Constrictive pericarditis in the new millennium.

Constrictive pericarditis (CP) is a complex clinical syndrome in which an inflamed pericardium becomes fibrotic and non-compliant, ultimately reducing cardiac pump performance. Although we have known about CP for centuries, it remains a challenge to diagnose. Recent advances in cardiac imaging, along with an expanding armamentarium of treatment options, have improved the quality and precision of care for patients with CP. This article reviews important historical and contemporary perspectives on the pathophysiology of CP, as well as our approach to diagnosis and management.

Chronic constrictive pericarditis: a rare cardiac involvement in primary Sjögren's syndrome.

BACKGROUND: Constrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren's syndrome, usually occurring in association with pericardial effusion or pericarditis. We report a case of constrictive pericarditis with an insidious course and unusual evolution associated with primary Sjögren's syndrome. Due to the challenging nature of the diagnosis, clinical suspicion and multimodality imaging are essential for early identification and prompt initiation of treatment. Long-term outcomes remain uncertain. To the best of our knowledge, no other cases linking this autoimmune disease to constrictive pericarditis have been reported. CASE PRESENTATION: We present the case of a 48-year-old male patient with moderate alcohol habits and a history of two prior hospitalizations. On the first, the patient was diagnosed with primary Sjögren's syndrome after presenting with pleural effusion and ascites, and empirical corticosteroid regiment was initiated. On the second, two-years later, he was readmitted with complaints of dyspnea and abdominal distension. Thoracic computed tomography revealed a localized pericardial thickening and a thin pericardial effusion, both of which were attributed to his rheumatic disease. A liver biopsy showed hepatic peliosis, which was considered to be a consequence of glucocorticoid therapy. Diuretic therapy was adjusted to symptom-relief, and a tapering corticosteroid regimen was adopted. Four years after the initial diagnosis, the patient was admitted again with recurrent dyspnea, orthopnea and ascites. At this time, constrictive pericarditis was diagnosed and a partial pericardiectomy was performed. Although not completely asymptomatic, the patient reported clinical improvement since the surgery, but still with a need for baseline diuretic therapy. CONCLUSION: Albeit uncommon, connective tissue disorders, such as primary Sjögren's syndrome, should be considered as a potential cause of constrictive pericarditis, especially in young patients with no other classical risk factors for constriction. In this case, after excluding possible infectious, neoplastic and autoimmune conditions, a primary Sjögren´s syndrome in association with constrictive pericarditis was assumed. This case presents an interesting and challenging clinical scenario, highlighting the importance of clinical awareness and the use of multimodal cardiac imaging for early recognition and treatment.

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.

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.

Multimodal Imaging of Constrictive Pericarditis Induced by Long-term Pergolide Treatment for Parkinson's Disease.

We herein report the first case of constrictive pericarditis (CP) induced by long-term pergolide treatment for Parkinson's disease that was assessed using multimodal imaging in a 72-year-old patient with leg edema and dyspnea. The patient was correctly diagnosed with CP using multimodal imaging and successfully treated with pericardiectomy. The treatment history of Parkinson's disease and pathological findings of the removed pericardium suggested that long-term pergolide was the cause of CP. Properly recognizing pergolide as the cause of CP and accurately diagnosing CP using multimodal imaging may contribute to the early detection and treatment of pergolide-induced CP.

Primary pericardial mesothelioma complicated by pericardial calcification.

BACKGROUND: Pericardial calcification is usually a marker of chronic diseases, and its occurrence in rapidly progressing malignant primary pericardial mesothelioma (PPM) is extremely rare. Therefore, this atypical imaging appearance contributes to more frequent misdiagnosis of PPM. However, no systematic summary currently exists of the imaging characteristics of malignant pericardial calcification in PPM. In our report, its clinical characteristics are discussed in detail, to provide a reference to reduce the misdiagnosis rates of PPM. CASE PRESENTATION: A 50-year-old female patient was admitted to our hospital, presenting primarily with features suggestive of cardiac insufficiency. Chest computed tomography revealed significant pericardial thickening and localized calcification, suspicious of constrictive pericarditis. A chest examination performed through a midline incision showed a chronically inflamed and easily-ruptured pericardium that was closely adherent to the myocardium. Post-operative pathological examination confirmed a diagnosis of primary pericardial mesothelioma. Six weeks postoperatively, the patient experienced symptom recurrence and abandoned chemotherapy and radiation therapy. Nine months postoperatively, the patient died of heart failure. CONCLUSION: We report this case to highlight the rare finding of pericardial calcification in patients with primary pericardial mesothelioma. This case illustrated that confirmation of pericardial calcification cannot completely rule out rapidly developing PPM. Therefore, understanding the different radiological features of PPM can help to reduce its rate of early misdiagnosis.

