A haemorrhagic pericardial cyst compressing the right side of the heart: a case report

Abstract

Background

Pericardial cysts are rare and represent the third most common cystic mass of the mediastinum. The majority are asymptomatic and detected as incidental findings; however, they can be symptomatic and associated with life-threatening complications such as bronchial compression, congestive heart failure, cardiac tamponade, or even sudden death.

Case summary

We present a rare case of a haemorrhagic pericardial cyst with subtotal compression of the right side of the heart. A symptomatic male patient was referred due to progressive dyspnoea, signs of congestive heart failure for four months, and a transthoracic echocardiogram showing subtotal compression of the right heart side; the diagnosis was confirmed with thoracic computer tomography imaging and was removed surgically.

Discussion

Pericardial cysts are asymptomatic and benign in the majority of cases; however, they can be associated with life-threatening complications. Thus, regular follow-up is recommended, and in a minority of cases, minimal invasive intervention or surgery could be imperative.

Graphical Abstract

Graphical abstract.

Learning points.

• Pericardial cysts are asymptomatic and benign in the majority of cases, and close follow-up is usually sufficient. However, in a minority of cases, they can be associated with life-threatening complications, which could necessitate intervention.

• This case highlights the importance of understanding the anatomical location, the size of the cyst, the haemodynamic state, and the rarity of infection-induced haemorrhagic pericardial cysts in relation to the onset of symptoms.

• Of note, haemodynamic instability in case of congestive heart failure or cardiac tamponade includes clinical evidence of low cardiac output and stroke volume in the setting of elevated cardiac filling pressures (e.g. hypotension or even shock, dizziness, and oliguria), with evidence of increased sympathetic tone (e.g. tachycardia and peripheral vasoconstriction), and exclusion of other causes of shock.

Introduction

Pericardial cysts are rare and represent the third most common cystic mass of the mediastinum. Most of the cases are diagnosed as an incidental finding in chest X-rays. They can be congenital or acquired.

The estimated incidence of congenital pericardial cysts is 1:100,000, and they account for up to 7% of the mediastinal masses reported in the literature. They are formed by the incomplete coalescence of foetal lacunae during pericardial development.¹

They are rarely found in children, are discovered more commonly in the third or fourth decade of gender life. However, ∼30% of patients develop chest pain, shortness of breath, and or paroxysmal tachypnoea.¹

On the other hand, an acquired cyst can occur as a post-inflammatory process, infection, or even post-trauma.

Most cysts are asymptomatic. Symptoms occur if the cyst compresses on a nearby structure, such as the right side of the heart, causing congestive heart failure, or obstructs the right main stem bronchus; ingress of the cyst into the superior vena cava can occur, and recurrent syncope, atrial fibrillation, and even sudden cardiac death have been reported.² In addition, inflammation of the cyst can occur with pericarditis and pneumonia or can rupture into the pleural sac, mediastinal cavity, or in the pericardial sac causing cardiac tamponade.

Summary figure

Date/time	Case events
September 2022	Dyspnoea NYHA III.
11 December 2022/11:30 a.m.	Echocardiography showed a large intrapericardial mass compressing the right side of the heart.
11 December 2022/1 p.m.	Thoracic CT revealed that the intrapericardial cyst measured 89 × 58 mm.
12 December 2022/9:20 a.m.	Pericardiolysis with resection of the pericardial cyst.
18 December 2023	Discharge without symptoms. Follow-up in 90 days without quality-of-life limitation.

Case presentation

A 73-year-old man with known diabetes mellitus type II presented with a four-month history of progressive dyspnoea as well as leg and facial oedema without symptoms of cough, sputum, fever, or night sweats. Due to the non-improvement of symptoms on diuretic medication, his family doctor referred him to a cardiologist. A physical examination revealed markedly congestive neck veins. The transthoracic echocardiogram (TTE)) revealed subtotal compression of the right side of the heart from outside the heart border (see Supplementary material online, Video S1). The cardiologist referred the patient urgently to our clinic for further assessment. The routine blood test demonstrated markedly elevated creatinine (3,5 mg/dL, glomerular filtration rate 18 mL/min, 48 mg/dL), NT-Pro BNP 2543 pg/mL, and D Dimer 1393 µg/L. For further clarification, we conducted a thoracic contrast computer tomography (CT). It showed a large intrapericardial thick-walled mass with fluid accumulation in the cystic cavity and homogeneous enhancement of the cyst wall without visible air trapping. It measured 89 × 58 mm, and the maximal pericardial thickness was 2 mm (Figures 1 and 2). Consecutively, there was compression of the right side of the heart (Figure 3). Mediastinally, there were multiple supracarinal pretracheal lymph nodes enlarged, measuring 19.7 × 12.3 mm.

Figure 1.

Computer tomography coronal view revealed a large intrapericardial thick-walled mass with fluid accumulation in the cystic cavity and homogeneous enhancement of the cyst wall without visible air trapping. It measured 89 × 58 mm, and the maximal pericardial thickness was 2 mm. LA, left atrium; LV, left ventricle.

Figure 2.

Computer tomography sagittal view revealed a large intrapericardial thick-walled mass with fluid accumulation in the cystic cavity and homogeneous enhancement of the cyst wall without visible air trapping. It measured 89 × 58 mm, and the maximal pericardial thickness was 2 mm. LV, left ventricle.

Figure 3.

Computer tomography horizontal view revealed a large intrapericardial thick-walled mass with fluid accumulation in the cystic cavity. Consecutively, there was compression of the right ventricle as well as the right atrium. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

The imaging diagnosis was a large pericardial cyst with signs of concomitant infection (presence of discrete fluid collections usually related to increased vasodilation and vascular permeability, resulting in tissue oedema and stranding of normally fatty signal. However, these signs are non-specific).

