Constrictive Pericarditis as a Rare Cause of Recurrent Miscarriage

Background: Constrictive pericarditis is a rare but important cause of diastolic heart failure. Its uniqueness lies in the fact that surgery (pericardiectomy) remains the mainstay of treatment. Globally, the leading causes of constrictive pericarditis include idiopathic, mediastinal irradiation, post cardiac surgery and tuberculosis. Methods: Patients who underwent pericardiectomy at our tertiary hospital between January 2019 and December 2024 were retrospectively studied with the aim of discussing our experience with the procedure and its outcomes. Data including baseline demographics, preoperative conditions, intraoperative details, and postoperative outcomes were collected from clinical records and analysed. Results: Thirteen patients had total pericardiectomy during the period under review. The median age was 28 years with dyspnoea and ascites being the most common symptoms. Most patients (61.6%) presented in NYHA class III and IV and were above ASA II classification at the time of surgery. Pericardiectomy was done via median sternotomy and without cardiopulmonary bypass in all cases, with an average surgery duration of 284.5 mins. Postoperative complications included low cardiac output, acute kidney injury, coagulopathy, and prolonged pleural effusion. Median duration of intensive care unit (ICU) stay was 2days and there was 1 mortality. At twelve months follow up, more than 90% of surviving patients were in NYHA class I or II. Conclusion: Pericardiectomy offers symptomatic relief to patients with constrictive pericarditis. Early identification of this disease would prevent disease progression and offer improved outcomes. Keywords: Constrictive pericarditis; pericardiectomy; diastolic heart failure; New York heart association (NYHA).

Background: Blunt cardiac injury (BCI) encompasses a spectrum of conditions resulting from chest trauma. While most patients, particularly those with minor injuries, recover fully, a subset with severe trauma faces significant acute and delayed complications. The diagnosis and management of BCI are challenging due to its variable presentation and the lack of universal diagnostic criteria. Aim: This article aims to review the potential complications, long-term management strategies, and the critical role of interprofessional collaboration in optimizing outcomes for patients with BCI. Methods: The approach is based on a review of clinical practices and consensus. It advocates for a systematic management protocol involving initial screening with electrocardiography (ECG) and troponin levels, inpatient monitoring for high-risk patients, and structured medium-term follow-up. Results: Most patients with minor BCI recover without lasting consequences. However, severe injuries can lead to acute complications (e.g., chamber rupture) or delayed sequelae such as heart failure, arrhythmias, pericardial effusion, and constrictive pericarditis. Effective management hinges on a coordinated, interprofessional team approach, clear patient education on warning symptoms, and a recommended follow-up evaluation at 3 to 6 months post-injury. Conclusion: A proactive and collaborative approach, balancing the generally favorable prognosis with vigilance for rare but serious complications, is essential for the effective long-term care of patients with BCI.

Atrial septal defect with constrictive pericarditis: Report of a rare case

Radical Pericardiectomy and Use of Cardiopulmonary Bypass for Constrictive Pericarditis.

