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Eosinophilic Endocarditis and Strongyloides stercoralis
Eosinophilic Endocarditis and Strongyloides stercoralis Jeremy Thaden, MD*, Andrew Cassar, MD, Brianna Vaa, MD, Sabrina Phillips, MD, Harold Burkhart, MD, Marie Aubry, MD, and Rick Nishimura, MD A 40-year-old woman from El Salvador presented with 3 months of abdominal pain and diarrhea followed by 2 weeks of atypical chest pain and exertional dyspnea and was diagnosed with eosinophilic endocarditis secondary to Strongyloides stercoralis infection. Transthoracic echocardiogram revealed apical masses in the left and right ventricles and a thickened posterior mitral valve leaflet and cardiac magnetic resonance imaging confirmed the presence of a left ventricular apical mass with diffuse subendocardial delayed enhancement consistent with endocardial fibrosis. In conclusion, eosinophilic endocarditis is a rare cause of restrictive cardiomyopathy characterized by endomyocardial fibrosis and apical thrombosis and fibrosis with frequent involvement of the posterior mitral valve leaflet. Ó 2013 Elsevier Inc. All rights reserved. (Am J Cardiol 2013;112:461e462)
Mayo Clinic, Rochester, Minnesota. Manuscript received February 23, 2013; revised manuscript received and accepted March 24, 2013. See page 462 for disclosure information. *Corresponding author: Tel: (507) 284-3545; fax: (507) 284-8295. E-mail address: [email protected] (J. Thaden).
Eosinophilic endocarditis (Loeffler endocarditis) is an uncommon cause of restrictive cardiomyopathy resulting from endomyocardial thrombosis and fibrosis as a result of toxins released by infiltrating eosinophils. It is classically thought to be comprised of 3 stages: the necrotic
Figure 1. Transthoracic echocardiogrpahy, parasternal short axis (A) and apical 4 chamber (B) showing apical thrombus and cavity obliteration. Transmitral Doppler (C) demonstrates restrictive filling. Steady state free procession cardiac MRI (D) and short axis delayed enhancement cardiac MRI (E) reveal a relatively normal outer layer of myocardium, a subendocardial rim of delayed enhancement (E, arrows), and a nonenhancing endocardial thrombus (D, arrows). 0002-9149/13/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjcard.2013.03.053
www.ajconline.org
462
The American Journal of Cardiology (www.ajconline.org)
stage, the thrombotic stage, and the fibrotic stage. We describe typical echocardiographic and cardiac magnetic resonance imaging (MRI) findings in a patient with eosinophilic endocarditis related to Strongyloides stercoralis infection. Case Description A 40-year-old woman from El Salvador presented with 3 months of abdominal pain, diarrhea, nausea, vomiting, and weight loss followed by 2 weeks of atypical chest pain and exertional dyspnea. Initial chest x-ray revealed cardiomegaly and mild pulmonary edema. Further workup revealed a peripheral eosinophilia of 2.2 109/L (normal 0.05 to 0.50 109/L), a troponin I of 0.31 mg/L, and an electrocardiogram consistent with left ventricular (LV) hypertrophy. Transthoracic echocardiogram showed a large mass in the LV apex and a small mass in the right ventricular apex (Figure 1; Supplemental Videos 1, 2). The posterior mitral valve leaflet was thickened and had reduced mobility (Figure 1), resulting in significant mitral regurgitation. Doppler interrogation revealed a mitral inflow pattern consistent with restrictive filling (Figure 1) and an estimated right ventricular systolic pressure of 69 mm Hg. Cardiac MRI confirmed a 4.3 3.4 3.5 cm nonenhancing mass in the LV apex (Figure 1; Supplemental Video 3) and diffuse subendocardial delayed enhancement (Figure 1) with increased signal on edema and diffusion weighted sequences. Given the previously mentioned findings and her previous residence in El Salvador there was a suspicion for eosinophilic endocarditis related to parasitic infection. Serologies and subsequent stool cultures confirmed active infection with Strongyloides stercoralis. She was treated with ivermectin and started on warfarin for prevention of systemic embolization. Unfortunately, 3 weeks after treatment with ivermectin and therapeutic anticoagulation her dyspnea persisted and the LV mass remained unchanged on repeat echocardiography. Because of her persistent symptoms and the significant burden of thrombus and fibrosis in the LV she underwent surgical excision of right ventricular and LV thrombus and fibrosis with concurrent mitral valve replacement. Intraoperatively, extensive thrombosis and fibrosis of the LV cavity with involvement of both papillary muscles and both mitral valve leaflets was noted. Pathology revealed endocardial fibrosis with organizing and degenerating mural thrombus associated with a lymphoplasmacytic infiltrate extending into myocardium. Eosinophilic infiltration likely resolved in the setting of previous medical therapy, leaving residual postinflammatory fibrosis. Comments Eosinophilic endocarditis is classically comprised of 3 stages: the necrotic stage, the thrombotic stage, and the
