Semiquantitative 18F-FDG PET/CT in monitoring glucocorticoid response of immunoglobulin G4-related effusive constrictive pericarditis: a case report.

BACKGROUND: Immunoglobulin G4 (IgG4)-related effusive constrictive pericarditis (ECP) is a rare manifestation of IgG4-related disease (IgG4-RD). It can lead to persistent pericardial fibrosis, resulting in cardiac tamponade, diastolic dysfunction, and heart failure. Glucocorticoids are the primary treatment for effectively reducing inflammation and preventing fibrosis. However, guidelines for monitoring treatment response are lacking and tapering glucocorticoid therapy for specific target organs remains a challenge. Recent studies on IgG4-RD have demonstrated that semiquantitative measurements of fluorine-18 fluorodeoxyglucose (18F-FDG) uptake in the main involved organs in positron emission tomography/computed tomography (PET/CT) scanning are correlated to disease activity. We present a case of IgG4-related ECP to demonstrate the usefulness of 18F-FDG PET/CT for diagnosing and treatment follow-up of IgG4-related ECP. CASE PRESENTATION: Herein, a 66-year-old woman diagnosed with IgG4-related ECP presented with breathlessness, leg swelling, rales, and fever. Laboratory tests revealed markedly elevated levels of C-reactive protein, and transthoracic echocardiography revealed constrictive physiology with effusion. High IgG4 levels suggested an immune-related pathogenesis, while viral and malignant causes were excluded. Subsequent pericardial biopsy revealed lymphocyte and plasma cell infiltration in the pericardium, confirming the diagnosis of IgG4-related ECP. 18F-FDG PET/CT revealed increased uptake of 18F-FDG in the pericardium, indicating isolated cardiac involvement of IgG4-RD. Treatment with prednisolone and colchicine led to a rapid improvement in the patient's condition within a few weeks. Follow-up imaging with 18F-FDG PET/CT after 3 months revealed reduced inflammation and improved constrictive physiology on echocardiography, leading to successful tapering of the prednisolone dose and discontinuation of colchicine. CONCLUSION: The rarity of IgG4-related ECP and possibility of multiorgan involvement in IgG4-RD necessitates a comprehensive diagnostic approach and personalized management. This case report highlights the usefulness of 18F-FDG PET/CT in the diagnosis and treatment follow-up of isolated pericardial involvement in IgG4-RD.

Ultrasound analysis of cervical thoracic duct for patients with constrictive pericarditis and chylothorax.

PURPOSE: To analyze ultrasound features of cervical thoracic duct for patients with constrictive pericarditis and chylothorax. METHODS: Patients were retrospectively assessed. The patients were divided into a non-pleural effusion (PE) group (n = 54), a chylothorax group (n = 23), and non-chylothorax group (n = 28). Conventional ultrasound was used to obtain the maximum inner diameter and collapse of the inferior vena cava, the inner diameter of left cervical thoracic duct, and the frequency of opening of the valve at the end of the left thoracic duct. Contrast ultrasonography was used to score the reverse flow of the thoracic tube. RESULTS: The percentage of PE was 48.5%, and the percentage of chylothorax was 21.9%. The three groups had significant differences in five parameters. The inner diameter of left cervical thoracic duct was correlated with the degree of central venous pressure. Contrast ultrasonography was effective in quantitative assessment of the degree of intravenous-thoracic cord reverse flow which correlated with all parameters of central venous pressure. CONCLUSION: Thoracic duct dilation and regurgitation secondary to central venous pressure can lead to chyloreflux disorder and may be the mechanism of chylothorax occurrence in constrictive pericarditis.

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.

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.

Anesthesia management for pericardiectomy- a case series study.

BACKGROUND: Constrictive pericarditis (CP) is an uncommon disease that limits both cardiac relaxation and contraction. Patients often present with right-sided heart failure as the pericardium thickens and impedes cardiac filling. Pericardiectomy is the treatment of choice for improving hemodynamics in CP patients; however, the procedure carries a high morbidity and mortality, and the anesthetic management can be challenging. Acute heart failure, bleeding and arrhythmias are all concerns postoperatively. METHODS: After IRB approval, we performed the retrospective analysis of 66 consecutive patients with CP who underwent pericardiectomy from July 2018 to May 2022. RESULTS: Most patients had significant preoperative comorbidities, including congestive hepatopathy (75.76%), New York Heart Association Type III/IV heart failure (59.09%) and atrial fibrillation (51.52%). Despite this, 75.76% of patients were extubated within the first 24 h and all but 2 of the patients survived to discharge (96.97%). CONCLUSIONS: Anesthetic management, including a thorough understanding of the pathophysiology of CP, the use of advanced monitoring and transesophageal echocardiography (TEE) guidance, all played an important role in patient outcomes.

