Successful Visceral and Parietal Pericardiectomy for Refractory Effusive-Constrictive Pericarditis: A Challenging Case Report.

BACKGROUND Effusive-constrictive pericarditis (ECP) is an uncommon clinical syndrome characterized by the coexistence of pericardial effusion and constriction involving the visceral pericardium. This differs from constrictive pericarditis, which presents with thickening of the pericardium without effusions. Specific diagnostic criteria of ECP include the failure of right atrial pressure to decrease by 50% or reach a new level below 10 mmHg after normalization of intrapericardial pressure. CASE REPORT We present the case of a 32-year-old obese man with multiple comorbidities who initially presented with flu-like symptoms and pleural effusion with development of constrictive-like symptoms. Despite undergoing numerous pericardiocentesis and appropriate medical management, the patient's condition failed to improve, leading to the likely diagnosis of effusive-constrictive pericarditis. Cultures of pericardial fluid revealed E. -faecium, which required multiple antimicrobial therapy. Despite infection, the exact etiology of ECP remained unknown and likely idiopathic. Common causes of ECP include idiopathic, tuberculosis, cardiac surgery complications, radiation, or neoplasia. Ultimately, the patient underwent a pericardiectomy involving the visceral and parietal pericardium, resulting in hemodynamic stability and resolution of symptoms. CONCLUSIONS This case highlights the challenges in diagnosing and managing ECP, emphasizing the importance of considering surgical intervention in refractory cases. ECP initially presents as a pericardial effusion, often addressed through pericardiocentesis; however, in a small subset of patients, sustained symptoms and altered hemodynamics persist following pericardiocentesis, necessitating further evaluation and management. The success of pericardiectomy in our patient highlights the potential efficacy of surgical intervention in improving outcomes for patients with ECP.

A Rare Case of Immunoglobulin G4-Related Constrictive Pericarditis Diagnosed Via Multimodality Imaging.

Constrictive pericarditis (CP) presents as a pathophysiological state where the pericardium becomes inelastic due to fibrotic changes, most commonly secondary to a protracted inflammatory process. The disease is characterized by compromised diastolic cardiac function due to loss of pericardial compliance. Immunoglobulin G4 (IgG4)-related disease, an entity marked by the insidious proliferation of IgG4-positive plasma cells and subsequent fibrosis within various organs, is an infrequent but recognized cause of CP. A case of a 55-year-old male patient with clinical manifestations of dyspnea and edema in the lower extremities elucidates the diagnostic complexity inherent to CP. Echocardiography revealed a constellation of signs, including annulus reversus, septal bounce, and a congested inferior vena cava; cardiac magnetic resonance imaging (MRI) demonstrated diffuse pericardial thickening with delayed gadolinium enhancement, suggestive of a long-term inflammatory state; and right heart catheterization confirmed the hemodynamic hallmark of CP-equalization of diastolic pressures across the cardiac chambers. The serological analysis elicited elevated serum levels of IgG4 and IgE, pointing to the differential diagnosis of IgG4-related disease. Given the nonspecific clinical presentation of IgG4-related CP, a heightened index of suspicion combined with a systematic approach to imaging and serological evaluation is paramount.

A novel multi-task machine learning classifier for rare disease patterning using cardiac strain imaging data.

To provide accurate predictions, current machine learning-based solutions require large, manually labeled training datasets. We implement persistent homology (PH), a topological tool for studying the pattern of data, to analyze echocardiography-based strain data and differentiate between rare diseases like constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). Patient population (retrospectively registered) included those presenting with heart failure due to CP (n = 51), RCM (n = 47), and patients without heart failure symptoms (n = 53). Longitudinal, radial, and circumferential strains/strain rates for left ventricular segments were processed into topological feature vectors using Machine learning PH workflow. In differentiating CP and RCM, the PH workflow model had a ROC AUC of 0.94 (Sensitivity = 92%, Specificity = 81%), compared with the GLS model AUC of 0.69 (Sensitivity = 65%, Specificity = 66%). In differentiating between all three conditions, the PH workflow model had an AUC of 0.83 (Sensitivity = 68%, Specificity = 84%), compared with the GLS model AUC of 0.68 (Sensitivity = 52% and Specificity = 76%). By employing persistent homology to differentiate the "pattern" of cardiac deformations, our machine-learning approach provides reasonable accuracy when evaluating small datasets and aids in understanding and visualizing patterns of cardiac imaging data in clinically challenging disease states.

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.

