Erdheim-Chester Disease presenting with constrictive pericarditis: A case report and review of the literature.

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.

[Management of Constrictive Pericarditis : A study of 43 operated cases]. / Gestion de la péricardite constrictive : à travers l'étude de 43 cas opérés.

Chronic constrictive pericarditis is a rare condition characterized by clinical signs of right heart failure, due to the symphysis of the two pericardial leaflets. Our study focused on a retrospective analysis of 43 CCP surgery observations collected over an 11-year period (2003-2013). The mean age of the patients was 32 years; 65% were male; exercise dyspnea (95%) was the most frequent sign. Two main etiologies were observed: tuberculosis 58% and idiopathic causes 42%. All of our patients received a subtotal pericardectomy per median sternotomy, of which 95% had no cardiopulmonary bypass.

Novel role of cardiovascular MRI to contextualise tuberculous pericardial inflammation and oedema as predictors of constrictive pericarditis.

Tuberculosis (TB) and human immunodeficiency virus/acquired immunodeficiency syndrome have reached epidemic proportions, particularly affecting vulnerable populations in low- and middle-income countries of sub-Saharan Africa. TB pericarditis is the commonest cardiac manifestation of TB and is the leading cause of constrictive pericarditis, a reversible (by surgical pericardiectomy) cause of diastolic heart failure in endemic areas. Unpacking the complex mechanisms underpinning constrictive haemodynamics in TB pericarditis has proven challenging, leaving various basic and clinical research questions unanswered. Subsequently, risk stratification strategies for constrictive outcomes have remained unsatisfactory. Unique pericardial tissue characteristics, as identified on cardiovascular magnetic resonance imaging, enable us to stage and quantify pericardial inflammation and may assist in identifying patients at higher risk of tissue remodelling and pericardial constriction, as well as predict the degree of disease reversibility, tailor medical therapy, and determine the ideal timing for surgical pericardiectomy.

Acute onset of constrictive pericarditis due to acute myelomonocytic leukemia: A case and literature review.

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.

Cardiac magnetic resonance feature tracking derived left atrial strain in the diagnosis of patients with constrictive pericarditis and restrictive cardiomyopathy.

Objective: To explore the diagnostic value of cardiac magnetic resonance feature tracking (CMR-FT) divided left atrial (LA) strain in differentiating constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). Methods: Patients with CP (n = 40) and RCM (n = 40), and another 40 normal control group were retrospectively enrolled over a period of 8 years at a tertiary cardiac centre. Left ventricular (LV) and biatrial strain and strain rate (SR) were measured. Atrial strain was used to differentiate between patients with CP and RCM. Then, patients were grouped according to their left ventricular ejection fraction (LVEF), either ≥50% or < 50%. A deeper analysis was done to evaluate the diagnostic value of atrial strain in these subgroups. Receiver operating characteristic curves (ROC) were used to assess the accuracy of myocardial strain based on CMR FT for the differential diagnosis of CP and RCM. Results: LV and LA strain and SR were significantly lower in patients with CP and RCM than those in the normal controls (P < 0.05). LA strain and SR were significantly lower in the RCM group than in the CP group (P < 0.05). In patients with either LVEF≥50% or＜50%, LA strain were lower in the RCM group than in the CP group (P < 0.05). ROC analysis showed that LA stored strain (LA-εs) had a good differential diagnostic value for CP and RCM, with an area under the curve (AUC) of 0.811 and an optimal cutoff value of 6.98%, above this value it tends to develop CP. Further, an excellent differential diagnostic value was found in patients with LVEF＜50%, with an AUC of 0.955. Conclusion: LA strain analysis obtained by CMR-FT provides good differential diagnostic value for distinguishing CP from RCM, especially in patients with LVEF＜50%.

IgG4-Related disease with diffuse myopericardial involvement- value of CMR: a case report and literature review of cardiac involvement.

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. CASE PRESENTATION: 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.

Ultrasound analysis of cervical thoracic duct for patients with constrictive pericarditis and chylothorax.

PURPOSE: To analyze ultrasound features of cervical thoracic duct for patients with constrictive pericarditis and chylothorax. METHODS: Patients were retrospectively assessed. The patients were divided into a non-pleural effusion (PE) group (n = 54), a chylothorax group (n = 23), and non-chylothorax group (n = 28). Conventional ultrasound was used to obtain the maximum inner diameter and collapse of the inferior vena cava, the inner diameter of left cervical thoracic duct, and the frequency of opening of the valve at the end of the left thoracic duct. Contrast ultrasonography was used to score the reverse flow of the thoracic tube. RESULTS: The percentage of PE was 48.5%, and the percentage of chylothorax was 21.9%. The three groups had significant differences in five parameters. The inner diameter of left cervical thoracic duct was correlated with the degree of central venous pressure. Contrast ultrasonography was effective in quantitative assessment of the degree of intravenous-thoracic cord reverse flow which correlated with all parameters of central venous pressure. CONCLUSION: Thoracic duct dilation and regurgitation secondary to central venous pressure can lead to chyloreflux disorder and may be the mechanism of chylothorax occurrence in constrictive pericarditis.

