Asian Cardiovasc Thorac Ann 2007;15:422-426
© 2007 Asia Publishing EXchange Ltd
Surgical Experience with Cardiac Echinococcosis
Sami S Kabbani, MD,
Ahmed Ramadan, MD,
Loay Kabbani, MD,
Aref Sandouk, MD,
Fawzi Nabhani, MD,
Hisham Jamil, MD
Damascus University Cardiovascular Surgical Center, Damascus, Syria
For reprint information contact: Sami S Kabbani, MD, Tel: 963 11 373 8902, Fax: 963 11 373 8901, Email: dam-uncv{at}net.sy, Damascus University Cardiovascular Surgical Center, Mezza Str., PO Box 2837, Damascus, Syria.
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ABSTRACT
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Cardiac echinococcosis is a rare affliction of the heart encountered in areas where the tapeworm Echinococcus is endemic. During the 15 years to September 2005, 19 consecutive patients (11 female; mean age, 25.6 ± 12.8 years) with cardiac echinococcosis were operated on at Damascus University. Diagnosis was mainly established by 2D-echocardiography. Twelve patients also had extracardiac hydatidosis. All patients were operated on using cardiopulmonary bypass and cardioplegia. Intact cysts were generally enucleated, ruptured cysts were exteriorized, and interventricular septal cysts were managed by controlled puncture and aspiration. There was no operative mortality. All patients were placed on mebendazole postoperatively for periods appropriate to the extent of their hydatidosis. There were 4 known late deaths due to systemic extension of the disease. Cardiac echinococcus is a serious disease that should be managed surgically without delay. Enucleation of intact cysts under cardiopulmonary bypass and cardiac arrest is our preferred method of excision. Anthelmintic medical treatment is a valuable and necessary adjunct to surgical management.
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INTRODUCTION
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Echinococcosis (hydatid disease) is a parasitic disorder caused by the tapeworm Echinococcus that infests dogs and other canines. Mammals, chiefly herbivores and occasionally man, act as an intermediate host harboring its larval stage, the hydatid cyst, anywhere in the body, but especially in the liver and lungs.1 The heart is involved in less than 2% of cases of hydatidosis.2 We previously reported 4 surgical cases of cardiac echinococcosis.3 Our experience to date totals 19 consecutive operations.
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PATIENTS AND METHODS
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Between January 1989 and September 2005, 19 consecutive patients with cardiac hydatidosis were surgically managed at Damascus University Cardiovascular Surgical Center by the same medico-surgical team. There were 11 females, and ages ranged between 6 and 57 years, with a mean of 25.6 ± 12.8 years. Only 7 patients gave a history of contact with stray cats (6) or dogs (1). Clinical presentation depended on the organs affected by hydatid disease, cardiac site involved, and size of cysts (Table 1
). Respiratory symptoms and findings dominated the clinical picture when there was accompanying pulmonary hydatidosis. Abdominal pain and hepatomegaly were present when there was significant liver involvement. Both patients with history of anaphylactic shock (one with accompanying skin rash) had cysts lodged in the interventricular septum. A patient with a right atrial multicystic mass had recurrent bouts of urticaria. Two patients had documented cerebral involvement. Cardiac infestation either produced no distinct symptoms or was associated with exertional dyspnea or nonspecific chest pain and/or palpitations (due to recurrent ventricular tachycardia in 1 case). Aside from a positive indirect hemagglutination test (in 12 patients), elevated erythrocyte sedimentation rate (10), and eosinophilia (6), the laboratory findings were unremarkable. Chest radiographs were normal in 6 patients, and 4 had evidence of pulmonary hydatidosis (one with left pleural effusion). Cardiomegaly was present in 10 patients (generalized in 6, left ventricular in 3, and mainly involving the pulmonary artery in 1). Electrocardiograms were reported as: normal in 5 patients, left ventricular hypertrophy in 2, right ventricular hypertrophy in 1, incomplete right bundle branch block in 6, and incomplete left bundle branch block in 2. Eight patients had nonspecific ST/T changes on their electrocardiogram.
The diagnosis of cardiac echinococcosis was established mainly by transthoracic (and sometimes transesophageal) 2D-echocardiography (Table 2). The interventricular septum was the most frequent location for cardiac hydatid cysts (Figure 1), followed by the left ventricle (Figure 2). Ultrasound was also used to diagnose extracardiac hydatidosis; the diagnosis of hepatic cysts by echography in 2 cases alerted us to the possibility of cardiac involvement that was later confirmed by echocardiography. Computed tomography (CT) was carried out in addition to ultrasound in 9 patients, and magnetic resonance imaging (MRI) in 4; the results confirmed the echocardiographic findings and identified new extracardiac cyst locations in 5. Twelve patients had concomitant extracardiac hydatid disease: involvement of one or both lungs in 5; lungs and liver in 2; and liver and both kidneys in 1. One patient with multiple posterior left ventricular cysts had 3 cerebral (parietal) cysts on CT and MRI. Two patients, one with left atrial and one with interventricular septal cysts, showed foci of cerebral infarction on MRI, presumably due to hydatid cyst embolization.
