Ann Thorac Surg 2004;77:2183-2184
© 2004 The Society of Thoracic Surgeons
Case report
Single-stage repair of tetralogy of Fallot with pseudoaneurysm: a unique approach
Sreekanthan Sundararaghavan, MDa*,
Omar Khalid, MDa,
William A. Suarez, MDa,
Edward L. Bove, MDb
a Department of Pediatrics, Medical College of Ohio, Toledo, Ohio, USA
b Section of Cardiac Surgery, Department of Surgery, University of Michigan, Ann Arbor, Michigan, USA
Accepted for publication June 13, 2003.
* Address reprint requests to Dr Sundararaghavan, Department of Pediatrics, Medical College of Ohio, 2222 Cherry St, Suite 2800, Toledo, OH 43608, USA
e-mail: sraghavan{at}mco.edu
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Abstract
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Pseudoaneurysm is a potentially lethal complication after modified Blalock-Taussig shunt. This report describes a frequently misdiagnosed clinical presentation along with noninvasive diagnosis of pseudoaneurysm after a modified Blalock-Taussig shunt. We report a novel single-stage surgical management of the pseudoaneurysm with concomitant complete repair of tetralogy of Fallot.
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Introduction
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The Blalock-Taussig (BT) shunt procedure was the palliative procedure of choice for children with cyanotic congenital heart disease for many years [1]. More recently, the modified BT shunt utilizing a polytetrafluoroethylene (Gore-Tex; W.L. Gore and Assoc, Flagstaff, AZ) interposition graft has been used with increasing frequency for palliative care of selective patients. Pseudoaneurysm, although rare after BT shunts, is a potentially fatal complication. In this report, we describe the clinical presentation and the noninvasive diagnosis of an infant who underwent a modified BT Shunt with subsequent development of a pseudoaneurysm at the shunt anastomosis site. Additionally, the surgical management of the pseudoaneurysm with concomitant complete repair of the underlying cardiac pathology is presented.
An 8-month-old male infant with history of tetralogy of Fallot palliated with a modified BT shunt at another institution presented with a 1-week history of intermittent fever, cough, decreased oral intake, and bloody emesis. He was febrile, tachycardic, cyanotic, and in moderate respiratory distress. Examination revealed decreased breath sounds with bronchial breathing, coarse rhonchi over the right upper lobe, and a grade 3/6 high pitch systolic ejection murmur at the left sternal border without a diastolic component. The chest roentgenogram revealed right upper lobe consolidation with mild deviation of the trachea to the left side. The echocardiogram showed normal biventricular systolic function with an unrestrictive ventricular septal defect and antegrade flow through a small pulmonary artery. Turbulent flow was noted entering into the pulmonary artery by color Doppler. The modified BT shunt could not be visualized.
Laboratory studies showed leukocytosis and anemia. Blood culture grew Streptococcus pneumoniae. Respiratory distress worsened despite antibiotic therapy. A repeat chest roentgenogram showed more prominent tracheal deviation with right upper lobe consolidation (Fig 1).
Echocardiogram showed cavitations and soft tissue mass below the innominate vein and surrounding the ascending aorta extending to the right hemithorax. The turbulent continuous flow from the aortopulmonary shunt was absent. Computed tomography scan showed a large opacity occupying the upper half of the right hemithorax. A well circumscribed, uncalcified area of dense enhancement (shunt), extended both caudally and medially, but did not enhance at the point of anastomosis distally or proximally (Fig 2).
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Fig 1. Chest roentgenogram with a dense consolidated mass and site of shunt surgery (arrow) in the right thorax with deviation of the tracheal air column to the left.
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Fig 2. Computed tomography scan image of pseudoaneurysm surrounding the modified Blalock-Taussig shunt. (Ao = aorta; PA = pulmonary artery; S = spine; T = trachea.)
