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.

Pericardial Involvement in Sarcoidosis.

Pericardial disease secondary to sarcoidosis is a rare clinical entity with no observational studies in previous research. Therefore, we evaluated reported cases of pericarditis because of sarcoidosis to further understand its diagnosis and management. We performed a systematic review of previous research until December 16, 2020 in MEDLINE, Embase, Scopus, Cochrane Central Register of Controlled Trials, and Web of Science. Case reports and case series demonstrating pericardial involvement in sarcoidosis were included. Fourteen reports with a total of 27 patients were identified. Dyspnea (82%) was the most common presentation, with the lungs being the primary site of sarcoidosis in most patients (77%). The most frequently encountered pericardial manifestations were pericardial effusion (89%), constrictive pericarditis and cardiac tamponade (48%). Management of these patients included use of corticosteroids (82%), colchicine (11%), and nonsteroidal anti-inflammatory agents (7%). Similar to the general population, the most common intervention in these patients was pericardiocentesis (59%), pericardial window (30%), and pericardiectomy (19%). Overall, the majority of this population (70%) achieved clinical improvement during median follow-up time of 8 months. In conclusion, the prevalence and incidence of sarcoid-induced pericarditial disease remain unclear. Clinical manifestations of pericardial involvement are variable, though many patients present with asymptomatic pericardial effusions. No consensus exists on the treatment of this special population, but corticosteroids and combination therapies are considered first-line therapies because of their efficacy in suppressing pericardial inflammation and underlying sarcoidosis. Patients with refractory cases of pericarditis may also benefit therapeutically from the addition of nonsteroidal anti-inflammatory agents, colchicine, and/or biologics.

Constrictive Pericarditis after Open Heart Surgery: A 20-Year Case Controlled Study.

BACKGROUND: Constrictive pericarditis is a rare complication of open heart surgery (OHS), but little is known regarding the etiologic determinants, and prognostic factors. The purpose of this study was to investigate clinical predictors and long term prognosis of post-operative constrictive pericarditis (CP). METHODS: Using the Myocardial Infarction Data Acquisition System database, we analyzed records of 142,837 patients who were admitted for OHS in New Jersey hospitals between 1995 and 2015. Ninety-one patients were hospitalized with CP 30 days or longer after discharge from OHS. Differences in proportions were analyzed using Chi square tests. Controls were matched to cases for demographics, surgical procedure type, history of OHS, and propensity score. Cox proportional hazard models were used to evaluate the risk of all-cause death. Log-rank tests and Cox models were used to assess differences in the Kaplan-Meier survival curves with and without adjustments for comorbidities. RESULTS: Patients with CP were more likely to have history of valve disease (VD, p < 0.001), atrial fibrillation (AF, p = 0.024) renal disease (CKD, p = 0.028), hemodialysis (HD, p = 0.008), previous OHS (p < 0.001). Patients with CP compared to matched controls had a higher 7-year mortality (p < 0.001). This difference became statistically significant at 1-year after surgery. CONCLUSION: CP is a rare complication of OHS that occurs more frequently in patients with VD, AF, CKD, HD, multiple OHS, and it is associated with an unfavorable long-term prognosis. Given the large number of OHS performed every year, the results highlight the need for clinicians to recognize and properly manage this complication of OHS.

Pericarditis in Systemic Rheumatologic Diseases.

PURPOSE OF REVIEW: We review the epidemiology, pathophysiology, and management of pericarditis most commonly complicating autoimmune and autoinflammatory conditions. RECENT FINDINGS: Typically, pericarditis occurs in the context of a systemic flare of the underlying disease but infrequently, it is the presenting manifestation requiring a high index of suspicion to unravel the indolent cause. Pericardial involvement in rheumatic diseases encompasses a clinical spectrum to include acute, recurrent and incessant pericarditis, constrictive pericarditis, asymptomatic pericardial effusion, and pericardial tamponade. Direct evidence on the pathophysiology of pericarditis in the context of rheumatic diseases is scant. It is theorized that immune perturbations within pericardial tissue result from the underlying central immunopathology of the respective autoimmune or autoinflammatory disease. Pericarditis management depends on acuity, the underlying cause and epidemiological features such as patient's immune status and geographic prevalence of infections such as tuberculosis. Immunosuppressive medications including biologics such as interleukin 1 blockers emerge as possible steroid sparing agents for pericarditis treatment.

