differentiating constrictive pericarditis and

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...

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...

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...

INTRODUCTION: Mulibrey nanism (MUL) is a rare disorder caused by TRIM37 gene variants characterized by growth failure, dysmorphic features, congestive heart failure (CHF), and an increased risk of Wilms' tumor. Although immune system impairment has been documented in MUL, the underlying mechanisms remain poorly understood. METHODS: We present a case of MUL with progressive lymphopenia and review similar cases from the literature. RESULTS: Our patient presented with prenatal onset growth restriction, characteristic dysmorphic features, and Wilms' tumor...

BACKGROUND: Constrictive pericarditis (CP) is an uncommon but reversible cause of diastolic heart failure if appropriately identified and treated. However, its diagnosis remains a challenge for clinicians. Artificial intelligence may enhance the identification of CP. OBJECTIVES: The authors proposed a deep learning approach based on transthoracic echocardiography to differentiate CP from restrictive cardiomyopathy. METHODS: Patients with a confirmed diagnosis of CP and cardiac amyloidosis (CA) (as the representative disease of restrictive cardiomyopathy) at Mayo Clinic Rochester from January 2003 to December 2021 were identified to extract baseline demographics...

PURPOSE: The inherent characteristics of transthoracic echocardiography (TTE) images such as low signal-to-noise ratio and acquisition variations can limit the direct use of TTE images in the development and generalization of deep learning models. As such, we propose an innovative automated framework to address the common challenges in the process of echocardiography deep learning model generalization on the challenging task of constrictive pericarditis (CP) and cardiac amyloidosis (CA) differentiation...

BACKGROUND: To assess the diagnostic accuracy of the Mayo Clinic echocardiographic criteria for differentiating between constrictive pericarditis and restrictive cardiomyopathy. METHODS: We searched electronic databases for the date range from their inception to July 1, 2022. The index tests were the Mayo Clinic echocardiographic criteria. We performed a bivariate random-effects model to estimate the pooled sensitivity and specificity, each with 95% confidence interval (CI)...

In the evaluation of heart failure, 2 differential diagnostic considerations include constrictive pericarditis and restrictive cardiomyopathy. The often outwardly similar clinical presentation of these 2 pathologic entities routinely renders their clinical distinction difficult. Consequently, initial assessment requires a keen understanding of their separate pathophysiology, epidemiology, and hemodynamic effects. Following a detailed clinical evaluation, further assessment initially rests on comprehensive echocardiographic investigation, including detailed Doppler evaluation...

The Mulibrey (Muscle-liver-brain-eye) nanism caused by loss-of-function variants in TRIM37 gene is an autosomal recessive disorder characterized by severe growth failure and constrictive pericarditis. These patients also suffer from severe respiratory infections, co-incident with an increased mortality rate. Here, we revealed that TRIM37 variants were associated with recurrent infection. Trim37 FINmajor (a representative variant of Mulibrey nanism patients) and Trim37 knockout mice were susceptible to influenza virus infection...

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...

BACKGROUND: ECG is the initial diagnostic tool that in combination with typical symptoms often raises the suspicion of pericarditis. Echocardiography remains the first-line imaging modality for assessment of pericardial diseases, particularly effusion/tamponade, constrictive physiology, and assessment of regional wall motion abnormalities as differential diagnoses. However, cardiac CT and cardiac magnetic resonance may be necessary in complicated cases, and to identify pericardial inflammation in specific settings (atypical presentation, new onset constriction), as well as myocardial involvement and monitoring the disease activity...

We describe a case of a 67-year-old African American man who presented to the emergency department with a sharp, pleuritic chest pain and shortness of breath. After several admissions and extensive workup, he ultimately was diagnosed with a persistent pleural effusion, pericardial effusion, and secondary constrictive pericarditis due to rheumatoid arthritis. By highlighting immunological disorders such as rheumatoid arthritis in the differential diagnosis, in the setting of a refractory pericardial effusion and serositis, this case report addresses key aspects of the presentation both in the emergency and inpatient settings, reviews the criteria for a rheumatoid arthritis diagnosis, and emphasizes areas of importance in predominantly cardiopulmonary extra-articular manifestations of a typically musculoskeletal disease...

