c© 2011 Wiley Periodicals, Inc. 367

CASE REPORTS

Cardiac Tuberculoma of theRight Atrium

Mahmoud Momtahen, M.D., F.A.C.C.,∗Nader Givtaj, M.D.,∗Zahra Ojaghi, M.D.,∗Shabnam Momtahen, M.D.,∗Nooshin Almasi, M.D.,∗and Amir J. Momtahen, M.D.†∗Rajaie Interventional CardiovascularResearch Center, Tehran University ofMedical Sciences, Tehran, Iran; and†Department of Radiology, Saint LouisUniversity, St. Louis, Missouri

ABSTRACT We report a patient with a large mass inthe right atrium with tricuspid valve involvementresulting in significant stenosis, which was a tuber-culoma without active pulmonary disease. Cardiactuberculoma is rare and usually involves the peri-cardium. Myocardial involvement is a very rare oc-currence. doi: 10.1111/j.1540-8191.2011.01253.x(J Card Surg 2011;26:367-369)

Myocardial tuberculosis is a rare condition. Cardiactuberculomas are mostly located in the right heartand usually well circumscribed and sharply demarcatedfrom the surrounding parenchyma.1 Herein we report apatient with a large mass in the right atrium with tricus-pid valve involvement resulting in significant stenosis,which was a tuberculoma without active pulmonarydisease.

CASE REPORT

A 21-year-old female patient was admitted due toprogressive dyspnea and weight loss for six months.Her past medical history revealed documented cavi-tary pulmonary tuberculosis two years ago that wasdiagnosed by positive culture of the bronchial aspira-tions and treated for nine months. She did not have ahistory of diabetes mellitus, HIV, or other immunosup-pressive illnesses. The treatment regimen included aninitial phase (two months) with Isoniazid (300 mg/day),Rifampin (600 mg/day), Pyrazinamide (1.2 g/day), andEthambutol (1 g/day) and a continuation phase (sevenmonths) with Isoniazid (300 mg/day) and Rifampin(600 mg/day).

Conflict of Interest: None.

Address for correspondence: Amir J. Momtahen, M.D., Departmentof Radiology, Saint Louis University, 3635 Vista Ave, St. Louis, MO63110. Fax: 314-268-5116; e-mail: momtahen@yahoo.com

Figure 1. Transesophageal echocardiogram at 90◦ (bicavalview) shows a large echogenic right atrial mass (long arrows)attached to the right atrial free wall. Short arrow = SVC;arrowhead = IVC.

Clinical examination was unremarkable apart from anelevated jugular venous pressure (JVP) with no signsof peripheral edema. Results of laboratory investiga-tion of the blood and urine were unremarkable. Ery-throcyte sedimentation rate (ESR) was 51. The chestX-ray demonstrated enlargement of the right atrium.Transthoracic and transesophageal echocardiographyshowed dilated right atrium with a large homoge-nous mass measuring approximately 3.7 cm × 4 cm(Fig. 1). The mass extended to the base of the interatrialseptum and septal leaflet of the tricuspid valve, result-ing in severe functional tricuspid stenosis (transvalvu-lar mean pressure gradient of 10 mmHg). Computedtomography (CT) scan showed a nonenhancing low-density mass measuring 40 mm in maximal short axisat the level of tricuspid valve in the right atrium (Fig.2).

Percutaneous biopsy under echocardiographic guid-ance was performed. The pathologic result was non-specific. Surgical resection was planned with a possiblediagnosis of angiosarcoma. Following a median ster-notomy, aortic, superior vena cava (SVC), and inferiorvena cava (IVC) cannulation was performed. Cardiacarrest was achieved with application of cold crystalloidcardioplegia (St. Thomas II, Martindale Pharmaceuti-cals, Romford, Essex, UK). Subsequently, a right atri-otomy extending from the right atrial appendage to theright interatrial groove was performed. The intraatrialmass adherent to the right atrial free wall with grossappearance of caseation was resected. There was noinvolvement of the tricuspid valve or interatrial sep-tum. The surgical specimen was sent for pathologicevaluation and no frozen section was obtained. Patho-logic examination revealed caseous granuloma (Fig. 3).Granulomatous inflammation was compatible with tu-berculoma. However, Ziehl-neelsen staining failed toreveal acid-fast bacilli. The culture and smear of themass were negative for tuberculosis.

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Figure 2. Contrast-enhanced CT angiogram image of thechest shows a nonenhancing low-density mass (arrowhead)at the level of tricuspid valve in the right atrium (long arrow).Short arrow = right ventricle.

Considering the prior history of tuberculosis and thecurrent pathologic examination, a diagnosis of tuber-culosis relapse or reinfection was made and the pa-tient was started on standard four-drug antituberculo-sis therapy. Three weeks following medical therapy,the patient’s clinical condition significantly improvedwith resolution of fever and weight gain. Follow-upechocardiography revealed no residual tumor, mod-erate tricuspid regurgitation with normal pulmonaryartery pressure, and normal transtricuspid valve pres-sure gradient. The patient was followed up for twoyears with no evidence of disease recurrence.

DISCUSSION

Cardiac tuberculoma is rare and usually involves thepericardium. Myocardial tuberculosis is an extremelyrare condition and is usually diagnosed at autopsy.1-6

Most of the single or multiple well-circumscribed

Figure 3. Light microscopic image shows an ill-defined gran-uloma with central caseous necrosis (arrows).

cardiac tuberculomas are located in the right heartchambers, particularly in the right atrium. They areusually sharply demarcated from the surroundingparenchyma.7-9 Our case showed extension of themass to the tricuspid valve septal leaflet resulting insevere obstruction of the right ventricular inflow tract.Transthoracic echocardiography can reliably identifymass location, attachments, shape, size, and mobil-ity while transesophageal echocardiography frequentlyadds additional important information to the assess-ment of mass lesions and associated abnormalities.Although biopsy under echocardiographic guidance isa superior diagnostic method, it appears not to be sen-sitive enough to rule out the diagnosis as in our caseand other studies.10,11

Magnetic resonance imaging (MRI) can be obtainedfor the precise localization of the mass, its anatomi-cal relationship to the cardiac chambers, and extensionto the pericardium and contiguous mediastinal struc-tures. The imaging findings on T2W images include:an isointense central core, a hypointense rim, and ringenhancement following contrast administration. MRIfindings are often nonspecific and the differential diag-nosis in such cases includes right atrial myxoma andthrombus.12

The gold standard method for the diagnosis of tu-berculosis is culture isolation of the organism. Mostreports indicate that Ziehl-Neelsen staining has failedto reveal acid-fast bacilli and that the diagnosis restson the typical histologic changes.13 In our patient, con-sidering the previous history of pulmonary tuberculo-sis with cavitation, current sign and symptoms, andpathologic examination, reinfection or relapse of tuber-culosis was diagnosed and supported by response toantituberculosis therapy.

