Case presentation
A 10-month-old male infant was admitted to our hospital due to progressive compression of the right heart chambers secondary to a pericardial cyst initially detected during routine fetal echocardiography. The patient had no other significant medical history apart from this prenatally diagnosed cardiac lesion. Fetal ultrasound performed at 27 weeks of gestation revealed an anechoic area measuring 1.62 × 0.77 cm located lateral to the right heart (Figure 1(a)). Following birth, serial transthoracic echocardiography demonstrated progressive enlargement of the cystic mass. At 10 months of age, comprehensive cardiac imaging including transthoracic echocardiography, CT, and MRI revealed a large multiloculated cystic mass within the pericardium measuring approximately 5.3 × 3.5 × 3.9 cm with internal septations, causing significant compression of the right atrium and right ventricle (Figure 1(b), 1(c), 1(d)). Transthoracic echocardiography also demonstrated pulmonary valve stenosis and supravalvular stenosis with a significant pressure gradient.
Preoperative imaging demonstrating progressive enlargement of pericardial cyst in a 10-month-old infant. ( a ) Fetal ultrasound at 27 weeks of gestation showing an anechoic area (1.62 × 0.77 cm) located lateral to the right heart. ( b ) Transthoracic echocardiography at 10 months of age demonstrating a large multiloculated cystic mass causing significant compression of the right ventricle. ( c ) Cardiac MRI showing the cystic mass compressing the right ventricle. ( d ) Cardiac CT revealing a large cystic mass (5.3 × 3.5 × 3.9 cm), causing compression of the right atrium and right ventricle. LV = left ventricle; RV = right ventricle.

Given the progressive symptoms and significant compression of cardiac structures, surgical intervention was planned. A median sternotomy approach was selected, considering the complexity of the case and the need for concurrent repair of pulmonary valve stenosis. Intraoperative findings revealed a large cystic mass located on the epicardial surface between the right atrium and right ventricle. The mass contained multiple internal cystic compartments filled with clear yellowish fluid (Figure 2(a)). Careful dissection revealed that the right coronary artery was intimately related to the cystic wall, running along the surface of one of the cystic compartments (Figure 2(b)). Due to the intimate relationship between the right coronary artery and the cystic wall, complete excision of all cystic components was not feasible without risking coronary artery injury. Therefore, the surgical strategy was modified: the main cystic mass was excised, and the cystic cavity was obliterated, while the portion of the cyst adjacent to the right coronary artery was intentionally left unresected to preserve coronary artery integrity. Concurrent pulmonary valve commissurotomy and pulmonary artery patch augmentation were performed to address the associated pulmonary stenosis (Figure 2(c)).
Intraoperative findings and postoperative echocardiography. ( a ) Intraoperative view showing a giant cystic mass on the surface of the right atrium and right ventricle, causing severe compression and displacement of the right heart chambers. The massive cyst obscures direct visualisation of the right atrium and right ventricle. ( b ) After partial cyst excision, intraoperative dissection revealing the right coronary artery (RCA, red arrow) coursing along the wall of the residual cyst, demonstrating intimate relationship between the coronary artery and the cyst. ( c ) Postoperative view after subtotal cyst excision with cavity obliteration, concurrent pulmonary valve commissurotomy, and pulmonary artery patch augmentation. ( d ) Postoperative transesophageal echocardiography demonstrating a small residual cyst (red arrow) with significantly improved right heart chamber dimensions. AV = aortic valve; LA = left atrium; RA = right atrium; RAA = right atrial appendage; RV = right ventricle.

Postoperative transesophageal echocardiography demonstrated a small residual cyst with significantly improved right heart chamber dimensions and no significant pulmonary valve gradient (Figure 2(d)). Histopathological examination of the excised specimen revealed fibrous cystic wall tissue with inflammatory cell infiltration, lined by a single layer of flattened epithelial cells, consistent with a mesothelial-lined pericardial cyst (Figure 3). Immunohistochemical staining was positive for Calretinin (focal), WT-1 (focal), and MC (++), confirming the mesothelial origin of the cyst lining. The patient recovered uneventfully and was discharged.
