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cardiac echinococcosis should be diagnosed and undergosurgery without delay.
References
1. Miralles A, Bracamonte L, Pavie A, Bors V, Rabago G,Gandjbakhch I, Cabrol C. Cardiac echinococcosis. Surgicaltreatment and results. J Thorac Cardiovasc Surg 1994;107:184–90.
2. Alehan D, Celiker A, Aydingoz U. Cardiac hydatid cyst ina child: diagnostic value of echocardiography and magneticresonance imaging. Acta Paediatr Jpn 1995;37:645–7.
3. Chadly A, Krimi S, Mghirbi T. Cardiac hydatid cyst rupture ascause of death. Am J Forensic Med Pathol 2004;25:262–4.
4. Marci M, Ponari A, Finazzo F, Battaglia A. Echocardio-graphically diagnosed cardiac echinococcus complicated by
embolic intraventricular thrombus. J Am Soc Echocardiogr1998;11:1158–60.
5. Karabay O, Onen A, Yildiz F, Yilmaz E, Erdal CA, Sanli A, KilciG, Algin I, Itil O, Acikel U. The case of a cyst hydatid localizedwithin the interatrial septum. Jpn Heart J 2004;45:703–7.
6. Oliver JM, Sotillo JF, Dominguez FJ, Lopez de Sa E, Calvo L,Salvador A, Paniagua JM. Two-dimensional echocardiographicfeatures of echinococcosis of the heart and great blood ves-sels. Clinical and surgical implications. Circulation 1988;78:327–37.
7. Desnos M, Brochet E, Cristofini P, Cosnard G, Keddari M,Mostefai M, Gay J. Polyvisceral echinococcosis with cardiacinvolvement imaged by two-dimensional echocardiography,computed tomography and nuclear magnetic resonance imag-ing. Am J Cardiol 1987;59:383–4.
Papillary Fibroelastoma of Aortic Valve:Diagnosis and Surgical Management
Pankaj Saxena, MCh, DNB ∗, Andrew Lee, MBBS,Igor E. Konstantinov, MD, PhD and Mark A.J. Newman, FRACS
Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, Nedlands, WA 6009, Australia
Papillary fibroelastoma (PFE) is a rare and benign cardiac tumour that mainly affects the valves. This tumour has theotential to cause serious life threatening thromboembolic complications. Herein, we describe successful excision of anortic valve papillary fibroelastoma. The importance of valve sparing tumour resection is emphasised.
rimary cardiac tumours are rare. Papillary fibroe-lastoma (PFE) is the third most common primary
ardiac tumour following myxomas and lipomas and theommonest tumour of the cardiac valves.1–3 This is aenign tumour. This tumour has the potential to result
n thromboembolic phenomena to the brain or coronaryirculation.
ase Report
68-year-old woman presented to her general practi-ioner with a three-month history of episodic dizziness,alpitations, pre-syncope and one episode of amaurosis
ugax. There were no focal neurological symptoms duringhese episodes. Past medical history was unremarkable,part from curative excision of a superficial malignantelanoma from the left leg. Physical examination was
ormal. She underwent 24 h Holter electrocardiography
eceived 15 March 2006; received in revised form 11 October006; accepted 19 October 2006; available online 8 March 2007
(ECG) monitoring. This demonstrated two isolated briefepisodes of atrial fibrillation. The patient subsequentlyunderwent echocardiography, which demonstrated amobile mass attached by a thin stalk to the aortic valve.Trans-oesophageal echo demonstrated a 1.5–2 cm longpedunculated mobile mass involving the non-coronarycusp of aortic valve. The valve was tricuspid in struc-ture. There was no aortic insufficiency. All other cardiacchambers were normal in structure and function of leftventricle was normal. Exercise stress test was performedwhich was negative. Her coronary angiogram was nor-mal. As a part of the pre-operative work up in the settingof ischaemic cerebral symptoms, she underwent carotidDoppler, computed tomographic (CT) scan and magneticresonance imaging (MRI) scan of the brain. These inves-tigations were normal. She was then referred for urgentsurgical resection.
The patient underwent resection of a 15 mm longtumour mass, which was 2 mm in diameter. Myocardialprotection was achieved with cold blood cardioplegiadelivered in antegrade and retrograde fashion with sys-temic hypothermia to 28 ◦C. Terminal warm cardioplegiawas delivered in retrograde manner. Aortic cross clamp
2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society ofustralia and New Zealand. Published by Elsevier Inc. All rights reserved.
350 Case Reports Heart, Lung and Circulation2008;17:330–351
Figure 1. Papillary fibroelastoma attached to aortic valve. Patient’shead is towards the lower part of the picture.
time was 29 min and cardiopulmonary bypass time was58 min.
