Cardiac Hydatid Cysts- Review of Recent Literature
- 1. Department of Cardiovascular Surgery, Nev?ehir State Hospital, Turkey
- 2. Department of Cardiovascular Surgery, Ankara University School of Medicine, Turkey
ABSTRACT
Echinococcus is a parasiticsource of infection that occurs in humans in endemic regions of the world. It causes medical, veterinary and economic problems for endemic developing countries.Primary echinococcosis of the heart represents 0,5-2% of all hydatid disease. This is a retrospective review of recent literature that consists information about 35 case report studies from different regions and aims to emphasize the significant points of approach principals to cardiac echinococcosis.
CITATION
Yaman ND, Sirlak M (2017) Cardiac Hydatid Cysts- Review of Recent Literature. J Vet Med Res 4(8): 1102.
KEYWORDS
• Cardiac echinococcosis
• Hydatid cyst
• Echinococcus granulosus
• Albendazole
ABBREVIATIONS
CT: Computerized Tomography; MRI: Magnetic Resonance Imaging; IHA: Indirect Hemagglutinin; ELISA: Enzyme-Linked Immunosorbent Assay
INTRODUCTION
Human cardiac echinococcosis is of worldwide severity and causes medical, veterinary and economic problems for endemic developing countries. The diagnosis of cardiac echinococcosis can be hard and must be suspected in any patient from endemic or farming areas. It should be considered in the differential diagnosis of tumoral and cystic masses and treat properly with combination of different methods. We report a review of recent literature, and we aim to emphasize the significant points of approach principals to cardiac echinococcosis.
DISCUSSION
Review
We retrospectively reviewed the latest reports of cardiac echinococcosis cases from different regions of the World between 2014 and 2017. Searching the terms: ‘cardiac hydatid cysts and cardiac echinococcosis’. All papers in English were included. 41 were females and 45 were males. 42 patients lived in Turkey and, the others were from Iran (five cases), Saudi Arabia (one case), Italy (two cases), Pakistan (one case), China (26 cases), South West Bengal (one case), Greece (one case), Tunusian (one case), India (four cases), Peruvian (one case) [1-35]. Cases are described in detail on (Table 1).
Five patients had refused to undergo surgery, they had been treated with chemotherapy of albendazole. Others were operated for total excision of cysts in elective surgery under cardiopulmonary bypass.
Albendazole was used in 77 patients pre and postoperatively, mebendazole was used in one patient.We could not have data from reports about medication of eight patients.
At postoperative course, all survived and operated cases had good health, and hadno recurrence. Two patients had died, one of them was because of anaphylactic shock during surgery and one was immediate death while on the waiting list for surgery.
Etiology and epidemiology
Echinococcus is a parasiticdisease that occurs in humans in endemic regions of the world and is caused by the larval stage of Echinococcus granulosus, E.multilocularis, or E.voceli [35]. Infection arises from handling dogs or ingesting cyst-containing meat from an intermediate host in an endemic area. Echinococcus multilocularis is adjusted to circulate between wild and domestic canids as definitive hosts and small mammals as intermediate hosts. Small mammals combine limited space for the metacestode growth with a short life time, so that morphological differences to metacestodes of other Echinococcus spp. (compact, vesicular growth with high density of protoscoleces) and the short time needed for the development of protoscoleces can be explained as an evolutionary response to conditions. In the largest part of the endemic area E. multilocularis life cycles are based on rodents, predominantly voles (Arvicolinae), and different species of canids that prey on them. Based on field data and/or experimental infections, other families of Carnivora are caused to be partially or completely refractory to infection. The high number of human cases certainly reflects the wide distribution and high frequency in dogs and livestock, but an apparently low specificity at the intermediate host level may also contribute to an enhanced infectivity or pathogenicity for humans compared to other Echinococcus spp. causing cystic echinococcus. Epidemiological data suggest that this species is particularly well adapted to sheep as intermediate hosts, which is reflected in high prevalence [36]. That seems as one of the main reasons of the prevalence and severity of Echinococcus manifestations.
In the following paragraph, we briefly discuss the general life cycle on the definitive and intermediate hosts. The adult forms lay eggs in the small bowel of the definitive host (commonly dog), which are passed in the feces. After ingestion by an intermediate host (sheep or goat), the egg releases an oncosphere that migrates via the circulation into different organs. The oncosphere develops into a cyst, producing protoscolices and daughter cysts that fill the interior of cyst. The common non-urban areas’ practice of feeding sheep organs to dogs in turn makes easier transmission back to the dog. Humans become infected through contact with dogs via a fecal-oral route in an incidental manner. Intermediary transmission of eggs by flies and arthropods may also result in infection of humans [37]. The average duration of the life of cardiac echinococosis in humans is 10–20 years and the annual growth rate of the cyst is commonly about 1–5 cm in diameter [38,39].
