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Endomyocardial fibrosis from a surgical standpoint
J
THoRAc CARDIOVASC SURG
80:437-440, 1980
Endomyocardial fibrosis from a surgical standpoint Successful surgical correction was accomplished in a 12-year-old boy with proved endomyocardial fibrosis. Pathological features and the role of surgical therapy in the management of this lesion are discussed. This report presents one of the youngest patients who has undergone endocardiectomy and valve replacement for right-sided endomyocardial fibrosis.
Stanley John, M.S., M.S. (Thor.), F.A.C.C., F.A.C.S., F.A.M.S., Ganesh K. Mani, M.Ch. (Thor.), S. Muralidharan, M.Ch. (Thor.), S. Krishnaswamy, D.M., F.A.C.C., and G. Cherian, D.M., F.A.C.C., Vellore, India
EndOmYOCardial fibrosis is a progressive restrictive endocardial disease of unknown origin more commonly occurring in tropical countries. Most cardiologists have resigned themselves to treating this clinicopathologic entity as being incurable. Prigent and colleagues! in 1971 were the first to accomplish surgical cure for endomyocardial fibrosis by endocardiectomy and prosthetic replacement of the affected valve. We present our experience with a corrective operation of this lesion carried out in a young boy at the Department of Cardiothoracic Surgery of the Christian Medical College Hospital, Vellore, India.
Case report A 12-year-old boy was admitted for evaluation of progressive dyspnea of less than I year's duration with gross congestive failure. He had no history of rheumatic fever. He was poorly nourished and pale. Pronounced cardiomegaly with a heaving apex and prominent outflow tract pulsations were evident. No murmurs were heard. There was gross hepatomegaly, the liver's edge being palpable 8 em below the right costal margin. V waves in the jugular veins rose up to the angle of mandible. A roentgenogram of the chest showed a grossly enlarged cardiac silhouette with a prominent right atrium and pulmonary conus (Fig. I, A). The electrocardiographic findings were atrial fibrillation, an axis of +60 degrees, incomplete right bundle branch block, and left ventricular hypertrophy. Cardiac catheterization studies revealed a mean right atrial From the Departments of Cardiothoracic Surgery and Cardiology, Christian Medical College and Hospital, Vellore, India. Received for publication Jan. 29, 1980. Accepted for publication March 26, 1980. Address for reprints: Dr. Stanley John, Professor and Head of Department of Cardiothoracic Surgery, C.M.C. Hospital, Vellore 632004, India.
pressure of 23 mm Hg with an elevated right ventricular end-diastolic pressure of 15 mm Hg. The catheter in the right atrium was touching the right border of the cardiac silhouette, so that there was no possibility of any pericardial effusion. The systemic index was 2.97 L'rnin/rn". The status of the left ventricle as assessed by pressures showed satisfactory function with an end-diastolic pressure of 10 mm Hg. A right atriogram revealed a grossly dilated right atrium (Fig. 2) and an obliterated right ventricular cavity with vigorous pulsations of the outflow tract. Angiographically, the left ventricular end-systolic size was normal (Fig. 3), with a normal ejection fraction (estimated 0.55) and no filling defects. The diagnosis of endomyocardial fibrosis affecting the right ventricular body and tricuspid valve was thus well documented. He was treated with digitalis and diuretics for a month but, as his congestive failure was not controlled, he was scheduled for an operation. Method. Through a median sternotomy, and with the subject on cardiopulmonary bypass, the large right atrium was opened. A few clots were present and the tricuspid valve was grossly puckered and atrophic. A transverse paracoronary right ventriculotomy revealed a grayish-white, thick, fibrous membrane, varying from 6 to 8 mm in thickness, lining the interior and binding down the papillary muscles but not involving the outflow tract or pulmonary valve. Despite extensive involvement of the ventricle, the atrium was free from fibrosis. However, this fibrous membrane was peeled off the right ventricular myocardium, and the entire papillary muscle complex was excised; only the septal leaflet of the tricuspid valve was left, and this was subsequently reefed. The tricuspid valve was replaced with a No. 27 Bjork-Shiley tilting disc prosthesis. Cardiopulmonary bypass was discontinued, and myocardial electrodes were inserted as a precautionary measure, although the patient was in atrial fibrillation with no evidence of heart block. Postoperatively, congestive failure and effort intolerance regressed remarkably in due course and he was discharged from the hospital receiving digitalis, diuretics, and anticoagulants. His condition has continued to improve since, and he is leading a normal life I year following valve replacement (Fig. 1, B).