Relapse or reinfection of tuberculosis after medicaltreatment is not uncommon, particularly among pa-tients with a history of cavitary pulmonary tuberculo-sis.14 Our patient showed significant clinical improve-ment following medical treatment. Since most of theprior reports demonstrate that tuberculomas are usu-ally tumor like and most of them are initially misdi-agnosed as a tumor, we suggest that tuberculosis ofthe heart be considered as a differential diagnosis forintracardiac masses particularly in patients with a pre-vious history of tuberculosis.1,10,11,15

Several studies have demonstrated a reduction insize of cardiac tuberculoma after antituberculosis med-ical therapy.1-3,7,10,15-18 Hence, it is recommendedthat pharmacotherapy be considered as a safe anduseful method both to confirm the diagnosis andfor the treatment. Surgical therapy can be mostlyavoided unless pathomorphological diagnosis remainsunclear.11,19 In our case, due to severe tricuspidstenosis, inderterminate results of the percutaneousbiopsy, and an initial diagnosis of angiosarcoma, med-ication therapy was not started prior to the surgery.In conclusion, a case of right atrial tuberculoma wasdescribed. It should be considered as a differential di-agnosis of an intracardiac mass particularly in patientswith a prior history of tuberculosis.

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REFERENCES

1. Licht J, Diefenbach C, Stang A, et al: Tuberculoma ofmyocardium: A rare case of intra-vitam diagnosis. ClinRes Cardiol 2009;98:331-333.

2. Gaultier Y, Alou A, Cenac A, et al: Tuberculoma of theheart. Contribution of echography. Arch Mal Coeur Vaiss1987;80:1413-1416.

3. Immer F, Pirovino M, Saner H: Isolated tuberculosisof the heart: A clinical and echocardiography follow-up.Z Kardiol 1997;86:15-19.

4. Rose AG: Cardiac tuberculosis: A study of 19 patients.Arch Pathol Lab Med 1987;111:422-426.

5. Horn H, Saphir O: The involvement of the myocardiumin tuberculosis: A review of literature and report of threecases. Am Rev Tuberc 1935;32:492-506.

6. Kannangara DW, Salem FA, Rao BS, et al: Cardiac tu-berculosis: TB of the endocardium. Am J Med Sci1984;287:45-47.

7. Vaideeswar P, Pandit SP, Deshpande JR: Tuberculomaof the heart. Cardiovasc Pathol 2006;15:55-56.

8. Claiborne TS: Caseating granulomas of the myocardium.JAMA 1974;39:1081-1086.

9. Kapoor OP, Mascarenhas E, Rananaware M, et al: Tu-berculoma of the heart. Report of 9 cases. Am Heart J1973;86:334-340.

10. Alkhulaifi AM, Carr CS: Right atrial tuberculoma: Com-puted tomography and magnetic resonance imaging.J Thorac Cardiovasc Surg 2007;133:808.

11. Chang BC, Ha JW, Kim JT, et al: Intracardiac tuberculoma.Ann Thorac Surg 1999;67:226-228.

12. Dixit R, Chowdhury V, Singh S. Case report: Myocardialtuberculosis-MRI. Indian J Radiol Imaging 2009;19:57-59.

13. Schnitzer R. Myocardial tuberculosis with paroxysmalventricular tachycardia. Br Heart J 1947;9:213-219.

14. Mandell GL, Bennett JE, Dolin R: Mandell, Douglas, andBennett’s principles and practice of infectious diseases.Elsevier, Philadelphia, 2010.

15. Jagia P, Gulati GS, Sharma S, et al: MRI features of tu-berculoma of the right atrial myocardium. Pediatr Radiol2004;34:904-907.

16. Jeilan M, Schmitt M, McCann G, et al: Images in car-diovascular medicine. Cardiac tuberculoma. Circulation2008;117:984-986.

17. O’Neill PG, Rokey R, Greenberg S, et al: Resolution ofventricular tachycardia and endocardial tuberculoma fol-lowing antituberculosis therapy. Chest 1991;100:1467-1469.

18. Baretti R, Eckel L, Beyersdorf F. An isolated tuber-culoma in the left ventricle. Dtsch Med Wochenschr1994;119:102-106.

19. Batra R, Trehan V, Salwan R, et al: Antemortem diagnosisof cardiac tuberculoma. Indian Heart J 1998;50:87-89.

Acute Bioprosthetic Mitral ValveStenosis in a Patient with HITS

Maninder Sidhu, M.D.,Pankaj Goel, M.Ch., H.P. Singh, M.D.,Arun K. Chopra, M.D., Rajesh Arora, M.D.,and Shailpreet Sidhu, M.D.

Department of Cardiology, Fortis-EscortsHeart and Superspeciality Institute, MajithaVerka By-Pass, Amritsar, Punjab, India

ABSTRACT Acute thrombosis involving the leftatrium and a bioprosthetic valve during the earlypostoperative period is an extremely rare compli-cation of heparin-induced thrombocytopenia syn-drome (HITS). We present a patient with earlybioprosthetic mitral valve stenosis complicated byHITS in a patient with severe mitral regurgitation,atrial fibrillation, and severe left ventricular dys-function. doi: 10.1111/j.1540-8191.2011.01259.x(J Card Surg 2011;26:369-371)

Acute thrombosis involving the left atrium and a bio-prosthetic valve during the early postoperative periodis an extremely rare complication of heparin-inducedthrombocytopenia syndrome (HITS). We present a pa-tient with early bioprosthetic mitral valve stenosis com-plicated by HITS in a patient with severe mitral regurgi-tation, atrial fibrillation (AF), and severe left ventriculardysfunction.