Pericardiectomy after constrictive pericarditis associated with second dose of BNT162b2 vaccine: A case report.

Constrictive pericarditis is quite rare complication of messenger ribonucleic acid-based severe acute respiratory syndrome-Coronavirus 2 (SARS-CoV-2) vaccine. It is a severe clinical picture with clinical symptoms of right ventricular failure. Initial physical examination, laboratory work-up, and chest X-ray may yield non-specific findings. Echocardiography, computed tomography, and cardiac catheterization are other diagnostic tools. Surgery with pericardiectomy is the definitive treatment option. Herein, we report a case of pericardiectomy after constrictive pericarditis associated with the second dose of BNT162b2 vaccine.

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

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

Early postoperative constrictive pericarditis in China: a single-center retrospective observational study.

Background: The diagnosis, treatment, and prognosis of early postoperative constrictive pericarditis (EPCP) have not been discussed in depth. The objective of this study was to devise and propose a management strategy for EPCP. Methods: In this study, constrictive pericarditis (CP) within 6 months after cardiac surgery was defined as EPCP, and patients were divided into two groups based on intraoperative findings: a parietal thickening group and a visceral thickening group. Results: A total of 20 patients were included in this study, and the incidence rate of recurrent pericardiectomy was 0.32% among all patients undergoing cardiovascular surgery. EPCP after valve surgery occurred in 85.0% of patients. Pleural effusion was the most common preoperative symptom, occurring in 90% of patients. Pericardial thickening occurred in the visceral layer in seven cases and in the parietal layer in 13 cases. There were no differences in comorbidities, C-reactive protein (CRP) level, or erythrocyte sedimentation rate (ESR) between the two groups. Most patients with visceral thickening (83.3%) needed cardiopulmonary bypass (CPB) assistance during surgery and had a longer hospital stay than those with parietal thickening (52.8±21.8 vs. 34.9±13.8 days, P=0.049). Central venous pressure (CVP) was decreased in all patients after pericardiectomy (24.9±6.96 vs. 8.9±2.92 cmH2O, P<0.001), and the cardiac function improved significantly in patients with parietal thickening [New York Heart Association (NYHA) grade ≥ III accounted for 28.6% of patients]. The long-term survival rate of patients with parietal thickening was 92.3% and that of patients with visceral thickening was 57.1%, and there was no significant difference between them (P=0.056). Conclusions: Recurrent episodes of chest tightness, pleural effusion, and elevated CVP within 6 months after cardiac surgery should be considered highly suggestive of EPCP. There are few points of difference between pericarditis with thickening of the parietal and visceral layers. After failure of conservative medical treatment, pericardiectomy results in significant improvements in cardiac function and quality of life, especially in patients with thickening of the parietal layer.

Clinical symptoms, pathogenesis and postoperative course of non-specific constrictive pericarditis with dumbbell-shaped heart.

Non-specific chronic constrictive pericarditis is a rare and debilitating chronic infection in developed countries and its rapid diagnosis and treatment has not affected its outcome and complication. A 15-year-old male, well nourished, negative HIV test, and without a history of previous pulmonary tuberculosis, was admitted to our hospital for exertional dyspnea (New York Heart Association, NYHA, functional class II). Our patient had had no pulmonary tuberculosis during childhood, had received anti-tuberculosis treatment, and was referred to our center for further surgical pericardiectomy.

Calcific constrictive pericarditis following tumour necrosis factor-alpha inhibition.

