Pericardial Calcification: An Uncommon Case with Intraventricular Extension.

An 80-year-old man presented to the cardiology outpatient clinic due to shortness of breath. His past medical history included alcohol intake, hypertension, inferior wall myocardial infarction (five years ago), an ischemic stroke, and permanent atrial fibrillation (diagnosed three years before the current examination). A physical exam revealed a decreased intensity of S1 and S2, irregular rate and rhythm, and no murmurs nor friction rub. X-rays, Computed Tomography, and echocardiography exhibited pericardial calcification, involving mostly the inferior wall and protruding into the left ventricle. A diagnosis of constrictive pericarditis due to pericardial calcification was established and considered idiopathic. Even when it may be related to ischemic heart disease, post-infarction pericarditis could explain how the calcification extended to adjacent territory perfused by the circumflex coronary artery. Combined imaging studies were crucial not only for identifying calcium deposits in the pericardium but also in assessing a patient inherently prone to co-existing and exacerbating conditions. Even though pericardiectomy allows for removal of the clinical manifestations of congestive pericarditis in the most symptomatic patients with pericardial calcification, among patients like ours, with tolerable symptoms, cardiologists should discuss the therapeutic options considering the patient's choices, potentially including a rehabilitation plan as part of non-pharmacological management.

Clinicopathological features of immunoglobulin G4-related constrictive pericarditis.

AIM: Constrictive pericarditis (CP) is characterised by scarring fibrosis and a loss of pericardial elasticity, which causes heart failure. IgG4 (immunoglobulin G4)-related disease (IgG4-RD) is a systemic fibro-inflammatory disease characterised by the infiltration of IgG4-immunopositive plasmacytes and high serum IgG4 levels that frequently shape tumorous lesions. Although pericardial involvement of IgG4-RD is rare, with indications of CP, pericardial effusion and irregular masses, the clinical and pathological features remain unclear. In this study, we examined the relationship between CP and IgG4-RD. METHODS: Among 35 thick-walled CP cases (histologically pericardial thickening ≥2 mm), eight cases were aetiology identified. Using the diagnostic criteria for IgG4-RD, 11 cases were classified as IgG4-CP, whereas the remainder were considered true idiopathic CP (16 cases) and the clinical pathological features were evaluated. RESULTS: Compared with the other groups, the IgG4-CP group was more common in men and associated with low-grade fever and massive pericardial effusion with frequent recurrence. Deaths resulting from heart failure occurred in a few cases of the IgG4-CP group, but not in other groups. An increase in C-reactive protein and a high positivity rate of anti-nuclear antibodies frequently occurred in the IgG4-CP group. Histologically, the IgG4-CP group included lymphoid follicle, eosinophil infiltration and few calcifications. CONCLUSIONS: Pericardial IgG4-RD occurs not only as nodular lesions, but also as thick-walled CP, and accounts for approximately 40% of thick-walled CP cases of unknown cause. The predominant clinical characteristic was refractory and recurrent pericardial effusion. Recognising IgG4-RD as a cause of CP is important to initiate appropriate therapy.

Evaluation of Pericardial Thickening and Adhesion Using High-Frequency Ultrasound.

BACKGROUND: Routine echocardiography using a standard-frequency ultrasound probe has insufficient spatial resolution to clearly visualize the parietal pericardium (PP). High-frequency ultrasound (HFU) has enhanced axial resolution. The aim of this study was to use a commercially available high-frequency linear probe to evaluate apical PP thickness (PPT) and pericardial adhesion in both normal pericardium and pericardial diseases. METHODS: From April 2002 to March 2022, 227 healthy individuals, 205 patients with apical aneurysm (AA) and 80 patients with chronic constrictive pericarditis (CP) were recruited to participate in this study. All subjects underwent both standard-frequency ultrasound and HFU to image the apical PP (APP) and pericardial adhesion. Some subjects underwent computed tomography (CT). RESULTS: Apical PPT was measured using HFU and found to be 0.60 ± 0.01 mm (0.37-0.87 mm) in normal control subjects, 1.22 ± 0.04 mm (0.48-4.53 mm) in patients with AA, and 2.91 ± 0.17 mm (1.13-9.01 mm) in patients with CP. Tiny physiologic effusions were observed in 39.2% of normal individuals. Pericardial adhesion was detected in 69.8% of patients with local pericarditis due to AA and 97.5% of patients with CP. Visibly thickened visceral pericardium was observed in six patients with CP. Apical PPT measurements obtained by HFU correlated well with those obtained by CT in those patients with CP. However, CT could clearly visualize the APP in only 45% of normal individuals and 37% of patients with AA. In 10 patients with CP, both HFU and CT demonstrated equal ability to visualize the very thickened APP. CONCLUSIONS: Apical PPT measured using HFU in normal control subjects ranged from 0.37 to 0.87 mm, consistent with previous reports from necropsy studies. HFU had higher resolution in distinguishing local pericarditis of the AA from normal individuals. HFU was superior to CT in imaging APP lesions, as CT failed to visualize the APP in more than half of both normal individuals and patients with AA. The fact that all 80 patients with CP in our study had significantly thickened APP raises doubt regarding the previously reported finding that 18% of patients with CP had normal PPT.