fibrotic stage. It is thought that eosinophilic degranulation and release of major basic protein and cationic proteins lead to myocyte injury.1e3 Ongoing eosinophilic infiltration and degranulation leads to thrombosis followed by fibrosis of the endocardium resulting in a restrictive cardiomyopathy. Fibrosis typically involves the left and right ventricular apices and may extend to the posterior wall of the left ventricle, chordae tendinea, and the posterior mitral leaflet.4 Noninvasive testing relies largely on echocardiography and MRI. Echocardiography may be normal in the early stages of disease but later may show evidence of left or right ventricular apical thrombosis and cavity obliteration as was seen quite dramatically in our patient. Frequently, there is involvement of the posterior mitral valve leaflet and the chordae tendinea, which may result in significant mitral valve regurgitation.4 The aortic valve is spared in most cases. Doppler echocardiography may reveal evidence of restrictive filling. When the diagnosis is in question, contrast-enhanced MRI can confirm evidence of active myocarditis and subendocardial fibrosis. The MRI findings have been described as a “3-layered” appearance, which is well illustrated in our patient. The outer layer consists of relatively hypointense normal myocardium. The middle layer may show hyperenhancement with gadolinium, increased signal on T2-weighted sequences and increased signal on delayed enhancement sequences secondary to inflammation or fibrosis, depending on the stage of disease. The innermost layer consists of nonenhancing endocardial thrombus.5
Disclosures The authors have no conflicts of interest to disclose.
Supplementary Data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/ j.amjcard.2013.03.053. 1. Moosbauer C, Morgenstern E, Cuvelier SL, Manukyan D, Bidzhekov K, Albrecht S, Lohse P, Patel KD, Engelmann B. Eosinophils are a major intravascular location for tissue factor storage and exposure. Blood 2007;109:995e1002. 2. Tai PC, Ackerman SJ, Spry CJ, Dunnette S, Olsen EG, Gleich GJ. Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease. Lancet 1987;1:643e647. 3. Ogbogu PU, Rosing DR, Horne MK III. Cardiovascular manifestations of hypereosinophilic syndromes. Immunol Allergy Clin N Am 2007;27: 457e475. 4. Ommen SR, Seward JB, Tajik AJ. Clinical and echocardiographic features of hypereosinophilic syndromes. Am J Cardiol 2000;86:110e113. 5. Syed IS, Martinez MW, Feng DL, Glockner JF. Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease. Int J Cardiol 2008;126:e50ee52.
Mayo Clinic, Rochester, Minnesota. Manuscript received February 23, 2013; revised manuscript received and accepted March 24, 2013. See page 462 for disclosure information. *Corresponding author: Tel: (507) 284-3545; fax: (507) 284-8295. E-mail address: [email protected] (J. Thaden).
Eosinophilic endocarditis (Loeffler endocarditis) is an uncommon cause of restrictive cardiomyopathy resulting from endomyocardial thrombosis and fibrosis as a result of toxins released by infiltrating eosinophils. It is classically thought to be comprised of 3 stages: the necrotic
Figure 1. Transthoracic echocardiogrpahy, parasternal short axis (A) and apical 4 chamber (B) showing apical thrombus and cavity obliteration. Transmitral Doppler (C) demonstrates restrictive filling. Steady state free procession cardiac MRI (D) and short axis delayed enhancement cardiac MRI (E) reveal a relatively normal outer layer of myocardium, a subendocardial rim of delayed enhancement (E, arrows), and a nonenhancing endocardial thrombus (D, arrows). 0002-9149/13/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjcard.2013.03.053
www.ajconline.org
462
The American Journal of Cardiology (www.ajconline.org)