Effect of Anti-Inflammatory Drugs on Clinical Outcomes in Patients With Malignant Pericardial Effusion.

BACKGROUND: Pericardiocentesis (PCC) with extended catheter drainage has become a relatively safe procedure to control pericardial effusion (PE), but little is known about long-term outcomes after PCC in malignant PE. OBJECTIVES: This study evaluated the effects of anti-inflammatory agents on long-term outcomes after effective drainage of PE in active cancer patients. METHODS: From May 2007 to December 2018, 445 patients with malignant PE who underwent echocardiography-guided PCC were enrolled. Clinical, laboratory, echocardiographic and procedural findings, and clinical outcome data were collected. Use of anti-inflammatory agents including colchicine, nonsteroidal anti-inflammatory drugs, or steroids after PCC was also analyzed. Colchicine was administered in a dose of 0.6 mg orally, twice a day for 2 months. The primary outcome was defined as a composite of all-cause death and re-PCC or pericardial window operation due to recurred PE. RESULTS: The procedure was successful in 97.0% of the cases, with 1 procedure-related death. During the follow-up of 2 years, 26.1% of patients developed recurrent PE, and 46.0% developed constrictive pericarditis. The colchicine treatment group showed a significantly lower risk of composite events (adjusted hazard ratio [aHR]: 0.65; 95% confidence interval [CI]: 0.49 to 0.87; p = 0.003) as well as all-cause death (aHR: 0.60; 95% CI: 0.45 to 0.81; p = 0.001) than did the noncolchicine group. On propensity score matching, colchicine after PCC was consistently associated with a lower composite events (aHR: 0.55; 95% CI: 0.37 to 0.82; p = 0.003). CONCLUSIONS: In cancer patients with malignant PE, PCC with extended drainage can be an appropriate therapeutic option and shows low complication rate. Patients receiving colchicine after successful PCC showed significant improvement in clinical outcome.

Pericardial diseases in patients with cancer: contemporary prevalence, management and outcomes.

Neoplastic pericardial effusion is a common and serious manifestation of advanced malignancies. Lung and breast carcinoma, haematological malignancies, and gastrointestinal cancer are the most common types of cancer involving the pericardium. Pericardial involvement in neoplasia may arise from several different pathophysiological mechanisms and may be manifested by pericardial effusion with or without tamponade, effusive-constrictive pericarditis and constrictive pericarditis. Management of these patients is a complex multidisciplinary problem, affected by clinical status and prognosis of patients.

US National Trends in the Management and Outcomes of Constrictive Pericarditis: 2005-2014.

BACKGROUND: Patient characteristics, trends in the management strategy, and outcomes of patients with constrictive pericarditis have not been characterized at the national scale. METHODS: Annual trends of patients admitted to hospitals in the United States with constrictive pericarditis were evaluated using the National Inpatient Sample dataset between 2005 and 2014. Poisson regression models adjusting for the US census population estimate were fitted to evaluate trends in the incidence of constrictive pericarditis, isolated pericardiectomy, and cardiopulmonary bypass (CPB) use. Descriptive analyses were performed to compare patient characteristics and in-hospital mortality rates between surgically and medically managed cohorts. RESULTS: During 2005-2014, 29,487 patients were admitted with constrictive pericarditis. Sixteen percent underwent isolated pericardiectomy. The prevalence of constrictive pericarditis remained stable between 2005 and 2014 at 9-10 cases per million, but proportion of patients undergoing isolated pericardiectomy decreased from 18% in 2005 to 15% in 2014 (P = 0.001 for trend). CPB use increased from 15% to 29% (P < 0.001). Compared with medically managed patients, the pericardiectomy cohort was younger (age 57 vs 61 years, P < 0.001), less likely to be female (25% vs 41%, P < 0.001), and harboured fewer comorbidities. In-hospital mortality was 7.3% for those undergoing pericardiectomy and 6.8% for a medically managed cohort (P = 0.58) and operative mortality was stable across years (P = 0.99 for trend). CONCLUSIONS: The prevalence of constrictive pericarditis remained stable between 2005 and 2014 at 9-10 cases per million. Surgical management was infrequent, with younger and less comorbid patients being more likely to be managed operatively. Increasing use of CPB without a change in operative mortality highlights the persisting challenge of this complex disease.

