Abstract Objective: To study the epidemiological, clinical, paraclinical and therapeutic profile of patients who underwent pericardial decortication. Patients and methods: Retrospective study of 11 patients operated on at the Cuomo Cardiothoracic Surgery Center of Fann University Hospital between 2017 and 2022. Results: Male predominance (n = 7). Mean age = 30.5 years. Exertional dyspnea (9 patients) and right heart failure (7 patients) were the most common presenting symptoms.The main etiology was idiopathic. Pericardial calcifications, observed in 5 patients, were associated with a poor prognosis. Subtotal pericardiectomy via median sternotomy was performed in 7 cases, without extracorporeal circulation. Eight patients had uneventful postoperative courses. Cardiac rhythm disorders and low cardiac output were the main complications. There were 3 in-hospital deaths and one late death at 6 months. After 6 years, 4 of the 7 followed-up patients were asymptomatic. Conclusion: Although tuberculosis remains classically the leading cause of chronic constrictive pericarditis in developing countries, our series did not confirm this predominance, with idiopathic etiology being the most frequent. Pericardiectomy remains the curative treatment of choice, allowing significant improvement in symptoms and functional outcomes when the resection is complete and performed early. Keywords: Chronic constrictive pericarditis – Calcifications – Decortication.

Pericardiectomy remains the standard treatment in constrictive pericarditis. The study was aimed to assess the clinical characteristics, etiology and the outcome of patients who underwent pericardiectomy for chronic constrictive pericarditis. Single center based retrospective cohort study was conducted on the patients who underwent standard pericardiectomy at our center from January 2021 to December 2023. Structured questionnaire was used to observe the record of the participants. Data was entered in Epi-data and exported to IBM SPSS Statistics version 16 for analysis. Ethical approval was obtained from Institute Review Committee, Nobel Medical College and Teaching Hospital (Ref no: 121/2024). The study involved 17 patients, with a mean age of 41.76±13.16 years. Male were higher in number with 12 (70.60%) of total cases. The echocardiography findings included annulus reversus, septal bounce, calcified pericardium, and congested Inferior Vena-Cava. Post-operatively, all 17 patients improved to New York Heart Association (NYHA) function class I or II (11 and 6 patients respectively) from (NYHA) functional class III or IV. The average intensive care unit stay was 3±0.7 days and hospital stay were 9.88±2.86 days. Histopathology report revealed tuberculosis in 15 (88.23%) cases. The most common cause of constrictive pericarditis was tuberculosis and symptomatic improvement was noticed in all patients.

Constrictive pericarditis is a condition in which inflammation of the pericardium results in the loss of pericardial elasticity, leading to restricted ventricular filling. This case reports a male in his 50s who presented with symptoms of bilateral pedal oedema and dyspnoea. Examination revealed a raised jugular venous pulse, abdominal dullness and crepitations in both lungs. Echocardiography and cardiac computed tomography revealed the characteristic features of chronic constrictive pericarditis, including septal involvement, calcific deposits and right-sided heart failure. The patient was also diagnosed with chronic liver cirrhosis. The patient underwent a surgical pericardiectomy. Histopathological examination of the pericardial tissue confirmed a tuberculous aetiology. The patient was postoperatively managed for heart failure and antitubercular medication and was subsequently discharged. The patient was readmitted with symptoms of right-sided heart failure and eventually died. This case highlights that constrictive pericarditis with myocardial involvement has poor outcomes after intervention, emphasising the need for early diagnosis.

Normal pericardium plays a fundamental role in the maintenance of left ventricular (LV) twists. Pericardial diseases are among the important causes of morbidity and mortality in cases with cardiovascular diseases. The thorough management of suspected pericardial diseases requires early diagnosis using optimal imaging modalities for each patient. Strain and strain rate could be calculated using two-dimensional speckle-tracking echocardiography (STE) through tracking frame-to-frame movements of myocardial tissue. This modality could also determine the LV torsion and rotation. Ultrasonic strain imaging can evaluate LV function without being influenced by the whole heart motion or even tethering forces. This imaging modality could also identify the disease at earlier stages. This report reviews the role of strain imaging by echocardiography in the assessment of pericardial diseases including acute pericarditis, perimyocarditis, chronic constrictive pericarditis, effusive constrictive pericarditis, and transient constriction, pericardial tamponade, post pericardiectomy, pericardial cysts, masses, diverticula and congenital absence of the pericardium. Being familiar with obtaining data from strain imaging using STE could help pericardial specialists working in established pericardial centers of excellence.

