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Pediatric idiopathic pericardial effusions are common and often have a prolonged clinical course. We hypothesized that these effusions have no standardized diagnostic workup, and ultimately have a final etiology not initially appreciated. To test these hypotheses, a hospital system-wide retrospective (1/1/1990–10/1/2019) cohort study of pericardial effusions in children (< 18 years) was conducted. Effusions were grouped by etiology and patients receiving an initial idiopathic diagnosis were further analyzed. Effusion size, diagnostic workup, final diagnosis, and time to resolution were abstracted. In total, 42/366 effusions were initially diagnosed as idiopathic. Workup was not standardized and included up to six laboratory tests including pericardial fluid analysis and infectious, metabolic, rheumatologic and thyroid workups. Treatment course involved 1 readmission in 24%, and > 1 readmission in 12%. Resolution of effusion occurred in 83% of patients within a median of 1 admission (range 1–4). Of those effusions initially deemed idiopathic, 12/42 (29%) were later found to have an underlying etiologic diagnosis including: autoimmune (7, 58%), neoplastic (2, 16%), infectious (2, 16%), and renal (1, 8%). Children initially diagnosed with idiopathic pericardial effusions have an underlying etiologic diagnosis 29% of the time, and a standardized workup may prevent delays in definitive diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Purpose of Review: We aim to give a concise overview of the different clinical manifestations of both acute and long-term radiotherapy-related pericardial diseases, the underlying pathophysiology as well as the diagnosis and treatment options. Recent Findings: Radiotherapy-related pericardial disease is common, but despite radiotherapy being a cornerstone of many cancer treatments, this disease entity is relatively underrepresented in clinical trials, resulting in a paucity of research data on pathophysiology and management. Summary: Since the development of innovative cancer treatments, survival has significantly improved. Therefore, the importance of long-term treatment-related side effects increases, most notably cancer treatment-related cardiovascular toxicity. In patients undergoing radiotherapy as a part of their cancer treatment, radiotherapy-related pericardial disease can manifest early (during or shortly after radiotherapy administration) or very late (several years to decades after treatment). This exceptionally long latency period confronts physicians with treatment-related side effects of radiotherapy regimens that may have been abandoned already. [ABSTRACT FROM AUTHOR]

Purpose of the Review: We review the pathophysiology, diagnosis, and contemporary treatment for recurrent pericarditis, with focus on interleukin-1 (IL-1) inhibitors. Recent Findings: Recurrent pericarditis occurs in about 15–30% of patients who have acute pericarditis. With increased understanding of the autoinflammatory pathophysiology of recurrent pericarditis, IL-1 inhibitors including anakinra, canakinumab, and rilonacept have been applied to this condition with great promise. In particular, the RHAPSODY trial found rilonacept significantly improves pain and inflammation, while also reducing recurrence with few adverse events. The next IL-1 inhibitor on the block for pericarditis, goflikicept, is also discussed. Summary: Understanding the role of the inflammasome via the autoinflammatory pathway in pericarditis has led to incorporation of IL-1 inhibitors in the treatment of recurrent pericarditis, with proven efficacy and safety and randomized trials. This will lead to increase uptake of this agent which demonstrated lower rates of recurrence and faster time to resolution. [ABSTRACT FROM AUTHOR]

Purpose of Review: Immune checkpoint inhibitors have reshaped the treatment of cancer, but they are characterized by peculiar toxicity consisting of immune-related adverse events that may potentially affect any organ or system. In this review, we summarize data on clinical presentation, diagnosis, pathogenesis, and management of the main immune-related cardiovascular toxicities of immune checkpoint inhibitors. Recent Findings: The most relevant immune-related cardiovascular toxicity is myocarditis, but other non-negligible reported events include non-inflammatory heart failure, conduction abnormalities, pericardial disease, and vasculitis. More recently, growing evidence suggests a role for immune checkpoint inhibitors in accelerating atherosclerosis and promoting plaque inflammation, thus leading to myocardial infarction. Summary: Immune checkpoint inhibitors are associated with several forms of cardiovascular toxicity; thus, an accurate cardiovascular baseline evaluation and periodical monitoring are required. Furthermore, the optimization of cardiovascular risk factors before, during, and after treatment may contribute to mitigating both short-term and long-term cardiovascular toxicity of these drugs. [ABSTRACT FROM AUTHOR]

