insufficiency, and congestive heart failure, necessitating elective treatment to avoid aortic rupture. This case indicates that elective surgical repair of aortic root aneurysms resulting from GCA can safely be performed on octogenarians.
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REFERENCES
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ABSTRACT
INTRODUCTION
