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Mitral valve replacement for mitral regurgitation due to Libman-Sacks endocarditis
Mitral valve replacement for mitral regurgitation due to Libman-Sacks endocarditis Report 0/ a case A case of mitral valve replacement jor Libman-Sacks endocarditis is described. The incidence of this lesion and the difficulty in diagnosis is discussed. Due to the frequency of this lesion, the ineffectiveness ot steroid therapy in preventing its occurrence, and the increasing longevity of patients with systemic lupus erythematosus (SLE), more patients with hemodynamically significant valvular lesions may require valve replacement. The advantages of the porcine xenograft are discussed. This case demonstrates that patients with SLE can tolerate an open-heart operation and should be considered for operative intervention when their cardiac disease warrants it.
P. David Myerowitz, M.D., Lawrence L. Michaelis, M.D., and Charles L. McIntosh, M.D., Bethesda, Md.
The first clinical description of cardiovascular involvement in lupus erythematosus was by Kaposi,' who in 1872 reported cardiac irregularity and dyspnea in two patients. Pernet," in 1908, described a small vegetation on the mitral valve of a patient with lupus erythematosus. The classic report by Libman and Sacks- in 1924 presented the clinical course and pathological findings in four patients with systemic lupus erythematosus (SLE) and nonbacterial verrucous endocarditis. They also clearly differentiated this entity from rheumatic and bacterial endocarditis. Libman-Sacks endocarditis is a recognized pathological entity and is found at autopsy in 13' to 63 per cent" of patients with SLE. Characteristically, the lesions are small, 1 to 3 mm. vegetations occurring on the valve From the Clinic of Surgery, National Heart and Lung Institute, National Institutes of Health, Bethesda, Md. 20014. Received for publication Feb. 25, 1974.
leaflets, chordae tendineae, and mural endocardium. They occur most frequently on the mitral valve but can be found on any valve. The verrucae consist of areas of proliferating and degenerating valve substance mixed with fibrin and platelet thrombi. Microscopically, the lesion is the hematoxylin body consisting of blue-staining debris from the necrosis of cell nuclel.v- 11 It has been thought that the vegetations of the mitral valve seen in Libman-Sacks endocarditis do not produce significant alterations in hemodynamic function.':" Shulman' reported 3 cases of SLE and aortic regurgitation. One of these patients underwent aortic valve replacement; fenestrations and mucoid degeneration of the aortic valve were present, but no vegetations were seen. There were no vegetations on the mitral valve, but thickened leaflets and dilated annulus necessitated mitral annuloplasty to correct moderate mitral regurgitation. This report describes a patient with large 869
The Journol of
870
Myerowitz, Michaelis, Mcintosh
Thoracic and Cardiovascular Surgery
Fig. 1. A transseptal left atrial angiogram shows a multilobulated filling defect (arrows) at the basilar aspect of the inferior wall of the left ventricle.
Libman-Sacks valve, chordae cle; the result tion requiring
vegetations of the mitral tendineae, and papillary muswas severe mitral regurgitamitral valve replacement.
