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CURRICULUM IN CARDIOLOGY Endomyocardial fibrosis: Report on the hemodynamic data in 29 patients and review of the results of surgery George Cherian, D.M., G. Vijayaraghavan, D.M., S. Krishnaswami, D.M., I. P. Sukumar, D.M., S. John, M.S., P.S. Jairaj, F.R.A.C.S., and A. Bhaktaviziam, M.D. Vellore, India Endomyocardial fibrosis (EMF) is an unusual type of cardiomyopathy characterized by a restriction to ventricular filling and an obliteration of the inflow portion of the ventricular cavity by fibrosis and often by thrombus formation. The outflow tract is spared. The fibrosis involves the base of the papil- lary muscles and at times the chordae or cuspsl** The fibrous strands extend into the myocardium, resulting in further restriction of filling and pump failure. The presentation is complicated by varying degrees of primary atrioventricular valvar regurgita- tion, pump failure, and pericardial effusion. Recent interest in this condition is due to the increasing recognition of this condition outside the “tropical” zone,3-6 a possible role for abnormal eosinophils in the pathogenesis,7-g and the good long-term results of an aggressive surgical approach with endocardial stripping and valve replacement.5, lo* l1 For purposes of surgical management, it is impor- tant to distinguish between biventricular, dominant right ventricular, and dominant left ventricular dis- ease. This report deals with the hemodynamic data in 29 of our patients and also reviews the results of surgery in this series and from the literature. METHODOLOGY Patients studied. This report is based on 29 patients with EMF seen at the Christian Medical College Hospital, Vellore, India. Twelve others with incomplete data have been excluded. Six patients From the Departments of Cardiology, Cardiac Surgery, and Pathology, Christian Medical College Hospital. Received for publication May 3, 1982; revision received Oct. 13. 1982; accepted Oct. 28, 1982. Reprint requests: George Cherian, D.M., Cardiology Dept. (691/111E), Wadsworth VA Medical Center, Wilshire and Sawtelle Blvds., Los Angeles, CA 90073. underwent surgical procedures, and autopsies were performed in two. Cardiac catheterization. All patients had cardiac catheterization and selective angiocardiography using standard techniques. Simultaneous pressures from the pulmonary artery and right atrium and between the right ventricle and right atrium were recorded in five patients through equisensitive transducers. Diagnosis. The diagnosis of RVEMF was based on previously described angiographic criteria.12s l3 There is obliteration of the apical inflow portion of the right ventricle contrasted with a vigorously con- tracting and often dilated right ventricular outflow tract and varying degrees of tricuspid regurgitation. Filling defects and calcification may be present in the apical portion. The diagnosis of LVEMF was made from angio- graphic criteria. l4 The apical portion of the left ventricle is obliterated giving it a globular or square shape, and it may be further distorted with out- pouchings like small aneurysms. Varying degrees of mitral regurgitation will be present. Filling defects or calcification may be present at the apex. A raised ventricular end-diastolic pressure and dip-diastolic pressure are present in symptomatic patients. In the early or mild cases with only slight distortion of the left ventricle and or mild mitral regurgitation, the diagnosis can be suspected only when these signs are associated with the characteristic changes in the right ventricle. The diagnosis of biventricular EMF (BVEMF) was based on evidence of involvement of both ventricles. The severity of mitral and tricuspid regurgitation was graded using angiographic crite- ria.15 The left ventricular ejection fraction was not calculated because of the mitral regurgitation and distortion of the left ventricle. Contractility was graded as good (+++), fair (++), or poor (+). 659
Transcript
Page 1: Endomyocardial fibrosis: Report on the hemodynamic data in 29 patients and review of the results of surgery

CURRICULUM IN CARDIOLOGY

Endomyocardial fibrosis: Report on the hemodynamic data in 29 patients and review of the results of surgery

George Cherian, D.M., G. Vijayaraghavan, D.M., S. Krishnaswami, D.M., I. P. Sukumar, D.M., S. John, M.S., P.S. Jairaj, F.R.A.C.S., and A. Bhaktaviziam, M.D. Vellore, India

