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.

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.

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.

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.

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.

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.

Constrictive pericarditis in the setting of repeated chest trauma in a mixed martial arts fighter.

BACKGROUND: Constrictive pericarditis (CP) is characterized by scarring and loss of elasticity of the pericardium. This case demonstrates that mixed martial arts (MMA) is a previously unrecognized risk factor for CP, diagnosis of which is supported by cardiac imaging, right and left heart catheterization, and histological findings of dense fibrous tissue without chronic inflammation. CASE PRESENTATION: A 47-year-old Caucasian male former mixed martial arts (MMA) fighter from the Western United States presented to liver clinic for elevated liver injury tests (LIT) and a 35-pound weight loss with associated diarrhea, lower extremity edema, dyspnea on exertion, and worsening fatigue over a period of 6 months. Past medical history includes concussion, right bundle branch block, migraine headache, hypertension, chronic pain related to musculoskeletal injuries and fractures secondary to MMA competition. Involvement in MMA was extensive with an 8-year history of professional MMA competition and 13-year history of MMA fighting with recurrent trauma to the chest wall. The patient also reported a 20-year history of performance enhancing drugs including testosterone. Physical exam was notable for elevated jugular venous pressure, hepatomegaly, and trace peripheral edema. An extensive workup was performed including laboratory studies, abdominal computerized tomography, liver biopsy, echocardiogram, and cardiac magnetic resonance imaging. Finally, right and left heart catheterization-the gold standard-confirmed discordance of the right ventricle-left ventricle, consistent with constrictive physiology. Pericardiectomy was performed with histologic evidence of chronic pericarditis. The patient's hospital course was uncomplicated and he returned to NYHA functional class I. CONCLUSIONS: CP can be a sequela of recurrent pericarditis or hemorrhagic effusions and may have a delayed presentation. In cases of recurrent trauma, CP may be managed with pericardiectomy with apparent good outcome. Further studies are warranted to analyze the occurrence of CP in MMA so as to better define the risk in such adults.

Constrictive pericarditis following heart transplantation: Reality or fiction?

Constrictive pericarditis (CP) is a curable cause of diastolic heart failure with prior cardiac surgery being a recognizable etiology. We report a patient who developed CP one year following heart transplantation. Several clinical and imaging related factors may lead to diagnostic delays in similar patients, including the mistaken belief that transplanted hearts are devoid of pericardium and thus do not develop constriction. Post-transplantation pericardial effusion, mediastinitis, and cardiac rejection predispose to future CP. Caretakers should consider this entity in allograft recipients who develop heart failure symptoms of unclear etiology.

[Constrictive Pericarditis with Multiple Organ Failure after Coronary Artery Bypass Grafting].

A 67-year-old male patient, who had undergone coronary artery bypass grafting (CABG) 16 years before, developed congestive heart failure 5 years after surgery. Three years later, he developed repeated heart failure, sepsis by methicillin-resistant Staphylococcus aureus (MRSA), renal failure, repeated thrombophlebitis on his right leg and atrial fibrillation. He also suffered from clouding of consciousness and flapping tremor caused by hyperammonemia. The three bypass grafts showed normal flow, but the pericardium was severely thickened. Therefore, pericardiotomy was performed via median sternotomy with additional left thoracotomy without using cardiopulmonary bypass. Although, he developed MRSA mediastinitis after surgery, he recovered after a month of continuous negative pressure wound therapy. His liver function and septic conditions gradually recovered. No recurrence of heart failure has been observed for 8 years since his second surgery.

Outcomes of pericardiectomy for constrictive pericarditis following mediastinal irradiation.

BACKGROUND: Pericardiectomy for postradiation constrictive pericarditis has been reported to generally have unfavorable outcomes. This study sought to evaluate surgical outcomes in a large cohort of patients undergoing pericardiectomy for radiation-associated pericardial constriction. METHODS: A retrospective analysis of all patients (≥18 years) who underwent pericardiectomy for a diagnosis of constrictive pericarditis with a prior history of mediastinal irradiation from June 2002 to June 2019 was conducted. There were 100 patients (mean age 57.2 ± 10.1 years, 49% females) who met the inclusion criteria. Records were reviewed to look at the surgical approach, the extent of resection, early mortality, and late survival. RESULTS: The overall operative mortality was 10.1% (n = 10). The rate of operative mortality decreased over the study period; however, the test of the trend was not statistically significant (p = .062). Hodgkin's disease was the most common malignancy (64%) for which mediastinal radiation had been received. Only 27% of patients had an isolated pericardiectomy, and concomitant pericardiectomy and valve surgery were performed in 46% of patients. Radical resection was performed in 50% of patients, whereas 47% of patients underwent subtotal resection. Prolonged ventilation (26%), atrial fibrillation (21%), and pleural effusion (16%) were the most common postoperative complications. The overall 1, 5-, and 10-years survival was 73.6%, 53.4%, and 32.1%, respectively. Increasing age (hazard ratio, 1.044, 95% confidence interval 1.017-1.073) appeared to have a significant negative effect on overall survival in the univariate model. CONCLUSION: Pericardiectomy performed for radiation-associated constrictive pericarditis has poor long-term outcomes. The early mortality, though high (~10%), has been showing a decreasing trend in the test of time.