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Hypothermic cardiopulmonary bypass was established before any dissection. The pseudoaneurysm was then identified through the median sternotomy approach. Due to the large mass of necrotic tissue, the proximal right subclavian artery was exposed with difficulty. On low flow bypass, the proximal anastomotic site was repaired by direct suture and the pseudoaneurysm was completely excised. The shunt was floating freely in the middle of the aneurysm, having completely dehisced from both anastomotic sites. The distal shunt anastomotic site was identified at the take off of the right upper lobe branch, which was sacrificed due to extensive involvement, and the defect in the pulmonary artery was repaired with a patch of autologous pericardium. A small patent ductus arteriosus was divided. Obstructing right ventricular muscle bundles were divided transatrially and the ventricular septal defect closed with a Gore-Tex patch. Because of significant hypoplasia of the pulmonary valve annulus, an incision was performed crossing the pulmonary valve into the distal right ventricular outflow tract. Further exploration revealed an infected posterior leaflet of the pulmonary valve with extension posteriorly outside the pulmonary artery. The area of infection was debrided and found to be well incorporated by surrounding tissue. A transannular patch was placed. Cultures were obtained from all the infected tissues. The right ventricular pressure was 65% of systemic pressure on completion of the repair.
A postoperative transesophageal echocardiogram demonstrated good biventricular systolic function, no residual shunt, and no right ventricular outflow tract obstruction. Although the cultures were negative to date, he received 6 weeks of intravenous antibiotics. On follow-up 4 months later, he was asymptomatic with mild-to-moderate pulmonic insufficiency.
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Comment
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The medical and surgical experience with modified BT shunt is extensive. However, complications of this procedure still occur and include inadequate palliation, excessive shunt flow, chylopericardium, infection, aneurysm, early thrombosis, and pulmonary artery distortion [2, 3]. Pseudoaneurysm secondary to infection is a rare but well-recognized complication.
Our patient presented with right upper lobe consolidation and positive blood cultures, suggesting the diagnosis of pneumonia. The initial chest roentgenogram and echocardiogram did not show significant mass effect, possibly due to the lack of confinement in the superior mediastinum and minimal distortion of surrounding structures. However, within 48 hours the mass effect became pronounced radiographically. The diagnosis was established by echocardiogram and confirmed using computed tomography scan with contrast, which clearly delineated the pseudoaneurysm with shunt discontinuity (Fig 2). Unlike previous case reports, the need for a cardiac catheterization to establish the diagnosis was not necessary [4, 5] and a noninvasive investigative approach was considered to be adequate before surgical repair.
This case indicates that a single-stage repair in such patients is feasible and can be accomplished with minimum morbidity. Importantly, exploration of all the cardiac tissues and valves for occult vegetations proved essential. In this patient, the posterior leaflet of the pulmonary valve and the surrounding tissue was noted to be infected and was removed in its entirety. The extent of the infection and the involvement of the pulmonary valve were not appreciated on preoperative studies. Inadequate exploration might have undermined the success of the surgery. Performing the initial portions of the surgery on hypothermic low-flow bypass facilitated evaluation of all the cardiac pathology while avoiding potentially catastrophic hemorrhage entering the pseudoaneurysm itself.
This unusual case alerts physicians to consider shunt-related pathology if the patient presents with respiratory symptoms and ipsilateral signs of consolidation with or without signs of compression of mediastinal structures. Despite the extensive nature of the operation, a single-stage repair should be considered if the cardiac anatomy is favorable. Every effort to completely remove the infected material and cardiac tissue is essential.
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References
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- Blalock A., Taussig H.B. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 1945;128:189-202.[Abstract/Free Full Text]
- Opie J.C., Traverse L., Hayden R.I., Ho C.Y., Culham J.A.G., Ashmore P.G. Experience with polytetrafluoroethylene grafts in children with cyanotic congenital heart disease. Ann Thorac Surg 1986;41:164-168.[Abstract/Free Full Text]
- Gladman G., McCrindle B.W., Williams W.G., Freedom R.M., Benson L.N. The modified Blalock-Taussig Shunt: clinical impact and morbidity in Fallot's tetralogy in the current era. J Thorac Cardivasc Surg 1997;114:25-30.[Abstract/Free Full Text]
- Valliattu J., Jairaj P., Delamie T., Subramanyam R., Menon S., Vyas H. False aneurysm following modified Blalock-Taussig shunt. Thorax 1994;49:383-384.[Abstract/Free Full Text]
- Jan S.L., Hwang B., Fu Y.C., Chi C.S. Pseudoaneurysm formation after infected modified Blalock-Taussig shunt: echocardiographic finding. Echocardiography 2000;17:187-191.[Medline]
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Abstract
Introduction
