Asian Cardiovasc Thorac Ann 1999;7:238-240
© 1999 Asia Publishing EXchange Pte Ltd
Ankylosing Spondylitis and the Mitral Valve
Alexander John, MD,
Christoph Glauner, MD,
Henning Warnecke, MD
Department of Heart Surgery
Schüchtermann Clinic
Bad Rothenfelde, Germany
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For reprint information contact: Alexander John, MD Tel: 49 5424 64 1651 Fax: 49 5424 64 1653 Department of Heart Surgery, Schüchtermann Clinic, Ulmenallee 11, Bad Rothenfelde 49214, Germany.
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Abstract
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Recurrent valve dehiscence was encountered in a patient with ankylosing spondylitis who had undergone mitral valve replacement. A method of reinforcing the native annulus with bovine pericardium and reattaching the valve is described.
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Introduction
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The most common cardiac lesions in patients with ankylosing spondylitis are aortic regurgitation and conduction abnormalities. Involvement of the mitral valve is less common but severe lesions requiring mitral valve replacement have been reported. The sclerosing inflamma-tory lesion is said to originate in the region of the aortic root but progressive involvement of the mitral valve with prolapse of the anterior leaflet has been found in a small number of patients. We encountered an unusual problem involving only the mitral valve, which is reported because of the rarity of this condition.
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Case Report
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A 44-year-old male engineer was found to have a heart murmur during a routine examination in 1991. He was known to have ankylosing spondylitis for nearly 20 years and had severe kyphosis of the cervicodorsal spine. His heart condition was routinely followed up and he underwent cardiac catheterization and angiography in 1995. The mitral regurgitation was severe and the obtuse marginal branch of the circumflex artery was blocked. Pulmonary and wedge pressures were above normal limits and rose markedly with prominent V waves on moderate exertion. Although the symptoms were mild, it was decided to replace the mitral valve and revascularize the obtuse marginal.
Severe kyphosis of the cervicodorsal spine made endotracheal intubation difficult. The tube was guided over a bronchoscope. The mitral valve was found to be floppy and dilated and it was replaced with a 29-mm bileaflet valve (Sulzer Carbomedics, Austin, TX, USA). The valve was fixed with 16 interrupted pledgeted mattress sutures and the annulus appeared quite strong. A vein graft was placed on the obtuse marginal. The patient recovered well and was sent for rehabilitation on the 6th postoperative day. After 4 weeks of rehabilitation, he was discharged fully recovered. Pathological findings in the excised mitral valve were postinflammatory fibrosis with mononuclear cellular infiltration and areas of calcification.
Three weeks later, the patient was readmitted with weakness and breathlessness on exertion. Echocardio-graphy revealed moderately severe periprosthetic leakage. There was laboratory evidence of hemolytic anemia. A second operation was performed. The valve showed severe dehiscence along the entire caudal half of the circum-ference. The cranial half had healed well and was holding the valve strongly. Along the dehiscent portion, the pledgeted sutures had cut through the annulus and were lying on the rim of the valve (Figure 1
). We presumed it was a technical problem and reattached the valve using 6 or 7 of the usual pledgeted sutures taken carefully through the annulus and the prosthesis. The annulus appeared to be strong enough to hold the sutures. After refixation of the valve, it looked quite satisfactory. After the heart resumed beating and cardiopulmonary bypass was terminated, a transesophageal echocardiogram confirmed proper functioning of the valve with no periprosthetic leakage. After two weeks, the patient was transferred to the rehabilitation center in a stable condition. Sinus rhythm that had been lost temporarily, was restored.
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Figure 1. Mitral valve dehiscence. The caudal half of the valve has separated from the annulus. Note the pledgets lying on the rim of the valve.
