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peration, the patient was hemodynamically stable with-ut catecholamines. The patient received 500 mL ofacked red blood cells and 600 mL of plasma.The patient was extubated at 12 hours postoperatively.is postoperative course was complicated by broncho-neumonia and a transitory psychotic syndrome. Theatient was discharged on postoperative day 20.The histopathologic workup showed that the surgicalargins were negative in the IVC (R0). In the pulmonary

ndarterectomy specimen, the neoplastic nature of thembolus was proven; however, margins could not beudged properly.

On follow-up visitation at 6 months, the patient was ingood clinical condition, without any complaints, and

ad returned to work. The follow-up computed tomog-aphy imaging showed no tumor recurrence or metasta-is. The reconstructed IVC was patent.

omment

he IVC is subdivided into 3 segments delineated by thenflow of the hepatic and renal veins [2]. Tumors of thenferior or middle segment can be resected duringlamping of the IVC and by performing the Pringleaneuver. As soon as the upper segment or the RA is

nvolved, CPB is mandatory.The techniques used for the resection and reconstruc-

ion of the IVC include ligation, graft replacement [3],atch cavoplasty, or primary repair [2]; however, thehoice of techniques to be used is controversial. Iferivascular infiltration is observed, a preferably radicalpproach with wide resection and ligation or replace-ent of the IVC is mandatory [4]. However, a predomi-

antly intravascular growth pattern is shown in a rele-ant portion of leiomyosarcomas [2]. In this patient, noerivascular infiltration was seen intraoperatively or dur-

ng the pathologic workup; therefore, we determined thatartial resection with patch reconstruction was adequate.To prevent thrombosis of the reconstructed IVC, the

onstruction of a temporary arteriovenous fistula is oftendvised. We and others [3] think that this is not necessary inase of suprarenal cavoplasty. In our experience of morehan 30 cases of cavoplasty using a native pericardial patch,e have never seen a thrombosis; in addition, the lowest

ates of leg edema are reported for patch reconstruction [2].owever, we advise anticoagulation with phenprocoumon

or 3 months after IVC reconstruction.In the described case, IVC resection and repair were

erformed during HCA because the suprahepatic seg-ent and the RA had to be inspected. After RA closure,e initially tried to complete the cavoplasty during CPB;owever, massive backflow through the hepatic veinsnd collaterals was extremely hindering and led us toomplete the reconstruction during HCA. For manage-ent of possible complications, especially intraoperative

umor embolization, cannulation was done before luxa-ion of the liver. Cerebral oxygen saturation monitoringas used to prevent critical desaturation during HCA.Although pulmonary embolectomy for acute pulmo-

ary embolism can be done as a beating-heart procedure,ae

2010 by The Society of Thoracic Surgeonsublished by Elsevier Inc

ulmonary endarterectomy is required for chronic pul-onary embolic disease, regardless of whether it is

hromboembolic or neoplastic. The latter procedure usu-lly requires HCA to facilitate a blood-free field inatients with compensatory increased blood flow inronchial arteries and collaterals [5].In the tumor in the pulmonary vasculature, unlike the

umor in the IVC, it was impossible to prove R0 resection byeans of histopathology, whereas intraoperatively, all mac-

oscopic parts of the tumor were removed completely.owever, the patient’s history and symptoms indicated a

low and less-invasive growth pattern of the tumor. There-ore, pulmonary recurrence of the tumor should be de-ected early by follow-up computed tomography and op-ionally could be treated by appropriate lung resection.

We conclude that the combined removal of a largeeiomyosarcoma of the IVC and pulmonary endarterec-omy for extensive intravascular neoplastic disease usinghe described approach of intermittent HCA is feasiblend can produce a good perioperative and midtermostoperative result.

eferences

. Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ,Cavallari N. International registry of inferior vena cavaleiomyosarcoma: analysis of a world series on 218 patients.Anticancer Res 1996;16(5B):3201–5.

. Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF.Surgical treatment and outcomes of patients with primaryinferior vena cava leiomyosarcoma. J Am Coll Surg 2003;197:575–9.

. Bower TC, Nagorney DM, Cherry KJ, Jr., Toomey BJ, HallettJW, Panneton JM et al. Replacement of the inferior vena cavafor malignancy: an update. J Vasc Surg 2000;31:270–81.

. Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L. Leiomy-osarcoma of the inferior vena cava: experience in 22 cases.Ann Surg 2006;244:289–95.