Epidemiology of pericardial diseases in Africa: a systematic scoping review.

OBJECTIVES: This scoping review sought to summarise available data on the prevalence, aetiology, diagnosis, treatment and outcome of pericardial disease in Africa. METHODS: We searched PubMed, Scopus and African Journals Online from 1 January 1967 to 30 July 2017 to identify all studies published on the prevalence, aetiologies, diagnosis, treatment and outcomes of pericardial diseases in adults residing in Africa. RESULTS: 36 studies were included. The prevalence of pericardial diseases varies widely according to the population of interest: about 1.1% among people with cardiac complaints, between 3.3% and 6.8% among two large cohorts of patients with heart failure and up to 46.5% in an HIV-infected population with cardiac symptoms. Tuberculosis is the most frequent cause of pericardial diseases in both HIV-uninfected and HIV-infected populations. Patients with tuberculous pericarditis present mostly with effusive pericarditis (79.5%), effusive constrictive pericarditis (15.1%) and myopericarditis (13%); a large proportion of them (up to 20%) present in cardiac tamponade. The aetiological diagnosis of pericardial diseases is challenging in African resource-limited settings, especially for tuberculous pericarditis for which the diagnosis is not definite in many cases. The outcome of these diseases remains poor, with mortality rates between 18% and 25% despite seemingly appropriate treatment approaches. Mortality is highest among patients with tuberculous pericarditis especially those coinfected with HIV. CONCLUSION: Pericardial diseases are a significant cause of morbidity and mortality in Africa, especially in HIV-infected individuals. Tuberculosis is the most frequent cause of pericardial diseases, and it is associated with poor outcomes.

Effusive-Constrictive Pericarditis After Pericardiocentesis: Incidence, Associated Findings, and Natural History.

OBJECTIVES: This study sought to investigate the incidence, associated findings, and natural history of effusive-constrictive pericarditis (ECP) after pericardiocentesis. BACKGROUND: ECP is characterized by the coexistence of tense pericardial effusion and constriction of the heart by the visceral pericardium. Echocardiography is currently the main diagnostic tool in the assessment of pericardial disease, but limited data have been published on the incidence and prognosis of ECP diagnosed by echo-Doppler. METHODS: A total of 205 consecutive patients undergoing pericardiocentesis at Mayo Clinic, Rochester, Minnesota, were divided into 2 groups (ECP and non-ECP) based on the presence or absence of post-centesis echocardiographic findings of constrictive pericarditis. Clinical, laboratory, and imaging characteristics were compared. RESULTS: ECP was subsequently diagnosed in 33 patients (16%) after pericardiocentesis. Overt clinical cardiac tamponade was present in 52% of ECP patients and 36% of non-ECP patients (p = 0.08). Post-procedure hemopericardium was more frequent in the ECP group (33% vs. 13%; p = 0.003), and a higher percentage of neutrophils and lower percentage of monocytes were noted on pericardial fluid analysis in those patients. Clinical and laboratory findings were otherwise similar. Baseline early diastolic mitral septal annular velocity was significantly higher in the ECP group. Before pericardiocentesis, respiratory variation of mitral inflow velocity, expiratory diastolic flow reversal of hepatic vein, and respirophasic septal shift were significantly more frequent in the ECP group. Fibrinous or loculated effusions were also more frequently observed in the ECP group. Four deaths occurred in the ECP group; all 4 patients had known malignancies. During median follow-up of 3.8 years (interquartile range: 0.5 to 8.3 years), only 2 patients required pericardiectomy for persistent constrictive features and symptoms. CONCLUSIONS: In a large cohort of unselected patients undergoing pericardiocentesis, 16% were found to have ECP. Pre-centesis echocardiographic findings might identify such patients. Long-term prognosis in those patients remains good, and pericardiectomy was rarely required.