stage, the thrombotic stage, and the fibrotic stage. We describe typical echocardiographic and cardiac magnetic resonance imaging (MRI) findings in a patient with eosinophilic endocarditis related to Strongyloides stercoralis infection. Case Description A 40-year-old woman from El Salvador presented with 3 months of abdominal pain, diarrhea, nausea, vomiting, and weight loss followed by 2 weeks of atypical chest pain and exertional dyspnea. Initial chest x-ray revealed cardiomegaly and mild pulmonary edema. Further workup revealed a peripheral eosinophilia of 2.2 109/L (normal 0.05 to 0.50 109/L), a troponin I of 0.31 mg/L, and an electrocardiogram consistent with left ventricular (LV) hypertrophy. Transthoracic echocardiogram showed a large mass in the LV apex and a small mass in the right ventricular apex (Figure 1; Supplemental Videos 1, 2). The posterior mitral valve leaflet was thickened and had reduced mobility (Figure 1), resulting in significant mitral regurgitation. Doppler interrogation revealed a mitral inflow pattern consistent with restrictive filling (Figure 1) and an estimated right ventricular systolic pressure of 69 mm Hg. Cardiac MRI confirmed a 4.3 3.4 3.5 cm nonenhancing mass in the LV apex (Figure 1; Supplemental Video 3) and diffuse subendocardial delayed enhancement (Figure 1) with increased signal on edema and diffusion weighted sequences. Given the previously mentioned findings and her previous residence in El Salvador there was a suspicion for eosinophilic endocarditis related to parasitic infection. Serologies and subsequent stool cultures confirmed active infection with Strongyloides stercoralis. She was treated with ivermectin and started on warfarin for prevention of systemic embolization. Unfortunately, 3 weeks after treatment with ivermectin and therapeutic anticoagulation her dyspnea persisted and the LV mass remained unchanged on repeat echocardiography. Because of her persistent symptoms and the significant burden of thrombus and fibrosis in the LV she underwent surgical excision of right ventricular and LV thrombus and fibrosis with concurrent mitral valve replacement. Intraoperatively, extensive thrombosis and fibrosis of the LV cavity with involvement of both papillary muscles and both mitral valve leaflets was noted. Pathology revealed endocardial fibrosis with organizing and degenerating mural thrombus associated with a lymphoplasmacytic infiltrate extending into myocardium. Eosinophilic infiltration likely resolved in the setting of previous medical therapy, leaving residual postinflammatory fibrosis. Comments Eosinophilic endocarditis is classically comprised of 3 stages: the necrotic stage, the thrombotic stage, and the
fibrotic stage. It is thought that eosinophilic degranulation and release of major basic protein and cationic proteins lead to myocyte injury.1e3 Ongoing eosinophilic infiltration and degranulation leads to thrombosis followed by fibrosis of the endocardium resulting in a restrictive cardiomyopathy. Fibrosis typically involves the left and right ventricular apices and may extend to the posterior wall of the left ventricle, chordae tendinea, and the posterior mitral leaflet.4 Noninvasive testing relies largely on echocardiography and MRI. Echocardiography may be normal in the early stages of disease but later may show evidence of left or right ventricular apical thrombosis and cavity obliteration as was seen quite dramatically in our patient. Frequently, there is involvement of the posterior mitral valve leaflet and the chordae tendinea, which may result in significant mitral valve regurgitation.4 The aortic valve is spared in most cases. Doppler echocardiography may reveal evidence of restrictive filling. When the diagnosis is in question, contrast-enhanced MRI can confirm evidence of active myocarditis and subendocardial fibrosis. The MRI findings have been described as a “3-layered” appearance, which is well illustrated in our patient. The outer layer consists of relatively hypointense normal myocardium. The middle layer may show hyperenhancement with gadolinium, increased signal on T2-weighted sequences and increased signal on delayed enhancement sequences secondary to inflammation or fibrosis, depending on the stage of disease. The innermost layer consists of nonenhancing endocardial thrombus.5
Disclosures The authors have no conflicts of interest to disclose.
Supplementary Data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/ j.amjcard.2013.03.053. 1. Moosbauer C, Morgenstern E, Cuvelier SL, Manukyan D, Bidzhekov K, Albrecht S, Lohse P, Patel KD, Engelmann B. Eosinophils are a major intravascular location for tissue factor storage and exposure. Blood 2007;109:995e1002. 2. Tai PC, Ackerman SJ, Spry CJ, Dunnette S, Olsen EG, Gleich GJ. Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease. Lancet 1987;1:643e647. 3. Ogbogu PU, Rosing DR, Horne MK III. Cardiovascular manifestations of hypereosinophilic syndromes. Immunol Allergy Clin N Am 2007;27: 457e475. 4. Ommen SR, Seward JB, Tajik AJ. Clinical and echocardiographic features of hypereosinophilic syndromes. Am J Cardiol 2000;86:110e113. 5. Syed IS, Martinez MW, Feng DL, Glockner JF. Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease. Int J Cardiol 2008;126:e50ee52.