Case report The diagnosis of SLE was made in a 19-year-old black woman in 1970; she had arthritis, pericarditis, pleuritis, and the nephrotic syndrome, for which she was treated with Imuran and prednisone . She had multiple admissions from 1970 to 1971 for arthritis, nephritis, chemical pancreatitis, hepatitis, and impetigo. During an admission in January, 1972, a Grade 2/6 systolic ejection murmur was first heard. There were no other cardiac symptoms or physical findings . The chest x-ray study showed a slight increase in the cardiothoracic ratio, and the electrocardiogram revealed nonspecific T-wave changes. In November, 1972, a mid-systolic click, a Grade 1/6 late decrescendo systolic murmur, and a high pitched mid-diastolic sound were heard. Renal status remained stable, and hypertension was well controlled on Aldomet. In August of 1973 she was admitted for the acute onset of cough, chills, shortness of breath, hemoptysis, and paroxysmal nocturnal dyspnea. Physical examination revealed a palpable gallop and systolic thrill at the apex; a Grade 3/6 holosystolic decrescendo murmur audible at the apex radiated to the axilla . The mid-systolic click and diastolic sound previously heard were not present. The electrocardiogram showed voltage criteria for left ventricular hypertrophy, and the chest x-ray film revealed diffuse interstitial alveolar infiltrates
with slight prominence of the left atrial appendage. An open lung biop sy showed interstitial pneumonitis and multiple small vessel thrombi. Right heart catheterization revealed a pulmonary artery wedge mean pressure of 20 to 25 mm. Hg, a pulmonary artery pressure of 45/15 mm. Hg, a right ventricular pressure of 45/5-10 mm. Hg, and a right atrial mean pressure of 5 to 7 mm. Hg, Both pulmonary edema and pulmonary infiltrates gradually resolved with mild diuresis and digitalis therapy, and she was discharged from the hospital in 2 weeks on a regimen of digitalis and diuretics. She was readmitted I week later after the acute recurrence of shortness of breath, cough, and wheezing. On admission she was afebrile, with tachycardia and premature atrial contractions. Chest examination revealed bilateral rales and dullness on the left. The murmur of mitral regurgitation had increased in intensity to Grade 4/6. No other murmurs were heard. The electrocardiogram showed left ventricular hypertrophy and left atrial enlargement. Chest x-ray study showed marked pulmonary venous engorgement with increased left atrial prominence. A standard echo cardiogram revealed multiple reflections beneath the mitral valve, and a two-dimensional echocardiogram revealed a mass in this location. Following diuresis her condition improved, and on Oct. 10, 1973, she underwent cardiac catheterization. The following results were obtained: right atrial mean pressure 5 mm . Hg ; right ventricular pressure 50/9 mm . Hg ; pulmonary artery pressure 50/30 mm. Hg; and pulmonary artery wedge mean pressure 28 mm. Hg. After an angiocardiogram the left atrial mean pressure was 50
Volume 67 Number 6
Libman-Sacks endocarditis
87 1
June, 1974
Fig. 2. Serial chest x-ray films: A, Preoperative film shows bilateral pulmonary infiltrates. B, Film taken immediately after operation shows marked resolution. C, Film taken 2 months postoperatively shows complete resolution of infiltrates and decreased cardiomegaly. mm, Hg, with v waves to 80 mm. Hg. The left ventricular pressure was 100/22 mm. Hg. There was a mean gradient across the mitral valve of 14 mm. Hg, but the pressure equilibrated at enddiastole. The cardiac index was 1.8 L. per minute per square meter. A transseptal left atrial angiogram revealed a multilobulated irregular mass at the basilar aspect of the inferior wall of the left ventricle (Fig. 1). The mass extended between the posterior mitral valve leaflet and the left ventricular wall inferiorly as far as the posterior papillary muscle. Superiorly, it extended to the atrioventricular junction. The chordae tendineae and anterior mitral valve leaflet were thickened. There was little motion of the anterior and no
motion of the posterior mitral valve leaflet. The patient experienced chest pain during the catheterization, and electrocardiographic changes consistent with myocardial infarction were noted. It was postulated that a piece of the left ventricular mass may have been embolized to a coronary artery. Subsequent electrocardiograms and serum enzyme determinations were consistent with myocardial infarction. Operation was felt necessary to correct the mitral regurgitation, but, since her condition seemed stable, a 6 week, delay was thought advisable following a possible myocardial infarction. Over the next 4 days, however, her condition deteriorated rapidly with the development of bilateral pulmonary infiltrates (Fig. 2), spiking
872
Myerowitz, Michaelis , McIntosh
The Journal of Thoracic and Card iovascular Surgery
Fig. 3. An artist's conception of the vegetations surrounding the papillary muscles and chordae of the mitral valve and projecting through the orifice (upper drawings). The area of resection and placement of sutures of the porcine xenograft (lower drawing).