Background: Constrictive pericarditis (CP) impairs diastolic filling, commonly manifesting as right-sided heart failure. Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect virtually every organ system and rarely cause CP. Case Presentation: A 62-year-old man from India presented with insidious onset dyspnea, leg swelling, abdominal distension and weight gain. Physical exam revealed pedal edema, tense ascites, and Kussmaul’s sign. Initial labs showed cholestatic liver injury pattern. Imaging demonstrated bilateral pleural effusions, pericardial effusion, liver cirrhosis, and splenomegaly. Pleural fluid showed exudate, and high SAAG, low protein ascites were noted. Transthoracic echocardiogram (TTE) with Doppler showed septal bounce, annulus reversus, increased respiratory variation in mitral inflow E velocity, and atrial systolic notch on M-mode of the interventricular septum. Cardiac Magnetic Resonance imaging demonstrated patchy T2 hyperintensity with diffuse late gadolinium enhancement out of proportion to T2. Right heart catheterization (RHC) confirmed elevated and equalized diastolic pressures, confirming the diagnosis of CP. Despite the high initial concern for TB, extensive microbiological workup was negative. Absence of cavitary lung lesions, neutrophil-predominant pleural effusion, and an uncharacteristic ascitic fluid analysis for TB suggested an alternate diagnosis. Liver biopsy showed interface hepatitis, bile duct injury, and bridging fibrosis, suggesting autoimmune hepatitis/cholangitis. Serum IgG4 subclass levels returned markedly elevated at 366.4 mg/dL. Per 2020 Revised Comprehensive Diagnostic Criteria, the patient met clinical and serologic criteria for IgG4-RD. After multidisciplinary discussion regarding risks/benefits, pericardial stripping was not pursued. The patient received diuresis and paracentesis for symptom relief, and ibuprofen with colchicine for pericarditis. Conclusion: IgG4-RD should remain an important diagnostic consideration in idiopathic CP, especially in the setting of multi-system involvement. In our patient, IgG4-related CP possibly co-occurred with autoimmune cholangitis. While pericardial surgery would yield maximum therapeutic benefit and histopathologic confirmation of the underlying diagnosis, our case highlights a rare form of CP caused by an infiltrative disease process.

Introduction: Constrictive pericarditis (CP) is a rare but reversible cause of diastolic heart failure caused by chronic pericardial inflammation, fibrosis, and calcification that impairs ventricular filling. Often misdiagnosed as HFpEF (heart failure with preserved ejection fraction), CP has an estimated U.S. prevalence of 9–10 cases per million. Early recognition is crucial, as surgical pericardiectomy can be curative. Case Presentation: A 60-year-old male with hypertension, type 2 diabetes, and obstructive sleep apnea presented with progressive exertional dyspnea, fatigue, bilateral lower extremity edema, intermittent exertional chest pain, orthostatic dizziness, and palpitations. Initial evaluation in 2022 showed preserved left ventricular ejection fraction and no pericardial abnormalities on transthoracic echocardiogram. Myocardial perfusion SPECT revealed a mild right coronary artery perfusion defect. EKG showed right bundle branch block with nonspecific ST-T changes. In February 2024, left heart catheterization revealed mild coronary artery disease and incidental pericardial calcifications. By June 2024, CT angiography confirmed diffuse pericardial thickening and dense calcifications around the left ventricular apex, consistent with prior pericarditis. Right heart catheterization in July 2024 demonstrated equalization of diastolic pressures (Right Atrium: 25 mmHg, Right Ventricular End Diastolic Pressure: 24 mmHg, Pulmonary Capillary Wedge Pressure: 24 mmHg, Left Ventricular End Diastolic Pressure: 24–25 mmHg), reduced cardiac output (1.6 L/min), low cardiac index (1.7 L/min/m 2 ), and mildly elevated Pulmonary Vascular Resistance (3.2 Wood units), confirming CP. The patient underwent pericardiectomy without cardiopulmonary bypass in August 2024. Pathology showed pericardial calcifications and chronic inflammation. Postoperatively, the patient reported improved exercise tolerance and resolution of symptoms. Discussion: This case highlights the diagnostic challenge of CP, especially in patients without classic risk factors such as prior surgery, radiation, or tuberculosis. The clinical picture mimicked HFpEF, but subtle clues—pericardial calcification and invasive hemodynamic findings—were critical for diagnosis. Timely pericardiectomy led to significant clinical improvement, emphasizing the importance of early recognition and intervention in CP.

