Rosai-Dorfman Disease as Constrictive Pericarditis: An Unusual Childhood Presentation.

Rosai-Dorfman disease (RDD) is a rare, multisystemic, histiocytic disorder that usually presents with painless cervical lymphadenopathy. We describe a case of constrictive effusive pericarditis with congestive cardiac failure in a six-year-old child as an initial presentation of RDD. The child underwent pericardiectomy and was treated with steroids, following which the symptoms resolved entirely. While tuberculosis accounts for the majority of cases presenting with constrictive pericarditis in developing countries, the presentation of RDD can be easily missed if not kept in mind as a possibility. This case report documents the unique presentation of a rare disease.

Corrigendum: Case Report: Epstein-Barr virus and constrictive pericarditis-an unusual combination.

[This corrects the article DOI: 10.3389/fped.2023.1215928.].

Semiquantitative 18F-FDG PET/CT in monitoring glucocorticoid response of immunoglobulin G4-related effusive constrictive pericarditis: a case report.

BACKGROUND: Immunoglobulin G4 (IgG4)-related effusive constrictive pericarditis (ECP) is a rare manifestation of IgG4-related disease (IgG4-RD). It can lead to persistent pericardial fibrosis, resulting in cardiac tamponade, diastolic dysfunction, and heart failure. Glucocorticoids are the primary treatment for effectively reducing inflammation and preventing fibrosis. However, guidelines for monitoring treatment response are lacking and tapering glucocorticoid therapy for specific target organs remains a challenge. Recent studies on IgG4-RD have demonstrated that semiquantitative measurements of fluorine-18 fluorodeoxyglucose (18F-FDG) uptake in the main involved organs in positron emission tomography/computed tomography (PET/CT) scanning are correlated to disease activity. We present a case of IgG4-related ECP to demonstrate the usefulness of 18F-FDG PET/CT for diagnosing and treatment follow-up of IgG4-related ECP. CASE PRESENTATION: Herein, a 66-year-old woman diagnosed with IgG4-related ECP presented with breathlessness, leg swelling, rales, and fever. Laboratory tests revealed markedly elevated levels of C-reactive protein, and transthoracic echocardiography revealed constrictive physiology with effusion. High IgG4 levels suggested an immune-related pathogenesis, while viral and malignant causes were excluded. Subsequent pericardial biopsy revealed lymphocyte and plasma cell infiltration in the pericardium, confirming the diagnosis of IgG4-related ECP. 18F-FDG PET/CT revealed increased uptake of 18F-FDG in the pericardium, indicating isolated cardiac involvement of IgG4-RD. Treatment with prednisolone and colchicine led to a rapid improvement in the patient's condition within a few weeks. Follow-up imaging with 18F-FDG PET/CT after 3 months revealed reduced inflammation and improved constrictive physiology on echocardiography, leading to successful tapering of the prednisolone dose and discontinuation of colchicine. CONCLUSION: The rarity of IgG4-related ECP and possibility of multiorgan involvement in IgG4-RD necessitates a comprehensive diagnostic approach and personalized management. This case report highlights the usefulness of 18F-FDG PET/CT in the diagnosis and treatment follow-up of isolated pericardial involvement in IgG4-RD.

Case report: Epstein-Barr virus and constrictive pericarditis-An unusual combination.

Constrictive pericarditis is a chronic inflammatory process that can lead to heart failure if not diagnosed and treated correctly. Although Epstein-Barr virus (EBV)-related pericarditis is a very rare condition, it should still be considered for a differential diagnosis. We report the case of an 18-year-old male, who was surgically treated for constrictive pericarditis, in which in situ hybridization to Epstein-Barr virus-encoded RNA (EBER) probe of the excised pericardium led to the subsequent etiological diagnosis of chronic pericarditis caused by EBV.

Pericardial effusions: perspective of the acute cardiac care physician.

Pericardial effusions can result in acute haemodynamic compromise and require rapid intervention. Understanding pericardial restraint is essential to determine the approach to newly identified pericardial effusions in the intensive care unit. As pericardial effusions stretch the pericardium, the pericardial compliance reserve is eventually exhausted, with an exponential rise in compressive pericardial pressure. The severity of pericardial pressure increase depends on both the rapidity and volume of pericardial fluid accumulation. This increase in pericardial pressure is reflected in an increase in measured left- and right-sided 'filling' pressures, but paradoxically left ventricular end-diastolic volume (the true left ventricular preload) is decreased. This uncoupling of filling pressures and preload is the hallmark of pericardial restraint. When this occurs acutely from a pericardial effusion, rapid recognition and pericardiocentesis can be lifesaving. In this review, we will discuss the haemodynamics and pathophysiology of acute pericardial effusions, provide a physiological guide to determine the need for pericardiocentesis in acute care, and discuss important caveats to management.