temperatures, paroxysmal supraventricular tachycardia, and oliguria progressing to 24 hours of anuria. Progressive cardiac decompensation required inotropic support, and respiratory insufficiency necessitated intubation and respir ator support. Operative procedure. Operation was carried out Oct. 15, 1973, through a median sternotomy. The right pleural space was opened and the right lung appeared edematous with diffuse petechial hemorrhages. A lung biopsy was obtained . Care was taken not to manipulate the heart for fear of dislodging the calcified mass in the left ventricle. The chambers and great vessels appeared grossly normal, with no evidence of dyskinesia or recent myocardial infarction. The left atrium and left ventricle were not grossly enlarged. Cardiopulmonary bypass with moderate hypothermia was instituted, the aorta was cross-clamped, and the
mitral valve was visualized. The middle portion of the anterior leaflet of the mitral valve was obviously flail due to ruptured chordae tendineae at the level of the anterior papillary muscle. The anterior leaflet and the small residual posterior leaflet were somewh at thickened. A mass of calcium , ar ising from the base of the anterior papillary muscle , could be seen projecting through the orifice of the mitral valve (Fig. 3). Further inspection of the calcified mass revealed that it extended to the posterior atrioventricular groove and entrapped the chordae tendineae of the posterior leaflet, rendering this leaflet incompetent. The mass had burrowed into the mural endocardium. The mitral valve and papillary muscles were excised, and the friable calcified mass was remo ved with a pituitary rongeur and bone curet, except in the region of the posterior atrioventricular groove. The left atrium and ventricle were
Volume 67 Number 6 June, 1974
then lavaged with large quannties of saline to remove all debris. Frozen section of the mass revealed no evidence of malignancy. Because of the normal size of the left ventricle, a 29 mm. Reis-Hancock porcine xenograft was utilized for mitral valve replacement. The patient was weaned from cardiopulmonary bypass satisfactorily, requiring minimal inotropic support. Permanent sections of the mass revealed typical fibrinoid degeneration mixed with fibrin and platelet thrombi. No evidence of hematoxylin bodies, bacteria, or leukocyte infiltration was seen. The anterior papillary muscle showed evidence of recent (3 to 7 days) infarction. The ruptured chordae tendineae of the anterior leaflet were clubbed, indicating some healing had occurred. Microscopic examination of the lung revealed fibrosis. Postoperative course. The patient's immediate postoperative course was benign. She required minimal inotropic and ventilatory support for 48 hours. Her arterial blood gases improved immediately with marked radiographic clearing of bilateral pulmonary infiltrates within 36 hours (Fig. 2). After the patient had been essentially anuric for 24 hours prior to operation, diuresis began while she was still in the operating room. Her only significant postoperative problems were a transient thrombocytopenia from the third to the ninth postoperative day and transient decrease in renal function. The blood urea nitrogen rose to 132 mg. per cent with a creatinine of 5.8 mg. per cent 1 week postoperatively, but 2 weeks postoperatively the blood urea nitrogen was 46 mg. per cent and the creatinine 2.0 mg. per cent. She was given digitalis on the second postoperative day and was maintained on a mild diuretic regimen with no evidence of heart failure or pulmonary edema. She was hypertensive in the immediate postoperative period, but the blood pressure was easily controlled with Aldomet. She was given large doses of steroid initially, but these were quickly tapered to a maintenance dose of prednisone. The patient was discharged the fourth postoperative week. On a follow-up visit 2 months postoperatively, the blood urea nitrogen was 25 mg. per cent and creatinine 2.0 mg. per cent. The chest x-ray film (Fig. 2) showed decreased cardiomegaly and pulmonary vascularity, and the electrocardiogram showed no evidence of left atrial enlargement but continued left ventricular hypertrophy. She was in functional Class I (New York Heart Association classification) .
