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Triple-valve endocarditis with unusual echocardiographic findings
March,
598
Brief
Communications
Table
II. Noninvasive data
CUSP
Scimitar sign on chest radiograph
ECG
1
RBBB
American
2 3 4 5
Normal Incomplete Normal Normal Incomplete
Only on tomography Present Present Present Present
6 7 8
RVH RVH
Present Absent Absent
9
Normal
Present
RBBB
RBBB -
= right
bundle
branch
block;
RVH
= right
ventricular
Heart
1984
Journal
M-mode echocardiograph and sector scan Normal Not done Normal Normal Abnormal vessel penetrating the diaphragm Not done Not done Abnormal vessel penetrating the diaphragm Normal
hypertrophy
is probably not indicated, the high incidence of surgery in this seriesbeing a reflection of our initial enthusiasmfor operative treatment. As with uncomplicated atria1 septal defects, however, there are borderline caseswith moderate shunts in which the decision concerning surgery is not clear-cut. Surgical correction of those caseswith infradiaphragmatic drainage posesspecial problems and the demonstration of this anatomy on ultrasound is of value in deciding on the best management. From the experience of nine casesof the scimitar vein syndrome in this unit it can be concluded that: (1) the anomaly is most frequently detected by massradiographic screeningand its true incidence may be underestimated; (2) most patients have very mild or no symptomssymptoms may develop or increase with time; (3) in patients with symptomsattributable to a shunt and who have supradiaphragmatic drainage, surgery carries a low risk and is successful;and (4) infradiaphragmatic drainage can be confidently predicted by ultrasound examination even when catheter studiesare equivocal-the demonstration of this anatomy favors conservative management. REFERENCES
1. Cooper G: Case of malformation of the thoracic viscera: Consisting of imperfect development of right lung and transposition of the heart. London Med Gaz l&600, 1836. 2. Chassinat R: Observation d’anomalies anatomiques remarquables de l’appareil circulatoire avec hapatocele congeniale, n’ayant donne lieu pendant la vie a aucun symptom particulier. Arch Gen Med Paris (2nd series) 11:80, 1836. 3. Folger GM: The scimitar syndrome. Angiology 27:373, 1976. 4. Halasz NA, Halloran KH, Liebow AA: Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 14:826, 1956. 5. Kiely B, Filler J, Stone S, Doyle EF: Syndrome of anomalous venous drainage of the right lung to the inferior vena cava. A review of 67 reported cases and three new cases in children. Am J Cardiol 20~102, 1967. 6. Mathey J, Galey JJ, Logeais Y, Santoro E, Vanetti A: Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome). Report of three cases and review of the literature. Thorax 23:398, 1968.
Triple-valve endocarditis with unusual echocardiographic findings JamesM. Rippe, M.D., Frederick Curley, M.D., John A. Paraskos,M.D., Frederick J. Schoen, M.D., Ph.D., Lawrence H. Cohn, M.D., and Joseph S. Alpert, M.D. Worcester and Boston, Mass.
The value of echocardiography in demonstrating valvular vegetations in infective endocarditis (IE) is well established.‘-“’ Since the first reports of echocardiographically demonstrated vegetations in 1973,‘~~numerous investigators have defined echocardiographic criteria for vegetations on the mitral”, ‘* and aortic valves.13-2n While the tricuspid valve is more difficult to visualize by M-mode echocardiography, the development of two-dimensional sector scanning(2DE), with resultant improved tricuspid valve visualization, has led to improved accuracy in defining vegetations on this structure.21The majority of echocardiographically demonstrated vegetations occur on a single valve, while demonstration of involvement of two valves occurs much lessfrequently; triple-valve involvement is extremely rare. In a recent seriesof 47 patients with clinically proven IE and echocardiographicevidence of valvular vegetations, only one patient had involvement of three valves (2%).3 In a seriesof 25 opiate addicts with echocardiographic evidence of valvular vegetations, no casesof triple-valve involvement were found.22We recently had the opportunity to perform serial M-mode and 2DE on a patient with surgically proven triple-valve endocardi-
From the Division of Cardiovascular Medicine, University of Massachusetts Medical Center; and Department of Pathology, and Division of Thoracic and Cardiac Surgery, Brigham and Women’s Hospital. Reprint Medicine, Worcester,
requests: James M. Rippe, University of Massachusetts MA 01605.
M.D., Division of Cardiovascular Medical Center, 55 Lake Ave.,
Volume Number
107 3
Brief
a
Communications
599
b
C Fig. 1. Two-dimensional (long-axis projection) and M-mode echocardiograms of the mitral and aortic valve on patient TT (March 20, 1981). Asymmetric thickening of the posterior leaflet of the aortic valve with a prolapsing mass of shaggy echoes during diastole typical of aortic valvular vegetation is seen on both 2DE and M-mode echocardiogram (open arrows, a and cl. The mitral valve (MV) is asymmetrically thickened, with a mass of shaggy echoes prolapsing into the left atrium (LA) during systole, again typical of valvular vegetations (closed arrows, b and c). Left atria1 and right ventricular enlargement are also present. Ao = aorta; LV = left ventricle.
