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Endocarditis Due to Tropheryma Whipplei
Letters to the Editor
aorta, patent mammary artery grafts, or a technically complex resternotomy. Rocío Casais Pampín,a Francisco Calvo Iglesias,b José Antonio Baz Alonso,c and Gonzalo Pradas Montillaa
Servicio de Cirugía Cardiaca, Hospital do Meixoeiro, Vigo, Pontevedra, Spain b Servicio Cardiología, Hospital do Meixoeiro, Vigo, Pontevedra, Spain c Servicio Hemodinámica, Hospital do Meixoeiro, Vigo, Pontevedra, Spain a
REFERENCES
1. Walther T, Falk V, Borger MA, Dewey T, Wimmer-Greinecker G, Schuler G, et al. Minimally invasive transapical beating heart aortic valve implantation — proof of concept. Eur J Cardiothorac Surg. 2007;31:9-15. 2. Lichtenstein SV, Cheung A, Ye J, Thompson CR, Carere RG, Pasupati S, et al. Transapical transcatheter aortic valve implantation in humans: Initial clinical experience. Circulation. 2006;114:591-6. 3. Klaaborg KE, Egeblad H, Jakobsen CJ, Terp K, Lindskov C, Andersen HR, et al. Transapical transcatheter treatment of a stenosed aortic valve bioprosthesis using the Edwards SAPIEN transcatheter heart valve. Ann Thorac Surg. 2009;87:1943-6. 4. Walther T, Falk V, Dewey T, Kempfert J, Emrich F, Pfannmüller B, et al. Valve-in-a-valve concept for transcatheter minimally invasive repeat xenograft implantation. J Am Coll Cardiol. 2007;50:56-60. 5. Walther T, Dewey T, Borger MA, Kempfert J, Linke A, Becht R, et al. Transapical aortic valve implantation: step by step. Ann Thorac Surg. 2009;87:276-83.
Endocarditis Due to Tropheryma Whipplei To the Editor, Although 20%-55% of people with Whipple’s disease present cardiac involvement,1 the episodes of endocarditis due to Tropheryma whipplei are uncommon,2-5 and thus this agent is rarely taken into account during etiologic differential diagnosis when endocarditis is suspected but hemocultures are negative. We present a patient treated in our hospital, where the suspected initial diagnosis was infective endocarditis (IE) with hemocultures negative for papillary fibroelastoma on the native aortic valve. Endocarditis due to T. whipplei was later diagnosed by PCR analysis. This 57-year-old woman without known heart disease has a prolonged history of polyarthralgias, pretibial pain and peripheral neuropathy classified as palindromic rheumatism, long-term hypertransaminasemia, chronic cough with negative functional and allergy tests, transient episodes of diplopia and frequent headaches, without diarrhea 250 Rev Esp Cardiol. 2010;63(2):240-52
Figure 1. Transesophageal echocardiogram showing a 1.2 cm mass from the aortic valve.
or other clinical digestive disorders, and had received oral corticoid treatment only. She was admitted to the cardiology unit due to congestive heart failure. During examination she had no fever, but did present enlarged latero-cervical lymph nodes and grade II/ IV diastolic murmur in the right parasternal border. The echocardiogram showed a 1.2-cm mass on the left coronary aortic leaflet that caused severe aortic regurgitation (Figure 1). Hemocultures were taken but the results were negative. Given the suspicion of IE with negative hemoculture for papillary fibroelastoma, surgical treatment was prescribed. During the intraoperative phase, large fibrous adhesions were found in the pericardium and a mass with a polypoid appearance (1.2×0.5 cm) was confirmed in the aortic face of the left coronary leaflet (Figure 2). The valve was resected and replaced with a mechanical prosthesis.
Figure 2. Polypoid mass on the left coronary leaflet of the aortic valve.
