Heart Vessels (2005) 20:164–166 DOI 10.1007/s00380-004-0791-7
© Springer-Verlag 2005
CASE REPORT . Ibrahim Özsöyler · Levent Yilik · S¸ahin Bozok Cengiz Özbek · Ali Gürbüz
Cardiovascular involvement in Crohn’s disease in the absence of ankylosing spondylitis
Received: May 6, 2004 / Accepted: July 30, 2004
Abstract We describe a patient who had aortic regurgitation associated with Crohn’s disease in the absence of ankylosing spondylitis. Aortitis and aortic insufficiency are fairly uncommon in Crohn’s disease. The patient required aortic valve replacement because of severely uncoated cusps secondary to inflammation of the aortic wall and aortic valve. There was a saccular formation just above the right non-coronary commissure. This sac was closed with a pericardial patch. Pledgeted sutures were used for implantation of the prosthetic valve to avoid periprosthetic leakage. The right coronary ostium had narrowed due to aortic wall thickening. A right internal thoracic artery to right coronary artery bypass was done since there was no necessity for proximal anastomosis. Key words Crohn’s disease · Aortitis · Aortic valve replacement
Introduction Inflammatory aortic root disease causes regurgitation by alternation of the symmetry of the valve because of dilatation and distortion of aorta and aortic annulus.1,2 Cardiac valvular dysfunction is well known to be associated with ankylosing spondylitis. The association of cardiovascular lesions with “secondary” forms of ankylosing spondylitis, including spondyloarthropaties associated with psoriasis and regional enteritis (Crohn’s disease), is less frequent.3 Cardiovascular involvement in Crohn’s disease without ankylosing spondylitis has occasionally been reported
. I. Özsöyler1 (*) · L. Yilik · S¸. Bozok · C. Özbek · A. Gürbüz Department of Cardiovascular Surgery, Atatürk Education and . Research Hospital, Izmir, Turkey Correspondence address: . 1 T. Aktas¸ Cad. No: 2/10, Narlıdere, Izmir 35320, Turkey Fax ⫹90-232-243-4848 e-mail:
[email protected]
previously.1,3–5 We report a case of Crohn’s Disease, aortitis, severe aortic valve insufficiency, and serious narrowing of the right coronary ostium, without ankylosing spondylitis.
Case report A 40-year-old man was admitted to our hospital 4 months after the onset of dyspnea and angina pectoris. Examination results showed signs of severe aortic insufficiency. There were no symptoms or history of endocarditis. He had a history of histologically proven Crohn’s disease (diagnosed 6 years before). Ischemic alternations were detected in electrocardiograms and severe aortic regurgitation by echocardiography. Results of cardiac catheterization and coronary angiography confirmed the diagnosis of severe aortic regurgitation and serious narrowing of the right coronary ostium. There was no history of syphilis, rheumatic heart disease, or ankylosing spondylitis. Radiographs of the sacroiliac joints were normal and autoantibodies, including rheumatoid factor, as well as the tissue typing for HLAB27, were negative.
Surgery The right internal thoracic artery was harvested and cardiopulmonary bypass was established under general anesthesia. Antegrade cardioplegic arrest and retrograde cardioplegic maintenance after cross-clamping the aorta were used for myocardial protection. The aorta was opened after diastolic cardiac arrest. The aortic wall was 6 mm thick. There was minimal ascending aortic dilatation, but aortic root enlargement was evident. The aortic valve leaflets were thickened and they had obvious coaptation defects. There was a 2 ⫻ 2 ⫻ 2-cm3 saccular formation just above the right noncoronary commissure. This sac was closed with a pericardial patch (Fig. 1). The aortic valve was then replaced with 2 prosthetic mechanical valve using pledgeted sutures
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in order to prevent periprosthetic leakage. The ostium of the right coronary artery was very narrow. Cardiopulmonary bypass was terminated after a right internal thoracic artery to right coronary artery bypass was done. Specimens taken from the aortic wall, leaflets, and pericardium were sent for pathologic evaluation.
around the small vessels. There was inflammation of the adventitia to a similar extent, which intensified in the media especially around the vasa vasorum. The wall thickening and the lumen contraction in the vasa vasorum were significant (Fig. 2a). Basophilic degeneration and occasional lymphocytes were detected on the aortic leaflets (Fig. 2b).
