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. 1998 Apr;79(4):362–367. doi: 10.1136/hrt.79.4.362

Long term follow up of patients with endomyocardial fibrosis: effects of surgery

U Schneider 1, R Jenni 1, J Turina 1, M Turina 1, O Hess 1
PMCID: PMC1728653  PMID: 9616343

Abstract

Aims—To determine the long term outcome of patients with endomyocardial fibrosis and to compare echocardiographic and haemodynamic data before and after ventricular endocardial resection.
Patients—Seventeen patients (11 women and six men; mean age 35.5 years) diagnosed with endomyocardial fibrosis at the University Hospital in Zurich, Switzerland from 1971 to 1995. Twelve patients (70%) had partial obliteration of both ventricles and in five patients (30%) the fibrotic lesions were limited to the left ventricle. 
Methods—Fourteen of the 17 patients had surgical resection: fibrosis was resected from both ventricles in five patients and from the left ventricle only in nine patients. Ten patients had mitral valve replacement and two had tricuspid valve replacement. Left ventricle endocardial resection was done without reconstruction or replacement of the atrioventricular valve in three patients. Preoperative and postoperatve echocardiographic data were available for 11 patients and haemodynamic data for six patients. Patients were followed up for 0.4-19 years (mean 8.6).
Results—Preoperatively four patients were NYHA functional class IV and 10 were class III; postoperatively one patient was class III, seven class II, and six class I. Preoperatively, echocardiography showed obliteration of the left ventricular apex and inflow tract in all patients, which decreased or disappeared after surgery. Left ventricular end diastolic pressure decreased from 25 mm Hg before surgery to 14 mm Hg after successful resection of the fibrosis. Left ventricular end diastolic volume (normal 93 (17) ml/m2) increased from 65 ml/m2 to 97 ml/m2 (p < 0.05) after surgery. Ejection fraction was normal preoperatively (57%) and decreased slightly (52%) after surgery. One patient died five months after surgery from heart failure. Four surgically treated patients died during the follow up period: one each from systolic dysfunction, recurrence of endomyocardial fibrosis, pneumonia, and food poisoning. Overall survival was 65% at five years and 59% at 10 years; the survival rates of the operated patients was 72% and 68%, respectively. Only one of the medically treated patients survived longer than three years from diagnosis.
Conclusions— Endomyocardial fibrosis is a rare disease in European countries and is found mainly in women. The clinical picture is characterised by severe congestive heart failure but heart size is only moderately increased. Systolic performance is normal or only slightly depressed despite severe restriction to filling, atrioventricular valve regurgitation or both. Partial obliteration of the right and/or left ventricle may be detected by echocardiography. Endocardial resection with atrioventricular valve replacement is the treatment of choice with appreciable postoperative improvement and 10 year survival of approximately 70%.

 Keywords: endomyocardial fibrosis;  atrioventricular valve regurgitation;  endocardial resection;  hypereosinophilic syndrome

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Figure 1  .

Figure 1  

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Preoperative (top) and postoperative (bottom) cross sectional echocardiogram (apical four chamber view) of a 52 year old Swiss patient with left ventricular endomyocardial fibrosis and hypereosinophilic syndrome (myeloproliferative disease). The obliteration of the apex (arrow) can be clearly seen. The left and right atria are enlarged owing to the restriction to filling. Postoperatively the obliteration is no longer present (arrow).

Figure 2  .

Figure 2  

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Echocardiographic follow up data in the same patient as fig 1. Arrow indicates time of operation. Preoperatively, there is progressive enlargement of the left atrium (A) and a decrease in systolic shortening (B). Postoperatively, left atrial size decreases and systolic shortening improves. LVED, left ventricular end diastolic diameter; LA, left atrial end systolic diameter; LVES, left ventricular end systolic diameter; Ao, aortic diameter.

Figure 3  .

Figure 3  

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Left ventricular end diastolic pressure (LVEDP) before and after endocardial resection. There is a significant fall in left ventricular end diastolic pressure after surgery nearly in all patients.

Figure 4  .

Figure 4  

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Cumulative survival rates in medically and surgically treated patients with endomyocardial fibrosis. After five and 10 years approximately two thirds of all operated patients were alive, but only one of three medically treated patients.

Figure 5  .

Figure 5  

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Left ventricular end diastolic pressure (LVEDP) volume (EDVI) relation in six patients before and after endocardial resection. There is a clear tendency for normalisation of LV diastolic dysfunction (high LVEDP and small LV end diastolic volume) after surgical removal of the fibrotic plaque. Normal values are taken from reference 17. 

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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