Purpose of Review: While there have now been a variety of large reviews on adult pericarditis, this detailed review specifically focuses on the epidemiology, clinical presentation, diagnosis, and management of pediatric pericarditis. We have tried to highlight most pediatric studies conducted on this topic, with special inclusion of important adult studies that have shaped our understanding of and management for acute and recurrent pericarditis. Recent Findings: We find that the etiology of pediatric pericarditis differs from adult patients with pericarditis and has evolved over the years. Also, with the current COVID-19 pandemic, it is important for pediatric clinicians to be aware of pericardial involvement both due to the infection and from vaccination. Oftentimes, pericarditis maybe the only cardiac involvement in children with COVID-19, and so caregivers should maintain a high index of suspicion when they encounter children with pericarditis. Summary: Large-scale contemporary epidemiological data regarding incidence and prevalence of both acute and recurrent pericarditis is lacking in pediatrics, and future studies should focus on highlighting this important research gap. Most of the current management strategies for pediatric pericarditis are from experiences gathered from adult data. Pediatric multicenter trials are warranted to understand the best management strategy for those with acute and recurrent pericarditis. Case Vignette: A 6-year-old child with a past history of pericarditis almost 2 months ago comes in with a 2-day history of chest pain and fever. Per mother, he stopped his steroids about 2 weeks ago, and for the last 2 days has had a temperature of 102F and has been complaining of sharp mid-sternal chest pain that gets worse when he lies down and is relieved when he sits up and leans forward. On examination, he is tachycardic (heart rate 160 bpm), with normal blood pressure for age. He appears to be in pain (5/10), and on auscultation has a pericardial friction rub. His lab studies are notable for elevated white blood cell count and inflammatory markers (CRP and ESR). His electrocardiogram reveals sinus tachycardia and diffuse ST-elevation in all precordial leads. His echocardiogram demonstrates normal biventricular function and a trace pericardial effusion. His cardiac MRI confirms recurrent pericarditis. He is started on indomethacin and colchicine. He has complete resolution of his symptoms by day 3 of admission and is discharged with close follow-up. [ABSTRACT FROM AUTHOR]

Purpose of Review: Coronavirus disease 2019 (COVID19) involves the heart, including pericardium. This article reviews the possible pathophysiological mechanisms in pericardial involvement in COVID19 and pericardial manifestations of COVID19. It also summarizes the patients with pericarditis secondary to COVID19 and outlines the contemporary treatment strategies in this patient population. Recent Findings: A high degree of suspicion is required to identify the pericardial involvement in COVID19 patients. It is proposed that an underlying hyperinflammatory reaction in COVID19 leads to pericardial inflammation. Acute pericarditis with or without myocardial involvement is diagnosed on clinical presentation, serum inflammatory markers, electrocardiogram, and echocardiogram. Multimodality imaging may also have an additional diagnostic value. Patients are usually managed medically, but some patients develop a life-threatening pericardial tamponade necessitating pericardial drainage. Summary: Pericardial involvement is an important clinical manifestation of COVID19 requiring a proper workup. Timely diagnosis and a specific management plan based on the presentation and concomitant organ involvement usually lead to a complete recovery. [ABSTRACT FROM AUTHOR]