tis. The unusual echocardiographic demonstration of vegetations on tricuspid, mitral, and aortic valves illustrates the value and limitations of serial echocardiography in multivalvular IE. T.T., a previously healthy 59year-old female, was
admitted to a community hospital on January 28, 1981, complaining of 1 month of anorexia, malaise, myalgias, and intermittent fever. There was no history of recent surgical or dental procedure or intravenous drug abuse. Physical examination revealed a new III/VI holosystolic
600
Brief
Communications
American
a
March, 1984 Heart Journal
b
Fig. 2. 2DE (four-chamber projection) and M-mode echocardiograms on patient TT (March 20, 1981). The tricuspid valve is abnormally thickened on M-mode echocardiogram cc). 2DE in the four-chamber projection shows a mass of echoes prolapsing into the right atrium (RA) during systole (a) and falling into the right ventricle (RV) during diastole (b).
murmur, clear lungs, and no peripheral signs of IE. The clinical suspicion of IE was supported when two blood cultures grew Streptococcus viridans. Echocardiography was said to be normal. The patient was treated with a &week course of high-dose penicillin and streptomycin with prompt resolution of symptoms. Outpatient echocardiography performed at our institution 3 weeks after discharge (March 20, 1981) showed evidence of valvular vegetations involving mitral, aortic, and tricuspid valves in addition to right ventricular and left atria1 enlarge-
ment (Figs. 1 and 2). In April, 1981, the patient began to experience dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. In July, 1981, after a week of increasing abdominal girth and pedal edema, she was admitted to University of Massachusetts Medical Center. Physical examination at that point revealed marked jugular venous distention, evidence of pulmonary congestion, murmurs of aortic and mitral regurgitation, hepatomegaly, and marked pitting edema, all suggestive of severe biventricular heart failure.
Volume
107
Number
3
Brief
a
601
b
Fig. 3. 2DE (long-axis projection) and M-mode echocardiograms of the mitral and aortic T.T. (July 14,198l). Marked, asymmetric thickening of the posterior leaflet of the aortic on M-mode echocardiogram (open arrow, c). Long-axis 2DE reveals diastolic prolapse of echoes (open arrow, a). A prominent mass of echoes on the anterior leaflet of the mitral prolapse into the left atrium during systole (closed arrows, b and c).
M-mode and 2DE performed on July 14, 1981, once again revealed findings typical of valvular vegetations on aortic, mitral, and tricuspid valves (Figs. 3 and 4). Cardiac catheterization and angiography after diuresis revealed marked elevation of pulmonary artery pressures (86/36 mm Hg), left ventricular end-diastolic pressure of 24 mm Hg, and evidence of severe mitral, aortic, and tricuspid regurgitation. Emergency cardiac surgery showed a thickened, rolled posterior mitral valve leaflet and three ruptured chordae tendineae of the anterior mitral leaflet with healed vegetations on the valve. The aortic valve also contained healed vegetations, particularly on the noncor-
Communications
valve on patient valve is present a mass of shaggy valve is seen to
onary leaflet which was prolapsing into the left ventricle. The tricuspid valve revealed two vegetations, while inspection of the pulmonic valve revealed a grossly normal structure. Aortic and mitral valve replacement was performed together with excision of the tricuspid vegetations. Pathologic examination of mitral and aortic valves demonstrated small post-inflammatory lesions characterized by focal disruption of the normal layered valvular architecture, evolving fibrosis with irregular thick-walled blood vessels, and a moderate, mixed inflammatory infiltrate composed of mononuclear and polymorphonuclear leukocytes and pIasma cells (Fig. 5). The uninvolved portions of
602
Brief
Communications
American
March, 1994 Heart Journal
Fig. 4. 2DE (four-chamber projection) and M-mode echocardiograms on patient T.T. (July 14, 1981). A mass of echoes is seen during systole on M-mode echocardiography (closed arrow, c). 2DE shows this mass to prolapse into the right atrium during systole (closed arrow, a) and to disappear from view during diastole (b).
the valves had preserved architecture except for focal vascularization of the spongiosa of the normally avascular cusps. No organisms were seen in sections specifically stained for bacteria and fungi. The patient tolerated the operation well and was discharged from the hospital 2 weeks later. On follow-up visit 6 weeks after surgery, the patient continued to recover without difficulty and she was free of evidence of heart failure or recurrent IE. Although echocardiography has a well-established role in demonstrating valvular vegetations of IE, a number of
controversies remain. The sensitivity of echocardiography with respect to the demonstration of valvular vegetations remains in question.3, ‘.‘I In several series of clinically proven IE, echocardiography has demonstrated vegetations in only 54% to 77% 3.5*?2of cases. This relatively low sensitivity may result from technical difficulties (echocardiograms are unable to detect valvular lesions smaller than 2 mm in diameter), problems distinguishing other valvular pathologic changes from those of vegetations, and from problems in the clinical definition of IE. In one
Volume
107
Number
3
Brief Communications
603
Fig. 5. Morphology of mitral valve. a, Low magnification photomicrography demonstrating thickened and deformed leaflet. (Voerhoffs elastic tissue stain; original magnification x17.) b, Higher magnification photomicrograph of healing inflammatory lesion characterized by numerous thick and thin-walled blood vessels (arrows) and mixed cellular inflammatory infiltrate (asterisk). (Hematoxylin and eosin; original magnification x160.) autopsy series of patients with the clinical diagnosis of IE, only 53% of individuals had pathologically proven valvular vegetations. 23,24 Most reports indicate that patients with echocardiographic evidence of valvular vegetation suffer a higher incidence of embolic phenomena and congestive heart failure than patients with clinical evidence of IE, but echocardiographically normal valves3,”