Surgical treatment of constrictive pericarditis at a single center: 10 years of experience.

BACKGROUND: Constrictive pericarditis (CP) is a pericardial disease characterized by the pericardium becoming calcified or fibrotic as a result of chronic inflammation, which impairs diastolic filling by compressing the cardiac chambers. Pericardiectomy is a promising surgical option for treating CP. In this study, we reviewed over 10 years of preoperative, perioperative, and short-term postoperative follow-ups of patients who underwent pericardiectomy for constrictive pericarditis at our clinic. METHODS: Between January 2012 and May 2022, 44 patients were diagnosed with constrictive pericarditis. Twenty-six patients underwent pericardiectomy for CP. Median sternotomy is the surgical approach of choice because it provides easy access for complete pericardiectomy. RESULTS: The patient median age was 56 (min: 32, max: 71), and 22 out of 26 patients (84.6%) were male. Twenty-one patients (80.8%) complained of dyspnea, which was the most common reason for admission. Twenty-four patients (92.3%) were scheduled for elective surgery. Cardiopulmonary bypass (CPB) was used during the procedure in six patients (23%). The duration of intensive care stay was two days (min: 1, max: 11), and the total hospitalization was six days (min: 4, max: 21). No in-hospital mortality was observed. CONCLUSION: The median sternotomy approach provides a critical advantage in terms of performing a complete pericardiectomy. Although CP is a chronic condition, early diagnosis and planning of pericardiectomy before irreversible deterioration of cardiac function leads to a notable reduction in mortality and morbidity.

[An exemplificative case of constrictive pericarditis]. / Un caso esemplare, ma soprattutto esemplificativo, di pericardite costrittiva.

Constrictive pericarditis is a rare, but fatal disease, leading to heart failure due to diastolic dysfunction resulting from the fibrotic and non-elastic pericardium. Clinical presentation is sneaky, with initial symptoms of splanchnic and peripheral venous congestion, then with hepatomegaly and ascites: this kind of presentation is not often recognized, delaying diagnosis. We report the case of a young male adult with no previous cardiovascular history, but with a diagnosis of hepatic cirrhosis: investigations in our Centre led to the diagnosis of constrictive pericarditis, successfully treated with pericardiectomy; however, despite the effective venous decongestion, it was not possible to spare the patient from liver transplant.

Cardiac tamponade.

Cardiac tamponade is a medical emergency caused by the progressive accumulation of pericardial fluid (effusion), blood, pus or air in the pericardium, compressing the heart chambers and leading to haemodynamic compromise, circulatory shock, cardiac arrest and death. Pericardial diseases of any aetiology as well as complications of interventional and surgical procedures or chest trauma can cause cardiac tamponade. Tamponade can be precipitated in patients with pericardial effusion by dehydration or exposure to certain medications, particularly vasodilators or intravenous diuretics. Key clinical findings in patients with cardiac tamponade are hypotension, increased jugular venous pressure and distant heart sounds (Beck triad). Dyspnoea can progress to orthopnoea (with no rales on lung auscultation) accompanied by weakness, fatigue, tachycardia and oliguria. In tamponade caused by acute pericarditis, the patient can experience fever and typical chest pain increasing on inspiration and radiating to the trapezius ridge. Generally, cardiac tamponade is a clinical diagnosis that can be confirmed using various imaging modalities, principally echocardiography. Cardiac tamponade is preferably resolved by echocardiography-guided pericardiocentesis. In patients who have recently undergone cardiac surgery and in those with neoplastic infiltration, effusive-constrictive pericarditis, or loculated effusions, fluoroscopic guidance can increase the feasibility and safety of the procedure. Surgical management is indicated in patients with aortic dissection, chest trauma, bleeding or purulent infection that cannot be controlled percutaneously. After pericardiocentesis or pericardiotomy, NSAIDs and colchicine can be considered to prevent recurrence and effusive-constrictive pericarditis.

Post-cardiac injury syndrome after surgical repair of atrial septal defect: Reporting a rare occurrence.

A 16-year-old male with past medical history of congenital atrial septal defect surgical repair, presented with recurrent pericarditis secondary to post-cardiotomy injury syndrome (PCIS), After failing medical therapy, he ultimately underwent pericardiectomy for symptom resolution, PCIS is underdiagnosed in children and should be considered in patients with recurrent chest, pain.

Pericardial Disease in the Developing World.