Uremic pericarditis, pericardial effusion, and constrictive pericarditis in end-stage renal disease: Insights and pathophysiology.

A rising prevalence of end-stage renal disease (ESRD) has led to a rise in ESRD-related pericardial syndromes, calling for a better understanding of its pathophysiology, diagnoses, and management. Uremic pericarditis, the most common manifestation of uremic pericardial disease, is a contemporary problem that calls for intensive hemodialysis, anti-inflammatories, and often, drainage of large inflammatory pericardial effusions. Likewise, asymptomatic pericardial effusions can become large and impact the hemodynamics of patients on chronic hemodialysis. Constrictive pericarditis is also well documented in this population, ultimately resulting in pericardiectomy for definitive treatment. The management of pericardial diseases in ESRD patients involves internists, cardiologists, and nephrologists. Current guidelines lack clarity with respect to the management of pericardial processes in the ESRD population. Our review aims to describe the etiology, classification, clinical manifestations, diagnostic imaging tools, and treatment options of pericardial diseases in this population.

A prospective investigation into the effect of colchicine on tuberculous pericarditis.

INTRODUCTION: Tuberculous (TB) pericarditis carries significant mortality and morbidity rates, not only during the primary infection, but also as part of the granulomatous scar-forming fibrocalcific constrictive pericarditis so commonly associated with this disease. Numerous therapies have previously been investigated as adjuvant strategies in the prevention of pericardial constriction. Colchicine is well described in the treatment of various aetiologies of pericarditis. The aim of this research was to investigate the merit for the use of colchicine in the management of tuberculous pericarditis, specifically to prevent constrictive pericarditis. METHODS: This pilot study was designed as a prospective, double-blinded, randomised, control cohort study and was conducted at a secondary level hospital in the Northern Cape of South Africa between August 2013 and December 2015. Patients with a probable or definite diagnosis of TB pericarditis were included (n = 33). Study participants with pericardial effusions amenable to pericardiocentesis underwent aspiration until dryness. All patients were treated with standard TB treatment and corticosteroids in accordance with the South African Tuberculosis Treatment Guidelines. Patients were randomised to an intervention and control group using a web-based computer system that ensured assignment concealment. The intervention group received colchicine 1.0 mg per day for six weeks and the control group received a placebo for the same period. Patients were followed up with serial echocardiography for 16 weeks. The primary outcome assessed was the development of pericardial constriction. Upon completion of the research period, the blinding was unveiled and data were presented for statistical analysis. RESULTS: TB pericarditis was found exclusively in HIV-positive individuals. The incidence of pericardial constriction in our cohort was 23.8%. No demonstrable benefit with the use of colchicine was found in terms of prevention of pericardial constriction (p = 0.88, relative risk 1.07, 95% CI: 0.46-2.46). Interestingly, pericardiocentesis appeared to decrease the incidence of pericardial constriction. CONCLUSION: Based on this research, the use of colchicine in TB pericarditis cannot be advised. Adjuvant therapy in the prevention of pericardial constriction is still being investigated and routine pericardiocentesis may prove to be beneficial in this regard.

An approach to the patient with suspected pericardial disease.

Diseases of the pericardium commonly manifest in one of three ways: acute pericarditis, pericardial effusion and constrictive pericarditis. In the developed world, the most common cause of acute pericarditis is viral or idiopathic disease, while in the developing world tuberculous aetiology, particularly in sub-Saharan Africa, is commonplace owing to the high prevalence of HIV. This article provides an approach to the diagnosis, investigation and management of these patients.

