Cardiac hydatid cyst is a rare disease, especially in children. An 11-year-old boy with a previous anaphylactic reaction and episodes of abdominal pain was admitted for workup of an acquired long systolic murmur. Echocardiographic investigation disclosed a tumor of the right ventricular anterior wall, with multiple loculations. Magnetic resonance imaging characterized it as a multilobular tumor with cyst formation and disclosed another cyst in the right pulmonary artery. With a positive ELISA reaction the child was admitted for surgery with the diagnosis of cardiac and pulmonary hydatid cysts. Cardiac surgery was performed with good results, followed by medical treatment with albendazole

Bento, L

Jalles, N

Lima, M

Macedo, AJ

Magalhães, MP

Sampayo, F

Repository of the Hospital Center of Central Lisbon

Cardiac Hydatid Cyst in a Child

Repositório do Centro Hospitalar de Lisboa Central, EPE

Cardiac Hydatid Cyst in a ChildA.J. Macedo, M.P. Magalha˜es, N. Jalles Tavares, L. Bento, F. Sampayo, M. LimaDepartment of Pediatric Cardiology, Hospital de Santa Marta, Santa Marta Street, 1150 Lisbon, PortugalAbstract. Cardiac hydatid cyst is a rare disease, espe-cially in children. An 11-year-old boy with a previousanaphylactic reaction and episodes of abdominal painwas admitted for workup of an acquired long systolicmurmur. Echocardiographic investigation disclosed a tu-mor of the right ventricular anterior wall, with multipleloculations. Magnetic resonance imaging characterized itas a multilobular tumor with cyst formation and dis-closed another cyst in the right pulmonary artery. With apositive ELISA reaction the child was admitted for sur-gery with the diagnosis of cardiac and pulmonary hyda-tid cysts. Cardiac surgery was performed with good re-sults, followed by medical treatment with albendazole.Key words: Cardiac hydatid cyst — Echocardiography— Magnetic resonance imaging — Pediatric cardiologyHydatid cyst in the heart is uncommon, cardiac involve-ment is reported in fewer than 2% of all cases [5, 9].Diagnosis in children is rare due to the cyst’s slowgrowth [1]. Cardiac cysts are reported to be primary le-sions in 80% of cases [4, 5]. Most frequently hydatidcysts in children occur within the lung parenchyma. InPortugal the incidence of hydatid cysts is estimated to beapproximately 1 per 710,000 [3], and a recent 10 yearstudy reported 20 cases of hydatid cyst in children, butnone with cardiac involvement [2]. We report a myocar-dial hydatid cyst of the right ventricle that was treatedsuccessfully with surgery.Case ReportAn 11-year-old boy was originally seen by our department because ofa cardiac murmur. There was no relevant family history, and pregnancyand delivery had been normal with a birth weight of 3400 g. He hadbeen under a regular pediatric surveillance and received the recom-mended immunizations, BCG included. During infancy he had hadseveral episodes of bronchiolitis and urticaria. Beginning at 2 years ofage there was marked alleviation of the respiratory problems, althoughthe atopic dermatitis did not resolve. At the age of 8 years he had beenadmitted to the Santa Maria University Hospital in Lisbon on an emer-gency basis owing to a serious anaphylactic crisis of unknown origin.Therapy with intravenous corticosteroids and dopamine was necessary.At the age of 10 he was referred to the same hospital because of acuteright upper abdominal pain. No diagnosis was established, and at thattime a soft cardiac murmur was found and was interpreted as an inno-cent murmur. Various episodes of upper abdominal pain, interpreted aspsychosomatic problems, took him to various emergency rooms. Onemonth before returning to our department, because of left lower rib painand dry cough for 2 weeks with no fever his pediatrician diagnosedleft-side pneumonia on the basis of a chest radiograph. At that time, aloud cardiac systolic murmur was heard.At the current admission the clinical examination, including skin,chest, and abdomen, revealed no