Download Huge Pericardial Cyst with Compressing Effects on the Right

Arch Cardiovasc Imaging. 2016 August; 4(3):e45736.
Published online 2016 August 28.
doi: 10.5812/acvi.45736.
Case Report
Huge Pericardial Cyst with Compressing Effects on the Right-Heart
Chambers
Mehmet Onur Omaygenc,1,* Ibrahim Oguz Karaca,1 Beytullah Cakal,1 Haci Murat Gunes,1 Filiz
Kizilirmak,1 and Bilal Boztosun1
1
Cardiology Department, Istanbul Medipol University Hospital, Istanbul, Turkey
*
Corresponding author: Mehmet Onur Omaygenc, Istanbul Medipol University Hospital, Cardiology Department, TEM Otoyolu Goztepe Cikisi No:1, 34214 Bagcilar, Istanbul,
Turkey. Tel: +90-2124607774; +90-5054424718, Fax: +90-2124607059, E-mail: [email protected]@medipol.edu.tr
Received 2016 July 02; Revised 2016 July 30; Accepted 2016 August 20.
Abstract
Introduction: Pericardial cysts are rare abnormalities of the mediastinum and generally have a benign course. Patients are mostly
asymptomatic, and diagnosis is established incidentally in a majority of cases. The management of this asymptomatic population
is also controversial.
Case Report: We report a huge pericardial cyst, located at the right cardiophrenic angle impairing ventricular filling properties
without causing evident symptoms. The cyst was almost 13 cm in the largest diameter. Echocardiography and computed tomography scan were utilized not only to confirm the diagnosis, but also to determine the treatment strategy.
Conclusions: Surgical or percutaneous interventional treatment for pericardial cysts might be occasionally necessary, depending
on the location of the cyst and its relationship with the adjacent structures. Unfortunately, our patient refused any kind of treatment
option. Based on this report, the course of the disease, diagnostic modalities, and treatment strategies in general manner were
additionally discussed.
Keywords: Mediastinal Cyst, Pericardial Cyst, Pericardial Fluid
1. Introduction
Pericardial cysts are rare abnormalities of the mediastinum and generally have a benign course. Patients are
mostly asymptomatic unless major anatomic structures
are compressed or rupture of the cyst occurs. Unfortunately, the management of this rare situation is controversial. The presence of large or gradually enlarging cysts and
suspicion for a diagnosis other than a benign cyst might
necessitate therapeutic intervention (1, 2).
2. Case Report
A 42-year-old man was admitted to our outpatient
clinic with mild exertional dyspnea. He was a heavy
smoker and had a previous history of coronary stent implantation. He did not report a recent chest trauma. In
his physical examination, the heart sounds were almost
inaudible and the respiratory sounds were markedly reduced with rhonchi in all the lung fields. Chest X-ray, aside
from pulmonary parenchymal abnormal findings, demonstrated a mass adjacent to the right ventricle (Figure 1A).
ECG was normal, and the treadmill stress test result did not
reveal significant myocardial ischemia.
In echocardiographic examination, a large cystic structure compressing not only the right atrium but also the
right ventricular base was observed in the apical and subcostal views (Video 1, Video 2, and Figure 1B). A significant
respiratory variation was detected in the tricuspid in-flow
Doppler recordings. The mitral in-flow was also affected,
mainly due to impaired right ventricular filling. Accordingly, an undulation in preload and a slight ventricular interdependence were established (Figure 1C). The left ventricular volumes and functions were preserved. The inferior vena cava collapse during inspiration was within normal limits.
Multislice computed tomography (CT) scan confirmed
the diagnosis. A contrast-free cyst (127 × 68 × 100 mm),
adjacent to the right-heart chambers, was detected. (Figure 1D). The cyst was located in the pericardial cavity, and
the contents were postulated to be in fluid density (9
HU). Diffuse paraseptal and centrilobular emphysematous
regions, pleuroparenchymal bands, and nodular lesions
were also noticed in the pulmonary parenchymal sections.
Apart from these, neither altered levels of serum inflammatory markers or serologic tests nor abnormal imaging
data consistent with echinococcosis or tuberculosis were
detected.
The patient was consulted by the local heart team and
Copyright © 2016, Iranian Society of Echocardiography. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0
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Onur Omaygenc M et al.
due to the huge size of the cyst and reduced filling properties, a consensus on the necessity of surgical or percutaneous intervention was achieved. Unfortunately, he refused these treatment options. At the 6th month’s visit, no
symptom progression was observed and the echocardiographic findings were similar.
3. Discussion
Pericardial cysts are considerably rare, with an overall
incidence of 1 in 100,000 patients, and constitute 6% of
all mediastinal masses (3). In line with this statement and
to the best of our knowledge, approximately 25 striking
cases and literature reviews have been published and cited
in conventional citation indices since 2001 (1, 4, 5). These
cysts are usually unilocular, well-delineated spaces encapsulated by a fibrous wall which is lined with mesothelium.