Pericarditis constrictiva: espectro etiológico, presentaciones clínicas, factores pronósticos y seguimiento a largo plazo / Constrictive pericarditis: etiologic spectrum, patterns of clinical presentation, prognostic factors, and long-term follow-up

Introducción y objetivos: Algunos estudios han descrito un cambio en el espectro etiológico de la pericarditis constrictiva. Además, no hay datos sobre la relación entre la forma de presentación clínica y la etiología. El objetivo de este estudio es describir las etiologías de la enfermedad, su relación con la forma de presentación clínica y los hallazgos quirúrgicos, así como identificar los factores predictivos de una mala evolución. Métodos: Se analizó a un total de 140 pacientes consecutivos a los que se practicaron intervenciones quirúrgicas por pericarditis constrictiva en un mismo centro en un periodo de 34 años. Resultados: La etiología fue idiopática en 76 pacientes (54%) y tras pericarditis aguda idiopática en 24 (17%), pericarditis tuberculosa en 15 (11%), pericarditis purulenta en 10 (7%) y cirugía cardiaca en 5 (4%), radioterapia en 3 (2%) y uremia en 2 (1%). La duración media de los síntomas antes de la pericardiectomía fue de 19 meses (desviación estándar: 44 meses); la forma de presentación clínica más aguda fue la de las pericarditis purulentas (26 [intervalo, 7-60] días) y la más crónica, la de los casos idiopáticos (29 meses [4 días-360 meses]). La mortalidad perioperatoria fue del 11%. No hubo diferencias en la mortalidad según etiologías. La mediana de seguimiento fue de 12 (0,1-33,0) años, durante los cuales fallecieron 50 pacientes. En un análisis de regresión de Cox, la edad en el momento de la operación, la clase funcional de la New York Heart Association avanzada (III–IV) y los antecedentes de pericarditis aguda idiopática se asociaron a una mayor mortalidad durante el seguimiento. Conclusiones: La mayoría de los casos de pericarditis constrictiva son idiopáticas. La cirugía cardiaca y la radioterapia causan una minoría de los casos. Las presentaciones aguda y subaguda merecen un estudio etiológico. La edad, la clase funcional avanzada y la pericarditis aguda idiopática previa se asocian a mayor mortalidad (AU)

Introduction and objectives: Some reports have described a change in the etiologic spectrum of constrictive pericarditis. In addition, data on the relationship between its clinical presentation and etiology are lacking. We sought to describe the etiologies of the disease, their relationship with its clinical presentation and surgical findings, and to identify predictors of poor outcome. Methods: We analyzed 140 consecutive patients who underwent surgery for constrictive pericarditis over a 34-year period in a single center. Results: The etiology was idiopathic in 76 patients (54%), acute idiopathic pericarditis in 24 patients (17%), tuberculous pericarditis in 15 patients (11%), purulent pericarditis in 10 patients (7%), and cardiac surgery, radiation and uremia in 5, 3 and 2 patients respectively (4%, 2% and 1%).Mean duration of symptoms before pericardiectomy was 19 months (standard deviation=44 months), the most acute presentation being for purulent pericarditis (26 days [range, 7-60 days]) and the most chronic for idiopathic cases (29 months [range, 4 days-360 months]). Perioperative mortality was 11%. There was no difference in mortality between etiologies. Median follow-up was 12 years (range, 0.1-33.0 years) in which 50 patients died. In a Cox-regressionanalysis, age at surgery, advanced New York HeartAssociation functional class (IIIto IV) and previous acute idiopathic pericarditis were associated with increased mortality during follow-up. Conclusions: Most cases of constrictive pericarditis are idiopathic. Cardiac surgery and radiation accounted for a minority of cases. Etiologic investigations are warranted only in acute or subacute presentations. Age, advanced functional class, and previous acute idiopathic pericarditis are associated with increased mortality (AU)

Long-term outcomes of pericardiectomy for constrictive pericarditis.

