Pericardial Diseases: International Position Statement on New Concepts and Advances in Multimodality Cardiac Imaging.

Pericardial diseases have gained renewed clinical interest, leading to a renaissance in the field. There have been many recent advances in pericardial diseases in both multimodality cardiac imaging of diagnoses, such as recurrent, transient constrictive and effusive-constrictive pericarditis, and targeted therapeutics, especially anti-interleukin (IL)-1 agents that affect the inflammasome as part of autoinflammatory pathophysiology. There remains a large educational gap for clinicians, leading to variability in evaluation and management of these patients. The latest pericardial imaging (American Society of Echocardiography, European Association of Cardiovascular Imaging) and clinical guidelines (European Society of Cardiology) are >8-10 years of age and may not reflect current practice. Recent clinical trials involving anti-IL-1 agents in recurrent pericarditis, including anakinra (AIRTRIP), rilonacept (RHAPSODY), and goflikicept have demonstrated their efficacy. The present document represents an international position statement from world leaders in the pericardial field, focusing on novel concepts and emphasizing the role of multimodality cardiac imaging as well as new therapeutics in pericardial diseases.

Constrictive pericarditis in a patient with fiberglass lung disease: a case report.

BACKGROUND: Fiberglass has a larger aerodynamic diameter and is less likely to be inhaled into the lungs. Further, it will be cleared even if it is mechanically broken into smaller pieces and inhaled into the lungs. Fiberglass lung disease has been well documented if long term exposure but was thought reversible and would not cause severe diseases. The diagnosis of fiberglass lung disease depends on exposure history and histopathological findings. However, the exact occupational exposure history is often difficult to identify because mixed substance exposure often occurs and fiberglass disease is not as well-known as asbestosis. CASE PRESENTATION: A 66-year-old man had unexplained transudative pericardial effusion requiring pleural pericardial window operation twice at another medical center where asbestosis was told because of his self-reported long-term asbestosis exposure and the histopathological finding of a ferruginous body in his lung. Constrictive pericarditis developed two years later and resulted in congestive heart failure. Radical pericardiectomy combined with lung biopsy was performed following chest computed tomography imaging and the transudative nature of pericardial effusion not compatible with asbestosis. However, the histopathologic findings of his lung and pericardium at our hospital only showed chronic fibrosis without any asbestosis body. The patient's lung was found to be extremely fragile during a lung biopsy; histopathologic specimens were reviewed, and various fragments of fiberglass were found in the lung and pericardium. The patient's occupational exposure was carefully reevaluated, and he restated that he was only exposed to asbestosis for 1-2 years but was heavily exposed to fiberglass for more than 40 years. This misleading exposure history was mainly because he was only familiar with the dangers of asbestos. Since most fiberglass lung diseases are reversible and the symptoms of heart failure resolve soon after surgery, only observation was needed. Ten months after radical pericardiectomy, his symptoms, pleural effusion, and impaired pulmonary function eventually resolved. CONCLUSION: Fiberglass could cause inflammation of the pericardium, resulting in pericardial effusion and constrictive pericarditis, which could be severe and require radical pericardiectomy. Exact exposure history and histopathological examinations are the key to diagnosis.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome.

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is a rare autosomal recessive disease caused by mutation in proteoglycan 4 (PRG4) gene on chromosome 1q25-q31. We faced a dilemma and delay in diagnosis in two sisters. The elder sister had pericardial effusion with constrictive pericarditis, underwent pericardiectomy and received empirical treatment for suspected tuberculosis. After 2 years, she developed bilateral knee swelling with restriction of movement. At the same time, her younger sister also presented with bilateral knee swelling which aroused the suspicion of genetic disease. The whole-genome sequencing revealed homozygous PRG4 mutation suggestive of CACP syndrome.

Calcific pericarditis strangling the heart, an answer to unexplained heart failurediagnostic modalities.

Pericardial calcification is often found incidentally from imaging studies and may be a clue to constrictive pericarditis. Constrictive pericarditis often mimics other causes of heart failure, pulmonary, or liver disease, making it hard to diagnose. Tuberculosis is the most common infectious aetiology of Constrictive Pericarditis. Living in developing countries, such as Indonesia, should warn us of the possibility of tuberculous constrictive pericarditis as a differential diagnosis of unexplained heart failure. The presented case came with complaints of shortness of breath, especially on exertion for five years, which worsened in the last 6 months. The past history of pulmonary Tuberculosis with the Cardiac CT findings confirmed the diagnosis of Constrictive Pericarditis.

Pericardial Diseases and Best Practices for Pericardiectomy: JACC State-of-the-Art Review.

