Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with diverse clinical features. It is characterized by systemic histiocyte infiltration of the bone, skin, central nervous system, lung, kidney, and cardiovascular system. Pericardial involvement is frequently revealed through either pericardial effusion or pericardial thickening in patients with ECD. Although most patients remain asymptomatic, progressive pericarditis, effusion, or cardiac tamponade may occur. Herein, we report a rare and unusual presentation of ECD in a 51-year-old man who experienced severe constrictive pericarditis. The patient presented with uncontrolled fluid retention and heart failure. After the diagnosis of ECD, interferon alpha treatment was administered. The patient recovered dramatically with decreased pleural and pericardial effusion, as well as improvements in the echocardiographic signs of constrictive pericarditis. Despite several therapeutic options described in the literature for managing ECD-related pericardial disease, a standard treatment has not been established. This report highlights the importance of early treatment based on accurate diagnosis of an unusual ECD complication.

Remarkable advances have occurred in the understanding of the pathophysiology of pericardial diseases and the role of multimodality imaging in this field. Medical therapy and surgical options for pericardial diseases have also evolved substantially. Pericardiectomy is indicated for chronic or irreversible constrictive pericarditis, refractory recurrent pericarditis despite optimal medical therapy, or partial agenesis of the pericardium with a complication (eg, herniation). A multidisciplinary evaluation before pericardiectomy is essential for optimal patient outcomes. Overall, given the good outcomes reported, radical pericardiectomy on cardiopulmonary bypass, if feasible, is the preferred approach. Due to patient complexity, as well as the technical aspects of the surgery, pericardiectomy should be performed at high-volume centers that have the required expertise. The current review highlights the essential features of this multidisciplinary approach from diagnosis to recovery in patients undergoing pericardiectomy.

Advances in multi-modality cardiac imaging have aided the evaluation, surveillance and treatment guidance of pericardial diseases, which have traditionally been a challenging group of conditions to manage. Although echocardiography remains the first-line imaging modality to assess the pericardium, both computed tomography (CT) and magnetic resonance imaging (MRI) have valuable complimentary roles. It is critical for clinicians to have a clear understanding of the utilities, advantages and disadvantages of these cardiac imaging modalities in pericardial pathologies. This contemporary review provides an update regarding the applications of multi-modality cardiac imaging in the evaluation of pericardial syndromes including acute/recurrent pericarditis, effusion/tamponade, constriction, masses and congenital anomalies.

Purulent pericarditis is a rare and potentially life-threatening condition characterized by infection of the pericardial space. We describe a case of purulent bacterial pericarditis in a 41-year-old male with no significant medical or surgical history who had concomitant pulmonary Histoplasma infection. Streptococcus intermedius was the bacteria directly responsible for the pericardial infection, though co-infection with histoplasmosis likely predisposed him to develop purulent pericarditis. We hypothesize histoplasmosis caused mediastinal lymphadenopathy, facilitating contact between a necrotic lymph node and the pericardium and contiguous suppuration of bacteria to the pericardial space. We treated S. intermedius and Histoplasma capsulatum with ceftriaxone and amphotericin B, respectively. Additionally, the patient presented in cardiac tamponade requiring emergent pericardiocentesis and drain placement. His course was also complicated by pericardial constriction. Cardiac magnetic resonance confirmed this, showing inflamed pericardium and abnormal septal motion with inspiration, and he had symptoms refractory to antimicrobials and anti-inflammatories. As such, he required pericardiectomy. This case demonstrates maintaining suspicion for secondary infectious foci as a contributor to the pathogenesis of purulent pericarditis is important, as pulmonary histoplasmosis played a pivotal role in allowing S. intermedius to spread to the pericardium but was not the primary infection. It also highlights the multifaceted evaluation and management of purulent pericarditis, highlighting the role of echocardiography and emergent pericardial drainage if cardiac tamponade is present, the importance of targeted antimicrobial therapy, the superior ability of cardiac magnetic resonance to identify pericardial constriction as a sequela of purulent pericarditis, and indications for pericardiectomy.