Stewart et a1.3 report that 30% of patients with de !mon&rated valvular vegetations suffered systemic er nboli, while 30 % of such individuals developed congestive heart failure, ultimately requiring valve replacement. The size and location of valvular vegetations appc 3ar to carry no prognostic significance.3 Serial echocardiog why appears to have limited value in following patients after
604
Brief
Communications
adequate antibiotic therapy for IE.3 Vegetations are generally not visible until 2 weeks after the onset of symptoms of IE, and they may develop during the course of adequate antibiotic therapy.6 Serial echocardiography in patients with clinically resolved IE has shown decrease in size of valvular vegetations in 67% of cases, no change in size in 8%, and either increased size or inability to evaluate (because of complications of IE such as ruptured chordae tendineae or valvular disruption) in 25% .3 The chief role of serial echocardiography resides in its ability to provide repeat noninvasive assessment of cardiac chamber size and ventricular function. In the current case, initial echocardiograms done at an outside hospital were read as “normal.” Possible explanations for this include: a technically poor study, or a study done early in the course of IE before valvular vegetations or fibrin deposition had developed. Repeat echocardiography at our institution on March 20,1981, showed evidence of valvular vegetations on aortic, mitral, and tricuspid valves (Figs. 1 and 2). The findings of right ventricular enlargement and left atria1 enlargement on this echocardiogram, although nonspecific, provide early clues to the presence of tricuspid and mitral valvular incompetence. By the time the patient was admitted to University of Massachusetts Medical Center in July, 1981, she had developed marked biventricular heart failure. Echocardiographic examination performed on July 14, 1981, showed marked thickening of the noncoronary cusp of the aortic valve and a shaggy mass of echoes attached to the posterior leaflet of the mitral valve which was prolapsing into the left atrium during systole (Fig. 3). The apparently increased size of the mitral vegetation (Fig. 3) was artifactual and was demonstrated at surgery to be due to ruptured chordae tendineae. Marked thickening of the tricuspid valve (Fig. 4) and evidence of marked right ventricular volume overload as well as marked left atria1 enlargement was also present. Emergency surgery confirmed the presence of vegetations on all three valves, as well as ruptured chordae tendineae of the posterior leaflet of the mitral valve. This unusual case of echocardiographically demonstrated and surgically confirmed triple-valve endocarditis illustrates the importance of performing both M-mode and 2DE in patients with clinical evidence of IE. While previous reports have suggested that only marginally improved detection of vegetations on the mitral and aortic valves is achieved with the addition of ZDE,‘“,?” improved visualization of tricuspid valve vegetations is well established.21 While several large studies have demonstrated that patients with echocardiographically demonstrated vegetations suffer more systemic emboli and a higher incidence of congestive heart failure, the experience with vegetations demonstrated on multiple valves remains too small to assess if this confers a still higher risk. In our patient with vegetations on aortic, mitral, and tricuspid valves, rapid hemodynamic deterioration occurred. REFERENCES
1. Dillon JC, Feigenbaum H, Konecke LL: Echocardiographic manifestations of valvular vegetations. AM HEART J 86:698, 1973.
American
March, 1984 Heart Journal
2. Spangler
3.
4.
5t .
6.
7. 8.
9.
10.
11.
12. 13.
14. 15.
16.
17.
18.
19.
RD, Johnson ML, Holmes JH, Blount SG: Echocardiographic demonstration of bacterial vegetations in active infective endocarditis. J Clin Ultrasound 1:128, 1973. Stewart JA, Silimperi D, Harris P, Wise NK, Fraker TD, Kisslo JA: Echocardiographic documentation of vegetative lesions in infective endocarditis: Clinical implications. Circulation 61:374, 1980. Thomson KR, Nanda NC, Gramiak R: The reliability of echocardiography in the diagnosis of infective endocarditis. Radiology 125:473, 1977. Roy P, Tajik AJ, Guiliani ER, Schattenberg TT, Gan GT, Frye RL: Spectrum of echocardiographic findings in bacterial endocarditis. Circulation 53:474, 1976. Assey ME, Usher BW: Development of aortic valvular vegetations during appropriate antibiotic therapy. Chest 76:223, 1979. Dillon JC: Echocardiography in valvular vegetations. Am J Med 62:856, 1977. Wann LS, Dillon JC, Weyman AE, Feigenbaum H: Echocardiography in bacterial endocarditis. N Engl J Med 295135, 1976. Pietto DA, Parisi AF: Intracardiac masses: Tumors, vegetations, thrombi and foreign bodies. Med Clinics North Am 64:239, 1980. Perini GP, Marino P, Salazzare GC, Mancuso M, Potti A: Echocardiographic diagnosis of subacute bacterial endocarditis. Eur J Cardiol 11:315, 1980. Boucher CA, Fallon JT, Myers GS, Hutter AM, Buckley MJ: . The value and limitations of echocardiography in recording mitral valve vegetations. AM HEART J 94:37, 1977. Kisslo J: Echocardiography of the mitral valve. Med Clin North Am 64:215, 1980. Vignola PA, Swaye PS: Echocardiographic evaluation of aortic valve and root disease. Med Clin North Am 64:205, 1980. Wray TM: The variable echocardiographic features in aortic valve endocarditis. Circulation 52:658, 1975. Mintz GS, Kotler MN, Segal BL, Parry WR: Survival of patients with aortic valve endocarditis: The prognostic implications of the echocardiogram. Arch Intern Med 139:862, 1979. Mardelli TJ, Ogawa S, Hubbard FE, Dreifus LS, Meixell LL: Cross-sectional echocardiographic detection of aortic ring abscess in bacterial endocarditis. Chest 74:576, 1978. Sternberg L, Sole MJ, Joza P, Scully HE: Echocardiographic features of an unusual case of aortic valve endocarditis. CMA Journal 115:1022,1976. Reder RF, Yeh HC, Dimick I, Steinfield L: Serial echocardiography of the aortic valve in bacterial endocarditis: A case report. Mt Sinai J Med 44:521, 1977. Wray TM: Echocardiographic manifestations of flail aortic valve leaflets in bacterial endocarditis. Circulation 51:832, 1975.