The clinical spectrum of pericardial effusions varies from innocuous serous fluid to life-threatening hemopericardium. A misdiagnosis may be made by similar clinical presentation of acute chest pain/hypotension. Echocardiography is the first-line test for diagnosis of pericardial effusion and its etiology, but sometimes there are different drawbacks to the correct cardiovascular ultrasound diagnosis. Radiologists are reporting an increasing amount of thoracic Multidetector CT examinations at the emergency department. Multidetector CT has now become an established and complementary method for cardiac imaging, and diseases of the pericardium can now be quickly identified with increasing certainty. The aim of this review is to discuss the hemopericardium key Multidetector CT features in acute clinical setting which indicate the need to proceed with predominantly medical or surgical treatment, however, being able to identify forms of bleeding pericardial effusion for which only "a watch and wait strategy" and/or deferred treatment is indicated. In the emergency care setting, radiologists must be aware of different findings of hemopericardium in order to address a tailored and timely management approach. [ABSTRACT FROM AUTHOR]

Purpose of review: To discuss invasive cardiovascular procedures as they relate to onco-cardiology, as well as recent additions to anti-cancer therapies and their subsequent effect on cardiovascular toxicity. Recent findings: The development of immune checkpoint inhibitors and chimeric antigen receptor T cell therapy has been linked to cardiotoxicity and represents an emerging area of concern. Recent advances in transcatheter valve replacement have shown benefits compared with surgical management regardless of malignancy type, stage, or treatment. Summary: With the increasing use of immunotherapy and increasing recognition of cardiotoxicity, there is a need for identifying mortality-improving strategies. The use of a transcatheter approach for aortic valve replacement looks to be a safer alternative when compared with surgical replacement despite the presence of cancer. Pericardial disease is frequent in the cancer population and pericardiocentesis represents a valid option for the treatment of significant pericardial effusions. Endomyocardial biopsy is performed for various indications in the cancer population and is the gold standard for diagnosing myocarditis and infiltrative diseases. [ABSTRACT FROM AUTHOR]

Pericardial diseases have changed their epidemiology in the past few years. With the aging population and decreasing incidence of communicable diseases, the causes of pericardial diseases have significantly changed from infectious and malignant to postradiation and cardiac surgery causes. Despite that, pericardial diseases remain difficult to diagnose. The accurate and timely diagnosis of these diseases is essential to avoid the late sequela of pericardial constriction and pericardial cirrhosis. Echocardiography remains the first test of choice for the assessment of patients with suspected pericardial diseases. Most patients with acute pericarditis have a self-limiting course and do not need further imaging. However, in the era of multimodality imaging, other modalities, namely, computed tomography (CT) and magnetic resonance imaging (CMR), are often utilized in complex cases. These two modalities provide a wide-open view of the pericardium and adjacent structures. They have high resolution to assess pericardial calcification, a hallmark of many diseases especially tuberculous constrictive pericarditis. CMR is also unique in its ability to assess pericardial late gadolinium enhancement (LGE) and edema. These have been recently suggested to be very important in the progression from acute pericarditis to constrictive pericarditis. In addition, they provide prognostic value to assess which patients are at high risk of developing heart failure and resource utilization. Thus, in the current era, patients with suspected complex pericardial diseases will need a multimodality approach rather than a single modality approach. [ABSTRACT FROM AUTHOR]

An insidious progression of cardiovascular (CV) involvement is generally associated with rheumatologic diseases and finally regarded as a major source of morbidity and mortality in Juvenile idiopathic arthritis (JIA) patients. JIA could involve all of the cardiac structures, including pericardium, myocardium, endocardium; coronary vessels; valves and conduction system. Development of pericarditis, myocarditis, endocarditis and ventricular dysfunction are not unexpected issues in the progress of JIA. It is essential to ensure a comprehensive follow-up with advanced and up-to-date diagnostic and therapeutic modalities for prevention of CV complications in JIA patients. Since these are all associated with an unfavorable prognosis, it is necessary to detect subclinical cardiac involvement in CV asymptomatic patients, in order to start adequate management and treatment. Furthermore, controlling chronic inflammatory state of JIA by new treatment modalities will also significantly reduce the overall morbidity and mortality related to CV diseases. In this review, we aimed to investigate CV involvement patterns in patients with JIA. [ABSTRACT FROM AUTHOR]