BACKGROUND: Constrictive pericarditis is a rare and disabling disease that can result in chronic fibrous thickening of the pericardium. The purpose of this study was to evaluate the long-term outcomes following treatment of constrictive pericarditis by pericardiectomy. METHODS: Between September 1992 and May 2014, 47 patients who underwent pericardiectomy for constrictive pericarditis were retrospectively examined. Demographic, pre-, intra- and postoperative data and long-term outcomes were analyzed. RESULTS: Thirty of the patients were male, the mean age was 45.8 ± 16.7. Aetiology of constrictive pericarditis was tuberculosis in 22 (46.8 %) patients, idiopathic in 15 (31.9 %), malignancy in 3 (6.4 %), prior cardiac surgery in 2 (4.3 %), non-tuberculosis bacterial infections in 2 (4.3 %), radiotherapy in 1 (2.1 %), uraemia in 1 (2.1 %) and post-traumatic in 1 (2.1 %). The surgical approach was achieved via a median sternotomy in all patients except only 1 patient. The mean operative time was 156.4 ± 45.7 min. Improvement in functional status in 80 % of patients' at least one New York Heart Association (NYHA) functional class was observed. In-hospital mortality rate was 2.1 % (1 of 47 patients). The cause of death was pneumonia leading to progressive respiratory failure. The late mortality rate was 23.4 % (11 of 47 patients). The mean follow-up time was 61.2 ± 66 months. The actuarial survival rates were 91 %, 85 % and 81 % at 1, 5 and 10 years, respectively. Recurrence requiring a repeat pericardiectomy was developed in no patient during follow-up. CONCLUSION: Pericardiectomy is associated with high morbidity and mortality rates. Cases with neoplastic diseases, diminished cardiac output, cases in need of reoperation are expected to have high mortality rates and less chance of functional recovery.

Los síndromes pericárdicos agudos en la unidad de cuidados intensivos / Acute pericardial syndromes in the intensive care unit

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Prevalence, hemodynamics, and cytokine profile of effusive-constrictive pericarditis in patients with tuberculous pericardial effusion.

BACKGROUND: Effusive constrictive pericarditis (ECP) is visceral constriction in conjunction with compressive pericardial effusion. The prevalence of proven tuberculous ECP is unknown. Whilst ECP is distinguished from effusive disease on hemodynamic grounds, it is unknown whether effusive-constrictive physiology has a distinct cytokine profile. We conducted a prospective study of prevalence and cytokine profile of effusive-constrictive disease in patients with tuberculous pericardial effusion. METHODS: From July 2006 through July 2009, the prevalence of ECP and serum and pericardial levels of inflammatory cytokines were determined in adults with tuberculous pericardial effusion. The diagnosis of ECP was made by combined pericardiocentesis and cardiac catheterization. RESULTS: Of 91 patients evaluated, 68 had tuberculous pericarditis. The 36/68 patients (52.9%; 95% confidence interval [CI]: 41.2-65.4) with ECP were younger (29 versus 37 years, P=0.02), had a higher pre-pericardiocentesis right atrial pressure (17.0 versus 10.0 mmHg, P<0.0001), serum concentration of interleukin-10 (IL-10) (38.5 versus 0.2 pg/ml, P<0.001) and transforming growth factor-beta (121.5 versus 29.1 pg/ml, P=0.02), pericardial concentration of IL-10 (84.7 versus 20.4 pg/ml, P=0.006) and interferon-gamma (2,568.0 versus 906.6 pg/ml, P=0.03) than effusive non-constrictive cases. In multivariable regression analysis, right atrial pressure > 15 mmHg (odds ratio [OR] = 48, 95%CI: 8.7-265; P<0.0001) and IL-10 > 200 pg/ml (OR=10, 95%CI: 1.1, 93; P=0.04) were independently associated with ECP. CONCLUSION: Effusive-constrictive disease occurs in half of cases of tuberculous pericardial effusion, and is characterized by greater elevation in the pre-pericardiocentesis right atrial pressure and pericardial and serum IL-10 levels compared to patients with effusive non-constrictive tuberculous pericarditis.