Abstract
Background
Hydatidosis is a parasitic infection caused by Echinococcus granulosus. Hydatidosis has been reported in the lungs, brain, musculoskeletal system, and heart. Cystic hydatid cases observed in the pulmonary artery are quite rarely seen, and cystic hydatid is considered one of the rare causes of nonthrombotic pulmonary embolism.
Case Summary
A 53-year-old female patient with shortness of breath and coughing was examined and found to have a cystic structure measuring 4.1 × 4.9 mm with thrombus on the right atrium. Pulmonary computed tomography angiography revealed a 52 × 55 × 33-mm multiseptate structure containing cystic vesicles within the right atrium and bilateral intrapulmonary cystic lesions measuring 2 to 3 cm. The patient, whose hemodynamics were compromised, underwent emergency surgery, and the pathology result was consistent with hydatid cyst.
Discussion
Cardiac hydatid cysts can be fatal, but due to rare occurrence, there are no defined treatment algorithms. Each case must be evaluated individually using a multidisciplinary approach.
Take-Home Messages
Hydatid cysts are rare, but cardiac involvement is also possible. If cardiac involvement is on the right side of the heart, it is beneficial to check the pulmonary arteries as well.
Key words: echocardiography, imaging, pulmonary hypertension
Graphical Abstract
History of Presentation
A 53-year-old patient with no known medical history, who had recently arrived from abroad, presented to the outpatient clinic with complaints of coughing and shortness of breath. The patient, who was tachypneic and orthopneic with an oxygen saturation of 80, was admitted to the intensive care unit.
Take-Home Messages
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Although rare, cardiac hydatid cysts can spread to the pulmonary arteries and, when they spread and rupture, can be fatal.
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In such cases, it is important to evaluate the pulmonary arteries and lung parenchyma according to the location of the cardiac cysts.
Investigations
The electrocardiography examination revealed sinus rhythm. Echocardiography revealed a 4.1 × 4.9-cm mass in the right atrium near the interatrial septum (the mobile part was consistent with a thrombus, and the lower part had a cystic appearance), the interatrial septum was thin, tricuspid regurgitation severe systolic pulmonary artery pressure 80 mm Hg, aortic regurgitation second degree, mitral regurgitation second degree, and ejection fraction 55% (Video 1). Pulmonary computed tomography angiography was performed based on the observation of thrombus appearance on echocardiography and elevated pulmonary artery pressure. Pulmonary computed tomography angiography revealed a cystic lesion measuring approximately 52 × 55 × 33 mm in the right atrium, which could be related to peripheral cystic vesicles. In addition, multiple cystic lesions measuring 2 to 3 cm in diameter were observed in both lungs, both parahilar and intrapulmonary. The findings primarily support the presence of intracardiac and pulmonary hydatid cysts. The pulmonary trunk diameter was measured at 42 mm. In addition, filling defects consistent with cystic contents or thrombosis were observed in the segmental pulmonary artery branches of the right upper and lower lobes (Figure 1).
Figure 1.
Cystic Masses Seen in the Pulmonary Arteries and Right Atrium on Pulmonary Computed Tomography Angiography
Management
Due to hypotension and general deterioration observed during patient follow-up, emergency coronary angiography was performed prior to surgery, and the patient underwent emergency surgery. As no significant lesions were observed in the coronary angiography, cyst excision was planned first. No cysts were observed in the accessible areas of the pulmonary arteries. A 5 × 5-cm cystic mass filled with vesicles, extending from the lateral wall of the right atrium to the septum, was excised (Video 2, Figure 2). The mass was irrigated with hypertonic solution. A De Vega annuloplasty procedure was performed on the patient, who had a wide tricuspid valve annulus.
Figure 2.
Cystic Masses Removed Intraoperatively
Outcome and Follow-Up
The patient was admitted to the postoperative intensive care unit and remained there for 6 days, followed by 2 days of follow-up in the service after the intensive care unit. The patient, who was in good general condition, was discharged with albendazole 2 × 400 mg and rivaroxaban 20 mg 1 × 1. The pathology report showed findings consistent with hydatid cyst and foreign body reaction with focal necrosis and suppurative inflammation in fibrous tissue fragments.