Pseudomonas aeruginosa-related effusive-constrictive pericarditis diagnosed with echocardiography: A case report.

BACKGROUND: Effusive-constrictive pericarditis (ECP) is an uncommon pericardial syndrome. Careful echocardiographic examination may provide helpful information not only for diagnosing but also for managing ECP. ECP has various etiologies; however, Pseudomonas aeruginosa (P. aeruginosa) infection has not been reported as a cause to date. Herein, we present a rare case of ECP caused by P. aeruginosa infection, which was followed up using echocardiography. CASE SUMMARY: A 30-year-old man was admitted to our hospital with a 2-mo history of cough, dyspnea, bloating, palpitations, and lower-extremity edema. The patient was initially diagnosed with pericardial effusion by transthoracic echocardiography. Drainage of pericardial effusion was performed to relieve the clinical symptoms. A follow-up echocardiogram showed that the pericardial effusion had decreased; however, the right atrial pressure continued to increase, and signs of constrictive pericarditis were observed upon a more comprehensive inspection. Therefore, the diagnosis of ECP was established based on the comprehensive pre- and post-pericardiocentesis echocardiographic findings. An urgent pericardectomy was subsequently performed, which significantly relieved the patient's clinical symptoms, and the signs of pericardial constriction on echocardiography improved. Pericardial effusion and pericardial culture showed growth of P. aeruginosa. CONCLUSION: ECP induced by P. aeruginosa infection remains a rare disease. The presence of echocardiographic features of constrictive pericarditis after pericardiocentesis therapy is highly indicative of ECP.

Effusive-constrictive pericarditis after the second dose of BNT162b2 vaccine (Comirnaty): a case report.

BACKGROUND: Whereas effusive-constrictive pericarditis (ECP) can rarely occur in coronavirus disease 2019 (COVID-19), to date no cases of ECP related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) vaccine have been documented. CASE SUMMARY: A 59-year-old Caucasian man presented to our emergency department with ECP. Symptoms occurred shortly after the second dose of BNT162b2 (Comirnaty) vaccine. No other aetiological causes were identified. Guidelines-directed therapy for acute pericarditis was implemented, with clinical benefit. DISCUSSION: Systemic inflammatory response to COVID-19 can rarely trigger pericarditis. In our case, a strong temporal relationship between the second dose of BNT162b2 vaccine and symptoms occurrence was documented, indicating a possible rare adverse reaction to the vaccine, similarly to natural infection. Further research is needed to confirm a causal relationship.

Rituximab therapy in pericarditis associated with rheumatoid arthritis.

In rheumatoid arthritis, pericarditis is commonly asymptomatic, but rarely, it progresses to a morbid complication, like cardiac tamponade or restrictive pericarditis. Current studies have indicated that conventional drugs have limited ability to reverse these lethal conditions. To date, invasive surgical measures remain the only definitive therapy for patients who are unresponsive to drugs. Recently, anti-tumor necrosis factor-α and anti-interleukin-1 antibody-based drugs have shown limited success. Consequently, given the importance of pericarditis, we need new treatment methods. Here, we describe a patient with rheumatoid arthritis and effusive pericarditis, which progressed to life-threatening cardiac tamponade. The patient responded very well to rituximab. Thus, rituximab represents a potential new therapy for this rarely treated complication of rheumatoid arthritis.

A rare cause of effusive-constrictive pericarditis.

Effusive-constrictive pericarditis (ECP) is an uncommon diagnosis, frequently missed due to its heterogeneous presentation, but a potentially reversible cause of heart failure. A 62-year-old Caucasian male presented with remittent right heart failure and mild-moderate pericardial effusion. Following an initial diagnosis of idiopathic pericarditis, indomethacin was started, but the patient shortly relapsed, presenting with severe pericardial effusion and signs of cardiac tamponade, requiring pericardiocentesis. ECP was diagnosed on cardiac catheterization. Cardiac computed tomography showed non-calcified, mildly thickened and inflamed parietal pericardium. Pericardiectomy was performed with symptoms remission. On histological examination of pericardium, chronic non-necrotizing granulomatous inflammation was noted. Polymerase chain reaction assay was positive for non-tuberculous mycobacteria. This case represents a rare finding of ECP with unusual presentation due to atypical mycobacteriosis in a non-immunocompromised patient and in a non-endemic area. Pericardiectomy can be an effective option in cases unresponsive to anti-inflammatory treatment, even in the absence of significant pericardial thickening or calcification.