. Jamieson SW, Kapelanski DP, Sakakibara N, et al. Pulmonaryendarterectomy: experience and lessons learned in 1,500cases. Ann Thorac Surg 2003;76:1457–62.

evelopment of a Subaorticneurysm Secondary to Disseminateduberculosis in a Child

arirai F. Takawira, FCPaed(SA),ayneel A. Joshi, FCPaed(SA), andirk J. Du Plessis, MMed

epartments of Pediatric Cardiology and Cardiothoracicurgery, Steve Biko Academic Hospital and University ofretoria, Pretoria, South Africa

ubvalvular aneurysms of the left ventricle are very rarend often the cause is uncertain. Most of the cases ofubvalvular left ventricular aneurysms described in the

ccepted for publication Jan 4, 2010.

ddress correspondence to Dr Takawira, Department of Pediatric Cardi-logy, Level D3 – Department of Pediatrics, Steve Biko Academic Hospital

nd University of Pretoria, P Bag X 169, Pretoria, 0001 South Africa;-mail: fari.takawira@up.ac.za.

0003-4975/$36.00doi:10.1016/j.athoracsur.2010.01.070

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iterature are due to congenital weakness of the fibromus-ular annuli. We describe a unique case of a child with auberculous subaortic aneurysm observed at differenttages of development by serial transthoracic echocardiog-aphy. The patient underwent successful cardiac surgeryfter the initial conservative treatment for tuberculosis.

(Ann Thorac Surg 2010;90:644–7)© 2010 by The Society of Thoracic Surgeons

ubvalvular aneurysms of the left ventricle are veryrare and often the cause is uncertain. Very little data

n the literature describes subvalvular aneurysms inhildren and most of the data are derived from caseeports. We describe a unique case of a human immu-odeficiency virus negative child with a tuberculousubaortic aneurysm observed at different stages of devel-pment by serial transthoracic echocardiography. Theatient underwent successful cardiac surgery after the

nitial conservative treatment for tuberculosis (TB).

3-year-old black boy who was negative for humanmmunodeficiency virus presented to our institution with

1-week history of coughing, fever, night sweats, andeneralized body swelling. On examination he appearedhronically ill and was underweight for his age. He hadeneralized lymphadenopathy in the axillary, supracla-icular, cervical, and submandibular regions. He hadscites, hepatomegaly (liver span, 12 cm), and spleno-egaly (3 cm below costal margin). The boy was tachy-

neic with a respiratory rate of 50 breaths/min. He hadntercostal and subcostal recession and audible bilateralhonchi. His heart rate was150 beats/min, with a gallophythm present and with muffled heart sounds. Thereere no murmurs, but he was in congestive cardiac

ailure. His blood pressure was 80/40 mm Hg.A chest roentgenogram demonstrated cardiomegaly, aidened mediastinum, and paratracheal lymphadenop-

thy. The C-reactive protein, lactate dehydrogenase lev-

ig 1. Apical four-chamber view and parasternal long-axis viewchocardiogram (inset) demonstrating the organized loculated peri-ardial effusion. (AO � aorta; LA � left atrium; LV � left ventricle;E � loculated organized pericardial effusion; RA � right atrium;

aV � right ventricle.)

ls, and erythrocyte sedimentation rate were elevated.chocardiography (Fig 1) revealed normal intracardiacnatomy with a large organized and loculated pericardialffusion. A biopsy of a supraclavicular lymph node dem-nstrated caseating necrosis with sites of granulomatous

nflammation. Epitheloid cell granulomas and Langhansiant cells were also seen. The Ziehl Neelsen stainighlighted occasional acid and alcohol fast bacilli, whichonfirmed the TB. There were no features of lymphomar malignancy present. Mycobacterium TB was also iso-

ated on cultures of gastric aspirates. A diagnosis ofisseminated TB with organized TB pericarditis wasade.The patient was treated with anti-TB drugs (isoniazid,

ifampicin, pyrazinamide, and ethambutol), prednisone,nd appropriate cardiac failure therapy. His clinical con-ition improved with resolution of the generalizeddema and ascites and with a decreased size of his lymphodes. A repeat echocardiogram (Fig 2) after 6 weeks of

