Continuing Education Activity

A pericardial cyst is typically considered a congenital anomaly, with most patients diagnosed via incidental findings on routine chest imaging. Rarely, they can 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:

• Summarize the most common presentation of a patient with a pericardial cyst.
• Outline the etiology of a pericardial cyst.
• Describe the typical imaging findings associated with pericardial cysts.
• Review the importance of communication amongst interprofessional team members to enhance the delivery of care for patients with pericardial cysts.

Introduction

A pericardial cyst is typically considered a congenital anomaly, with most patients diagnosed via incidental findings on routine chest imaging. Rarely, they can 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 either 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 5.4cm.[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 an incidental finding 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] In the remainder of patients, symptoms may occur via compression and mass effect 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, and/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 an incidental finding on chest radiographs, which can demonstrate a round, circumscribed mass adjacent to the heart, statistically most common at the right cardiophrenic 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 non-enhancing, thin-walled, ovoid homogenous mass without solid component. A disadvantage occurs if protein content increases (such as with hemorrhage), leading to errors in reporting.[7] 

Some authors suggest that echocardiography is a superior modality for delineating pericardial borders and characterization of 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, i.e., in the case of obesity. 

Cardiac MRI is recommended to further 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 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 in size, continued surveillance and conservative management generally 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]

Recurrence rates of pericardial cyst after aspiration is about 33%. There are no available data regarding the adhesion formation or recurrence after alcohol sclerosis. Otherwise, surgical options include surgical resection of the cyst via thoracotomy, sternotomy, VATS, or mediastinoscopy.[7] The recommendation for the treatment of pericardial cysts has its basis in observational data. As such, tailored management is necessary. 

Differential Diagnosis

On plain chest radiographs, pericardial cysts may simulate pericardial fat or ventricular aneurysm. 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, which 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, a plethora of different types of congenital cysts can be considered, such as esophageal duplication cyst or neurenteric cysts, but their typical location can sometimes differentiate these.[10][7]

Prognosis

The general prognosis is excellent for pericardial cysts, not unexpected when one considers that the vast majority of 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 is dependent upon the tailored management of symptoms.

Complications

Complications from pericardial cysts are often due to compression of adjacent structures, which include but are 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

It is essential to educate the patient that most pericardial cysts are incidental findings, with no long-term concerning sequelae, to alleviate their anxiety about the diagnosis. Patients should also understand that these cysts rarely cause complications. Scheduled follow-up at certain time intervals can assess for impending complications or growth of the cyst.

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 patience-centered care and patient participation and expectations.