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Ann Thorac Surg 2001;71:2034-2035
© 2001 The Society of Thoracic Surgeons


Case report

Hydatid disease of the interventricular septum causing pericardial effusion

Julio G. Tejada, MDa, Jesus Saavedra, MDa, Luis Molina, MDa, Alberto Forteza, MDa, Casimiro Gomez, MDa

a Department of Cardiology and Cardiac Surgery, Doce de Octubre Hospital, Madrid, Spain

Accepted for publication April 13, 2000.

Address reprint requests to Dr Tejada, Servicio de Cardiología, Hospital Doce de Octubre, Carretera de Andalucía Km 5.400, 28041 Madrid, Spain
e-mail: juliogtejada{at}yahoo.com


    Abstract
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 Abstract
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 References
 
Echinococcal infestation of the heart is uncommon. We report a case of a 35-year-old man with an hydatid cyst located in distal interventricular septum. Clinical presentation was chest pain and urticaria. Diagnosis was made by echocardiography and magnetic resonance imaging. Surgical resection was performed; the cyst was punctured and its content was drained, hypertonic glucose solution was instilled for sterilization, and it was removed. The patient did well and remains asymptomatic. Diagnosis and ultimate surgical treatment of this disease prevented potentially lethal complications such as cyst rupture with embolic phenomena and anaphylactic shock.


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Hydatidosis is a zoonosis which is endemic in areas of the Middle East, South America, Oceania, and the Mediterranean coast. Cardiac involvement accounts for approximately 0.5% to 2% [1]. When the embryo reaches the myocardium, the cyst is formed within a period of 1 to 5 years. The possible occurrence of severe complications, such as cyst rupture and sudden death, makes cyst extirpation the most appropriate treatment [2].

A 35-year-old man, without medical history, presented with urticarial exanthema, sweat, and intense itchiness 1 month before admission. During that month, he complained of evening fever and episodes of precordial pain which increased with deep breathing and postural changes (in lying position).

Physical examination revealed urticarial exanthema in both legs. A pericardial rub was heard at the left lower sternal border. A blood test showed an elevated erythrocyte sedimentation rate, eosinophilia, and normal liver and cardiac enzymes. Pericarditis was the initial diagnosis, and the patient was treated with aspirin (4 grams/day).

The chest x-ray film showed enlargement of the heart and deformation of the cardiac silhouette. The electrocardiogram revealed sinus rhythm, presence of Q-waves in leads V1–V3 and steep symmetrical T-wave inversion in leads II, III, aVF, V2–V6. Transthoracic echocardiography demonstrated a cystic mass (6 cm in diameter) having well-defined edges and internal trabeculations in distal interventricular septum that protruded inside the cavity of the left ventricle, as well as mild pericardial effusion. This mass was thought to be an echinococcal cyst, as this patient lived in an endemic area and had been exposed to dogs. Differential diagnosis included cystic degenerating tumors, pericardial cyst, myocardial abscess communicated with pericardium or pericardial hematoma. Magnetic resonance imaging confirmed the echocardiographic finding, showing small cystic structures corresponding to daughter membranes inside the liquid intramyocardial mass (Fig 1). Serologic test (agglutination) for Echinococcus granulosus was positive. Abdominal ultrasonography was normal.



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Fig 1. Magnetic resonance imaging: axial view showing a cystic, well-defined rounded mass (arrowheads).

 
Extirpation of the cyst was performed with medial sternotomy and cardiopulmonary bypass. Surgical treatment included puncture and aspiration of the cyst content, previous sterilization with hypertonic saline solution, and excision of the cyst. A Dacron (C. R. Bard, Haverhill, MA) patch implantation was needed to close a ventricular septal defect created after excision. Surgical findings were hematic pericardial effusion and a 6-cm diameter cyst, full of daughter vesicles, located in septum, communicating with the pericardial cavity. The cyst was formed of a laminated outer layer and an inner germinal layer filled with a transparent fluid in which scolices were suspended (Fig 2). Histopathological analysis of the surgical specimen confirmed a hydatid cyst.



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Fig 2. Surgical image showing an intramyocardial cyst, filled with daughter vesicles and the surrounding layers.