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One week later, a systolic murmur could be heard over the apex and an echocardiogram confirmed a new periprosthetic leak. There was evidence of hemolytic anemia (hemoglobin 95 g•L–1, hydroxybutyrate dehydro-genase 455 U•L–1, lactate dehydrogenase 668 U•L–1, and bilirubin 22 mg•L–1). By now it was a real surgical problem. The native annulus failed to hold the pledgeted sutures that gradually worked their way through it, causing dehiscence. Fortunately, the patient and his family were very cooperative and understood the problem quite well. In spite of the uncertain outcome, they agreed to another operation, the third within a period of six months. Mobilizing the heart and accessing the valve was not very difficult. The same part of the valve had opened up. This time we had to improvise a better technique. A large piece of bovine pericardium (Medtronic, Inc., Irvine, CA, USA) was used to reinforce the native annulus. Specially prepared sutures with large pledgets were taken through the pericardial patch, the annulus, and the prosthesis and tied carefully to fix the valve in place. The outer border of the pericardial patch was fixed to the inside of the left atrial wall using a running 4/0 polypropylene suture (Figure 2). After closure, valve function was checked by transesophageal echocardiography and found to be good. The patient made a good recovery and was sent home after a satisfactory rehabilitation of 5 weeks. Three months later, he underwent a detailed cardiological examination including a stress test. He was in stable sinus rhythm and fully fit, the valve was functioning normally, and there was no periprosthetic leakage. Right and left ventricular function was normal. He had already gone back to work.
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Figure 2. Bovine pericardium reinforcing the annulus where the sutures tend to cut through. The pericardium was fixed peripherally to the atrial wall using a running polypropylene suture.
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Discussion
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Ankylosing spondylitis is a disease of unknown etiology, manifesting with axial articular involvement, peripheral asymmetric arthritis, and various extra-articular lesions in a number of organs including heart, lungs, eyes, and the skin. Essentially, it is a chronic and progressive inflammation leading to fibrosis and calcification. There is a known familial disposition and a tendency to affect young men.1 Cardiac involvement has been described and although all parts of the heart including pericardium, myocardium, the conduction system, and the valves can be affected, the most common lesion is aortic incom-petence occurring in 3% to 10% of patients.2 Involvement of the mitral valve is very rare and constitutes thickening and fibrosis mainly of the anterior leaflet, leading to prolapse and regurgitation. So far, such mitral lesions have been reported only in association with aortic incompetence. This is the first report of isolated involvement of the mitral valve, except for one previous report of mitral stenosis and incompetence, in which rheumatic origin of the mitral lesion could not be ruled out.3
The pathology of the valvular lesion has been described in detail.4 It starts at the aortic root with thickening and dilatation of the proximal aorta in the region of the sinuses of Valsalva. There is adventitial scarring and intimal proliferation. Progressive involvement of the membranous septum and the anterior leaflet of the mitral valve also occurs. A characteristic thickening of the base of the anterior leaflet gives rise to the subvalvular ridge seen angiographically and anatomically. The microscopic findings are of acute inflammation with perivascular mononuclear cellular infiltration or various stages of chronicity with fibrosis and calcification. These gross and microscopic findings have been contrasted with the findings in other causes of aortic incompetence such as rheumatic fever, Marfan syndrome, syphilis, and rheumatoid arthritis.5
There are various reports of aortic valve replacement for the characteristic aortic lesion of ankylosing spondylitis.6,7 There are no reports of any technical difficulties or valve dehiscence leading to paravalvular leak. In the case of mitral valve replacement, the late development of periprosthetic leak was reported.8 For some reason, the mitral annulus in ankylosing spondylitis appears to be unable to hold the sutures that fix the implanted prosthetic valve. The problem of pledgeted sutures working their way through the annulus and causing recurrent dehiscence has not been reported before. We had to improvise a technique to effectively reattach the mitral valve and the use of bovine pericardium was a good solution to the problem. Unlike rheumatic lesions where dense fibrosis makes suturing of valves easy, the tissues have a different texture in cases of ankylosing spondylitis. This should be kept in mind during valve operations in patients with ankylosing spondylitis.
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References
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Kotelnikova GP, Kamova NN, Polyanskaya IP. Characterisation of cardiac pathology in ankylosing spondylitis. Terapevticheskii Arkhiv 1993;65:34–7.
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Gregersen PK, Gallerstein P, Jaffe W, Enlow RW. Valvular heart disease associated with juvenile onset ankylosing spondylitis. A case report and review of the literature. Bull Hosp Joint Dis Orthop Inst 1982;42:103–14.
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Montes J, Anguita M, Pulpon LA, Taveras R, Alonso A, Teresa ED, et al. Manifestationes cardiacas de la espondilitis anquilosante. Revision a proposito de un caso poco frequente. Revista Espanola de Cardiologia 1983;36:501–4.[Medline]
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Roberts WC, Hollingsworth JF, Bulkley BH, Jaffe RB, Epstein SE, Stinson EB. Combined mitral and aortic regurgitation in ankylosing spondylitis, angiographic and anatomic features. Am J Med 1974;56:237–43.[Medline]
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Abstract
Introduction
