Cardiac hydatid cyst of the right ventricle: Severe localization

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Case Report

Cardiac hydatid cyst of the right ventricle: Severe localization

Aziza L ’ aarje

^a,

*, Selma Lyazidi

^b

, Younes Kitane

^c

, Aziz Alami

^b

, Rachida Habbal

^a

aCardiologyCenter,UniversityHospitalIbnRushd,Casablanca,Morocco

bSurgeryofCardiovascularDiseaseCenter,UniversityHospitalIbnRushd,Casablanca,Morocco

cInternalMedicineDepartment,UniversityHospitalIbnRushd,Casablanca,Morocco

Introduction

Human echinococcosis (hydatidosis or hydatid disease) is caused by the larval stages of cestodes (tapeworms) of the genus Echinococcus. Echinococcus granulosus causes cystic echinococcosis (CE), the form most frequently encountered. Liver and lungs are the primary localization of the disease, the heart is rarely involved [1]. The incidence is low in North America and Northern Europe, but it ranges from 1 to 220 cases per 100 000 inhabitants in other endemic areas.

We report an original case of a heart hydatid cyst of the right ventricle (RV) presenting with syncope and gastrointestinal symptoms and fever, to underline the atypical presentations and diagnosis challenges of this rare disease.

Case report

We present the case of a 24-year-old patient, with no past medical history, who presented to the emergency department of

our hospital with syncope and palpitations associated with fever and a decline in general health started one month before admission. He also reported epigastric pain, nausea, and vomiting.

On physical examination the patient was fully oriented. The blood pressure was 110/75 mmHg, the temperature 38.3

C, and the pulse was regular at 108 per minute. Cardiovascular, pulmonary, neurological, and abdominal examinations were normal.

Electrocardiography revealed sinus tachycardia at a rate of 108 beats per minute, right axis deviation, and right bundle-branch block without ST segment or T wave abnormalities.

Routine laboratory test examination showed leukocytosis of 20 400/mm

³

and marked eosinophilia. The erythrocyte sedimen- tation rate was 57 mm and C-reactive protein was in the normal range. The chest radiography was unremarkable.

Regarding the syncope and fever, we performed a computed tomography (CT) of the head that was normal.

We performed a transthoracic echocardiography that showed a 56 50 mm heterogeneous cystic mass occupying the basal and middle segments of the free wall of the RV, compressing the right cavities, causing RV out ﬂ ow obstruction and a tricuspid regurgita- tion stage II (Fig. 1). Thoracic CT scan con ﬁ rmed the location in the free wall of the RV, with no attachment to the adjacent organs and no other cyst was present.

JournalofCardiologyCasesxxx(2017)xxx–xxx

ARTICLE INFO

Articlehistory:

Received2March2017

Receivedinrevisedform12June2017 Accepted16June2017

Keywords:

Hydatidcyst Rightventricle Endemiccountries Clinicalpolymorphism Surgicalemergency

ABSTRACT

A 24-year-old male was admitted for syncope and epigastric pain and fever. Biological examination showed leukocytosis and marked eosinophilia, the brain computed tomography (CT) was normal. The echocardiography revealed a voluminous mass in the free wall of the right ventricle compressing the right cavities without communication conﬁrmed with thoracic CT. Hydatid serology was positive. Early and urgent surgery was decided. The postoperative period was uneventful and the patient was discharged home on albendazole.

Hydatid cyst is an endemic parasitic infection in cattle-breading countries, and migration has played a signiﬁcant role to expand this infection worldwide. Cardiac hydatidosis is rare (0.5–2%) in comparison with hydatidosis of the liver (65%) and lung (25%). The right ventricle location is even rarer, 10% versus 60% for the left ventricle, and can cause fatal complications such as anaphylactic shock, dissemination, and pulmonary embolism.

<Learningobjective:

Report of a rare occurrence of right ventricle hydatid cyst, and underlining the atypical clinical presentation and difﬁculties of treatment.>

© 2017 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

*Correspondingauthorat:CardiologyCenter,IbnRushdUniversityHospital, 8Elyassmine,MersSultan,Casablanca20140,Morocco.Fax:+212522277124.

E-mailaddress:azizalaarje@gmail.com(A.L’aarje).

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Please cite this article in press as: L ’ aarje A, et al. Cardiac hydatid cyst of the right ventricle: Severe localization. J Cardiol Cases (2017), http://dx.doi.org/10.1016/j.jccase.2017.06.009

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Given the echocardiography data, the eosinophilia, and the demographic origin of the patient diagnosis of a hydatid cystic was highly suggested. It was con ﬁ rmed by a positive enzyme-linked immunosorbent assay E. granulosus antibody assay test, at 1/1300.

The patient was placed on cardiopulmonary bypass. The cyst attached to the lateral wall and the apex of the RV was directly reached and without opening adjacent cardiac chambers we aspirated the entire contents of the cyst, removed its germinative membrane, and washed the cavity with 20% hypertonic saline solution (Fig. 2). Capitonnage was performed, and the incision was closed with the use of polytetra ﬂ uoroethylene felt.

The patient recovered uneventfully. He was discharged home one week later on albendazole 400 mg twice a day for 6 months.

Discussion

CE is endemic in Mediterranean countries, Middle East, southern part of South America, Iceland, Australia, New Zealand, the southern parts of Africa, and central Asia. The incidence of CE in these areas ranges from 1 to 220 cases per 100 000 inhabitants [2,3]. North America and northern Europe are not endemic areas, but the change in the migration patterns and the marked increase in transcontinental transportation over the past 4 decades have caused a rise in the prevalence of this previously rare disease.