Non-pulmonary Disseminated Tuberculosis Complicated by Constrictive Pericarditis and Cutaneous Gumma.

A 23-year-old previously healthy male presented to the hospital with symptoms of heart failure. He was diagnosed with pericarditis and found to have a reduced left ventricular ejection fraction of 25%. He was noted to have mediastinal lymphadenopathy. Pulmonary and abdominal sampling were non-diagnostic for infection, autoimmune disease, or malignancy. A QuantiFERON Gold returned positive. After a thorough travel history and detailed exam, the patient was diagnosed with disseminated tuberculosis after the discovery of a cutaneous gumma that was found to have acid-fast bacilli present on biopsy with Fite's stain. 18F-FDG PET CT and cardiac MRI were pursued given that pericardial and myocardial biopsy could not be safely performed due to the patient's hemodynamics. 18F-FDG PET CT and cardiac MRI did not demonstrate any myocardial pathology responsible for the left ventricular ejection fraction. This case highlights that pulmonary involvement is not necessary for disseminated TB, Fite's stain may be used to identify M. tuberculosis, and that cardiac MRI and 18F-FDG PET CT may be useful to delineate myocardial involvement in high-risk situations.

Constrictive pericarditis: portal, splenic, and femoral venous Doppler pulsatility: a case series.

PURPOSE: Pulsatile flow of the portal vein has been implicated as an indicator of right ventricular dysfunction in cardiac patients. In patients with significantly elevated right atrial pressure, pulsatile venous flow may be transmitted to the portal, splenic, renal, and femoral veins. We describe the evolution of these echocardiographic findings in four patients with constrictive pericarditis (CP) undergoing pericardiectomy with simultaneous hemodynamic waveform and cerebral oximetry monitoring in the operating room and in the intensive care unit. CLINICAL FEATURES: Patient 1 presented classic signs of CP, including equalization of left and right diastolic pressures, a "square root" sign on the diastolic portion of the right ventricular pressure curve, and elevated right atrial pressure. Preoperative transesophageal echocardiography showed a hyperdynamic left ventricle and dilated right ventricle with abnormal pulsatile waveforms in the portal and splenic veins. Surgical decompression of the pericardium gradually normalized the Doppler waveforms. Increased venous return following pericardiectomy during surgery in patients 2 and 3 and during the postoperative period in patient 4 resulted in right ventricular (RV) failure due to significantly increased preload. Venous pulsatility was also observed in the portal, splenic, and femoral veins. CONCLUSION: In patients with CP, changes in hemodynamic and echocardiographic signs of RV dysfunction are rapidly reflected by changes in peripheral venous velocities. Identifying signs of splanchnic and peripheral vascular venous congestion could help identify patients at higher risk of developing postoperative complications following pericardiectomy.

RéSUMé: OBJECTIF : Le flux pulsatile de la veine porte a été impliqué comme indicateur de dysfonctionnement ventriculaire droit chez les patients de chirurgie cardiaque. Le flux veineux pulsatile pourrait être transmis aux veines porte, splénique, rénale et fémorale chez les patients présentant une pression auriculaire droite significativement élevée. Nous décrivons l'évolution de ces observations échocardiographiques chez quatre patients atteints de péricardite constrictive (PC) bénéficiant d'une péricardectomie avec monitorage simultané de la forme d'onde hémodynamique et de l'oxymétrie cérébrale en salle d'opération et à l'unité de soins intensifs. CARACTéRISTIQUES CLINIQUES: Le patient 1 présentait des signes classiques de PC, y compris l'égalisation des pressions diastoliques gauche et droite, un signe de « racine carrée ¼ sur la partie diastolique de la courbe de pression ventriculaire droite, et une pression auriculaire droite élevée. L'échocardiographie transœsophagienne préopératoire a montré un ventricule gauche hyperdynamique et un ventricule droit dilaté, avec des formes d'onde pulsatiles anormales dans les veines porte et splénique. La décompression chirurgicale du péricarde a progressivement normalisé les formes d'onde Doppler. L'augmentation du retour veineux suivant une péricardectomie, survenue pendant la chirurgie chez les patients 2 et 3 et en période postopératoire chez le patient 4, a entraîné une défaillance ventriculaire droite (VD) due à l'augmentation significative de la précharge. La pulsatilité veineuse a également été observée dans les veines porte, splénique et fémorale. CONCLUSION: Chez les patients atteints de péricardite constrictive, les changements dans les signes hémodynamiques et échocardiographiques de dysfonctionnement du VD sont rapidement reflétés par des changements dans la vélocité veineuse périphérique. L'identification des signes de congestion veineuse splanchnique et vasculaire périphérique pourrait aider à identifier les patients présentant un risque plus élevé de manifester des complications postopératoires après une péricardectomie.