apparent disease, except for a thrill onthe left anterior chest wall. Cardiac auscultation disclosed a loud leftsternal ejection murmur with no click and maximal intensity on thethird intercostal space. The first heart sound was normal, and there wasa normal split second sound with a soft pulmonary component. Bloodtests disclosed 16% eosinophilia. The chest radiograph demonstrated anormal cardiac image and nonspecific bilateral lung opacities. Theelectrocardiogram (ECG) was normal. On two-dimensional echocardi-ography a large multicystic mass was apparent within the anterior walland outflow tract of the right ventricle (Fig. 1). The mass was non-movable with cardiac cycles, and a Doppler gradient of 40 mmHg wasmeasurable in the outflow tract of the right ventricle. A right ventricularhydatid cyst was suspected. ECG gated magnetic resonance imaging(MRI) demonstrated a lobulated, multiseptated mass in the right ven-tricle that involved the anterior wall; it exhibited high signal intensityon T2-weighted images (Fig. 2). In addition, a right main pulmonarycyst was shown. The enzyme-linked immunosorbent assay (ELISA) forEchinococcus was positive. Abdominal ultrasonography and braincomputed tomography (CT) were normal.The patient was submitted to open heart surgery, and a large cystwas demonstrated invading the right ventricle anterior wall and extend-ing to the epicardium and the outflow tract (Fig. 3). There was a smallrupture on the cyst layer, with an opening to the right ventricularinfundibulum. The cyst was resected, and the anterior right ventriclewall closed with direct stretches with Teflon pledget support. The rightpulmonary artery cyst was extracted by needle aspiration.Albendazole 300 mg/day was administered for 4 months aftersurgery. At 6 and 12 months after surgery the ELISA was repeated, aswere clinical and echocardiographic evaluations. The cardiac murmurhad almost disappeared, as had the previous right ventricular echocar-diographic images, but the ELISA for Echinococcus continued to showCorrespondence to: A.J. MacedoPediatr Cardiol 18:226–228, 1997 PediatricCardiology© Springer-Verlag New York Inc. 1997high values at 18 months after surgery. MRI was repeated, and a rightpulmonary artery cyst was demonstrated, which was resected througha right thoracotomy.DiscussionThe presence of portacaval and Retzius space shunts inchildren favors the occurrence of hydatid cysts predomi-nantly in the lung [2, 9]. In its early stages the Echino-coccus larva arrives at the left cardiac cavities througheither the pulmonary capillary network or the rightatrium due to the patency of the foramen ovale. Reachingthe coronary arteries the larva invades the myocardium,requiring 1–5 years to attain adult form. The clinicalsymptoms induced by hydatid cysts are due to relatedcomplications, such as rupture, obstruction, or compres-sion. Although more frequently located on the left ven-tricle, right ventricular hydatid cysts more frequentlyrupture, leading to pulmonary embolism, an anaphylacticreaction, or sudden dead. Rupture into the pericardialcavity can also lead to pericarditis, effusion, or cardiactamponade [4, 5, 9].In the case reported here, detection of the cardiacmurmur and echocardiography provided the most helpfuldata. The previous anaphylactic reaction could be inter-preted afterward as related to rupture of the cyst into theright ventricle. The ECG was normal, as is usually thecase when the cyst is right-sided [9]; the chest radiographfailed to reveal the typical opacity of a pulmonary hyda-tid cyst. Echocardiographic features of an intracardiacmulticystic mass that is relatively fixed associated witheosinophilia and the fact that the child was in an endemicregion led to the strong suspicion of a cardiac hydatidcyst. The two-dimensional echocardiographic findingsdistinguished this mass from other, more frequent car-diac tumors during childhood, namely rhabdomyoma, fi-broma, and myxoma [7].Both CT and MRI have been used to diagnose car-diac