Chronic constrictive pericarditis (CCP) is a debilitating condition characterized bythickening and fibrosis of the pericardium, leading to impaired diastolic filling and reduced cardiac output. Patients typically present with symptoms of right-sided heart failure, such as peripheral edema, ascites, and dyspnea. Definitive treatment involves surgical pericardiectomy, which aims to remove the constrictive pericardium and restore normal cardiac function. This study aims to assess the operative and short-term outcome of this rare disease in the northeastern part of India. Retrospective data records of patients who underwent pericardiectomy were analyzed from 2011 to 2020. Of the patients,20 (47.6%) were inNew York Heart Association (NYHA) class III, and 12 (28.6%) were in NYHAclass IV. Hyperbilirubinemiawas seen in 29 (69%) patients, and hypoalbuminemiain 18 (42.9%) patients. Radical pericardiectomy was done in 36 (85.7%) cases, and the waffle operation was done in five (12%) cases. The mean ICU stay post procedure was 5.81 days (2-18 days), and the mean hospital stay was 19.6 days (2-49 days). The in-hospital mortality rate was 14.6% (six cases), and the cause of death in all cases was persistent low cardiac output. Pericardiectomy is the only definitive treatment for symptomatic CCP, and it gives a chance to the patient for a full recovery from the chronic morbidity and mortality it is associated with. The right time for surgical intervention is still not clear and needs to be individualized for each patient, but the earlier the intervention, the better the short-term survival.

Myocarditis and pericarditis represent inflammatory conditions of cardiac tissue. A variety of etiologies can result in myocarditis and pericarditis, including inflammatory and infectious causes. This chapter aims to provide a detailed overview of the various diagnostic methods for these conditions, focusing on the utility of imaging tests—echocardiography, cardiac CT, 18F FDG-PET, and CMR in the diagnostic work-up, utility, diagnostic features, and limitations of the various modalities. The role of imaging in the diagnosis of recurrent pericarditis and chronic constrictive pericarditis is discussed in this chapter. A brief overview of the management of myocarditis and pericarditis is also presented.

Constrictive pericarditis, an uncommon cause of diastolic heart failure, is reversible if identified and treated promptly and appropriately. The treatment approach may vary significantly depending on the disease stage of constrictive pericarditis. Here, we report a case of 18 F-FDG and 68 Ga-FAPI-04 PET/CT imaging in a 70-year-old woman with chronic constrictive pericarditis. The thickened pericardium showed mild uptake of 18 F-FDG but intense uptake of 68 Ga-FAPI-04, indicating that the lesion was at the fibrotic phase. This case suggests a promising role of combination of 18 F-FDG and 68 Ga-FAPI PET/CT in accurately and timely assessing the lesion stage in constrictive pericarditis.

About 15 – 30% of patients develop recurrent episodes of pericarditis following an acute pericarditis attack. In developed countries, most cases of pericarditis are of idiopathic etiology. First-line therapy typically includes non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine, with corticosteroids being the traditional second-line agents. Anti-interleukin-1 (IL-1) agents are a novel treatment option increasingly utilized due to their high efficacy as an alternative second-line therapy or for resistant cases, while pericardiectomy remains the last resort. Special populations with recurrent pericarditis (RP), including patients at the extremes of age or during pregnancy, have been understudied. In some cases, pericarditis may develop secondary to infections (including viral infections, such as coronavirus disease 2019, bacterial infections, such as tuberculosis, and fungal infections), autoimmune diseases (such as systemic lupus erythematosus, rheumatoid arthritis, vasculitis, and inflammatory bowel disease), post-cardiac injury syndromes, cancer, and other rare conditions. Non-idiopathic etiologies are associated with a higher risk of RP, chronic constrictive pericarditis, and cardiac tamponade. The general treatment algorithm may not be applicable to these special populations due to patient-related or etiological factors. For example, NSAIDs or corticosteroids are often contraindicated in older patients due to comorbidities. Bacterial or fungal purulent pericarditis requires aggressive treatment of infection followed by pericardial fluid drainage, with corticosteroids and anti-IL-1 agents contraindicated in these cases. Therefore, management often requires a multidisciplinary approach and must take place at a specialized pericardial center to optimize patient outcomes. In this review, we present current evidence on the evaluation and management of RP in the aforementioned special populations.