Yoshikawa J, Tanaka K, Owaki T, Kato H: Cord-like aortic valve vegetation in bacterial endocarditis: Demonstration by cardiac ultrasonography. Circulation 53:911, 1976. 21. Kisslo J, Von Ramm OT, Haney R, Jones R, Juk SS, Behar VS: Echocardiographic evaluation of tricuspid valve endocarditis. Am J Cardiol 38:502, 1976. 22. Andy dJ, Sheikh MU, Barnes BO, Fox LM, Curry CL, Roberts WC: Echocardiographic observations in opiate addicts with active infective endocarditis. Am J Cardiol 40:17, 1977. 23. Buckbinder NA, Roberts WC: Left-sided valvular active infective endocarditis: A study of forty-five necropsy patients. Am J Med 53:20, 1972. 24. Arnett EN, Roberts WC: Active infective endocarditis: A clinicopathologic analysis of 137 necropsy patients. In Current Problems in cardiology. vol 1. No. 7, 1976. 25. Minta GS, Kotler MN, Segal BL, Parry WR: Comparison of two-dimensional and M-mode echocardiography in the evaluation of patients with infective endocarditis. Am J Cardiol 43:739, 1979. 20.
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26. Gilbert BW, Honey RS, Crawford F, McClellan J, Gallio HA, Johnson ML, Kisslo JA: Two-dimensional echocardiographic assessment of vegetative endocarditis. Circulation 55:346, 1977. 27. Wann LS, Hallam CC, Dillon JC, Weyman AE, Feigenbaum H: Comparison of M-mode and cross-sectional echocardiography in infective endocarditis. Circulation 60:728, 1979. 28. Pedersen JF, Berning J, Hauns S: Single and multiple beam echocardiography in aortic valve endocarditis. Acta Med Stand 204:315, 1978.
Visualization of pericardial thymoma evaluation of chemotherapy by two-dimensional echocardiography
and
Sabino Iliceto, M.D., Donato Quagliara, M.D., Piero Calabrese,M.D., and Paolo R&on, M.D. Bari, Italy
A 27-year-old woman was hospitalized with a 2-month history of fever and chest pain. On admission,auscultation revealed faint normal first and second heart sounds without murmurs or rubs. Percussion of the cardiac outline suggestedcardiac enlargement. Laboratory data were normal. Chest x-ray findings were consistent with a large pericardial effusion. ECG showed normal sinus rhythm and Q wavesin leadsII, III and aVF. A wide-angle
From the Division of Cardiology, University ogy Section of the Oncologic Hospital. Reprint requests: Sabino Iliceto, M.D., Medica I, Universiti di Bari, Policlinico,
of Bari; and the Cardiol-
Cattedra di Cardiologia, Viale Ennio, Bari, Italy.
Clinica
two-dimensional echocardiogram(2DE) was made with a Toshiba SL-53M phased-array system which demonstrated a pericardial effusion and a large irregular cauliflower-like mass protruding into the pericardial space from the epicardium (Fig. 1). Median sternotomy was performed, which revealed absenceof anterior mediastinal involvement. However, there was a large intrapericardial mass arising from the anterior wall of the heart. The tumor was not removed and a biopsy of the tumor was taken which showed histologic findings consistent with the diagnosisof an epithelial spindle cell thymoma (Fig. 2). Chemotherapy with BACOP (bleomycin, Adriamycin (doxorubicin HCl), cyclophosphamide, vincristine, prednisone) was begun and 2 months later 2DE showed no pericardial effusion and marked regressionof the tumor mass (Fig. 3). On chest x-ray examination, there was marked reduction of the cardiothoracic ratio and ECG revealed normal axis orientation. Thymomas mainly involve the anterior mediastinum and rarely arise from thymic rests which are not infrequently encountered in the pericardium.’ A diagnosisof primary pericardial thymoma can be made only when there is no evidence of involvement of the anterior mediastinum. Therefore, this casewas considered a primary pericardial thymoma. M-mode echocardiography is useful for detecting pericardial effusions.’ Wide-angle 2DE, becauseof its better spatial orientation, permits assessment of the size and distribution of fluid and recognition of loculated effusions3; furthermore, intrapericardial abnormalities can be detected.4 It has been recently demonstrated that 2DE is a useful tool in the recognition of intrapericardial tumom5 Our caseconfirms this ability; a thymoma not suspectedclinically waseasily detected by 2DE and this technique wasvaluable in the assessment of the effects of chemotherapy.
Fig. 1. Two-dimensional echocardiogrambefore chemotherapy. A pericardial effusion (PE) and a large cauliflower-like tumor mass(T) protruding from the visceral pericardium are present. The arrows indicate a fibrinous strand. RV = right ventricle; LV = left ventricle; RA = right atrium.