Discussion
The prognosis of patients with SLE is improving. Merrell" reported a mortality rate of approximately 50 per cent 4 years
Libman-Sacks endocarditis
873
after diagnosis in 1955. Leonhardt" in 1966 reported a 51 per cent survival 10 years after diagnosis. A significant percentage of patients with SLE demonstrate Libman-Sacks vegetations at autopsy. The effectiveness of steroid therapy in preventing Libman-Sacks endocarditis is doubtful, according to Heijtmancik.? Of 16 patients with SLE who were examined at autopsy, 10 had endocardial vegetations and 8 had valvular vegetations. Five of 10 patients with Libman-Sacks vegetations and only 1 of 6 patients without vegetations had received steroid therapy. The possibility arises, therefore, that with greater longevity and no reduction in incidence of vegetations, a greater number of patients with SLE will survive long enough to develop hemodynamically significant valvular lesions. The diagnosis of Libman-Sacks endocarditis is difficult clinically due to the complexity of the disease. A murmur of mitral regurgitation does not correlate with the presence of vegetations at necropsy." G Indeed, in the original description of the lesions by Libman and Sacks, 2 of 4 patients had no murmur of mitral regurgitation. In Shearn's" series, the murmur of patients with proved Libman-Sacks vegetations was in no way different from the murmur in patients without lesions. It was postulated that this other murmur might be due to various other factors including anemia and tachycardia. Other complicating factors in the diagnosis are the presence of pericardial disease, primary myocardial disease, hypertension, secondary bacterial endocarditis, and coronary arteritis. Echocardiography may prove useful in screening SLE patients for the presence of significant endocardial or valvular lesions. Cardiac catheterization and operative intervention should be considered in this high-risk population of patients when indicated. Our case also demonstrates that vegetations can be quite large, Although usually described as 1 to 3 mm., the vegetations in this patient were 2.5 em. in diameter. Certainly the hemodynamic significance of the
The Journal of
874
Myerowitz, Michaelis, Mcintosh
vegetations is well documented in the catheterization data. The earlier Grade 2/6 holosystolic murmur probably represented moderate mitral regurgitation caused by the entrapped posterior leaflet. The acute onset of symptoms and signs of severe mitral insufficiency probably correlates with the rupturq of the anterior papillary muscle. A porcine xenograft was chosen in this patient for two reasons: ( 1) It offered hemodynamic advantages in a normal-sized left ventricular cavity and (2) eliminated the need for anticoagulation in a patient who would be on chronic steroid therapy. REFERENCES Kaposi, M.: Neue Beitriige zur kenntnis des lupus erythematosus, Arch. DermatoI. Syph. 4: 36, 1872. 2 Pernet, G.: Le lupus erythemateux aigu dernblee, Thesis, Paris, 1908, No. 20. 3 Libman, E., and Sacks, B.: A Hitherto Undescribed Form of Valvular and Mural Endocarditis, Arch. Intern. Med. 33: 701, 1924. 4 Kong, T. Q., Kellum, R. E., and Haserick,
Thoracic and Cardiovascular Surgery
5
6 7 8
9 10 11
J. R.: Clinical Diagnosis of Cardiac Involvement in Systemic Lupus Erythematosus: A Correlation of Clinical and Autopsy Findings in Thirty Patients, Circulation 26: 7, 1962. Heijtmancik, M. R., Wright, J. C., Quint, R., and Jennings, F. L.: The Cardiovascular Manifestations of Systemic Lupus Erythematosus, Am. Heart J. 68: 119, 1964. Shearn, M. A.: The Heart in Systemic Lupus Erythematosus, Am. Heart J. 58: 452, 1959. Shulman, H. J., and Christian, C. L.: Aortic Insufficiency in Systemic Lupus Erythematosus, Arthritis Rheum. 12: 138, 1969. Merrell, M., and Shulman, L. E.: Determination of Prognosis in Chronic Disease Illustrated by Systemic Lupus Erythematosus, J. Chron. Dis. 1: 12, 1955. Leonhardt, T.: Long-Term Prognosis of Systemic Lupus Erythematosus, Acta Med. Scand. 179: 440, 1966 (SuppI. 445). Klemperer, P., Pollack, A. D., and Baehr, G.: Pathology of Disseminated Lupus Erythematosus, Arch. PathoI. 32: 569, 1941. Shulman, E. E., and Harvey, A. M.: Systemic Lupus Erythematosus, in Hollander, J. L., and McCarty, D. J., editors: Arthritis and Allied Conditions, chap. 51, Philadelphia, 1972, Lea & Febiger, Publishers.
