Ann Thorac Surg 1995;59:753-755
© 1995 The Society of Thoracic Surgeons
Case Reports
Endocarditis With Multiple Intracardiac Shunts: Identification and Repair
Masood Sadiq, MRCP,
Narayanswami Sreeram, MRCP,
Joseph V. de Giovanni, FRCP,
John G. Wright, MRCP,
William J. Brawn, FRCS,
Edmund J. Ladusans, MRCP,
Babulal Sethia, FRCS
Heart Unit, Birmingham Children's Hospital, Birmingham, United Kingdom
Accepted for publication July 9, 1994.
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Abstract
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An 8-year-old boy who suffered from Hirschsprung's disease had development of tricuspid valve endocarditis that progressed to aortic root abscess formation, development of a fistulous communication between aorta and right atrium, atrial and ventricular septal defects, and a left ventricle to right atrium defect. Several surgical procedures were required. Operation consisted initially of closure of the septal defects and aortic valve repair. This was followed by homograft replacement of the aortic valve for persistent infection, and further closure of a left ventricle to right atrium fistula.
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Introduction
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Infective endocarditis of the tricuspid valve in children with structurally normal hearts is a recognized complication of indwelling central venous lines [1]. In children in whom hemodynamically significant intracardiac shunts develop, there are few reports of a successful outcome after operation. We describe the diagnostic features and surgical treatment of multiple intracardiac shunts and fistulas in a child.
The patient, an 8-year-old boy with Hirschsprung's disease and no known previous heart problem, who had many previous bowel operations, underwent creation of a permanent colostomy and insertion of a central venous line for parenteral feeding. Pyrexia developed on the eighth postoperative day, and two strains of coagulase-negative staphylococcus (Staphylococcus epidermidis) were isolated from blood cultures. Despite treatment with appropriate antibiotics he remained pyrexial, and 5 days later a systolic heart murmur developed in the third left intercostal space. Two-dimensional echocardiography demonstrated a large vegetation with cavitation in the right atrium, attached to the mid and superior portions of the atrial septum (Fig 1A
). Two days later a continuous murmur developed associated with hemodynamic compromise. Two-dimensional echocardiography demonstrated an aortic root abscess with prolapse of the noncoronary cusp of the valve. Doppler color flow imaging showed severe aortic regurgitation, an acquired atrial septal defect, a fistulous communication between the aorta and right atrium, and a further communication between the left ventricle and both right ventricle and right atrium (Fig 1B).
Operative repair was performed under local and systemic hypothermia to 25°C. The findings were free purulent pericardial fluid and an abscess at the base of the ascending aorta extending from the noncoronary sinus toward the interatrial septum. There was a large lump of thrombus in the right atrium attached to an infected abscess cavity, which extended from the base of the septal leaflet of the tricuspid valve superiorly to the aortic root. There was a communication from the right atrium into the aorta and a further communication into the right ventricle beneath the septal leaflet of the tricuspid valve between the left and right ventricles, and from left ventricle to right atrium. The noncoronary cusp of the aortic valve was detached from the aortic annulus, resulting in aortic-left ventricular discontinuity. The infected thrombus was excised and infected tissues debrided. Bovine pericardium was used to close the atrial and ventricular septal defects. The aortic valve was repaired by resuspension of the noncoronary cusp to the pericardial patch superiorly. Postoperative echocardiography showed minimal aortic and tricuspid valve regurgitation with no residual intracardiac shunts.
Ten days later his clinical condition deteriorated. Transthoracic and transesophageal echocardiograms with color flow imaging showed a defect in the region of the atrioventricular membranous septum, producing shunting between the left ventricle and both the right ventricle and right atrium. The aortic root abscess had recurred with evidence of severe aortic regurgitation. These findings were confirmed at cardiac catheterization and angiography. At a second operation the previous septal defect patch was removed, the margins of the defect were debrided, and a new pericardial patch was used to close the ventricular and atrial septal defects. The aortic root was excised together with the valve leaflets and replaced with a 22 mm cryopreserved homograft.
Three weeks later, despite continuing antibiotic therapy, clinical deterioration was again evident, associated with a new pansystolic murmur but without laboratory evidence of persistent infection. Echocardiography showed a large communication between left ventricle and right atrium (Fig 1C
), which was confirmed on angiography. The operative findings were of a 5--6 mm diameter defect below the aortic valve, which communicated with the right atrium. There was no evidence of active infection. The defect was approached through the aorta and closed directly with a series of pledgeted sutures.
The patient was allowed home 22 days later (total duration of hospital stay of 69 days), with no clinical or laboratory evidence of persistent infection. The 12-lead electrocardiogram showed sinus rhythm with first-degree atrioventricular block. Echocardiography showed no residual fistulous communications or aortic regurgitation. At 4 months follow-up (3 months off antibiotics), the laboratory parameters of infection are normal and the patient remains well, with no clinical or echocardiographic evidence of aortic regurgitation or a fistulous communication.
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Comment
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Infective endocarditis is an uncommon disease in children with structurally normal hearts, although an increasing incidence has been associated with the use of indwelling catheters [1, 2]. In adult patients, aortic root abscesses have been reported to occur from infections due to organisms of low virulence. Previous studies have also shown that valvular destruction and heart failure are more common in patients with vegetations discernible by echocardiography [3]. The routine application of transthoracic and transesophageal echocardiography therefore plays an increasingly important role in the early diagnosis of endocarditis, in anticipating the associated complications, and in providing noninvasive assessment of the hemodynamic consequences of acquired intracardiac shunts or fistulas.
The indications for operation during active endocarditis include progressive and uncontrolled heart failure, embolization or extension of infection with destruction of cardiac structures. Aortic root replacement during active infection has been extensively practiced in adult patients with uncontrolled infection, and appears to be superior to prosthetic valve replacement [4, 5]. The major predictor of recurrence of infection appears to be the presence of ``extensive'' as opposed to ``localized'' infection [4]. In children who have not achieved their full growth, homograft valves are preferable to prosthetic valve replacement. The individual complications of endocarditis described in this report (aorta to atrial fistula, atrial and ventricular septal defect, and left ventricle to right atrium fistula) have been recognized previously, predominantly in older patients. This report documents correct echocardiographic identification of the multiple lesions and successful repair requiring several surgical procedures.
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Footnotes
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Address reprint requests to Mr Sethia, Heart Unit, Birmingham Children's Hospital, Ladywood Middleway, Birmingham B16 8ET, UK.
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References
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- Tornos MP, Castro A, Toran N, Girona J. Tricuspid valve endocarditis in children with normal hearts. Am Heart J 1989;118:624–5.[Medline]
- Stanton BF, Baltimore RS, Clemens JD. Changing spectrum of infective endocarditis in children. Am J Dis Child 1984;138:720–5.[Abstract/Free Full Text]
- Rohmann S, Erbel R, Gorge G, et al. Clinical relevance of vegetation localization by transesophageal echocardiography in infective endocarditis. Eur Heart J 1992;13:446–52.[Abstract/Free Full Text]
- Haydock D, Barratt-Boyes B, Macedo T, Kirklin JW, Blackstone E. Aortic valve replacement for active infectious endocarditis in 108 patients. A comparison of freehand allograft valves with mechanical prostheses and bioprostheses. J Thorac Cardiovasc Surg 1992;103:130–9.[Abstract]
- Tuna IC, Orszulak TA, Schaff HV, Danielson GK. Results of homograft aortic valve replacement for active endocarditis. Ann Thorac Surg 1990;49:619–24.[Abstract]
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Abstract
Introduction