CASE REPORT

A 70-year-old female with history of severe leftventricular (LV) dysfunction, severe mitral regurgita-tion (MR), and chronic AF was admitted with NewYork Heart Association (NYHA) class IV symptoms.Transthoracic echocardiography (TTE) revealed re-gional wall motion abnormality in an area supplied bythe left anterior descending artery (LAD) with severeMR due to apical displacement of the papillary mus-cle. LV ejection fraction was 30% with a pulmonaryartery systolic pressure of 70 mmHg. Coronary an-giography demonstrated 100% chronic total occlusionof LAD. She underwent coronary artery bypass graft-ing using a saphneous vein graft to bypass the LADand simultaneous mitral valve replacement (MVR) witha St Jude’s B100–25M tissue valve (St Paul, MN,USA). A routine TTE on the fourth postoperative dayshowed normal bioprosthetic valve function. She wasstarted on antiplatelet therapy and unfractionated hep-arin (5000 IU s/c thrice daily). On the second post-operative day, oral anticoagulation with warfarin was

Conflict of Interest: None.

Address for correspondence: Maninder S. Sidhu, M.D., Consultant,Department of Cardiology, Fortis-Escorts Heart and SuperspecialityInstitute, Majitha Verka By-Pass, Amritsar, Punjab 143004, India. Fax:0183-2573910; e-mail: maninder1976@hotmail.com

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Figure 1. Transesophageal ultrasound performed on the fifth postoperative day. Midesophageal view in diastole and systoleshowing layered thrombus adherent to left atrial wall and diffuse thrombosis of bioprosthetic mitral valve leaflets. LA = left atrium;RA = right atrium; RV = right ventricle; LV = left ventricle; IAS = interatrial septum; PMV = bioprosthetic mitral valve.

initiated. On the seventh postoperative day, the pa-tient became grossly dyspneic, cold, and sweaty. Thepulse rate was 140/min, systolic blood pressure was80 mmHg, and there were bilateral coarse crepitationswith raised jugular venous pressure. There was a de-cline in platelet count from 230 × 109/L preoperativelyto 55 × 109/L. International normalized ratio (INR) was2.2 and a heparin-induced thrombocytopenia (HIT) anti-body assay was positive. TTE was performed followedby transesophageal echocardiography, which showedlarge immobile thrombi adherent to the left atrial wallextending onto the atrial and ventricular surface ofthe bioprosthetic mitral valve leaflets, causing severevalvular stenosis due to leaflet restriction (Fig. 1). Trans-mitral continuous-wave Doppler showed severe mitralstenosis with estimated mitral valve area of 0.5 cm2

using the pressure half time (PHT), mean transvalvulargradient of 25 mmHg (Fig. 2), and severe pulmonaryhypertension. Heparin was stopped immediately, andintravenous danaparoid sodium, a factor Xa inhibitor,

Figure 2. Doppler on transesophageal ultrasound demon-strating turbulent flow across bioprosthetic mitral valve. Trans-mitral continuous wave Doppler showing severe mitral steno-sis with mitral valve area 0.5 cm2 using pressure half time,and the transvalvular maximum gradient are 26 mmHg andmean 14 mmHg. LA = left atrium; RA = right atrium; RV =right ventricle; LV = left ventricle.

was started. On the ninth postoperative day, the pa-tient was taken back to surgery due to hemodynamicinstability, and a redo MVR was done with a 27-mm,model 500 DM 27, ATS medical mechanical prostheticvalve (St Paul, MN, USA) after removing a large leftatrium (LA) thrombus and the thrombotic tissue valve(Fig. 3). The clot was present on both the atrial andventricular surfaces of the bioprosthesis. It was notpossible to remove all the clots on the ventricular sur-face without removing the entire bioprosthesis. Thesurgery was uneventful, and the patient was weanedoff cardiopulmonary bypass; however, she developedcoagulopathy and continued to bleed requiring multipletransfusions. She worsened over the next two daysand expired due to multi-organ failure.

DISCUSSION

HITS is a syndrome characterized by moderatethrombocytopenia, which is defined as drop in plateletcount to less than 150 × 109/L or a 50% decreasefrom baseline values. It usually develops 5 to 10 daysafter starting heparin therapy and it depends on thetype of heparin used. Its prevalence is approximately

Figure 3. Surgically excised bioprosthetic mitral valve demon-strating heavily thrombosed leaflets.

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1% to 5% with a preponderance for the venoussystem. HIT is a disorder in which platelet activationand thrombosis are caused by antibodies against com-plexes of platelet factor 4 (PF4) and heparin, as a resultof exposure to heparin.1 In patients at risk for HIT,immunoglobulin (Ig) G antibodies are formed againstthe complex of heparin and PF4. The IgG then bindsto the heparin/PF4 complex, forming immune com-plexes. In turn, the IgG/heparin/PF4 immune complexactivates platelets by binding to their Fc receptors.2

Activation of platelets results in the release of procoag-ulant platelet-derived micro particles, and the antigen–antibody complex activates the coagulation cascadeand the endothelial cells, thus releasing tissue factor,all of which lead to the thrombotic complications. Theoptical density (OD) value of the HIT enzyme-linked im-munosorbent assay (ELISA) test may contribute to thediagnostic value of the HIT ELISA test. Zwicker et al.9

found that higher ELISA OD measurements correlatedsignificantly with thrombosis and that patients with anOD level of 1.0 or more had a six-fold increased riskof thrombosis compared with patients who had ODvalues between 0.4 and 0.99.

In this unique case, thrombosis was caused by thecombination of platelet activation by HIT, the unen-dothelialized bioprosthetic material, severe LV dysfunc-tion, and AF. Although left atrial thrombi in a patientwith a bioprosthetic MV and HIT have been described,3

acute bioprosthetic valve dysfunction caused by HIT-induced thrombosis is a rare phenomenon.

