Ann Thorac Surg 2009;88:1672-1674. doi:10.1016/j.athoracsur.2009.03.018
© 2009 The Society of Thoracic Surgeons
Case Reports
Syphilitic Aortitis: An Uncommon Cause of Acquired Aortopulmonary Fistula
Srilakshmi M. Adhyapak, DNBa,*,
Arun K. Haridas, Mchb,
Mogalur C. Yeriswamy, MDc,
Mysore J. Santosh, DNBa,
Gurappa G. Shetty, DMa,
Kiron Varghese, DMa,
Chandrakant B. Patil, DMa,
Shamanna S. Iyengar, DMa,
Sunil Joshi, MSd
a Department of Cardiology, St. John's Medical College Hospital, Bangalore, India
b Department of Cardiothoracic Surgery, St. John's Medical College Hospital, Bangalore, India
c Department of General Medicine, St. John's Medical College Hospital, Bangalore, India
d Department of Vascular Surgery, St. John's Medical College Hospital, Bangalore, India
Accepted for publication March 6, 2009.
* Address for correspondence Dr Adhyapak, Department of Cardiology, St. John's Medical College Hospital, Sarjapura Rd, Bangalore, 560034, India (Email: srili2881967{at}yahoo.com).
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Abstract
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Cardiovascular syphilis has become a medical curiosity with the advent of widespread use of penicillin for the treatment of early syphilis. We report a case of a 37-year-old man who presented with sudden onset breathlessness that worsened insidiously for 1 year. Diagnosis of syphilitic aortopulmonary fistula was confirmed by aortography, contrast computed tomography, and histopathology. The patient underwent successful surgical correction. A high level of suspicion and awareness is needed for the diagnosis of this now rare disease. This entity is only amenable to surgical correction, even as existing surgical techniques need constant improvisation and individualization to each patient.
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Introduction
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Syphilitic aortic aneurysms typically present as saccular or fusiform swellings of the ascending aorta [1–3]. They can compress and erode surrounding vascular, visceral, and bony structures. We report the successful repair of a syphilitic aortic aneurysm compressing the pulmonary artery presenting as an aortopulmonary fistula.
A 37-year-old man was referred to our institute with history of sudden onset breathlessness that worsened insidiously to New York Heart Association functional class 3 for 10 months. There was a history of exposure to venereal disease 15 years ago. Clinical examination revealed tachycardia and wide pulse pressure. There were signs of congestive heart failure and a continuous murmur in the left second and third intercostal spaces. His hematologic and biochemical investigations were normal. The serum Venereal Disease Research Laboratory and Treponema Pallidum Hemagglutination Antibody were positive. The cerebrospinal fluid Venereal Disease Research Laboratory was negative. The enzyme-linked immunosorbent assay for human immunodeficiency virus (HIV) was negative. The two-dimensional transthoracic and transesophageal echocardiograms revealed dilatation of the ascending aorta adjacent to the sinotubular junction with a fistula communicating to the main pulmonary artery and moderate aortic insufficiency (Fig 1).
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Fig 1. Echocardiographic image at the basal short-axis showing the fistula with flow from the aorta-to-pulmonary artery.
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The contrast computed tomographic scan revealed a saccular aneurysm of the ascending aorta compressing the pulmonary artery (Fig 2). Cardiac catheterization revealed normal coronaries with a significant oxygen step-up in the pulmonary artery confirming a left-to-right shunt with a Qp/Qs of 6.1:1. Aortography demonstrated simultaneous opacification of the aorta and pulmonary artery with the catheter slipping from aorta to pulmonary artery through the fistula. The opinion of a venereologist was sought and the patient was treated with long-acting penicillin. He was subjected to surgery after 6 weeks of diagnosis and completion of the penicillin course.
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Fig 2. Contrast computed tomographic image demonstrating the saccular aortic aneurysm (arrow) compressing the main pulmonary artery.