Endomyocardial fibrosis (EMF) is an unusual type of cardiomyopathy characterized by a restriction to ventricular filling and an obliteration of the inflow portion of the ventricular cavity by fibrosis and often by thrombus formation. The outflow tract is spared. The fibrosis involves the base of the papil- lary muscles and at times the chordae or cuspsl** The fibrous strands extend into the myocardium, resulting in further restriction of filling and pump failure. The presentation is complicated by varying degrees of primary atrioventricular valvar regurgita- tion, pump failure, and pericardial effusion. Recent interest in this condition is due to the increasing recognition of this condition outside the “tropical” zone,3-6 a possible role for abnormal eosinophils in the pathogenesis,7-g and the good long-term results of an aggressive surgical approach with endocardial stripping and valve replacement.5, lo* l1

For purposes of surgical management, it is impor- tant to distinguish between biventricular, dominant right ventricular, and dominant left ventricular dis- ease. This report deals with the hemodynamic data in 29 of our patients and also reviews the results of surgery in this series and from the literature.

METHODOLOGY

Patients studied. This report is based on 29 patients with EMF seen at the Christian Medical College Hospital, Vellore, India. Twelve others with incomplete data have been excluded. Six patients

From the Departments of Cardiology, Cardiac Surgery, and Pathology, Christian Medical College Hospital.

Received for publication May 3, 1982; revision received Oct. 13. 1982;

accepted Oct. 28, 1982.

Reprint requests: George Cherian, D.M., Cardiology Dept. (691/111E), Wadsworth VA Medical Center, Wilshire and Sawtelle Blvds., Los Angeles, CA 90073.

underwent surgical procedures, and autopsies were performed in two.

Cardiac catheterization. All patients had cardiac catheterization and selective angiocardiography using standard techniques. Simultaneous pressures from the pulmonary artery and right atrium and between the right ventricle and right atrium were recorded in five patients through equisensitive transducers.

Diagnosis. The diagnosis of RVEMF was based on previously described angiographic criteria.12s l3 There is obliteration of the apical inflow portion of the right ventricle contrasted with a vigorously con- tracting and often dilated right ventricular outflow tract and varying degrees of tricuspid regurgitation. Filling defects and calcification may be present in the apical portion.

The diagnosis of LVEMF was made from angio- graphic criteria. l4 The apical portion of the left ventricle is obliterated giving it a globular or square shape, and it may be further distorted with out- pouchings like small aneurysms. Varying degrees of mitral regurgitation will be present. Filling defects or calcification may be present at the apex. A raised ventricular end-diastolic pressure and dip-diastolic pressure are present in symptomatic patients. In the early or mild cases with only slight distortion of the left ventricle and or mild mitral regurgitation, the diagnosis can be suspected only when these signs are associated with the characteristic changes in the right ventricle. The diagnosis of biventricular EMF (BVEMF) was based on evidence of involvement of both ventricles. The severity of mitral and tricuspid regurgitation was graded using angiographic crite- ria.15 The left ventricular ejection fraction was not calculated because of the mitral regurgitation and distortion of the left ventricle. Contractility was graded as good (+++), fair (++), or poor (+).

659

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Table I. Hemodynamic data __--

RVEMF BVEMF L VEMF 14 cases (A) 12 cases (B) 3 cases (C) Significance (p value)

Variable Mean SD Mean SD Mean SD A us B B us C C us A

Cardiac index 2.56 + 0.45 2.4 + 0.39 2.9 a 1.59 NS NS NS RA (mean) 13.6 !I 4.10 16.4 + 6.28 4.7 + 1.53 NS <0.05 <O.Ol RV dip-diastolic 8.83 2 4.87 9.54 k 5.66 0 NS co.05 co.05 RVEDP 14.2 c 4.14 16.5 rt 4.92 7.0 k 2.65 NS <0.05 co.05 RVEDP/Systolic ratio 67.9 5 17.02 57.1 AZ 25.5 9.5 -i- 0.87 NS <0.05 <O.Ol PA diastolic 11.5 k 2.55 19.4 f 9.63 44.7 k 15.01 <0.05 <O.Ol <O.OOl

PA (mean) 15.0 k 2.61 24.5 + 12.14 57.7 + 15.63 <0.05 co.01 <O.OOl

LV dip-diastolic 1.7 f 2.49 4.7 t 4.51 12.5 + 4.95 <0.05 <0.05 <O.OOl LVEDP 8.9 + 3.25 18.4 AZ 11.42 26 + 2.83 <0.05 NS <O.OOl

Cardiac index in L/min/m*; all pressures in mm Hg. RA = right atria1 pressure; RV = right ventricular; PA = pulmonary artery pressure; LV = left ventricular; EDP = end-diastolic pressure; BV = biventricu-

lar: EMF = endomyocardial fibrosis.