598
Brief
Communications
Table
II. Noninvasive data
CUSP
Scimitar sign on chest radiograph
ECG
1
RBBB
American
2 3 4 5
Normal Incomplete Normal Normal Incomplete
Only on tomography Present Present Present Present
6 7 8
RVH RVH
Present Absent Absent
9
Normal
Present
RBBB
RBBB -
= right
bundle
branch
block;
RVH
= right
ventricular
Heart
1984
Journal
M-mode echocardiograph and sector scan Normal Not done Normal Normal Abnormal vessel penetrating the diaphragm Not done Not done Abnormal vessel penetrating the diaphragm Normal
hypertrophy
is probably not indicated, the high incidence of surgery in this seriesbeing a reflection of our initial enthusiasmfor operative treatment. As with uncomplicated atria1 septal defects, however, there are borderline caseswith moderate shunts in which the decision concerning surgery is not clear-cut. Surgical correction of those caseswith infradiaphragmatic drainage posesspecial problems and the demonstration of this anatomy on ultrasound is of value in deciding on the best management. From the experience of nine casesof the scimitar vein syndrome in this unit it can be concluded that: (1) the anomaly is most frequently detected by massradiographic screeningand its true incidence may be underestimated; (2) most patients have very mild or no symptomssymptoms may develop or increase with time; (3) in patients with symptomsattributable to a shunt and who have supradiaphragmatic drainage, surgery carries a low risk and is successful;and (4) infradiaphragmatic drainage can be confidently predicted by ultrasound examination even when catheter studiesare equivocal-the demonstration of this anatomy favors conservative management. REFERENCES
1. Cooper G: Case of malformation of the thoracic viscera: Consisting of imperfect development of right lung and transposition of the heart. London Med Gaz l&600, 1836. 2. Chassinat R: Observation d’anomalies anatomiques remarquables de l’appareil circulatoire avec hapatocele congeniale, n’ayant donne lieu pendant la vie a aucun symptom particulier. Arch Gen Med Paris (2nd series) 11:80, 1836. 3. Folger GM: The scimitar syndrome. Angiology 27:373, 1976. 4. Halasz NA, Halloran KH, Liebow AA: Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 14:826, 1956. 5. Kiely B, Filler J, Stone S, Doyle EF: Syndrome of anomalous venous drainage of the right lung to the inferior vena cava. A review of 67 reported cases and three new cases in children. Am J Cardiol 20~102, 1967. 6. Mathey J, Galey JJ, Logeais Y, Santoro E, Vanetti A: Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome). Report of three cases and review of the literature. Thorax 23:398, 1968.
Triple-valve endocarditis with unusual echocardiographic findings JamesM. Rippe, M.D., Frederick Curley, M.D., John A. Paraskos,M.D., Frederick J. Schoen, M.D., Ph.D., Lawrence H. Cohn, M.D., and Joseph S. Alpert, M.D. Worcester and Boston, Mass.
The value of echocardiography in demonstrating valvular vegetations in infective endocarditis (IE) is well established.‘-“’ Since the first reports of echocardiographically demonstrated vegetations in 1973,‘~~numerous investigators have defined echocardiographic criteria for vegetations on the mitral”, ‘* and aortic valves.13-2n While the tricuspid valve is more difficult to visualize by M-mode echocardiography, the development of two-dimensional sector scanning(2DE), with resultant improved tricuspid valve visualization, has led to improved accuracy in defining vegetations on this structure.21The majority of echocardiographically demonstrated vegetations occur on a single valve, while demonstration of involvement of two valves occurs much lessfrequently; triple-valve involvement is extremely rare. In a recent seriesof 47 patients with clinically proven IE and echocardiographicevidence of valvular vegetations, only one patient had involvement of three valves (2%).3 In a seriesof 25 opiate addicts with echocardiographic evidence of valvular vegetations, no casesof triple-valve involvement were found.22We recently had the opportunity to perform serial M-mode and 2DE on a patient with surgically proven triple-valve endocardi-
From the Division of Cardiovascular Medicine, University of Massachusetts Medical Center; and Department of Pathology, and Division of Thoracic and Cardiac Surgery, Brigham and Women’s Hospital. Reprint Medicine, Worcester,
requests: James M. Rippe, University of Massachusetts MA 01605.
M.D., Division of Cardiovascular Medical Center, 55 Lake Ave.,
Volume Number
107 3
Brief
a
Communications
599
b
C Fig. 1. Two-dimensional (long-axis projection) and M-mode echocardiograms of the mitral and aortic valve on patient TT (March 20, 1981). Asymmetric thickening of the posterior leaflet of the aortic valve with a prolapsing mass of shaggy echoes during diastole typical of aortic valvular vegetation is seen on both 2DE and M-mode echocardiogram (open arrows, a and cl. The mitral valve (MV) is asymmetrically thickened, with a mass of shaggy echoes prolapsing into the left atrium (LA) during systole, again typical of valvular vegetations (closed arrows, b and c). Left atria1 and right ventricular enlargement are also present. Ao = aorta; LV = left ventricle.