reatment demonstrated a small pouch (2 mm � 3 mm) inhe subaortic area with mild aortic regurgitation. Cardiacurgery was not considered at this stage because of thective TB and the possibility of friable tissue. He waslosely monitored while receiving TB treatment, and heemained clinically asymptomatic. Another follow-upchocardiogram (Fig 3) 3 months later demonstrated aarge subaortic aneurysm just below the left coronaryusp that measured 30 mm � 24 mm and compressed theeft atrium. The neck of the aneurysm measured 5 mmnd a color Doppler echocardiogram demonstrated flown and out of the aneurysm from the left ventricle. Theulsed wave spectral Doppler echocardiogram demon-trated restrictive bidirectional flow into the aneurysmith a flow velocity of 4 m/s. There was no distortion of

he aortic valve and only mild aortic regurgitation wasvident. These findings were confirmed on transesopha-eal echocardiography (Fig 4). A left ventriculogram (Fig) demonstrated the large aneurysm clearly, which also

ig 2. Repeat transthoracic echocardiogram 6 weeks after presenta-ion. Parasternal long axis view demonstrating a small pouch in theubaortic area (arrow). (AO � aorta; LA � left atrium; LV � leftentricle; RV � right ventricle.)

ppeared to be compressing the left coronary artery.

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The boy underwent successful cardiac surgery 5onths after TB therapy was initiated. There was exten-

ive fibrosis in the thoracic cavity and pericardial space.he aneurysm was approached through an incision in thescending aorta and through the aortic valve. The neck ofhe aneurysm was sutured and closed to isolate theneurysm, and blood was allowed to resorb spontane-usly. Due to the extensive fibrosis and the posteriorosition of the aneurysm, evacuation was deemed tooisky. The boy was doing well 4 months after the surgerynd had completed the anti-TB therapy.

omment

ubvalvular left ventricular aneurysms can either beubaortic or submitral. The submitral aneurysms are

ig 3. Repeat transthoracic echocardiogram 3 months after presenta-ion. (A) Modified parasternal long-axis view demonstrating a largeubaortic aneurysm. (B) Modified apical three-chamber view demon-trating the large subaortic aneurysm compressing the left atriumLA). The opening of the aneurysm is seen just below the aorticalve (arrow). (AN � aneurysm; AO � aorta; LV � left ventricle;

aV � right ventricle.)

ore common than the subaortic type [1]. Subaortic andubmitral aneurysms arise in the fibro-muscular rings ofhe aortic valve and mitral valve annuli, respectively [2].

Most of the cases of subvalvular left ventricular aneu-ysms described in the literature are due to congenitaleakness of the fibro-muscular annuli. Chesler and col-

eagues [2] postulate that a dehiscence of the fibro-uscular union will result in aneurysm formation. Theajority of these patients are of African ancestry, although

imilar conditions have been found to a lesser degree inther race groups [1, 3, 4]. They have also been described ascomplication in patients with infective endocarditis, tu-

erculosis, and syphilis [4–7]. Du Toit and colleagues [6] inheir series of 12 patients found an association with rheu-

atic carditis in 2 patients. The causal effect of all thesenfections has always been speculative, as it may just reflecthe high incidence of the infections in endemic areas, ratherhan a causal mechanism [6].

Our patient had disseminated mycobacterium TB in-ection with cardiac involvement. There was no aneu-ysm noted on the initial echocardiogram, thus excludingcongenital origin. The aneurysm was initially identified

ig 4. Transesophageal echocardiogram: (A) demonstrating the largeubaortic aneurysm compressing the left atrium and narrow neckarrow). (B) With color Doppler showing the restrictive flow throughhe neck of the aneurysm (arrow). (AN � aneurysm; AO � aorta;A � left atrium; LV � left ventricle; RV � right ventricle.)

s a small pouch 6 weeks after presentation. By 4 months

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647Ann Thorac Surg CASE REPORT KASAHARA ET AL2010;90:647–9 TRICUSPID ATRESIA AND ABSENT PULMONARY VALVE

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he small pouch had grown into a large aneurysm requir-ng surgical intervention.

We believe this case is the first report in which a congen-tal cause could be excluded by echocardiogram, and aneu-ysm development was linked to disseminated TB. We alsoelieve that this is the youngest patient reported with aubaortic aneurysm secondary to TB. Disseminated TB isften associated with acquired immunodeficiency syn-rome, but our patient was negative for human immuno-eficiency virus and was immunocompetent. There wereo vegetations identified on any of the valves to suggest

nfective endocarditis as the cause.Subaortic aneurysms must be distinguished from sinus

f Valsalva aneurysms. The aortic valve and the sinusesere normal, both on transesophageal echocardiogram andn angiogram, excluding a sinus of Valsalva aneurysm.Surgical repair is the treatment of choice for large

ubaortic aneurysms, which are at risk of rupture, calci-cation, and infective endocarditis if left untreated [8].arge aneurysms have been reported to compress theoronary arteries, causing myocardial infarction and sud-en death [1, 2], or to compress the conduction system,ausing heart blocks [9]. The ventriculogram in ouratient demonstrated compression of the left coronaryrtery, although the electrocardiogram did not reveal anyschemic changes.