Letters to the Editor
No microorganisms were found in the pericardial and aortic valve cultures, but a subsequent general PCR analysis isolated T whipplei in pericardial and valvular tissue. Given the diagnosis of T whipplei endocarditis, antibiotic treatment with ceftriaxone IV was given for 1 week, followed by oral trimethoprim-sulfamethoxazole. The patient was admitted 1 month after hospital discharge for nausea and food-associated vomiting due to prerenal acute kidney failure. At a subsequent check-up the patient demonstrated functional class I, with a normally functioning prosthesis and substantial improvement in lumbar and ankle pain; the only persistent problem was intermittent right-knee pain. The patient does not present diarrhea or other digestive clinical symptoms. Currently, she is still receiving antibiotic treatment with oral cotrimoxazole that will be maintained for at least 18 months. The first case of her diagnosed disease was described in 1907 by Whipple,6 who called it intestinal lipodystrophy. In 1952 the first effective guideline of antibiotic treatment was achieved. In 2000, the causal bacillus was successfully isolated and cultivated and in 2003 the full sequence of the genome was analyzed. The main symptoms consist of arthralgias, weight loss, diarrhea and abdominal pain, although other parts of the body can also be affected, such as the central nervous system, lungs, and heart. The incidence of this disorder is around 30 new cases per year. It is more common in men (86%) than in women.7 If treatment is not received, the disease is lethal. There is cardiac involvement in 20%-55% of the cases1 of the Whipple’s disease and can affect all the layers of the heart. It can occur with thoracic pain and pericardial friction, interstitial myocarditis and endocarditis, and is more common on the mitral valve than on the aortic valve. Endocarditis due to T whipplei has also been described on a mitral valve bioprosthesis.8 Treatment consists in valve replacement and longterm antibiotic treatment (around 1-2 years) with trimethoprim-sulfamethoxazole; no recurrences have been described. Javier Miguelena, Rafael Muñoz, Ramón Maseda, and Antonio Epeldegui
Servicio de Cirugía Cardiaca Adultos, Hospital Ramón y Cajal, Madrid, Spain
REFERENCES
1. McAllister HA Jr, Fenoglio JJ Jr. Cardiac involvement in Whipple’s disease. Circulation. 1975;52:152-6. 2. Aïouaz H, Célard M, Puget M, Vandenesch F, Mercusot A, Fenollar F, et al. Whipple’s disease endocarditis: report of 5 cases and review of the literature. Rev Med Interne. 2005;26:78490.
3. Marrakchi C, Abdennadher M, Blin D. Endocarditis caused by Tropheryma whippelii. Tunis Med. 2004;82:781-4. 4. Schneider T, Salamon-Looijen M, Von Herbay A, Schwerdt H, Weg-Remers S, Stallmach A, et al. Whipple’s disease with aortic regurgitation requiring aortic valve replacement. Infection. 1998;26:178-80. 5 Khairy P, Graham AF. Whipple’s disease and the heart. Can J Cardiol. 1996;12:831-4. 6. Whipple GH. A hither to undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Bull Johns Hopkins Hosp. 1907;18:382-91. 7. Dobbins WO. Whipple’s disease. Mayo Clin Proc. 1988;63:6234. 8. Ratliff NB, McMahon JT, Naab TJ, Cosgrove DM. Whipple’s disease in the porcine leaflets of a Carpentier-Edwards prosthetic mitral valve. N Engl J Med. 1984;311:902-3.