Management of such a patient is complicated be-cause severe mitral stenosis causes a vicious cycleof further stasis and thrombosis in the LA causingmore stenosis. There are reports of spontaneous reso-lution of LA thrombus in non-HIT patients;3 the thrombiin these patients were significantly smaller and lim-ited only to the left atrial appendage. The manage-ment of intracardiac thrombus in HIT4,5 depends onthe site of the thrombus and the clinical situation. TheAmerican College of Physicians6 has recommendedthat heparin should be discontinued and anticoagula-tion therapy should be started using direct thrombininhibitors such as lepirudin, argatroban, bivalirudin, ordanaparoid.7 Stopping heparin alone does not protectHIT patients against thrombotic complications. It is esti-mated that 40% to 50% of HIT cases show thromboticcomplications despite cessation of heparin.8 The long-term anticoagulant of choice is warfarin, only afternormalization of platelet counts. For patients receivingwarfarin at the time of diagnosis of HIT, reversal of anti-coagulation is recommended with the use of vitamin K(10 mg po or 5 to 10 mg IV). Starting warfarin as mono-therapy during acute phases of HIT before the normal-ization of the platelet count can induce the venous limbgangrene syndrome. This complication is related to adecrease in the natural anticoagulant protein C and isoften associated with an elevated INR,7 so proper tim-ing for initiating warfarin for HIT treatment should beindividualized. It has been recommended that warfarinuse should be postponed until platelet counts are re-stored with at least five days of adequate anticoagu-lation with alternative agents. Usually, long-term anti-

coagulation is recommended for at least two to threemonths, and stopped only after the LA thrombi hascompletely resolved. In the case of thrombus interfer-ing with the function of the valve, surgical interventionshould be considered. In the present case, it was HIT-associated thrombosis, which is usually more resistantthan other thrombotic conditions. The use of fibrinolytictherapy in such cases can be devastating due to riskof embolism, so slow lysis of the clot using danaparoidsodium and long-term warfarin therapy is the best pos-sible and safest management. Unfortunately, this didnot work favorably in the present case and a high-risksurgical intervention was necessary. Because of ex-tensive clot formation on the ventricular surface of thebioprosthesis, it was not possible to simply declot thevalve, and replacement was necessary.

REFERENCES

1. Arepally GM, Ortel TL: Clinical practice: Heparin-inducedthrombocytopenia. N Engl J Med 2006;355:809-817.

2. Greinacher A, Potzsch B, Amiral J, et al: Heparin-associated thrombocytopenia: Isolation of the antibodyand characterization of a multimolecular PF4-heparin com-plex as the major antigen. Thromb Haemost 1994;71:247-251.

3. Abraham BK, Chow CM, Latter DA, et al: Natural course ofleft atrial thrombi after bioprosthetic mitral valve replace-ment in a patient with heparin-induced thrombocytopenia.Can J Cardiol 2005;21:1307-1308.

4. Corrado G, Tadeo G, Beretta S, et al: Atrial thrombi resolu-tion after prolonged anticoagulation in patients with atrialfibrillation. Chest 1999;115:140-143.

5. Vazquez-Jimenez JF, Jansens U, Sellhaus B, et al: Throm-bosis of a mitral valve prosthesis in a patient with heparininduced thrombocytopenia type 11. J Thorac CardiovascSurg 1999;118:751-753.

6. Jack Hirsh, Gordon Guyatt, Gregory W, et al: AmericanCollege of Chest Physicians evidence-based clinicalpractice guidelines (8th Edition). Chest 2008;133:71S-109S.

7. Warkenten TE, Greinacher A: Heparin induced thrombocy-topenia: Recognition, treatment and prevention: The sev-enth ACCP conference on antithrombotic and thrombolytictherapy. Chest 2004;126(3 Suppl):311S-337S.

8. Wallis DE, Workmen DL, Lewis BE, et al: Failure of earlyheparin cessation as treatment for heparin-induced throm-bocytopenia. Am J Med 1999;106:629-635.

9. Zwicker JI, Uhl L, Huang WY, et al: Thrombosis andELISA optical density values in hospitalized patients withheparin-induced thrombocytopenia. J Thromb Haemost2004;2:2133-2137.

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Repair of Intervalvular FibrousBody Rupture During AorticValve Replacement

Abdallah K. Alameddine, M.D.,∗†Victor K. Alimov, M.D.,∗and Joseph E. Flack, M.D.∗

∗Division of Cardiac Surgery, BaystateMedical Center, Springfield, Massachusetts;and †Department of Surgery, Tufts MedicalSchool, Boston, Massachusetts

ABSTRACT The intervalvular fibrosa, the structurethat connects the mitral and the aortic valves,can be injured during aortic valve replacement. Al-though this complication is rare, it may requirecomplex and extensive repair. Herein, we describemanagement of a damaged aortic-mitral curtainand fibrous body during tissue aortic valve replace-ment in a patient with mixed connective tissuedisorder. A “U” suture repair combining an ex-ternal and internal reinforcement repair techniquevia the aorta, without explanting the prostheticvalve technique, is described. The purpose of us-ing the “externalized” buttressed U-stitch was toobliterate any residual cavity and to assure con-trol of hemorrhage externally. Repair of damagedfibrous body during aortic valve replacement (AVR)is challenging. We managed this difficult situa-tion in a satisfactory surgical approach without ex-planting the aortic prosthesis. doi: 10.1111/j.1540-8191.2011.01269.x (J Card Surg 2011;26:372-374)

CASE REPORT

A 54-year-old female with nonsignificant coronaryartery disease on cardiac catheterization was sched-uled for a minimally invasive aortic valve replacement(AVR) because of severe aortic stenosis. Her echocar-diogram showed an aortic valve area of 0.6 cm2 anda transaortic mean gradient of 70 mmHg. Her medi-cal history included Sjogren’s syndrome, Hashimoto’sthyroiditis, rheumatoid arthritis, and celiac disease. Un-der cardiopulmonary bypass (CPB) with moderate hy-pothermia, a partial “L” sternotomy was performed inthe right fourth intercostal space using a 3′′ skin inci-sion. Exploration revealed a severely stenotic bileaflet

Conflict of interest: There are no conflicts of interest for any authors.

Address for correspondence: Abdallah K. Alameddine, M.D.,Division of Cardiac Surgery, Baystate Medical Center, 759 Chest-nut Street, Springfield, MA 01107 and Department of Surgery, TuftsMedical School, Boston, MA. Fax: (413) 794-4212; e-mail: akalamed-dine@gmail.com

valve with fibrosis and calcification. A size 21 MagnaEase bioprosthetic valve (Edwards Lifesciences, Irvine,CA, USA) was seated using 12 noneverting Teflon feltpledgetted sutures around the annulus. The patientwas weaned-off CPB without difficulty. However, uponthe removal of the pulmonary vein vent, a large amountof bleeding was noted coming from behind the ascend-ing aorta. When CPB was reinstituted, a 2-cm tear wasvisualized in the left atrial dome adjoining the aorticroot. Despite placement of several pledgetted suturesin this area, full hemostasis was not achieved.