Dominant or lone disease. In this report the term the disease. Of the 10 patients in sinus rhythm, right or left ventricular EMF refers to patients in seven (70 % ) had an “a” wave impression on the whom the disease (after cardiac catheterization and right ventricular tracing (Figs. 1 and 2.). A plateau angiography) was judged to be confined to one or the in diastole was found only during the longer diastol- other ventricle, or with dominant involvement of ic pauses with atria1 fibrillation. The right ventricu- one ventricle with only minimal angiographic lar systolic pressure ranged from 15.7 to 28 mm Hg changes in the contralateral ventricle. and averaged 21 mm Hg.

OBSERVATIONS

General. The basic hemodynamic data are shown in Table I. The mean right atrial pressure and the right ventricular end-diastolic pressure were signif?- cantly higher in RVEMF and BVEMF. The left ventricular end-diastolic pressure was raised in LVEMF and BVEMF and pulmonary hypertension was present in both these groups.

Right ventricular EMF. Fourteen patients with an average age of 16.9 years had dominant involvement of the right ventricle. Four were in atrial fibrillation and 10 were in sinus rhythm. None showed any hemodynamic alterations on the left side, while in 3 of the 14 there were minimal changes in the shape of the left ventricle or trivial mitral regurgitation. In some the right ventricular and right atrial pressure tracings were similar (Fig. 1). A pericardial effusion, confirmed at cardiac catheterization, was present in three cases.

The pulmonary artery systolic pressure ranged from 14 to 25 mm Hg and averaged 19.3 mm Hg. Of the 10 patients in sinus rhythm, six (60%) had an “a” wave impression on the pulmonary artery (Fig. 2). Fig. 3 is a catheter tip transducer tracing showing an “a” wave in the right atrium, right ventricle, and pulmonary artery. There was a diastolic pressure gradient between the right ventricle and pulmonary artery ranging from 2 to 5.7 mm Hg and averaging 3.6 mm Hg, being higher in the right ventricle in 11 out of the 14 patients.

The right atrial mean pressure was higher with atrial fibrillation (average 16.6 mm Hg) than in those patients in sinus rhythm (average 12.3 mm Hg), and was less (average 10.1 mm Hg) in those patients with a dominant “a” wave, as compared to those with a dominant “v” wave (14.6 mm Hg). The patients with the higher right ventricular dip- diastolic pressures were all in atrial fibrillation and the majority had prominent “v” waves in the right atria1 tracing, suggesting a more advanced stage of

Selective angiocardiography. The right atrium was enlarged, usually markedly, in all but one patient who had no tricuspid regurgitation. Filling defects were present in the right atrium in four patients (28.5 % ). The distinctive obliteration of the apical inflow portion of the right ventricle was seen in all 14 patients and calcification in this area was present in three patients (21.4 % ). The characteristic increased contractions of the right ventricular out- flow tract were seen in all 14 patients and in seven the outflow tract was also dilated. Tricuspid regurgi- tation was present in all but one patient. It was judged to be grade II/IV in four and grade III/IV in nine patients.

Left ventricular EMF. Three patients with an aver- age age of 14.8 years had dominant LVEMF and no angiographic or hemodynamic evidence of right ventricular involvement. Two were in sinus rhythm and 1 was in atrial fibrillation. All had pulmonary

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Number 4 Endomyocardial fibrosis: Hemodynamic types/Surgical procedures 661

RV i- RA

mmHg I

Fig. 1. These tracings shows similar pressures in the right ventricle (RV) and right atrium (RA) in a patient in sinus rhythm. The dip and end-diastolic pressures are high in the RV and both tracings show a prominent “a” wave, except for the atrial premature beat (arrow).

k b

“-as-.. mmHg

Fig. 2. Panel A shows simultaneous pulmonary arterial (PA) and right atrial (RA) pressures with a dominant “a” wave in the RA, which is seen also in the PA tracing. Panel B shows simultaneous right ventricular (RV) and RA pressures with a dominant “a” wave in the RA, which is seen also in the RV tracing.

hypertension. One patient had a 6 mm Hg diastolic gradient across the mitral valve. None had a pericar- dial effusion.