My Right to Reply: Inaccurate References To the Editor, Recently, this Journal published a “Letter to the Editor1” referring to an article published by my research group.2 The letter expressed regret for the lack of rigor in the references. When I learned about this letter while reading in the Journal, I was shocked. Regarding the essence of the letter, I can do nothing but accept the criticism and apologize. The software for dealing with references is far from perfect and I should have reviewed the references one by one. In any case, the zeal of José L. Zambrana seems excessive when he includes “Sunol” as an error. Although I love the letter eñe and consider it something specifically Spanish, the article by Suñol was published in an English journal and the author appears as “Sunol.” The responsibility José L. Zambrana attributes to the referees is debatable. Their task of substantially improving the articles in an altruistic way is already sufficient. Perhaps someone dedicated to reviewing references could be considered in a journal of this standing. The problem of errors in references has been widely addressed in the literature. The percentage of errors in highly prestigious scientific journals is around 30%,3-5 and in some cases it reaches 56%.6 Even authors as sensitive to this issue as José L. Zambrana lie within these ranges, as can be appreciated by reviewing the references in one of their articles mentioned in his letter.7 José A. San Román
Servicio de Cardiología, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
Rev Esp Cardiol. 2010;63(2):240-52 251
aorta, patent mammary artery grafts, or a technically complex resternotomy. Rocío Casais Pampín,a Francisco Calvo Iglesias,b José Antonio Baz Alonso,c and Gonzalo Pradas Montillaa
Servicio de Cirugía Cardiaca, Hospital do Meixoeiro, Vigo, Pontevedra, Spain b Servicio Cardiología, Hospital do Meixoeiro, Vigo, Pontevedra, Spain c Servicio Hemodinámica, Hospital do Meixoeiro, Vigo, Pontevedra, Spain a
REFERENCES
1. Walther T, Falk V, Borger MA, Dewey T, Wimmer-Greinecker G, Schuler G, et al. Minimally invasive transapical beating heart aortic valve implantation — proof of concept. Eur J Cardiothorac Surg. 2007;31:9-15. 2. Lichtenstein SV, Cheung A, Ye J, Thompson CR, Carere RG, Pasupati S, et al. Transapical transcatheter aortic valve implantation in humans: Initial clinical experience. Circulation. 2006;114:591-6. 3. Klaaborg KE, Egeblad H, Jakobsen CJ, Terp K, Lindskov C, Andersen HR, et al. Transapical transcatheter treatment of a stenosed aortic valve bioprosthesis using the Edwards SAPIEN transcatheter heart valve. Ann Thorac Surg. 2009;87:1943-6. 4. Walther T, Falk V, Dewey T, Kempfert J, Emrich F, Pfannmüller B, et al. Valve-in-a-valve concept for transcatheter minimally invasive repeat xenograft implantation. J Am Coll Cardiol. 2007;50:56-60. 5. Walther T, Dewey T, Borger MA, Kempfert J, Linke A, Becht R, et al. Transapical aortic valve implantation: step by step. Ann Thorac Surg. 2009;87:276-83.
Endocarditis Due to Tropheryma Whipplei To the Editor, Although 20%-55% of people with Whipple’s disease present cardiac involvement,1 the episodes of endocarditis due to Tropheryma whipplei are uncommon,2-5 and thus this agent is rarely taken into account during etiologic differential diagnosis when endocarditis is suspected but hemocultures are negative. We present a patient treated in our hospital, where the suspected initial diagnosis was infective endocarditis (IE) with hemocultures negative for papillary fibroelastoma on the native aortic valve. Endocarditis due to T. whipplei was later diagnosed by PCR analysis. This 57-year-old woman without known heart disease has a prolonged history of polyarthralgias, pretibial pain and peripheral neuropathy classified as palindromic rheumatism, long-term hypertransaminasemia, chronic cough with negative functional and allergy tests, transient episodes of diplopia and frequent headaches, without diarrhea 250 Rev Esp Cardiol. 2010;63(2):240-52
Figure 1. Transesophageal echocardiogram showing a 1.2 cm mass from the aortic valve.
or other clinical digestive disorders, and had received oral corticoid treatment only. She was admitted to the cardiology unit due to congestive heart failure. During examination she had no fever, but did present enlarged latero-cervical lymph nodes and grade II/ IV diastolic murmur in the right parasternal border. The echocardiogram showed a 1.2-cm mass on the left coronary aortic leaflet that caused severe aortic regurgitation (Figure 1). Hemocultures were taken but the results were negative. Given the suspicion of IE with negative hemoculture for papillary fibroelastoma, surgical treatment was prescribed. During the intraoperative phase, large fibrous adhesions were found in the pericardium and a mass with a polypoid appearance (1.2×0.5 cm) was confirmed in the aortic face of the left coronary leaflet (Figure 2). The valve was resected and replaced with a mechanical prosthesis.
Figure 2. Polypoid mass on the left coronary leaflet of the aortic valve.