Transthoracic and transesophageal echocardiographylayed a pivotal role in identifying, monitoring, andlanning surgical intervention in our patient.

eferences

. Normann SJ. Annular subaortic aneurysm resulting in sud-den death. Clin Cardiol 1991;14:68–72.

. Chesler E, Mitha AS, Edwards JE. Congenital aneurysms

ig 5. Left ventriculogram and ascending aortogram (inset) demon-trating the large aneurysm compressing on the left coronary arteryarrows). (AO � aorta; AN � aneurysm; LV � left ventricle.)

adjacent to the annuli of the aortic and/or mitral valves. Chest1982;82:334–7.

Ke

2010 by The Society of Thoracic Surgeonsublished by Elsevier Inc

. Inoue Y, Kiso I, Takahashi R, Mori A, Nakajima H. Aorticsubannular left ventricular aneurysm in a patient of Asianancestry. JJTCVS 2001;49:325–6.

. Deshpande J, Vaideeswar P, Sivaraman A. Subvalvular leftventricular aneurysms. Cardiovasc Pathol 2000;9:267–71.

. Muller H, Cikirikcoglu M, Lerch R. Subaortic aneurysmcaused by Paecilomyces lilacinus endocarditis. Arch Cardio-vas Dis 2008;101:803–4.

. Du Toit HJ, Von Oppell UO, Hewiston J, Lawrenson J, DaviesJ. Left ventricular sub-valvar mitral aneurysms. Interact Car-diov Thorac Surg 2003;2:547–51.

. Kamble MS, Kamat SV. Subaortic aneurysm in a case of aorticcoarctation: case report. Cardiovasc Intervent Radiol 1992;15:189–91.

. Rose AG, Bortz D, Commerford PJ. Severe para-ortic regur-gitation due to ruptured congenital subaortic aneurysm. S AfrMed J 1984;66:230–2.

. Sivasankaran S, Kannan BRJ, Kumar A, Tharakan JA. Coex-istence of congenital subaortic and sinus of Valsalva aneu-rysms. Indian Heart J 2002;54:432–4.

enestrated Exclusion of the Rightentricle for Tricuspid Atresia andbsent Pulmonary Valve

irofumi Kasahara, MD, Ryo Aeba, MD, andyohei Yozu, MD

ivision of Cardiovascular Surgery, Keio University, Tokyo,apan

he case of a boy who had an absent pulmonary valve,ricuspid atresia, intact ventricular septum, coronary-to-ight-ventricular fistula, and dysplasia of the right ven-ricular free wall is described. At the bidirectional cavo-ulmonary shunt procedure, the right ventricular freeall was opened and two major fistula orifices to the

avity were closed with sutures. A fenestrated circularatch was placed in the main pulmonary artery and theight ventricular free wall was plicated. The patient thennderwent completion for total cavopulmonary connec-

ion. Follow-up catheterization showed that the pulmo-ary artery was partially excluded with minimal pressureave conduction from the right ventricle, which signifi-

antly shrank. This new approach seems to be effectivend reproducible in this particular situation.

(Ann Thorac Surg 2010;90:647–9)© 2010 by The Society of Thoracic Surgeons

bsence of the pulmonary valve occurs infrequentlyin association with tricuspid atresia, intact ventric-

lar septum, and dysplasia of the right ventricular freeall. The right ventricle is nonfunctional and potentiallyazardous for Fontan completion because of nonphysi-logic backward flow in the systemic vein, and surgicalanagement is challenging and controversial [1]. In the

ollowing Fontan case, fenestrated exclusion of theathologic right ventricle (RV) is described.

ccepted for publication Feb 17, 2010.

ddress correspondence to Dr Aeba, Division of Cardiovascular Surgery,

eio University, 35 Shinano-machi, Shinjuku-ku, Tokyo, 160-8582 Japan;-mail: aeba@sc.itc.keio.ac.jp.

0003-4975/$36.00doi:10.1016/j.athoracsur.2010.02.044