Quadrivalvular marantic endocarditis (ME) mimicking acute bacterial endocarditis (ABE)

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ISSUES IN INFECTIOUS DISEASE

Quadrivalvular marantic endocarditis (ME) mimicking acute bacterial endocarditis (ABE) Nicole M. Durie, MD,c Lawrence E. Eisenstein, MD,a,c Burke A. Cunha, MD,a,c and Maria Maratta Plummer, MDb

Marantic endocarditis (ME) is defined by noninfectious valvular vegetations. The most common disorders associated with ME are malignancy with or without hypercoagulable state, intercardiac instrumentation, residual vegetations from previously treated infective endocarditis (IE), renal insufficiency, and burns. Another important cause of ME is systemic lupus erythematosus when accompanied by vegetations, that is, Libman-Sacks endocarditis. ME should be differentiated from IE because they may present with similar clinical features. Both ME and IE may present with fever and a heart murmur with or without embolic phenomenon. Leukocytosis and elevated erythrocyte sedimentation rate suggest the diagnosis of IE. The hallmark of IE is a cardiac vegetation and continuous high-grade bacteremia. After exclusion of the causes of culture negative endocarditis, the absence of bacteremia clearly differentiates ME from IE. We present a case of ME mimicking acute bacterial endocarditis (ABE). The differential diagnostic features of ME versus IE are discussed. To the best of our knowledge, this is the first reported case of quadrivalvular ME with massive vegetations on all cardiac valves, as well as the aorta, atria, and pulmonary artery. (Heart Lung® 2007;36:154 –158.)

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arantic endocarditis (ME) is defined as having one or more noninfected intracardiac vegetations often accompanied by fever, heart murmur, and embolic phenomenon mimicking infective endocarditis (IE).1-5 As in IE, ME typically involves one cardiac valve, and the composition of the vegetations in ME resemble those of IE in composition and location. Marantic vegetations heal by becoming endothelialized, and subsequent calcification is not uncommon. Lambl’s excrescences (ie, tumor-like masses) are most likely to be found on the valve cusps, which usually become thickened and stenotic. The involvement of two or three cardiac valves in ME is rare.1,3 Because the

From the aInfectious Disease Division and bDepartment of Pathology, Winthrop-University Hospital, Mineola, New York, and c State University of New York School of Medicine, Stony Brook, New York. Reprint requests: Burke A. Cunha, MD, Chief, Infectious Diseases Division, Winthrop-University Hospital, Mineola, NY 11501. 0147-9563/$ – see front matter Copyright © 2007 by Mosby, Inc. doi:10.1016/j.hrtlng.2006.08.009

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vegetations in ME are by definition uninfected, bacteremia is not associated with ME. Continuous, prolonged, high-grade bacteremia is characteristic of IE, particularly when it is caused by Streptococcus viridans or Staphylococcus aureus.6 Because ME and IE have many overlapping features (eg, fever, heart murmur with or without embolic phenomenon), the vegetations resulting from ME or IE on echocardiography of the heart have an identical appearance, either by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE).7,8 Therefore, the critical determinant in differentiating ME from IE is the presence of a continuous high-grade bacteremia caused by a usual endocarditis pathogen. The most common predisposing factors to ME are malignancy with or without an associated hypercoagulable state. Up to 75% of marantic lesions at autopsy may be found in patients with cancer, most notably those with mucin-producing adenocarcinomas or lymphomas.1,3 A variety of other disorders (ie, giant cell arteritis, but most notably systemic lupus erythematosus when accompanied by classic Libman-Sacks vegetations) are important causes of ME.9 Libman-Sacks vegetations are found

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at autopsy in approximately 50% of patients with systemic lupus erythematosus, most often on the mitral and aortic valves. Multivalvular involvement is common in Libman-Sacks ME. Although most often found incidentally, Libman-Sacks vegetations often result in valvular regurgitation compared with nonmarantic lesions, which more commonly cause stenosis.10-15 Indwelling cardiac catheter procedures are another risk factor for right-sided marantic vegetations,16 particularly in right-sided marantic vegetations. Valvular damage by the catheter may result in vegetation formation. Other risk factors for marantic vegetations include renal failure and burns. It is interesting that given the same pathophysiology of IE and ME, the sterile vegetations of ME are not infected more often. The incidence of antecedent ME and subsequent IE is rare.10-13 The best way to differentiate ME from IE in patients with a fever and heart murmur is the absence of bacteremia with ME.6 Other tests may be useful in differentiating these two entities that closely resemble each other. The erythrocyte sedimentation rate (ESR) is regularly elevated in IE. Elevations of the ESR in patients with a fever and heart murmur may be reflective of a cardiac process (eg, IE, atrial myxoma, cardiac tumor, myocardial abscess) or a noncardiac source (eg, malignancy, osteomyelitis, collagen-vascular disease). Therefore, the sedimentation rate can be helpful in differentiating IE from ME. The ESR will be elevated in IE, but not ME, unless the ME is secondary to a malignancy. If malignancy can be excluded, the ESR is a useful, preliminary, nonspecific laboratory test to differentiate IE from ME pending blood culture results.6 We present an autopsy-proven case of quadrivalvular ME. To the best of our knowledge, this is the first case of quadrivalvular ME that also involved the pulmonary artery, aorta, and atria.

