Case Report

Thrombotic cardiac apex hydatid cyst

Feridoun Sabzi a, Hamid Madani b, Samsam Dabiri a, Alireza Pormotabed a,Reza Faraji a,*a Preventive Cardiovascular Research Centre Kermanshah, Kermanshah University of Medical Sciences, Kermanshah,IranbDepartment of Pathology, Faculty of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran

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a r t i c l e i n f o

Article history:

Received 22 February 2015

Accepted 12 September 2015

Available online 27 October 2015

Keywords:

Hydatid cyst

Chest pain

Echocardiography

a b s t r a c t

Hydatid cyst (HC) is an endemic infestation in the cattle-breading countries such as in Iran.

The involvement of heart by HC is rare; however, nesting of larva in the left ventricular apex

with subsequent rupture to the systemic circulation and thrombus formation in the

remaining cyst cavity is an exceedingly rare phenomenon. A 45-year-old man referred to

our emergency cardiac room with chest pain and a transthoracic echocardiography (TTE)

that showed a cardiac apex cystic lesion. The differential diagnosis of a cystic tumor, a HC, or

aneurysm in the apex of the left ventricular walls was considered and evaluated by TTE and

magnetic resonance imaging. However, the thrombotic HC was confirmed at the surgery.

The cyst with its thrombotic component was excised surgically by on-pump cardiac surgery.

The postoperative period was uneventful and the patient was discharged to home and

treated with a full course of Albendazole therapy for 4 weeks. Six-month follow-up with TTE

revealed complete healing of the apex defect without recurrence of the cyst.

# 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

Available online at www.sciencedirect.com

ScienceDirect

journal homepage: www.elsevier.com/locate/ihj

1. Introduction

Cardiac involvement by Hydatid cyst (HC) is rare comprisingless than 3% of cases of human hydatidosis. Cardiac involve-ment by HC is usually manifested in the muscular part of theheart as in the interventricular septum or the left or rightventricular free wall. The right ventricle, the right atrium, orthe left atrium involvement has also been reported in themedical literature. The cardiac apex as a part of septum issupplied by both left and right coronary artery and isconsidered as a high risk location for larva nesting.1 The HC

* Corresponding author.E-mail address: r.faraji61@gmail.com (R. Faraji).

http://dx.doi.org/10.1016/j.ihj.2015.09.0220019-4832/# 2015 Cardiological Society of India. Published by Elsevier

of the apex has equal chances to rupture into the right or leftventricle, but the interventricular septal HC commonlyruptures into the left ventricle. After HC ruptures to a cardiaccavity, two scenarios are likely. If the cavity has a wideconnecting orifice to cardiac chamber, the risks of cardiogenicshock and systemic emboli are high, but if the connectingorifice is a narrow, the risk of thrombus formation due tostagnation of blood in the cavity is higher than the risk ofemboli.2 Thrombus formation in remnant cavity could presentas a cardiac apex pseudo-aneurysm containing a thrombus.3

We present a case of left ventricular thrombotic HC that waspreoperatively diagnosed as a pseudo-aneurysm.

B.V. All rights reserved.

Fig. 2 – TTE in three-chamber view shows apical cyst (blackarrow).

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2. Case report

A 45-year-old woman was referred to our hospital from a localhospital with chest pain and dyspnea for the last two months.A physical examination revealed normal cardiac and respira-tory sounds. In abdominal palpation, no mass or hepatosple-nomegaly was found. Chest X-ray was normal. On ECG, T-wave inversion was seen in the anterior leads, but his rhythmwas sinuous and regular. A transthoracic echocardiography(TTE) showed a left apical cystic mass with intra-cavityheterogeneous content (Figs. 1 and 2). Magnetic resonancefinding revealed a large cystic mass in the apex of the leftventricle (Figs. 3 and 4).3,4 An HC haemagglutination test waspositive and its titer was 120 IU. Coronary arteries were normalin angiographic study. The patient was scheduled for opencardiac surgery by a median sternotomy. Cardiopulmonarybypass (CPB) was instituted using bicaval and ascending aortacannulation. The ascending aorta was cross-clamped andantegrade crystalloid cardioplegia was infused in the aorticroot. The cyst was easily found in the left ventricular apexmuscle oriented towards the lateral wall (Fig. 5). With highsuspicion of HC, cardiac apex was isolated from the surround-ing pericardial cavity and covered circumferentially withhypertonic saline soaked sponge gauze. The cardiac apexwas opened and thrombotic content of the cyst was removed.The cavity contained multiple daughter cysts and membraneswith an organized thrombus. The connecting narrow orifice ofthe cavity to the main left ventricle chamber was blocked by aclot. Narrow connection of the cyst to the left ventricular cavityand its block by thrombotic material prevented free dischargeof cavity's content to circulation, so a major anabolic eventwas avoided as per past medical history of the patient. After

Fig. 1 – Echocardiography in multiple views shows apicalcyst. (1a, left upper image): long axis view shows cyst withblack vertical arrow. (2a, right upper image): four chamberview shows cyst with black transverse arrow. (3a, left lowerimage): four chamber view shows wall of cyst with verticalblack arrow. (4a, right lower image): four chamber viewshows cyst with transverse black arrow.