Primary echinococcosis of the heart represents 0,5-2% of all hydatid disease causes in endemic regions [40].
Echinococcus granulosus is widespread in regions of Eurasia, South America and Africa. In the sole animal study for the moment in literature of 39,738 domestic livestock in Pakistan, 6.67% of animals were found to be infected. The prevalence of hydatid cysts was highest in camels (17.29%) followed by sheep (7.52%), buffalo (7.19%), goats (5.48%), and cattle (5.18%) [41]
Localizations
Hydatid cysts usually affect liver and lungs, although any part of the body can be involved. These cysts can arise as part of an extensive systemic infection or as a separated event [42].
Cardiac hydatid cysts are very uncommon found in fewer than 2% of cases of hydatidosis and most usual cardiac localizations are in the myocardium of the left ventricle wall. The most common cardiac locations are the left ventricular wall (60%), followed by the right ventricle (10%), pericardium (7%), left atrium (6-8%), right atrium (3-4%), and the interventricular septum (4%) [43]. In 50% of such cardiac cases, there is multiple organ inclusion [44].
In this review of literature, 25 cysts were in the left ventricule, 3 were in the right ventricule, 11 were in the interventricular septum, one was in the aortic lümen and others were extended to multiple localizations. All serologic tests were positive except three of them and the ones that were not given data.
Larvae usually extend to the myocardium through the systemic or pulmonary circulation or as direct extension from near organs and that is a uncommon localisation.
Clinical presentation
The clinical presentation in cardiac hydatid cyst is commonly spreading in a hidden way. The slowly growing larger echinococcal cyst generally remains asymptomatic until the space-occupying effect in an included organ brings out symptoms. Early diagnosis and management are very significant given the lethal risk of cyst perforation. The endocardial infection occurs infrequently in comparison to the myocardial involvement. Again, hydatid cysts may seldom develop within pulmonary arteries following ruptured cardiac or hepatic cysts [45].
The disease can stay asymptomatic (90%), but it may cause change of position of the coronary vessels, arrhythmias and mechanical interference with the atrioventricular valves and ventricular function via cysts’ mass effect. Compression of the coronary arteries by a cyst can cause myocardial infarction. Pulmonary embolism could complicate the course. It may result from rupture of cardiac or hepatic cysts and may only be diagnosed postmortem [46-50].
Common symptoms were dyspnea, chest pain, palpitation, loss of appetite and fever. Two patients had acute peripheral arteriel embolic events and two had cerebral events with loss of conciousness and seizures.
Cardiac echinococcosis can be manifested by rhythm disturbances, angina pectoris, valvular malfunction, pulmonary or systemic embolism, and right or left ventricular outflow tract obstruction. Cyst perforation is the most hazardous complication, ending in anaphylactic shock, thrombo embolism, and death in approximately 75% of patients [51,52].
Table 1: Studies of cardiac echinococcosis world wide.
Author | Age Gender Region | Symptoms | Size of cyst | Localisation | Serology | Syrgery | Medication | Follow up |
Poorzand H. et al. 2014 (1) | 29 Female Iran | Shortness of breathe | 50x47 mm | RV | + | OHS | Albendazole 400 mg/day | Good, 1 year |
Gocen U. et al. 2014 (2) | 6 Female Turkey | Chest pain Dyspnea | 40x30 mm | IVS | + | OHS | Albendazole 400 mg/day | Good, 6 mo. |
Alshehri H. et al. 2014 (3) | 29 Female Saudi Arabia | Palpitation | 70x60 mm | IVS | _ | OHS | Albendazole 400 mg/day | Good, 4 mo. |
Naeem S. et al. 2013 (4) | 48 Female Pakistan | Shortness of breathe Loss of appetite | 22x30 mm | IVS | _ | OHS | Albendazole 400 mg/day | Good, 6 mo. |
Inzirillo F. et al. 2013 (5) | 85 Female Italy | Dyspnea | nd | Pericardial | nd | OHS | nd | Good, 6 mo. |
Suner A.et al. 2014 (6) | 21 Female Turkey | Dyspnea Arryhtmia (RBBB) | nd | LA, sec.ASD, PA | nd | OHS | Albendazole | Good |