CE is the larval cystic stage of a small cyclophyllidea cestode E. granulosus that cause illness in intermediate hosts, generally herbivorous animals and humans who are infected accidentally. In the normal life cycle of Echinococcus species, adult tapeworms inhabit the small intestine of carnivorous de ﬁ nitive hosts, such as dogs, coyotes, or wolves, and echinococcal cyst stages occur in herbivorous intermediate hosts, such as sheep, cattle, and goats.

Although most often found in the liver and lung, hydatid cysts occur in any organ or tissue. Involvement of the heart can occur from systemic or pulmonary circulation or as a direct extension from adjacent structures. Isolated cardiac involvement is rare and occurs in only 0.5 – 2% [4,5]. It can be located at any part of the heart and the manifestations depend on the size, location, the vicinity of the valve ori ﬁ ce areas and the conduction tissue [1].

Cysts grow slowly, usually at a rate of 1 cm/year. When cardiac infection is the case, cysts are encased in an adventitial pericystic coat and they reach maturity in 1 – 5 years.

Hydatid cysts are usually localized within the free wall of the left ventricle (LV) and interventricular septum where the blood

supply is ample. Pericardium, RV, and interatrial septum are rarely affected [4].

RV hydatid cyst is rare. Prevalence is about 10%, compared to LV cysts which are 60%, favored by the large muscle mass, richer vascular supply [5].

Left untreated, the cyst volume gradually increases; it may compress and erode the heart chambers and adjacent organs. The RV location seems to be severe, given the low pressure level, with dire complications, such as hydatid pulmonary embolism, tamponade, and anaphylactic shock (30% of cases) [1].

Our patient had epigastric pain, nausea, and vomiting which would be a referred free wall RV and apex pain.

RV compression may result in decreased RV compliance, reduced ﬁ lling, and decreased RV stroke volume, diminished LV ﬁ lling and drop in cardiac output that could result in cerebral hypoperfusion and syncope.

Eosinophilia is a suggestive but not speci ﬁ c element for the parasitic infection.

In our case, the transthoracic echocardiography was the main examination that helped to make the diagnosis for this atypical presentation. This examination is key to diagnosis, and guides the surgical technique. The transthoracic echocardiography and transesophageal echocardiography as well as CT and magnetic resonance imaging (MRI) can contribute to diagnosis, staging, and therapeutic options [6].

Cardiac hydatid cyst is a surgical emergency. We recommend excising the cyst on cardiopulmonary bypass surgery. The contents of the cyst must be entirely aspirated and germinative membrane removed [7].

Fig.1. Cysticheterogeneouscontent,atthefreewalloftherightventriclein echocardiography.

Fig.2.

Peroperative image. Piping corresponds to the extracorporeal circulation circuitfor discharging theheart. Itshowsthe hydatid cystatthetipoftherightventricle.

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Please cite this article in press as: L ’ aarje A, et al. Cardiac hydatid cyst of the right ventricle: Severe localization. J Cardiol Cases (2017),

http://dx.doi.org/10.1016/j.jccase.2017.06.009

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Under general anesthesia, the patient is placed in the supine position. The most commonly used route is the median sternotomy rather than the lateral or transverse thoracotomy. Under the condition of normothermic cardiopulmonary bypass by cannu- lating the ascending aorta and the right atrium, the aorta was cross clamped and the heart was arrested with a dose of antegrade cold blood-based cardioplegia.

Although many protoscolicidal agents have been introduced, none is ideally effective and safe for intraoperative use. A 20%

hypertonic saline solution is one of the few effective and relatively nontoxic protoscolicidal agents. This solution destroys protosco- lices by creating a signi ﬁ cant osmotic gradient. We have also used this solution to wash out the remaining cavity. It is prudent to place solution-soaked sponges in the pericardial cavity during the operation to prevent local dissemination [8].

For cystopericystectomy, the hydatid cyst is ﬁ rst punctured using a trocar connected to the aspiration, when the cyst is unilocular it immediately collapses. The peri-cyst is then incised with the electric scalpel and it appears as a de ﬂ ated white balloon.

This very fragile membrane is then carefully enucleated using instrumental detachment and aspiration. This cystectomy is facilitated by the virtual cleavage plane ﬁ lled with a thin layer of mucus located between the hydatid and the adventitia.

For multilocular cysts, the evacuation is much more laborious, as the punctured cyst does not collapse entirely.

While perkystectomy consists of the resection of ﬁ brous scar tissue, it must be the least invasive as possible the remaining cavity is erased by pad points which reinforce the thinned heart wall, in order to avoid rupture.

Capitonnage of the residual cavity can also be performed.

Microscopic examination of the cyst contents usually reveals protoscolices with hooklets, ﬁ ndings consistent with echinococcus larvae.

Albendazole (400 mg twice daily) is typically prescribed for at least 4 days preoperatively and 4 – 12 weeks postoperatively

[7,8]. In case of disseminated disease, inoperable and ruptured cysts, medical therapy with albendazole is the only option [9,10].

Cardiac localization of hydatid disease is rare. The clinical polymorphism, latency and severity of complications are the essential characteristics. Echocardiography, CT and lately MRI represent the main examinations for early diagnosis. Treatment is primarily surgical. The risk of recurrence is present, hence the importance of biological and radiological monitoring. Good prevention in endemic countries is key to the global eradication of this parasitic infection.

Con ﬂ ict of interest None.

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