Fig. 3 – Magnetic resonance imaging revealed apical cystfilled with contrast (black arrow).

removal of cyst contents, the cyst cavity was irrigated withhypertonic saline solution. The orifice of the cyst cavity to theleft ventricle was sutured by two 4/0 proline sutures and therest of cyst was closed with the capitonage method. Thepatient recovered from the surgery and his postoperativecourse was uneventful. Culture and smear study of cystcontents showed negative bacterial results. Albendazole wasgiven in the postoperative period for 4 weeks. The patient wasdischarged on the 13th day of surgery. Echocardiographyrevealed no further recurrence of the cyst during 6 postoper-ative months. Histopathological examination showed

Fig. 5 – Intra-operative view shows thrombotic material incyst space.

Fig. 4 – Magnetic resonance imaging shows apical cyst(black arrow).

Fig. 6 – Hematoxylin eosin examination of hydatid cyst wallshows scolices (transverse arrow), germinal layer (verticalarrow), and laminated layer or ectocyst layer (curved arrow)subsequently.

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germinative and keratinized layers with thrombotic materi-als (Fig. 6).

3. Discussion

The HC cestode, echinococcusgranulosis, causes hydatiddisease of the heart in 0.5–5% of body organ involvement by

HC. The diagnosis of heart HC is not an easy task and could beconsidered in any case with a cystic mass in the pericardialspace in a patient from a sheep-breading province. The clinicalpresentation may differ from asymptomatic mass diagnosedincidentally during routine work-up of a patient for anotherpathology to a stormy clinical picture presenting withtamponade, complete heart block, or anaphylactic shock,and congestive heart failure. TTE not only has high sensitivityand accuracy in diagnosis of the cyst but also localizes the siteand size of the cysts. If the patient had chest pain, coronaryangiography should be performed in symptomatic patientswith clinical diagnosis of pseudo-aneurysm. In some cases,image of HC in TTE resembles solid masses by frequent intra-cystic daughter cysts, which makes differentiation of HC fromother primary or secondary metastatic tumors even moredifficult.4 Oliver et al. demonstrated the similarity betweenecho images of HC and solid tumors related to degeneration ofintra-cystic content such as germinative layers, daughter cyst,and scolex to necrotic materials that gives a picture like a solidtumor with its heterogeneous echo signals. This necroticinflammatory mass was surrounded by a degenerative layer ofHC that leads to severe inflammation in the wall of the cyst. Inaddition to natural inflammatory reaction, in some casessecondary infection of cyst cavity causes difficulty in accuratepathologic diagnosis.5 Acute coronary syndrome like clinicalsituation due to pressure effects of the cyst on surroundingtissues and arteries could be another manifestation of apicalcyst. Coronary artery involvement caused by kinking orinflammation induced intra-vascular thrombosis is anotherlikelihood. Some cases of apical HC rupture into the pericardialcavity and may cause pericarditis, effusion, constrictivepericarditis, and multiple intra-pericardial daughter cysts ormay even drain into the systemic circulation and subsequent-ly cause anaphylactic shock. Differential diagnosis of solidmass in the apical region of the heart includes fibroma,rhabdomyoma, rhabdomayosarcoma, lipoma, teratoma, and

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thrombotic apical pseudo-aneurism. TTE has a critical role inthe diagnosis of non-infected HC by determination of the cysticmass with distinct wall and heterogeneous intra-cystic materi-als.6–8 Differential diagnosis of infected HC of solid tumors isdifficult and in most patients, the final diagnosis is establishedby pathological exam. However, the non-surgical interventionalmethod is now being used in the non-cardiac cases. Surgerywith or without CPB use is the treatment of choice for cardiacinvolvement of cyst. Preoperative TTE in the apical HC definesthe plane of surgery by distinguishing the communication ofcyst to surrounding chambers and therefore gives a plane toremove the cyst by off-pump or by CPB.9 Apical HC in our casehad a small communication into the left ventricle and,therefore, we removed cyst content via CPB and aortic cross-clamp and cardioplegia arrest and by capitonage method.

4. Conclusion

Cardiac HC should be considered in the differential diagnosisof any intra-pericardial mass, especially in sheep-breadingprovinces where hydatidosisis endemic infestation is ob-served. Although apical HCs are rare and often asymptomaticin non-ruptured stage of the cyst, they may be fatal ifinterventricular septal necrosis or subsequent to rupture tosystemic circulation as an aortic or pulmonary emboli.Therefore, clinical suspicion is important for a correctdiagnosis. Echocardiography, CT, and MRI are useful in thediagnosis and location of cardiac hydatid cysts. Combinedsurgical removal of an apical cardiac hydatid cyst, irrigation ofthe remaining cavity with hypertonic saline solution or 96%alcohol, and concurrent albendazole therapy typically yieldexcellent results.

Conflicts of interest

The authors have none to declare.

Contributors

All authors have taken a significant and active part in thepreparation of the article. They contributed to the intellectualcontext and approved the final version.

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