0022-5223/801090437+04$00.4010 © 1980 The C. V. Mosby Co.
437
438
John et
The Journal of Thoracic and Cardiovascular Surgery
at.
Fig. 1. A. Plain film shows an enormously enlarged cardiac contour. Note the slight bulge from the dilated outflow tract of the right ventricle. B. Postoperative roentgenogram shows striking regression of cardiomegaly following endocardiectomy and valve replacement. Lanka, Malaysia, Brazil, and India. The high incidence in Kerala in South India was detected by Gopi" in 1967. The cause is not known yet. Malnutrition, virus, microfilaria, and excessive intake of serotonin-rich substances such as plantains have all been incrim-
inated."
Fig. 2. Opacified right atrium showing its vast size and very small right ventricle (between arrows).
Comment Endomyocardial fibrosis is an obliterative cardiomyopathy and is known to occur endemically in Uganda, Nigeria, Sudan, Congo, Kenya, Tanzania, Sri
The basic process of fibrosis may involve either ventricle individually or both ventricles together. In Uganda, left ventricular or biventricular involvement is more common," but it seems likely that isolated right ventricular endomyocardial fibrosis is frequently encountered in India." Characteristically, only the inflow tract and bases of papillary muscles are involved, while the outflow tract remarkably is free from disease. The peripheral limit of the lesion presents as a ridge." The survival after diagnosis is usually about 2 years.' Symptoms are caused by endocardial rigidity, atrioventricular valve dysfunction, and progressive obliteration of the ventricular cavity. Dubost': 8 was one of the first to report the excellent result achieved by endocardiectomy and valve replacement. Lepley and associates" accomplished success with this procedure in 1973. Their subject underwent operation with a diagnosis of cardiac tumor, and endomyocardial fibrosis of the left side of the heart was diagnosed at operation. Through an apical left ventriculotomy, the mass of fibrous endocardium was dis-
Volume 80
Endomyocardial fibrosis
Number 3
43 9
September. 1980
® Fig. 3. Diastolic (A) and systolic (B) frames of left ventriculogram showing normal left ventricular end-systolic size and contractility. sected from the myocardium along with the papillary muscles; then the mitral valve was excised and replaced with a low-profile disc prosthesis. In our patient we had documented the diagnosis of endomyocardial fibrosis of the right ventricle with tricuspid regurgitation. This was confirmed at operation when the right ventricle was found to be small and indented along the inferior margin. The giant-sized right atrium revealed some clots but no fibrous thickening of the endocardium. The tricuspid valve was incompetent and scarred. Excellent clinical improvement occurred in this patient, which was a gratifying feature. He has maintained this progress over the last year and is going to school. We believe that in selected cases operation does have a role in the management of endomyocardial fibrosis, an otherwise incurable malady. We have carried out cavopulmonary shunt (Glenn's operation) for right ventricular endomyocardial fibrosis. This has offered reasonable palliation in some young patients. 10 Tricuspid valve replacement has offered significant hemodynamic benefits in those in whom the pathological process is confined to the right side. Nonetheless, we emphasize the need for prolonged anticoagulant therapy for a tricuspid valve prosthesis. Our experience
heretofore following valve replacement for rheumatic valvular disease, especially in young patients, has shown an extremely low incidence of thromboembolic cornplications.!': 12 The present case report presents one of the youngest patients with this entity in whom successful surgical correction has been accomplished. To our knowledge, this is the first subject reported from our country.
