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Eur J Cardiothorac Surg 1999;15:309-313
© 1999 Elsevier Science NL

Surgery for endomyocardial fibrosis revisited¹

Division of Thoracic Surgery, Department of Surgery, Federal University of Pernambuco, Heart Institute of Pernambuco, Recife, Brazil

Received 21 September 1998; received in revised form 11 January 1999; accepted 12 January 1999.

Corresponding author. Tel.: +55-81-2315-968; fax: +55-81-2210-382.

	Abstract

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Objective: To identify life expectancy after surgery for endomyocardial fibrosis (EMF) and the events that influence it. Methods: Eighty-three patients with EMF underwent endocardial decortication and atrioventricular valve replacement or repair, between December 1977 and December 1997. There were 66 (79.6%) female and 17 (20.4%) male patients, ranging in age from 4 to 59 years (mean, 31). Thirty-seven (44.5%) had biventricular disease, 34 (41.0%) had disease of the right ventricle alone and 12 (14.5%) had EMF confined to the left ventricle. All were in functional class III or IV (New York Heart Association classification). Results: Sixty-eight (81.9%) patients survived the operation and were followed up for periods ranging from 2 months to 17 years. The total follow-up time was 6290 patient/months (mean, 92 months). There were 15 late deaths, but in six, the cause was not related to the underlying disease. Four (5.8%) patients presented recurrence of the fibrosis and were reoperated on and in six (8.8%), EMF appeared in the other ventricle. Five (7.3%) patients were reoperated on to replace either a valve prosthesis or a native valve which had been preserved during the first procedure. Only 24 (45%) of the 53 surviving patients are in functional class I or II. The actuarial probability of survival at 17 years, including operative mortality, was 55%. Conclusion: Surgical treatment of EMF should be considered a palliative procedure because surgery does not alter the progressive nature of the disease. However, surgical therapy is recommended for patients with EMF and heart failure as it is their only hope of survival.

Key Words: Endomyocardial fibrosis • Cardiomyopathy • Surgery

	Introduction

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Surgical treatment of endomyocardial fibrosis (EMF) introduced by Dubost [1] in 1971, consists of endocardial decortication and atrioventricular valve replacement or repair and is a well-established procedure. The operative technique as well as the early and intermediate results of surgery have been extensively described [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15], but there are few reports regarding the long-term outlook of the operated patients [16] [17]. Herein is a review of our experience over the last 20 years with surgery for EMF, with the aim of identifying the life expectancy of the operated patients and the events that influence it.

	Patients and methods

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From December 1977 to December 1997, 83 patients with EMF were operated upon in our institutions. There were 66 (79.6%) female and 17 (20.4%) male patients, ranging in age from 4 to 59 years (mean, 31).

According to the New York Heart Association Functional Classification, all patients were in class III or IV. Thirty-seven (44.5%) had biventricular disease, 34 (41.0%) had involvement of the right ventricle alone, and 12 (14.5%) had EMF confined to the left ventricle.

The clinical and hemodynamic aspects of the patients, the surgical technique and the early results of our experience have been previously reported in detail [5] [8] [14]. Follow-up assessments of the surviving patients was performed in our institution and included clinical examination, chest X-ray, electrocardiogram and echocardiogram. Postoperative hemodynamic and angiographic studies were performed in 22 patients. Detailed pre- and postoperative clinical and hemodynamic aspects of the patients have been reported [5] [14], and in this study we have analyzed the late mortality and its causes, the progression of the disease characterized either by recurrence of the fibrosis or appearance of EMF in the other ventricle, the need for reoperation, the functional status and, finally, the actuarial probability of survival by the Kaplan–Meier method.

	Results

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Sixty-eight (81.9%) patients survived the operation and were followed up for periods ranging from 2 months to 17 years. The total follow-up time was 6290 patient/months (mean, 92 months).

Mortality
Fifteen (18.0%) late deaths occurred in periods ranging from 2 months to 15 years after operation (Table 1). The causes of late deaths were intractable heart failure (n=4), cerebral embolism (n=2), pulmonary embolism (n=2), low cardiac output after reoperation for recurrence of fibrosis (n=2), sepsis (n=2), infectious endocarditis (n=1), hepatic cirrhosis (n=1) and gastrointestinal infection (n=1).

Late mortality was not affected by the localization (uni-or biventricular) of the disease or by whether the atrioventricular valves were replaced or preserved. Among the 68 survivors, 41 had univentricular EMF with nine deaths (21.9%) and 27 had biventricular disease with six deaths (22.2%). In 51 surviving patients in whom atrioventricular valve was replaced, late mortality was 23.5% (12 cases) while in 17 who had the valves preserved the mortality was 17.6% (three patients).

Progression of the disease
Six (8.8%) out of 68 survivors presented echo and/or angiographic evidence of EMF in the non-operated ventricle, which was normal at the time of surgery. None have been reoperated on yet.

Four (5.8%) other patients with biventricular disease presented recurrence of the fibrosis in the left ventricle 3, 11, 12 and 15 years after surgery. One of them also had dysfunction of the mitral bioprosthesis and another, in whom the native valve was preserved in the first operation, had severe mitral incompetence. All were reoperated on and two died. The surgical and microscopic aspects of the fibrous tissue were similar to those seen in primary cases. Both operations were performed by the same surgical team which eliminates the possibility that recurrence could be the result of insufficient resection at the initial operation.

None of the patients who developed progression of EMF in the late postoperative period presented episodes of eosinophilia.

Need for reoperation
Besides the four patients mentioned above in whom recurrence of the fibrosis has occurred, five (7.3%) others have been reoperated on either to replace a valve prosthesis (n=4) or a native mitral valve preserved at the first operation (n=1).