Surgical Treatment of Constrictive Pericarditis.

INTRODUCTION: The mainstay of the treatment of constrictive pericarditis is pericardiectomy. However, surgery is associated with high early morbidity and mortality and low long-term survival. The aim of this study is to describe our series of pericardiectomies performed over 30 years. METHODS: A descriptive, observational, and retrospective analysis of all pericardiectomies performed at the Institute of Cardiology and Cardiovascular Surgery of the Favaloro Foundation was performed. RESULTS: A total of 45 patients underwent pericardiectomy between June 1992 and June 2022, mean age was 52 years (standard deviation ± 13.9 years), and 73.3% were men. Idiopathic constrictive pericarditis was the most prevalent (46.6%). The variables significantly associated with prolonged hospitalization were preoperative advanced functional class (incidence of 38.4%, P<0.04), persistent pleural effusion (incidence of 81.8%, P<0.01), and although there was no statistical significance with the use of cardiopulmonary bypass, a trend in this association is evident (P<0.07). We found that 100% of the patients with an onset of symptoms greater than six months had a prolonged hospital stay. In-hospital mortality was 6.6%, and 30-day mortality was 8.8%. The preserved functional class is 17 times more likely to improve their symptomatology after pericardiectomy (odds ratio 17, 95% confidence interval 2.66-71; P<0.05). CONCLUSION: Advanced functional class at the time of pericardiectomy is the variable most strongly associated with mortality and prolonged hospitalization. Onset of the symptoms greater than six months is also a poor prognostic factor mainly associated with prolonged hospitalization; based on these data, we strongly support the recommendation of early intervention.

Severely Calcified Pericardium Causing Constrictive Pericarditis.

Constrictive pericarditis (CP) is a type of diastolic heart failure caused by an inelastic pericardium that impairs cardiac filling. Diagnosing CP can be challenging, and a variety of imaging techniques may be necessary. We present a unique case of severely calcified pericardium leading to CP.

Left Ventricular Angiogram in Constrictive Pericarditis.

A 14-year-old boy presented with dyspnea on exertion and easy fatiguability for 1 year. He also had an episode of pedal edema 6 months prior, which resolved with diuretics. He had a history of tuberculosis treated with a 6-month course of antitubercular therapy. After a series of tests and analyses, it was evident that longitudinal contraction of the left ventricle was preserved, whereas the circumferential contraction was severely impaired. Pericardial calcification also could be seen encircling the left and right ventricles. Pericardial calcification is evident in around 25% of cases of constrictive pericarditis and predominantly seen in those with tubercular and pyogenic etiology. The subepicardial myocardial fibers are responsible for radial shortening and subendocardial fibers are responsible for longitudinal shortening. In constrictive pericarditis, the involvement of the subepicardial fibers leads to reduction in the circumferential shortening. The patient was referred for pericardiectomy.

Right Heart Failure With Recurrent Pericardial Effusion Mimicking Effusive-Constrictive Pericarditis Several Years After Renal Transplantation.

Pericardial disease is a rare complication after renal transplantation. We present a patient who developed high-output cardiac failure from a large arteriovenous (AV) fistula with recurrent pericardial effusion resulting in a constrictive hemodynamic pattern that was revealed during cardiac catheterization. Pericardiectomy was considered for recurrent effusive pericarditis, but per cardiac surgery recommendations, closure of the AV fistula dramatically cured the patient's heart failure, and no recurrence of pericardial effusion was seen during follow-up almost a year later.

Ascitic Fluid Analysis Leading to the Diagnosis of Constrictive Pericarditis in 2 Patients.

Although well documented, constrictive pericarditis is a rare entity and an uncommon cause of heart failure. A stiff and noncompliant pericardium creates the disease's unique hemodynamics and leads to elevated venous pressures, hepatic sinusoidal congestion, and draining of protein-rich fluid into the peritoneal cavity presenting as ascites. The low incidence in addition to its varied and subtle clinical presentations can often lead to a delay in diagnosis. Here, we present 2 clinical cases of constrictive pericarditis in which ascitic fluid analysis was important-one patient who presented with new-onset ascites with concern for cirrhosis and another patient who presented with symptoms concerning for heart failure with ascites. Through their hospital course and workup, we highlight the importance of diagnostic sampling of ascitic fluid to prompt the consideration of constrictive pericarditis followed by utilizing advanced diagnostics, such as echocardiogram and cardiac catheterization to reach the correct diagnosis in an otherwise often overlooked pathology.

Pericardial Manifestations of Thyroid Diseases.