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.

Pericardial Calcification: An Uncommon Case with Intraventricular Extension.

An 80-year-old man presented to the cardiology outpatient clinic due to shortness of breath. His past medical history included alcohol intake, hypertension, inferior wall myocardial infarction (five years ago), an ischemic stroke, and permanent atrial fibrillation (diagnosed three years before the current examination). A physical exam revealed a decreased intensity of S1 and S2, irregular rate and rhythm, and no murmurs nor friction rub. X-rays, Computed Tomography, and echocardiography exhibited pericardial calcification, involving mostly the inferior wall and protruding into the left ventricle. A diagnosis of constrictive pericarditis due to pericardial calcification was established and considered idiopathic. Even when it may be related to ischemic heart disease, post-infarction pericarditis could explain how the calcification extended to adjacent territory perfused by the circumflex coronary artery. Combined imaging studies were crucial not only for identifying calcium deposits in the pericardium but also in assessing a patient inherently prone to co-existing and exacerbating conditions. Even though pericardiectomy allows for removal of the clinical manifestations of congestive pericarditis in the most symptomatic patients with pericardial calcification, among patients like ours, with tolerable symptoms, cardiologists should discuss the therapeutic options considering the patient's choices, potentially including a rehabilitation plan as part of non-pharmacological management.

Impact of pericardial calcification on early postoperative outcomes after pericardiectomy: a retrospective observational study.

BACKGROUND: Owing to the lack of understanding of the clinical significance of pericardial calcification during pericardiectomy, whether pericardial calcification should be considered when determining the optimal timing for pericardiectomy is debatable. We aimed to investigate the effect of pericardial calcification on early postoperative outcomes in patients who underwent pericardiectomy for constrictive pericarditis. METHODS: Altogether, 44 patients who underwent pericardiectomy for constrictive pericarditis were enrolled. After excluding three patients who underwent concurrent surgeries, a total of 41 patients were categorized into two groups based on the presence of pericardial calcification as determined by preoperative computed tomography and pathological examination. Preoperative clinical and imaging characteristics, intraoperative data, and early postoperative outcomes were compared between the two groups. A multivariable analysis was performed to identify the factors associated with postoperative complications. RESULTS: The group with and without PC comprised 21 and 20 patients, respectively. No significant differences were observed in 30-day mortality (n = 1 [5%]) in the group with pericardial calcification and no mortality in the group without pericardial calcification (p > 0.999). Other early postoperative outcome variables did not demonstrate any significant differences between the two groups. However, the use of cardiopulmonary bypass was associated with postoperative complications (p < 0.009, odds ratio: 63.5, 95% confidence interval: 5.13-3400). CONCLUSIONS: Pericardial calcification did not significantly affect the postoperative outcomes after pericardiectomy. Further comprehensive studies, including those with larger sample sizes and longitudinal designs, are necessary to determine whether pericardial calcification can significantly influence the timing of surgical intervention.

COVID-related constrictive pericarditis requiring pericardiectomy: a case report.

BACKGROUND: The COVID-19 pandemic was primarily considered a respiratory malady in the early phases of the outbreak. However, as more patients suffer from this illness, a myriad of symptoms emerge in organ systems separate from the lungs. Among those patients with cardiac involvement, myocarditis, pericarditis, myocardial infarction, and arrhythmia were among the most common manifestations. Pericarditis with pericardial effusion requiring medical or interventional treatments has been previously reported in the acute setting. Notably, chronic pericarditis with pericardial thickening resulting in constriction requiring sternotomy and pericardiectomy has not been published to date. CASE PRESENTATION: A patient with COVID-19-associated constrictive pericarditis three years after viral infection requiring pericardiectomy was reported. The COVID-19 infection originally manifested as anosmia and ageusia. Subsequently, the patient developed dyspnea, fatigue, right-sided chest pressure, bilateral leg edema, and abdominal fullness. Following recurrent right pleural effusions and a negative autoimmune work-up, the patient was referred for cardiothoracic surgery for pericardiectomy when radiographic imaging and hemodynamic assessment were consistent with constrictive pericarditis. Upon median sternotomy, the patient's pericardium was measured to be 8 mm thick. Descriptions of the clinical, diagnostic, and therapeutic features are provided. Within the first week after the operation, the patient's dyspnea resolved; one month later, leg edema and abdominal bloating were relieved. CONCLUSIONS: Although an association between COVID-19 and cardiac complications has been established, this case adds another element of virus severity and chronic manifestations. The need for sternotomy and pericardiectomy to treat COVID-19-related constrictive pericarditis is believed to be the first reported diagnosis.