Abstract Export Introduction: The leading cause of chronic constrictive pericarditis differs according to geographic location. Tuberculosis remains the most common cause of constrictive pericarditis in Africa and Asia, whereas in western countries, it remains a rare entity and idiopathic causes remain the most common etiology. Here, our aim was to study the clinical and echocardiography outcomes postpericardiectomy and compare it with other previous clinical studies. Materials and Methods: This is a retrospective study of 12 months with a mean follow-up period of 2.40 ± 2.01 years. PHILIPS EPIQ 7C machine was used for echocardiographic analysis. Mitral and tricuspid inflow velocities were detected using pulsed-wave Doppler in apical four-chamber view with a sample volume of 2–4 mm. Results: All the patients in the study population presented with dyspnea 19 (100%). Annulus reversus was observed in all our patients (19, 100%), which resolved completely postoperatively. The mean duration of hospital stay was 26.63 ± 13.09 days with a mean intensive care unit stay of 5.89 ± 2.4 days. Discussion: CVP decreased from a mean of 31.9±4.89mmHg preoperatively to 12.95 ± 3.84 mmHg in the immediate postoperative period. Significant reduction (P < 0.001) was noted in inspiratory mitral E velocity without significant change in expiratory mitral E velocity. Significant respirophasic variation was noted in all patients’ mitral and tricuspid E velocity (P < 0.001). Discussion: Different from international studies, we observed pericarditis in younger age group with a mean age of 26.58 ± 11.9 years which could be accountable to tuberculosis in young generation in India, as also observed by other Indian studies. In our study, there was significant respirophasic variation in the mitral and tricuspid E velocity in all patients (P < 0.001). Conclusions: Studies on detailed echocardiographic evaluation in terms of various parameters like mitral and tricuspid E velocities and tissue Doppler imaging are sparse. This study adds to the important role of echocardiography in assessment of chronic constrictive pericarditis.

Background: Constrictive pericarditis (CP) is a rare condition characterized by pericardial rigidity, impairing cardiac filling and causing systemic congestion. Pericardiectomy is the definitive treatment but carries significant morbidity and mortality. Limited data exist on predictors of outcomes following surgery. Methods: This retrospective study analyzed 53 patients with CP who underwent pericardiectomy at IGIMS, Patna, between December 2021 and October 2024. Clinical, laboratory, imaging, and intraoperative data were collected. Mortality predictors were evaluated using univariate, multivariate, and survival analyses. Results: The study cohort (mean age: 59 years; 67.9% male) showed high prevalence of NYHA class III–IV symptoms, peripheral oedema (54.7%), and pericardial thickening (88.7%). Over 70% had preserved LV systolic function, but 67% had diastolic dysfunction. Complete pericardiectomy was performed in 54.7% of patients. The 30-day mortality was 18.9%, with significant postoperative complications including dialysis (30.2%) and reintubation (26.4%). Conclusion: Constrictive pericarditis presents with apparently preserved LV function but complex haemodynamics and significant comorbidities. Surgical pericardiectomy, though effective, carries considerable perioperative risk.