Based on this, a transesophageal echocardiogram(TEE) was done that showed disruption of the fibrouscontinuity between the aortic and mitral valves. Furtherinvestigation using color Doppler demonstrated a smallcavity, lateral to the aortic root, extending out towardthe dome of the left atrium with free communicationwith the left ventricular outflow tract; see Panels A, B,and C in Figure 1.

For those reasons, the mini-sternal incision wasconverted to a full sternotomy, the cross-clamp wasreapplied, and the aorta reopened. After the pros-thetic leaflets were retracted, there was an entrancetear consisting of a 1.5-cm separation of the fibroustrigone 2 to 3 mm beneath the aortic annulus wherea prosthesis suture had torn through. Two Teflon feltpledgetted stitches of 2-0 Ethibond were used frominside the aorta to close the defect bringing the ante-rior mitral valve leaflet up to the prosthetic cuff. Thenfrom inside the aorta, a U-stitch was added. The nee-dle of this stitch went through the base of the ante-rior mitral leaflet, through the residual aortic rim belowthe prosthesis, and then was anchored externally onthe left atrial dome adjacent to the aortic root. Thusthe mitral leaflet was brought up to the residual aorticrim that was then externalized to buttress the uppertear as well (see Fig. 2A ).

The original cross-clamp time was 155 minutes,which included both the clamp time for the AVR(90 min) as well as the clamp time needed for the initialattempt to control the bleeding exit site located at thedome of the LA (65 min). The additional cross-clampand bypass times needed for the final repair were54 minutes and 80 minutes, respectively. The totalbypass time was 240 minutes. The patient was dis-charged on postoperative day 17. The pathologicalanalysis of the resected aortic leaflets showed myx-ofibrotic degenerative changes and calcification but nolymphocytic infiltrate was identified.

Transthoracic echocardiography and computed to-mography of the chest seven months after AVRshowed good prosthetic and mitral valve functions withno evidence of pseudoaneurysm formation.

DISCUSSION

The heart is one of the many organs that can beaffected by a variety of connective tissue diseases in-cluding Sjogren’s syndrome.1-3 As a consequence oftissue friability due to the inflammatory process3 or to

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Figure 1. Cardiac images. Mid-transesophageal three-chamber views acquired after the aortic valve bioprosthesis was implanted.Panel A shows the presence of a disruption of the fibrous continuity between the aortic annulus and the anterior mitral leafletposteriorly (arrow). Color Doppler in panel B (during systole) and in panel C (during diastole) confirms free flow from the leftventricular outflow tract (LVOT) into the cavity between the aortic root and left atrial (LA) dome as a consequence of thefirst repair. Panel D shows the postrepair study. There was no longer communication with the LVOT (arrow). AOR = aorticroot.

corticosteroids therapy, patients with these disordersmay have a predisposition to tissue dehiscence duringaortic valve replacement.

The intervalvular fibrosa is a structure where the leftatrial dome, the aortic root, and the anterior leaflet ofthe mitral valve conjoin.4 Normally, the area below theaortic root is densely attached to the left atrial dome. Inour case, we believe that a combination of factors hascontributed to this potentially fatal injury including thelimited exposure, excessive tissue retraction for bet-ter visualization, and perhaps disproportionate annulardebridement.

TEE was crucial in determining the precise locationof the intractable bleeding, as shown in Figure 2B.

We selected a definitive internal repair, an approachakin to the repair of the left ventricular rupture follow-ing mitral valve replacement, but without removing theprosthetic device to minimize the perfusion time.

The benefit of the “externalized” buttressed U-stitchwas to obliterate the residual cavity, decrease the ten-sion and stress in this area, and to prevent externalbleeding. The early outcome of this technique was fa-

vorable and without sequelae. Although the pathologyof the index case reported in the article was a systemicconnective tissue disease, damage to the fibrous bodyduring AVR is not unique to this illness.5 Thus, it re-mains to be determined under what circumstances thistechnique can be applied to a larger group of patientswith similar situations.

We believe that in younger patients with absence ofinfection and with a localized tearing (as in this patient),and no significant tissue friability this technique can beadapted. For larger and complex tears, the valve mustbe removed, and the fibrous trigone reconstructed witha patch of Dacron fabric or bovine pericardium as usedfrequently in patients with bacterial endocarditis.6

In retrospect, the limited exposure from the “min-imally invasive” incision contributed to this com-plication. Patients with connective tissue disordersundergoing AVR might be better served with a full ster-notomy to avoid excessive traction on the annulus andthe fibrous trigone.

In conclusion, in patients with autoimmune pro-cesses, bleeding of the aortic root following AVR can

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Figure 2. Schematic drawing of the intraoperative repair. Panel A: The left ventricular outflow tract is shown throughthe leaflets of the aortic bioprosthesis (P); the leaflets and a segment of the sewing ring are not shown for better clar-ity. Complete closure (capittonage) of the residual cavity behind the aortic root and reattachment of the aortic root arecompleted by an additional pledgetted U-stitch. The needle of this stitch went through the anterior mitral leaflet, throughthe residual aortic rim below the prosthesis, and then was anchored through the roof of the left atrial dome. T1 ar-row = upper, exit tear; T2 arrow = lower, entrance tear. Anatomically, the distance between T1 and T2 is <10 mm.Panel B: drawing shows the path of the bleeding through the intervalvular fibrous body (FB).

result from disruption of the fibrous continuity betweenthe aortic and mitral valves. In selected situations, inter-nal repair can be considered as a viable option, withoutremoving the aortic bioprosthesis. However, long-termfollow-up is needed to evaluate this technique beforecardiac surgeons adopt this alternative method to theirsurgical armamentarium.

Acknowledgments: The authors wish to thank Dr. Leng Jiangof Cardiology Department for providing the echocardiographicimages, Todd A. Lajoie for the artwork, and the editorial as-sistance of Anthony Desjardins.

REFERENCES

1. Vassiliou VA, Myossakis I, Boki KA, et al: Is the heartaffected in primary Sjogren’s syndrome? An echocar-diographic study. Clin Exp Rheumatol 2008;26:109-112.

2. Alameddine AK, Schoen FJ, Yanagi H, et al: Aortic or mitralvalve replacement in systemic lupus erythematosus. AmJ Cardiol 1992;70:955-956.

3. Bridge K, Farivar RS. A case of Sjogren’s syndrome leadingto mitral and aortic valve replacement. J Thorac CardiovascSurg 2010;139:e139-e140.