The tumour was attached to the non-coronary cusp ofthe aortic valve (Fig. 1). Macroscopically, the specimen wasa fine, white branching structure with multiple papillaryfronds projecting from a central stalk (Fig. 2). The tumourwas excised at its attachment, preserving the valve. Anintra-operative trans-oesophageal echo did not demon-strate any residual tumour or the presence of any aorticregurgitation.
She made a good recovery post-operatively and wasdischarged home on day 5. The diagnosis of papillaryfibroelastoma was confirmed on histopathological exam-ination (Fig. 3). As she was in sinus rhythm, she wasmaintained on aspirin. A follow-up echocardiogram at 4weeks did not demonstrate any tumour recurrence or aor-tic regurgitation. The patient will be followed-up in oneyear with repeat echo.
Figure 3. Microphotograph demonstrates tumour fronds with centralcore of dense, hyalinised, acellular collagen, and a single layer ofendothelial cells lining the papillae (elastin stain, magnification×100).
Discussion
PFE is the most common tumour of the heart valves,accounting for 75% of cardiac valvular tumours.3 PFE hasalso been documented to arise from the endocardiumof non-valvular areas of the heart including the papil-lary muscles, the ventricular septum, chordae tendinaeand endocardium of the right atrium.4,5 They usuallytend to affect left sided valves. The aortic valve is mostcommonly involved. The risk of associated thromboem-bolism with these tumours is very significant because ofthe predominant left side valve involvement in high flowcardiovascular circulation.
There are certain other tumours affecting the cardiacvalves that can mimic fibroelastomas. Valvular myxo-mas may be quite difficult to differentiate from PFEs.Lambl’s excrescences are usually multiple, are foundalong the area of valvular coaptation and are dis-tinguished histologically.6 They can be confused withfibroelastomas.
The majority of patients with PFE are asymptomatic andas a result, diagnosis of these tumours has historicallybeen an incidental finding upon autopsy or during car-diac surgery.7 This has changed with widespread use oftrans-thoracic and trans-oesophageal echocardiography,and has facilitated early diagnosis and curative surgicaltreatment.
The presentation of symptomatic fibroelastoma is vari-
Figure 2. The excised tumour.
able. It is dependent upon tumour size, location, andtendency for embolisation, but clinical presentation isgenerally related to cardiovascular sequelae. The mostcommon presentation of cardiac papillary fibroelastomais through cerebral embolisation of a fibrin thrombusor fragment of tumour, resulting in symptoms rangingfrom vague neurological complaints to transient ischaemicattacks or stroke. PFE can also cause angina and acutemyocardial infarction through direct occlusive effects ofthe coronary arteries or embolisation to a coronary vessel.7
Despite the usually small size of these tumours, they
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can cause recurrent significant symptoms like cerebralischaemic symptoms.
Surgical treatment is curative. Valve sparing surgerylike shave excision is commonly performed as a part ofsurgical treatment with good results due to the pedun-culated nature of the tumour.7 Anticoagulation has beenproposed as a means of treatment for patients who areeither unfit for surgery3 or are awaiting surgical treat-ment with the aim of reducing the risk of embolisation. Ithas been recommended that surgery should be performedwithin two days following the diagnosis to minimise therisk of embolisation.8
In summary, cardiac papillary fibroelastoma is rare witha high risk of embolic phenomena. A valve-sparing surgi-cal excision is curative and prevents the development ofpotentially serious complications.
References
1. Chitwood Jr WR. Cardiac neoplasms: current diagnosis,pathology, and therapy. J Cardiac Surg 1988;3:119–54.
2. Prichard RW. Tumors of the heart: review of the subject andreport of 150 cases. Arch Pathol Lab Med 1951;51:98–128.
4. Abu Nassar SG, Parker Jr JC. Incidental papillary endocardialtumour. Its potential significance. Arch Pathol 1971;92:370–6.
5. Gallas MT, Reardon MJ, Reardon PR, DeFelice CA, Raizner AE,Mody DR. Papillary firbroelastoma. A right atrial presentation.Tex Heart Inst J 1993;20:293–5.
6. McAllister Jr HA. Primary tumours and cysts of heart and peri-cardium. Curr Probl Cardiol 1979;4:8.
7. Ngaage DL, Mullany CJ, Daly RC, Dearani JA, Edwards WD,Tezelaar HD, et al. Surgical treatment of cardiac papillaryfibroelastoma: a single center experience with eighty-eightpatients. Ann Thorac Surg 2005;80:1712–8.
8. Bossert T, Gummert JF, Mohr F. Papillary fibroelastomas andother cardiac tumours should be resected on an urgent basis.Ann Thorac Surg 2005;79:756.