Histopathological examination of the excised pericardial cyst. Histopathological examination (H&E staining) showing fibrous cystic wall tissue with inflammatory cell infiltration, lined by a single layer of flattened mesothelial cells, consistent with a mesothelial-lined pericardial cyst.

At 3-month follow-up, transthoracic echocardiography revealed a well-defined anechoic area measuring 1.77 cm × 0.467 cm within the right pericardial space, with regular morphology and clear borders (Figure 4(a)). Importantly, the residual cyst showed no interval enlargement compared to the immediate postoperative imaging. Colour Doppler echocardiography demonstrated patent pulmonary valve and main pulmonary artery with no stenosis and normal antegrade pulmonary flow (Figure 4(b)). The patient remained asymptomatic and continues under close surveillance with planned serial echocardiographic follow-up.
Three-month postoperative follow-up echocardiography. ( a ) Transthoracic echocardiography demonstrating a well-defined residual anechoic area measuring 1.77 cm × 0.467 cm within the right pericardial space with regular morphology and clear borders, showing no interval enlargement compared to immediate postoperative imaging. ( b ) Colour Doppler echocardiography showing patent pulmonary valve and main pulmonary artery with no stenosis and normal antegrade pulmonary flow.

Discussion
Pericardial cysts are uncommon congenital cardiac anomalies that arise from developmental abnormalities during embryogenesis. Such cysts are thought to originate embryologically from disconnected mesenchymal lacunae, which normally unite to form the pericardial coelom. The majority of cases (50–75%) are asymptomatic and are discovered incidentally during routine radiological investigations performed for other clinical indications. Reference Qamar, Gulzar, Qamar, Sabry and Minhas1 Most pericardial cysts are located at the right (70%) or left (22%) cardiophrenic angle, with the remainder found in the anterior or posterior mediastinum. Reference Kar and Ganguly2
Epicardial cysts are much rarer than pericardial cysts, and their surgical management has seldom been reported. Reference Gupta, Shah, Naik, Chandan, Sheth and Shah3,Reference Wang, Zhang, Zi and Xu4 While CT and MRI are useful for characterising cystic lesions and assessing their relationship to surrounding structures, the definitive distinction is often made intraoperatively. In our case, the cyst was found to be located on the epicardial surface with intimate coronary artery involvement, similar to the epicardial cysts described by Wang et al. Reference Wang, Zhang, Zi and Xu4
The surgical management of pericardial and epicardial cysts varies depending on their size, location, and relationship to vital cardiac structures. Reported cases have been managed through complete surgical excision via median sternotomy or video-assisted thoracoscopic surgery. Reference Wang, Zhang, Zi and Xu4,Reference Kim, Kim, Choi and Jeong5 However, when cysts are intimately associated with coronary arteries or cardiac chambers, complete excision may not be feasible without risking significant complications. In the case reported by Gupta et al., an epicardial cyst was found to be eroding into the left ventricular wall, necessitating cardiopulmonary bypass for safe resection and reconstruction of the attenuated ventricular wall. Reference Gupta, Shah, Naik, Chandan, Sheth and Shah3 Gupta et al. emphasised that cardiopulmonary bypass may be required when cysts compress surrounding structures or erode the ventricular wall and coronaries, and suggested that the surgeon must be aware of the possible need for cardiopulmonary bypass in such situations. Similarly, Wang et al. reported an epicardial cyst originating from the roof of the left atrium, which was successfully resected using an off-pump technique after careful assessment of coronary artery involvement. Reference Wang, Zhang, Zi and Xu4 They emphasised that coronary CT angiography or coronary angiography should be considered based on the degree of adhesion and location of coronary vessels, and that when invasion of important structures, including the left ventricle and coronary artery, is suspected, median sternotomy with cardiopulmonary bypass should be considered.
