Ann Thorac Surg 2006;82:1115-1117
© 2006 The Society of Thoracic Surgeons
Case report
Successful Reoperation After 17 Years in a Case of Endomyocardial Fibrosis
Sanjay Mammen Cherian, MSa,*,
Baylal Ragavendra Jagannath, DNBa,
Sushma Nayar, MDb,
Kotturathu Mammen Cherian, FRACSa
a Department of Cardiovascular Surgery, Chennai, India
b Department of Pathology, Frontier Lifeline, International Centre for Cardiothoracic and Vascular Diseases & Dr KM Cherian Heart Foundation, Chennai, India
Accepted for publication December 8, 2005.
* Address correspondence to Dr Cherian, Frontier Lifeline, International Centre for Cardiothoracic and Vascular Diseases & Dr KM Cherian Heart Foundation, R-30-C, Ambattur Industrial Estate Rd, Mogappair, Chennai, 600101 India (Email: frontierlifeline{at}yahoo.co.in).
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Abstract
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Endomyocardial fibrosis is an uncommon cause of congestive cardiac failure characterized by severe diffuse thickening of the endocardium, and the overall long-term prognosis of this disease is generally considered poor. We report a case of endomyocardial fibrosis that was initially treated by endocardial resection and mitral valve replacement, which was regularly followed-up. The patient underwent successful repair of a paravalvular leak after a period of 17 years. The treatment of left ventricular endomyocardial fibrosis with associated mitral valve disease by endocardial resection and mitral valve replacement is a good surgical option that provides good long-term results.
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Introduction
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Endomyocardial fibrosis (EMF) is an uncommon idiopathic disorder characterized by severe diffuse thickening of the endocardium causing obliteration of the ventricular cavity, restricted ventricular filling, and congestive cardiac failure [1]. The fibrotic process is usually located at the apex of the affected ventricle, and involvement of the chordae tendinea may cause mitral or tricuspid valve dysfunction, or dysfunction of both valves simultaneously. The treatment of EMF is primarily surgical, and the overall long-term prognosis in severe cases is poor [2].
We report a case of EMF involving the left ventricle and mitral valve, initially treated by endocardial resection and prosthetic valve replacement, which was regularly followed-up, and underwent successful repair of a paravalvular leak after 17 years.
A 26-year-old man presented to us in November 1987 with symptoms suggestive of class III dyspnea. His pulse was regular with a rate of 106 per minute. Systemic blood pressure was 140/100 mm Hg. Transthoracic echocardiography revealed thickening of the inferior and basal ventricular wall, apical obliteration, a restrictive left ventricular filling pattern, severe mitral regurgitation, thickening of the anterior and posterior mitral leaflets, and moderate pulmonary artery hypertension. The left ventricular end-systolic volume was 39 mL, left ventricular end-diastolic volume was 176 mL, and the ejection fraction was 78%. No cardiac catheterization was performed.
On table inspection, diffuse fibrosis of the left ventricular endocardium and chordae tendinea was revealed. The mitral valve was fibromyxomatous with spotty calcification, and repair of the valve was not feasible due to the extensive fibrosis. On excision of the valve, a well-defined plane of cleavage was observed between the endocardium and myocardium. This permitted excision of the fibrous tissue starting from the mitral valve annulus to the apex of the left ventricle. The weight of the excised tissue was approximately 8 to 10 grams. After endocardial resection, the mitral valve was replaced using a 25-mm Bjork Shiley (Shiley Inc, Irvine, CA) mechanical valve. His postoperative course was uneventful. Histopathologic examination of the endocardial tissue revealed endomyocardial fibrosis.
Postoperatively, he was treated with anticoagulants and diuretics, and he was on a regular annual follow-up. He remained asymptomatic for 16 years. He had atrial fibrillation develop during that year for which he was started on oral amiodarone. He gave a history of recurrent fever that was treated with antibiotics, and had class II dyspnea for 1 month. His blood culture was negative. Echocardiography revealed moderate paravalvular leak. Repeat echocardiography done after 1 month demonstrated that the leak had progressed to severe paravalvular leak, with a maximum gradient of 34 mm Hg, mean of 16 mm Hg, pannus formation, dilated left atrium and left ventricle, mild tricuspid regurgitation, severe pulmonary artery hypertension, and good left ventricular function. Coronary angiography demonstrated normal epicardial coronary arteries, and grade IV paravalvular leak. Blood investigations were unremarkable with an eosinophil count of 4%, and an international normalized ratio of 1.94.