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.

Milk of calcium pericardial effusion confirmed with postmortem computed tomography and autopsy findings: A case report.

We present the postmortem computed tomography and autopsy findings of a 60-year-old man who developed milk of calcium pericardial effusion and died of constrictive epicarditis. He experienced out-of-hospital cardiopulmonary arrest, and spontaneous circulation returned at the hospital. However, 7 h after recovery, the patient died. He had a swollen neck, had been experiencing coughing from 4 to 5 days earlier, and had no significant medical or surgical history. On computed tomography (CT), highly dense pericardial fluid (CT value: 130-150 Hounsfield units) and multiple calcifications along the epicardium and pericardium were visualized. The epicardium and pericardium were thick, hard, rough, and widely calcified with mild adhesions on autopsy. The pericardial cavity contained a pale, reddish brown, turbid, and highly viscous liquid. Bacteriological tests of pericardial fluid cultures revealed the presence of group G ß-hemolytic streptococci. Hence, we diagnosed the patient's cause of death as heart failure due to constrictive epicarditis. We believe that this case of milk of calcium pericardial effusion is the first case confirmed with postmortem CT and autopsy findings.

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.

Constrictive epicarditis.

Constrictive pericarditis is relatively uncommon. Constrictive phenomenon involves in the majority of cases the two layers of the pericardium namely the parietal pericardium and the visceral one. Chronic epicarditis is a distinct and very scarce form where only the visceral pericardium is interested by the pathologic process. We present herein the case of a 25 years old patient admitted in our department for surgical treatment of a chronic visceral pericarditis. We discuss along some important clinical and therapeutic points related to this specific presentation with a special interest to the right ventricular dysfunction after pericardiectomy.

Surgical resection of enlarged calcification formed by idiopathic localized constrictive pericarditis during total aortic arch replacement for aortic dissection.

A 75-year-old woman presented at a prior hospital with persistent cough and was treated conservatively for a thrombosed-type aortic dissection (Stanford A). One-year after discharge, follow-up computerized tomography revealed a DeBakey type II, chronic dissecting aortic aneurysm enlarged to 54 mm. She was referred to our hospital with slight edema in the face and extremities and chest radiography showed calcification around the heart. Computerized tomography performed at the prior hospital showed a large spherical mass in the anterior pericardium in addition to the aortic dissection. We therefore resected the mass immediately before a total aortic arch replacement. Surgery was successful and uneventful with patient discharge on postoperative day 21. The final differential diagnosis was idiopathic, localized, constrictive pericarditis.

Severe Calcific Chronic Constrictive Pericarditis as an Unexpected Cause of Death: An Autopsy Case.

Chronic calcific constrictive pericarditis is a rare condition. It can cause severe morbidity and even mortality. The diagnosis may be difficult to establish due to its variable clinical signs. We report an autopsy case of a 54-year-old male with a past medical history of well treated hypertension, diabetes and dyspnea present of 2 weeks, who was discovered dead in his bed. The postmortem examination showed a large band of calcification of the pericardium with obliteration of the pericardial space. Both pericardium and epicardium were thickened with bread-and-butter appearance. Microscopic examination showed thickened pericadium which is formed by a fibrous paucicellular tissue containing extensive basophilic calcifications.

Autoinflammatory constrictive pericarditis and chronic myelomonocytic leukaemia: when one speciality is not enough.

