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Ann Thorac Surg 1999;68:1290-1294
© 1999 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

A clinical dilemma: cardiac and pericardiac echinococcosis

C. Levent Birinciolu, MD^a, Hamet Bardakci, MD^a, eref A. Küçüker, MD^a, Ahmet T. Ulus, MD^a, Kemal Arda, MD^a, Birol Yamak, MD^a, Ouz Tademir, MD^a

^a Department of Cardiovascular Surgery, Türkiye Yüksek htisas Hospital, Ankara, Turkey

	Abstract

Top Abstract Introduction Patients and methods Operative technique Results Comment References

 
Background. Cardiac and pericardial echinococcosis as a life-threatening disease may present with a clear picture most of the time, however it may also become a clinical puzzle.

Methods. In the period between 1977 and 1998, 14 patients were operated on with the diagnosis of cardiac and pericardial echinococcosis. Nine patients were operated on with standard cardiopulmonary bypass (CPB) techniques, and the remaining 5 patients were operated on without CPB. Transesophageal echocardiography (TEE) or intraoperative surface echocardiography were used to plan and perform the operation for the late cases.

Results. One patient died during the postoperative period due to the rupture of interventricular septum. All other patients survived the perioperative period, received mebendazole treatment, and exhibited no recurrence during the follow-up.

Conclusions. The definitive treatment is the surgical extraction of the cyst. Because the clinical picture may vary according to the number, size, and location of cysts, as well as complications, cardiac echinococcosis should be remembered and included in the differential diagnosis to achieve the treatment. Intraoperative surface echocardiography is of paramount value for diagnosis and planning the management of a successful surgery.

	Introduction

Top Abstract Introduction Patients and methods Operative technique Results Comment References

 
In some parts of the world, echinococcosis remains a serious health problem. Although sporadic cases have been identified in western countries, most cases are found in immigrants from endemic areas [1, 2]. Cardiac echinococcosis is extremely rare. The incidence, even in countries where it is endemic, is less than 2 percent [3].

Most cases can easily be diagnosed, and with simple surgery, treatment can be achieved. However, some cases may be misdiagnosed and echinococcosis may be the unexpected etiology for some patients.

We present 14 cases of cardiac echinococcosis, illustrating different clinical pictures. Apart from uncomplicated forms, the disease may present with forms mimicking atrial myxoma, ruptured sinus of Valsalva aneurysm, left ventricular aneurysm, pericardial tamponade, and subdiaphragmatic abscess.

	Patients and methods

Top Abstract Introduction Patients and methods Operative technique Results Comment References

 
From 1977 to 1998, 14 patients with cardiac and pericardial cysts were operated on. Eight were female, 6 were male, with ages ranging from 10 to 57 years (mean 28.9 ± 12.6 years). Four of the patients (30%) in this series were sheep farmers and nearly all of them stayed in an endemic area at least once. Seven patients (50%) had preoperative treatment for hydatid disease involving other organs. Six patients (48.8 %) had positive serologic tests.

The following clinical forms were identified: myocardial cyst of the left ventricle, right atrial cyst mimicking myxoma, cyst between the sinus of Valsalva and the right atrium, mimicking the rupture of the sinus of Valsalva aneurysm to the left ventricle, cysts causing tamponade by compressing the right atrium, and pericardial hydatid cyst. Each of these clinical forms is detailed in a case report.

Myocardial cyst of left ventricle
Eight of fourteen cysts were present in patients at the left ventricular myocardium (Table 1). In 6 patients (6 of 8, 75%), there were electrocardiogram (ECG) changes initially diagnosed as coronary artery disease. Two-dimensional echocardiography revealed cystic mass of the left ventricular wall in 7 of 8 patients and 1 had posterolateral left ventricular aneurysm. In the eldest 3 patients, (35, 36, and 46-years-old), including 1 whom had posterolateral left ventricular aneurysm, coronary angiography and ventriculography were performed. Coronary angiograms were normal but 1 patient had a filling defect at ventriculography. The patient who was hospitalized for unstable angina and diagnosed echocardiographically to have left ventricular posterolateral aneurysm had normal coronary angiography and ventriculography. These conflicting findings forced us to perform a computerized tomography (CT), which diagnosed a cardiac cyst (Fig 1). This finding was confirmed at the time of surgery with surface echocardiography (Fig 2 ) and operative findings.

View larger version (139K): [in this window] [in a new window]   Fig 1. Computed tomography image of case 8, a cystic mass containing membranes, located at the cardiac apex.

View larger version (73K): [in this window] [in a new window]   Fig 2. Intraoperative surface echocardiographic view, cyst mass containing membranes, located at the posterolateral part of left ventricle, without any connection with ventricular cavity. (CHD = chordae; PM = papillary muscle; LV = left ventricular cavity; MEMB = membranous structure.)

