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modalities employed in Gorham’s disease are surgery andradiation therapy.2

Acknowledgment

Thank you to Professor K. Krishnan Unni at the MayoClinic in Rochester, Minnesota, USA, for reviewing the his-tology in this case. This case report required no externalfinancial assistance.

References

1. Gorham WL, Stout AP. Massive osteolysis (acute spon-taneous absorption of bone, phantom bone, disappearingbone): its relation to hemangiomatosis. J Bone Joint Surg Am1955;37A:985–1004.

2. Patel DV. Gorham’s disease or massive osteolysis. Clin Med Res2005;3:65–74.

3. Duffy BM, Manon R, Patel RR, Welsh JS. A case of Gorham’sdisease with chylothorax treated curatively with radiation ther-apy. Clin Med Res 2005;3:83–6.

4. Jackson JBS. A boneless arm. Boston Med Surg J 1838;18:368–9.5. Devlin RD, Bone 3rd HG, Roodman GD. Interleukin-6: a

potential mediator of the massive osteolysis in patients withGorham-Stout disease. J Clin Endocrinol Metab 1996;81:1893–7.

6. Heyden G, Kindblom LG, Nielsen JM. Disappearing bone dis-ease: a clinical and histological study. J Bone Joint Surg Am1977;59-A:57–61.

7. Colucci S, Taraboletti G, Primo L, Viale A, Roca C, Valdembri D,Geuna M, Pagano M, Grano M, Pogrel AM, Harris AL, Athana-sou NN, Mantovani A, Zallone A, Bussolino F. Gorham-Stoutsyndrome: a monocyte-mediated cytokine propelled disease. JBone Miner Res 2006;21:207–18.

8. Tie ML, Poland GA, Rosenow 3rd EC. Chylothorax in Gorham’ssyndrome. A common complication of a rare disease. Chest1994;105:208–13.

Coronary Artery Aneurysms Associatedwith a Paclitaxel Coated Stent

Dennis Wang, MBBS, Brendan Gunalingam, FRACP, FCSANZ, FSCAI ∗,

A

Department of Cardiology, Gosford Hospital, Holden Street, Gosford, NSW 2250, Australia

Coronary artery aneurysm associated with the Paclitaxel drug eluting stent (Taxus) is a rare complication. We describethe case of a 71-year-old female, who developed two coronary artery aneurysms in her right coronary artery associatedwith the insertion of a Taxus stent. Although no adverse clinical outcome resulted, this case highlights a potential problemgoing forward.

(Heart, Lung and Circulation 2008;17:62–79)© 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and

New Zealand. Published by Elsevier Inc. All rights reserved.

Keywords. Coronary artery aneurysm; Drug eluting stent; Paclitaxel; Taxus

Case Report

71-year-old female presented to hospital in April 2004with a 24 h history of ischaemic chest pain associ-

ated with nausea and diaphoresis. An ECG performed onadmission revealed sinus rhythm with inferior Q wavesand T wave inversion. Admission Trop I was elevated at3.83 ng/ml. Her background medical history included non-insulin dependant diabetes mellitus, hyperlipidaemia andhypertension. She was also an ex-smoker and drank alco-hol socially.

The provisional diagnosis was that of a non ST elevationinfarct and she was commenced on medical therapy which

Received 24 January 2007; received in revised form 14 December2006; accepted 16 February 2007; available online 6 April 2007

∗ Corresponding author. Tel.: +61 243233060; fax: +61 243237062.E-mail address: gunalingam@bigpond.com (B. Gunalingam).

included Aspirin, Clopidogrel, Metoprolol and thera-peutic intravenous Heparin. A coronary angiogram wassubsequently arranged.