We present a case of constrictive pericarditis with concomitant blood and bone marrow appearances of chronic myelomonocytic leukaemia (CMML). Despite surgical treatment with pericardiectomy, the patient deteriorated into multiorgan failure. Pericardial histology disclosed a typical inflammatory picture with no evidence of monocytic or malignant infiltrate. Following intensive collaboration between cardiologists, haematologists and rheumatologists via daily email exchanges, a diagnosis was reached of autoinflammatory constrictive pericarditis with a non-infiltrative coexisting CMML. The key to achieving a rapid and sustained response was a trial of high-dose steroids followed by intravenous immunoglobulins. This achieved restoration of cardiac function, resolution of symptoms and near normalisation of inflammatory markers. A diagnosis of concurrent CMML was confirmed at 3 months. The patient remains well, taking colchicine and steroids.

A rare cause of ascites in a renal transplant recipient.

Thirty-five-year-old man, underwent renal transplantation 4 years back and was doing well. He now presented with complaints of ascites with engorged neck veins and dyspnoea on exertion for last 6 months. Examination showed elevated jugular venous pressure with two prominent descents, high pitched diastolic heart sound (pericardial knock). Echocardiography showed characteristic features of thickened pericardium, septal bounce, expiratory flow reversal in hepatic veins and phasic variation of mitral inflow, suggestive of constrictive pericarditis. The patient was started on empirical antitubercular therapy and diuretics. The patient symptomatically improved, but in view of persisting constrictive physiology he was planned for pericardiectomy.

Clinical Utility of Cardiac Magnetic Resonance Imaging in Pericardial Diseases.

BACKGROUND: Pericardial diseases are relatively common in clinical practice and encountered in various clinical settings with consequent significant morbidity and mortality. However, the diagnosis as well as management can be complex and challenging, as the clinical presentation is usually non-specific. Therefore, there is an increasing role for Cardiac Magnetic Resonance Imaging (CMR) as an imaging tool to facilitate the diagnosis of pericardial diseases. CONCLUSION: Herein we describe conventional and unique CMR approaches to provide an increased non-invasive understanding of the pericardium in health and disease including a novel method to diagnose constrictive pericarditis via radio-frequency tissue tagging by defining unique visceralparietal adherence patterns easily learned by the cardiologist and radiologist.

Incremental Value of the Tissue Motion of Annular Displacement Derived From Speckle-Tracking Echocardiography for Differentiating Chronic Constrictive Pericarditis From Restrictive Cardiomyopathy.

OBJECTIVES: The tissue motion of annular displacement provides an accurate and rapid assessment of left ventricular (LV) systolic function. However, it has rarely been used in patients with chronic constrictive pericarditis and restrictive cardiomyopathy. This study aimed to assess the differences in LV systolic function in patients with constrictive pericarditis and restrictive cardiomyopathy using tissue motion of annular displacement derived from speckle-tracking echocardiography. METHODS: Twenty-four patients with constrictive pericarditis, 24 with restrictive cardiomyopathy, and 25 healthy volunteers (controls) were enrolled. The septal and lateral mitral annular longitudinal displacements, displacement at the midpoint, and normalized midpoint displacement of the mitral ring were calculated. RESULTS: Mitral annular tracking and quantification of the tissue motion of annular displacement were achieved within 10 seconds. In patients with constrictive pericarditis, the lateral mitral annular longitudinal displacement, displacement at the midpoint, and midpoint displacement of the mitral ring were decreased, whereas the septal mitral annular longitudinal displacement was preserved compared to controls, indicating that the reduction of systolic function in constrictive pericarditis was caused by pericardial adhesion and calcium. In patients with restrictive cardiomyopathy, tissue motion of annular displacement was more reduced compared to patients with constrictive pericarditis and controls. The correlation between the septal mitral annular longitudinal displacement and left ventricular ejection fraction was 0.67 (P < .001). A cutoff value of 8.45 mm for the septal mitral annular longitudinal displacement could effectively differentiate constrictive pericarditis from restrictive cardiomyopathy with 95.2% sensitivity and 91.7% specificity. CONCLUSIONS: The tissue motion of annular displacement was decreased in patients with constrictive pericarditis, which indicated early impairment of longitudinal function in constrictive pericarditis; adhesion and calcium in the pericardium might account for the reduction. The septal mitral annular longitudinal displacement provides a fast and effective method for the assessment of LV systolic function in patients with constrictive pericarditis and restrictive cardiomyopathy.

Transitory effusive-constrictive pericarditis.

