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´ NDEZ ET AL CASE REPORT BAUTISTA-HERNA CONSTRICTIVE PERICARDITIS AND Q FEVER
pulmonary artery hypertension. This report also highlights the importance of careful evaluation of ECG in the preoperative assessment of congenital heart disease. Severe LV dysfunction in a neonate, especially with ECG changes, should be interrogated thoroughly for ALCAPA including its origin from unusual sites.
References 1. Levin SE, Dansky R, Kinsley RH. Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta. Int J Cardiol 1990;27:31–6. 2. Bharati S, Chandra N, Stephenson LW, Wagner HR, Weinberg PM, Lev M. Origin of the left coronary artery from the right pulmonary artery. J Am Coll Cardiol 1984;3: 1565–9. 3. Schmidt KG, Cooper MJ, Silvermann NH, Stanger P. Pulmonary artery origin of the left coronary artery: diagnosis by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow mapping. J Am Coll Cardiol 1988;11: 396 –402.
CASE REPORTS
Constrictive Pericarditis Due to Coxiella burnetii Vı´ctor Bautista-Herna´ ndez, MD, Francisco Gutierrez, MD, PhD, Vı´ctor G. Ray, MD, Jose´ M. Arribas, MD, Julio Garcı´a-Puente, MD, Norberto Casinello, MD, PhD, and Ramo´ n Arcas, MD, PhD Regional Service of Cardiovascular Surgery, University Hospital “Virgen de la Arrixaca,” Murcia, Spain
Q fever is characterized by its clinical polymorphism. Cardiac involvement in acute Q fever is rare. We report a case of pleuro-pericarditis that rapidly evolved to pericardial constriction during an acute episode of Coxiella burnetii infection. Constrictive pericarditis was confirmed by hemodynamic measurements, echocardiography, and magnetic resonance. Indirect immunofluorescence assay revealed positive serology for acute Q fever. The patient underwent a successful pericardiectomy and was given antibiotics. The histopathologic study of the excised pericardium showed C. burnetii in a large cluster of organisms. After a 6-month follow-up period, the individual was asymptomatic. (Ann Thorac Surg 2004;78:326 – 8) © 2004 by The Society of Thoracic Surgeons
C
onstrictive pericarditis is an uncommon complication of disorders of diverse etiology. Although most often idiopathic, constrictive pericarditis also may occur after acute pericarditis, infection, chest trauma, radiation Accepted for publication July 18, 2003. Address reprint requests to Dr Bautista-Herna´ ndez, Servicio Regional de Cirugı´a Cardiovascular, Hospital Universitario “Virgen de la Arrixaca,” 30120 El Palmar, Murcia, Spain; e-mail: vbautista_hernandez@hotmail. com.
© 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2004;78:326 – 8
therapy, or granulomatous disease [1]. Q fever is a worldwide zoonosis due to infection with the rickettsia Coxiella burnetii, an obligate intracellular organism first described in 1937 by E.H. Derrick [2]. C. burnetii main reservoirs are cattle, sheep, and goats. Humans contract the disease generally by inhaling contaminated particles or by ingesting nonpasteurized milk from infected animals. C. burnetii exhibits a phase-variation phenomenon caused by the partial loss of a lipopolysaccharide. Diagnosis is usually made by mean of an indirect immunofluorescence test, as described previously [3]. A serum sample with titers of phase II IgG of 1:200 or higher and titers of phase II IgM of 1:50 or higher is 100% predictive of recent Q fever [4]. In untreated cases, phase II antigenspecific IgM appears in the circulating blood between 7 and 15 days after onset of the disease and reaches its maximum titer in 4 to 8 weeks. Phase II IgG appears somewhat later but can persist for years. Clinical presentation of the disease is nonspecific. In acute cases, the major recognized forms are a self-limited febrile illness, atypical pneumonia, and granulomatous hepatitis [5]. Heart involvement has been reported in less than 1% of the patients during the acute phase. The main cardiac manifestations are pericarditis and myocarditis [4]. We report a case of constrictive pericarditis attributable to C. burnetii, successfully treated with pericardiectomy and antibiotic therapy. Constrictive pericarditis has been rare after an episode of acute pericarditis of this etiology. In early October 2001, a 45-year-old man presented with a 4-day history of temperature of 39°C, headaches, cough, asthenia, anorexia, together with myalgia, arthralgia, weight loss, and pleuritic pain. The patient worked in a pharmaceutical factory. He resided in a city suburb and occasionally spent some weekends in the countryside. He did not recall having been in contact with farm animals or animal hides or having ingested raw milk or farm goat cheese. His past medical history was unremarkable and he denied use of alcohol, illicit drugs, or tobacco. He had no family history of heart diseases. Cardiac auscultation revealed a pericardial friction rub. A chest roentgenogram revealed bilateral pleural effusion (Fig 1). On echocardiography, pericardial effusion without signs of constriction was observed. The electrocardiogram showed sinus rhythm with nonspecific STsegment and T wave abnormalities. The patient was diagnosed with idiopathic pericarditis and treated with nonsteroid antiinflammatory agents. The man recovered progressively and was discharged home in good physical condition. The patient’s progress at home was unsatisfactory, and he was reassessed 1 month later. Indirect serum immunofluorescence antibody test results revealed acute C. burnetii infection. Titers of phase II IgG and IgM antibodies were 1:2,048 and 1:192, respectively. Titers of IgA and IgM antibodies against phase I were negative. HIV, hepatitis A, B, and C were excluded. The patient received antibiotic therapy with doxycycline (200 mg/day) for 1 month. 0003-4975/04/$30.00 doi:10.1016/S0003-4975(03)01361-4
Fig 1. Chest roentgenogram showing pleural effusions.