4. Anderson RH. Clinical anatomy of the aortic root. Heart2000;84:670-673.

5. De Oliveira NC, David TE, Armstrong S, et al: Aortic andmitral valve replacement with reconstruction of the inter-

valvular fibrous body: An analysis of clinical outcomes. JThorac Cardiovasc Surg 2005;129:286-290.

6. David TE, Kuo J, Armstrong S. Aortic and mitral valvereplacement with reconstruction of the intervalvular fi-brous body. J Thorac Cardiovasc Surg 1997;114:766-772.

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Atrial Myxoma and BoneChanges: A ParaneoplasticSyndrome?

Marcus J. Smith, M.D.,∗Muhammad A. Chaudhry, M.D.,∗Mary Beth Humphrey, M.D., Ph.D.,†and Pedro M. Lozano, M.D.∗

∗Department of Internal Medicine, Sectionof Cardiovascular Disease, University ofOklahoma Health Sciences Center,Oklahoma City, Oklahoma; and†Department of Internal Medicine, Sectionof Rheumatology, Immunology and Allergy,University of Oklahoma Health SciencesCenter, Oklahoma City, Oklahoma

ABSTRACT Atrial myxomas are the most commonbenign tumors of the heart and are difficult to di-agnose due to a wide variety of presenting symp-toms. We present a patient with a five-year his-tory of visual loss, vertigo, ataxia, tinnitus, andbone lesions that resolved after diagnosis and re-section of an atrial myxoma. This case not onlyhighlights an unusual presentation of atrial myxo-mas but also raises the question of whether atrialmyxomas can produce paraneoplastic syndromes,including bone abnormalities. doi: 10.1111/j.1540-8191.2011.01277.x (J Card Surg 2011;26:375-377)

Atrial myxomas frequently present with neurolog-ical symptoms secondary to embolic events; however,skeletal abnormalities are rare. We present an unusualcase of an atrial myxoma presenting with recurrentneurological symptoms as well as boney lesions. Themajority of boney lesions resolved with resection ofthe primary myxoma suggesting that these lesions areparaneoplastic manifestations of the myxoma.

CASE REPORT

A 64-year-old African American male, with a pastmedical history of hypertension, was admitted with afive-year history of intermittent vertigo, tinnitus, ataxia,and visual loss. These symptoms lasted 7 to 8 min-utes, each with sudden appearance and spontaneousresolution. They occurred several times a year but inthe previous months the symptoms began occurringon a daily basis.

Conflict of interest: None of the authors have any conflict of interest,financial or otherwise.

Address for correspondence: Marcus Smith, M.D., CardiovascularMedicine WP 3010, University of Oklahoma Health Sciences Cen-ter, Oklahoma City, OK 73104. Fax: 405-271-3521; e-mail: marcus-smith@ouhsc.edu

Several months earlier, the patient had presented tohis primary care physician’s office with these symp-toms. A computed tomography (CT) scan of the brainwas negative for any acute or chronic stroke. However,a thickened calvarium with a mottled density was notedraising the concern for a malignant process. A bonescan confirmed increased activity in the calvarium aswell as the right scapula and left femur. CT scans of thechest, abdomen, and pelvis revealed a circumscribedsoft tissue defect in the left atrium (Fig. 1).

The patient was admitted for further evaluation ofa left atrial mass and a transthoracic echocardiogram(TTE) showed a large echogenic mobile mass that mea-sured 4.3 × 3 cm in the left atrium, raising the proba-bility of a left atrial myxoma (Fig. 2). The patient under-went resection of the left atrial mass on the third dayof hospitalization. The mass was noted to be attachedto the septum secundum (Fig. 3). A portion of the in-teratrial septum was resected with the mass but didnot require an atrial patch. The pathology ultimatelyconfirmed the diagnosis of atrial myxoma (Figs. 4and 5).

At a six-month follow-up the patient reported the in-termittent visual loss, vertigo, tinnitus, and ataxia hadresolved. A bone scan performed three-months afterthe myxoma resection revealed resolution of the in-creased activity in the right scapula and left femur.The increased activity in the calvarium persisted andprompted additional malignancy evaluation. Results ofthe evaluation included a negative serum and urine pro-tein electrophoresis (SPEP/UPEP), as well as normalhemoglobin, creatinine, alkaline phosphatase, calcium,and serum kappa and lambda light chain ratio. Basedon these data, the calvarial lesion was believed to benonmalignant. The patient declined biopsy of his skulland is being followed closely for changes in this lesion.

DISCUSSION

This case is an example of an extremely unusualpresentation of atrial myxoma with symptoms ofintermittent visual loss, vertigo, tinnitus, and bonechanges. These symptoms resolved with the resec-tion of the tumor. Given the sporadic nature of thepatient’s symptoms, coupled with the fact that therewere no signs of acute or chronic stroke on brain imag-ing, these symptoms cannot be explained by embolicphenomenon, but rather by systemic manifestations ofa paraneoplastic process.

A paraneoplastic process has been described in pre-vious studies and has shown an association betweenatrial myxomas and vasculitis, amyloidosis, nephriticsyndrome, systemic lupus erythematosus, and de-myelinating neuropathy.1-5 Elevations of interleukin6 (IL-6), tumor necrosis factor, and vascular endothe-lia growth factor (VEGF) have also been linked to atrialmyxoma and may play a part in the production of sys-temic manifestations.6-8

Skeletal metastases of cardiac myxomas is an ex-tremely rare event with very few cases reported in theliterature. Skeletal sites of metastases have includedthe humerus, pelvis, femur, scapula, tibia, and skull.

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Figure 1. CT scan (axial view) showing left atrial myxoma (arrow).

Figure 2. TTE (apical four chamber view) showing left atrial myxoma (arrow).

In each of these cases, biopsies proved that thesebone lesions were indeed myxomas and all were ul-timately excised.9-12 The possibility remains that thepersistently thickened calvarium seen in this case mayrepresent a metastatic myxoma. The rapid resolutionof the scapular and femur changes, however, points toanother mechanism besides metastasis that may beresponsible for these changes.

CONCLUSION

Bone changes in conjunction with intermittent visualloss, vertigo, and tinnitus appear to represent possi-ble systemic manifestations of atrial myxomas. In thiscase, these symptoms all resolved with the removalof the atrial myxoma. This gives further evidence tothe thought that atrial myxomas not only exert theireffect by the local presence of the tumor but also by

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Figure 3. Intraoperative TEE showing left arial myxoma (arrow).

