Continuing Education Activity
A pericardial cyst is typically considered a congenital anomaly, with most patients diagnosed via incidental findings on routine chest imaging. Rarely can they become symptomatic and require treatment or intervention. This activity reviews the diagnosis, evaluation, and management of pericardial cysts and highlights the interprofessional team's role in evaluating and managing patients with this condition.
Objectives:
Identify the most common presentation of a patient with a pericardial cyst.
Determine the etiology of a pericardial cyst.
Assess the typical imaging findings associated with pericardial cysts.
Communicate the importance of interprofessional team strategies to enhance care delivery for patients with pericardial cysts.
Access free multiple choice questions on this topic.
Introduction
A pericardial cyst is typically considered a congenital anomaly, with most patients diagnosed via incidental findings on routine chest imaging. Rarely can they become symptomatic and require treatment or intervention.
Etiology
Classically, a pericardial cyst is considered a congenital anomaly whereby incomplete fusion in embryogenesis leads to herniation or weakness in the pericardial sac, forming a diverticulum. This outpouching can persist as a pericardial diverticulum or form a pericardial cyst when the communication to the pericardial sac becomes obliterated. These cysts usually contain clear fluid.
Some retrospective data suggest the average diameter of these cysts is about 5.4 cm.[1] Less frequently, pericardial cysts are acquired, such as after cardiothoracic surgery, after an inflammatory process such as pericarditis or echinococcosis, post-trauma, or reportedly, in patients on chronic hemodialysis.[2][3][4]
Epidemiology
Pericardial cysts are considered rare, with an approximate incidence of 1 in 100,000 persons and comprising an estimated 33% of all mediastinal cysts and 7% of mediastinal masses.[5] They are usually discovered as incidental findings on routine imaging and have been diagnosed in all ages, including antenatally and in patients up to 102 years of age.[6]
Pathophysiology
Patients with pericardial cysts are mostly asymptomatic, with the discovery of reportedly up to 75% of pericardial cysts being incidentally.[1] Symptoms may occur via compression and mass effect in the remainder of patients when the cyst impinges upon or erodes into adjacent structures. This symptomatology can present with cardiac compression and subsequent retrosternal pain radiating to the shoulder, compression of the right side of the heart with a deviation of the septum, diastolic dysfunction, right ventricular outflow tract obstruction, pulmonary stenosis, mitral valve prolapse, or congestive heart failure.
Compression of the lung and hila can also occur with obstruction of the right mainstem bronchus and compression of the adjacent lobes of the lung.[7] This obstruction can cause repeated respiratory infections, cough, dyspnea, and cyanosis. In some instances, cyst infections can result in pericarditis. Rupture of the pericardial sac can also occur, causing pleuropericarditis and pneumonitis. Even more rarely, atrial fibrillation or erosion of the cyst into the SVC and right ventricular wall can occur, along with recurrent syncope.[7] There are rare reported cases of torsion at the neck of the diverticula or cyst, causing chest pain.[1]
Histopathology
Histopathologically, a pericardial cyst has a relatively histologically simple wall of fibrous tissue lined by simple cuboidal mesothelial cells without specialized epithelium or smooth muscle cells.[8]
History and Physical
As mentioned, patients with pericardial cysts are predominantly asymptomatic, with reportedly <25% of patients presenting with symptoms due to compression or erosion of adjacent structures. There have been reports of a large 11 x 11 cm pericardial cyst, causing substernal pain radiating to the bilateral shoulders after a long road trip, only discovered incidentally on imaging ordered to assess for pulmonary embolism.[9]
Common symptoms are vague and can include chronic cough, chest pain, dyspnea, and retrosternal pressure. Rarely, dysrhythmias, syncope, and pneumonia can occur.[7] Exceedingly rare life-threatening complications such as pericardial tamponade have been reported, associated with pericardial cysts.[2] Physical examination typically yields no clues in the diagnosis of pericardial cysts.
Evaluation
As mentioned earlier, clinicians diagnose most cases as incidental findings on chest radiographs, which can demonstrate a round, circumscribed mass adjacent to the heart, statistically most common at the right cardiogenic angle, with a reported incidence of 51% to 70%. They occur less commonly at the left cardiophrenic angle, with a reported incidence of 22% to 38%. Finally, mediastinal locations not adjacent to the diaphragm are approximately 8% to 11%.[10]
Laboratory tests and electrocardiography are generally inconclusive in assessment for pericardial cysts.[1] Computerized tomography (CT) scan without contrast has been reported as the modality of choice for delineating the pericardial anatomy and subsequent diagnosis, as it can aid in precise localization and characterization. Findings typically include a single nonenhancing, thin-walled, ovoid homogenous mass without solid components. A disadvantage occurs if protein content increases (such as with bleeding), leading to errors in reporting.[7]
Some authors suggest that echocardiography is a superior modality for delineating pericardial borders and characterizing the cyst from adjacent structures.[2] Other researchers indicate echocardiography is not a preferred method because of the narrow window for visualization, operator-dependent modality, and technical difficulties (ie, in the case of obesity).
A cardiac MRI is recommended further to evaluate the possible compressive effects of the pericardial cysts, though this exam is costly and time-consuming. Additional errors in reporting can be seen in this modality, as well, if cyst protein content is high, thus altering signal characteristics. Pericardial cysts cannot enhance in contrast imaging of either a CT scan or MRI.[7]
Treatment / Management
As the majority of patients are asymptomatic, treatment is usually conservative. Serial transthoracic echocardiography can assess for stability. If a patient remains asymptomatic and the cyst has not enlarged, continued surveillance and conservative management merit consideration.