P. David Myerowitz, M.D., Lawrence L. Michaelis, M.D., and Charles L. McIntosh, M.D., Bethesda, Md.
The first clinical description of cardiovascular involvement in lupus erythematosus was by Kaposi,' who in 1872 reported cardiac irregularity and dyspnea in two patients. Pernet," in 1908, described a small vegetation on the mitral valve of a patient with lupus erythematosus. The classic report by Libman and Sacks- in 1924 presented the clinical course and pathological findings in four patients with systemic lupus erythematosus (SLE) and nonbacterial verrucous endocarditis. They also clearly differentiated this entity from rheumatic and bacterial endocarditis. Libman-Sacks endocarditis is a recognized pathological entity and is found at autopsy in 13' to 63 per cent" of patients with SLE. Characteristically, the lesions are small, 1 to 3 mm. vegetations occurring on the valve From the Clinic of Surgery, National Heart and Lung Institute, National Institutes of Health, Bethesda, Md. 20014. Received for publication Feb. 25, 1974.
leaflets, chordae tendineae, and mural endocardium. They occur most frequently on the mitral valve but can be found on any valve. The verrucae consist of areas of proliferating and degenerating valve substance mixed with fibrin and platelet thrombi. Microscopically, the lesion is the hematoxylin body consisting of blue-staining debris from the necrosis of cell nuclel.v- 11 It has been thought that the vegetations of the mitral valve seen in Libman-Sacks endocarditis do not produce significant alterations in hemodynamic function.':" Shulman' reported 3 cases of SLE and aortic regurgitation. One of these patients underwent aortic valve replacement; fenestrations and mucoid degeneration of the aortic valve were present, but no vegetations were seen. There were no vegetations on the mitral valve, but thickened leaflets and dilated annulus necessitated mitral annuloplasty to correct moderate mitral regurgitation. This report describes a patient with large 869
The Journol of
870
Myerowitz, Michaelis, Mcintosh
Thoracic and Cardiovascular Surgery
Fig. 1. A transseptal left atrial angiogram shows a multilobulated filling defect (arrows) at the basilar aspect of the inferior wall of the left ventricle.
Libman-Sacks valve, chordae cle; the result tion requiring
vegetations of the mitral tendineae, and papillary muswas severe mitral regurgitamitral valve replacement.
Case report The diagnosis of SLE was made in a 19-year-old black woman in 1970; she had arthritis, pericarditis, pleuritis, and the nephrotic syndrome, for which she was treated with Imuran and prednisone . She had multiple admissions from 1970 to 1971 for arthritis, nephritis, chemical pancreatitis, hepatitis, and impetigo. During an admission in January, 1972, a Grade 2/6 systolic ejection murmur was first heard. There were no other cardiac symptoms or physical findings . The chest x-ray study showed a slight increase in the cardiothoracic ratio, and the electrocardiogram revealed nonspecific T-wave changes. In November, 1972, a mid-systolic click, a Grade 1/6 late decrescendo systolic murmur, and a high pitched mid-diastolic sound were heard. Renal status remained stable, and hypertension was well controlled on Aldomet. In August of 1973 she was admitted for the acute onset of cough, chills, shortness of breath, hemoptysis, and paroxysmal nocturnal dyspnea. Physical examination revealed a palpable gallop and systolic thrill at the apex; a Grade 3/6 holosystolic decrescendo murmur audible at the apex radiated to the axilla . The mid-systolic click and diastolic sound previously heard were not present. The electrocardiogram showed voltage criteria for left ventricular hypertrophy, and the chest x-ray film revealed diffuse interstitial alveolar infiltrates