Incidentally detected dual right coronary artery in patient with constrictive pericarditis.

We report a case of a 29-year-old man with constrictive pericarditis where CT angiography incidentally demonstrated a dual right coronary artery (RCA). The present case highlights the diagnostic criterion for dual RCA as well as the potential clinical implications of the anomaly.

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.

Calcified constrictive pericarditis resulting in tamponade in a patient with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multiorgan involvement, including heart. Pericarditis-the most common cardiac manifestation-occurs in up to 50% of cases, resulting in positive treatment outcomes. Rarely, it evolves to hazardous complications. A 50-year-old woman with SLE in clinical remission, receiving hydroxychloroquine 400 mg/day, presented to us with severe chest pain and low-grade fever. Physical examination revealed a friction rub and decreased breath sounds at the right lung base. Laboratory evaluation demonstrated leukopenia, thrombocytopenia, low C4 levels, and high acute phase reactants. Chest X-ray exhibited cardiomegaly, calcified pericardium, and right pleural effusion, confirmed by CT scan. PPD skin test and IGRA were both negative. Pericardial fluid, blood, and urine cultures for bacteria and fungi, as well as Gram and Ziehl-Neelsen stains were negative. Serological tests for viruses were also negative. The patient was diagnosed with calcified constrictive pericarditis (CP) due to SLE. She was treated with cyclophosphamide and methylprednisolone pulses, without improvement. Her clinical condition deteriorated, developing signs and symptoms compatible with cardiac tamponade (TMP), which was confirmed by Doppler echocardiography. The patient underwent pericardiectomy. A dramatic response was noted and she was discharged with prednisone 50 mg/day and azathioprine 100 mg/day. Thus, we review and discuss the relevant literature of SLE cases with CP or TMP. When an SLE patient presents with CP, infectious causes should be excluded first. To the best of our knowledge, this is the only case of SLE and calcified CP leading to TMP, hence physicians should be aware of this complication.

Multimodality imaging of constrictive pericarditis in H syndrome.

This is the first report of constrictive pericarditis (CP) in a 16-year-old boy with H syndrome with pericardial involvement predominantly over the right ventricle with favorable response to anti-inflammatory treatment. H syndrome, first reported in 2008, is a new auto-inflammatory syndrome with multiorgan involvement due to mutation in the SLC29A3 gene. We described the echocardiographic characteristics of asymmetric pericardial involvement and presented the cardiac computed tomography angiographic and magnetic resonance imaging findings. We reviewed the echocardiographic signs of CP, introduced tricuspid E/A respiratory alternans as a novel echocardiographic sign of right ventricular dominant CP, and explained the underlying mechanism.

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.

Pericardiectomy for Constrictive Pericarditis: Analysis of Outcomes.

Constrictive pericarditis is caused by pericardial inflammation and fibrosis, leading to diastolic heart failure. The diagnosis requires a high index of suspicion because it often can mimic restrictive myocardial disease and cardiac tamponade and can be associated with severe tricuspid regurgitation and chronic liver disease. Patients who remain undiagnosed can experience a 90% mortality rate, and for those who undergo pericardiectomy, the survival rate varies significantly, depending on the underlying etiology and preoperative functional class of the patient. In this article, the authors review the pathophysiology, echocardiographic findings, management, and surgical outcomes of constrictive pericarditis to aid the cardiothoracic anesthesiologist in the perioperative management of this disorder.

Fluid Balance of the Second Day Following Operation is Associated with Early Mortality and Multiorgan Failure After Pericardiectomy for Constrictive Pericarditis.

