Ann Thorac Surg 2009;88:2027-2029. doi:10.1016/j.athoracsur.2009.04.141
© 2009 The Society of Thoracic Surgeons
Case Reports
Tricuspid and Aortic Valve and Ventricular Septal Defect Endocarditis: An Unusual Presentation of Acute Q Fever
Sebastian Pagni, MD*,
Anthony Dempsey, MD,
Erle H. Austin, III, MD
Division of Thoracic and Cardiovascular Surgery, Department of Surgery, University of Louisville, Louisville, Kentucky
Accepted for publication April 15, 2009.
* Address correspondence to Dr Pagni, Division of Thoracic and Cardiovascular Surgery, University of Louisville, 201 Abraham Flexner Way, Suite 1200, Louisville, KY 40202 (Email: spagni{at}ucsamd.com).
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Abstract
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Q fever is a rare systemic infection caused by Coxiella Burnetii. The presentation with endocarditis is insidious, with negative blood cultures, and oftentimes it is not obvious in diagnostic imaging studies until hemodynamic changes or valve destruction is reached [1]. We report a case of Q fever endocarditis involving the tricuspid and aortic valves and a congenital ventricular septal defect. Surgical treatment and distinct aspects of this unusual case are herein described.
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Introduction
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Q fever is a worldwide zoonosis caused by Coxiella Burnetii. Farmers and animal caretakers are more susceptible to this rare infection. Blood culture negative endocarditis is typically a common presentation with single valve involvement. Clinical diagnosis and surgical treatment are challenging.
A 39-year-old Caucasian man who is a farmer was referred to our institution with a 3-month history of malaise, fever, chills, and nonproductive cough. The symptoms worsened in the last month, with a new onset of myalgia and right pleuritic chest pain. His medical history revealed a ventricular septal defect (VSD) (Qp:Qs = 1.5:1), but he had not returned for follow-up since age 20. On presentation, his temperature was 37°C, his blood pressure was 120/75 mm Hg, and his heart rate was 80 bpm. His physical examination showed a 4/6 holosystolic murmur, best heard along the left sternal border. There was mild jugular vein distention and no signs of peripheral or cutaneous embolization. A micro-papular rash was noted in the lower extremities. The blood cultures at 5 days were negative and the white count was 6.9. A computed tomographic scan showed few small nodules in the right lung. A transesophageal echocardiogram (TEE) showed severe tricuspid regurgitation with papillary muscle rupture and multiple vegetations (Fig 1). A 1.2-cm vegetation was attached to the endocardium below the septal leaflet. A cardiac catheterism documented a perimembranous VSD with a Qp:Qs of 2.1:1 and pulmonary artery pressures of 43/22 mm Hg. His farming background and atypical presentation prompted serologic assays for rickettsiosis. Serology was positive for Coxiella Burnetti with titers of immunoglobular G phases I and II of 1/64 and positive for phase II immunoglobular M titers. At the operation (Fig 2), all tricuspid valve leaflets and subvalvular apparatus, VSD, and adjacent endocardium were involved with vegetations. Extensive debridement was performed, and the VSD was closed using autologous pericardium and a 31-mm bovine pericardial bioprosthesis (Carpentier-Edwards, Edwards Lifesciences LLC, Irvine, CA) was implanted.
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Fig 1. A 4-chamber transesophageal echocardiographic view shows the involvement of the tricuspid valve and subvalvar apparatus. A large vegetation sits on the atrial side of anterior tricuspid leaflet (arrow).
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Fig 2. (A) Right atrial exposure with a view of the large ventricular septal defect (arrow), anterior leaflet vegetation, and endocardial vegetations after removal of the septal and part of the anterior leaflet of the tricuspid valve (*). (B) Tricuspid valve specimen shows multiple vegetations involving the leaflets, cordi, and septal papillary muscle.
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The results of an intraoperative transesophageal echocardiography also noted an 8-mm nodule on the undersurface of the noncoronary aortic leaflet. The mass was exposed through an aortotomy and had a healed fibrotic appearance; it was surgically shaved from the leaflet. An epicardial ventricular pacemaker lead was placed and was then postoperatively connected to an abdominal generator. The cardiopulmonary bypass time was 101 min and the cross clamp time was 86 min. Pathologic findings were inflammation changes with multiple histiocytoses of the leaflets and the vegetations, and the cultures were negative.