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Figure 1. 2D-echocardiogram revealing a multilocular cyst in the interventricular septum. LV = Left ventricle; LA = Left atrium; RA = Right atrium; RV = Right ventricle; Cyst = Interventricular septal multilocular cyst.
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Figure 2. 2D-echocardiogram showing an intact cyst in the left ventricular wall. Cyst = intact cyst in left ventricular wall; LV = Left ventricle; RV = Right ventricle; RVS = Interventricular septal; MV = Mitral valve; TV = Tricuspid valve.
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All operations were carried out under cardiopulmonary bypass, moderate hypothermia, and crystalline or blood cardioplegia. Unruptured cysts were generally enucleated after placing pads impregnated with 1% formaldehyde around them to protect against dissemination and growth of scolices in case of rupture (Figures 3 and 4). This could be accomplished carefully without cyst rupture in the left and right ventricles, starting with an incision in the epicardium (or myocardium) where the cyst was nearest the surface of the heart. Enucleation of one right ventricular outflow cyst inadvertently caused damage to the left anterior descending artery, which was subsequently repaired without ill effects to the patient. The enucleated cysts measured 1.5 to 12 cm in diameter. The larger the cyst, the easier it was to enucleate because of the relative thickness of its outer layer. The epicardial or myocardial pouch resulting from cyst enucleation was closed by primary suturing. In the ventricular septum, exposed through the right atrium, the cyst could be enucleated safely in 2 patients; however, it ruptured in one because it was under intense pressure. This accident made us change our technique in that particular site to one of aspiration of cyst contents, injection of hypertonic saline as a larvicidal agent (allowing 10 min for death of scolices), and aspiration and removal of the cyst wall and daughter cysts through an endocardial incision. The interventricular space after cyst removal was filled with saline, and the septal incision was closed by primary suture. When the cyst had degenerated and ruptured, the wall and cyst contents were removed without concern about dissemination (1 left ventricular, 1 interventricular septal, and 2 pericardial cysts). The cardiac operation was carried out in combination with removal of bilateral infected pulmonary cysts in a child with a left ventricular cyst, and in tandem with an abdominal operation to remove multiple hepatic cysts in a child with a large interventricular septal cyst (Table 3).
All patients received mebendazole (the benzimidazole available in Syria) for a period of at least 1 month postoperatively. This was then discontinued in cases of single or degenerate cysts and when there was no evidence of extracardiac hydatidosis. Otherwise, the anthelmintic drug was prescribed for at least 6 months, and as long as there remained evidence of inoperable systemic hydatidosis.
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RESULTS
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There was no operative mortality. Pathology reports all indicated intact or degenerated hydatid cysts. Patients were checked postoperatively at least every 6 months, except for 2 who were lost to follow up (1 and 2.5 years after operation) because of change of address. Both were well and free of hydatid disease at their last appointment. In none of the patients subjected to cyst removal did we detect deterioration of left or right ventricular function as a result of the operation, either in the short or long term. We also found by follow-up echocardiography or CT scan that the residual cyst pouches decreased in size over the years, but never disappeared.
There were 4 known late deaths. Cerebral hydatidosis claimed the life of a young woman 1 year postoperatively. Her cyst had resembled a myxoma attached to the posterior wall of the left atrium. She had a cerebrovascular accident 4 months before her cardiac operation, with the finding of left parietal lobe infarction on brain CT. Although she was discharged in good condition after her cardiac operation and kept on mebendazole, she was readmitted 1 year later with increased intracranial pressure and MRI evidence of multiple cysts in the brain, from which she succumbed soon afterwards. Cerebral hydatidosis was also the cause of the late death of a young man with multiple cysts in the posterior left ventricular wall, identified by CT, 8 months after his cardiac operation. The third fatality was in a middle-aged man who had multiple pulmonary and hepatic cysts at the time of excision of his interventricular septal and degenerated small left ventricular cysts. He died 20 months after his operation from hepatic failure, despite continued medical therapy and an abdominal operation to remove the larger hepatic cysts. The last death was in a child with an interventricular septal cyst and multiple cysts in the liver and both kidneys, who died presumably of multiple organ failure 6 months after his cardiac operation.