The cavity is filled with a clear fluid. The most common locations of these benign cysts are both (mainly right) cardiophrenic angles (1, 2, 4). Most of these lesions range between 1 and 5 cm in diameter, but rarely cysts measuring
over 20 cm have also been reported in the literature (4).
Pericardial cysts are almost invariably located at the
middle mediastinum. Aside from lymphoma, various benign cysts like bronchogenic, enteric, and rarely thymic
cysts can be counted in the differential diagnosis. Cardiac or mediastinal hydatid cysts may also cause confusion. Calcification of the cyst wall and characteristic imaging properties such as floating membranes and daughter
cysts may help distinguish them from other primary mediastinal masses (4, 6).
In our patient, we did not observe any radiologic appearance consistent with echinococcosis inside the cavity.
The inflammatory markers were within the normal limits,
and the serum analysis for specific antibodies was also negative. Meanwhile, concomitant presence of tuberculosis
was investigated and relevant biochemical and radiologic
evidence was not observed. As for the exclusion of major
differential diagnoses, the cyst was conclusively defined as
a gradually expanding congenital cyst.
Patients with pericardial cysts are generally asymptomatic, and the diagnosis is established incidentally in a
majority of cases. Nevertheless, dyspnea, palpitations, and
persistent cough may exist as initial symptoms. Hemoptysis, syncope, and fever are uncommon presentations and
might indicate a local complication. Cyst rupture, cardiac tamponade, congestive heart failure, atrial fibrillation, right ventricular outflow tract obstruction, and obstruction of the right main bronchus can be considered
possible complications at a glance (1, 2, 4). In our patient,
mild symptoms and pulmonary auscultation findings necessitated a chest X-ray and diagnostic workup was started
2
Figure 1. A, Elliptical Mass is Seen Adjacent to the Right Ventricle (Arrows); B, Pericardial Cyst is Demonstrated by Transthoracic Echocardiography in the Subcostal View
(Star); C, Significant Respiratory Variation is Seen in Mitral In-Flow Doppler Recordings; D, A Contrast-Free Pericardial Cyst is Located at the Right Cardiophrenic Angle.
henceforward. Retrospectively speaking, almost absent
heart sounds on the right of the sternum might be associated with the localization of the cyst. However, on the left
side, it should probably be interpreted as the solidification
of the pulmonary parenchyma and the masking of roaring
expiratory auscultation findings.
The key point of the further investigation process is
echocardiographic examination. Pericardial cysts are observed as echolucent spaces adjacent to the parietal pericardium. As was the case in our patient, due to the mass
effect of the cyst, filling properties might be impaired (1,
3, 5). The definition of the actual size of the cyst and its
relationship with the neighboring structures requires CT
or magnetic resonance imaging (MRI) as the method of
choice after a comprehensive echocardiographic examination. The cysts appear as thin-walled, usually unilocular
and well-delineated homogeneous cavities. In MRI imaging, they have distinctive signal-intensity alterations in T1
and T2 weighted sequences. Pericardial cysts fail to display
contrast enhancement in both methods, and this condition helps distinguish these lesions from other pericardial
disorders (1, 3). Our case absolutely met these features, and
the localization of the cyst was typical. Since probable alternative pathologic conditions were unlikely, a congenital pericardial cyst was the definitive diagnosis.
The management strategy vis-a-vis pericardial cysts is
based on symptomatology. Asymptomatic patients can be
followed up by serial echocardiographic e examinations.
However, extensive cyst size, compression of the adjacent
structures, and suspicion of a solid component may raise
the need for intervention. Percutaneous aspiration is the
1st-line treatment for appropriate cases. In case of recurrence or possible malignant disease, surgical resection
(thoracotomy or video-assisted thoracoscopic surgery) is
required (1-3).
It is mandatory to note that recommendations regarding the diagnostic process and treatment algorithms
Arch Cardiovasc Imaging. 2016; 4(3):e45736.
Onur Omaygenc M et al.
mainly rely upon large case series and literature reviews.
Thus, the management of asymptomatic or mildly symptomatic patients is still a matter for debate and more evidence is required particularly as regards follow-up data on
those with bulky masses.
Footnotes
Authors’ Contribution: Mehmet Onur Omaygenc: corresponding author; Ibrahim Oguz Karaca: language editing, data collection; Beytullah Cakal: patient follow-up, formation of figures; Haci Murat Gunes: data collection; Filiz
Kizilirmak: structural editing; Bilal Boztosun: senior author.
Conflict of Interest: None.
Financial Disclosure: None.
Arch Cardiovasc Imaging. 2016; 4(3):e45736.
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