Cardiac catheterisation revealed triple vessel diseasewith an estimated left ventricular ejection of 35% as a resultof anterolateral akinesis. The left main artery showed a dis-tal 50% stenosis. The left anterior descending (LAD) arterywas occluded after a very large septal branch which hadan ostial 95% lesion. The left circumflex (LCx) artery was anon dominant vessel with a 90% stenosis at its origin. Theright coronary artery (RCA) was a dominant vessel withdiffuse disease and subtotal mid-vessel occlusion. (Fig. 1)The recommendation at the time was to refer this lady forcoronary artery bypass grafting surgery.

However during her hospital admission she had ongo-ing post-infarct angina and after much discussion, went onto have coronary intervention to her right coronary artery,

© 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society ofAustralia and New Zealand. Published by Elsevier Inc. All rights reserved.

1443-9506/04/$30.00doi:10.1016/j.hlc.2007.02.093

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Figure 1. Subtotal RCA occlusion on initial diagnostic angiogram.

which was considered to be the culprit artery. Myocardialviability in the LAD and LCx territories was in question,so PCI to the RCA was felt to be appropriate in the firstinstance.

The previously identified subtotally occluded rightcoronary artery was predilated with a 2.25 mm × 20 mmballoon and subsequently stented with a Taxus 3.0 mm ×28 mm stent (Fig. 2a and b). At the end of the procedurethere was no significant residual stenosis.

This lady was successfully discharged from hospital sev-eral days after the procedure. At a routine three monthfollow-up, she complained of further chest and left armpain and hence a myocardial perfusion study was per-

formed to determine the extent of residual reversibleischaemia. This study revealed a left ventricular ejectionfraction of 34%, with anteroseptal and apical infarction andminimal peri-infarctional apical ischaemia.

Routine follow-up at the end of November 2004 revealedthat she had had a further hospital admission with decom-pensated left ventricular failure, without evidence offurther infarction or ischaemia.

Given that this lady was a diabetic with known triplevessel disease and moderately severe left ventriculardysfunction, a decision was made to refer her for coro-nary artery bypass grafting surgery. Despite the lack ofsignificant reversible ischaemia on the perfusion scan,ongoing symptoms justified this strategy. Another coro-nary angiogram was arranged to reassess her coronaryanatomy, especially her right coronary artery which hadbeen stented seven months earlier.

An outpatient coronary angiogram performed in Jan-uary 2005, nine months after the original stent insertionrevealed that her left coronary anatomy was essentiallyunchanged from the original diagnostic angiogram. How-ever, in the right coronary artery, at the location ofthe Taxus stent, two coronary aneurysms had developed(Fig. 3). These aneurysms were not present on the originaldiagnostic angiogram, or the subsequent interventionalprocedure. The aneurysms were located in the proximaland mid segments of the Taxus stent. No further percuta-noro

D

Du

F raphi

igure 2. (a) Taxus stent in RCA prior to deployment. (b) Final angiog

eous intervention was undertaken in this lady, who wentn to have successful coronary artery bypass grafting. Theight coronary artery aneurysm was left alone at the timef surgery.

iscussion

e novo coronary artery aneurysms constitute a relativelyncommon clinical entity, defined by a vessel diameter

c result after deployment of Taxus stent.

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68 Case Reports Heart, Lung and Circulation2008;17:62–79

Figure 3. (a) Follow-up angiogram demonstrating the two coronary aneurysms at the site of the Taxus stent (arrows) (b) RAO view of RCAdemonstrating the two coronary artery aneurysms (arrows).

1.5–2 times the reference vessel. In an autopsy series byDauod et al., the incidence of coronary artery aneurysmwas 1.4% in 694 patients.1 In the CASS (Coronary arterysurgery study) registry, the angiographic incidence ofthis abnormality was 4.9% in a group of 20,087 patients.2

Potential complications associated with these abnormali-ties include thrombus formation, distal embolisation and,rarely, rupture, although the prognosis is not well known.This case highlights this rare complication of coronaryaneurysm formation after insertion of a Paclitaxel coatedstent (TAXUS).