On February 2001, the patient was admitted to the hospital complaining of dyspnea and cough. He did not recall having had a fever. Physical examination revealed bibasilar hypoventilation, hepatomegaly, and jugular vein distension. No cyanosis, rash, or lymphadenopathy was elicited. An electrocardiogram was normal. Magnetic resonance imaging revealed pleural effusion, ascites, and pericardial thickening (Fig 2), which was more marked over the right ventricular free wall and the lateral and posterior left ventricular wall. In these areas, echocardiography revealed impaired cardiac contractility. The global systolic function was preserved. A right thoracentesis yielded 2,900 mL of transudate. Pleural fluid analyses ruled out tuberculosis and malignancy. An echocardiogram showed pericardial thickening and signs of pericardial constriction. Right cardiac catheterization with rapid volume infusion demonstrated a high right
Fig 2. Magnetic resonance imaging demonstrating thickening of the pericardium.
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327
atrial pressure with a rapid Y descent. A “dip-andplateau” pattern was also observed. With these findings, our patient was diagnosed as having constrictive pericarditis due to C. burnetii. A pericardiectomy was carried out through midsternotomy access without cardiopulmonary bypass. Cardiac decortication was performed from the level of the diaphragm to the great vessels and from the left to the right phrenic nerve. Histopathologic examination of the excised pericardium showed dense interstitial fibrosis with chronic inflammatory cell infiltrate. Silver staining of the biopsy specimen demonstrated small clusters of infected cells. Indirect immunofluorescent antibody examination of the excised portion of pericardium showed C. burnetii in large clusters of organisms. High titers of antibodies against phase II of C. burnetii were also found in pericardial and pleural fluids. An echocardiography performed 7 days after surgery revealed no signs of constriction. Cardiac contractility remained impaired on the posterior areas of the left ventricle, where the pericardium had not been removed. The ejection fraction was normal. The patient’s postoperative evolution was uneventful and he was discharged home 8 days after the operation. Six months after pericardiectomy the patient remained asymptomatic.
Comment The spectrum of acute Q fever clinical presentation is extremely broad. Pulmonary, hepatic, neurologic, and dermatological manifestations have been documented. Although rare, cardiac involvement can be lifethreatening. Myocarditis has been reported to be the main cause of death in young patients with acute Q fever [4]. Pericarditis may produce cardiac tamponade or constrictive pericarditis, as in our patient [6]. The most striking aspect in our case was the rapid progression to pericardial constriction after acute pericarditis. Q fever was not initially considered in our patient due to its rarity as a cause of pericarditis. Accordingly, the patient was first managed with the standard treatment for idiopathic pericarditis. The antiinflammatory treatment was helpful for the relief of symptoms, but was ineffective for preventing the development of constrictive pericarditis. In this individual, cardiac decortication “from phrenic to phrenic nerve” and antibiotic therapy proved to be an effective management strategy, although contractility in the areas where pericardium had not been removed remained impaired. One solution might have been performing on-pump pericardiectomy, which could have allowed total pericardial decortication, and perhaps could have achieved a better functional result. Presently, no data are available about the possible long-term effects of constrictive pericarditis due to C. burnetii. Although pericarditis due to C. burnetii is rare, its true incidence is probably underestimated. Diagnosis seems to depend on the thorough search by local physicians and cardiologists and on the availability of a specialized laboratory that is capable of performing the appropriate
CASE REPORTS
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CASE REPORT AGARWALA ET AL RESECTION OF RIGHT VENTRICULAR FIBROMA
serologic tests. Given the potential severity of the cardiac complications of Q-fever and the lack of response of this type of pericarditis to conventional treatments, we suggest that physicians and cardiologists should be aware of this diagnostic possibility and perform routine tests to detect Q-fever in patients with pericarditis, especially in patients with an unsatisfactory evolution or coexistent risk factors.
We thank Dr Martı´nez-Lage for helpful review of the manuscript and Carmen Valera for her assistance in the preparation of this article.