Background: Tumour necrosis factor (TNF)-alpha inhibition is a core therapeutic avenue for a broad range of inflammatory and autoimmune disorders including rheumatoid arthritis and inflammatory bowel disease, as well as dermatological conditions such as hidradenitis suppurativa. Adalimumab has become one of the most common TNF-alpha-inhibiting agents, which is used for many of these conditions. Treatment with such agents is associated with numerous systemic side effects, though cardiac complications remain relatively rare. These include reports of pericarditis and pericardial effusions1-3. Case summary: A 63-year-old lady was referred to the outpatient respiratory clinic with a 1-year history of increasing breathlessness, on a background of 4 years of treatment with adalimumab for Stage III hidradenitis suppurativa. A high-resolution computed tomography (CT) thorax revealed evidence of pericardial calcification. Subsequent left and right heart catheterization study revealed equalization of intraventricular pressures, consistent with constrictive pericarditis. A QuantiFERON test was negative, and rheumatological serology was unremarkable. The patient was initially managed conservatively with close follow-up, before undergoing surgical pericardectomy when she developed signs of cardiac failure. Discussion: Adalimumab is associated with a range of systemic side effects, though cardiac complications are relatively rare. This case highlights a potentially novel complication associated with prolonged adalimumab therapy. Given that there are reports in the literature of pericarditis and pericardial effusions associated with TNF-alpha inhibition1-3, it is reasonable to hypothesize that the calcific constrictive pericarditis seen in this case may demonstrate a novel cardiac phenomenon associated with this therapy, given the lack of any traditional aetiological factors.

The clinical value of P-wave terminal force in lead V1 in evaluating pericardial thickness in tuberculous constrictive pericarditis.

AIM: To investigate the relationship between p wave terminal force (Ptfv1) and pericardial thickness in patients with tuberculous constrictive pericarditis. METHODS: From January 2018 to October 2022, 95 patients with tuberculous constrictive pericarditis who needed pericarditis dissection in a hospital were collected, and 3 patients who did not meet the criteria were excluded, a total of 92 cases. The absolute value of Ptfv1 in conventional electrocardiogram was tested before surgery, and pericardial thickness was measured by echocardiography and chest CT. Pericardial thickness was measured after pericardial dissection. Pearson correlation analysis was used, R software was used to make scatter plot, and non-parametric square test was used. The correlation of postoperative measurements with echocardiography, chest CT and absolute value of Ptfv1 was analyzed. RESULTS: Pearson correlation analysis was conducted with postoperative measurements and echocardiography measurements, postoperative measurements and chest CT measurements, and postoperative measurements and absolute value of Ptfv1. Pearson correlation analysis showed that the correlation coefficients between postoperative measurements and echocardiography, chest CT and Ptfv1 values were statistically significant. Scatter plot and nonparametric Chi-square test showed that postoperative measurements were consistent with absolute values of echocardiography, chest CT and Ptfv1 (p < 0.05). And this study found that the distribution of the value of Ptfv1 ≥ 5 was higher than the value of Ptfv1 < 5 after pericardiectomy (0.95:0.05) in the absolute value of Ptfv1 ≥ 0.04 which measured before pericardiectomy. The hypothesis was statistically significant (p < 0.05). CONCLUSION: The absolute value of Ptfv1 in electrocardiogram can be used as an auxiliary diagnostic index to evaluate pericardial thickness in tuberculous constrictive pericarditis.

Pericardiectomy for Constrictive Pericarditis with or without Cardiopulmonary Bypass.

Aim: We aim to access the effect of pericardiectomy for constrictive pericarditis with or without cardiopulmonary bypass. Methods: This was a review of pericardiectomy for constrictive pericarditis. Results: Cardiopulmonary bypass is actually an important maneuver to attain complete relief of the constriction. The short additional time of cardiopulmonary bypass during the procedure has very little effect on the risk of morbidity of the main operation. Conclusion: Incomplete pericardiectomy perhaps was the cause of postoperative remnant constriction and high diastolic filling pressure leading to multiorgan failure. Complete pericardiectomy (removal of phrenic-to-phrenic and the postero-lateral and inferior wall pericardial thickening) using cardiopulmonary bypass should be the routine for total relief of the constriction of the heart.

A classical case report of constrictive pericarditis, highlighting the role of magnetic resonance imaging and haemodynamic assessment.

Background: Constrictive pericarditis is a rare complication of pericarditis and is difficult to diagnose due to non-specific presentation. It mostly presents with right-sided heart failure as a consequence of a rigid pericardium that encases the heart causing impaired diastolic filling. Case summary: We present the case of a patient with signs and symptoms of dyspnoea and right-sided heart failure who was initially diagnosed with heart failure with preserved ejection fraction (HFpEF) but remained symptomatic despite being euvolaemic after treatment. A septal bounce and shudder on echocardiogram prompted further investigation. Eventually, cardiac magnetic resonance (CMR) imaging and invasive biventricular pressure measurements led to the diagnosis of constrictive pericarditis. A pericardiectomy was performed after which the patient was relieved of symptoms. Discussion: Constrictive pericarditis can mimic HFpEF. Due to its potentially curable character, timely recognition is of cardinal importance. In patients with symptoms of severe right-sided heart failure not resolving after diuretic therapy, a septal shudder on echocardiography should trigger further investigation, with e.g. CMR and cardiac catheterization.