Selective angiocardiography. There was no angio- graphic evidence of right ventricular involvement. Apical obliteration and filling defects in the left ventricle were present in all three patients. The left ventricle showed multiple outpouchings in all, with a square shape in one and was judged to be slightly dilated. The overall contractility of the distorted ventricle was fair (++) in two patients and poor (+) in one patient. All patients had grade III/IV mitral

regurgitation. None had prolapse of the mitral valve.

Biventricular EMF. Twelve patients with an average age of 19.7 years had biventricular disease with significant involvement of both ventricles, as judged by angiography and hemodynamic changes. Six were in atrial fibrillation and six were in sinus rhythm. Three patients had a pericardial effusion. Patients could be divided into two groups, eight without and four with pulmonary hypertension.

In the eight patients without pulmonary hyper- tension, the pulmonary artery mean pressure aver-

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Fig. 3. Catheter-tip transducer recording of withdrawal from pulmonary artery (PA) to the right atrium (RA). An “a” wave is seen throughout with a raised dip and end-diastolic pressure in the right ventricle.

Table II. Serial cardiac catheterization

Variable Age 7 years Age 13 years

Cardiac index 3.1 2.7 Right atrium a 12; v 8; 5 AF; v 22.4; 18.8 Right ventricle 18/O-12 21/11-15.5 Pulmonary artery 16/10; n 21/11.5; i7 Left ventricle 104/O-12 10015-8 Arterial pressure 104175; ss 103/63; @

Cardiac index in L/min/m$ all pressures in mm Hg. AF = atria1 fibrillation.

aged 16.9 mm Hg and all had a high right ventricular systolic to end-diastolic pressure ratio (average 73% ) and a diastolic pressure gradient across the pulmonary valve (average 4.3 mm Hg). These fea- tures were not seen in the four patients with pulmo- nary hypertension, whose mean pressure averaged 39.8 mm Hg.

Selective angiocardiography. There was angio- graphic evidence of biventricular involvement. Fill- ing defects in the left ventricle were present in five patients. The left ventricle showed outpouchings in seven patients, with a square shape in five, was slightly dilated in six patients, was normal in five, and was small in one patient. The overall contractil- ity of the ventricle was good (+++) in 11 patients and poor (+) in one. All patients had mitral regurgi- tation which was grade I/IV in eight, grade II/IV in two, and grade III/IV in the remaining two. No patient had prolapse of the mitral valve.

Progression. Serial cardiac catheterization was performed in one male patient; the results are shown

in Table II. He was asymptomatic at 7 years when first seen and was in congestive cardiac failure at the time of the second study, 6 years later. Surgical correction was advised but was refused. He died 2 years later and an autopsy was not permitted.

Results of medical treatment. The follow-up infor- mation, though incomplete, is available on 17 of the 23 patients who did not undergo operations. Two were asymptomatic when first seen and one contin- ues to be so during a 3-year follow-up, while the other patient died 8 years later (see preceding paragraph). Out of the .three patients who were grade II symptomatically, one has not progressed over a 4-year period and the other two were lost to follow-up. Eighteen patients were in cardiac failure when seen. Eight of them have been followed up at another hospital, where five had repeated hospital admissions and three died over a 2-year period. Of the remaining 10 six are known to have died, be- cause the letters were returned as “addressee dead,” but the length of survival is not known; the other four were lost to follow-up.

Results of surgery. Six patients, who were all in congestive heart failure, underwent surgical proce- dures. Three patients aged 12,18, and 22 years with RVEMF had right ventricular endocardiectomy and tricuspid valve replacement, two with a Bjiirk-Shiley and one with an Ionescu-Shiley prosthesis. One patient died in the early postoperative period from a low cardiac output state. The 12-year-old has been followed up for 3 years and the 22-year-old for 8 months. Both are asymptomatic.

Three patients aged 4, 10, and 12 years with

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Table III. Surgical series: Endocardiectomy and valve replacement

Race Ventricles

Author and country Number Age (~4 White Other One Both

Dubost”’ France

Hess et al.s Switzerland

Moraes et al.” Brazil

Bertrand et al.”