Patients with cancer can present with difficult management issues, as the medicine can sometimes cause sequelae destructive to healthy tissue. As this population lives longer, cardiotoxic effects are beginning to emerge, but the early recognition of this signal can prove difficult, with too late a recognition leading to lifelong cardiac impairment and dysfunction. Cardio-oncology can bridge this difficulty, and echocardiography and its newer imaging abilities are proving efficacious in this population. This article will address common sequelae of cardiotoxic treatment regimens and offer recommendations for echocardiographic surveillance. We recommend echocardiography, preferably three-dimensional and strain imaging, to monitor for cardiotoxic myocardial effects before, during, and after chemotherapy with cardiotoxic drug regimens, particularly anthracycline derivatives. A reduction in left ventricular (LV) global longitudinal strain in all patients, or reduction in LV global circumferential strain or global radial strain in patients at intermediate to high risk for cardiotoxicity, despite normal LV ejection fraction warrants a clinical assessment on the benefits of continuing cardiotoxic chemotherapeutic agents. Lifelong surveillance using echocardiography for cardiotoxicity and radiation-related valvular, pericardial, and coronary artery disease is prudent. [ABSTRACT FROM AUTHOR]

Pericardial effusion (PE) is the presence of an excess of fluid in the pericardial cavity. PE symptoms depend from the rate of fluid accumulation, ranging from mild dyspnea on exertion to shock due to cardiac tamponade. Echocardiography is usually the primary diagnostic tool when PE is suspected, as it is accurate, non-invasive, widely available, and feasible also with pocket size devices. Studies have shown a high degree of sensitivity and specificity in the detection of PE using focused cardiac ultrasound (FOCUS), which can be performed also by non-cardiologist in emergency setting or at bedside. A PE is visualized as an echo-free space between the heart and the parietal layer of the pericardium. A semi-quantification of the PE may be obtained measuring the distance between the two pericardial layers. Once PE diagnosis has been made, characterization of fluid and search for signs of possible cardiac tamponade have to be performed. While unechogenic space is usually associated with serous fluid, hemorrhagic, and purulent effusions may be suspected in the presence of corpuscolated/echogenic fluid. Echocardiography may identify cardiac tamponade before it is clinically evident, and can guide pericardiocentesis. B-mode echocardiographic signs of cardiac tamponade include cardiac chambers collapse (with right chambers collapse occurring at earlier stages), opposite changes in right and left cardiac chamber filling during respiratory cycle, inferior vena cava and hepatic vein plethora. Doppler analysis of tricuspidalic and mitral flow velocities are used for a more detailed analysis of ventricular interdependence, even though more advanced operator expertise is required. [ABSTRACT FROM AUTHOR]

Constrictive pericarditis is a disorder of cardiac filling caused by an inelastic pericardium. This treatable cause of heart failure should be considered in all patients with unexplained right heart failure symptoms or signs, especially when the left ventricular ejection fraction is preserved. Diagnosing constrictive pericarditis remains challenging, and the most effective tools are designed to identify its unique pathophysiologic mechanisms: dissociation of intrathoracic and intracardiac pressures and enhanced ventricular interaction. The cornerstone of the diagnostic work-up remains comprehensive echocardiography with Doppler, but cross-sectional imaging and invasive hemodynamic assessment may be necessary in some cases. Cardiac MRI is particularly helpful in identifying those patients who may have inflammatory constriction that would resolve with anti-inflammatory therapy. Complete surgical pericardiectomy remains the only definitive treatment for patients with chronic constriction. [ABSTRACT FROM AUTHOR]