We referred the patient to a tertiary heart centre. One day later, the patient underwent pericardiolysis with resection of the pericardial cyst (Figure 4) via an anterolateral right side mini thoracotomy incision and received symptomatic treatment, including inhalation, breathing exercises, and antibiotic therapies. The subsequent post-operative histopathological and microscopic findings revealed a cyst containing haemorrhagic fluid with low cellular collagenous connective tissue with chronic inflammation (lymphoplasmacytic infiltration, many plasma cells, minor mixed eosinophils and granulocytes) without evidence of malignant cells.

Figure 4.

Intraoperative view of open-heart surgery via an anterolateral right side mini thoracotomy incision revealed a huge pericardial cyst.

The patient recovered post-surgery with no deficits, the TTE showed no compression of right heart side, and the patient was discharged 7 days later. The discharge medications were painkillers as necessary. Three months after the procedure, the follow-up did not show quality-of-life limitations.

Discussion

We present a rare case of a haemorrhagic pericardial cyst with compression of the right side of the heart causing congestive heart failure, which was removed surgically.

In general, pericardial cysts represent the most common benign tumour of the pericardium. However, the presence of a pericardial cyst in a typical location or, less frequently, in an unusual location, still poses a diagnostic challenge in distinguishing it from other intracardiac or mediastinal benign or malignant lesions. Further image studies are required to complete the study of these lesions.

TTE characteristics include a homogeneous echolucent mass with minor attenuation of the ultrasound through a low-density fluid-filled structure.

It is easy to perform, especially in unstable patients, provides information about the cardiac functional state, follow-up, and image-guided percutaneous aspiration.

In a short acquisition time, thoracic CT provides clear and sharp images with excellent delineation of the pericardial anatomy and can aid in the precise localization and characterization of various pericardial lesions, which is the key for optimal management.

The cyst’s characteristics are a single thin-walled, sharply defined round, homogenous, and radiodense lesion without septation or solid component seen at the cardiovascular fluid-filled sac and not enhanced with contrast. Two-thirds occur on the right side, followed by the left costophrenic angle (10–40%) or unusual localizations such as the posterior mediastinum.³

Magnetic resonance imaging provides an excellent soft-tissue architecture demonstration. It shows a low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. However, an altered signal occurs when the cyst protein is high, and the calcification is not as well visualized as in a CT.²

The differentiation of pericardial cysts from diverticula occurs with the presence of a communicating tract between the pericardium and the cyst cavity, which is not usually recognized in any of the imaging modalities.²

Differential diagnoses can sometimes be confused with a coronary artery aneurysm, dextrocardia, pneumonia, pleural effusion, granulomatous lesions or abscess, benign or malignant lung, mediastinal, or pericardial tumours, phrenic nerve palsy, bronchogenic cyst, diaphragmatic hernia, ventricular aneurysm, and valsalva sinus.⁴

In most patients, close follow-up is enough and is performed by serial transthoracic echocardiography. Some pericardial cysts resolve spontaneously, most likely from rupture into the pleural space.

Decision-making in the management of a pericardial cyst should be based on the presence or the absence of symptoms, the size of the mass, the haemodynamic state, an unclear diagnosis or aggressive behaviour, or the compression of important structures. In such scenarios, treatment could be necessary to confirm the diagnosis and to prevent life-threatening emergencies, such as cardiac tamponade.¹

Of note, haemodynamic instability in case of congestive heart failure or cardiac tamponade includes clinical evidence of low cardiac output in the setting of elevated cardiac filling pressures (e.g. hypotension, dizziness, and oliguria), with evidence of increased sympathetic tone (e.g. tachycardia and peripheral vasoconstriction), and exclusion of other causes of shock.

Treatment includes conservative management with follow-up, percutaneous aspiration of the cyst, and excision of the cyst. The task force on the diagnosis and management of pericardial diseases of the European Society of Cardiology recommended percutaneous aspiration and ethanol sclerosis as initial treatment for congenital and inflammatory cysts.⁴

Video-assisted thoracotomy or surgical resection in the case of complications, the potential for malignant transformation, haemodynamic compromise, or even to prevent life-threatening emergencies could be mandatory in a minority of cases.

This case highlights the importance of understanding the anatomical location, the size of the cyst, the haemodynamic state, and the rarity of infection-induced haemorrhagic pericardial cysts in relation to the onset of symptoms.

Conclusion

Pericardial cysts are asymptomatic and benign in the majority of cases, and close follow-up is usually sufficient. However, in a minority of cases, they can be associated with life-threatening complications, which could necessitate intervention.

Lead author biography

Abdelrahman Elhakim is an interventional cardiologist and head of catheter laboratory at Schoen hospital Neustadt in Holstein in Germany. He completed his cardiovascular medicine residency and interventional cardiology fellowship in many hospitals in Germany. He is also subspecialized in coronary artery intervention, intensive care, and emergency medicine in Germany. He is a lead of pulmonary embolism workshops and did a medical doctoral study on pulmonary embolism at Schleswig-Holstein University Hospital in Luebeck. He also holds master in cardiovascular medicine at Al-Azhar University, Egypt and another master’s in business and health administration at Nuernberg University in Germany.

Supplementary material

Supplementary material is available at European Heart Journal – Case Reports online.

Consent: Written informed consent was obtained from the patient in line with COPE for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Funding: This case report was not supported by any funding.

Data availability

All data related to the case are available on request. The paper is not under consideration elsewhere. None of the paper’s contents have been previously published. All authors have read and approved the manuscript.