Yan F.et al. 2014 (7) | 26 patients 11 female, 15 male Mean age:28±7,6 China | Dyspnea(15) Chest pain(11) Palpitation(8) Cough(7) Fever(2) | 50x40 mm (range 10- 135 mm) | LV (7) RA (2) IVS (1) | + (15) | OHS CBP(15) | Albendazole (10-15 mg/kg/ day) | 5 death, 1 recurrence, 20 good. 68±21 mo. |
Fiergo L.et al. 2014 (8) | 11 Female Turkey | Chest pain Palpitation | 90x60 cm | LV | + | OHS | Albendazole 400 mg/day | Good, 18 mo. |
Sava? G.et al. 2014 (9) | 44 Female Turkey | Dyspnea Chest pain | 53x45 mm | LV | + | OHS | Albendazole | Good, 2 mo. |
Altas O.et al. 2014 (10) | 3 patients (3 males) mean age:39 (24-44years) | Shortness of breathe Fatigue Chest pain | 48x28, 25x21, 36x24 mm | IVS(1) LV(1) IVS(1) | nd | OHS | Albendazole 800 mg/day | Good |
Y?ld?z CE.et al. 2014 (11) | 27 Male Turkey | Dyspnea Chest pain Fatigue,Fever | 100x60 mm | LV | + | OHS | Albendazole | Good, 6 mo. |
Khosravi A.et al. 2014 (12) | 13 Male Iran | Dyspnea Chest pain | 73x53 mm | LV | + | OHS | Albendazole | Good, 2 years |
Sahin I.et al. 2015 (13) | 31 Female Turkey | Dyspnea Limb edema Chest pain Palpitation | 55x49 cm | RV | + | OHS | Albendazole | Good, 1 year |
Dasbaksi K.et al. 2015 (14) | 39 Female South West Bengal | Asymptomatic | 120x50 mm | LV | + | OHS | Albendazole 800 mg/day | Good, 5 years |
Mirzaie A.et al. 2014 (15) | 14 Male Iran | Acute lower extremity pain | 40x35 mm | LV | + | OHS | Albendazole 400 mg/day | Good, 7 years |
Tosya A.et al. 2015 (16) | 53 Male Turkey | Recurrent arteriel embolic events | 30x40 mm | Aortic lumen | nd | OHS | Mebendazole (50 mg/kg/day) | Good, 2 years |
Aggeli C.et al. 2016 (17) | 56 Female Greece | Asymptomatic | 35x35 mm | LV | nd | No surgery | Albendazole | Good, 6 mo. |
Parakh N.,et al. 2016 (18) | 52 Female India | Shortness of breathe Dizziness | 100x80 mm | IVS | nd | OHS | nd | Exitus on the day of surgery |
Jain N.et al. 2015 (19) | 50 Female India | Dyspnea | 78x69 mm | LV | + | OHS | nd | Good |
Charfeddine S.et al. 2015 (20) | 36 Female Tunusian | Chest pain Lack of appetite | 50x48 mm | LV | + | OHS | Albendazole | Good, 6 mo. |
Salehi R.et al. 2015 (21) | 54 Female Iran | Asymptomatic | 85x65 mm | IVS | nd | OHS | nd | Good |
Ohri S.et al. 2015 (22) | 67 Male India | Chest heaviness | 23x21 mm | LV | + | OHS | Albendazole, 800 mg/day | Good, 6 mo. |
Sabzi F.et al. 2015 (23) | 45 Male Iran | Chest pain Dyspnea | nd | LV | + | OHS | Albendazole | Good, 6 mo. |
Celik M.et al. 2016 (24) | 23 Male Turkey | Exertional dyspnea | 41x32 mm | RV | nd | OHS | Albendazole | Good |
Saglican Y.et al. 2016(25) | 35 Female Turkey | Palpitation Dyspnea | 65x50 mm | IVS | nd | OHS | Albendazole Good, | 1 year |
Sarli B.et al.2016 (26) | 62 Female Turkey | Palpitation Dyspnea | 90x60 mm | IAS | + | OHS | nd | Died of anaphylactic shock |
Demirel M.,et al. | 2016 (27) 55 Female Turkey | Dyspnea | nd | LV | + | No surgery | Albendazole 800 mg/day | Good, 6 mo |
Patrizia C.et al. 2016 (28) | 40 Male Peruvian | Asymptomatic | nd | LV | + | No surgery | nd | Good |
Mirijello A.et al. 2016 (29) | 23 Male Italy | Loss of consciousness | 51x43 mm | IVS | + | No surgery | Albendazole 400 mg/day | Good |
Yasim A.et al. 2016 (2005-2013) (30) | 25 patients 15 male, 10 female Mean:33,4±12,6 years (15-75) | Dyspnea | nd | 11 intracaviter 14 extracaviter | +(22) _(3) | OHS | Nd | 51±29 mo. Good, Except 1 death |
Alonso J.et al. 2016 (31) | 21 Male Romanian | Loss of Memory, seizures | nd | LV | nd | OHS | Albendazole | Good |
Kocabay G.et al. 2016 (32) | 51 Male Turkey | Asymptomatic | nd | LV | + | OHS | Albendazole | Good |
Yaman M.et al. 2016 (33) | 33 Female Turkey | Chest pain During ceaseraen surgery | 28x36 mm | IVS | + | No surgery | nd | Exitus |
Kothari J,et al. 2016 (34) | 28 Male India | Chest pain | 92x96 mm | LV | nd | OHS Albendazole, | 400 mg/day | Good, 2 mo. |
Sirlak M.et al. 2007 (35) | 32 Male Turkey | Chest pain Weight loss Lethargy | 50x45 mm | LV | + | OHS | Albendazole | Good, 1 year. |
LV: Left ventricle; RV: Right ventricle; LA: Left atrium; RA: Right atrium; nd: no data; OHS: Open heart surgery; IVS: inter ventricular septum; IAS: Interatrial septum |
DIAGNOSIS