In view of his increasing paravalvular leak, he had an elective reoperation in December 2004. The prosthetic mitral valve was inspected, and a paravalvular leak measuring 2 to 3 cms was noted at the 8 o'clock position. Minimal pannus on the superior aspect of the valve annulus was excised. The rest of the valve annulus was normal. There was no evidence of active infective endocarditis such as vegetation, unhealthy granulation tissue, and so forth. The valve function was tested and found to be normal. The paravalvular leak was repaired using four interrupted pledgetted 2-0 Ticron (Tyco Healthcare, Mansfield, MA) sutures, and was further reinforced with continuous 3-0 Prolene sutures (Ethicon, Somerville, NJ). A small bit of paravalvular tissue was excised and sent for histopathologic analysis, which confirmed EMF (Fig 1). Culture of this tissue was negative. His postoperative course was uneventful, and he was discharged on postoperative day 8. He was reviewed 6 months after his second surgery when he was noted to be clinically asymptomatic. Electrocardiogram revealed first degree heart block. Transthoracic echocardiography demonstrated no paravalvular leak, a maximum gradient of 5 mm Hg, a mean gradient of 3 mm Hg, effective valve area of 2.5 sq cm, mild to moderate pulmonary artery hypertension, and good left ventricular function.
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Fig 1. Section of paravalvular tissue showing areas of endocardial fibrosis with inflammatory infiltration. (Elastic Van Gieson stain magnification, x400.)
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Comment
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Endomyocardial fibrosis is an uncommon cause of restrictive cardiomyopathy that carries a poor long-term prognosis in severe cases [2]. We report a case of EMF involving the left ventricle with severe mitral regurgitation, which was treated by endocardial resection and mitral valve replacement at the initial surgery. The patient was followed-up regularly and underwent successful repair of a paravalvular leak after a period of 17 years. This patient probably represents one of the longest follow-up cases of EMF that underwent successful reoperation after 17 years.
At initial surgery, the endocardium was noted to be densely fibrotic, whereas the underlying myocardium appeared normal. A well-defined line of cleavage was observed between the endocardium and the myocardium after valve excision that facilitated endocardial resection. Histologic features of EMF, namely, subendocardial infarction, fibrosis, granulation tissue formation, and inflammatory cell infiltration [3], were observed in the biopsy specimens obtained that confirmed the presence of EMF. Recurrence after complete excision does not occur [4], and the evidence of EMF in our patient at second surgery could probably represent residual disease.
Although valve repair has been previously described as a form of treatment for valvular involvement in EMF [5], because the mitral valve was grossly dysfunctional and irreparable in our patient, the valve was replaced at the initial surgery. We believe that endocardial resection can be performed better once the valve is excised, because it is easier to develop the plane for dissection.
At repeat operation, because there was no evidence of active infective endocarditis, the clean, well-defined paravalvular leak was repaired without explanting the previous prosthetic valve.
The exact cause of the late paravalvular leak is unclear. Paravalvular tissue excised at the second surgery that was sent out for culture did not grow any organism in any media. However, histopathologic examination revealed inflammatory cells in the areas of fibrosis. This prompts us to consider sterile infective endocarditis as a probable cause.
Endomyocardial fibrosis usually occurs in children and young women [4, 6], and although the precise cause of EMF is unknown, eosinophilia and parasitic infections have been implicated [4]. However, our patient was a young man who had no such history.
In conclusion, the treatment of left ventricular endomyocardial fibrosis with associated mitral valve disease by endocardial resection and mitral valve replacement is a good surgical option that provides good long-term results.
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Acknowledgments
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We would like to thank Meena Rani for helping with the preoperative and postoperative echocardiography, Indira Balaji and Vidya Sundar for help with references, Geetha Ananthakrishnan for clerical assistance, and Dr Vimala Jesudian and Dr Binoy John for help with critical review and comments during the preparation of the manuscript.
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References
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- Schneider U, Jenni R, Turina J, Turina M, Hess OM. Long term follow up of patients with endomyocardial fibrosiseffects of surgery. Heart 1998;79:362-367.[Abstract/Free Full Text]
- Moraes F, Lapa C, Hazin S, et al. Surgery for endomyocardial fibrosis revisited Eur J Cardiothorac Surg 1999;15:309-313.[Abstract/Free Full Text]
- Chopra P, Narula J, Talwar KK, et al. Histomorphologic characteristics of endomyocardial fibrosisan endomyocardial biopsy study. Hum Pathol 1990;21:613-616.[Medline]
- Shaper AG. The aetiology of endomyocardial fibrosisIn: Valiathan MS, Somers K, Kartha CC, editors. Endomyocardial fibrosis. 1st ed.. Oxford, England: Oxford University Press; 1993. pp. 113.
- Uva MS, Jebara VA, Acar C, et al. Mitral valve repair in patients with endomyocardial fibrosis Ann Thorac Surg 1992;54:89-92.[Abstract]
- Radhakumary C, Kumari TV, Kartha CC. Endomyocardial fibrosis is associated with selective deposition of Type 1 collagen Indian Heart J 2001;53:486-489.[Medline]
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Abstract
Introduction
