Management and Outcome of Cardiac and Endovascular Cystic Echinococcosis Marta Dı´az-Mene ´ ndez, Jose ´ Antonio Pe ´ rez-Molina, Francesca Florence Norman, Ana Pe ´ rez-Ayala, Begon ˜ a Monge-Maillo, Pilar Zamarro ´ n Fuertes, Rogelio Lo ´ pez-Ve ´ lez* Tropical Medicine and Clinical Parasitology, Infectious Diseases Department, Ramo ´ n y Cajal Hospital, Instituto Ramo ´ n y Cajal de Investigacio ´ n Sanitaria (IRYCIS), Madrid, Spain Abstract Background: Cystic echinococcosis (CE) can affect the heart and the vena cava but few cases are reported. Methods: A retrospective case series of 11 patients with cardiac and/or endovascular CE, followed-up over a period of 15 years (1995–2009) is reported. Results: Main clinical manifestations included thoracic pain or dyspnea, although 2 patients were asymptomatic. Cysts were located mostly in the right atrium and inferior vena cava. Nine patients were previously diagnosed with disseminated CE. Echocardiography was the diagnostic method of choice, although serology, electrocardiogram, chest X-ray, computed tomography/magnetic resonance imaging and histology aided with diagnosis and follow-up. Nine patients underwent cardiac surgery and nine received long-term antiparasitic treatment for a median duration of 25 months (range 4–93 months). One patient died intra-operatively due to cyst rupture and endovascular dissemination. Two patients died 10 and 14 years after diagnosis, due to pulmonary embolism (PE) and cardiac failure, respectively. One patient was lost to follow-up. Patients who had cardiac involvement exclusively did not have complications after surgery and were considered cured. There was only one recurrence requiring a second operation. Patients with vena cava involvement developed PEs and presented multiple complications. Conclusions: Cardiovascular CE is associated with a high risk of potentially lethal complications. Clinical manifestations and complications vary according to cyst location. Isolated cardiac CE may be cured after surgery, while endovascular extracardiac involvement is associated with severe chronic complications. CE should be included in the differential diagnosis of cardiovascular disease in patients from endemic areas. Citation: Dı ´az-Mene ´ndez M, Pe ´rez-Molina JA, Norman FF, Pe ´rez-Ayala A, Monge-Maillo B, et al. (2012) Management and Outcome of Cardiac and Endovascular Cystic Echinococcosis. PLoS Negl Trop Dis 6(1): e1437. doi:10.1371/journal.pntd.0001437 Editor: Hector H. Garcia, Universidad Peruana Cayetano Heredia, Peru Received December 15, 2010; Accepted October 30, 2011; Published January 3, 2012 Copyright: ß 2012 Dı ´az-Mene ´ndez et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: This study was partially supported by the Instituto de Salud Carlos III within the Research Network of Tropical Diseases (RICET RD06/0021/0020 and RD06/0021/0009). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Competing Interests: The authors have declared that no competing interests exist. * E-mail: rlopezvelez.hrc@salud.madrid.org Introduction Cystic echinococcosis (CE) is a zoonotic infection caused by the larval stage of the tapeworm Echinococcus granulosus. This parasitic disease occurs in humans (who act as intermediate hosts) when the ova of E. granulosus from canine (definitive host) faeces are accidentally swallowed. After ingestion, the parasite crosses the duodenal wall and spreads via the portal and systemic circulation to the liver, lungs and other organs. CE is endemic in Europe, including the Mediterranean area and the Balcanic nations, North and East Africa, India, China, Indonesia and the Southern Cone of the Americas [1]. Hydatid cysts may be found at almost any site of the body, but the liver (60–70%) and lungs (10–15%) are most frequently affected [2–4]. The heart is reported to be involved in less than 2% of cases [5–9]. Endovascular extracardiac CE is very rare, only a handful of cases have been reported and these are thought to be secondary to the rupture of the primary germinative membrane of cysts located at other sites, mostly in the heart or vena cava (including the intrahepatic portion of the cava), with consequent embolization to the pulmonary or systemic circulation [10–16]. We describe 11 patients who were diagnosed and treated for cardiac and endovascular CE. Cases from the literature were also reviewed and recommendations for the management of patients are discussed. Methods Ethics statement This was a retrospective analysis, the data were analyzed anonymously and written informed consent was not obtained for individual participants. The database from which patients’ information was obtained has been approved by the Ramo ´n y Cajal Hospital’s Ethics Committee (Comite ´E ´ tico de Investigacio ´n Clı ´nica, CEIC, Hospital Ramo ´n y Cajal) and is used in accordance with the current laws in Spain (Ley Orga ´nica de Proteccio ´n de Datos de Cara ´cter Personal 15/1999) which guarantee patient confidentiality. www.plosntds.org 1 January 2012 | Volume 6 | Issue 1 | e1437