References
CASE REPORTS
1. Myers RB, Spodick DH. Constrictive pericarditis: clinical and pathophysiologic characteristics. Am Heart J 1999;138:219 –32. 2. Derrick EH. “Q” fever, new fever entity: clinical features, diagnosis, and laboratory investigation. Med J Aust 1937;2: 281–99. 3. Dupont HT, Thirion X, Raoult D. Q fever serology: cut-off determination for microimmunofluorescence. Clin Diagn Lab Immunol 1994;1:189 –96. 4. Raoult D, Tissot-Dupont H, Foucault C, et al. Q fever 1985– 1998. Clinical and epidemiologic features of 1,383 infections. Medicine (Baltimore) 2000;79:109 –23. 5. Tissot-Dupont H, Raoult D, Brouqui P, et al. Epidemiologic features and clinical presentation of acute Q fever in hospitalized patients: 323 French cases. Am J Med 1992;93:427–34. 6. Levy PY, Carrieri P, Raoult D. Coxiella burnetii pericarditis: report of 15 cases and review. Clin Infect Dis 1999;29:393–7.
Surgical Issues in Giant Right Ventricular Fibroma Brojendra N. Agarwala, MD, Joanne P. Starr, MD, Eloise Walker, MD, and Emile A. Bacha, MD Sections of Pediatric Cardiology, Cardiothoracic Surgery, Pediatric and Congenital Cardiac Surgery, University of Chicago Children’s Hospital, Chicago, Illinois
Cardiac fibroma is a rare and benign tumor usually seen in infants and children. We report a 3-year-old asymptomatic child who presented with a giant right ventricular (RV) fibroma. He underwent complete surgical resection and is doing well at follow-up. Because of the size of the tumor and the potential need for resection of RV free wall, it is essential to have contingency plans to deal with postoperative RV failure. (Ann Thorac Surg 2004;78:328 –30) © 2004 by The Society of Thoracic Surgeons
Ann Thorac Surg 2004;78:328 –30
C
ardiac fibroma, while overall an exceedingly rare lesion, is the second most common tumor in the pediatric population after rhabdomyomas [1, 2]. The tumor consists of benign growth of myocardial connective tissue and more commonly originates from the left ventricular free wall. It is predominantly a solitary lesion. Patients can be asymptomatic, or present with congestive heart failure, sudden death, or arrhythmias [1, 2]. When feasible, complete resection is usually recommended [1, 2]. Debulking (partial resection), heart transplantation, or conservative management have also been advocated [2, 3]. This report describes a patient with a giant right ventricular (RV) free wall fibroma. Pertinent surgical issues addressed in this report are whether surgery is indicated, the need (and risk) of partial versus complete resection, and the surgical management of RV failure resulting from an extensive RV resection. A 3-year-old previously healthy child presented with a heart murmur. Physical examination revealed a healthy appearing child with normal vital signs, and a prominent left precordium. A grade 1–2/6 atypical ejection systolic murmur was heard along the left upper sternal border. Electrocardiogram revealed normal sinus rhythm, left axis deviation, and right bundle branch block. Twentyfour hour Holter monitor showed no evidence of arrhythmia. Crosssectional echocardiogram (Fig 1) and magnetic resonance imaging (MRI) of the heart (Fig 2) demonstrated a 7 ⫻ 5 cm cardiac tumor located in the inlet portion of the RV free wall, severely compressing the RV cavity. The extent of the tumor in-growth into the endocardium or involvement of the anterior papillary muscle of the tricuspid valve could not be determined. Colorflow Doppler at the RV inflow indicated mild obstruction. At surgery a 7 ⫻ 4.5 cm sessile white appearing poorly vascularized intramural tumor mass was noted in the inlet portion of the anterior wall of the RV, severely compressing the RV cavity. After cardioplegic arrest and right atriotomy, the RV endocardium was noted to be completely intact. Frozen section biopsy of the tumor was consistent with benign fibroma. Starting at the margin between tumor and normal myocardium, the tumor was completely excised using sharp dissection, staying within 1 to 2 mm of healthy myocardium. The endocardium was breached at the lowest point of the tumor creating a 5-mm hole. The right coronary artery and atrioventricular groove were preserved. Once the resection was completed, the remaining cavity was collapsed onto itself and the epicardium closed with a double layer of 5-0 polypropelene. Cross clamp time was 57 minutes. His postoperative course was uneventful. Postoperative echocardiogram did not reveal residual tumor and the RV cavity was normal in size with normal tricuspid Doppler color-flow pattern (Fig 3). At 18-months follow-up, he is doing well.
Accepted for publication June 13, 2003. Address reprint requests to Dr Bacha, Pediatric and Congential Cardiac Surgery, The University of Chicago Children’s Hospital, 5841 S Maryland Ave, MC 5040, Chicago, IL 60637; e-mail:
[email protected]. uchicago.edu.
© 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
Comment Although surgical resection of symptomatic cardiac fibromas is universally accepted, the role of surgery in 0003-4975/04/$30.00 doi:10.1016/S0003-4975(03)01368-7