tis. The unusual echocardiographic demonstration of vegetations on tricuspid, mitral, and aortic valves illustrates the value and limitations of serial echocardiography in multivalvular IE. T.T., a previously healthy 59year-old female, was
admitted to a community hospital on January 28, 1981, complaining of 1 month of anorexia, malaise, myalgias, and intermittent fever. There was no history of recent surgical or dental procedure or intravenous drug abuse. Physical examination revealed a new III/VI holosystolic
600
Brief
Communications
American
a
March, 1984 Heart Journal
b
Fig. 2. 2DE (four-chamber projection) and M-mode echocardiograms on patient TT (March 20, 1981). The tricuspid valve is abnormally thickened on M-mode echocardiogram cc). 2DE in the four-chamber projection shows a mass of echoes prolapsing into the right atrium (RA) during systole (a) and falling into the right ventricle (RV) during diastole (b).
murmur, clear lungs, and no peripheral signs of IE. The clinical suspicion of IE was supported when two blood cultures grew Streptococcus viridans. Echocardiography was said to be normal. The patient was treated with a &week course of high-dose penicillin and streptomycin with prompt resolution of symptoms. Outpatient echocardiography performed at our institution 3 weeks after discharge (March 20, 1981) showed evidence of valvular vegetations involving mitral, aortic, and tricuspid valves in addition to right ventricular and left atria1 enlarge-
ment (Figs. 1 and 2). In April, 1981, the patient began to experience dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. In July, 1981, after a week of increasing abdominal girth and pedal edema, she was admitted to University of Massachusetts Medical Center. Physical examination at that point revealed marked jugular venous distention, evidence of pulmonary congestion, murmurs of aortic and mitral regurgitation, hepatomegaly, and marked pitting edema, all suggestive of severe biventricular heart failure.
Volume
107
Number
3
Brief
a
601
b
Fig. 3. 2DE (long-axis projection) and M-mode echocardiograms of the mitral and aortic T.T. (July 14,198l). Marked, asymmetric thickening of the posterior leaflet of the aortic on M-mode echocardiogram (open arrow, c). Long-axis 2DE reveals diastolic prolapse of echoes (open arrow, a). A prominent mass of echoes on the anterior leaflet of the mitral prolapse into the left atrium during systole (closed arrows, b and c).
M-mode and 2DE performed on July 14, 1981, once again revealed findings typical of valvular vegetations on aortic, mitral, and tricuspid valves (Figs. 3 and 4). Cardiac catheterization and angiography after diuresis revealed marked elevation of pulmonary artery pressures (86/36 mm Hg), left ventricular end-diastolic pressure of 24 mm Hg, and evidence of severe mitral, aortic, and tricuspid regurgitation. Emergency cardiac surgery showed a thickened, rolled posterior mitral valve leaflet and three ruptured chordae tendineae of the anterior mitral leaflet with healed vegetations on the valve. The aortic valve also contained healed vegetations, particularly on the noncor-
Communications
valve on patient valve is present a mass of shaggy valve is seen to
onary leaflet which was prolapsing into the left ventricle. The tricuspid valve revealed two vegetations, while inspection of the pulmonic valve revealed a grossly normal structure. Aortic and mitral valve replacement was performed together with excision of the tricuspid vegetations. Pathologic examination of mitral and aortic valves demonstrated small post-inflammatory lesions characterized by focal disruption of the normal layered valvular architecture, evolving fibrosis with irregular thick-walled blood vessels, and a moderate, mixed inflammatory infiltrate composed of mononuclear and polymorphonuclear leukocytes and pIasma cells (Fig. 5). The uninvolved portions of
602
Brief
Communications
American
March, 1994 Heart Journal
Fig. 4. 2DE (four-chamber projection) and M-mode echocardiograms on patient T.T. (July 14, 1981). A mass of echoes is seen during systole on M-mode echocardiography (closed arrow, c). 2DE shows this mass to prolapse into the right atrium during systole (closed arrow, a) and to disappear from view during diastole (b).
the valves had preserved architecture except for focal vascularization of the spongiosa of the normally avascular cusps. No organisms were seen in sections specifically stained for bacteria and fungi. The patient tolerated the operation well and was discharged from the hospital 2 weeks later. On follow-up visit 6 weeks after surgery, the patient continued to recover without difficulty and she was free of evidence of heart failure or recurrent IE. Although echocardiography has a well-established role in demonstrating valvular vegetations of IE, a number of
controversies remain. The sensitivity of echocardiography with respect to the demonstration of valvular vegetations remains in question.3, ‘.‘I In several series of clinically proven IE, echocardiography has demonstrated vegetations in only 54% to 77% 3.5*?2of cases. This relatively low sensitivity may result from technical difficulties (echocardiograms are unable to detect valvular lesions smaller than 2 mm in diameter), problems distinguishing other valvular pathologic changes from those of vegetations, and from problems in the clinical definition of IE. In one
Volume
107
Number
3
Brief Communications
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Fig. 5. Morphology of mitral valve. a, Low magnification photomicrography demonstrating thickened and deformed leaflet. (Voerhoffs elastic tissue stain; original magnification x17.) b, Higher magnification photomicrograph of healing inflammatory lesion characterized by numerous thick and thin-walled blood vessels (arrows) and mixed cellular inflammatory infiltrate (asterisk). (Hematoxylin and eosin; original magnification x160.) autopsy series of patients with the clinical diagnosis of IE, only 53% of individuals had pathologically proven valvular vegetations. 23,24 Most reports indicate that patients with echocardiographic evidence of valvular vegetation suffer a higher incidence of embolic phenomena and congestive heart failure than patients with clinical evidence of IE, but echocardiographically normal valves3,”