BACKGROUND: The operative mortality of pericardiectomy still is high. This retrospective study was conducted to determine the risk factors of early mortality and multiorgan failure. METHODS: We retrospectively analyzed patients undergoing pericardiectomy from January 2009 to June 2020 at our hospital. Pericardiectomy was performed via sternotomy. Histopathologic studies of pericardium tissue from every patient were done. All survivors were monitored to the end date of the study. RESULTS: Ninety-two consecutive patients undergoing pericardiectomy for constrictive pericarditis were included in the study. Postoperatively, central venous pressure significantly decreased, and left ventricular end diastolic dimension and left ventricular ejection fractions significantly improved. The overall mortality rate was 5.4%. The common postoperative complications include acute renal injury (27.2%), and multiorgan failure (8.7%). Analyses of risk factors showed that fluid balance of the second day following operation is associated with early mortality and multiorgan failure. In this series from Guangxi, China, characteristic histopathologic features of tuberculosis (60/92, 65.2%) of pericardium were the most common histopathologic findings, and 32 patients (32/92, 34.8%) had the histopathologic findings of chronic nonspecific inflammatory changes. The functional status of the patients improved after pericardiectomy; 6 months later postoperatively 85 survivors were in class I (85/87, 97.7%) and two were in class II (2/87, 2.3%). CONCLUSIONS: Tuberculosis is the most common cause of constrictive pericarditis in Guangxi, China. Fluid balance of the second day following operation is associated with early mortality and multiorgan failure after pericardiectomy for constrictive pericarditis in our study.

Features on cardiac magnetic resonance imaging and accuracy of echocardiographic findings for diagnosing constrictive pericarditis.

Constrictive pericarditis is a rare disease with a difficult diagnosis. Cardiac magnetic resonance (CMR) imaging data of Aga Khan University Hospital from January 2011 to March 2020 was retrospectively reviewed and patients with the diagnosis of constrictive pericarditis were included. A total of 22 patients were included with the mean age of 46±16 years and majority 17(77%) were male. The most common findings on transthoracic echo were significant respiratory variation in mitral and tricuspid inflow velocities in 20(91%), and septal annular e'>9 in 10 (86%). The most common finding on CMR was respiratory septal shift in 22(100%), followed by septal bounce in 21(95%) and thickened pericardium in 18(82%). Nearly two-third of the patients, 15(70%) were considered for pericardiectomy but it was deferred in 5 patients due to high surgical risk. Ten patients underwent pericardiectomy, with no mortality on a mean follow up of 4±2 years.

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.

[Constrictive Pericarditis Associated with Pericardial Hematoma:Report of a Case].

A 59-year-old man was admitted to our hospital due to keto-acidosis. Electric cardiogram showed history of myocardial infarction. Cardiac echogram showed severe left ventricular hypokinesis, thickened pericardium and pericardial effusion. Right ventricular pressure curve showed dip and plateau pattern, and coronary angiography showed severe three vessel disease. So, we diagnosed with constrictive pericarditis accompanying pericardial effusion and ischemic heart disease. After cardiac catheterization, as the hemodynamics was getting worse, we inserted intraaoric balloon pumping (IABP) immediately and performed an emergent operation. The heart was compressed by hematoma and thickened pericardium. The surrounding tissue of hematoma was organized including fibrous tissue. We resected hematoma and pericardium, and the cardiac function was improved remarkably. His postoperative course was uneventful. The cause of hematoma was thought to be intrapericardial bleeding after cardiac infarction. He had cardiac tamponade and constrictive pericarditis, and the diagnosis of this case was considered to effusive constrictive pericarditis.

Hemodynamics of constrictive pericarditis and restrictive cardiomyopathy.

Constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) are indolent disabling diseases of diastolic function. The two conditions share common pathophysiologic features, resulting in similar and overlapping clinical presentations, echocardiographic findings, and hemodynamic characteristics. However, their clinical course differs, as CP is surgically curable whereas RCM is a chronic condition managed medically. Separating these two entities is based on delineation of anatomic and physiologic derangements employing multimodality hemodynamic interrogation by advanced imaging techniques (Echo-Doppler, CT, and especially MRI) combined with sophisticated invasive hemodynamics.

Subacute pericardial abscess after aortic valve replacement: a case report.

BACKGROUND: Purulent pericarditis is an infectious disease, frequently caused by gram-positive bacteria, that is rarely observed in healthy individuals, and is often associated with predisposing conditions. CASE PRESENTATION: Here, we present the case of an Escherichia coli post-surgical localized purulent pericarditis complicated by transient constrictive pericarditis and its diagnostic and therapeutic management. CONCLUSIONS: Our case report focuses on the importance of imaging-guided treatment of purulent pericardial diseases, in particular on the emerging role of 18 F-labelled 2-fluoro-2-deoxy-D-glucose Positron Emission Tomography/Computed Tomography in pericardial diseases and on the management of transient constrictive pericarditis, often seen after thoracic surgery.