Chronic constrictive pericarditis results in the signs and symptoms of heart failure due to the chronic compression of the myocardium. When the pericardium becomes thickened and fibrosed, surgery is the sole curative management strategy. Surgery leads to an improvement in functional status and survival, but it is associated with significant mortality and morbidity. Over the years, the surgical approach to this pathology has largely remained unchanged, but there has been a shift in the etiological mechanism of constrictive pericarditis from predominantly tuberculous to post-cardiac surgery and idiopathic pathologies. This review offers an overview of the surgical management of constrictive pericarditis.

Erdheim-Chester Disease (ECD) is a rare form of histiocytosis characterized by xanthomatous infiltration of affected organs. We present a case of a 62-year-old man with ECD initially presenting with constrictive pericarditis. Comprehensive imaging revealed systemic involvement, including the skeleton, orbit, pituitary, lung, kidney, and retroperitoneum, despite the absence of related symptoms. The diagnosis of ECD was eventually confirmed through histopathological evidence from a CT-guided biopsy. The patient responded well to interferon-α2b treatment, with gradual symptom amelioration and improvement in imaging and laboratory findings over a 5-month follow-up period. This case highlights the importance of considering ECD in the differential diagnosis of constrictive pericarditis and the utility of multimodal imaging for accurate diagnosis and management of this rare disease. The patient\'s positive response to treatment also highlights the potential for effective management of ECD, particularly with early diagnosis and intervention.

We herein present a fatal case of constrictive pericarditis (CP) due to acute myelomonocytic leukemia (AMML) in a patient who initially complained of an acute onset of chest pain two days after COVID-19 vaccination. An autopsy revealed pericardial infiltration of leukemic cells. CP is rarely associated with leukemia and only 14 cases have been reported in the literature. The etiology of CP in previous reports included leukemic infiltration, graft-versus-host disease, drug-induced, post-radiation, autoimmune, and otherwise unidentified. This case indicates that leukemic infiltration can cause CP and that clinicians should include leukemia in the differential diagnosis of CP.

BACKGROUND: IgG4-related disease is a fibro-inflammatory disorder with an unknown etiology, which can affect multiple organ systems, including the cardiovascular system. While most reported cases of cardiovascular involvement are primarily associated with the aorta, there have been sporadic reports of isolated cardiac involvement.
METHODS: This paper presents a documented case of IgG4-related systemic disease with symptoms indicative of restrictive cardiomyopathy. Subsequent Cardiac Magnetic Resonance imaging revealed diffuse myopericardial involvement, characterized by pericardial thickening and enhancement, accompanied by subepicardial and myocardial infiltration. Considering the rarity of cardiac involvement in our case, we conducted a thorough review of the existing literature pertaining to various patterns of cardiac involvement in IgG4-related disease, as well as the diagnostic modalities that can be employed for accurate identification and assessment.
CONCLUSIONS: This case report sheds light on the importance of recognizing and evaluating cardiac manifestations in IgG4-related systemic disease to facilitate timely diagnosis and appropriate management.

The data on constrictive pericarditis following heart transplantation are scarce. Herein, the authors present 2 patients who developed a constrictive pericarditis 19, and 55 months after heart transplantation. They underwent several diagnostic procedures and successfully recovered after a radical pericardiectomy. In addition, the authors review the literature and report the incidence, aetiology, diagnostic features, and management of this rare and challenging condition.

Purulent pericarditis is the infection of the pericardial space with pus formation. High mortality and morbidity can be explained by cardiac tamponade and septic shock in the acute phase, while chronically, it can lead to recurrent purulent pericarditis and constrictive pericarditis. We present two cases of purulent pericarditis treated with intrapericardial recombinant tissue plasminogen activator (r-tPA) for three consecutive days in addition to surgical pericardial drainage. In both instances, loculated effusions and re-accumulation of pericardial fluid persisted despite adequate antibiotic coverage and surgical drainage. Intrapericardial fibrinolysis was considered a less invasive alternative to extensive surgery to prevent constrictive pericarditis and improve clinical outcomes. Both patients had complete clinical recovery and there was no evidence of constrictive pericarditis during follow-up. There is scant literature regarding r-tPA therapy for purulent pericarditis, most of which is limited to case reports or case series. The most commonly used regimen is three doses of tPA administered into the pericardial space over three days. It is a safe and potentially effective therapy in preventing constrictive pericarditis and need of pericardiectomy.