Pathologic findings
Outcome
An evident degree of thickening was detected in the aortic wall. There was thickening of the intimal layer, slight basophilic degeneration, and sparse lymphocytic infiltration. In the media there was an inflammatory infiltration which was composed of histiocytes and plasma cells, which were much more intense than lymphocytes that showed multifocal settlement with irregular boundaries and intensified neutrophils in places. Necrobiosis was observed in between. No giant cells were detected. Inflammation was concentrated
No complications developed during the early postoperative period. The patient was discharged on oral anticoagulation and antiaggregant treatment for a mechanical prosthetic heart valve. Following the surgery, the patient has remained asymptomatic for the last 6 months. Evaluations for ankylosing spondylitis and cardiovascular progress are ongoing.
Discussion
Fig. 1. Thickened aortic wall and cavitation closed by a pericardial patch
a
Ankylosing spondylitis has been reported to be present in 2%–16% of patients with Crohn’s disease. In a few patients with ankylosing spondylitis and Crohn’s disease, aortic valvular insufficiency has been noted.3 However, there are few reports on the association of valvular heart disease and Crohn’s disease in the absence of ankylosing spondylitis. In our patient, the morphologic and histologic valvular findings were similar to the previously reported cases.3,4 Clinical, immunological, and radiological findings of ankylosing spondylitis were absent in our case. Burdick et al.3 and Leung4 reported that in certain individuals with Crohn’s disease, associated cardiovascular lesions might occur independent from the ankylosing spondylitis. Burdick et al.4 reported one periprosthetic leakage in their study. It may even be true that periprosthetic leakage is the most important occurrence during the postoperative period due to continuous inflammation. We used pledgeted
b
Fig. 2. a Light microscopic view of the aortic wall. b Light microscopic view of the aortic leaflet
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sutures to avoid periprosthetic leakage. In addition, inflammation may cause destruction of the aortic wall. In our case, saccular formation had been detected in the aortic wall. Detecting such a formation, although there was no diagnosis of infective endocarditis, was very useful in revealing the severity of the inflammation. We have no evidence of any previous reports of aortic valve and coronary osteal impairment with Crohn’s disease in the absence of ankylosing spondylitis. In our patient, increased aortic wall thickness had affected the right coronary ostium. We did not apply patch-plasty for osteal narrowing, because the aortic wall was very thick and friable, which might have caused hemorrhage. We used the right internal thoracic artery for revascularization of the right coronary artery, since there was no requirement for proximal anastomosis. Nishiyama et al.2 reported that proximal saphenous vein graft anastomosis to an aorta with inflammation should be avoided. For this reason, we suggest that internal thoracic arteries should be used for coronary revascularization in such patients.
We believe that frequent outpatient follow-up is necessary for these patients because the inflammation is continuous and new lesions such as other coronary ostial narrowing, aortic dilatation or aneurysms, mitral valve insufficiency, abnormal atrioventricular conduction, and periprosthetic leakage can develop.
References 1. O’Mahony SA, Moss F, Jepson E (1990) Letter to the editor. Am Heart J 199:1444 2. Nishiyama A, Matsubara S, Toyama J (2001) Takayasu arteritis with multiple cardiovascular complications. Heart Vessels 16:23–27 3. Burdick S, Tresch DD, Komokowsky RA (1989) Cardiac valvular dysfunction associated with Crohn’s disease in the absence of ankylosing spondylitis. Am Heart J 118:174–176 4. Leung W-H (1990) Letter to the editor. Am Heart J 119:145 5. Wackerlin A, Zund G, Maggiorini M, Jenni R, Turina M, Follath F (1997) Aortic valve insufficiency in Crohn disease. Schweiz Med Wochenschr 127:935–939