Discussion
Hydatid cyst is a parasitic infection that is endemic in areas with poor sanitation conditions and commonly affects the liver and lungs.1,2 The prevalence of cardiac hydatid cysts ranges from 0.5% to 2%, and they are most commonly observed in the left ventricle.3 Involvement of the right atrium and pulmonary arteries is less common. Depending on its location, it can cause symptoms such as chest pain, palpitations, shortness of breath, and coughing.1,4 It can also lead to arrhythmia, valve disease, systemic or pulmonary embolism, and pulmonary hypertension.1,4
Our patient had a rare cardiac hydatid cyst, which was located in the right atrium. In addition, hydatid cysts were also seen in the bilateral pulmonary arteries.
Hydatid cysts in the pulmonary artery can form in different ways. First, they can be seen as emboli from the primary cardiac focus. Second, it can be seen as the spread of cyst embryos from the inferior vena cava to the right atrium and then to the pulmonary arteries. Finally, it can also spread to the pulmonary artery by invading the artery wall from the lung.5,6 In our patient, cysts were present in the right atrium and lung parenchyma, so cysts could be seen in the pulmonary arteries through all these pathways.
Cardiac hydatid cysts can cause cerebrovascular disorders by embolizing in the left heart chambers, and in the right heart chambers, it can cause pulmonary embolism by embolizing. Depending on its location, it can cause valve regurgitation by exerting pressure or cause death by causing anaphylaxis due to sudden rupture. For these reasons, it is a disease that requires early diagnosis and early treatment with a multidisciplinary approach.7, 8, 9
Albendazole (ABZ) (15 mg/kg twice a day) is recommended as a drug for the medical treatment of hydatid cysts.10 ABZ is more effective in liver cystic hydatids, but its effectiveness varies depending on the size, location, and stage of the cyst. It has been reported to be more beneficial in C1, C2, and C3a cysts in the liver.10,11 It has been reported that hydatid liver cysts smaller than 5 cm may benefit from medical treatment.10 Mebendazole, 40 to 50 mg/kg/d, is recommended as an alternative to ABZ.12 Other treatment options for hydatid cysts of the liver are surgery and the PAIR (puncture, aspiration, injection of a scolicidal agent, and re-aspiration) method.11,13
Surgery is recommended in cases of cardiac hydatid cyst treatment where there is a life-threatening tamponade, anaphylactic reaction, or rupture. It has been reported that the use of ABZ prior to the surgery increases the effectiveness of treatment in reported cases of cardiac hydatid cysts.10 Irrigation with hypertonic saline is also recommended during surgery. In addition, continuation of medical treatment is recommended after surgery.14
In our patient, surgical intervention was performed urgently due to hypotension and deterioration of the general condition.
According to World Health Organization data, more than 1 million people are affected by echinococcosis at any given time.15 According to World Health Organization data from 2015, echinococcosis is estimated to cause 19,300 deaths and approximately 871,000 disabilities worldwide each year.15 To prevent hydatid cysts, which are among the causes of morbidity and mortality, attention should be paid to the cleanliness of fruit and vegetable consumption, care should be taken not to eat vegetables raw, contact with infected animals should be avoided, and local veterinary visits and inspections should be increased for the purpose of early detection of infected animals.16
Conclusions
When hydatid cysts are diagnosed in patients living in endemic regions, the heart chambers and lung parenchyma should be examined in detail. When found in the lung parenchyma, the pulmonary arteries should be examined in detail for direct passage from the parenchyma, and when found in the heart chambers, for passage via embolism.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
For supplemental videos, please see the online version of this paper.
Appendix
Cystic Mass Seen in the Right Atrium on Echocardiography
Intraoperative Removal of Cystic Vesicles
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Associated Data
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Supplementary Materials
Cystic Mass Seen in the Right Atrium on Echocardiography
Intraoperative Removal of Cystic Vesicles