Idiopathic Effusive-Constrictive Pericarditis Presented by Variant Angina.

An 80-year-old man presented to our hospital complaining of loss of appetite. During the medical examination, he developed variant angina accompanied with heart failure. Oral calcium channel blocker therapy controlled his variant angina, but medical management of heart failure became increasingly difficult due to gradually increasing pericardial effusion, and pericardiocentesis leading to the diagnosis of effusive-constrictive pericarditis (ECP). Here, we report a rare case of idiopathic pericarditis caused variant angina with already having endothelial dysfunction and eventually developed ECP.

Effusive-constrictive cholesterol pericarditis: a case report.

BACKGROUND: Cholesterol pericarditis (CP) remains a rare pericardial disease characterized by chronic pericardial effusions with high cholesterol concentrations with or without the formation of cholesterol crystals. Effusions are often large and can cause ventricular compression and subsequent pericardial adhesion formation. CP can be idiopathic but has associations with rheumatoid arthritis (RA), tuberculosis and hypothyroidism. CASE SUMMARY: We present a case of a 72-year-old male with a background of seropositive RA with a finding of an incidental pericardial effusion on computed tomography thorax abdomen and pelvis. Transthoracic echocardiogram demonstrated a large effusion with echocardiographic features of tamponade. On review, he was breathless with a raised venous pressure, bilateral ankle oedema, and pulsus paradoxus was present. Pericardial drainage was performed with fluid analysis demonstrating a cholesterol concentration of 8.3 mmol/L and numerous cholesterol crystal formation. Interval imaging demonstrated recurrence of the effusion with pericardial thickening and progressive constriction. He remained asymptomatic and underwent a successful pericardial window. At present, he is under close clinical outpatient surveillance with symptoms guiding a future pericardiectomy if warranted. DISCUSSION: CP can present as an emergent situation with signs and symptoms of acute heart failure with prompt pericardiocentesis required in cases of clinical tamponade. However, the disease course is often one of chronicity with relapsing large effusions that tend to recur following drainage, with the development of pericardial constriction necessitating pericardiectomy for definitive management.

Effusive-constrictive pericarditis secondary to pneumopericardium associated with gastropericardial fistula.

A 66-year-old man with a history of gastric pull-up reconstruction for oesophageal cancer was hospitalized because of prolonged chest pain. Chest X-ray demonstrated pneumopericardium. Computed tomography revealed ulceration and abscess in the gastric conduit adjacent to the heart, suggesting gastropericardial fistula. As the patient did not show tamponade physiology, he was conservatively treated with antibiotics. The pneumopericardium diminished; however, he developed effusive-constrictive pericarditis with overt heart failure symptoms. Because pericardiocentesis failed to relieve the symptoms, pericardiectomy was performed. Intraoperative exploration revealed remarkably thickened pericardium and epicardium constituting multiple layers with purulent effusion. Epicardiectomy as well as pericardiectomy were required to achieve the effective reduction of central venous pressure. Perforation of the gastric conduit into the pericardial cavity was identified and repaired. Histopathology demonstrated thickened pericardium composed of hyalinized stroma, collagenous bundles, and infiltration of inflammatory cells. Streptococcus anginosus and Candida tropicalis were identified by culture of the resected tissue.

Postcardiotomy effusive-constrictive pericarditis in a 65-year-old man: Noteworthy echocardiographic signs.

Effusive-constrictive pericarditis (eCP) is a rare entity where constriction of the heart occurs through the visceral pericardium, with concomitant presence of a tense effusion in the pericardial space. We present the case of a 65-year-old patient with shortness of breath and pericardial effusion, 4 months after aortic and mitral valve replacement for Streptococcus agalactiae endocarditis. The present report focuses on the echocardiographic features of constrictive pericarditis (CP) and provides a brief overview of the pathophysiology, work-up, and management guidelines.