Stewart et a1.3 report that 30% of patients with de !mon&rated valvular vegetations suffered systemic er nboli, while 30 % of such individuals developed congestive heart failure, ultimately requiring valve replacement. The size and location of valvular vegetations appc 3ar to carry no prognostic significance.3 Serial echocardiog why appears to have limited value in following patients after
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adequate antibiotic therapy for IE.3 Vegetations are generally not visible until 2 weeks after the onset of symptoms of IE, and they may develop during the course of adequate antibiotic therapy.6 Serial echocardiography in patients with clinically resolved IE has shown decrease in size of valvular vegetations in 67% of cases, no change in size in 8%, and either increased size or inability to evaluate (because of complications of IE such as ruptured chordae tendineae or valvular disruption) in 25% .3 The chief role of serial echocardiography resides in its ability to provide repeat noninvasive assessment of cardiac chamber size and ventricular function. In the current case, initial echocardiograms done at an outside hospital were read as “normal.” Possible explanations for this include: a technically poor study, or a study done early in the course of IE before valvular vegetations or fibrin deposition had developed. Repeat echocardiography at our institution on March 20,1981, showed evidence of valvular vegetations on aortic, mitral, and tricuspid valves (Figs. 1 and 2). The findings of right ventricular enlargement and left atria1 enlargement on this echocardiogram, although nonspecific, provide early clues to the presence of tricuspid and mitral valvular incompetence. By the time the patient was admitted to University of Massachusetts Medical Center in July, 1981, she had developed marked biventricular heart failure. Echocardiographic examination performed on July 14, 1981, showed marked thickening of the noncoronary cusp of the aortic valve and a shaggy mass of echoes attached to the posterior leaflet of the mitral valve which was prolapsing into the left atrium during systole (Fig. 3). The apparently increased size of the mitral vegetation (Fig. 3) was artifactual and was demonstrated at surgery to be due to ruptured chordae tendineae. Marked thickening of the tricuspid valve (Fig. 4) and evidence of marked right ventricular volume overload as well as marked left atria1 enlargement was also present. Emergency surgery confirmed the presence of vegetations on all three valves, as well as ruptured chordae tendineae of the posterior leaflet of the mitral valve. This unusual case of echocardiographically demonstrated and surgically confirmed triple-valve endocarditis illustrates the importance of performing both M-mode and 2DE in patients with clinical evidence of IE. While previous reports have suggested that only marginally improved detection of vegetations on the mitral and aortic valves is achieved with the addition of ZDE,‘“,?” improved visualization of tricuspid valve vegetations is well established.21 While several large studies have demonstrated that patients with echocardiographically demonstrated vegetations suffer more systemic emboli and a higher incidence of congestive heart failure, the experience with vegetations demonstrated on multiple valves remains too small to assess if this confers a still higher risk. In our patient with vegetations on aortic, mitral, and tricuspid valves, rapid hemodynamic deterioration occurred. REFERENCES
1. Dillon JC, Feigenbaum H, Konecke LL: Echocardiographic manifestations of valvular vegetations. AM HEART J 86:698, 1973.
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RD, Johnson ML, Holmes JH, Blount SG: Echocardiographic demonstration of bacterial vegetations in active infective endocarditis. J Clin Ultrasound 1:128, 1973. Stewart JA, Silimperi D, Harris P, Wise NK, Fraker TD, Kisslo JA: Echocardiographic documentation of vegetative lesions in infective endocarditis: Clinical implications. Circulation 61:374, 1980. Thomson KR, Nanda NC, Gramiak R: The reliability of echocardiography in the diagnosis of infective endocarditis. Radiology 125:473, 1977. Roy P, Tajik AJ, Guiliani ER, Schattenberg TT, Gan GT, Frye RL: Spectrum of echocardiographic findings in bacterial endocarditis. Circulation 53:474, 1976. Assey ME, Usher BW: Development of aortic valvular vegetations during appropriate antibiotic therapy. Chest 76:223, 1979. Dillon JC: Echocardiography in valvular vegetations. Am J Med 62:856, 1977. Wann LS, Dillon JC, Weyman AE, Feigenbaum H: Echocardiography in bacterial endocarditis. N Engl J Med 295135, 1976. Pietto DA, Parisi AF: Intracardiac masses: Tumors, vegetations, thrombi and foreign bodies. Med Clinics North Am 64:239, 1980. Perini GP, Marino P, Salazzare GC, Mancuso M, Potti A: Echocardiographic diagnosis of subacute bacterial endocarditis. Eur J Cardiol 11:315, 1980. Boucher CA, Fallon JT, Myers GS, Hutter AM, Buckley MJ: . The value and limitations of echocardiography in recording mitral valve vegetations. AM HEART J 94:37, 1977. Kisslo J: Echocardiography of the mitral valve. Med Clin North Am 64:215, 1980. Vignola PA, Swaye PS: Echocardiographic evaluation of aortic valve and root disease. Med Clin North Am 64:205, 1980. Wray TM: The variable echocardiographic features in aortic valve endocarditis. Circulation 52:658, 1975. Mintz GS, Kotler MN, Segal BL, Parry WR: Survival of patients with aortic valve endocarditis: The prognostic implications of the echocardiogram. Arch Intern Med 139:862, 1979. Mardelli TJ, Ogawa S, Hubbard FE, Dreifus LS, Meixell LL: Cross-sectional echocardiographic detection of aortic ring abscess in bacterial endocarditis. Chest 74:576, 1978. Sternberg L, Sole MJ, Joza P, Scully HE: Echocardiographic features of an unusual case of aortic valve endocarditis. CMA Journal 115:1022,1976. Reder RF, Yeh HC, Dimick I, Steinfield L: Serial echocardiography of the aortic valve in bacterial endocarditis: A case report. Mt Sinai J Med 44:521, 1977. Wray TM: Echocardiographic manifestations of flail aortic valve leaflets in bacterial endocarditis. Circulation 51:832, 1975.