View larger version (67K): [in this window] [in a new window]   Fig 3. Transesophageal echocardiography view of case 9, a tumoral mass within the right atrium resembling myxoma. (RA = right atrium; APP = right atrial appendix; TM = tumoral mass.)

View larger version (174K): [in this window] [in a new window]   Fig 4. Macroscopic appearance of the extirpated mass from the right atrium of case 9. Gateaux formation made by the nonvital, degenerated, dry hydatid daughter cyst membranes.

View larger version (131K): [in this window] [in a new window]   Fig 5. Lateral chest x-ray of case 10, a mass with calcified contours located at the anterior mediastinum.

A 57-year-old male patient (case 12) presented to our hospital with paroxysmal nocturnal dyspnea, orthopnea, peripheral edema, hepatomegaly, and NYHA class IV functional capacity. Two-dimensional echocardiography demonstrated a large cystic mass, pushing the right atrium. CT also confirmed this lesion as 12.3 x 6.2 cm cystic mass in the anterior mediastinum just anterior to the vena cava superior and ascending aorta. Right atriography demonstrated an external mass pushing the right atrium. Coronary angiography was normal. At operation, a 15 x 10 x 10 cm mass was extracted from the right anterior hemithorax, compressing the right atrium extrapericardially.

Pericardial hydatid cyst
There were 4 patients in this group, 2 of which also presented with myocardial cysts of the left ventricle. Another patient (case 13) had two pericardial cysts (6 x 6 cm and 3 x 2 cm). Another patient had a 3 x 5 x 7 cm pericardial cyst, in addition to his apical cyst (case 4). The third patient (case 3) had a 3 x 3 x 4 cm pericardial cyst in addition to the left ventricular lateral wall cyst. Another patient (case 14), who was operated on for hepatic hydatidosis 3 years ago, was admitted to our hospital with signs of weakness, fever, and abdominal pain. Blood tests showed leukocytosis. Abdominal ultrasonography identified subdiaphragmatic collection and a 4 x 5 cm cystic mass in the liver. CT additionally showed some pericardial irritation. General surgeons operated on him, and during the operation, it was observed that the cyst was extending and had ruptured into the pericardial cavity. At this point, cardiovascular surgeons were called in to join the operation.

	Operative technique

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Median sternotomy was performed in 13 patients. Posterolateral thoracotomy was performed in 1 patient who had two pericardial cysts, which were resected with the adjacent pericardium. Planning of the operation was reassessed following pericardial sectioning. Especially for late cases, TEE and intraoperative surface echocardiography were used during this reassessment.

Standard cardiopulmonary bypass (CPB) techniques were used in 9 patients, of which 8 had moderate hypothermia, cardioplegic arrest, and topical cooling. Following the cardioplegic arrest, cysts were reached via left ventriculotomy in 6, aortotomy in 1 and right atriotomy in 1. The left ventriculotomy was closed primarily in all patients but 1 (case 1), in whom a polytetrafluorethylene (PTFE) patch was used due to a large defect. In another patient (case 9), a right atrial cyst was removed with atrial wall attachment and atriotomy was repaired with pericardial patch. In another patient (case 10), a ruptured cyst was located between the noncoronary sinus of Valsalva and right atrium, deforming and perforating the noncoronary cusp, and there was a connection between left ventricle and sinus of Valsalva via this ruptured and calcified cyst. It was removed and aortic cusps were resected. The aortic root was repaired with collagen coated Dacron (C.R. Bard, Haverhill, PA) patch and aortic valve was replaced with a 25-mm bileaflet mechanical prosthesis (St. Jude Medical, St. Paul, MN). Another patient (case 2) had a large hydatic cyst mass at the posterior wall of left ventricle. There was no connection between the cyst lumen and the ventricular cavity (some fluid content of the cyst was aspirated with injector and only cystic fluid was obtained) so the cyst was removed with the beating heart on CPB.

In the remaining 5 cases, cysts were exposed and removed without CPB. After checking the fluid content to identify that no communication was present between cyst cavities and cardiac lumens, polyvinylpyrrolidone iodine was injected into the cysts. The fluid content of the cyst was than aspirated and the cavity was opened. Multiple daughter cysts were removed and the cavity was irrigated. If the cyst mass was free from the myocardium, the cysts were directly removed. One patient (case 14), while being operated on by general surgeons with the diagnosis of subdiaphragmatic abscess and recurrent hepatic cyst, was found to have a hydatic cyst, which crossed the diaphragm and ruptured into the pericardial cavity. Following the polyvinylpyridine iodine injection, the cyst was aspirated and germinative membrane extracted transdiaphragmatically, and the pericardial cavity was washed and drained.