with slight prominence of the left atrial appendage. An open lung biop sy showed interstitial pneumonitis and multiple small vessel thrombi. Right heart catheterization revealed a pulmonary artery wedge mean pressure of 20 to 25 mm. Hg, a pulmonary artery pressure of 45/15 mm. Hg, a right ventricular pressure of 45/5-10 mm. Hg, and a right atrial mean pressure of 5 to 7 mm. Hg, Both pulmonary edema and pulmonary infiltrates gradually resolved with mild diuresis and digitalis therapy, and she was discharged from the hospital in 2 weeks on a regimen of digitalis and diuretics. She was readmitted I week later after the acute recurrence of shortness of breath, cough, and wheezing. On admission she was afebrile, with tachycardia and premature atrial contractions. Chest examination revealed bilateral rales and dullness on the left. The murmur of mitral regurgitation had increased in intensity to Grade 4/6. No other murmurs were heard. The electrocardiogram showed left ventricular hypertrophy and left atrial enlargement. Chest x-ray study showed marked pulmonary venous engorgement with increased left atrial prominence. A standard echo cardiogram revealed multiple reflections beneath the mitral valve, and a two-dimensional echocardiogram revealed a mass in this location. Following diuresis her condition improved, and on Oct. 10, 1973, she underwent cardiac catheterization. The following results were obtained: right atrial mean pressure 5 mm . Hg ; right ventricular pressure 50/9 mm . Hg ; pulmonary artery pressure 50/30 mm. Hg; and pulmonary artery wedge mean pressure 28 mm. Hg. After an angiocardiogram the left atrial mean pressure was 50
Volume 67 Number 6
Libman-Sacks endocarditis
87 1
June, 1974
Fig. 2. Serial chest x-ray films: A, Preoperative film shows bilateral pulmonary infiltrates. B, Film taken immediately after operation shows marked resolution. C, Film taken 2 months postoperatively shows complete resolution of infiltrates and decreased cardiomegaly. mm, Hg, with v waves to 80 mm. Hg. The left ventricular pressure was 100/22 mm. Hg. There was a mean gradient across the mitral valve of 14 mm. Hg, but the pressure equilibrated at enddiastole. The cardiac index was 1.8 L. per minute per square meter. A transseptal left atrial angiogram revealed a multilobulated irregular mass at the basilar aspect of the inferior wall of the left ventricle (Fig. 1). The mass extended between the posterior mitral valve leaflet and the left ventricular wall inferiorly as far as the posterior papillary muscle. Superiorly, it extended to the atrioventricular junction. The chordae tendineae and anterior mitral valve leaflet were thickened. There was little motion of the anterior and no
motion of the posterior mitral valve leaflet. The patient experienced chest pain during the catheterization, and electrocardiographic changes consistent with myocardial infarction were noted. It was postulated that a piece of the left ventricular mass may have been embolized to a coronary artery. Subsequent electrocardiograms and serum enzyme determinations were consistent with myocardial infarction. Operation was felt necessary to correct the mitral regurgitation, but, since her condition seemed stable, a 6 week, delay was thought advisable following a possible myocardial infarction. Over the next 4 days, however, her condition deteriorated rapidly with the development of bilateral pulmonary infiltrates (Fig. 2), spiking
872
Myerowitz, Michaelis , McIntosh
The Journal of Thoracic and Card iovascular Surgery
Fig. 3. An artist's conception of the vegetations surrounding the papillary muscles and chordae of the mitral valve and projecting through the orifice (upper drawings). The area of resection and placement of sutures of the porcine xenograft (lower drawing).