Complete resolution of constrictive pericarditis after coronary bypass surgery.

Classical constrictive pericarditis (CP) is an unusual and rare complication after coronary artery bypass grafting. It can be transient, progressive or fixed form of cardiac constriction. However recently recognized transient variant of constrictive pericarditis can be managed with medical therapy, though other progressive and irreversible forms may require pericardiectomy. We describe a 65-year-old male patient who developed a classical but a very early transient CP, just within two weeks as a result of post cardiac injury syndrome after coronary bypass surgery. The patient had a complete recovery following medical treatment.

A rare cause of effusive-constrictive pericarditis.

Effusive-constrictive pericarditis (ECP) is an uncommon diagnosis, frequently missed due to its heterogeneous presentation, but a potentially reversible cause of heart failure. A 62-year-old Caucasian male presented with remittent right heart failure and mild-moderate pericardial effusion. Following an initial diagnosis of idiopathic pericarditis, indomethacin was started, but the patient shortly relapsed, presenting with severe pericardial effusion and signs of cardiac tamponade, requiring pericardiocentesis. ECP was diagnosed on cardiac catheterization. Cardiac computed tomography showed non-calcified, mildly thickened and inflamed parietal pericardium. Pericardiectomy was performed with symptoms remission. On histological examination of pericardium, chronic non-necrotizing granulomatous inflammation was noted. Polymerase chain reaction assay was positive for non-tuberculous mycobacteria. This case represents a rare finding of ECP with unusual presentation due to atypical mycobacteriosis in a non-immunocompromised patient and in a non-endemic area. Pericardiectomy can be an effective option in cases unresponsive to anti-inflammatory treatment, even in the absence of significant pericardial thickening or calcification.

Rheumatic pericarditis: a rare cause of constrictive pericarditis.

Constrictive pericarditis is a relatively uncommon form of cardiac failure and presents due to scarring and consequent loss of the normal elasticity of the pericardial sac. This results in abnormal/limited ventricular filling and symptoms of heart failure. The aetiology is varied, from infective causes to idiopathic causes, or can manifest after cardiothoracic surgery. This case involves a 46-year-old man presenting with acute group A beta haemolytic streptococcus infection, and over the subsequent 6 months develops constrictive pericarditis due to what is believed to be a rheumatic aetiology. The patient subsequently underwent pericardiectomy and had restoration of normal filling dynamics confirmed on follow-up echocardiography. This case provides a subject matter for the review of the features of constrictive pericarditis and its investigation and management. This case is that it highlights the fact that pericarditis is not a benign condition. Emerging evidence suggests that pericarditis is due to a failure in inflammatory regulatory mechanisms, and patients suffering this condition have a preponderance to 'autoinflammation'. Pericarditis should be recognised early and treated fully with anti-inflammatory agents.

Pericardial Involvement in Cancer.

Despite the monumental advances in the diagnoses and therapeutics of malignancy, several cancer patients have presented with pericardial involvement, including acute pericarditis, constrictive pericarditis, and pericardial effusion. Multiple factors can contribute to acute pericarditis, including direct metastasis to the heart, pericardial hemorrhage, infections due to immunosuppression, and cancer therapies that include chemotherapy, immunotherapy, and radiation. Pericardial effusion, either due to cancer invasion or cancer treatment, is one of the most common incidental findings in cancer patients, which significantly worsens morbidity and mortality. If left untreated, pericardial effusion is known to cause complications such as pericardial tamponade. Constrictive pericarditis can be due to radiation exposure, chemotherapy, or is a sequela of a previous episode of acute pericarditis. In conclusion, early detection, prompt treatment, and understanding of pericardial diseases are necessary to help improve the quality of life of cancer patients, and we aim to summarize the knowledge of pericardial involvement in patients with cancer.

Exacerbation of severe constrictive pericarditis after prior inadequate pericardiectomy.

Pericardiectomy is commonly used to treat constrictive pericarditis (CP); however, persistent calcification can complicate recovery. An 82-year-old man presented with CP following an inadequate pericardiectomy at another hospital two years earlier. He was referred to our hospital with a diagnosis of recurrent CP. Pre-operative computed tomography revealed that the pericardium was not calcified on the anterior of the heart, while the inferior, posterior and lateral surfaces exhibited calcification. Notably, calcification along the inferior portion of the heart formed a calcium envelope structure. Pericardiectomy via re-sternotomy without cardiopulmonary bypass was performed. While dissecting the calcium envelope, a paste-like substance was exuded. Cardiac function improved after pericardiectomy, although the postoperative recovery from heart failure was prolonged. Calcified pericardium should be removed to the extent possible to enhance the efficacy of pericardiectomy, which contributes to improved early surgical results and prevents CP recurrence.