Nonbacterial thrombotic endocarditis
- Kenneth A Bauer, MD
Kenneth A Bauer, MD
- Professor of Medicine
- Harvard Medical School
Nonbacterial thrombotic endocarditis (NBTE) is a rare condition that refers to a spectrum of noninfectious lesions of the heart valves that is most commonly seen in advanced malignancy. NBTE is often an autopsy finding. However, some patients are diagnosed antemortem presenting with the signs and/or symptoms of systemic embolization and require therapy.
The epidemiology, pathogenesis, clinical presentation, evaluation, diagnosis, and treatment of NBTE are presented in this topic. The clinical manifestations and treatment of infective and culture negative endocarditis are discussed separately. (See "Epidemiology, microbiology, and diagnosis of culture-negative endocarditis" and "Antimicrobial therapy of native valve endocarditis" and "Clinical manifestations and evaluation of adults with suspected native valve endocarditis".)
Nonbacterial thrombotic endocarditis (NBTE) is a rare condition most often found postmortem with rates in autopsy series ranging from 0.9 to 1.6 percent [1-6]. It has been reported in every age group, most commonly affecting patients between the fourth and eighth decades of life with no sex predilection [1,7-9]. Patients with advanced malignancy and those with systemic lupus erythematosus are the most common populations affected by NBTE.
One autopsy series reported that, compared to the general population, patients with underlying malignancy have a higher rate of NBTE (1.25 versus 0.2 percent) [2,3]. When compared to other malignancies, higher rates were reported in those with adenocarcinoma (eg, lung, colon, ovary, biliary and prostate) (2.7 versus 0.47 percent) with the highest rates observed in patients with mucin-secreting and pancreatic adenocarcinoma (10 percent) [3,7,10].
In patients with systemic lupus erythematosus, observational studies using transthoracic echocardiography have reported prevalence rates of 6 to 11 percent, with higher rates (43 percent) observed when the more sensitive transesophageal echocardiography was performed [11,12]. (See "Non-coronary cardiac manifestations of systemic lupus erythematosus in adults", section on 'Verrucous endocarditis (Libman-Sacks endocarditis)'.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Deppisch LM, Fayemi AO. Non-bacterial thrombotic endocarditis: clinicopathologic correlations. Am Heart J 1976; 92:723.
- Rosen P, Armstrong D. Nonbacterial thrombotic endocarditis in patients with malignant neoplastic diseases. Am J Med 1973; 54:23.
- González Quintela A, Candela MJ, Vidal C, et al. Non-bacterial thrombotic endocarditis in cancer patients. Acta Cardiol 1991; 46:1.
- Steiner I. [Nonbacterial thrombotic endocarditis--a study of 171 case reports]. Cesk Patol 1993; 29:58.
- Llenas-García J, Guerra-Vales JM, Montes-Moreno S, et al. [Nonbacterial thrombotic endocarditis: clinicopathologic study of a necropsy series]. Rev Esp Cardiol 2007; 60:493.
- Eiken PW, Edwards WD, Tazelaar HD, et al. Surgical pathology of nonbacterial thrombotic endocarditis in 30 patients, 1985-2000. Mayo Clin Proc 2001; 76:1204.
- el-Shami K, Griffiths E, Streiff M. Nonbacterial thrombotic endocarditis in cancer patients: pathogenesis, diagnosis, and treatment. Oncologist 2007; 12:518.
- Mazokopakis EE, Syros PK, Starakis IK. Nonbacterial thrombotic endocarditis (marantic endocarditis) in cancer patients. Cardiovasc Hematol Disord Drug Targets 2010; 10:84.
- Lopez JA, Ross RS, Fishbein MC, Siegel RJ. Nonbacterial thrombotic endocarditis: a review. Am Heart J 1987; 113:773.
- Borowski A, Ghodsizad A, Cohnen M, Gams E. Recurrent embolism in the course of marantic endocarditis. Ann Thorac Surg 2005; 79:2145.
- Roldan CA, Shively BK, Crawford MH. An echocardiographic study of valvular heart disease associated with systemic lupus erythematosus. N Engl J Med 1996; 335:1424.
- Roldan CA, Qualls CR, Sopko KS, Sibbitt WL Jr. Transthoracic versus transesophageal echocardiography for detection of Libman-Sacks endocarditis: a randomized controlled study. J Rheumatol 2008; 35:224.
