Chronic Periaortitis of Ascending Aorta With Bilateral Ostial Coronary Stenosis in a Young Female Gauranga Majumdar, MCh, Aditya Kapoor, DM, Surendra K. Agarwal, MCh, Navneet Srivastava, MS, Ram Nawal Rao, MD, and Neetu Soni, MD Departments of Cardiovascular and Thoracic Surgery, Cardiology, Pathology, and Radiology, Sanjay Gandhi PGIMS, Lucknow, India
An 18-year-old woman without any risk factors for coronary artery disease or systemic vasculitis and infection presented with recurrent angina at rest. Coronary angiography revealed 100% occlusion of the ostial left main coronary artery and severe ostial right coronary artery stenosis. She underwent successful coronary artery bypass graft surgery. At surgery, the external surface of the ascending aorta was covered with a soft-tissue thickened mass; histopathology of the mass revealed chronic nonspecific inflammatory aortitis. (Ann Thorac Surg 2017;103:e427–9) Ó 2017 by The Society of Thoracic Surgeons
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omplete obstruction of the left main coronary artery (LMCA) is rare and is reported to occur in approximately 0.06% of patients undergoing coronary angiography [1]. Isolated stenosis of one or both coronary ostia with normal distal vessels has been reported in aortitis secondary to syphilis, Takayasu’s arteritis, connective tissue disorders, infections, congenital anomalies, and homozygous familial hypercholesterolemia [2–4]. Rarely, iatrogenic ostial coronary artery stenosis may occur after coronary angiography or after cardioplegia [5]. Ostial stenosis in the absence of these risk factors is often assumed to be atherosclerotic in origin [6]. A sex-based predilection with predominance in premenopausal women has also been reported. We report the case of an 18-year-old woman with angina secondary to 100% ostial LMCA occlusion and severe ostial right coronary artery (RCA) occlusion in the setting of aortitis. An 18-year-old nonhypertensive woman with no diabetes mellitus and no risk factors for coronary artery disease presented with exertional angina for the past year and recurrent rest angina for 3 months. There was no family history of coronary artery disease. Her clinical examination was normal; resting 12-lead electrocardiogram showed marked ST-segment depression in precordial and inferior leads (Fig 1). Echocardiography revealed normal left ventricular function with no regional wall motion abnormalities. There was no evidence of aortic
Accepted for publication Nov 6, 2016. Address correspondence to Dr Kapoor, Department of Cardiology, Sanjay Gandhi PGIMS, Lucknow 226014, India; email:
[email protected].
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
regurgitation, and the aortic valve and aortic root appeared unremarkable. Coronary angiography demonstrated complete obstruction of the LMCA, severe ostial stenosis of the RCA, and faint retrograde filling of the left anterior descending and left circumflex arteries through collaterals from the RCA (Fig 2, Videos 1 and 2). Complete blood count, renal and liver function, lipid profile, and blood cultures were normal. The erythrocyte sedimentation rate was 6 mm per hour, and the C-reactive protein was 0.08 mg/dL. Serologic testing for syphilis was also negative. Computed tomography imaging of the aorta was performed to rule out aortitis. This confirmed bilateral ostial coronary artery stenosis and revealed a soft-tissue, thickened mass encasing the ascending aorta (Fig 3A). Successful anaortic off-pump coronary artery bypass graft surgery was performed, with left and right internal mammary artery pedicled graft to the left anterior descending artery and RCA. At surgery, the entire ascending aorta was found to be encased in a soft-tissue thickened mass (Fig 3B, Video 3). Histopathology examination revealed fibroadipose tissue with interspersed arterioles and small nerve bundles along with perivascular infiltration by polymorphonuclear cells and nuclear dust. Venulitis with focal dense collection of plasma cells and lymphocytes was also demonstrable. The findings suggested chronic nonspecific inflammatory aortitis (Fig 3C and D).
Comment Ascending aortitis with ostial LMCA and RCA involvement without any underlying infection, vasculitis, or dyslipdemia, as happened in our case, is a rare entity. Involvement of both coronary ostia with normal distal coronary arteries has occasionally been reported in patients with syphilitic aortitis, Takayasu’s arteritis, and homozygous familial hypercholesterolemia [7, 8]. Ostial coronary stenosis in the absence of these risk factors is often assumed to be atherosclerotic in origin, especially in
Fig 1. Twelve-lead electrocardiogram showing ST-segment depression in precordial and inferior leads.
The Videos can be viewed in the online version of this article [http://dx.doi.org/10.1016/j.athoracsur.2016. 11.029] on http://www.annalsthoracicsurgery.org.
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2016.11.029
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Fig 2. Coronary angiography revealed (A) 100% ostial left main coronary artery disease and (B) severe ostial right coronary artery disease.
adults. Because in the present case the aortitis was probably indolent, the patient had slowly progressive obstructive coronary artery disease, due to involvement of coronary ostia. Most such cases are diagnosed incidentally by histopathology at the time of surgery. Histopathology of the specimen in our case, too, revealed chronic nonspecific inflammatory aortitis. Fig 3. (A) Computed tomography image revealed a soft tissue mass engulfing the ascending aorta (asterisk); (B) intraoperative findings confirmed the ascending aorta infiltration (asterisk); and histopathology findings revealed (C) fibroadipose tissue with perivascular infiltration by polymorphonuclear cells and nuclear dust (blue arrow) and (D) focal dense collections of plasma cells and lymphocytes (black arrow) suggesting aortitis.
Because the ascending aorta was not dilated either on echocardiography or computed tomography imaging, the surgeon did not consider replacing the entire ascending aorta. A more aggressive approach with ascending aorta replacement may be exercised in cases where the dilation is extreme. The obtuse marginal vessel of the left circumflex artery was found to be relatively small (less
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than 1 mm) and deemed to be not graftable. At the patient’s last 6-month follow-up, she was asymptomatic and doing well. The clinical presentation of aortitis is governed by the underlying cause of aortitis (vasculitis or infection). The site of aortic inflammation (ascending thoracic versus abdominal aorta) determines the location of pain (chest, back, or abdominal); and patients may have fever, shortness of breath, symptoms of aortic regurgitation, or angina due to coronary artery involvement. Occasionally, aortic dissection, aortic rupture, aortic thrombus formation, and peripheral embolization may occur. Diagnostic modalities for suspected aortitis include computed tomography, magnetic resonance angiography, or positron emission tomography scanning to image the entire aortic tree. As our patient had severe angina, a diagnostic coronary angiography was performed first. The ostial occlusion of LMCA and severe ostial RCA disease in a nonhyperlipidemic young woman without any evidence of generalized infection or vasculitis prompted us to suspect aortitis. Computed tomography imaging, in our case, confirmed the ostial coronary artery involvement and also revealed a soft tissue mass encasing the ascending aorta. In conclusion, because the clinical presentation of aortitis is often variable, it is important to have a high index of suspicion to establish a timely and accurate
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diagnosis. The present case was that of a 18-year-old woman with severe ostial LMCA and RCA disease. In the absence of conventional risk factors for coronary artery disease, no evidence of generalized vasculitis or infection aortitis was suspected. Computed tomography imaging followed by surgery and histopathology confirmed the diagnosis of nonspecific inflammatory aortitis.
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