If the patient becomes symptomatic or the cyst appears to be enlarging, the patient may become a candidate for surgery, mainly to prevent life-threatening emergencies and compressive effects. In the minority of those patients that become symptomatic, the patient can become a minimally invasive surgical candidate at the surgeon's discretion. These procedures can include percutaneous aspiration (recommended by the European Society of Cardiology) or ablation/ethanol sclerosis.[1]
The recurrence rate of pericardial cysts after aspiration is about 33%. There are no available data regarding adhesion formation or recurrence after alcohol sclerosis. Otherwise, surgical options include resection of the cyst via thoracotomy, sternotomy, VATS, or mediastinoscopy.[7] The recommendation for the treatment of pericardial cysts is based on observational data. As such, tailored management is necessary.
Differential Diagnosis
Pericardial cysts may simulate pericardial fat or ventricular aneurysm on plain chest radiographs. Differential diagnoses would also include diaphragmatic tumors, such as teratoma, which one would expect to see both solid and cystic components. Lymphangioma can also be a consideration, as it would appear multilocular or multicystic.
Morgagni hernia and eventration of the diaphragm could be additional considerations. Bronchial cysts are also possible, but one would expect to see bronchial epithelium lining the cyst on histology. Localized pericardial effusion is an additional differential in which one would expect to see fluid between the pericardium layers. Additionally, many different types of congenital cysts can be considered, such as esophageal duplication cysts or neurenteric cysts, but their typical location can sometimes differentiate these.[7][10][7]
Prognosis
The general prognosis is excellent for pericardial cysts, which is not unexpected considering that most patients are asymptomatic. Spontaneous resolution of lesions has even been reported.[11] Though exceedingly rare, life-threatening conditions can occur. In these cases, the prognosis varies and depends upon the tailored management of symptoms.
Complications
Complications from pericardial cysts are often due to compression of adjacent structures, including but not limited to the lung, esophagus, and heart. As such, symptoms are often vague and nonspecific. Rarely, complications can manifest as cyst infection, compression of the superior vena cava, and hemorrhage into the pericardial space, causing tamponade and death.[1]
Deterrence and Patient Education
To alleviate the patient's anxiety about the diagnosis, it is essential to educate them that most pericardial cysts are incidental findings with no long-term sequelae. Patients should also understand that these cysts rarely cause complications. Scheduled follow-up at certain time intervals can assess for impending complications or cyst growth.
Enhancing Healthcare Team Outcomes
The primary care physician can coordinate with the radiologist and the cardiologist to formulate a tailored management plan centered on the patient and their presentation. Clear communication of this tailored management plan to all involved, especially the patient, will enhance patient-centered care, participation, and expectations.
References
- 1.
Parmar YJ, Shah AB, Poon M, Kronzon I. Congenital Abnormalities of the Pericardium.
Cardiol Clin. 2017 Nov;35(4):601-614. [
PubMed: 29025550]
- 2.
Patel J, Park C, Michaels J, Rosen S, Kort S. Pericardial cyst: case reports and a literature review.
Echocardiography. 2004 Apr;21(3):269-72. [
PubMed: 15053790]
- 3.
King JF, Crosby I, Pugh D, Reed W. Rupture of pericardial cyst.
Chest. 1971 Dec;60(6):611-2. [
PubMed: 5126196]
- 4.
Pugliatti P, Donato R, Crea P, Zito C, Patanè S. Image Diagnosis: Pericardial Cyst in a Dialysis Patient.
J Cardiovasc Ultrasound. 2016 Jun;24(2):177-8. [
PMC free article: PMC4925400] [
PubMed: 27358715]
- 5.
Davis RD, Oldham HN, Sabiston DC. Primary cysts and neoplasms of the mediastinum: recent changes in clinical presentation, methods of diagnosis, management, and results.
Ann Thorac Surg. 1987 Sep;44(3):229-37. [
PubMed: 2820323]
- 6.
Lennon Collins K, Zakharious F, Mandal AKJ, Missouris CG. Pericardial Cyst: Never Too Late to Diagnose.
J Clin Med. 2018 Oct 30;7(11) [
PMC free article: PMC6262575] [
PubMed: 30380699]
- 7.
Kar SK, Ganguly T. Current concepts of diagnosis and management of pericardial cysts.
Indian Heart J. 2017 May-Jun;69(3):364-370. [
PMC free article: PMC5485391] [
PubMed: 28648435]
- 8.
Comoglio C, Sansone F, Delsedime L, Campanella A, Ceresa F, Rinaldi M. Mesothelial cyst of the pericardium, absent on earlier computed tomography.
Tex Heart Inst J. 2010;37(3):354-7. [
PMC free article: PMC2879185] [
PubMed: 20548822]
- 9.
Masood AM, Ali OM, Sequeira R. A hiding in the lining: painful pericardial cyst.
BMJ Case Rep. 2013 Apr 22;2013 [
PMC free article: PMC3645330] [
PubMed: 23608846]
- 10.
Stoller JK, Shaw C, Matthay RA. Enlarging, atypically located pericardial cyst. Recent experience and literature review.
Chest. 1986 Mar;89(3):402-6. [
PubMed: 3948553]
- 11.
Moffa AP, Stoppino LP, Loizzi D, Milillo P. Spontaneous Disappearance of a Pericardial Cyst: Case Report and Literature Review.
Korean J Thorac Cardiovasc Surg. 2018 Feb;51(1):72-75. [
PMC free article: PMC5796623] [
PubMed: 29430434]
Disclosure: Amanda Meredith declares no relevant financial relationships with ineligible companies.
Disclosure: Ibrahim Zazai declares no relevant financial relationships with ineligible companies.
Disclosure: Chris Kyriakopoulos declares no relevant financial relationships with ineligible companies.