Diagnostic procedure for localized constrictive pericarditis leading to bilateral pleural effusion: two case reports.

Constrictive pericarditis is a rare disease. Localized constrictive pericarditis leading to bilateral pleural effusion is more difficult to recognize, and the diagnostic procedure can be ambiguous. Here, we report two patients diagnosed with localized constrictive pericarditis who presented with bilateral pleural effusion. A thorough work-up showed that the pleural effusion was nonspecific, as was the pathology of the pleura. One patient had a history of pericardial effusion 2 years ago, and the other had undergone surgery for an anterior mediastinum teratoma. Pericardial scarring was found on their chest CT scans. The patients underwent pericardiectomy, and localized pericardial thickening was excised. The bilateral pleural effusion was effectively cured, and the patients showed satisfactory recovery on follow-up. Physicians should be aware of localized pericarditis leading to bilateral pleural effusion, and pericardiectomy is an effective diagnostic and therapeutic procedure.

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.

Cardiac tamponade associated with primary myelofibrosis: a case report.

Background: Cardiac tamponade is a life-threatening condition that occurs when an abnormal amount of fluid accumulates in the pericardial sac and impedes the cardiac filling process. Although extremely rare, haematological diseases have the potential to trigger an extramedullary haematopoiesis (EMH) process within the pericardium, resulting in a substantial build-up of pericardial effusion. Case summary: We present the case of a 29-year-old male previously diagnosed with primary myelofibrosis (PMF), who presented to the emergency unit with cardiac tamponade. An emergent pericardiocentesis procedure was performed, successfully evacuating 850 mL of haemorrhagic fluid. Over the course of 3 days, a total of 1.5 L of haemorrhagic effusion were drained from the pericardial space. Analysis of the pericardial fluid revealed evidence of haematopoietic activity, suggesting a potential association with the EMH process occurring within the pericardium. Following a 7-day hospitalization, the patient was discharged in stable condition but later experienced the development of constrictive pericarditis. Discussion: Haemorrhagic pericardial effusion is a rare occurrence. The majority of cases stems from complications of medical procedures (iatrogenic), malignancies, or side effects of antiplatelet/anticoagulant medications. In patients with PMF, the impaired haematopoietic ability caused by the fibrotic process in the bone marrow compels the body to produce blood components elsewhere, a phenomenon known as EMH. On very rare occasions, EMH can develop in the pericardial space, potentially leading to life-threatening cardiac tamponade. Our patient was successfully managed through pericardial fluid evacuation and drainage but later developed constrictive pericarditis.

Clinical Significance of Preoperative Pyrazinamide-Containing Therapy in Tuberculous Constrictive Pericarditis.

Background: Tuberculous constrictive pericarditis (TCP) is recommended to be treated with anti-tuberculosis (TB) therapy before pericardiectomy. Whether different preoperative anti-TB regimens may lead to different outcomes is unclear. Methods: We retrospectively collected patients diagnosed as TCP and received pericardiectomy from April 2016 to June 2023. The study patients were assigned into the active TCP (A-TCP) group and the inactive TCP (IA-TCP) group according to the results of Mycobacterium tuberculosis (MTB) culture and MTB RNA assay. Baseline characteristics including anti-TB regimens and surgical outcomes were compared between the two groups. Logistic regression analysis and subgroup analysis were conducted to identify the protective factors of A-TCP. Results: Of the 102 study patients, 24 was in the A-TCP group and 78 was in the IA-TCP group. The rate of preoperative anti-TB regimen containing pyrazinamide was 37.5% in the A-TCP group, as compared with 74.4% in the IA-TCP group (P = 0.001). Multivariate analysis showed that preoperative use of pyrazinamide was the protective factor of A-TCP (OR 0.194, 95% CI 0.053-0.703, P = 0.013). Subgroup analysis based on age also showed consistent findings. In the analyses of surgical outcomes, A-TCP was the independent risk factor of postoperative cardiac complications (OR 4.231, 95% CI 1.317-13.593, P = 0.015) and associated with longer hospital stay (P = 0.004) and higher hospitalization cost (P = 0.001). Conclusion: A strategy involving anti-TB regimen containing pyrazinamide before pericardiectomy was superior to that without pyrazinamide in the patients with TCP. The strategy was associated with lower risk of A-TCP and might lead to better postoperative recovery and cost-effectiveness.