Yoshikawa J, Tanaka K, Owaki T, Kato H: Cord-like aortic valve vegetation in bacterial endocarditis: Demonstration by cardiac ultrasonography. Circulation 53:911, 1976. 21. Kisslo J, Von Ramm OT, Haney R, Jones R, Juk SS, Behar VS: Echocardiographic evaluation of tricuspid valve endocarditis. Am J Cardiol 38:502, 1976. 22. Andy dJ, Sheikh MU, Barnes BO, Fox LM, Curry CL, Roberts WC: Echocardiographic observations in opiate addicts with active infective endocarditis. Am J Cardiol 40:17, 1977. 23. Buckbinder NA, Roberts WC: Left-sided valvular active infective endocarditis: A study of forty-five necropsy patients. Am J Med 53:20, 1972. 24. Arnett EN, Roberts WC: Active infective endocarditis: A clinicopathologic analysis of 137 necropsy patients. In Current Problems in cardiology. vol 1. No. 7, 1976. 25. Minta GS, Kotler MN, Segal BL, Parry WR: Comparison of two-dimensional and M-mode echocardiography in the evaluation of patients with infective endocarditis. Am J Cardiol 43:739, 1979. 20.
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26. Gilbert BW, Honey RS, Crawford F, McClellan J, Gallio HA, Johnson ML, Kisslo JA: Two-dimensional echocardiographic assessment of vegetative endocarditis. Circulation 55:346, 1977. 27. Wann LS, Hallam CC, Dillon JC, Weyman AE, Feigenbaum H: Comparison of M-mode and cross-sectional echocardiography in infective endocarditis. Circulation 60:728, 1979. 28. Pedersen JF, Berning J, Hauns S: Single and multiple beam echocardiography in aortic valve endocarditis. Acta Med Stand 204:315, 1978.
Visualization of pericardial thymoma evaluation of chemotherapy by two-dimensional echocardiography
and
Sabino Iliceto, M.D., Donato Quagliara, M.D., Piero Calabrese,M.D., and Paolo R&on, M.D. Bari, Italy
A 27-year-old woman was hospitalized with a 2-month history of fever and chest pain. On admission,auscultation revealed faint normal first and second heart sounds without murmurs or rubs. Percussion of the cardiac outline suggestedcardiac enlargement. Laboratory data were normal. Chest x-ray findings were consistent with a large pericardial effusion. ECG showed normal sinus rhythm and Q wavesin leadsII, III and aVF. A wide-angle
From the Division of Cardiology, University ogy Section of the Oncologic Hospital. Reprint requests: Sabino Iliceto, M.D., Medica I, Universiti di Bari, Policlinico,
of Bari; and the Cardiol-
Cattedra di Cardiologia, Viale Ennio, Bari, Italy.
Clinica
two-dimensional echocardiogram(2DE) was made with a Toshiba SL-53M phased-array system which demonstrated a pericardial effusion and a large irregular cauliflower-like mass protruding into the pericardial space from the epicardium (Fig. 1). Median sternotomy was performed, which revealed absenceof anterior mediastinal involvement. However, there was a large intrapericardial mass arising from the anterior wall of the heart. The tumor was not removed and a biopsy of the tumor was taken which showed histologic findings consistent with the diagnosisof an epithelial spindle cell thymoma (Fig. 2). Chemotherapy with BACOP (bleomycin, Adriamycin (doxorubicin HCl), cyclophosphamide, vincristine, prednisone) was begun and 2 months later 2DE showed no pericardial effusion and marked regressionof the tumor mass (Fig. 3). On chest x-ray examination, there was marked reduction of the cardiothoracic ratio and ECG revealed normal axis orientation. Thymomas mainly involve the anterior mediastinum and rarely arise from thymic rests which are not infrequently encountered in the pericardium.’ A diagnosisof primary pericardial thymoma can be made only when there is no evidence of involvement of the anterior mediastinum. Therefore, this casewas considered a primary pericardial thymoma. M-mode echocardiography is useful for detecting pericardial effusions.’ Wide-angle 2DE, becauseof its better spatial orientation, permits assessment of the size and distribution of fluid and recognition of loculated effusions3; furthermore, intrapericardial abnormalities can be detected.4 It has been recently demonstrated that 2DE is a useful tool in the recognition of intrapericardial tumom5 Our caseconfirms this ability; a thymoma not suspectedclinically waseasily detected by 2DE and this technique wasvaluable in the assessment of the effects of chemotherapy.
Fig. 1. Two-dimensional echocardiogrambefore chemotherapy. A pericardial effusion (PE) and a large cauliflower-like tumor mass(T) protruding from the visceral pericardium are present. The arrows indicate a fibrinous strand. RV = right ventricle; LV = left ventricle; RA = right atrium.