Figure 4. Gross speciman of left atrial myxoma.

Figure 5. Myxoma cells surrounding dilated vessels and scat-tered in a myxoid matrix.

release of humoral factors. Further studies need to bedone to determine any additional cytokines that maybe contributing to the systemic manifestation seen inatrial myxomas.

REFERENCES

1. Feldman A, Keeling III J. Cutaneous manifestations ofatrial myoma. J Am Acad Dermatol 1980;21(5):1080-1084.

2. Molstad P, Smith G, Aukrust P. Left atrial myxoma andsystemic AL amyloidosis. Eur Heat J 1992;13(1):143-144.

3. Kalra PA, Raghavan C, Hassan R, Lawson R, et al:Nephrotic range proteinuria associated with right atrialmyxoma. Clin Nephrol 1993;39(2):115.

4. Marmade L, Tribak M, Laaroussi M, et al: Lupus ery-thematosus misleads left atrial myxoma’s diagnosis. RevMed Interne 2006;27(5):424-426.

5. Santangeli P, Pieroni M, Marzo F, et al: Cardiac myx-oma presenting with sensory neuropathy, Int J Cardiol2010;143(1);e14-e16.

6. Mendoza CE, Rosado MF, Bernal L. The role of interleukin6 in cases of cardia myxoma: Clinical features, immuno-logic abnormalities and a possible role in recurrence. TexHeart Inst J 2001;28(1):3-7.

7. Franchimont N, Wertz S, Malaise M. Interleukin-6: Anosteotropic factor in bone formation. Bone 2005;37:601-606.

8. Ausakamoto H, Sakamaki T, Kanda T, et al: Vascular en-dothelial growth factor is an autocrine growth factor forcardiacmyxoma cells. Circ J 2004;68(5):488-493.

9. Jungreis CA, Sekhar LN, Martinez AJ, et al:Cardiac myxoma metastatic to temporal bone. Radiology1989;170:244.

10. Moran C, Braunstein EM, Ulbright T, et al: Case report689. Metastatic atrial myxoma to the pubis. Skeletal Ra-diol 1991;20:465-467.

11. Rupp GM, Heyman RA, Martinez AJ, et al: The pathol-ogy of metastatic cardiac myxoma. Am J Clin Pathol1989;91:221-227.

12. Uppin SG, Jambhekar N, Puri A, et al: Bone metastasisof glandular cardiac myxoma mimicking a metastatic car-cinoma. Skeletal Radiol 2011;40(1):107-111.

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Unusual Association of LeftVentricular Diverticulum andHypertrophic Cardiomyopathyin an Adult

Francesco Onorati, M.D., Ph.D.,∗Daniela Bacich, M.D.,†Enrico Amoncelli, M.D.,∗Francesco Santini, M.D.,∗Pietro Martire, M.D.,†Alessandro Mazzucco, M.D.,∗and Giuseppe Faggian, M.D.∗

∗Division of Cardiac Surgery, University ofVerona Medical School, Verona, Italy;and †Cardiology Unit, “Madonna dellaSalute” Hospital, Porto Viro, Italy

ABSTRACT A 68-year-old female with hyper-trophic obstructive cardiomyopathy (HOCM), fi-brous subaortic stenosis, mitro-aortic valveinsufficiencies, and congenital left ventricular di-verticulum (CLVD) at apical level was diagnosedafter syncope. Although the association betweenHOCM and CLVD has been previously reported, nocase has ever been disclosed in advanced adult-hood. doi: 10.1111/j.1540-8191.2011.01279.x (JCard Surg 2011;26:378-380)

Congenital left ventricular diverticulum (CLVD) is arare disease affecting 0.04 to 0.4% of the cardiac sur-gical population.1 Although CLVD is often associatedwith congenital anomalies of the sternum, diaphragm,pericardium, and abdominal wall (so-called Cantrell syn-drome), it can be rarely diagnosed as an isolated malfor-mation.2,3 Occasionally, CLVD is associated with othercongenital heart diseases.2 Of these, the associationwith hypertrophic obstructive cardiomyopathy (HOCM)seems very rare, having been reported in only threecases.4 Given the congenital nature of the disease,CLVD is almost always considered a disease of thenewborn, the infant or, at the most, the young. No caseof extremely delayed diagnosis, as in the 7th decadeof life, has ever been reported.

CASE REPORT

A 68-year-old female was admitted at our Insti-tution because of severe dyspnea with recurrent

Disclosure: The authors disclose that they have no conflicts of interest,financial or otherwise, with any of the products mentioned herein.

Address for correspondence: Giuseppe Faggian, M.D., Divi-sion of Cardiac Surgery, University of Verona Medical School,Piazzale Stefani 1, 37126 Verona, Italy. Fax: 0039-045-8123308; e-mail:giuseppe.faggian@univr.it

episodes of palpitations and a previous history ofacute pulmonary edema. She had been diagnosedwith HOCM and severe subvalvular aortic stenosissince 1985, when surgical correction was refused.In 2005 a follow-up transthoracic echocardiographyconfirmed HOCM with moderate subaortic mid-ventricular stenosis, but also revealed mild mitralregurgitation and a small (0.8 × 0.5 cm) CLVD atthe level of the apex. Surgery was again refused.Since then progressive dyspnea with signs andsymptoms of congestive heart failure developed.On admission, physical examination demonstratedparoxysmal atrial fibrillation (150 beats/min), systemichypertension (blood pressure of 130/100 mm/Hg),a systolic murmur at the base of the heart, andbilateral basal rales. ECG further demonstrated com-plete left bundle branch block. Chest X-ray disclosedcardiomegaly and pulmonary edema. Transthoracicechocardiography disclosed HOCM, a hypertrophiedleft ventricle, and a large cavity (5 × 4 cm) at the levelof the left ventricular apex communicating with themain left ventricular chamber through a narrow neck(0.8 cm). Doppler analysis showed severe subaorticstenosis (mean gradient of 55 mm/Hg), moderateaortic valve regurgitation, and moderate-to-severemitral regurgitation, related to the restricted motion ofthe anterior mitral leaflet, and the right coronary aorticcusp. Magnetic resonance imaging (MRI) showedHOCM and a large apical bulging (5.6 × 4.5 × 5.8 cm)synchronously contracting with the left ventricle(Fig. 1A). Delayed gadolinium-enhanced imaging didnot show intramural myocardial fibrosis or intraluminalthrombosis, confirming CLVD (Fig. 1B). No signs ofprevious myocardial infarction nor inflammation weredemonstrated at MRI. Preoperative coronary angiog-raphy confirmed the absence of any coronary disease.