The diagnosis of cardiac echinococcosis can be hard and must be suspected in any patient from endemic or sheep farming areas with a cystic tumour of heart. The diagnosis is usually achieved by combination of clinical findings, imaging and serology. For radiological image part of cardiac echiococcosis, chest X-ray, echocardiography, computerized tomography (CT) or magnetic resonance imaging (MRI) might be used.
Transthoracic echocardiography is showing the cyst with echo-negative components and regular contours is the most effective method of diagnosing the hydatid cyst. It is possible to get more detailed images with transoesefageal echocardiography. Echocardiography is the preferred diagnostic method because of its low cost and availability. Nevertheless, echocardiography is operator-dependent, with a limited field of view to see the area behind the sternum may be difficult or impossible to examine [53].
Other diagnostic steps to be taken accordingly enclose CT scan and MRI, and the performance of serologic tests. CT and MRI are superior to echocardiography for evaluation of pericardial masses and their relationship to surrounding tissues and extension to myocardium. CT can identify cysts, but it often fails to detect small lesions, it best shows wall calcification. MRI is the technique chosen for follow up. Specific signs of hydatid cyst include calcification of wall, the presence of daughter cysts, and membrane detachment [54].
Routine laboratory tests are not specific and may reveal normal or abnormal values. The blood count may show eosinophilia. Serologic tests are of limited value in diagnosis. The Caroni intradermal test and serology (indirect hemagglutin in (IHA) or enzyme-linked immune sorbent assay (ELISA) techniques) are useful but are subject to false negativity [55].
ELISA has sensitivity and specificity of 94% and 99%, respectively, and along with IHA test, it has proved to be the best choice for follow-up. Indirect fluorescent antibody and enzyme immunoassay complement fixation (Weinberg] tests are also used [56].
In patients with initial negative serology, serotesting using IHA, latex agglutination or radioallergosorbent testing should be done as a combination of several tests can improve the diagnostic exactness [57].
TREATMENT
Surgery has customarily been the primary method of treatment due to the high risk of associated complications, even in asymptomatic cases, and the safest way to remove the cyst definitely is under cardiopulmonary bypass. The principles of surgery are to avoid handling of heart before cross clamping, and complete excision of germinal layer. Some prefer to let the residual cavity heal by secondary intent to avoid complications. Preventing contamination of the surgical field is extremely important [58]. Surgical risks include anaphylaxis and dissemination due to the cystic fluid.
Multiple agents have been intended as helminthicides: 2%formalin, 0.5% silver nitrate solution, 20% hyper tonic saline solution, 1% iodine solution, or 5% cetrimonium bromide solution. There is no evident superiority and preference among these substances. It has been suggested that hypertonic saline solution-soaked pads be distributed within the pericardial cavity in order to prevent dissemination intra operatively. Albendazole, an active agent against Echinococcus, should be administered adjunctively. It is cautious to begin albendazole several days before surgery and continue for several weeks after resection [59-61]. About 10% of all hydatid cysts tend to occur again after surgery, but may decrease with convenient medical treatment [62,63]. To reduce the recurrence, supplemental medical treatment with mebendazole or albendazole is recommended. Chemotherapy is contraindicated for large, inactive or calcified cysts with a risk of rupture [64]. There are also few systemic conditions that cause contradiction of pharmacotherapy as bone marrow depression and pregnancy. Prophylactic chemotherapy of albendazole duration can be regulated according to the number of cystic lesions and organ extension [65].
As conclusion, cardiac hydatidosis should be considered in the differential diagnosis of tumoral and cystic masses and treat properly with combination of different methods.