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The aortic aneurysm was exposed through a midline sternotomy. It involved the left anterior aortic wall being adherent to the main pulmonary artery, with impending rupture (Fig 3). The aortic root and right atrial appendage were cannulated, and the patient was cooled to 28°C. The aorta was cross clamped and direct ostial cardioplegia was given. After institution of bypass, there was no pulmonary congestion. The aneurysm was opened and the aortopulmonary fistula (measuring approximately 1 x 1 cm) was identified approximately 3 cm above the sinotubular junction; it was directly sutured using 4.0 Prolene (Ethicon, Somerville, NJ) in two layers (Fig 4). There was minimal dilatation of the aortic annulus with prolapse of the noncoronary cusp. Due to the dense adhesions between the posterior aortic wall and surrounding structures, the coronary ostia were left in situ. A 28-Hemashield graft was anastomosed distally using 4.0 Prolene (Ethicon) and proximally to the aortic annulus including the coronary ostia. The initial plan was to spare the aortic valve. Hence, noncoronary cusp re-suspension with annuloplasty was done. However, due to persistence of moderate aortic regurgitation and financial constraints of the patient for a probable re-do valve surgery at a later date, the valve was replaced with a 22 ATS bi-leaflet mechanical valve (ATS Medical Inc, Minneapolis, MN). The air was removed from the patient. He was rewarmed, his cross clamp was removed after 139 minutes, and he was weaned from cardiopulmonary bypass after 180 minutes. The postoperative course of the patient was uneventful. Doppler echocardiography of the aorta showed no leak into the pulmonary artery and a normally functioning prosthetic valve. He was discharged on postoperative day 10, and he was asymptomatic.
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Comment
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Syphilitic aortitis manifests clinically more than 10 years after the primary infection as either aortic insufficiency, coronary ostial stenosis, or an aortic aneurysm.
These aneurysms are typically saccular, although fusiform aneurysms have been described [4]. Small aneurysms are asymptomatic. They become symptomatic due to compression or erosion into the surrounding structures. An aortopulmonary fistula is a rare complication that has been reported by Clapp and colleagues [5] and Vaideeswar and colleagues [6]. The first successful surgical repair was accomplished by Panday and colleagues [7].
The spirochaetes invade mucous membranes and form histopathologic hallmarks of obliterative endarteritis and adventitial mononuclear infiltrates (Fig 5). The vasa vasorum are inflamed with fibrotic intimal narrowing, leading to ischemia and focal medial necrosis; this leads to weakening of media and aneurysm formation. These focal necrotic areas cause elastin destruction and intimal shrinkage, resembling the typical tree bark appearance.
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Fig 5. Histopathology slide showing syphilitic endarteritis obliterans. (Hematoxylin & eosin stain, x40.)
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Unrepaired syphilitic aneurysms have a mortality of 36% [1]. The surgical technique of aortic root replacement needs to be individualized for each patient. As detailed for our patient, femoral cannulation was avoided, and the native coronary ostia were included in the vascular graft with replacement of the aortic valve.
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Acknowledgments
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We acknowledge the Department of Pathology for their help in the histopathology slide preparation and reporting.
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References
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- Jackman Jr JD, Radolph JD. Cardiovascular syphilis Am J Med 1989;87:425-433.[Medline]
- Kampmeier RH, Morgan HJ. The specific treatment of syphilitic aortitis Circulation 1952;5:771-778.[Abstract/Free Full Text]
- Cohen MG, Andersen D, Navia D, Belloso W, Gallo A, Grinfeld L. Syphilitic aortitis Catheter Cardiovasc Interv 2001;52:237-239.[Medline]
- Heggtveit HA. Syphilitic aortitis—a clinicopathological autopsy study of 100 cases Circulation 1964;29:346-355.[Abstract/Free Full Text]
- Clapp BR, Ranatunga CP, Nihoyannpoulos P. An unusual aortopulmonary communication secondary to syphilitic aortitis Int J Cardiol 1997;62:155-159.[Medline]
- Vaideeswar P, Deshpande JR, Sivaraman A. Aortic-pulmonary fistulization-an unusual complication of syphilitic aortic aneurysm Rontgenblatter 1983;36:26-29.[Medline]
- Panday S, Hishikar A, Karbhase J. Rupture of syphilitic aneurysm of ascending aorta into main pulmonary artery: successful emergency repair J Thorac Cardiovasc Surg 1982;83:470-471.[Medline]
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Abstract
Introduction
