Asian Cardiovasc Thorac Ann 2001;9:240-242
© 2001 Asia Publishing EXchange Pte Ltd
Right Atrial Hydatid Cyst With Multiple Organ Involvement
Firat Kardelen, MD,
Gayaz Akçurin, MD,
Halil Ertu
, MD,
Ömer Beyazit, MD1
Department of Pediatric Cardiology
1 Department of Cardiovascular Surgery Akdeniz University Faculty of Medicine Antalya, Turkey
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For reprint information contact: Firat Kardelen, MD Tel: 90 242 227 4343 Fax: 90 242 227 4320 email: kardelen{at}med.akdeniz.edu.tr Department of Pediatric Cardiology, Akdeniz University Faculty of Medicine, Dumlupinar Bulvari, Arapsuyu, Antalya 07059, Turkey.
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Abstract
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Cardiac hydatid disease is uncommon, especially in children. A 13-year-old girl had pulmonary and renal hydatidosis diagnosed by chest radiography, computed tomography, and magnetic resonance imaging. Serologic tests for hydatidosis were positive. A right atrial hydatid cyst was diagnosed on two-dimensional echo-cardiography. Medical treatment with albendazole was started and the cyst was successfully removed.
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INTRODUCTION
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Hydatid cysts tend to form in the liver or lung, but they may be found in any organ including brain, heart, and bones. Cardiac hydatid cysts are rare and represent 0.5% to 2% of all hydatid cysts in humans.1,2 They are usually found in the left or right ventricle, and rarely in the interventricular septum. Pericardial hydatid cyst is extremely rare. The cysts may remain asymptomatic until rupture, and infection is usually only detected incidentally on imaging studies.3
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CASE REPORT
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A 13-year-old girl was admitted with chest pain of 3 months duration and hemoptysis that had recurred after 10 days. Her arterial blood pressure was 100/70 mm Hg, and her heart rate was 98 beats•min–1. On auscultation of the lung, crackling rales were audible on the left side. Routine blood counts and biochemical tests were normal. Chest radiography showed a well-delineated round opacity on the left side and smaller opacities on the right side of the lung (Figure 1
). Hemagglutination tests for hydatidosis were positive with a titer of 1/1600. Transthoracic echocardiography showed a 22 
24-mm tumor of the right atrial posterior wall, extending to the inferior vena cava (Figure 2). Magnetic resonance imaging characterized the mass as a unilobular cyst. Computed tomography was consistent with a diagnosis of hydatid disease of the heart, and revealed an additional cyst located in the left kidney inferior pole and others in the lung. Medical treatment with albendazole was started. After 2 cycles of oral albendazole 400 mg twice daily for 2 months, cardiac surgery was performed under cardiopulmonary bypass. Through a median sternotomy, the hydatid cyst was successfully removed from the right atrium. Pathologic examination of the surgical material confirmed the diagnosis of hydatid cyst due to Echinococcus granulosus. Albendazole therapy was prescribed for one year. Albendazole was given in cycles of 400 mg twice daily for 4 weeks, followed by 2 weeks off. There was significant regression of the hydatid cysts in the lung. The patient became completely asymptomatic and her transthoracic echocardiogram was normal.
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Figure 1. Chest radiograph showing a round opacity on the right side and smaller opacities on the left side.
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Figure 2. Echocardiography of the right atrial hydatid cyst in apical 4-chamber view. LA = left atrium, LV = left ventricle, RA = right atrium, RV = right ventricle.
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DISCUSSION
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Echinococcus species are carried as tapeworms by canines including dogs, wolves, and foxes worldwide, but human infection is most prevalent in countries such as ours where sheep and cattle are raised. Humans incidentally acquire infection by ingesting parasite eggs that are discharged into the soil, vegetables, or water. The disease results from the expansion of parasite cysts in the visceral organs. Cysts form in 1 to 5 years. Approximately 50% to 70% of hydatid cysts occur in the liver, 20% to 30% in the lungs, and less than 10% in other organs. In contrast to liver lesions, pulmonary hydatid cysts are more commonly seen in children who may present with hemoptysis, cough, and dyspnea. The cyst may rupture into the bronchial tree and spread by discharging its contents into the respiratory secretions. Cardiac in-volvement is rare.1–4 The embryo usually reaches the myocardium via the coronary circulation from the left side of the heart. Our patient had pulmonary, renal, and cardiac hydatid cysts, but there was no cyst in the liver. Most individuals with hydatid cysts are asymptomatic. When symptoms occur, they are usually due to the cyst enlarging in a confined space. Symptoms occur earlier when the cysts are located in less well-supported tissues such as brain and lung, where there is also an increased risk of rupture into adjacent structures. The initial symptom in our patient was hemoptysis due to lung involvement.
Echocardiography is the method of choice for diagnosing cardiac cysts. Computed tomography and magnetic resonance imaging are useful for demonstrating extra-cardiac involvement. As seen in our patient, echocardio-graphic views of cardiac hydatid cysts are better than magnetic resonance images which are poor unless T-wave-triggered electrocardiographic gating is used. Infection suspected on imaging studies may be confirmed by specific hemagglutination tests, enzyme-linked immu-nosorbent assay, or Western blot serology. Serology is 80% to 100% sensitive and 88% to 96% specific for liver cysts, but less sensitive for lung (50% to 56%) or other organ (25% to 56%) involvement.5,6 Therefore, imaging methods are more sensitive than serodiagnosis, although the hemagglutination test for hydatidosis was positive in our patient. Jeridi and colleagues7 reported a case of multiorgan hydatid disease including a hydatid cyst of the right atrium, which was confirmed at surgery. They emphasized the latency and clinical polymorphism of right cardiac sites of hydatid cyst and the severity of its prognosis. On echocardiography, a right atrial cyst and its relationship with the septal leaflet of the tricuspid valve could be confused with myxoma of the tricuspid valve.7 Miralles and colleagues4 described 8 patients with hydatid disease of the heart and one who had alveolar hydatid disease with right atrial involvement.
Surgical treatment of cardiac echinococcosis does not always cure the disease. Late sudden death is not exceptional and is generally caused by rupture of a hidden secondary pericardial cyst.8 If the primary cyst is ruptured, the surgeon must explore the pericardium and remove cystic components completely as this is a possible source of hidden secondary cysts and of microscopic fertile elements. Optimal treatment of symptomatic cysts is surgical resection to remove the cyst completely. Medical therapy of inoperative cysts with either albendazole or mebendazole produces improvements in most patients, but the cure rate is approximately 33%. Albendazole is preferred due to its greater absorption from the gastro-intestinal tract and higher plasma levels. The initial therapy should be 3 or more cycles of 400 mg albendazole twice daily for 4 weeks, followed by 2 weeks off. Although rare, cardiac echinococcosis should be considered in the differential diagnosis of cardiac tumors, particularly in patients originating from endemic areas or with manifesta-tions of hydatid disease in other organs.
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REFERENCES
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Abstract
INTRODUCTION