- Aryana A, Esterbrooks DJ, Morris PC. Nonbacterial thrombotic endocarditis with recurrent embolic events as manifestation of ovarian neoplasm. J Gen Intern Med 2006; 21:C12.
- Ferrans VJ, Rodríguez ER. Cardiovascular lesions in collagen-vascular diseases. Heart Vessels Suppl 1985; 1:256.
- Wada H, Sase T, Yamaguchi M. Hypercoagulant states in malignant lymphoma. Exp Oncol 2005; 27:179.
- Hughson MD, McCarty GA, Sholer CM, Brumback RA. Thrombotic cerebral arteriopathy in patients with the antiphospholipid syndrome. Mod Pathol 1993; 6:644.
- Sharma S, Mayberry JC, Deloughery TG, Mullins RJ. Fatal cerebroembolism from nonbacterial thrombotic endocarditis in a trauma patient: case report and review. Mil Med 2000; 165:83.
- Roldan CA, Sibbitt WL Jr, Qualls CR, et al. Libman-Sacks endocarditis and embolic cerebrovascular disease. JACC Cardiovasc Imaging 2013; 6:973.
- Graus F, Rogers LR, Posner JB. Cerebrovascular complications in patients with cancer. Medicine (Baltimore) 1985; 64:16.
- Johnson JA, Everett BM, Katz JT, Loscalzo J. Clinical problem-solving. Painful purple toes. N Engl J Med 2010; 362:67.
- Taniyama D, Yamamoto R, Kawasaki M, et al. Nonbacterial thrombotic endocarditis leading to acute heart failure due to aortic stenosis in a patient with lung cancer. Intern Med 2013; 52:1617.
- Bhimani AA, Hoit BD. Extensive nonbacterial thrombotic endocarditis isolated to the tricuspid valve in primary antiphospholipid syndrome. J Am Soc Echocardiogr 2010; 23:107.e5.
- Strauss HW, Nunn A, Linder K. Nitroimidazoles for imaging hypoxic myocardium. J Nucl Cardiol 1995; 2:437.
- Nuñez M, Peinado-Onsurbe J, Vilaró S, Llobera M. Lipoprotein lipase activity in developing rat brain areas. Biol Neonate 1995; 68:119.
- Kurdi M, Beanlands DS, Chan KL, Veinot JP. Nonbacterial thrombotic endocarditis presenting as aortic stenosis with suspected infective endocarditis: clinicopathological correlation. Can J Cardiol 2004; 20:549.
- Singhal AB, Topcuoglu MA, Buonanno FS. Acute ischemic stroke patterns in infective and nonbacterial thrombotic endocarditis: a diffusion-weighted magnetic resonance imaging study. Stroke 2002; 33:1267.
- Biller J, Challa VR, Toole JF, Howard VJ. Nonbacterial thrombotic endocarditis. A neurologic perspective of clinicopathologic correlations of 99 patients. Arch Neurol 1982; 39:95.
- Reagan TJ, Okazaki H. The thrombotic syndrome associated with carcinoma. A clinical and neuropathologic study. Arch Neurol 1974; 31:390.
- de Bruijn SF, Agema WR, Lammers GJ, et al. Transesophageal echocardiography is superior to transthoracic echocardiography in management of patients of any age with transient ischemic attack or stroke. Stroke 2006; 37:2531.
- Dutta T, Karas MG, Segal AZ, Kizer JR. Yield of transesophageal echocardiography for nonbacterial thrombotic endocarditis and other cardiac sources of embolism in cancer patients with cerebral ischemia. Am J Cardiol 2006; 97:894.
- Whitlock RP, Sun JC, Fremes SE, et al. Antithrombotic and thrombolytic therapy for valvular disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 2012; 141:e576S.
- Rogers LR, Cho ES, Kempin S, Posner JB. Cerebral infarction from non-bacterial thrombotic endocarditis. Clinical and pathological study including the effects of anticoagulation. Am J Med 1987; 83:746.
- Rabinstein AA, Giovanelli C, Romano JG, et al. Surgical treatment of nonbacterial thrombotic endocarditis presenting with stroke. J Neurol 2005; 252:352.
- Ong ML, Veerapen K, Chambers JB, et al. Cardiac abnormalities in systemic lupus erythematosus: prevalence and relationship to disease activity. Int J Cardiol 1992; 34:69.