Pericardial effusion of various sizes is a quite common clinical finding, while its progression to effusive-constrictive pericarditis occurs in about 1.4-14% of cases. Although available evidence on prevalence and prognosis of this rare pericardial syndrome is poor, apparently a considerable proportion of patients conservatively managed has a spontaneous resolution after several weeks. A 61-year-old female presented to our emergency department reporting fatigue, effort dyspnea and abdominal swelling. The echocardiography showed large pericardial effusion with initial hemodynamic impact, so she underwent a pericardiocentesis with drainage of 800-850cm3 of exudative fluid, on which diagnostic investigations were undertaken: possible viral and bacterial infections, medical conditions, iatrogenic causes, neoplastic and connective tissue diseases were all excluded. Despite empirical therapy with NSAIDs and colchicine, after about one week she had a recurrence of pericardial effusion and progressive development of constriction. Echocardiography performed after a few weeks of anti-inflammatory therapy showed resolution of constriction and PE, with clinical improvement. If progression of pericardial syndromes to a constrictive form is rarely described in literature, cases of transitory effusive-constrictive phase are even more uncommon, mainly reported during the evolution of pericardial effusion. According to the available data, risk of progression to a constrictive form is very low in case of idiopathic pericardial effusion. We report a case of large idiopathic subacute pericardial effusion, treated with pericardiocentesis and then evolved into an effusive-constrictive pericarditis. A prolonged anti-inflammatory treatment leads to complete resolution of pericardial syndrome without necessity of pericardiectomy.

Pericardial interstitial cell senescence responsible for pericardial structural remodeling in idiopathic and postsurgical constrictive pericarditis.

OBJECTIVE: Idiopathic and postsurgical constrictive pericarditis is characterized by pericardial structural remodeling that involves fibrosis, calcification, and inflammation. This study aimed to determine whether cell senescence was responsible for pericardial structural remodeling. METHODS: Pericardial interstitial cells derived from patients with idiopathic or postsurgical pericarditis (pericarditis cells) were harvested. Timing of senescence and differences in telomere length were compared between age- and sex-matched controls (nonpericarditis cells). Pericardial interstitial cells derived from normal pericardia were serially passaged until senescence (senescent cells). Apoptosis, collagen matrix, calcium deposition, chemoattractant properties, gene expression profiles, and paracrine effects of senescent cells were compared with nonsenescent cells of passage 2 (nonsenescent cells). RESULTS: Pericarditis cells displayed senescent changes, including short telomere length, large flattened cell sizes, positive staining for senescence-associated ß-galactosidase, and limited growth capacity. These senescent cells were resistant to apoptosis, produced more collagen matrix, deposited more calcium, and attracted more monocytes/lymphocytes than the nonsenescent cells. A cluster of genes involved in extracellular matrix deposition (connective tissue growth factor, fibronectin, collagen type I, collagen type III, and tissue inhibitors of metalloproteinase-1), calcium deposition (osteopontin, bone sialoprotein, osteonectin, and matrix Gla protein), and inflammatory cell recruitment (interleukin-6, chemoattractant protein-1, and tumor necrosis factor-α) were upregulated in senescent cells, whereas extracellular matrix-degrading enzyme (metalloproteinase-1 and metalloproteinase-3) was downregulated. Furthermore, senescent cells had the ability to promote the proliferation, differentiation, and senescence of neighboring cells. CONCLUSIONS: These findings suggest that senescent cells have characteristics promoting pericardial structural remodeling, but further work is needed to establish causation.

New Cardiac Imaging Algorithms to Diagnose Constrictive Pericarditis Versus Restrictive Cardiomyopathy.

PURPOSE OF REVIEW: Echocardiography is the mainstay in the diagnostic evaluation of constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM), but no single echocardiographic parameter is sufficiently robust to accurately distinguish between the two conditions. The present review summarizes the recent advances in echocardiography that promise to improve its diagnostic performance for this purpose. The role of other imaging modalities such as cardiac computed tomography, magnetic resonance imaging, and invasive hemodynamic assessment in the overall diagnostic approach is also discussed briefly. RECENT FINDINGS: A recent study has demonstrated improved diagnostic accuracy of echocardiography with integration of multiple conventional echocardiographic parameters in to a step-wise algorithm. Concurrently, the studies using speckle-tracking echocardiography have revealed distinct and disparate patterns of myocardial mechanical abnormalities in CP and RCM with their ability to distinguish between the two conditions. The incorporation of machine-learning algorithms into echocardiography workflow permits easy integration of the wealth of the diagnostic data available and promises to further enhance the diagnostic accuracy of echocardiography. New imaging algorithms are continuously being evolved to permit accurate distinction between CP and RCM. Further research is needed to validate the accuracy of these newer algorithms and to define their place in the overall diagnostic approach for this purpose.