2 3 4 5 6 7
REFERENCES Prigent C, Baculard P, Carpentier A, Passeleco J, Dubost C: Endocardite fibreuse du ventricule gauche chez un filarien: Cure chirurgicale. Arch Mal Coeur 66: 11011116, 1973 Gopi CK: Endomyocardial fibrosis. Bull WHO 38:939, 1968 Ojo GO: The pathogenesis of endomyocardial fibrosis: The question of 5-hydroxytryptamine. Br Heart J 32: 671-674, 1970 Shaper AG, Hut! MSR, Coles RM: Necropsy study of endomyocardial fibrosis and rheumatic heart disease in Uganda 1950-1965. Br Heart J 30:391-401, 1968 Nair DV: Endomyocardial fibrosis in Kerala. Ind Heart J 23:182-190, 1971 Davies JNP: The ridge in endomyocardial fibrosis. Lancet 1:631-634, 1968 D'Arbela PG, Mutazindva T, Patel AK, Somers K: Sur-
440 John et ai.
The Journal of Thoracic and Cardiovascular Surgery
vival after first presentation with endomyocardial fibrosis. Br Heart J 34:403-407, 1972 8 Dubost C, Maurice P, Gerbaux A, Bertrand R, Rulliere F, Vial A, Barrillon C, Prigent CL, Carpentier A, Soyer R: The surgical treatment of constrictive fibrous endocarditis. Ann Surg 184:303-307, 1976 9 Lepley D Jr, Alejandro A, Korms ME, Walker JA, D'Cunha RM: Endomyocardial fibrosis. A surgical approach. Ann Thorac Surg 18:626-631, 1974 10 Vibhakar BB, Vohra JK, Desai MG, Mehta MP, Shah SJ:
Right ventricular endomyocardial fibrosis (a palliative surgical approach). Ind Heart J 24:291-294, 1972 II John S, Munsi SC, Gupta RP, Ramachandran V, Milledge JS, Sukumar IP, Cherian G: Results of mitral valve replacement in young patients with rheumatic heart disease. J THORAC CARDIOVASC SURG 66:255-264, 1973 12 John S, Munsi SC, Sukumar IP, Cherian G: Mitral valve replacement in children and adolescents with rheumat,j~ heart disease. Jpn Heart J 17:570-579, 1976
Information for authors Most of the provisions of the Copyright Act of 1976 became effective on January I, 1978. Therefore, all manuscripts must be accompanied by the following written statement, signed by one author: "The undersigned author transfers all copyright ownership of the manuscript (title of article) to The C. V. Mosby Company in the event the work is published. The undersigned author warrants that the article is original, is not under consideration by another journal, and has not been previously published. I sign for and accept responsibility for releasing this material on behalf of any and all co-authors." Authors will be consulted, when possible, regarding republication of their material.
THoRAc CARDIOVASC SURG
80:437-440, 1980
Endomyocardial fibrosis from a surgical standpoint Successful surgical correction was accomplished in a 12-year-old boy with proved endomyocardial fibrosis. Pathological features and the role of surgical therapy in the management of this lesion are discussed. This report presents one of the youngest patients who has undergone endocardiectomy and valve replacement for right-sided endomyocardial fibrosis.
Stanley John, M.S., M.S. (Thor.), F.A.C.C., F.A.C.S., F.A.M.S., Ganesh K. Mani, M.Ch. (Thor.), S. Muralidharan, M.Ch. (Thor.), S. Krishnaswamy, D.M., F.A.C.C., and G. Cherian, D.M., F.A.C.C., Vellore, India
EndOmYOCardial fibrosis is a progressive restrictive endocardial disease of unknown origin more commonly occurring in tropical countries. Most cardiologists have resigned themselves to treating this clinicopathologic entity as being incurable. Prigent and colleagues! in 1971 were the first to accomplish surgical cure for endomyocardial fibrosis by endocardiectomy and prosthetic replacement of the affected valve. We present our experience with a corrective operation of this lesion carried out in a young boy at the Department of Cardiothoracic Surgery of the Christian Medical College Hospital, Vellore, India.
Case report A 12-year-old boy was admitted for evaluation of progressive dyspnea of less than I year's duration with gross congestive failure. He had no history of rheumatic fever. He was poorly nourished and pale. Pronounced cardiomegaly with a heaving apex and prominent outflow tract pulsations were evident. No murmurs were heard. There was gross hepatomegaly, the liver's edge being palpable 8 em below the right costal margin. V waves in the jugular veins rose up to the angle of mandible. A roentgenogram of the chest showed a grossly enlarged cardiac silhouette with a prominent right atrium and pulmonary conus (Fig. I, A). The electrocardiographic findings were atrial fibrillation, an axis of +60 degrees, incomplete right bundle branch block, and left ventricular hypertrophy. Cardiac catheterization studies revealed a mean right atrial From the Departments of Cardiothoracic Surgery and Cardiology, Christian Medical College and Hospital, Vellore, India. Received for publication Jan. 29, 1980. Accepted for publication March 26, 1980. Address for reprints: Dr. Stanley John, Professor and Head of Department of Cardiothoracic Surgery, C.M.C. Hospital, Vellore 632004, India.