PURPOSE OF REVIEW: Thyroid disorders, especially hypothyroidism, are known to be associated with pericardial diseases. The aim of this paper is to review the current knowledge of the pericardial manifestations of hypothyroidism and hyperthyroidism. RECENT FINDINGS: Many reports have described associations between dysthyroidism, which encompasses hypothyroidism and hyperthyroidism, and several pericardial diseases, including acute pericarditis, constrictive pericarditis, pericardial effusion, and tamponade. The diagnosis of dysthyroidism-induced pericardial diseases consists of a combination of thyroid blood levels that fall outside of the normal range and the exclusion of other causes. Treatment of the thyroid disorder is key, along with treatment of the pericardial disease as recommended by the guidelines. Early recognition of the thyroid disorder is key in patients with pericardial diseases, since treating the underlying cause should assist resolution of the pericardial issues and ideally prevent recurrence and possible future complications of suboptimally treated pericarditis or pericardial effusions.

Constrictive pericarditis caused by pericardial substitution using expanded polytetrafluoroethylene.

BACKGROUND: Expanded polytetrafluoroethylene (ePTFE) is commonly used as a pericardial substitute during cardiac surgery to prevent cardiac injury during re-sternotomy. However, although rare, constrictive pericarditis associated with ePTFE has been reported. MATERIAL, METHODS AND RESULTS: Here, we report a rare case of constrictive pericarditis developed due to severe restriction of cardiac motion associated with the ePTFE membrane used as a pericardial substitute. Hemodynamic improvement has been achieved by surgical removal of the ePTFE membrane and exudates within the overlapped portion of the ePTFE membranes, and dissection of the epicardial fibrous thickening. CONCLUSION: Considering the risk of constrictive pericarditis, we believe that the use of ePTFE membranes as a pericardial substitute should be carefully indicated for only selected patients.

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 SARS-CoV-2 vaccination: A case report.

Coronavirus disease 2019 (COVID-19) and vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are associated with cardiovascular complications. Here, we report a case of right-sided heart failure caused by constrictive pericarditis that developed after the administration of messenger ribonucleic acid (mRNA) vaccine against SARS-CoV-2. A 70-year-old woman presented with body weight gain, peripheral edema, and dyspnea on effort, which developed over a period of 1 week after the second dose of vaccine. The jugular venous pressure was high with a prominent y descent (Friedreich's sign) and paradoxical increase on inspiration (Kussmaul's sign). The results of IgM and IgG testing specific to SARS-CoV-2 spike and nucleocapsid proteins indicated the presence of mRNA vaccine-induced antibody and were not suggestive of COVID-19 infection. Echocardiography showed pericardial thickening and septal bounce of the interventricular septum. Computed tomography (CT) also showed pericardial thickening compared with the results of the previous CT scan performed 4 months earlier. A diagnosis of right-sided heart failure due to constrictive pericarditis was confirmed on the basis of pressure analysis during cardiac catheterization.

Dialysis-related constrictive pericarditis: old enemies may sometimes come back.

Cardiovascular disease is the main cause of death in patients with chronic kidney disease (CKD). Several heart conditions have been associated with CKD, including myocardial and pericardial diseases. This paper describes a case of Dialysis-related constrictive pericarditis in a patient diagnosed with sudden hypotension during a hemodialysis session. A 65-year-old man diagnosed with hypertension, diabetes, obesity, and cirrhosis on hemodialysis for two years complained of symptoms during one of his sessions described as malaise, lipothymia, and confusion. The patient had a record of poor compliance with the prescribed diet and missed dialysis sessions. He was sluggish during the physical examination, and presented hypophonetic heart sounds, a blood pressure of 50/30mmHg, and a prolonged capillary refill time. The patient was referred to the intensive care unit and was started on antibiotics and vasoactive drugs. His workup did not show signs of infection, while electrocardiography showed low QRS-wave voltage. His echocardiogram showed signs consistent with a thickened pericardium without pericardial effusion. Cardiac catheterization showed equalization of diastolic pressures in all heart chambers indicative of constrictive pericarditis. The patient underwent a pericardiectomy. Examination of surgical specimens indicated he had marked fibrosis and areas of dystrophic calcification without evidence of infection, consistent with Dialysis-related constrictive pericarditis. Hypotension for unknown causes must be considered in the differential diagnosis of dialysis patients.

Annular Indentation of the Ventricles in a Stillborn: A Case Report and Literature Review.

BackgroundIdiopathic indentation of the cardiac ventricles in a fetus has not been previously reported. Reported cases of congenital ventricle indentation are either caused by pericardial abnormalities or myocardial defects. Case report: We describe an incidental finding of annular indentation of the lower part of both ventricles in a stillborn male. The fetus was well-developed and the cause of stillborn was pronounced cord entanglement twice around the neck. Conclusion: Circumferential indentation of ventricles is distinguished from constrictive pericarditis and other myocardial defects as histologically the three layers of endocardium, myocardium, and pericardium are intact.