Ivory Coast Cherian et al.*

India

15 12-58 6 9 RV 7 6 LV 2

4 19-22 - - LV3 1

6 14-48 - 6 RV 4 1 LV 1

31 - - -

6 4-23 - 6 RV 6

*present series includes three Glenn shunts. RV = right ventricle; LV = left ventricle.

RVEMF underwent the placement of Glenn shunts between the superior vena cava and the pulmonary artery. The lo-year-old died soon after surgery from a large pneumothorax and at autopsy was confirmed to have disease confined to the right side. The other two patients have been followed up for 4 years. There was symptomatic improvement, decrease in ascites, a reduction in the dose and frequency of diuretics, and both patients have continued to be in sinus rhythm. The results of surgical procedures performed for endomyocardial fibrosis are shown in Table III.

COMMENTS

EMF, first reported from equatorial Africa, is a common form of cardiomyopathy in Central Afri- ca17, 18 and is encountered less often in South Ameri- Cal9 and Asia.20 Recent reports from France,4*10 Swit- zerland,5 England,6*21 and the USA3p22s23 describing patients who have never been in the tropics, point to a worldwide distribution. The clinical features of EMF have been well described and the diagnosis is readily suspected at centers where the condition is not uncommon.‘4n 17, l8 Sometimes the diagnosis may be made only at the time of valve rep1acement24 or at surgery for a cardiac tumor,3* 23 or at autopsy.7, 25

Etiology and abnormal eosinophils. The etiology is not known. Endomyocardial disease has been de- scribed in a variety of conditions associated with eosinophilia, particularly from the West.7sg Eosino- philia has not been a feature in the African29 l* or South American reportslg and was found in only one patient in our series. Eosinophilia was found in 20% of the EMF patients reported from France’O and Switzerland.5 While it is known that abnormal, degranulated vacuolated eosinophils can be associ- ated with endomyocardial damage,8 the relationship

between Loffler’s endomyocardial disease and tropi- cal endomyocardial fibrosis is not clear.7pg

Diagnosis. The diagnosis of EMF is confirmed from the angiographic12* l4 and hemodynamic altera- tions. There are no reports on the value of endocar- dial biopsy in early cases,26 and minimal involve- ment of one or the other ventricle may be missed in the clinical series. Recent reports suggest that the diagnosis may be confirmed with two-dimensional echocardiography.“* 28

Hemodynamic changes. Some of the hemodynamic features of EMF have been reported in the past, but most series have dealt with mixed forms of the disease without separating them into right, left, and biventricular involvement.1g*29*30 The majority of patients have been in atrial fibrillation,1g~30 while in our series 62% were in sinus rhythm.

Right ventricular EMF. In RVEMF the normal or low right ventricular systolic pressure with a raised end-diastolic pressure resulted in a high systolic/ end-diastolic ratio (average 67.7 % ), higher than found in constrictive pericarditis.17

Brockington et al.,” using a catheter-tip transduc- er, found that in patients with RVEMF the proto- diastolic trough in the pulmonary artery was less deep than in the right ventricle, suggesting that the pulmonary valve closed for only about 0.02 second. We found a diastolic pressure gradient across the pulmonic valve both with atrial fibrillation and in sinus rhythm in 11 of 14 patients with RVEMF. This diastolic flow across the pulmonic valve would appear to be one way of augmenting forward flow in RVEMF as the end-diastolic ventricular pressure gradually increased. Seven out of the 10 patients with RVEMF in sinus rhythm had an “a” wave on the right ventricular tracing, and an “atrial kick” would be an important finding with restrictive ven-

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tricular disease. Six out of the 10 patients also had an “a” wave on the pulmonary artery tracing corre- sponding in height to the right atrial “a” wave with the expected phase delay. It is not clear if this represents further presystolic opening of the pul- manic valve, since an “a” wave can be recorded also on the normal pulmonary valve echograrn31 In the case reported by Blair et a1.,25 the peak pressure wave recorded in the right ventricle and pulmonary artery began after the P wave and before the QRS complex.

Tricuspid regurgitation was present in all but one of the patients with RVEMF. With the valve appa- ratus enmeshed in the fibrotic process resulting in an open tricuspid valve and with an open pulmonic valve from diastolic flow, the right ventricle may not be able to generate significant isometric rise in pressure. Thus even severe tricuspid regurgitation may be present without a systolic murmur and the pressure tracings from the pulmonary artery, right ventricle, and the right atrium may be similar in the late stages.

