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Ann Thorac Surg 2003;76:1303-1305
© 2003 The Society of Thoracic Surgeons


Case report

Infectious endocarditis of a Chiari network

Darrin M. Payne, BSa,b, Roger J. F. Baskett, MA, MD*a,b, Gregory M. Hirsch, MDa,b

a Division of Cardiac Surgery, The Maritime Heart Centre, Halifax, Nova Scotia, Canada
b Division of Cardiac Surgery, University of Western Ontario, London, Ontario, Canada

Accepted for publication March 5, 2003.

* Address reprint requests to Dr Baskett, The Maritime Heart Centre, Room 2269, 2nd Floor, 1796 Summer St, Halifax, NS, Canada B3H 3A7
e-mail: rogerbaskett{at}hotmail.com


    Abstract
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We present the case of a 62-year-old man with infectious endocarditis in a Chiari network. Chiari networks are present in 1.5% to 3% of the population. Although Chiari networks are usually clinically insignificant, they are associated with a number of conditions, including patent foramen ovale, thromboembolism, atrial aneurysm, and cardiac arrhythmias. Although there are rare reports of patients with a Chiari network who had endocarditis develop, this is the first report of a patient who had endocarditis develop solely within a Chiari network.


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Chiari networks were first described in 1897 by Hans Chiari as a network of threads and fibers found in the right atrium [1]. They are an embryologic remnant, resulting from incomplete resorption of the right sinus venosus valve and the persistence of a filamentous tissue network. They are rarely of clinical importance. Here we present the case of a 62-year-old man who presented with endocarditis involving a Chiari network.

A previously asymptomatic 62-year-old man with known hypertension and poorly controlled noninsulin dependent diabetes mellitus presented with a 1-week history of malaise, fatigue, fevers, chills, sweats, and decreased appetite. In addition, he had a 4-day history of a red, warm, swollen left knee, and left arm. He had no history of intravenous drug abuse, alcoholism, trauma, or any recent surgeries or dental work. Physical examination revealed the patient to be in no acute distress, afebrile, with bilateral basilar crackles at the lung bases, and bilateral pedal edema. His left forearm and knee were as he described. He had a moderate elevation of his liver enzymes and a white blood cell count of 13,600. He was admitted and treated for septic arthritis and cellulitis.

He continued to have fevers and chills, as well as a persistently elevated white blood cell count. Aspiration and eventual surgical exploration and irrigation of the left knee revealed negative bacterial cultures. Blood cultures were positive for group B streptococcus. Infectious endocarditis was suspected, and a transesophageal echocardiography was performed revealing four normal valves and a highly mobile right atrial mass of questionable cause and no patent foramen ovale (PFO). A computerized axial tomographic scan demonstrated a moderate right pleural effusion and multiple new lung nodules, likely representing an infectious and inflammatory cause.

At cardiac catheterization the patient was found to have critical three-vessel coronary disease. Considering the size and mobility of the mass and the evidence of recent pulmonary emboli, he was taken to the operating room for resection of a right atrial mass and coronary artery bypass grafting.

Intraoperative exploration of the right atrium revealed a 3 x 1 cm mass, tethered to the right atrial wall by two thin tissue strands (Figs 1, 2). The tricuspid valve was normal and showed no evidence of infection. Coronary revascularization was performed without complication. The postoperative course was unremarkable, the fevers and leukocytosis resolved.



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Fig 1. Intraoperative view of the Chiari network. Note the filamentous attachment (held by the forceps) and the associated thrombus. (Surgeon’s view, patient’s head to the left.)

 


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Fig 2. Chiari network with associated thrombotic vegetation.

 

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Chiari networks are an embryological remnant that persists as a reticular meshwork of right atrial tissue with variable attachments to the crista terminalis, eustachian valve, and thebesian valve. During early fetal life, after atrial absorption of the sinus venosus, the atrium is divided into two separate chambers by the right valve of the sinus venosus. This division separates the right atrium into a sinus portion, receiving the vena cavae, coronary sinus, and foramen ovale, and a muscular portion housing the tricuspid valve and atrial appendage [2]. Normally, the right sinus venosus valve almost completely regresses by the fifteenth week of gestation, with its remnants persisting as the thebesian and Eustachian valves [2]. Persistence of more tissue is referred to as a Chiari network.

Chiari networks are present in 1.5% to 4% of the population [3], they and are usually of no clinical significance. Associations have been made with the presence of a patent foramen ovale, intraatrial thrombus and thromboembolic events, atrial aneurysm, cardiac arrhythmias, and most recently with infectious endocarditis.

Chiari networks have a well-documented association with a PFO [1]. Using contrast echocardiography and provocative measures, Schneider and associates [1] demonstrated that a PFO was detected in 83% of patients with Chiari networks compared with 28% of controls. Significant left-to-right shunting through a PFO was also more common in the Chiari network group.

Although uncommon, Chiari networks can serve as a site for the formation of thrombus. This may be primary thrombus formation in the network or capture of venous thromboembolic matter [1]. Right atrial thrombus may be asymptomatic, result in variable degrees of pulmonary emboli, or in association with a PFO may become a source of cerebral and peripheral arterial emboli [3].

Schneider and associates [1] examined 29 patients with Chiari networks and observed that 7 patients (24%) also had atrial aneurysms. All patients were associated with a PFO. Similarly, among 1,436 patients undergoing transesophageal echocardiography for examination of their atrial aneurysms, 21% were found to have Chiari networks [1]. It remains unknown whether this association is causal or merely coincidental.

Cardiac arrhythmias in association with Chiari networks also have been reported [3] with termination of the arrhythmias being accomplished through surgical excision. There are also reports of Chiari networks acting as a physical barrier that interferes with the introduction of right-sided catheters and pacemakers [1].

There are rare reports of Chiari network infections associated with valvular endocarditis [4, 5]. However, we believe that our case report of a patient with normal valves who presented with endocarditis involving only the Chiari network and associated thrombus has not been previously reported. In our patient there was no valvular involvement and he experienced full resolution of his symptoms after surgical excision of the mass.

Chiari networks are often picked up incidentally with echocardiography as an asymptomatic right atrial mass. Echocardiography, in particular transesophageal echocardiography, is both a reliable and noninvasive means of identifying Chiari networks [1]. It should be considered in the differential diagnosis of endocarditis.


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 Abstract
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 References
 

  1. Schneider B., Hofmann T., Justen M.H., Meinertz T. Chiari’s network: normal anatomic variant or risk factor for arterial embolic events?. JACC 1995;26:203-210.[Abstract]
  2. Ducharme A., Tardif J.C., Mercier L.A., et al. Remnants of the right valve of the sinus venosus presenting as a right atrial mass on transthoracic echocardiography. Can J Cardiol 1997;13:573-576.[Medline]
  3. Goedde T.A., Conetta D., Rumisek J.D. Chiari network entrapment of thromboemboli: congenital inferior vena cava filter. Ann Thorac Surg 1990;49:317-318.[Abstract]
  4. Werner J.A., Cheitlin M.D., Gross B.W., Speck S.M., Ivey T.D. Echocardiographic appearance of the Chiari network: differentiation from right-heart pathology. Circulation 1981;63:1104-1109.[Abstract/Free Full Text]
  5. Lejko-Zupanc T., Kozelj M., Pikelj F. Right sided endocarditis: clinical and echocardiographic characteristics. J Clin Basic Cardiol 1999;2:81-84.



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