	Results

Top Abstract Introduction Patients and methods Operative technique Results Comment References

 
In case 6, the septal cystic cavity had no communication with ventricular cavity and it was closed primarily. The ventriculotomy was sutured by using Teflon strips (Impra, Inc, subsidiary of LR Bard, Tempe, AZ). Hemodynamic collapse occurred and the patient died in the intensive care unit. Autopsy showed septal rupture on the suture line closing the cystic cavity because of thinned wall and tight suturing.

The surviving 13 patients received mebendazole treatment postoperatively and echocardiographic controls were done at second and sixth postoperative months and then annually. Patients were followed up for a mean period of 7.3 ± 3.4 years (6 months–11 years). No recurrence was observed during follow-up.

	Comment

Top Abstract Introduction Patients and methods Operative technique Results Comment References

 
Cardiac hydatid cysts comprise only 0.5%–2% of all the hydatidosis cases [5]; consequently cardiologists may miss the diagnosis. In approximately 20,000 cardiac operations during 20 years, we had only 14 cases of cardiac echinococcosis, which is one of the largest series in literature. The presence of a cyst-like mass in persons with a history of exposure to sheep and dogs, in areas where echinococcus granulosus is endemic, weakly supports the diagnosis of hydatid disease (30% of our patients). Prior history of hydatid disease can facilitate the diagnosis of cardiac cysts (50% of our patients). Serologic tests are often useful but some patients with echinococcosis do not develop a detectable immune response (57% of our patients).

Echocardiography is a reliable technique for the diagnosis of intracardiac masses, but in some cases the echoluscent and multiseptate nature of echinococcal lesions may be absent. Thus, in the appropriate clinical settings, echinococcal infection should be included for the differential diagnosis of tumoral lesions of the heart [6]. Sometimes the kinetic pressure of the heart increases the tension around the cyst, restricting the enlargement so it contains less liquid and more scolexes, which gives a solid mass image. Echocardiography misdirected us on the planning of surgery for one of our cases (cyst at right atrium diagnosed as right atrial myxoma, both in two-dimensional echocardiography and TEE, case 9, Figure 3). There are similar cases in the literature [7–9]. The CT image of hydatid cysts typically shows sharp contours (Figure 1) and marginal calcifications, but sometimes CT identification of cyst is too difficult [10]. One of our patients was misdiagnosed with a pericardial adhesion on CT, but during the operation we found many cysts on the right ventricular wall. Furthermore, CT can only produce images in a single anatomical plane, limiting the morphologic analysis and often failing to detect small lesions [11]. Nuclear magnetic resonance (NMR) can produce images in slices comparable to CT, but in more than one anatomical plane and without using contrast material [11, 12]. CT and NMR as well as abdominal sonography are very useful for evaluation of hepatic, renal, and pelvic cystic diseases [11], but for cardiac cases, NMR may not give satisfactory results [11]. Diagnosis of multiple cysts containing less liquid and moving with every beat of the heart may obscure the NMR diagnosis, which requires motionless field. Furthermore, NMR is an expensive method for routine use in a clinical state, which may mimic many diseases. Nuclear magnetic resonance can be used for selected patients, especially those with other organ lesions.

Echocardiography is especially important for surgical planning. Kardaras and associates reported the rupture of an undiagnosed cyst in the right atrium during cannulation, which caused sudden death [13]. Intraoperative surface echocardiography will make the definitive diagnosis and can help us to plan the operation by helping us identify cannulation sites and make decisions such as whether to use the cardiopulmonary bypass and whether to perform the operation on a beating heart or under cross clamp. Consequently, with the guidance of intraoperative surface echocardiography, 4 patients were operated on without using CPB, and to reach the posterior surface of the heart in one case, the operation was performed with CPB on beating heart.

In our series, cardiac hydatic cysts were more common in the left heart (8 of 14 cases, of which 6 had ECG changes and chest pain) and, to differentiate from coronary disease, techniques to visualize cystic mass in the heart were used. Cardiac echinococcosis should be included in differential diagnosis in patients with normal coronary angiography, but having angina-like symptoms. Patients with cardiac echinococcosis may remain asymptomatic for many years or have minor nonspecific complaints, but it is associated with an increased risk of lethal complications if left undiagnosed and untreated [5, 14–18].

Although reports of patients successfully treated with benzimidazoles (albendazole, mebendazole) have been published [16, 17, 19], this does not prevent emergence of serious complications [18, 20]. In one of our patients, cardiac echinococcosis developed following pulmonary hydatid cyst operation inspite of albendazole treatment.

The awareness and suspicion of cardiac hydatidosis will facilitate its diagnosis, and the use of surface echocardiography will be a great help to plan the operation.

	References

Top Abstract Introduction Patients and methods Operative technique Results Comment References

 

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