Introduction: Leading cause of chronic constrictive pericarditis differs according to geographic location, tuberculosis remains the most common cause of constrictive pericarditis in Africa and Asia where as in western country it remains a rare entity and idiopathic causes remains the most common etiology. Here our aim was to study the clinical and echocardiography outcomes post pericardiectomy &amp; compare it with other previous clinical studies. Methods: This is a retrospective study of 12 months with mean follow up period of 2.40 ± 2.01 years. PHILIPS EPIQ7C machine was used for echocardiographic analysis. Mitral and tricuspid inflow velocities were detected using PW doppler in apical 4-chamber view with sample volume of 2-4 mm. Results: All the patients in the study population presented with dyspnea 19(100%). Annulus reversus was observed in all our patients (19, 100%), which resolved completely postoperatively. Mean duration of hospital stay was 26.63 ± 13.09 days with mean ICU stay of 5.89 ± 2.4 days. Discussion: Different from international studies, we observed pericarditis in younger age group with mean age of 26.58 ± 11.9 years which could be accountable to Tuberculosis in young generation in India, as also observed by other indian studies. In our study, there was significant respiro-phasic variation in the mitral and tricuspid E velocity in all patients. Conclusions: Studies on detailed echocardiographic evaluation in terms of various parameters like mitral and tricuspid E velocities and tissue doppler imaging are sparce. This study adds to the important role of echocardiography in assessment of chronic constrictive pericarditis.

The present study was designed to identify the preoperative clinical and imaging findings influencing adverse clinical outcomes in patients with chronic constrictive pericarditis after pericardiectomy. Patients with constrictive pericarditis who underwent pericardiectomy between January 2009 and September 2023 were retrospectively analyzed. Preoperative evaluations included assessments of clinical symptoms, comorbidities, laboratory tests, cardiac computed tomography (CT), and transthoracic echocardiography. The volume of pericardial calcifications was quantified on calcium scoring CT. Adverse clinical events were defined as cardiovascular death or hospitalization due to cardiac causes, and all-cause mortality was assessed. Univariable and multivariable Cox proportional hazard model analysis were performed to find factors associated with adverse clinical events. Among the 91 patients with available preoperative CT scans, 26 (28.6%) experienced adverse clinical events after pericardiectomy, with 19 (20.9%) experiencing cardiovascular deaths. On multivariable Cox analysis, larger pericardial calcium volume hazard ratio [HR], 1.004 (95% confidence interval [CI], 1.001–1.006) per 1cm3 increase; p = 0.005), higher E/E’ ratio (HR, 1.059, 95% CI, 1.015–1.105, p = 0.008), and lower albumin level (HR, 0.476, 95% CI, 0.229–0.986, p = 0.046) were significant factors associated with the adverse clinical events after pericardiectomy. The amount of pericardial calcification could be associated with the efficacy of pericardiectomy and potentially have implications for postoperative outcomes. Additionally, a high E/E ratio on echocardiography is indicative of unfavorable postoperative prognosis.

Introduction Chronic constrictive pericarditis (CCP) is a progressive disease characterized by thickening and fibrosis of the pericardium, leading to restricted diastolic filling and impaired cardiac function. In Northeast India, limited healthcare infrastructure poses challenges in diagnosing and managing CCP, potentially worsening patient outcomes. Therefore, this study aims to assess the demographic and clinical profiles of patients with CCPin this regionover 10 years and compare our findings to the relevant but limited published literature in South Asia. Methods We retrospectively analyzed the medical records of 42 patients diagnosed with CCP referred to our department from 2011 to 2020. Demographic, clinical, and paraclinical data during hospitalization and follow-up were collected. All patients underwent clinical examination imaging studies, including high-resolution chest computed tomography and echocardiography. Symptomatic patients underwent open pericardiectomy, and postoperative histopathological examination was performed. Results Of the 42 patients, 34 (81%) were male and eight (19%) were female. A significant proportion of the patients were under the age of 20 years (n = 18, 42.9%), followed by the 21- to 40-year age group (n = 13, 31%). Eighteen patients (42.9%) were from Meghalaya, 12 (28.6%) were from Assam, and seven (16.7%) were from Nagaland. Twenty-two patients (52.4%) had a prior history of pulmonary or extrapulmonary tuberculosis (TB) and had received antitubercular therapy for at least six months. Dyspnea on exertion was the most common symptom among the patients: 12 (28.6%) of them were classified as New York Heart Association class IV, 20 (47.6%) as class III, and 10 (23.8%) as class II. Clinical examination revealed pedal edema in 36(85.7%), hepatomegaly in 22 (52.4%), ascites in 15 (35.7%), and pleural effusion in 13 (31%) patients. Echocardiography showed septal bounce in 26 (61.9%), pericardial calcification in 17 (40.5%), and hepatic vein flow reversal in 12 (28.6%) patients. All patients were on diuretics and digoxin before surgery. Postoperative biopsy confirmed TBas the etiology in 28 (66.7%) patients and nonspecific etiology in 14 (33.3%) of them. Conclusions CCP in Northeast India predominantly affects young males and is largely associated with TB. Despite efforts to control TB, it remains a major contributor to CCP in this region. Recognizing the clinical presentation and diagnostic profile is essential for improving management strategies and patient outcomes.