Paclitaxel is an anti-neoplastic drug from the taxaneclass. It acts to block cells in the G2 phase and M phase ofthe cell cycle thereby inhibiting cell replication.

Neointimal hyperplasia resulting in restenosis afterinsertion of coronary stents results in recurrent anginaoften requiring percutaneous or surgical revascularisa-tion. Use of sirolimus and paclitaxel drug elution stentshas resulted in significant reductions in angiographic andclinical restenosis rates.3

The use of the Paclitaxel coated stent has resulted in sig-nificantly lower restenosis rates compared with bare metalstents, 4% versus 27%, respectively at six months.3 Theseresults are comparable to that involving the Sirolimuscoated stent (CYPHER).4

This rare complication involving a Taxus stent wasdescribed in clinical trials prior to market release in the

However, in clinical trials of the Cypher and Taxusstents, the angiographic follow-up was eight and ninemonths, respectively, with further clinical and telephonefollow-up only extending out to five years. Outside thesetting of these clinical trials there have been isolated casereports in the literature about this specific complication ofdrug eluting stents.6–12

The clinical significance of this finding is yet to be deter-mined, since these stents have only been on the market forless than five years. No doubt, as the use of drug elutingstents increases in the years to come, the clinical signifi-cance of these findings will become clearer.

Of note, if the true incidence of aneurysm formationis increased with drug eluting stents, it may retard theirwidespread use as there is no mortality advantage withthese stents in comparison to bare metal stents.

References

1. Daoud AS, Pankin D, Tulgan H, Florentin RA. Aneurysms ofthe coronary artery: report of ten cases and review of litera-ture. Am J Cardiol 1963;11:228–37.

2. Robertson T, Fisher L. Prognostic significance of coronaryartery aneurysm and ectasia in the coronary artery surgerystudy (CASS) registry. In: Shulman ST, editor. Kawasaki dis-ease. Proceedings of the second international symposium.New York: A.R. Liss; 1987. p. 325–39.

3. Stone GW, Ellis SG, Cox DA. One year clinical results with the

TAXUS-2 trial where a total of four cases were described,three in the Taxus-slow release group, and one in theTaxus-moderate release group.5 This was not significantlydifferent to the control arm of the study and did not resultin any clinical adverse events for this group of patients.In the subsequent TAXUS IV trial, two patients (0.7%) ineach group developed coronary artery aneurysms, withoutclinical adverse outcomes.

slow release, polymer-based, paclitaxel-eluting TAXUS stent.The TAXUS-IV trial. Circulation 2004;109:1942–7.

4. Morice MC, Serruys PW, Sousa JE, Fajadet J, Hayashi EB, PerinM, Colombo A, Schueler G, Barragan P, Guagliumi G, MolnarF, Falotico R, for the RAVEL Study Group. A randomised com-parison of a Sirolimus-eluting stent with a standard stent forcoronary revascularisation. N Engl J Med 2002;346(23):1773–80.

5. Colombo A, Drzewiecki J, Banning A, Grube E, Hawpt-mann K, Silber S, Dudek D, Fort S, Schiele F, Zmudka

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K, Guagliumi G, Russell ME, for the TAXUS II StudyGroup. Randomized study to assess the effectiveness ofslow- and moderate-release polymer-based Paclitaxel-elutingstents for coronary artery disease. Circulation 2003;108:788–94.

6. Anandaraja S, Naik N, Talwar K. Coronary artery aneurysmfollowing drug-eluting stent implantation. J Invasive Cardiol2006;18(1):E66–7.

7. Gupta RK, Sapra R, Kaul U. Early aneurysm formation afterdrug-eluting stent implantation: an unusual life-threateningcomplication. J Invasive Cardiol 2006;18(4):E140–2.

8. Alfonso F, Moreno R, Vergas J. Mycotic aneurysms aftersirolimus-eluting coronary stenting. Catheter Cardiovasc Interv2006;67(2):327–8.