Surgery was performed on full cardiopulmonarybypass with aortic cross-clamping (126 and 92 min,respectively) and blood cardioplegia. The procedureconsisted of septal myectomy, resection of thesubaortic fibrous curtain with full mobilization of theanterior mitral leaflet and of the right coronary aorticcusp, subcommissural aortic valve repair with threeinterrupted 4-0 polypropylene buttressed sutures,mitral annuloplasty with an autologous pericardialband, and CLVD excision with obliteration of theentry site by double 3-0 polypropylene stitching.Intraoperative macroscopic evaluation demonstratedthe muscular nature of the CLVD and the absenceof thrombi. The patient was successfully weanedfrom cardiopulmonary bypass. Intraoperative trans-oesophageal echocardiography demonstrated goodmitro-aortic valve function, trivial residual subaorticgradient (mean �p of 8 mm/Hg), the absence of mitralsystolic anterior motion, and the complete exclusionof CLVD. Pathological exam confirmed the “muscular”nature of the CLVD with the absence of endocardialscar. Pathological results of both diverticular and sep-tal specimens also confirmed the typical myocardialdisarray, with complete and complex whorl formation,glycogen accumulation in myocytes, and plexiformfibrosis.

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Figure 1. Basal MRI (A) demonstrated a 5.6 × 4.5 × 5.8 cm apical bulging (white arrow). Delayed gadolinium-enhanced imaging(B) showed transmural enhancement of the mass without intramural myocardial fibrosis or intraluminal thrombosis (arrow),consistent with a “muscular” type CLVD.

The postoperative course was uneventful and thepatient was discharged home on the 10th postopera-tive day. Eight-months clinical follow-up demonstratedclass I symptoms and a stable sinus rhythm. Follow-upechocardiography at 4th and 8th postoperative monthsdemonstrated normal septal thickness (10 mm), nor-mal mitral valve function (PHT 2.5 cmq, mean gradient4 mmHg), and subaortic maximum and mean gradientof 22 and 10 mm/Hg, respectively.

DISCUSSION

Cardiac diverticula are rare findings, consisting of a“true” protrusion of the endocardium and of the my-ocardium through a vulnerable portion of the free wallof the left or right ventricle.4 Although traditionally clas-sified into “muscular” type or “fibrous” type accord-ing to the histological nature of protruding tissue, the“muscular” nature is the only type unequivocally con-sidered “congenital” in its nature.4 Accordingly, thecongenital nature of the “muscular” type CLVD ex-plains the common association with other cardiac andextracardiac malformations.1-4 Of these, the associa-tion with HOCM has been described to date only inthree patients, two infants and one young male.4 Tothe best of our knowledge, this is the only report de-scribing this association in the elderly.4 Given the con-genital nature of this disease, it is almost always di-agnosed in the first years of life, and very rarely at ayoung age.1,2,4,5 Our case, however, proves that theactual occurrence of CLVD might be underestimated,and it highlights the potential occurrence also in theadulthood, including the late 7th decade of life.

CLVD has been described to potentially developanywhere in the left or right ventricles, and to be rarelylocalized at the level of the apex.1-3,5,6 However, all thethree cases associated with HOCM reported by Maronhad an apical component.4 The apical localization mayalso help to elucidate the pathogenesis of this rarecondition.1-6 Although CLVD of Cantrell’s pentalogyis postulated to be due to a developmental failureof the appropriate segments of the mesoderm, atthe time of its differentiation into the splanchnic andsomatic layers during the embryonic life2,7 thoseassociated with HOCM are thought to be consideredof different origin.4 In particular, the apical localizationof CLVD associated with HOCM with severe subaorticmid-ventricular severe stenosis suggests a “hemody-namic” etiology.4 The extremely high pressure in theapical portions of the left ventricle causes a bulgingof the thinnest and most vulnerable portion of theventricle itself, that is, the apex.4 The present caseseems to confirm this hypothesis, given the smalldimensions of the CLVD at early echocardiographyperformed 5 years before surgery. Moreover, theprogression of symptoms and the advanced age of thepatient at the time of the diagnosis seem to supportthe “progressive” and “hemodynamic” origin of CLVDassociated with HOCM. The absence of B-mode atthe time of the first echocardiography in 1985, or thepossibility of very small dimensions of the CLVD atthat time, could have misled to a missed diagnosis.

There is general agreement that symptomatic leftventricular diverticula should be treated by means ofsurgical intervention, whereas resection of asymp-tomatic diverticula is a matter of controversy.6 CLVD

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can potentially cause mitral insufficiency, as describedhere, can rupture spontaneously, or can trigger fatal ar-rhythmias, especially those of the “muscular” type.6

The potential “progressive” nature of CLVD associ-ated with HOCM, as in the case reported, should alertphysicians that this rare disease can also occur in thelast decades of life and that surgical intervention maybe indicated in order to avoid potential life-threateningcomplications.

REFERENCES

1. Okayama S, Uemura S, Soeda T, et al: Congenital isolatedbiventricular diverticula evaluated by cardiac computed to-mography and magnetic resonance imaging. Heart LungCirc 2010;19:630-632.

2. Herman TE, Siegel MJ: Cantrell syndrome. J Perinatol2010;30:298-299.

3. Pome G, Vignati G, Mauri L, et al: Isolated congeni-tal left ventricular diverticulum. Eur J Cardiothorac Surg1995;9:709-712.

4. Maron BJ, Hauser RG, Roberts WC: Hyperthropic car-diomyopathy with left ventricular apical diverticulum. AmJ Cardiol 1996;77:1263-1265.

5. Oruganti SS, Ayapati DR, James MA, et al: Congenitalgiant diverticulum of the left ventricle in an adult. AsianCardiovasc Thorac Ann 2005;13:79-81.

6. Paz Y, Fridman E, Shakalia FM, et al: Repair of anisolated huge congenital left ventricular diverticulum. JThorac Cardiovasc Surg 2004;128:313-314.

7. Pivnick EK, Kaufman RA, Velagaleti GV, et al: Infant withmidline thoracoabdominal schisis and limb defects. Tera-tology 1998;58:205-208.