Radiation-induced heart disease (RIHD) represents a spectrum of cardiovascular disease in patients who have undergone mediastinal, thoracic, or breast radiotherapy (RT). RIHD may involve any cardiac structure and is a major cause of morbidity and mortality in cancer survivors. While large cohort studies have demonstrated that symptomatic RIHD is a common late finding in this population, the incidence of asymptomatic disease is likely to be even higher. Long-term follow-up with regular screening for RIHD plays an important role in the management of cancer survivors who have undergone RT. Aggressive modification of traditional cardiovascular risk factors such as hypertension, dyslipidemia, and cigarette smoking is essential in patients at risk for RIHD, as these have been shown to potentiate the risks of radiation. In patients with symptomatic RIHD, medical and/or percutaneous therapies are often preferable to surgical interventions in view of the increased surgical risk associated with radiation damage to surrounding tissues. Percutaneous revascularization should generally be favored over surgical revascularization. Transcatheter valve replacements have not been widely used in this population but may offer an alternative to high-risk surgical valve procedures. Pericardiectomy is usually associated with extremely poor short-term and long-term outcomes in patients with RIHD and should be avoided in most cases. Heart transplantation is also higher risk in patients with RIHD than in patients with other etiologies of heart failure, but may be considered in young patients without other comorbidities. [ABSTRACT FROM AUTHOR]

Although echocardiography remains the standard diagnostic tool for identifying pericardial diseases, procedures with better delineation of morphology and heart function are often required. The pericardium consists of an inner visceral (epicardium) and outer parietal layer (pericardium), which constitute for the pericardial cavity. Pericardial effusion can occur as transudate, exudate, pyopneumopericardium, or hemopericardium. Potential causes are inflammatory processes, that is, pericarditis due to autoimmune or infective reasons, neoplasms, irradiation, or systemic disorders, chronic renal failure, endocrine, or metabolic diseases. Pericardial fat can mimic pericardial effusion. Using various image-acquisition sequences, MRI allows identifying and separating fluid and solid structures. Fast spin-echo T1-weighted sequences with black-blood preparation are favourably used for morphological evaluation. Fast spin-echo T2-weighted sequences, particularly with fat saturation, and short-tau inversion-recovery sequences are useful to visualize oedema and inflammation. For further tissue characterization, delayed inversion-recovery imaging is used. Therefore, image acquisition is performed at 5-20 min subsequent to contrast agent administration, the so-called technique of late gadolinium enhancement. Ventricular volumes and myocardial mass can be assessed accurately by steady-state free-precession sequences, which is required to measure cardiac function and ventricular wall stress. Constrictive pericarditis usually results from chronic inflammatory processes leading to increased stiffness, which impedes the slippage of both pericardial layers and thereby the normal cardiac filling. CT imaging can favourably assess pericardial calcification. Thus, MR and CT imaging allow a comprehensive delineation of the pericardium. Superior to echocardiography, both methods provide a larger field of view and depiction of the complete chest including abnormalities of the surrounding mediastinum and lungs. PET provides unique information on the in vivo metabolism of 18-fluorodeoxyglucose that can be superimposed on CT findings and is useful for identifying inflammatory processes or masses, for example neoplasms. These imaging techniques provide advanced information of anatomy and cardiac function to optimize the pericardial access, for example by the AttachLifter system, for diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Purpose of Review: Cardiovascular magnetic resonance (CMR) provides the most comprehensive imaging assessment of pericardial disease, providing a three-dimensional assessment of the pericardium, functional assessment of its impact on cardiac contractility, and pericardial tissue/fluid characterization. This review presents an update on the utility of CMR imaging in a wide variety of pericardial diseases. Recent Findings: CMR provides both qualitative and quantitative assessment of the pericardium through various imaging techniques. It can also be used as a guide therapy and delineate response to treatment in pericarditis. CMR is also useful for the assessment of rare congenital disorders and in defining pericardial tumors and differentiating some non-invasively. Summary: CMR is a powerful non-invasive diagnostic tool for evaluating and characterizing pericardial diseases. Ongoing optimization of imaging techniques allows for differentiation of subtypes of disease as well as progression. Ongoing research demonstrates continued expanding role of CMR in both the diagnosis and management of pericardial and cardiovascular disease. [ABSTRACT FROM AUTHOR]