9. Singh H, Singh C, Aggarwal N, Dugal JS, Kumar A, LuthraM. Mycotic aneurysm of left anterior descending artery aftersirolimus-eluting stent implantation: a case report. CatheterCardiovasc Interv 2005;65(2):282–5.

10. Vik-Mo H, Wiseth R, Hegbom K. Coronary aneurysm afterimplantation of a paclitaxel-eluting stent. Scand Cardiovasc J2004;38(6):349–52.

11. Kaul U, Gupta RK, Kachru R. Large coronary arteryaneurysms following sirolimus eluting stent implantation.Heart 2005;91(2):234.

12. Stabile E, Escolar E, Weigold G, Weissman NJ, Satler LF,Pichard AD, Suddath WO, Kent KM, Waksman R. Markedmalapposition and aneurysm formation after sirolimus-eluting stent implantation. Circulation 2004;110(5):e47–81.

Pulmonary Alveolar Proteinosis in Extremis: The Casefor Aggressive Whole Lung Lavage with

Extracorporeal Membrane Oxygenation SupportAlan D.L. Sihoe, FRCSEd(CTh) a,∗, Vivian M.W. Ng, FANZCA b,

Raymond W.T. Liu, FHKAM c, Lik-Cheung Cheng, FRCS a,a Division of Cardiothoracic Surgery, The University of Hong Kong, Grantham Hospital, Hong Kong SAR, China

ccufdP

(

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Pmep

R2

∗DHfE

©A

b Department of Anaesthesiology, The University of Hong Kong, Grantham Hospital, Hong Kong SAR, Chinac Department of Respiratory Medicine, Ruttonjee Hospital, Hong Kong SAR, China

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material is deposited in the alveoli,ompromising gaseous exchange. We report the case of a 29-year-old female patient presenting with the most extremease of PAP yet reported. She successfully managed by aggressive bilateral whole lung lavage (WLL) in a single sittingsing extracorporeal membrane oxygenation (ECMO) support. Despite critical hypercarbia and ventilator-dependenceor 12 days before lavage, the patient experienced rapid recovery of pulmonary function after WLL and ECMO could beiscontinued on-table. Aggressive WLL with ECMO support can be safe and effective even in the most severe cases ofAP.

(Heart, Lung and Circulation 2008;17:62–79)© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and

New Zealand. Published by Elsevier Inc. All rights reserved.

Keywords. Pulmonary alveolar proteinosis (PAP); Extracorporeal membrane oxygenation (ECMO); Whole lung lavageWLL)

ntroduction

ulmonary alveolar proteinosis (PAP) is a rare diseasecharacterised by the deposition of lipoproteinaceous

aterial in the alveoli, compromising pulmonary gasxchange. Whole lung lavage (WLL) is established as thealliative treatment of choice, but in those very rare cases

eceived 12 July 2006; received in revised form 15 November006; accepted 16 November 2006; available online 6 March 2007

Corresponding author at: Division of Cardiothoracic Surgery,epartment of Surgery, The University of Hong Kong, Granthamospital, Aberdeen, Hong Kong SAR, China. Tel.: +852 2518 2111;

ax: +852 2647 3512.-mail address: adls1@excite.com (A.D.L. Sihoe).

where the patient already has critical respiratory failureextracorporeal membrane oxygenation (ECMO) supportmay be required during WLL.1–9 Herein, we present themost extreme case of PAP-induced respiratory failure toour knowledge to be successfully managed by ECMO-supported bilateral WLL.

Case Report

A 29-year-old woman presented with a five-week historyof productive cough, anorexia and malaise. She was a non-smoker with an unremarkable past medical history and noidentifiable environmental risk factors. Her chest X-rayon admission showed diffuse pulmonary consolidations

2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society ofustralia and New Zealand. Published by Elsevier Inc. All rights reserved.

1443-9506/04/$30.00doi:10.1016/j.hlc.2006.11.007