CASE REPORT A 69-year-old man was admitted with a chief symptom of shortness of breath. His medical history included insulin-dependant diabetes mellitus, congestive heart failure, end-stage renal disease with hemodialysis, and coronary artery disease with a coronary artery stent and an automated internal cardiac defibrillator placement. He had recently been discharged from the hospital after a non-STelevation myocardial infarction. His hospital course was complicated by Clostridium difficile colitis, which resolved with therapy during his hospital stay. TTE showed no vegetations.

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The patient was admitted to the emergency department in a hemodynamically unstable condition. His blood pressure was 80/70 mm Hg, heart rate was 120 beats/min, and respiratory rate was 16 to 20 breaths/min. The patient was febrile to 101°F for several days, but on readmission his temperature was 95.6°F. The patient was awake and oriented. Head, ears, eyes, nose, and throat examination results were normal. Cardiovascular examination revealed an irregular pulse with holosystolic murmur. There were decreased bilateral breath sounds and basilar rales. The abdomen was soft with normal bowel sounds and no organomegaly or ascites. The patient had mild bilateral peripheral edema. The remainder of his physical examination results were unremarkable. All admission laboratory test results were within normal limits, including a white blood count of 7.5 K/mm3. Chest radiography was negative for infiltrates and cardiomegaly. The patient was given fluids and blood pressure support (dopamine). Blood cultures were drawn, and broad-spectrum antibiotics were started for possible acute bacterial endocarditis (ABE). The patient did not respond to fluids, and his condition quickly deteriorated. He became pulseless, had a cardiac arrest, and died within 8 hours of admission. The cause of death was cardiogenic shock secondary to acute massive inferolateral myocardial infarction. The autopsy showed extensive marantic vegetations on all heart valves, both atria, the aorta, and the pulmonary artery. Blood cultures were subsequently reported to be negative. Postmortem bacterial cultures of all vegetations were negative. Microscopically there was no evidence of infection in the vegetations (Fig 1-3).

DISCUSSION ME is most likely to be confused with IE in clinical practice. Most cases of ME are not suspected antemortem and are found at autopsy. ME is characterized by uninfected valvular vegetations. Univalvular involvement is the rule with ME, as it is with IE. ME is uncommon to begin with, and two-valve involvement is even more unusual, followed by trivalvular involvement, which is rare. In patients with fever and heart murmur, ME may be confused with IE. Both ME and IE have valvular vegetations, visualized by TTE or TEE. The appearance of cardiac vegetations by TTE/TEE in ME is indistinguishable from that in IE. Patients with ME and no fever or embolic phenomenon, and with only a heart mur-

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Fig 1 Autopsy results showing aortic valve with marantic vegetations.

Fig 2 Autopsy results showing mitral valve with marantic vegetations.

mur, are the subset who are undiagnosed antemortem. The hallmark of IE is continuous, highgrade bacteremia (ie, 3/4 – 4/4 positive blood cultures). Pending blood culture results in patients with a fever and heart murmur, the ESR may be useful in arguing against the diagnosis of ME. The ESR is moderately elevated in S. aureus ABE

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Fig 3 Autopsy results showing tricuspid valve with marantic vegetations.

(40 –50 mm/h) or may be highly elevated in SBE (80 –120 mm/h).6 The ESR is not elevated in patients with ME if the ME is not secondary to a malignancy, which would explain an elevated ESR (Table I). This is a unique case of quadrivalvular ME. The vegetations in both atria and in the aorta in this case of quadrivalvular ME make it even more remarkable. It is not known how quickly marantic vegetations are formed.1-5 In S. aureus ABE, vegetations may be visualized as soon as 3 days after intracardiac instrumentation. Because our patient had a cardiac history, he fortuitously had cardiac echocardiography approximately 1 month before his demise, and there were no vegetations at that time. There are no data on how quickly ME vegetations form, but in this case with quadrivalvular vegetations, as well as aortic and atrial involvement, it can be said that these extensive marantic vegetations formed within a few weeks. The patient presented was initially thought to have IE because of his fever, heart murmur, and hypotension, but hypotension does not occur with SBE in the absence of a coronary artery emboli but may occur with S. aureus ABE with valvular destruction and heart failure.6 The patient was administered empiric antimicrobial therapy for the possibility of ABE. Because of the patient’s rapid deterioration and demise, repeat cardiac echocardiography was not performed, and the diagnosis of ME was made at autopsy. No embolic phenomena were

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Table I Differential diagnosis of marantic endocarditis versus infectious endocarditis Marantic endocarditis

Infectious endocarditis

Fever



Heart murmur Vegetations on cardiac echocardiography Continuous/high-grade bacteremia Elevated WBC count Elevated ESR

⫹ ⫹ ⫺ ⫺ ⫺

⫹ (SBE ⬍ 102°F) (ABE ⬎ 102°F)* ⫹ ⫹ ⫹ ⫹ (SBE ⫽ 80–120 mm/h) (ABE ⫽ 40–50 mm/h)