temperatures, paroxysmal supraventricular tachycardia, and oliguria progressing to 24 hours of anuria. Progressive cardiac decompensation required inotropic support, and respiratory insufficiency necessitated intubation and respir ator support. Operative procedure. Operation was carried out Oct. 15, 1973, through a median sternotomy. The right pleural space was opened and the right lung appeared edematous with diffuse petechial hemorrhages. A lung biopsy was obtained . Care was taken not to manipulate the heart for fear of dislodging the calcified mass in the left ventricle. The chambers and great vessels appeared grossly normal, with no evidence of dyskinesia or recent myocardial infarction. The left atrium and left ventricle were not grossly enlarged. Cardiopulmonary bypass with moderate hypothermia was instituted, the aorta was cross-clamped, and the
mitral valve was visualized. The middle portion of the anterior leaflet of the mitral valve was obviously flail due to ruptured chordae tendineae at the level of the anterior papillary muscle. The anterior leaflet and the small residual posterior leaflet were somewh at thickened. A mass of calcium , ar ising from the base of the anterior papillary muscle , could be seen projecting through the orifice of the mitral valve (Fig. 3). Further inspection of the calcified mass revealed that it extended to the posterior atrioventricular groove and entrapped the chordae tendineae of the posterior leaflet, rendering this leaflet incompetent. The mass had burrowed into the mural endocardium. The mitral valve and papillary muscles were excised, and the friable calcified mass was remo ved with a pituitary rongeur and bone curet, except in the region of the posterior atrioventricular groove. The left atrium and ventricle were
Volume 67 Number 6 June, 1974
then lavaged with large quannties of saline to remove all debris. Frozen section of the mass revealed no evidence of malignancy. Because of the normal size of the left ventricle, a 29 mm. Reis-Hancock porcine xenograft was utilized for mitral valve replacement. The patient was weaned from cardiopulmonary bypass satisfactorily, requiring minimal inotropic support. Permanent sections of the mass revealed typical fibrinoid degeneration mixed with fibrin and platelet thrombi. No evidence of hematoxylin bodies, bacteria, or leukocyte infiltration was seen. The anterior papillary muscle showed evidence of recent (3 to 7 days) infarction. The ruptured chordae tendineae of the anterior leaflet were clubbed, indicating some healing had occurred. Microscopic examination of the lung revealed fibrosis. Postoperative course. The patient's immediate postoperative course was benign. She required minimal inotropic and ventilatory support for 48 hours. Her arterial blood gases improved immediately with marked radiographic clearing of bilateral pulmonary infiltrates within 36 hours (Fig. 2). After the patient had been essentially anuric for 24 hours prior to operation, diuresis began while she was still in the operating room. Her only significant postoperative problems were a transient thrombocytopenia from the third to the ninth postoperative day and transient decrease in renal function. The blood urea nitrogen rose to 132 mg. per cent with a creatinine of 5.8 mg. per cent 1 week postoperatively, but 2 weeks postoperatively the blood urea nitrogen was 46 mg. per cent and the creatinine 2.0 mg. per cent. She was given digitalis on the second postoperative day and was maintained on a mild diuretic regimen with no evidence of heart failure or pulmonary edema. She was hypertensive in the immediate postoperative period, but the blood pressure was easily controlled with Aldomet. She was given large doses of steroid initially, but these were quickly tapered to a maintenance dose of prednisone. The patient was discharged the fourth postoperative week. On a follow-up visit 2 months postoperatively, the blood urea nitrogen was 25 mg. per cent and creatinine 2.0 mg. per cent. The chest x-ray film (Fig. 2) showed decreased cardiomegaly and pulmonary vascularity, and the electrocardiogram showed no evidence of left atrial enlargement but continued left ventricular hypertrophy. She was in functional Class I (New York Heart Association classification) .
