A novel multi-task machine learning classifier for rare disease patterning using cardiac strain imaging data.

To provide accurate predictions, current machine learning-based solutions require large, manually labeled training datasets. We implement persistent homology (PH), a topological tool for studying the pattern of data, to analyze echocardiography-based strain data and differentiate between rare diseases like constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). Patient population (retrospectively registered) included those presenting with heart failure due to CP (n = 51), RCM (n = 47), and patients without heart failure symptoms (n = 53). Longitudinal, radial, and circumferential strains/strain rates for left ventricular segments were processed into topological feature vectors using Machine learning PH workflow. In differentiating CP and RCM, the PH workflow model had a ROC AUC of 0.94 (Sensitivity = 92%, Specificity = 81%), compared with the GLS model AUC of 0.69 (Sensitivity = 65%, Specificity = 66%). In differentiating between all three conditions, the PH workflow model had an AUC of 0.83 (Sensitivity = 68%, Specificity = 84%), compared with the GLS model AUC of 0.68 (Sensitivity = 52% and Specificity = 76%). By employing persistent homology to differentiate the "pattern" of cardiac deformations, our machine-learning approach provides reasonable accuracy when evaluating small datasets and aids in understanding and visualizing patterns of cardiac imaging data in clinically challenging disease states.

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.

Pericardiectomy for Constrictive Pericarditis with or without Cardiopulmonary Bypass.

Aim: We aim to access the effect of pericardiectomy for constrictive pericarditis with or without cardiopulmonary bypass. Methods: This was a review of pericardiectomy for constrictive pericarditis. Results: Cardiopulmonary bypass is actually an important maneuver to attain complete relief of the constriction. The short additional time of cardiopulmonary bypass during the procedure has very little effect on the risk of morbidity of the main operation. Conclusion: Incomplete pericardiectomy perhaps was the cause of postoperative remnant constriction and high diastolic filling pressure leading to multiorgan failure. Complete pericardiectomy (removal of phrenic-to-phrenic and the postero-lateral and inferior wall pericardial thickening) using cardiopulmonary bypass should be the routine for total relief of the constriction of the heart.

The clinical value of P-wave terminal force in lead V1 in evaluating pericardial thickness in tuberculous constrictive pericarditis.

AIM: To investigate the relationship between p wave terminal force (Ptfv1) and pericardial thickness in patients with tuberculous constrictive pericarditis. METHODS: From January 2018 to October 2022, 95 patients with tuberculous constrictive pericarditis who needed pericarditis dissection in a hospital were collected, and 3 patients who did not meet the criteria were excluded, a total of 92 cases. The absolute value of Ptfv1 in conventional electrocardiogram was tested before surgery, and pericardial thickness was measured by echocardiography and chest CT. Pericardial thickness was measured after pericardial dissection. Pearson correlation analysis was used, R software was used to make scatter plot, and non-parametric square test was used. The correlation of postoperative measurements with echocardiography, chest CT and absolute value of Ptfv1 was analyzed. RESULTS: Pearson correlation analysis was conducted with postoperative measurements and echocardiography measurements, postoperative measurements and chest CT measurements, and postoperative measurements and absolute value of Ptfv1. Pearson correlation analysis showed that the correlation coefficients between postoperative measurements and echocardiography, chest CT and Ptfv1 values were statistically significant. Scatter plot and nonparametric Chi-square test showed that postoperative measurements were consistent with absolute values of echocardiography, chest CT and Ptfv1 (p < 0.05). And this study found that the distribution of the value of Ptfv1 ≥ 5 was higher than the value of Ptfv1 < 5 after pericardiectomy (0.95:0.05) in the absolute value of Ptfv1 ≥ 0.04 which measured before pericardiectomy. The hypothesis was statistically significant (p < 0.05). CONCLUSION: The absolute value of Ptfv1 in electrocardiogram can be used as an auxiliary diagnostic index to evaluate pericardial thickness in tuberculous constrictive pericarditis.