WBC, White blood cell count; ESR, erythrocyte sedimentation rate; SBE, subacute bacterial endocarditis; ABE, acute bacterial endocarditis. Adapted from Cunha BA, Gill, MV, Lazar JM. Acute infectious endocarditis: diagnostic and therapeutic approach. Infect Dis Clin North Am 1996;10:811-34, with permission. *Excluding intravenous drug abusers.

present in this patient, and blood cultures were eventually reported to be negative. His ESR was not elevated, and at autopsy there were no signs of IE (eg, splenomegaly, septic emboli, and myocardial abscesses). Although the patient had chronic renal failure, ME developed a few weeks before his death. He did not have a malignancy or collagen vascular disease to explain his ME, and the cause of this patient’s quadrivalvular ME is unknown. Clinicians should be wary and keep ME in the differential diagnosis of IE when patients present with a fever and heart murmur. Cardiac echocardiography demonstrating vegetations with negative blood cultures confirms the diagnosis of ME. An unelevated ESR may occur with a variety of infectious and noninfectious disorders, but an elevated ESR in the setting of a fever and heart murmur essentially rules out ME from further diagnostic consideration. Quadrivalvular IE is rare, but quadrivalvular ME has not been reported.17-20 To the best of our knowledge, we believe this to be the first case of autopsy-proven quadrivalvular ME with aortic, pulmonary artery, and atrial involvement. This case illustrates the differential diagnostic features of IE versus ME.

REFERENCES 1. Waller BF, Knapp WS, Edwards JE. Marantic valvular vegetations. Circulation 1973;48:644-50.

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2. Olney BA, Schattenberg TT, Campbell JK, Okazaki H, Lie JT. The consequences of the inconsequential: marantic (nonbacterial thrombotic) endocarditis. Am Heart J 1979;98: 513-22. 3. Deppisch LM, Fayemi AO. Non-bacterial thrombotic endocarditis: clinicopathologic correlations. Am Heart J 1976;92: 723-9. 4. Geyer SJ, Franzini DA. Myxomatous degeneration of the mitral valve complicated by non-bacterial thrombotic endocarditis with systemic embolization. Am J Clin Pathol 1979;72: 489-92. 5. Borowski A, Ghodsizad A, Cohnen M, Gams E. Recurrent embolism in the course of marantic endocarditis. Ann Thorac Surg 2005;79:2145-7. 6. Cunha BA, Gill MV, Lazar JM. Acute infective endocarditis: diagnostic and therapeutic approach. Infect Dis Clin North Am 1996;10:811-34. 7. Gearty J, Kiely H, McCaughey WT. Infective and non-bacterial thrombotic endocarditis: experience in recent autopsy material. J Ir Med Assoc 1972;65:439. 8. Reisner SA, Brenner B, Haim N, Edoute X, Markiewicz W. Echocardiography in non-bacterial thrombotic endocarditis: from autopsy to clinical entity. J Am Soc Echocardiogr 2000; 13:876-81. 9. Hesselink DA, Van der Klooster JM, Schelfhart LS, Scheffer MG. Non-bacterial thrombotic (marantic) endocarditis associated with giant cell arteritis. Eur J Intern Med 2001;12: 454-8. 10. Mandell BF. Cardiovascular involvement in systemic lupus erythematosus. Semin Arthritis Rheum 1987;17:126-41. 11. Doherty NE, Siege RJ. Cardiovascular manifestations of systemic lupus erythematosus. Am J Cardiovasc Pathol 1987;1: 141-4. 12. Ansari A, Alrson PH, Bates HD. Cardiovascular manifestations of systemic lupus erythematosus: current perspective. Prog Cardiovasc Dis 1985;27:421-34. 13. Ferrans VJ, Rodriguez ER. Cardiovascular lesions in collagen-vascular diseases. Heart Vessels Suppl 1985;1: 256-61.

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Quadrivalvular marantic endocarditis mimicking ABE 14. Rose AG, Spracklen FH. Double valve disease in systemic lupus erythematosus. Case report and literature review. Am J Cardiovasc Pathol 1987;1:141-4. 15. Hojnik M, George J, Ziporen L, Schoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93:1579-87. 16. Tomimoto S, Ito S, Suzuki T, Mishama A, Suzumura H, Takeda Y, et al. Development of non-bacterial thrombotic endocarditis after percutaneous transvenous mitral commissurotomy for severely calcified mitral stenosis. Jpn Heart J 2000;411-16.

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Durie et al 17. Farrer W. Four-valve endocarditis caused by Corynebacterium CDC Group I1. South Med J 1987;80:923-5. 18. Hobbs RD, Downing SE, Andriole VT. Four-valve polymicrobial endocarditis caused by Pseudomonas aeruginosa and Serratia marcescens. Am J Med 1982;72:164-8. 19. Deonarine B, Lazar J, Gill MV, Cunha BA. Quadri-valvular endocarditis caused by Streptococcus mutans. Clin Microbiol Infect 1997;3:139-41. 20. Kim N, Lazar JM, Cunha BA, Liao W, Minnaganti V. Multivalvular endocarditis. Clin Micribiol Infect 2000;6:207-12.

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