These cases highlight the importance of thorough preoperative evaluation, particularly with coronary angiography or coronary CT angiography, to assess the relationship between the cyst and coronary vessels. Reference Gupta, Shah, Naik, Chandan, Sheth and Shah3,Reference Wang, Zhang, Zi and Xu4 In our case, preoperative imaging suggested close proximity between the cyst and the right coronary artery, which was confirmed intraoperatively. The decision to leave a portion of the cyst unresected was made to preserve coronary artery integrity, as complete excision would have required extensive dissection along the right coronary artery with potential risk of arterial injury or compromise. This approach is supported by Wang et al., who noted that video-assisted thoracoscopic surgery/thoracotomy may be the first option, but when invasion of important structures is suspected, median sternotomy with cardiopulmonary bypass should be considered, and off-pump resection may be indicated for uncomplicated cysts. Reference Wang, Zhang, Zi and Xu4
Similar to the case reported by Masuoka et al. Reference Masuoka, Sakurai, Shiraishi, Yoshiba, Katogi and Suzuki6 our patient presented in early infancy with symptomatic compression of cardiac structures. However, unlike their case, which presented as acute cardiac tamponade after chest trauma, our patient demonstrated progressive symptoms due to gradual cyst enlargement. The presence of concurrent pulmonary valve stenosis in our patient added complexity to the surgical management. While some reports have described isolated pericardial cyst resection, our case required combined procedures including cyst excision, pulmonary valve commissurotomy, and pulmonary artery augmentation. The decision to proceed with median sternotomy rather than video-assisted thoracoscopic surgery was influenced by the need for concurrent cardiac procedures and the anticipated complexity of dissection near the right coronary artery.
Regarding the natural history and recurrence risk of pericardial cysts, a retrospective observational study following 103 patients with confirmed pericardial cysts found that cysts most often regressed in size or remained the same, and those that grew required only continued monitoring due to asymptomatic presentations. Reference Alkharabsheh, Ayache and Halabi7 Incomplete excision may have led to early recurrence, as documented in a recent case report where a pericardial cyst recurred within 5 months after surgical resection. Reference Bachu, Mcdonald, Mandak, Moritz, Mandak and Murthy8 After recurrence, given further imaging suggesting that complete excision of the cyst would potentially injure nearby structures, repeat surgery was not recommended in that case, and fortunately, the patient reported no symptoms. Similarly, in our case, the intentionally retained cyst remnant adjacent to the right coronary artery necessitates close long-term follow-up with serial echocardiography to monitor for potential recurrence while balancing the risk of surgical complications against the benefits of complete excision.
This case underscores several important clinical lessons. First, pericardial cysts detected prenatally should be monitored closely for progressive enlargement and compression of cardiac structures. Second, comprehensive preoperative evaluation, including coronary artery assessment, is essential for surgical planning, particularly when cysts are located on the epicardial surface. Third, when complete excision poses excessive risk to vital structures such as coronary arteries, subtotal resection with cavity obliteration represents a reasonable alternative strategy. Finally, a flexible surgical approach with cardiopulmonary bypass standby should be considered for complex cases involving coronary artery proximity or concurrent cardiac lesions requiring repair.
Conclusion
We report a rare case of a giant pericardial cyst with intimate coronary artery involvement in an infant, successfully managed with subtotal resection and cavity obliteration combined with repair of concurrent pulmonary stenosis. This case highlights the diagnostic and surgical challenges posed by pericardial cysts in atypical locations with involvement of vital cardiac structures. Comprehensive preoperative evaluation, particularly coronary artery assessment, and a flexible surgical approach are essential for optimal outcomes. When complete excision poses excessive risk, subtotal resection represents a safe and effective alternative strategy.
Data availability statement
No data were generated or analysed for or in support of this paper.
Acknowledgements
We thank our multidisciplinary team members for their contributions to patient care.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
The authors have no conflict of interest.
Ethical standard
The patient’s guardians provided informed consent for the publication of this case report.