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.

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.

Clinicopathological features of immunoglobulin G4-related constrictive pericarditis.

AIM: Constrictive pericarditis (CP) is characterised by scarring fibrosis and a loss of pericardial elasticity, which causes heart failure. IgG4 (immunoglobulin G4)-related disease (IgG4-RD) is a systemic fibro-inflammatory disease characterised by the infiltration of IgG4-immunopositive plasmacytes and high serum IgG4 levels that frequently shape tumorous lesions. Although pericardial involvement of IgG4-RD is rare, with indications of CP, pericardial effusion and irregular masses, the clinical and pathological features remain unclear. In this study, we examined the relationship between CP and IgG4-RD. METHODS: Among 35 thick-walled CP cases (histologically pericardial thickening ≥2 mm), eight cases were aetiology identified. Using the diagnostic criteria for IgG4-RD, 11 cases were classified as IgG4-CP, whereas the remainder were considered true idiopathic CP (16 cases) and the clinical pathological features were evaluated. RESULTS: Compared with the other groups, the IgG4-CP group was more common in men and associated with low-grade fever and massive pericardial effusion with frequent recurrence. Deaths resulting from heart failure occurred in a few cases of the IgG4-CP group, but not in other groups. An increase in C-reactive protein and a high positivity rate of anti-nuclear antibodies frequently occurred in the IgG4-CP group. Histologically, the IgG4-CP group included lymphoid follicle, eosinophil infiltration and few calcifications. CONCLUSIONS: Pericardial IgG4-RD occurs not only as nodular lesions, but also as thick-walled CP, and accounts for approximately 40% of thick-walled CP cases of unknown cause. The predominant clinical characteristic was refractory and recurrent pericardial effusion. Recognising IgG4-RD as a cause of CP is important to initiate appropriate therapy.

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.

Pericardial Disease in Cardiac Amyloidosis: A Comprehensive Review.

In patients with cardiac amyloidosis, pericardial involvement is common, with up to half of patients presenting with pericardial effusions. The pathophysiological mechanisms of pericardial pathology in cardiac amyloidosis include chronic elevations in right-sided filling pressures, myocardial and pericardial inflammation due to cytotoxic effects of amyloid deposits, and renal involvement with subsequent uremia and hypoalbuminemia. The pericardial effusions are typically small; however, several cases of life-threatening cardiac tamponade with hemorrhagic effusions have been described as a presenting clinical scenario. Constrictive pericarditis can also occur due to amyloidosis and its identification presents a clinical challenge in patients with cardiac amyloidosis who concurrently manifest signs of restrictive cardiomyopathy. Multimodality imaging, including echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, is useful in the evaluation and management of this patient population. The recognition of pericardial effusion is important in the risk stratification of patients with cardiac amyloidosis as its presence confers a poor prognosis. However, specific treatment aimed at the effusions themselves is seldom indicated. Cardiac tamponade and constrictive pericarditis may necessitate pericardiocentesis and pericardiectomy, respectively.

Outcomes and Risk Factors Associated with Pericardiectomy in Patients with Constrictive Pericarditis: A Retrospective Study from China.

PURPOSE: Pericardiectomy is the definitive treatment option for constrictive pericarditis and is associated with a high prevalence of morbidity and mortality. However, information on the associated outcomes and risk factors is limited. We aimed to report the mid-term outcomes of pericardiectomy from a single center in China. METHODS: We retrospectively reviewed data collected from patients who underwent pericardiectomy at our institute from April 2018 to January 2023. RESULTS: Eighty-six consecutive patients (average age, 46.1 ± 14.7 years; 68.6 men) underwent pericardiectomy through midline sternotomy. The most common etiology was idiopathic (n = 60, 69.8%), and 82 patients (95.3%) were in the New York Heart Association function class III/IV. In all, 32 (37.2%) patients underwent redo sternotomies, 36 (41.9%) underwent a concomitant procedure, and 39 (45.3%) required cardiopulmonary bypass. The 30-day mortality rate was 5.8%, and the 1-year and 5-year survival rates were 88.3% and 83.5%, respectively. Multivariable analysis revealed that preoperative mitral insufficiency (MI) ≥moderate (hazard ratio [HR], 6.435; 95% confidence interval [CI] [1.655-25.009]; p = 0.007) and partial pericardiectomy (HR, 11.410; 95% CI [3.052-42.663]; p = 0.000) were associated with increased 5-year mortality. CONCLUSION: Pericardiectomy remains a safe operation for constrictive pericarditis with optimal mid-term outcomes.

[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.