We report on a 69-year-old man suffering from chronic progressive oligoarthritis (localized in metacarpal and knee joints), which clinically was interpreted as steroid-sensitive seronegative chronic arthritis. The patient died from sudden death at the emergency department after a 4-week history of increasing cough and dyspnea (meanwhile obtaining negative testing results for SARS-CoV-2). During the autopsy, we found massive pancarditis affecting all cardiac compartments, in particular exhibiting constrictive pericarditis, myocarditis, and multivalvular endocarditis...

BACKGROUND: Takayasu's disease (TM) is a vasculitis of the aorta and its main branches, Infectious diseases, especially Mycobacterium tuberculosis , can be a trigger for the development of TM by a hypersensitivity reaction, but paradoxically very few cases of the disease are reported in the literature review. MATERIALS AND METHODS: Through a retrospective study carried out in the departments of internal medicine and cardiology of the Centre hospitalier universitaire de Libreville (CHUL) in Gabon, we identified all the observations of TM diagnosed in the said departments from 03/2014 to 05/2022, and rediscussed them on the basis of the criteria of Sharma et al...

Background: Constrictive pericarditis (CP) can be one of the most challenging conditions to diagnose within cardiovascular medicine. Iatrogenic causes of CP are increasingly recognized in higher income countries. This case provides insight into the need for clinical suspicion when diagnosing this relatively under recognized clinical entity as well as the need for multimodality imaging combined with invasive haemodynamic assessment. Case summary: A 68-year-old man presented with decompensated heart failure 4 weeks after open-heart surgery...

Introduction and importance: Pericarditis is a common illness that can appear in a variety of clinical settings and has numerous causes. In developing nations where tuberculosis is still a serious public health issue, more than 50% of cases of pericarditis are related to tuberculosis. Case presentation: There was no history of TB, alcoholism, IV drug abuse, immunosuppressant, or corticosteroid use. On examination, she had a fever, tachycardia, pulsus paradoxus of 10 mmHg, hypotension, tachypnea, and a distended jugular vein...

PURPOSE OF REVIEW: Overlapping hemodynamics in constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) often pose difficulties in establishing accurate diagnosis. Echocardiography is the first-line imaging modality used for this purpose, but no single echocardiographic parameter is sufficiently robust for distinguishing between the two conditions. The newer developments may improve the diagnostic accuracy of echocardiography in this setting. RECENT FINDINGS: Recent studies have validated multiparametric algorithms, based on conventional echocardiographic parameters, which enable high sensitivity and specificity for distinguishing between CP and RCM...

Background: Constrictive pericarditis (CP) is a rare condition in which the pericardium becomes progressively fibrotic and non-compliant leading to impaired ventricular filling and overt heart failure. While CP shares many clinical and haemodynamic similarities with restrictive cardiomyopathy, differentiation of these diseases is crucial as CP is potentially curative through pericardiectomy. Here, we present a case of proven pericardial constriction with atypical haemodynamics in a patient presenting with heart failure and severe left main coronary artery disease (CAD)...

BACKGROUND: Primary malignant pericardial mesothelioma (PMPM) is a highly malignant tumor originating in the pericardium serosum with clinical manifestations presenting as constrictive pericarditis, with pericardial tamponade and heart failure. Malignant pericardial mesothelioma is rare and has a poor prognosis, with an average survival time of 6-10 months. CASE DESCRIPTION: Herein, we report the case of a 57-year-old female who developed chest tightness and panic for no obvious reason...

BACKGROUND: Restrictive cardiomyopathy (RCM) presents a high risk for sudden cardiac death in pediatric patients. Constrictive pericarditis (CP) exhibits a similar clinical presentation to RCM and requires differential diagnosis. While mutations of genes that encode sarcomeric and cytoskeletal proteins may lead to RCM, infection, rather than gene mutation, is the main cause of CP. Genetic testing may be helpful in the clinical diagnosis of RCM. METHODS: In this case series study, we screened for TNNI3, TNNT2, and DES gene mutations that are known to be etiologically linked to RCM in four pediatric patients with suspected RCM...