hydatid cysts with variable results [6, 12]. In thiscase, MRI clearly demonstrated the anatomic location ofthe mass, its relation to the cardiac chambers, and thepresence of a cyst in the pulmonary artery. Tissue char-acterization on the basis of a T2-weighted scan addedsome specificity to the diagnosis. After the diagnosis,because of its location and the risk of rupture with con-sequent pulmonary embolization, surgery was performedon an emergency basis.About 10% of all hydatid cysts tend to recur aftersurgery, but this rate may decrease with proper medicaltreatment [10]. The ELISA for Echinococcus proved tobe a valuable monitoring device to screen for recurrenceor undetected cysts. Medical treatment alone is not ef-fective, and surgery is the treatment of choice for cardiachydatid cyst [8]. Albendazole alone or in combinationwith praziquantel is effective prophylactic chemotherapyafter surgery [10, 11].We conclude that any child with an acquired loudcardiac murmur should be carefully studied. Two-dimensional echocardiography, MRI, and ELISA forEchinococcus proved to be excellent methods for estab-lishing the diagnosis in this case, which led to the im-mediate correct treatment of this rare condition.Fig. 1. Transthoracic two-dimensional echocardiography. Parasternalshort axis view at the level of the great arteries. A large multicysticmass is observed inside the right ventricle, narrowing the outflow tract.A small opening in the cyst is seen (arrow), confirmed later on surgery(see Fig. 3C). ao, aorta; rv, right ventricle.Fig. 2. Magnetic resonance imaging. A lobulated (arrows) and multi-septated (small arrows) mass is demonstrated in the anterosuperior wallof the right ventricle (RV).227Macedo et al.: Cardiac Hydatid CystReferences1. Ben-Musa AA, Singh H, Shembesh AH, Chugh JC (1990) Cardiachydatid cyst in a child. Clin Pediatr (Phila) 29:409–4112. Carvalhal A, Lourenc¸o L (1989) Hidatidose na crianc¸a. Arq HospEstefania 4:49–583. David de Morais JA, Rombert PC, Trinca AT (1985) Subsı´diospara o conhecimento da epidemiologia da hidatidose rural no altoalentejo. Rev Port Doenc Infecc 1–4:83–944. Di Bello R, Menendez H (1963) Intracardiac rupture of hydatidcysts of the heart: a study based on three personal observations and101 cases in the world literature. Circulation 27:366–3745. Dighiero J, Canabal EJ, Aguirre CV, Hazan Jacobo, Horjales JO(1958) Echinococcus disease of the heart. Circulation 17:127–1326. Elkouby A, Vaillant A, Comet B, Malmejac C, Houel J (1990)Cardiac hydatidosis: review of recent literature and report of 15cases. Ann Chir 44:603–6107. Farooki ZQ, Adelman S, Green EW (1977) Echocardiographicdifferentiation of a cystic and a solid tumour of the heart. Am JCardiol 39:107–1118. Heilbrunn A, Kittle CF, Dunn M (1963) Surgical management ofechinococcal cysts of the heart and pericardium. Circulation27:219–2289. Perez-Gomes F, Duran H, Tamames S, Perrote JL, Blanes A(1973) Cardiac echinococcosis: clinical picture and complications.Br Heart J 35:1326–133110. Taylor DH, Morris DL (1989) Combination chemotherapy is moreeffective in postspillage prophylaxis for hydatid disease than eitheralbendazole or praziquantel alone. Br J Surg 76:95411. Taylor DH, Morris DL, Reffin D, Richards KS (1989) Comparisonof albendazole, mebendazole and praziquantel chemotherapy ofEchinococcus multilocularis in a gerbil model. Gut 30:1401–140512. Vanjak D, Moutaoufik M, Leroy O, Beuscart C, Billiau V (1990)Cardiac hydatidosis: contribution of magnetic resonance imaging:report of a case. Arch Mal Coeur 83:1739–1742Fig. 3. Intraoperative findings. (A) Anteriorwall of the right ventricle, with an irregular as-pect caused by the cyst invading the epicardium.RV, right ventricle. (B) After opening the wall ofthe right ventricle, multiple cysts (arrow) can beseen. (C) Ruptured cyst can be seen throughoutthe pulmonary valve.228 Pediatric Cardiology Vol. 18, No. 3, 1997

Cardiac Hydatid Cyst in a Child

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