Pericardial disease in the developing world is dominated primarily by effusive and constrictive syndromes and contributes to the acute and chronic heart failure burden in many regions. The confluence of geography (location in the tropics), a significant burden of diseases of poverty and neglect, and a significant contribution of communicable diseases to the general burden of disease is reflected in the wide etiological spectrum of causes of pericardial disease. The prevalence of Mycobacterium tuberculosis in particular, is high throughout much of the developing world where it is the most frequent and important cause of pericarditis and is associated with significant morbidity and mortality. Acute viral/idiopathic pericarditis, which is the primary manifestation of pericardial disease in the developed world is believed to occur significantly less frequently in the developing world. Although diagnostic approaches and criteria to establish the diagnosis of pericardial disease are similar throughout the globe, resource constraints such as access to multimodality imaging and hemodynamic assessment are a major limitation in much of the developing world. These important considerations significantly influence the diagnostic and treatment approaches, and outcomes related to pericardial disease.

The Evolving Etiologic and Epidemiologic Portrait of Pericardial Disease.

Pericardial disease includes a variety of conditions, including inflammatory pericarditis, pericardial effusions, constrictive pericarditis, pericardial cysts, and primary and secondary pericardial neoplasms. The true incidence of this varied condition is not well established, and the causes vary greatly across the world. This review aims to describe the changing pattern of epidemiology of pericardial disease and to provide an overview of causative etiologies. Idiopathic pericarditis (assumed most often to be viral) remains the most common etiology for pericardial disease globally, with tuberculous pericarditis being most common in developing countries. Other important etiologies include fungal, autoimmune, autoinflammatory, neoplastic (both benign and malignant), immunotherapy-related, radiation therapy-induced, metabolic, postcardiac injury, postoperative, and postprocedural causes. Improved understanding of the immune pathophysiological pathways has led to identification and reclassification of some idiopathic pericarditis cases into autoinflammatory etiologies, including immunoglobulin G (IgG)4-related pericarditis, tumour necrosis factor receptor-associated periodic syndrome (TRAPS), and familial Mediterranean fever in the current era. Contemporary advances in percutaneous cardiac interventions and the recent COVID-19 pandemic have also resulted in changes in the epidemiology of pericardial diseases. Further research is needed to improve our understanding of the etiologies of pericarditis, using the assistance of contemporary advanced imaging techniques and laboratory testing. Careful consideration of the range of potential causes and local epidemiologic patterns of causality are important for the optimization of diagnostic and therapeutic approaches.

Constrictive pericarditis following cardiac transplantation: a report of two cases and a literature review.

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.

[Symptomatic atrial fibrillation revealing chronic constrictive pericarditis in a young patient: A case report with a review of the literature]. / Fibrillation atriale symptomatique révélant une péricardite chronique constrictive chez un patient jeune : à propos d'un cas avec revue de la littérature.

Chronic Constrictive pericarditis (CCP) is a rare clinical entity that can pose diagnostic problems. Constrictive pericarditis is the final stage of a chronic inflammatory process characterized by fibrous thickening and calcification of the pericardium that impairs diastolic filling, reduces cardiac output, and ultimately leads to right heart failure and to atrial dilatation which can caused supravetricular arrythmia. Transthoracic echocardiography, computed tomography, and cardiac magnetic resonance imaging each can reveal severe diastolic dysfunction, increased pericardial thickness and calcifications. The gold standard for diagnosis is cardiac catheterization with analysis of intracavitary pressure curves, which are high and, in end diastole, equal in all chambers. CCP is the commun cause of recurrent heart failure. At present, idiopathic or viral pericarditis is the predominant cause of CP in the Western world, followed by postcardiotomy irritation and mediastinal irradiation. Tuberculosis is still a cause of pericarditis in developing countries and in immunosuppressed patient. We present a patient with symptomatic atrial fibrillation revealing chronic constrictive pericardis. He underwent to drug cardioversion before radical pericardiectomy and to date has made a good recovery without palpitations with a sinus rythm. The diagnosis of CP is often neglected by physicians, who usually attribute the symptoms to another disease process. This case show the difficulty in diagnosing, illustrated as well as the role of multimodality imaging and the excellent outcome of pericardiectomy for total recovery.

Sclerotherapy of oesophageal varices may induce chronic constrictive pericarditis in children.

Constrictive pericarditis is rare in children and can be difficult to diagnose. It has been described in adults after sclerotherapy of oesophageal varices but not in children. We report two cases of chronic constrictive pericarditis after sclerotherapy of oesophageal varices in children with portal cavernoma. Constrictive pericarditis should be considered as a cause of refractory ascites.