Discussion
The prognosis of patients with SLE is improving. Merrell" reported a mortality rate of approximately 50 per cent 4 years
Libman-Sacks endocarditis
873
after diagnosis in 1955. Leonhardt" in 1966 reported a 51 per cent survival 10 years after diagnosis. A significant percentage of patients with SLE demonstrate Libman-Sacks vegetations at autopsy. The effectiveness of steroid therapy in preventing Libman-Sacks endocarditis is doubtful, according to Heijtmancik.? Of 16 patients with SLE who were examined at autopsy, 10 had endocardial vegetations and 8 had valvular vegetations. Five of 10 patients with Libman-Sacks vegetations and only 1 of 6 patients without vegetations had received steroid therapy. The possibility arises, therefore, that with greater longevity and no reduction in incidence of vegetations, a greater number of patients with SLE will survive long enough to develop hemodynamically significant valvular lesions. The diagnosis of Libman-Sacks endocarditis is difficult clinically due to the complexity of the disease. A murmur of mitral regurgitation does not correlate with the presence of vegetations at necropsy." G Indeed, in the original description of the lesions by Libman and Sacks, 2 of 4 patients had no murmur of mitral regurgitation. In Shearn's" series, the murmur of patients with proved Libman-Sacks vegetations was in no way different from the murmur in patients without lesions. It was postulated that this other murmur might be due to various other factors including anemia and tachycardia. Other complicating factors in the diagnosis are the presence of pericardial disease, primary myocardial disease, hypertension, secondary bacterial endocarditis, and coronary arteritis. Echocardiography may prove useful in screening SLE patients for the presence of significant endocardial or valvular lesions. Cardiac catheterization and operative intervention should be considered in this high-risk population of patients when indicated. Our case also demonstrates that vegetations can be quite large, Although usually described as 1 to 3 mm., the vegetations in this patient were 2.5 em. in diameter. Certainly the hemodynamic significance of the
The Journal of
874
Myerowitz, Michaelis, Mcintosh
vegetations is well documented in the catheterization data. The earlier Grade 2/6 holosystolic murmur probably represented moderate mitral regurgitation caused by the entrapped posterior leaflet. The acute onset of symptoms and signs of severe mitral insufficiency probably correlates with the rupturq of the anterior papillary muscle. A porcine xenograft was chosen in this patient for two reasons: ( 1) It offered hemodynamic advantages in a normal-sized left ventricular cavity and (2) eliminated the need for anticoagulation in a patient who would be on chronic steroid therapy. REFERENCES Kaposi, M.: Neue Beitriige zur kenntnis des lupus erythematosus, Arch. DermatoI. Syph. 4: 36, 1872. 2 Pernet, G.: Le lupus erythemateux aigu dernblee, Thesis, Paris, 1908, No. 20. 3 Libman, E., and Sacks, B.: A Hitherto Undescribed Form of Valvular and Mural Endocarditis, Arch. Intern. Med. 33: 701, 1924. 4 Kong, T. Q., Kellum, R. E., and Haserick,
Thoracic and Cardiovascular Surgery
5
6 7 8
9 10 11
J. R.: Clinical Diagnosis of Cardiac Involvement in Systemic Lupus Erythematosus: A Correlation of Clinical and Autopsy Findings in Thirty Patients, Circulation 26: 7, 1962. Heijtmancik, M. R., Wright, J. C., Quint, R., and Jennings, F. L.: The Cardiovascular Manifestations of Systemic Lupus Erythematosus, Am. Heart J. 68: 119, 1964. Shearn, M. A.: The Heart in Systemic Lupus Erythematosus, Am. Heart J. 58: 452, 1959. Shulman, H. J., and Christian, C. L.: Aortic Insufficiency in Systemic Lupus Erythematosus, Arthritis Rheum. 12: 138, 1969. Merrell, M., and Shulman, L. E.: Determination of Prognosis in Chronic Disease Illustrated by Systemic Lupus Erythematosus, J. Chron. Dis. 1: 12, 1955. Leonhardt, T.: Long-Term Prognosis of Systemic Lupus Erythematosus, Acta Med. Scand. 179: 440, 1966 (SuppI. 445). Klemperer, P., Pollack, A. D., and Baehr, G.: Pathology of Disseminated Lupus Erythematosus, Arch. PathoI. 32: 569, 1941. Shulman, E. E., and Harvey, A. M.: Systemic Lupus Erythematosus, in Hollander, J. L., and McCarty, D. J., editors: Arthritis and Allied Conditions, chap. 51, Philadelphia, 1972, Lea & Febiger, Publishers.