Functional status
Fifty-three (63.8%) patients are alive at the time of writing but only 24 (45.2%) of them are in functional class I or II ( Fig. 1 ).

View larger version (15K): [in this window] [in a new window]   Fig. 1. New York Heart Association functional classification of 53 survivors.

View larger version (19K): [in this window] [in a new window]   Fig. 2. Seventeen-year actuarial survival curve.

	Discussion

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The irreversible nature of EMF is well-known and the clinical course depends upon the severity of the ventricular fibrosis and the degree of mitral and/or tricuspid incompetence [22]. Surgical treatment should be recommended in all symptomatic patients. Early results of surgery have been reported in several series with an operative mortality of around 18% [2] [3] [4] [5] [6]. In one series, in which it was possible to preserve all valves, mortality was reduced to 4.6% [7]. It has also been reported that surgery improves functional capacity and the quality of life in the majority of surviving patients but their long-term outlook has still to be analyzed in detail.

In the present series, we have followed up 68 survivors for a mean period of time of 7 years and 8 months. Late mortality was 18% (15 patients) but in six cases the cause of death was not directly related to the underlying disease. The other nine patients presented two different types of clinical course: five patients remained in severe heart failure after surgery and died during the first postoperative year and four others died many years after the operation due to the progression of the disease after long periods of excellent clinical improvement.

In our opinion, the lack of clinical improvement after surgery observed in some patients with EMF can only be explained as being the result of severe infiltration of the myocardium by fibrous tissue. It is well-known that in this disease strands of fibrous tissue often extend to a varying degree into the inner myocardium [21]. Unfortunately the severity of myocardium involvement cannot be evaluated preoperatively. Therefore, it is difficult to predict which patients would really be benefited from endocardial resection from those suitable only for heart transplantation.

The appearance of endocardial fibrosis in the postoperative period either in the operated ventricle (recurrence) or in the non-operated one, demonstrates that EMF is an evolving disease and that surgery does not alter its progressive nature. Efforts should be concentrated on the elucidation of its etiology, which would permit the application of preventive measures or early medical treatment, thereby avoiding the development of the fibrosis.

Another factor that may adversely affect the postoperative course is valve bioprosthesis dysfunction and every effort should be made to preserve the native valve during the operation. Unfortunately, in our experience, this was only possible in 30% of the cases due to the advanced stage of the disease in the majority of the patients [14].

The analysis of the functional status of the patients in the late postoperative period showed that only 45.2% of the 53 actual survivors are in functional class I or II. The 17-year probability of survival was 55%. These results are similar to that recently reported [17]. Thus, results of surgical treatment of EMF are far less satisfactory than desired, but seems to be superior to the poor outlook of medically treated patients as previously reported [10]. In conclusion, surgery for EMF should be considered a palliative procedure because it does not alter the progressive nature of the disease. However, surgical therapy is recommended for patients with EMF and heart failure as it is their only hope of survival.

	Footnotes

 
Presented at 12th Annual Meeting of the European Association for Cardio-thoracic Surgery, Brussels, Belgium, September 20–23, 1998.

	Appendix A. Conference discussion

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Dr M. Turina (Zurich, Switzerland): The disease is rare in an endogenous population in Europe. Our own experience it is much smaller; I think it is 21 patients now. It is somewhat at variance and I would like to hear your opinion about it. We were lucky in having no operative mortality in these patients, but we have observed three patients who had a fairly rapid course of this disease with a recurrence of symptoms. It is a profound muscular infiltration, death within a year after surgery. All three of them had high eosinophilic counts, and I am questioning the wisdom of doing this operation in the patient with an acute phase of hypereosinophilic syndrome, which cannot be controlled by drugs, which are usually used to treat leukemia and similar disorders. So my question is, do you operate on the patients with an acute hypereosinophilic syndrome?

My second comment is a short one. In our experience gained with valve reconstruction, it is practically always possible to leave the valve reconstructed and still perform an adequate resection of the subendocardial fibrosis.

Dr Moraes: The first answer is no, we haven't operated on the acute phase of hypereosinophilia. However, regarding the preservation of the valve, perhaps we have operated on our patients in the end-stage of the disease where our valve apparatus is compromised, and in our hands we could not preserve most of the valves; only 30% of the valves in our experience were preserved. There are some groups that have recognized to operate on these patients earlier where the valve is not so compromised and can in these situations preserve the valve.

Dr U. Althaus (Bern, Switzerland): In your very impressive series, endomyocardial fibrosis predominantly affected the right ventricle. If I correctly have your figures, then 41% of your patients had this disease confined to the right ventricle but only 14% to the left ventricle alone. As you certainly know, in other reports endomyocardial fibrosis is affecting the left ventricle more frequently than the right ventricle. Could you comment on this discrepancy?

Dr Moraes: Well, this observation has been seen in many series. For instance, I have read the paper from the group of Professor Turina that he has presented and published in the Heart Journal, and I think all these series that have been presented have this proportion. I mean, most of the patients have the right ventricle compromised.

Dr H. Borst (Munich, Germany): As you said, you are now operating on patients with active disease. How did you define activity – clinically or by laboratory tests?

Dr Moraes: In our series we have made histology, and we haven't seen eosinophils under the microscope. Another observation is that only 10% of our patients present some degree of eosinophilia. Another observation was that between the first and the second operation in those patients who presented recurrence, we didn't observe periods of eosinophilia. So that is why we think that eosinophilia is not the main cause of this disease, the etiology, I mean.

Dr Althaus: You certainly know that some years ago, a fibroblast-stimulating hormone produced by eosinophils has been postulated, which may induce fibrosis of the ventricles. Based on your study, this hypothesis cannot be supported by your data. Dr Moraes: I cannot answer that.

	References

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