An observation from practice is given tuberculous constrictive pericarditis, which has been occurring for a long time with symptoms of polyserositis without identifying of etiology. The recurrent course of the disease, the constant accumulation of effusion in the abdominal and pleural cavities was the basis for the diagnosis: "compressive pericarditis complicated by calcification of unknown genesis and of constant patronage of cardiologists. Considering the tuberculous process suffered more than 10 years ago, to exclude the tuberculous etiology of polyserositis, the patient underwent pericardial resection. According to the results of microbiological examination of the surgical material, the tuberculous nature of pericarditis was established, however, according to the results of histological examination, no foci of necrosis and granuloma elements were found. The clinical case demonstrates the complexity of the diagnosis of tuberculous constrictive pericarditis with the phenomena of pronounced polyserositis, which was detected only as a result of diagnostic surgery: partial excision of the pericardium.

e23073 Background: Pericardial complications (PC) are common in malignancy as sequelae of metastasis, direct toxicity of radiation or chemotherapy, or pericardial infection/inflammation due to malignancy. The goal of this study was to assess the cardiovascular outcomes of pericardial complications among patients with solid and liquid cancer. Methods: The National Inpatient Sample from the years 2015 to 2018 was queried to assess cardiovascular outcomes in the setting of pericardial complications (acute pericarditis, chronic constrictive pericarditis, chronic adhesive pericarditis, hemopericardium, cardiac tamponade and other unspecified pericarditis) for patients that have any solid or liquid malignancy. Outcomes included in-hospital mortality, acute kidney injury (AKI), heart failure (HF), cardiogenic shock (CS), myocardial infarction (MI), sudden cardiac arrest (SCA), mechanical circulatory support (MCS), major adverse cardiovascular and cerebrovascular events (MACCE), and stroke. Outcomes were assessed using chi-square analysis using STATA. Results: A total of 1,183,619 cancer patients with PC (n = 20,410.00; 1.7%) and without PC (n = 1,163,209.60; 98.3%) were included. The mean age of patients with and without PC was 58 and 59 years, respectively. The average length of stay in the hospital was 14 and eight days in patients with and without PC, respectively. The most common complications were electrolyte disorders (51.2%, p = 0.000) and arrhythmias (40.4 %, p = 0.000) for patients with and without PC. Patients with PC had higher incidence of in-hospital mortality (11.7% vs 5.1%, p = 0.000), AKI (37.8% vs 22.1%, p = 0.000), HF (25.7% vs 15.5%, p = 0.000), CS (2.7% vs 0.4%, p = 0.000), MI (3.6% vs 2.0%, p = 0.000), SCA (3.2% vs 1.0%, p = 0.000), MCS (0.7% vs 0.1%, p = 0.000), and MACCE (17% vs 7.9%, p = 0.000) compared to cancer patients without PC. There was no difference in outcomes in terms of stroke (1.2% vs 1.2%, p = 0.879). The yearly trend was stable for length of stay among both groups while adjusted total hospital cost was progressively increasing from 2015 to 2018, with a greater net increase in those with PC. Yearly trends of complications were stable among both cohorts. Conclusions: Pericardial complications are prevalent in up to 1.7% of cancer patients, and they are associated with worse cardiovascular outcomes as compared to patients without pericardial complications. Patients with PC also have a higher incidence of in-hospital mortality.