Purpose of Review: To review the uses of echocardiography in patients with cancer and how it has expanded beyond the typical monitoring of systolic function during potentially cardiotoxic cancer therapeutics. Recent Findings: In addition to myocardial strain imaging being a predictor of subsequent left ventricular dysfunction, it can be used for pattern recognition to help identify patients with cardiac amyloidosis or Takotsubo cardiomyopathy. Echocardiography is essential for diagnosis and planning of intervention for aortic stenosis in radiation-induced valvular disease, for which transcutaneous aortic valve replacement that gives many cancer patients that are not surgical candidates an option for treatment. The safety of transesophageal echocardiography has recently been demonstrated in patients with cancer with thrombocytopenia and depleted white blood cell counts who are at increased risk of endocarditis. Summary: Echocardiography is an essential tool for evaluating common conditions in cancer patients such as pericardial disease, radiation-induced heart disease, and intracardiac tumors—with specific uses of specialized echocardiography techniques such as deformation imaging, transesophageal echocardiography, and point-of-care ultrasound. [ABSTRACT FROM AUTHOR]

Identification of the etiology of pericardial disease is challenging because the accessibility to pericardial fluid and tissue is limited and there is a relatively low yield of fluid and tissue analysis. Pericardial disease is associated with various systemic diseases and is frequently a first manifestation of other systemic diseases. Detecting the cause of pericarditis and minimizing the subsequent inflammatory process can possibly prevent long-term complications. Purpose of Review: To review the clinical utility of [18F]-2-deoxy-2-fluoro-D-glucose positron emission tomography/computed tomography ([18F]FDG-PET/CT) in the diagnosis and treatment of pericardial disease. Recent Findings: [18F]FDG-PET/CT can visualize the hypermetabolic tissues of both malignancy and inflammation. Distribution of [18F]FDG-PET/CT uptake can provide information for neoplastic disease. If malignancy is ruled out, high uptake of pericardium is associated with active inflammation of the pericardium, and thus response to anti-inflammatory agents can also be predicted with [18F]FDG-PET/CT imaging. Summary: [18F]FDG-PET/CT can be helpful for diagnosing and establishing prognosis and for planning for anti-inflammatory treatment in pericardial disease. [ABSTRACT FROM AUTHOR]

Purpose of Review: This review highlights the literature related to pericardial injury following radiation for oncologic diseases. Recent Findings: Radiation-associated pericardial disease can have devastating consequences. Unfortunately, there is considerably less evidence regarding pericardial syndromes following thoracic radiation as compared to other cardiovascular outcomes. Pericardial complications of radiation may arise acutely or have an insidious onset several decades after treatment. Transthoracic echocardiography is the screening imaging modality of choice, while cardiac magnetic resonance imaging further characterizes the pericardium and guides treatment decision-making. Cardiac CT can be useful for assessing pericardial calcification. Ongoing efforts to lessen inadvertent cardiac injury are directed towards the revision of radiation techniques and protocols. Summary: As survival of mediastinal and thoracic malignancies continues to improve, radiation-associated pericardial disease is increasingly relevant. Though advances in radiation oncology demonstrate promise in curtailing cardiotoxicity, the long-term effects pertaining to pericardial complications remain to be seen. [ABSTRACT FROM AUTHOR]

Purpose of Review: This is an in-depth review on the mechanism of action, clinical utility, and drug-drug interactions of colchicine in the management of pericardial disease.Recent Findings: Recent evidence about therapeutic targets on pericarditis has demonstrated that NALP3 inflammasome blockade is the cornerstone in the clinical benefits of colchicine. Such benefits extend from acute and recurrent pericarditis to transient constriction and post-pericardiotomy syndrome. Despite the increased utilization of colchicine in cardiovascular medicine, safety concerns remains unsolved regarding the long-term use of colchicine in the cardiac patient. Moreover, recent evidence has demonstrated that numerous cardiovascular medications, ranging from antihypertensive medication to antiarrhythmics, are known to interact with the CYP3A4 and/or P-gp system increasing the toxicity potential of colchicine.Summary: The use of adjunctive colchicine in the management of inflammatory pericardial diseases is standard of care in current practice. It is advised that a careful medication reconciliation with emphasis on pharmacokinetic is completed before prescribing colchicine in order to avoid harmful interaction by finding an alternative regimen or adjusting colchicine dosing. [ABSTRACT FROM AUTHOR]