Case Report

Cardiac hydatid disease, case report, and review of literature S. N. Younis, A. A. Faraj Hawler Medical School, Erbil, Kurdistan, Iraq Cardiac Hydatidosis is a rare but potentially very serious complication of hydatid disease. It is a diagnostic and therapeutic challenge due to the variability of signs and symptoms at presentation. A case of disseminated hydatidosis, affecting the heart muscle, which failed to surgical evacuation and followed by spread to the brain and elsewhere in the body, is presented, discussed, together with a review of the literature. The disease, hydatididosis, remains a challenge even in our days. Systematic radical control in the Middle East is required. Alternative surgical options include heart transplantation when the disease affects the heart muscles and there is more than one cyst which fails to surgical evacuation. Keywords: Hydatid disease, Heart, Surgery, Imaging, Options

Introduction

Case Report

Hydatid disease is a parasitic infection caused by larvae of Echinococcus granulosus, which is still endemic in many sheep-raising countries. Domestic dogs and cats are the primary carriers of echinococcal organisms. Humans are infected as intermediary carriers when they eat unwashed and uncooked vegetables and swallow the ova of the parasite. After the person digests the contaminated food, the embryo of the parasite is released into the intestinal tract and carried to the liver by the portal circulation. Hydatid cysts are surrounded by the periparasitic host tissue (pericyst) encompassing the endocyst of larval origin. Inside the laminated layer, or hyaline membrane, the cyst is covered by a multipotential germinal layer, which gives rise to the production of brood capsules and protoscolices.1 Hydatid cysts can be located in various tissues, although they are most common in the liver (50–70% of cases) and the lung (20–30% of cases) in humans.2 Cardiac involvement in hydatid disease is uncommon, constituting only 0.5–2% of all cases of hydatidosis.3 Experience with computed tomography (CT) and magnetic resonance imaging (MRI) of persons with cardiac hydatid disease is limited.4 A case of disseminated hydatidosis, affecting the heart muscle, which failed to surgical evacuation and followed by spread to the brain and elsewhere in the body, is presented.

MM, a 31-year-old man, was admitted in 2011. His principal complaint was chest pain of few months duration. Chest radiograph showed cardiomegaly and a round shadow projecting on the left ventricle. There were eosinophils in the peripheral blood smear; all other blood tests were within normal limits. Thoracotomy was performed through sternotomy, two cysts, 3 cm in diameter, were removed from the anterior surface of the right ventricle. Smaller cysts were isolated and removed from the right atrium. Four infected cysts were removed from the mediastinal aspect of the heart. His symptoms improved, however, soon after surgery, he started having ataxia, confusion, and memory loss, MRI of the brain revealed hydatidosis and chest CT revealed extensively recurrent cardiac hydatid cyst multiple localization and pulmonary hydatidosis with cystic masses within the right main pulmonary artery (Fig. 1). Echocardiography showed left atrial mass bulging through the mitral valve into the left ventricle.

Correspondence to: S N Younis, Department of Radiology, Hawler Medical School, Erbil, Kurdistan, Iraq. Email: [email protected]

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ß Acta Clinica Belgica 2014 DOI 10.1179/0001551213Z.0000000003

Discussion Hydatid cyst of the heart is an ominous disease which, in the absence of surgical treatment, is usually fatal.5 An improvement in the prognosis of these patients follows surgery, especially since open-heart techniques allow radical treatment.6,7 Surgical treatment of a cardiac hydatid cyst was first attempted by Marten and de Crespigny in 1921 and the first case successfully operated upon was described by Long in 1932.8 Cardiac involvement is an uncommon presentation of hydatid cyst disease, accounting for approximately

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Figure 1 CT chest (mediastinal and pulmonary windows) demonstrating multiple localization of cardiac hydatid cysts both in the left and right sides of the heart and both intra mural and intra cavitaory. Cyst is also noted within the right main pulmonary artery. The pulmonary window demonstrates intra pulmonary parenchymal nodule.

0.5–2% of all hydatidosis cases, and mainly occurring as part of a systemic infection.9 The presentation may also be unusual and late. Hydatid cyst of the myocardium can be asymptomatic; occasionally the first clinical manifestation is sudden death.10 More often precordial pain is present. Other possible signs are due to compression by the cyst. Mitral stenosis can be simulated when the cyst lies in the left atrium obstructing the cardiac outflow. Angina is a main complaint if the cyst compresses a coronary artery, it can also present like squeezing chest pain which could be caused by left ventricular aneurysm.11 Areas of cardiac involvement in hydatid disease include the left ventricle (60% of cases), right ventricle (10%), pericardium (7%), pulmonary artery (6%), and left atrial appendage (6%); involvement of the interventricular septum is rare (4% of cases).12 Cardiac involvement occurs by invasion of the

myocardium, first through the coronary artery circulation. The second route of infestation is the pulmonary vein from rupture of pulmonary echinococcal cysts into the vein.13 The left ventricle is more often involved than the right ventricle, possibly because of the dominance of the left coronary artery, which brings blood to the left ventricle; the greater myocardial mass in the left ventricle, which provides optimal conditions for development of the parasite; and the varying pressure conditions.14 Right-sided cardiac hydatid cysts have characteristics different from those of left sided cysts. Right-sided cysts have a tendency to expand intracavitarily and subendocardially, and right ventricular cysts rupture more frequently, so they lead to pulmonary embolus, anaphylaxis, or sudden death. Rupture into the pericardial cavity can lead to pericarditis, effusion, and cardiac tamponade, whereas left-sided cysts tend

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to grow subepicardially. The heart also can be secondarily affected by direct contact with hydatid cysts originating from the liver or the lung.15 Diagnosis of hydatid disease of the heart depends on a series of tests including hydatid serology, echocardiography, MRI, and CT. CT and MRI are helpful for localizing and defining the morphologic features of hydatid cysts. Specific signs include calcification of the cyst wall, presence of daughter cysts, and membrane detachment. CT best shows wall calcification, whereas MRI depicts the exact anatomic location and nature of the internal and external structures.9,10 Medical treatment of hydatid consists of Albendezol 800 mg/day, for cases who refuse surgery or when inoperable.9 Albendazole can also be given for 4 weeks before surgery to ensure that the hydatid fluid will not infect if the cyst accidentally bursts during surgery. Cystopericystectomy is the gold standard procedure but is sometimes unsuitable for particular sites. In that case, a conservative approach (partial pericystectomy) is mandatory to preserve organ function.10 Cardiac hydatid cysts with intracavitary expansion should be treated surgically without delay. Gentle handling of the heart during cardiopulmonary bypass minimizes operative risk. All patients should be investigated for systemic cysts. The surgical technique includes cavity cleaning and closure using multiple purse string sutures or partial resection and left ventricular patch plasty after removal of the cystic material in the left ventricle.16,17 Hydatid cysts located in the subepicardium can rupture and present like acute abdomen; the perforated cyst can be excised, while the heart is beating or with extracorporeal circulation. Computerized review of the last one hundred cases of hydatid cyst published in the literature in 1990, revealed a mortality of 23.47% in 98 recent cases.18

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Cardiac hydatid disease, case report, and review of literature.

Cardiac Hydatidosis is a rare but potentially very serious complication of hydatid disease. It is a diagnostic and therapeutic challenge due to the va...
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