The patient's recovery was uneventful; he was discharged home on postoperative day 9. He was treated for 12 months with oral doxycycline and hydrochloroquine. He was clinically free of reinfection at 3 years follow-up.
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Comment
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Endocarditis is a very unusual but severe complication of Q fever that occurs in 75% of chronic Coxiella Burnetti infections and has an associated mortality of 25% [1]. Only approximately 2% of patients develop the chronic form of endocarditis that represents the principal complication. Coxiella endocarditis is rare, with only 12 cases reported in the United States in 2000 [2]. Patients with acute Q fever and pre-existing valvular or congenital defects and previous prosthetic valve replacement have an estimated 40% risk of developing endocarditis. The chronic presentation is nonspecific, manifesting with constitutional symptoms, valve dysfunction, and often heart failure. The presence of large vegetations or aggressive leaflet destruction, abscess, or fistulae are rare.
Serology is the primary identification method for Coxiella Burnetti. Indirect immunofluorescence assay in acute Q fever may reveal a 4-fold increase in immunoglobular G titers between acute and convalescence samples or by the presence of phase II immunoglobular M antibodies [2].
Left-sided valves are more frequently involved, and the incidence is equally divided for both aortic and mitral valves. Mesana and colleagues [1] reported on 20 cases of Q fever endocarditis and only one case involved two valves. Tricuspid valve involvement has been reported in association with congenital coronary–cavitary fistula [3, 4], and VSD involvement was reported in only one report [5].
First-line antibiotic treatment consists of doxycicline (200 mg twice a day) and hydrochloroquine (200 mg three times a day) for a variable period of 1 to 3 years [6]. The risk of reinfection is approximately 8% for the aortic valve, as described by Agnihotri and colleagues [7]; however, it is unknown for the tricuspid valve or VSD locations.
The presentation with multiple valve and VSD involvement in this case most likely represented aggressive infection in anatomically contiguous structures. The principles of surgical treatment of endocarditis were applied, including debridement and valve replacement. This case also involved debridement of the right ventricular endocardium and VSD rim, and resection of a tricuspid valve papillary muscle and aortic vegectomy.
We conclude that Coxiella Burnetti infection should be highly suspected in cases of blood cultures negative for endocarditis. The association of extensive Q fever endocarditis (VSD, right ventricular endocardium, tricuspid and aortic valves) is extremely rare, and the diagnostic and surgical approach are challenging. Long-term antibiotic therapy is mandatory.
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References
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- Mesana TG, Collart F, Caus T, Salamand A. Q fever endocarditis: a surgical view and a word of caution J Thorac Cardiovasc Surg 2003;125:217-218.[Free Full Text]
- Gami AS, Antonios VS, Thompson RL, Chaliki HP, Ammash NM. Q fever endocarditis in the United States Mayo Clin Proc 2004;79:253-257.[Abstract/Free Full Text]
- Lupoglazoff JM, Brouqui P, Magnier S, Hvass U, Casasoprana A. Q fever tricuspid valve endocarditis Arch Dis Child 1997;77:448-449.[Abstract/Free Full Text]
- Hvass U, lansac E, Chatel D, Henri I. Mitral homograft for tricuspid valve endocarditis complicating a congenital fistula between the right coronary artery and right ventricle J Heart Valve Dis 1996;5:564-566.[Medline]
- Laufer D, Lew PD, Oberhansli I, Cox JN, Longson M. Chronic Q fever endocarditis with massive splenomegaly in childhood J Pediatri 1986;108:535-539.
- Levy PY, Drancourt M, Etienne J, et al. Comparison of different antibiotic regimens for therapy of 32 cases of Q fever endocarditis Antimicrob Agents Chemother 1991;35:533-537.[Abstract/Free Full Text]
- Agnihotri AK, McGiffin DC, Galbraith AJ, O'Brien MF. The prevalence of infective endocarditis after aortic valve replacement J Thorac Cardiovasc Surg 1995;110:1708-1720.[Abstract/Free Full Text]
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Abstract
Introduction