Serial catheterization showed a rise in right atrial mean pressure and the right ventricular dip and end-diastolic pressure. These pressures were also higher in patients with atrial fibrillation as com- pared to those in sinus rhythm, and may indicate a more advanced stage of the disease.

Left ventricular EMF. The possibility of EMF as an etiologic factor should be considered in patients with mitral regurgitation. 14yz4 In EMF, unlike other forms of mitral regurgitation, the left ventricle is not dilated and may be small. The average left ventricu- lar diastolic volume was 68 + 20 ml/m2 in one report,5 and angiographically the left ventricle was not dilated in any of our cases. Unlike rheumatic mitral regurgitation, the apical impulse in EMF is not prominent and the murmur is often an early systolic murmur with late systolic decrescendo.14 In EMF the symptoms and the raised ventricular dip and end-diastolic pressures are often out of propor- tion to the size of the left ventricle and the degree of regurgitation. Significant pulmonary hypertension was found in all our patients. A rheumatic etiology cannot be excluded with certainty without left ven- triculography. l4 The left ventricular ejection frac- tion was reported to be about 61 f 8% in one series5 and the end-systolic size was judged to be increased in the majority of our patients.

Biventricular EMF. Biventricular involvement is more common than left ventricular disease. In 8 of 12 patients the left ventricular and pulmonary artery pressures were normal and there was a dia-

stolic pressure gradient across the pulmonary valve, as with RVEMF.

Pericardial effusion. It is difficult to evaluate the hemodynamic consequences of pericardial effusion in a disease process associated with restriction to ventricular filling. There was no significant differ- ence in cardiac output in our patients with and without a pericardial effusion. There are, however, reports about patients presenting with a dominant or recurrent pericardial effusion, benefiting from pericardiocentesis, pericardial window, and pericar- dial shunts.32

Indications for surgery. The reported average sur- vival period on medical therapy after the onset of symptoms is 2 years,” and the prognosis was poor in our nonoperated cases as well. Digitalis and diuret- ics are initially useful, as with other patients in cardiac failure. The effect of vasodilators was not tried in our patients and there are no references to this form of therapy in EMF. In another series of 17 patients who were not operated, there were four deaths, three patients continued in cardiac failure, and 10 were in hepatic or renal failure, while there was improvement in all those who survived the operation.16 With the current surgical results (Table III), surgery would be indicated in all patients who are significantly incapacitated (functional grade III/ IV) or in congestive heart failure.

Pathologic evidence suggests that there is an active early stage with inflammatory edema, necro- sis, and cellular infiltration.2 The late stage is marked by fibrosis, thrombus formation, and an absence of cellular infiltration.‘12 There are no clear pointers to decide about “activity” in individual patients. Eosinophilia, when present, implies active disease and at times eosinophilia may reappear during the course of the disease.5’9 We found the erythrocyte sedimentation rate useful during follow- up. Atria1 fibrillation represents a late stage of the disease.

Surgical procedures. Surgery in EMF may be pal- liative or corrective. Pericardiectomy, pericardial windows/and pericardioperitoneal shunts have been done for patients with recurrent pericardial effu- sion.32 The Glenn shunt has been performed in cases of RVEMF to augment pulmonary flo~.~~ Hopefully, any decrease in the size of the right atrium will also delay the onset of atria1 fibrillation. Two of our patients improved during a 4-year follow-up and continued in sinus rhythm but required continuing treatment for cardiac failure.

Stripping of the diseased endocardium (endocar- diectomy) and atrioventricular valve replacement

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Endomyocardial fibrosis: Hemodynamic types ISurgical procedures 665 Volume 105

Number 4

was first reported by Dubost et al. in 1976.4 The major surgical series are shown in Table III. A variety of prosthetic and tissue values have been used. Endocardiectomy with2’ or withoutz3 valvulo- plasty has also been done in two patients in whom the degree of valve dysfunction did not warrant valve replacement.

