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.

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.

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.

Effusive-Constrictive Pericarditis: Doppler Findings.

PURPOSE OF REVIEW: To review the echo-Doppler findings in effusive-constrictive pericarditis (ECP). ECP corresponds to the coexistence of a hemodynamically significant pericardial effusion and markedly reduced compliance of the pericardium, manifested by constrictive physiology post-pericardiocentesis. RECENT FINDINGS: We summarize herein the recent observations regarding the prevalence of ECP based on echocardiography as well as the pre- and post-pericardiocentesis echo-Doppler features of ECP. ECP diagnosed by echocardiography was seen in approximately 15% of patients with ECP pre- and post-pericardiocentesis echo-Doppler findings sharing features with both cardiac tamponade and constrictive pericarditis. ECP post-pericardiocentesis is common but its natural history in the current era might be better than previously reported. Further studies and (particularly simultaneous echocardiography-cardiac catheterization) are still critically needed to better understand the underlying hemodynamics of ECP. Moreover, it remains to be determined whether pre- and post-pericardiocentesis echo-Doppler findings can be used to prognosticate or to guide therapy of those undergoing pericardiocentesis.

Transitory effusive-constrictive pericarditis.

Pericardial effusion of various sizes is a quite common clinical finding, while its progression to effusive-constrictive pericarditis occurs in about 1.4-14% of cases. Although available evidence on prevalence and prognosis of this rare pericardial syndrome is poor, apparently a considerable proportion of patients conservatively managed has a spontaneous resolution after several weeks. A 61-year-old female presented to our emergency department reporting fatigue, effort dyspnea and abdominal swelling. The echocardiography showed large pericardial effusion with initial hemodynamic impact, so she underwent a pericardiocentesis with drainage of 800-850cm3 of exudative fluid, on which diagnostic investigations were undertaken: possible viral and bacterial infections, medical conditions, iatrogenic causes, neoplastic and connective tissue diseases were all excluded. Despite empirical therapy with NSAIDs and colchicine, after about one week she had a recurrence of pericardial effusion and progressive development of constriction. Echocardiography performed after a few weeks of anti-inflammatory therapy showed resolution of constriction and PE, with clinical improvement. If progression of pericardial syndromes to a constrictive form is rarely described in literature, cases of transitory effusive-constrictive phase are even more uncommon, mainly reported during the evolution of pericardial effusion. According to the available data, risk of progression to a constrictive form is very low in case of idiopathic pericardial effusion. We report a case of large idiopathic subacute pericardial effusion, treated with pericardiocentesis and then evolved into an effusive-constrictive pericarditis. A prolonged anti-inflammatory treatment leads to complete resolution of pericardial syndrome without necessity of pericardiectomy.

Effusive-constrictive pericarditis, hepatitis, and pancreatitis in a patient with possible coxsackievirus B infection: a case report.

BACKGROUND: Coxsackie B is a viral pathogen that presents with various invasive diseases in adults. Historically, the majority of adult cases with pericarditis or myocarditis have been attributed to coxsackievirus B. The presentation of this viral infection causing effusive-constrictive pericarditis, hepatitis or pancreatitis is rare. This case report is the first to describe a patient with concomitant effusive-constrictive pericarditis, hepatitis and pancreatitis from possible coxsackievirus B infection. CASE PRESENTATION: A 26-year old female was admitted to our hospital with the diagnosis of effusive-constrictive pericarditis complicated by tamponade and cardiac arrest. An emergent pericardiocentesis was performed successfully. Hepatitis and pancreatitis were also identified in our patient. After an extensive workup, coxsackievirus B infection was suspected by positive serum complement fixation antibody titers. Our patient made a full recovery and was discharged from the hospital. CONCLUSION: Clinical suspicion of effusive-constrictive pericarditis with tamponade from coxsackievirus B should be considered in patients presenting with chest pain, dyspnea, jugular venous distention, hypotension, ST segment elevation on electrocardiogram, and ventricular interdependence with septal shift during diastole on transthoracic echocardiogram. Initial diagnoses of effusive-constrictive pericarditis resembling cardiac tamponade, hepatitis and pancreatitis can be challenging, and this case highlights the need for healthcare professionals to be cognizant of the association between these unusual clinical presentations and coxsackievirus B infection.

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.].

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.

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.

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.

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.

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.

Breaking hearts and taking names: A case of sarcoidosis related effusive-constrictive pericarditis.

INTRODUCTION: Pericardial involvement of sarcoidosis is a rare cause for acute heart failure, and usually occurs as a result of the development of a pericardial effusion leading to cardiac tamponade. Even rarer still, is the manifestation of constrictive pericarditis. We report a case of sarcoidosis with lung, pleural, and pericardial involvement with effusive-constrictive pericarditis leading to cardiac tamponade. CASE PRESENTATION: A 34-year-old Caucasian man presented for evaluation of a history of worsening exertional dyspnea, edema, and weight loss. A high-resolution chest computed tomography showed diffuse pulmonary nodules with upper lobe predominance and in a perilymphatic distribution; large right pleural effusion; and large pericardial effusion with pericardial thickening. A transthoracic echocardiogram demonstrated early tamponade physiology for which a pericardial drain was placed. After removal of the drain he developed cardiogenic shock from cardiac tamponade attributed to the reaccumulation of a pericardial effusion and urgent pericardial window was performed. Serial echocardiography was concerning for organization and localization of the pericardial fluid. Cardiac magnetic resonance imaging demonstrated a significant reduction in pericardial slippage between the parietal and visceral layers around the heart collectively suggestive of constrictive pericarditis. Confirmation of effusive-constrictive pericarditis was noted on right heart catheterization. He then underwent pericardiectomy, which on histopathologic evaluation demonstrated non-necrotizing granulomas, thus confirming pericardial involvement of sarcoidosis. CONCLUSIONS: We report a case demonstrating unique manifestations of sarcoidosis; effusive-constrictive pericarditis presenting with acute congestive heart failure.

A Complicated Case of Transient Constrictive Pericarditis Secondary to Rivaroxaban-Associated Hemopericardium.

A 72-year-old man on rivaroxaban developed effusive constrictive pericarditis secondary to hemopericardium. His condition improved with anti-inflammatory therapy supporting a diagnosis of transient constrictive pericarditis. On follow-up, residual constriction developed requiring surgical pericardiectomy. Although many cases with transient constrictive pericarditis resolve with medical management, some may progress and require pericardiectomy. (Level of Difficulty: Advanced.).