Echocardiography-Based Deep Learning Model to Differentiate Constrictive Pericarditis and Restrictive Cardiomyopathy.

BACKGROUND: Constrictive pericarditis (CP) is an uncommon but reversible cause of diastolic heart failure if appropriately identified and treated. However, its diagnosis remains a challenge for clinicians. Artificial intelligence may enhance the identification of CP. OBJECTIVES: The authors proposed a deep learning approach based on transthoracic echocardiography to differentiate CP from restrictive cardiomyopathy. METHODS: Patients with a confirmed diagnosis of CP and cardiac amyloidosis (CA) (as the representative disease of restrictive cardiomyopathy) at Mayo Clinic Rochester from January 2003 to December 2021 were identified to extract baseline demographics. The apical 4-chamber view from transthoracic echocardiography studies was used as input data. The patients were split into a 60:20:20 ratio for training, validation, and held-out test sets of the ResNet50 deep learning model. The model performance (differentiating CP and CA) was evaluated in the test set with the area under the curve. GradCAM was used for model interpretation. RESULTS: A total of 381 patients were identified, including 184 (48.3%) CP, and 197 (51.7%) CA cases. The mean age was 68.7 ± 11.4 years, and 72.8% were male. ResNet50 had a performance with an area under the curve of 0.97 to differentiate the 2-class classification task (CP vs CA). The GradCAM heatmap showed activation around the ventricular septal area. CONCLUSIONS: With a standard apical 4-chamber view, our artificial intelligence model provides a platform to facilitate the detection of CP, allowing for improved workflow efficiency and prompt referral for more advanced evaluation and intervention of CP.

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.

Multimodality Imaging in Differentiating Constrictive Pericarditis From Restrictive Cardiomyopathy: A Comprehensive Overview for Clinicians and Imagers.

In the evaluation of heart failure, 2 differential diagnostic considerations include constrictive pericarditis and restrictive cardiomyopathy. The often outwardly similar clinical presentation of these 2 pathologic entities routinely renders their clinical distinction difficult. Consequently, initial assessment requires a keen understanding of their separate pathophysiology, epidemiology, and hemodynamic effects. Following a detailed clinical evaluation, further assessment initially rests on comprehensive echocardiographic investigation, including detailed Doppler evaluation. With the combination of mitral inflow characterization, tissue Doppler assessment, and hepatic vein interrogation, initial differentiation of constrictive pericarditis and restrictive cardiomyopathy is often possible with high sensitivity and specificity. In conjunction with a compatible clinical presentation, successful differentiation enables both an accurate diagnosis and subsequent targeted management. In certain cases, however, the diagnosis remains unclear despite echocardiographic assessment, and additional evaluation is required. With advances in noninvasive tools, such evaluation can often continue in a stepwise, algorithmic fashion noninvasively, including both cross-sectional and nuclear imaging. Should this additional evaluation itself prove insufficient, invasive assessment with appropriate expertise may ultimately be necessary.

Transient pericardial constriction: A not so rare entity.

Constrictive pericarditis is a rare, potentially treatable, cause of heart failure with preserved ejection fraction that is characterized by insidious onset, challenging diagnosis and dismal prognosis, even following complete surgical pericardiectomy, particularly in advanced disease stages. In recent years it has been proposed that transient pericardial constriction may occur, with an even rarer frequency, during early phases of acute pericarditis and may resolve following specific treatment without progressing to the chronic, irreversible form. We recently observed two cases of well-documented transient pericardial constriction. In the present work we describe these two cases and provide a review on this rare condition, that, if unrecognized and left untreated, may lead to irreversible constrictive pericarditis.

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.

Pattern of pericardial calcification determines mid-term postoperative outcomes after pericardiectomy in chronic constrictive pericarditis.