Results of surgery. The early mortality with or without valve replacement has been 19%, being higher in those with biventricular disease.10s11*16 A high incidence of AV block following tricuspid valve replacement was found in one series,‘O but was not encountered in other reports.11*‘6 There were two late cardiac deaths, one from arrhythmias and the other from thrombosis of the prosthesis.‘O

The results in those patients who were followed up has been satisfactory, with either no or minimal symptoms. The follow-up period has ranged from 8 months to 8 years, and the results appear to be comparable to that of atrioventricular valve replace- ment.‘O In the largest series,” the mean follow-up was 15 months with “clinical, radiological and hemo- dynamic improvement.” Postoperative cardiac cath- eterization has shown an increase in cardiac output, a fall in ventricular diastolic pressures, an increased ventricular volume, and a normalization of the shape of the left ventricle.5T lo, I1 Since better results are found in unilateral and particularly in left-sided disease, the importance of complete preoperative evaluation is emphasized.

CONCLUSIONS

Fourteen patients had dominant RVEMF, 3 had LVEMF, and 12 had biventricular EMF disease. Patients with RVEMF had a normal or low puhno- nary artery pressure, high right ventricular systolic to end-diastolic pressure ratio (average 68%), and 11 of 14 had a diastolic gradient from the right ventricle to the pulmonary artery. All had apical distortion of the right ventricle and 13 of 14 had tricuspid regurgitation. Patients with LVEMF had pulmonary hypertension (mean pressure 58 mm Hg) as did also 4 of 12 with BVEMF. With left ventricu- lar involvement there was mitral regurgitation and distortion of the apex of the left ventricle. Three with RVEMF had Glenn shunts and three others had endocardiectomy and tricuspid valve replace- ment. The indications and results of surgical treat- ment have been reviewed.

We thank Dr. George Jacob, Professor of Cardiology, Medical College Hospital, Kottayam, India, for referral and follow-up of some of these patients, and Mrs. Kaye Cherry for typing the manuscript.

1. Davies JNP, Ball JD: The pathology of endomyocardial fibrosis. Br Heart J 17:337, 1955.

2. Shaper AG, Hutt MSR, Coles RM: Necropsy studies of endomyocardial fibrosis and rheumatic heart disease in Uganda. Br Heart J 30:391, 1968.

3. Lepley D, Jr, Aris A, Korus ME, Walker JA, D’Cunha RM: Endomyocardial fibrosis. A surgical approach. Ann Thorac Surg 18:626, 1974.

4. Dubost C, Maurice P, Gerbaux A, Bertrand E, Rulliere R, Vial F, Barrillon A, Prigent C, Carpentier A, Soyer R: The surgical treatment of constrictive fibrous endocarditis. Ann Surg 184:303, 1976.

5. Hess CM, Turina M, Senning A, Goebel NH, Scholer Y, Krayenbuehl HP: Pre and post operative findings in patients with endomyocardial fibrosis. Br Heart J 40~406, 1978.

6. Laing HC, Sharratt GP, Johnson AM, Davies MJ, Monro JL: Endomyocardial fibrosis in a European woman and its suc- cessful surgical treatment. J Thorac Cardiovasc Surg 74:803, 1977.

7. Roberts WC, Liegler DG, Carbone PP: Endocardial disease and eosinophilia. A clinical and pathologic spectrum. Am J Med 46:28, 1969.

8. Spry CJF, Tai PC: Studies on blood eosinophils. Patients with Loffler’s cardiomyopathy. Clin Exp Immunol 24:423, 1976.

9. Olsen EGJ, Spry CJF: The pathogenesis of Loffler’s endo- myocardial disease and its relationship to endomyocardial fibrosis. In Yu PN, Goodwin JF, editors: Progress in cardiol- ogy. no. 8. Philadelphia, 1979, Lea & Febiger, p 281.

10. Dubost C: Surgery for constrictive fibrous endocarditis. Compr Ther 5:28, 1979.

11. Moraes CR, Buffalo E, Victor E, Saravia L, Gomes JMP, Lira V, Lima R, Escobar M, Andrade JC: Endomyocardial fibrosis. Report of 6 patients and review of the surgical literature. Ann Thorac Surg 29:243, 1980.

12. Cockshott WP: Angiocardiography of endomyocardial fibro- sis. Br J Radio1 38:192, 1965.

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14. Vijayaraghavan G, Cherian G, Krishnaswami S, Sukumar IP: Left ventricular endomyocardial fibrosis in India. Br Heart J 39:563, 1977.

15. Grossman W: Cardiac catheterization and angiography. 2nd ed. Philadelphia, 1980, Lea & Febiger, pp 312, 323.

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