Letters to the Editor
No microorganisms were found in the pericardial and aortic valve cultures, but a subsequent general PCR analysis isolated T whipplei in pericardial and valvular tissue. Given the diagnosis of T whipplei endocarditis, antibiotic treatment with ceftriaxone IV was given for 1 week, followed by oral trimethoprim-sulfamethoxazole. The patient was admitted 1 month after hospital discharge for nausea and food-associated vomiting due to prerenal acute kidney failure. At a subsequent check-up the patient demonstrated functional class I, with a normally functioning prosthesis and substantial improvement in lumbar and ankle pain; the only persistent problem was intermittent right-knee pain. The patient does not present diarrhea or other digestive clinical symptoms. Currently, she is still receiving antibiotic treatment with oral cotrimoxazole that will be maintained for at least 18 months. The first case of her diagnosed disease was described in 1907 by Whipple,6 who called it intestinal lipodystrophy. In 1952 the first effective guideline of antibiotic treatment was achieved. In 2000, the causal bacillus was successfully isolated and cultivated and in 2003 the full sequence of the genome was analyzed. The main symptoms consist of arthralgias, weight loss, diarrhea and abdominal pain, although other parts of the body can also be affected, such as the central nervous system, lungs, and heart. The incidence of this disorder is around 30 new cases per year. It is more common in men (86%) than in women.7 If treatment is not received, the disease is lethal. There is cardiac involvement in 20%-55% of the cases1 of the Whipple’s disease and can affect all the layers of the heart. It can occur with thoracic pain and pericardial friction, interstitial myocarditis and endocarditis, and is more common on the mitral valve than on the aortic valve. Endocarditis due to T whipplei has also been described on a mitral valve bioprosthesis.8 Treatment consists in valve replacement and longterm antibiotic treatment (around 1-2 years) with trimethoprim-sulfamethoxazole; no recurrences have been described. Javier Miguelena, Rafael Muñoz, Ramón Maseda, and Antonio Epeldegui
Servicio de Cirugía Cardiaca Adultos, Hospital Ramón y Cajal, Madrid, Spain
REFERENCES
1. McAllister HA Jr, Fenoglio JJ Jr. Cardiac involvement in Whipple’s disease. Circulation. 1975;52:152-6. 2. Aïouaz H, Célard M, Puget M, Vandenesch F, Mercusot A, Fenollar F, et al. Whipple’s disease endocarditis: report of 5 cases and review of the literature. Rev Med Interne. 2005;26:78490.
3. Marrakchi C, Abdennadher M, Blin D. Endocarditis caused by Tropheryma whippelii. Tunis Med. 2004;82:781-4. 4. Schneider T, Salamon-Looijen M, Von Herbay A, Schwerdt H, Weg-Remers S, Stallmach A, et al. Whipple’s disease with aortic regurgitation requiring aortic valve replacement. Infection. 1998;26:178-80. 5 Khairy P, Graham AF. Whipple’s disease and the heart. Can J Cardiol. 1996;12:831-4. 6. Whipple GH. A hither to undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Bull Johns Hopkins Hosp. 1907;18:382-91. 7. Dobbins WO. Whipple’s disease. Mayo Clin Proc. 1988;63:6234. 8. Ratliff NB, McMahon JT, Naab TJ, Cosgrove DM. Whipple’s disease in the porcine leaflets of a Carpentier-Edwards prosthetic mitral valve. N Engl J Med. 1984;311:902-3.
My Right to Reply: Inaccurate References To the Editor, Recently, this Journal published a “Letter to the Editor1” referring to an article published by my research group.2 The letter expressed regret for the lack of rigor in the references. When I learned about this letter while reading in the Journal, I was shocked. Regarding the essence of the letter, I can do nothing but accept the criticism and apologize. The software for dealing with references is far from perfect and I should have reviewed the references one by one. In any case, the zeal of José L. Zambrana seems excessive when he includes “Sunol” as an error. Although I love the letter eñe and consider it something specifically Spanish, the article by Suñol was published in an English journal and the author appears as “Sunol.” The responsibility José L. Zambrana attributes to the referees is debatable. Their task of substantially improving the articles in an altruistic way is already sufficient. Perhaps someone dedicated to reviewing references could be considered in a journal of this standing. The problem of errors in references has been widely addressed in the literature. The percentage of errors in highly prestigious scientific journals is around 30%,3-5 and in some cases it reaches 56%.6 Even authors as sensitive to this issue as José L. Zambrana lie within these ranges, as can be appreciated by reviewing the references in one of their articles mentioned in his letter.7 José A. San Román
Servicio de Cardiología, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
Rev Esp Cardiol. 2010;63(2):240-52 251