 
The patient did well, was discharged after a 4-week albendazole cycle, and remains free of symptoms with normal echocardiography after 1 year of follow-up.


    Comment
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 Abstract
 Introduction
 Comment
 References
 
Hydatidosis is a parasitic infection caused by the encysted larvae of Echinococcus. Cardiac involvement is infrequent, appearing when the hexacanth embryo of Echinococcus reaches the right heart cavities through the portal or lymphatic routes. It lodges in the myocardium by way of coronary circulation after reaching the left side of the heart through the pulmonary circulation or a patent foramen ovale [3]. The most frequent location of the cysts is the myocardial region, especially the interventricular septum and left ventricular free wall; pericardial and paracardial sites of implantation are less common [4].

Clinical features depend on size, number, location of the cysts and presence of complications. Presenting symptoms include anaphylactic reactions such as fever, eruptions, or even circulatory collapse. A frequent complaint is chest pain of pericardial origin, but it can also be of ischemic origin when displacement or obstruction of coronary artery branches occurs [5]. Other presenting forms include systemic hydatid embolism, tricuspid stenosis or regurgitation, and multiple hydatid pulmonary embolism causing severe pulmonary hypertension [4].

Diagnosis must be suspected when the patient lives in an endemic area and hydatid cysts are also present in other locations (55% of multivisceral involvement has been reported) [2]. The presence of eosinophilia in some patients is very useful complementary data. Chest x-ray film often shows abnormal shape of the heart shadow, or a sometimes calcified spherical mass. Diagnosis is made by using echocardiography and serologic tests (agglutination or complement-fixation), but excision and pathological examination of the lesion are required to confirm it.

Echocardiography is the imaging method of choice for diagnosis of cardiac cysts, even better than computed tomography or magnetic resonance imaging, which are more useful in the study of extracardiac echinococcosis [6]. In most patients, two-dimensional echocardiographic images of hydatidosis are cystic masses having well-defined edges and internal trabeculations corresponding to daughter vesicles. Some patients present solid masses, very difficult to differentiate from heart tumors, corresponding to cysts that have undergone a degenerative process. Rarely, cardiac echinococcosis shows a large multilocular mass with poorly defined edges [4].

Given the significant risk of cyst rupture, extirpation of the lesion is recommended, even in asymptomatic patients. Complete and rapid sterilization of the cyst content must be performed with the instillation of hypertonic saline solution or other agents. If total excision of the cyst wall is not feasible, the remaining cavity should be closed by obliteration, plication, or both. Operative mortality is low and the postoperative evolution is usually satisfactory, but it is not free of complications [2, 3]. The most frightful of them is rupture during surgical manipulation, with subsequent dissemination and liberation of the content to systemic circulation or pericardium, producing anaphylactic shock.

Serologic and echocardiographic controls are recommended for 5 years after extirpation to detect recurrences after surgical manipulation or cysts that were not discovered at operation [4].


    References
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 Abstract
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 Comment
 References
 

  1. Dighiero J., Canabal E.J., Hazan J., Horlales J.O. Echinococcus: disease of the heart. Circulation 1958;17:128-131.
  2. Miralles A., Bracamonte L., Pavie A., et al. Cardiac echinococcosis. Surgical treatment and results. J Thorac Cardiovasc Surg 1994;107:184-190.[Abstract/Free Full Text]
  3. Ameli M., Mobarhan H.A., Nouraii S.S. Surgical treatment of hydatid cysts of the heart: report of six cases. J Thorac Cardiovasc Surg 1989;98:892-901.[Abstract]
  4. Oliver J.M., Sotillo J.F., Dominguez F.J., et al. Two dimensional echocardiographic features of echinococcosis of the heart and great blood vessels. Circulation 1988;78:327-337.[Abstract/Free Full Text]
  5. De Paulis R., Seddio F., Colagrande L., et al. Cardiac echinococcosis causing coronary artery disease. Ann Thorac Surg 1999;67:1791-1793.[Abstract/Free Full Text]
  6. Desnos M., Brochet E., Cristofini P., et al. Polyvisceral echinococcosis with cardiac involvement imaged by two dimensional echocardiography, computed tomography and nuclear magnetic resonance imaging. Am J Cardiol 1987;59:383-384.[Medline]

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