Chronic constrictive pericarditis (CP) is an uncommon form of heart failure in which a thickened and often calcified pericardium limits diastolic filling, ultimately reducing cardiac pump performance. Diagnosis can be challenging and may require integrating clinical data with meticulous echocardiographic assessment and cross-sectional cardiac imaging. If non-invasive testing is inconclusive, right heart catheterization remains the gold standard diagnostic test. Classic hemodynamic features of constrictive pericarditis may also be seen in restrictive cardiomyopathy and are often insufficient to establish the diagnosis. Therefore, the preferred hemodynamic criteria of CP include the finding of increased ventricular interdependence and intrathoracic and intracardiac pressure dissociation. However, the presence of concomitant pleural disease may interfere with the catheterization hemodynamics and decrease the accuracy of these criteria as we present in this case report.

Gestion de la péricardite constrictive : à travers l’étude de 43 cas opérés

Abstract Juvenile idiopathic arthritis (JIA), a common rheumatic disease of childhood, is a consolidated term used for all chronic childhood arthritis affecting joints and extra-articular structures. Involvement of skin and internal organs such as liver, spleen, intestine, peritoneum, pleura, pericardium and myocardium will cause systemic symptoms leading to disability and mortality. Despite advanced treatment options, significant morbidity still occurs due to an unpredictable clinical course and risk of joint restriction. We describe polyarticular rheumatoid factor-negative JIA with extra-articular manifestations involving pericardium causing chronic constrictive pericarditis in a 16-year-old girl and her sibling a 13-year-old boy. The female patient developed cardiac cirrhosis, portal hypertension and oesophageal varices. Screening evaluation of this patient’s asymptomatic sibling with similar articular manifestations revealed the rare occurrence of chronic constrictive pericarditis without symptoms. Both were treated with pericardiectomy and were relieved of symptoms. Two patients of JIA from the same family presenting with constrictive pericarditis is a rare association.

Introduction: Studies have been conducted on patients with constrictive pericarditis but there is no literature on the factors associated with mortality among hospitalized patients in this population. We examined the predictors of mortality among patients hospitalized with chronic constrictive pericarditis with or without arrhythmias. Hypothesis: Patient and hospital associated factors can predict mortality among hospitalized patients with chronic constrictive pericarditis. Methods: Retrospective cohort analyses were conducted using data from the National Inpatient Sample (NIS), 2016-2020. Multivariate logistic regression modelling was used to examine the factors associated with mortality among hospitalized patients with chronic constrictive pericarditis with or without arrhythmias. Results: Among those with arrhythmias, African Americans (AOR: 1.83; CI: 1.36-2.48) and Hispanics (AOR: 2.08; CI: 1.43-3.01) had higher odds of mortality compared to non-Hispanic whites. Those admitted electively were 62% (AOR: 0.38; CI: 0.27-0.53) less likely to die than the non-elective cases. Length of stay of more than 5 days, (AOR: 1.78; CI: 1.41-2.24) was associated with higher odds of mortality relative to 5 days or less. Other predictors of mortality were charlson comorbidity index and median household income national quartiles. Furthermore, among those without arrhythmias, older age (AOR: 1.88; CI: 1.37-2.59), and longer length of hospital stay (AOR: 2.87; CI: 2.27-3.63) were associated with higher odds of mortality. Conclusions: Our findings highlight the predictors of mortality among patients hospitalized with chronic constrictive pericarditis. Additional studies are needed to further explore patient and hospital related factors associated with mortality, especially among those with comorbid arrhythmias. This will enable physicians to identify high risk patients who may benefit from closer monitoring.

BackgroundConstrictive 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 presentationWe 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.ConclusionAlbeit 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.