One child (with a right ventricle cyst and confirmed left pulmonary cyst) had her infected pulmonary cyst removed 4 months after her cardiac operation. Two patients had hepatic cysts removed 3 and 8 months after their cardiac operations. The 13 surviving patients who are being followed up, remain well and free of evidence of cardiac hydatid disease. Some are still being medically managed for arrested pulmonary (2) or hepatic (1) hydatidosis.
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DISCUSSION
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The Mediterranean basin, among other sheep-raising areas in the world, is still considered endemic for Echinococcosis granulosus.4 The life cycle of this tapeworm and the histopathology of its lesions in man are now well established.1 The rare infestation of the heart is believed to occur either through the portal system from a hepatic cyst, or through the pulmonary veins from a pulmonary cyst. Once lodged in the heart, cysts affect cardiac function according to their size, number, and location. Ventricular septal cysts can interrupt normal nerve pathways, affect tricuspid valve function, cause atrioventricular obstruction, or produce myocardial ischemia.5–8 Ventricular cysts usually produce arrhythmias or cause myocardial ischemia or failure.9–11 They can also cause ventricular obstruction.12 Left and right atrial cysts can behave like cardiac tumors, with their attendant systemic or pulmonary embolization.3,13 Our experience confirms that available laboratory methods are not conclusively diagnostic of hydatidosis. Similarly, chest radiographs and electrocardiograms may be normal. 2D-echocardiography, whether via the chest or esophagus, corroborated with CT or MRI when ultrasound images are inadequate, affords the best diagnostic confirmation. As 2 of our patients were diagnosed on suspicion, having been confirmed with systemic hydatidosis, we recommend routine echocardiograms in all patients with hydatid disease.
Once diagnosed, surgical extirpation seems to be mandatory, because cyst rupture inside one of the heart chambers may cause anaphylactic shock, or cerebral, aortic, or pulmonary embolization, which could be fatal.14–17 Pericardial cysts, especially if they rupture, may also cause acute pericardial tamponade.18 Medical treatment with an anthelmintic regimen, a benzimidazole given either alone or combined with praziquantel, has been proven to give sometimes dramatic reduction in the number and size of hydatid cysts, but cannot be relied on to totally eradicate the disease.19 Thus its role should be either prophylactic (preoperatively) or palliative when the disease cannot be extirpated because of multiple cyst infestation of an organ, or multiorgan involvement. Although it is recommended that medical treatment be started a few weeks prior to hydatid cyst surgery, delay of operation may not be advisable in cases of cardiac hydatidosis. The decision to operate on a patient with cardiac echinococcosis who harbors extensive extracardiac dissemination is a difficult one. The benefits of the operation have to be weighed against the limited expected survival of these patients, even with ongoing drug therapy.
Enucleation (or extrusion) of solitary intact cysts remains our surgical treatment of choice because it completely obviates the possibility of dissemination through spillage. This can usually be performed safely and successfully in the case of epicardial cysts and those lodged in the left or right ventricular myocardium. However, this operation requires cardiac standstill as afforded by cardiopulmonary bypass and cardioplegia. If the surgeon estimates that there is danger of cyst rupture during enucleation, it is advisable to resort to controlled puncture, aspiration, and larvicidal compound injection before removing the cyst walls and contents. If this method is used in epicardial and pericardial cysts, it is possible to carry out the operation under beating heart conditions, but we believe this method cannot totally prevent recurrence from spillage, despite the precautionary measures taken.20
As demonstrated in 2 of our patients, it is possible to combine the cardiac operation with a thoracic or abdominal exploration to remove accompanying pulmonary or hepatic cysts. An alternative approach would be to tackle extracardiac lesions a few months after the cardiac operation, allowing for the benefit of preoperative medical therapy. We still recommend that all patients be discharged on anthelmintic therapy, the duration of which can be regulated according to the number of cystic lesions and extent of organ involvement. The fact that none of our patients with a solitary cyst treated by enucleation had a recurrence attests to the sufficiency of giving prophylactic medication for a limited period only in this group. In cases of multiple organ involvement, it may be wise to continue medical therapy as long as evidence of the disease persists.
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REFERENCES
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- Kabbani SS, Jokhadar M, Sandouk A, Nabhani F, Baba B, Shafik AI. Surgical management of cardiac echinococcosis. Report of four cases. J Cardiovasc Surg (Torino) 1992;33:505–10.[Medline]
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- Ottino G, Villani M, De Paulis R, Trucco G, Viara A. Restoration of atrioventricular conduction after surgical removal of a hydatid cyst of the interventricular septum. J Thorac Cardiovasc Surg 1987;93:144–7.[Medline]
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ABSTRACT
INTRODUCTION