pressure of 23 mm Hg with an elevated right ventricular end-diastolic pressure of 15 mm Hg. The catheter in the right atrium was touching the right border of the cardiac silhouette, so that there was no possibility of any pericardial effusion. The systemic index was 2.97 L'rnin/rn". The status of the left ventricle as assessed by pressures showed satisfactory function with an end-diastolic pressure of 10 mm Hg. A right atriogram revealed a grossly dilated right atrium (Fig. 2) and an obliterated right ventricular cavity with vigorous pulsations of the outflow tract. Angiographically, the left ventricular end-systolic size was normal (Fig. 3), with a normal ejection fraction (estimated 0.55) and no filling defects. The diagnosis of endomyocardial fibrosis affecting the right ventricular body and tricuspid valve was thus well documented. He was treated with digitalis and diuretics for a month but, as his congestive failure was not controlled, he was scheduled for an operation. Method. Through a median sternotomy, and with the subject on cardiopulmonary bypass, the large right atrium was opened. A few clots were present and the tricuspid valve was grossly puckered and atrophic. A transverse paracoronary right ventriculotomy revealed a grayish-white, thick, fibrous membrane, varying from 6 to 8 mm in thickness, lining the interior and binding down the papillary muscles but not involving the outflow tract or pulmonary valve. Despite extensive involvement of the ventricle, the atrium was free from fibrosis. However, this fibrous membrane was peeled off the right ventricular myocardium, and the entire papillary muscle complex was excised; only the septal leaflet of the tricuspid valve was left, and this was subsequently reefed. The tricuspid valve was replaced with a No. 27 Bjork-Shiley tilting disc prosthesis. Cardiopulmonary bypass was discontinued, and myocardial electrodes were inserted as a precautionary measure, although the patient was in atrial fibrillation with no evidence of heart block. Postoperatively, congestive failure and effort intolerance regressed remarkably in due course and he was discharged from the hospital receiving digitalis, diuretics, and anticoagulants. His condition has continued to improve since, and he is leading a normal life I year following valve replacement (Fig. 1, B).
0022-5223/801090437+04$00.4010 © 1980 The C. V. Mosby Co.
437
438
John et
The Journal of Thoracic and Cardiovascular Surgery
at.
Fig. 1. A. Plain film shows an enormously enlarged cardiac contour. Note the slight bulge from the dilated outflow tract of the right ventricle. B. Postoperative roentgenogram shows striking regression of cardiomegaly following endocardiectomy and valve replacement. Lanka, Malaysia, Brazil, and India. The high incidence in Kerala in South India was detected by Gopi" in 1967. The cause is not known yet. Malnutrition, virus, microfilaria, and excessive intake of serotonin-rich substances such as plantains have all been incrim-
inated."
Fig. 2. Opacified right atrium showing its vast size and very small right ventricle (between arrows).