OBJECTIVES: Although pericardiectomy is an effective treatment for constrictive pericarditis (CP), clinical outcomes are not always successful. Pericardial calcification is a unique finding in CP, although the amount and localization of calcification can vary. We investigated how the pattern and amount of pericardial calcification affect mid-term postoperative outcomes after pericardiectomy to treat CP. METHODS: All patients of total pericardiectomy in our hospital from 2010 to 2020 were enrolled. Preoperative Computed tomography (CT) scans of 98 consecutive patients were available and analyzed. Medical records were reviewed retrospectively. Cardiovascular events were defined as cardiovascular death or hospitalization associated with a heart failure symptom, and all-cause events were defined as any event that required admission. CT scans were analyzed, and the volume and localization pattern of peri-calcification were determined. Pericardium calcium scores are presented using Agatston scores. RESULTS: Of the 98 patients, 25 (25.5%) were hospitalized with heart failure symptoms after pericardiectomy. The median follow-up duration for all patients was 172 weeks. The group with a cardiovascular event had a lower calcium score than patients without an event. Multivariate Cox proportional analysis showed that high ln(calcium score+1) before pericardiectomy was a dependent predictor of cardiovascular event (hazard ratio, 0.90; p = 0.04) after pericardiectomy. When we set the cut-off value (ln(calcium score+1) = 7.22), there was a significant difference in cardiovascular events in the multivariate Cox proportional analysis (p = 0.04). CONCLUSION: A low burden of pericardial calcification was associated with a high rate of mid-term clinical events after pericardiectomy to treat CP.

Cardiac magnetic resonance imaging of pericardial diseases: a comprehensive guide.

Cardiac magnetic resonance (CMR) imaging has been established as a valuable diagnostic tool in the assessment of pericardial diseases by providing information on cardiac anatomy and function, surrounding extra-cardiac structures, pericardial thickening and effusion, characterization of pericardial effusion, and the presence of active pericardial inflammation from the same scan. In addition, CMR imaging has excellent diagnostic accuracy for the non-invasive detection of constrictive physiology evading the need for invasive catheterization in most instances. Growing evidence in the field suggests that pericardial enhancement on CMR is not only diagnostic of pericarditis but also has prognostic value for pericarditis recurrence, although such evidence is derived from small patient cohorts. CMR findings could also be used to guide treatment de-escalation or up-titration in recurrent pericarditis and selecting patients most likely to benefit from novel treatments such as anakinra and rilonacept. This article is an overview of the CMR applications in pericardial syndromes as a primer for reporting physicians. We sought to provide a summary of the clinical protocols used and an interpretation of the major CMR findings in the setting of pericardial diseases. We also discuss points that are less well clear and delineate the strengths and weak points of CMR in pericardial diseases.

Advances in multi-modality imaging for constrictive pericarditis and pericardial inflammation: role of imaging-guided therapy.

INTRODUCTION: Constrictive pericarditis (CP) can result from uncontrolled inflammation of the pericardium. This can be due to various etiologies. CP can lead to both left- and right-sided heart failure with associated poor quality of life, so early recognition is key. The evolving role of multimodality cardiac imaging allows for earlier diagnosis and facilitates management to help mitigate this adverse outcome. AREAS COVERED: This review discusses the pathophysiology of constrictive pericarditis, chronic inflammation and autoimmune etiologies, clinical presentation of CP, and advances in multimodality cardiac imaging for diagnosis and management. Echocardiography and cardiac magnetic resonance (CMR) imaging remain cornerstone modalities to evaluate this condition, whereas additional imaging modalities such as computed tomography and FDG-positron emission tomography can provide complementary information. EXPERT OPINION: Advances in multimodality imaging allow for a more precision diagnosis of constrictive pericarditis. There has been a paradigm shift in pericardial disease management with advances in multimodality imaging, especially CMR, to detect subacute and chronic inflammation. This has enabled imaging-guided therapy (IGT) to both help prevent and potentially reverse established constrictive pericarditis.

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.

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.