Comment Endomyocardial fibrosis is an obliterative cardiomyopathy and is known to occur endemically in Uganda, Nigeria, Sudan, Congo, Kenya, Tanzania, Sri
The basic process of fibrosis may involve either ventricle individually or both ventricles together. In Uganda, left ventricular or biventricular involvement is more common," but it seems likely that isolated right ventricular endomyocardial fibrosis is frequently encountered in India." Characteristically, only the inflow tract and bases of papillary muscles are involved, while the outflow tract remarkably is free from disease. The peripheral limit of the lesion presents as a ridge." The survival after diagnosis is usually about 2 years.' Symptoms are caused by endocardial rigidity, atrioventricular valve dysfunction, and progressive obliteration of the ventricular cavity. Dubost': 8 was one of the first to report the excellent result achieved by endocardiectomy and valve replacement. Lepley and associates" accomplished success with this procedure in 1973. Their subject underwent operation with a diagnosis of cardiac tumor, and endomyocardial fibrosis of the left side of the heart was diagnosed at operation. Through an apical left ventriculotomy, the mass of fibrous endocardium was dis-
Volume 80
Endomyocardial fibrosis
Number 3
43 9
September. 1980
® Fig. 3. Diastolic (A) and systolic (B) frames of left ventriculogram showing normal left ventricular end-systolic size and contractility. sected from the myocardium along with the papillary muscles; then the mitral valve was excised and replaced with a low-profile disc prosthesis. In our patient we had documented the diagnosis of endomyocardial fibrosis of the right ventricle with tricuspid regurgitation. This was confirmed at operation when the right ventricle was found to be small and indented along the inferior margin. The giant-sized right atrium revealed some clots but no fibrous thickening of the endocardium. The tricuspid valve was incompetent and scarred. Excellent clinical improvement occurred in this patient, which was a gratifying feature. He has maintained this progress over the last year and is going to school. We believe that in selected cases operation does have a role in the management of endomyocardial fibrosis, an otherwise incurable malady. We have carried out cavopulmonary shunt (Glenn's operation) for right ventricular endomyocardial fibrosis. This has offered reasonable palliation in some young patients. 10 Tricuspid valve replacement has offered significant hemodynamic benefits in those in whom the pathological process is confined to the right side. Nonetheless, we emphasize the need for prolonged anticoagulant therapy for a tricuspid valve prosthesis. Our experience
heretofore following valve replacement for rheumatic valvular disease, especially in young patients, has shown an extremely low incidence of thromboembolic cornplications.!': 12 The present case report presents one of the youngest patients with this entity in whom successful surgical correction has been accomplished. To our knowledge, this is the first subject reported from our country.
2 3 4 5 6 7
REFERENCES Prigent C, Baculard P, Carpentier A, Passeleco J, Dubost C: Endocardite fibreuse du ventricule gauche chez un filarien: Cure chirurgicale. Arch Mal Coeur 66: 11011116, 1973 Gopi CK: Endomyocardial fibrosis. Bull WHO 38:939, 1968 Ojo GO: The pathogenesis of endomyocardial fibrosis: The question of 5-hydroxytryptamine. Br Heart J 32: 671-674, 1970 Shaper AG, Hut! MSR, Coles RM: Necropsy study of endomyocardial fibrosis and rheumatic heart disease in Uganda 1950-1965. Br Heart J 30:391-401, 1968 Nair DV: Endomyocardial fibrosis in Kerala. Ind Heart J 23:182-190, 1971 Davies JNP: The ridge in endomyocardial fibrosis. Lancet 1:631-634, 1968 D'Arbela PG, Mutazindva T, Patel AK, Somers K: Sur-
440 John et ai.
The Journal of Thoracic and Cardiovascular Surgery
vival after first presentation with endomyocardial fibrosis. Br Heart J 34:403-407, 1972 8 Dubost C, Maurice P, Gerbaux A, Bertrand R, Rulliere F, Vial A, Barrillon C, Prigent CL, Carpentier A, Soyer R: The surgical treatment of constrictive fibrous endocarditis. Ann Surg 184:303-307, 1976 9 Lepley D Jr, Alejandro A, Korms ME, Walker JA, D'Cunha RM: Endomyocardial fibrosis. A surgical approach. Ann Thorac Surg 18:626-631, 1974 10 Vibhakar BB, Vohra JK, Desai MG, Mehta MP, Shah SJ:
Right ventricular endomyocardial fibrosis (a palliative surgical approach). Ind Heart J 24:291-294, 1972 II John S, Munsi SC, Gupta RP, Ramachandran V, Milledge JS, Sukumar IP, Cherian G: Results of mitral valve replacement in young patients with rheumatic heart disease. J THORAC CARDIOVASC SURG 66:255-264, 1973 12 John S, Munsi SC, Sukumar IP, Cherian G: Mitral valve replacement in children and adolescents with rheumat,j~ heart disease. Jpn Heart J 17:570-579, 1976
Information for authors Most of the provisions of the Copyright Act of 1976 became effective on January I, 1978. Therefore, all manuscripts must be accompanied by the following written statement, signed by one author: "The undersigned author transfers all copyright ownership of the manuscript (title of article) to The C. V. Mosby Company in the event the work is published. The undersigned author warrants that the article is original, is not under consideration by another journal, and has not been previously published. I sign for and accept responsibility for releasing this material on behalf of any and all co-authors." Authors will be consulted, when possible, regarding republication of their material.