Background: Coccidioidomycosis is primarily found in the southwestern United States and endemic regions of Central and South America, typically manifesting as a mild respiratory illness. Extrapulmonary manifestations are rare, occurring in approximately 1% of cases. Among these, coccidioidal pericarditis represents an exceptionally uncommon but potentially life-threatening complication. This condition can lead to serious outcomes including pericardial effusion, cardiac tamponade, constrictive pericarditis, and heart failure. Methods: We conducted a comprehensive literature review using PubMed to identify relevant articles published from January 1950 to July 2024. The search terms used were "Coccidioides AND Pericarditis" and "Coccidioidomycosis AND Pericarditis." We further evaluated citations in included articles and reviews to identify additional studies and expanded our search to international literature from endemic regions. Description of Case: A 53-year-old immunocompetent African American male presented with a 4-month history of dyspnea, fever, and night sweats. CT imaging showed miliary lung infiltration, a pulmonary mass, and spinal lytic lesions. Initial echocardiography revealed mild pericardial effusion that progressed to cardiac tamponade within 48 hours, necessitating a surgical pericardial window. Pericardial fluid and biopsy confirmed Coccidioides infection. He was treated with amphotericin B followed by fluconazole. MRI two months post-discharge revealed spinal osteomyelitis, which was confirmed on biopsy. Results: Our review identified 37 reported cases of coccidioidal pericarditis between 1950 and 2024. Most were from the United States, particularly California and Arizona. Common presenting symptoms included dyspnea (67.6%), chest pain (48.6%), and cough (37.8%). Complications included cardiac tamponade (27.0%), constrictive pericarditis (27.0%), and heart failure (21.6%). Among 36 cases with outcome data, the mortality rate was 25.0%, with most deaths occurring in earlier reports. Conclusion: Coccidioidal pericarditis is a rare but serious complication of disseminated coccidioidomycosis. It can rapidly progress to cardiac tamponade or constriction and carries significant morbidity and mortality. Clinicians should maintain a high index of suspicion in patients from or with travel to endemic areas presenting with unexplained pericardial disease. Early diagnosis and multidisciplinary management are essential to improving patient outcomes.

Background: Tuberculous pericarditis (TBP) remains a life-threatening extrapulmonary manifestation of tuberculosis (TB), particularly in regions with high TB prevalence. It often presents with pericardial effusion, cardiac tamponade, or progression to constrictive pericarditis (CP) and even hemodynamic compromise. While echocardiographic assessment of ventricular function is well established and left ventricular ejection fraction (LVEF) frequently preserved, subtle myocardial dysfunction may go undetected without advanced imaging. The prognostic role of atrial strain abnormalities, particularly left atrial (LA) and right atrial (RA) reservoir strain remains inadequately defined and underexplored. Objective: This study evaluates the prognostic implications of LA and RA strain abnormalities in patients with TBP, assessing their association with clinical outcomes and disease progression. Methods: A retrospective, multicenter study was conducted in 668 patients with confirmed TBP, (mean age = 37 years, mean LVEF= 56%, 57% male). All patients underwent comprehensive echocardiographic assessment, including two-dimensional speckle-tracking echocardiography for LA and RA reservoir strain analysis. Clinical outcomes were tracked over a defined follow-up period of 2-years. Multivariate Cox regression models were used to determine independent predictors of MACE. Results: Impaired LA and RA reservoir strain were observed in a significant proportion of patients despite preserved LVEF. Patients with reduced atrial strain values had higher rates of CP (p < 0.01), RV failure (p < 0.01), and all-cause mortality (p = 0.02). RA strain abnormalities were particularly associated with RV dysfunction and adverse hemodynamic profiles. Inflammatory markers (CRP, ESR) were significantly elevated in patients with atrial strain impairment, suggesting ongoing pericardial inflammation. In multivariate analysis, reduced LA and RA reservoir strain independently predicted MACE, even after adjusting for age, sex, HIV status, effusion size, and baseline ventricular function. Conclusion: In TBP, LA and RA strain abnormalities serve as sensitive indicators of subclinical cardiac dysfunction and strong independent predictors of adverse outcomes, including progression to constriction and death. These findings underscore the value of atrial strain imaging in early risk stratification and highlight the need for timely therapeutic intervention in those with strain abnormalities, even with preserved LVEF.

Introduction: Extensive vascular and valvular calcification is typically seen in older adults with longstanding cardiovascular disease. In younger patients, it often reflects complex systemic pathology. Case Presentation: A 44-year-old male presented with central, burning chest pain that worsened when lying flat and improved when upright, shortly after a dialysate adjustment and suspected leakage. His relevant history included cocaine-associated NSTEMI at age 22, non-ischemic cardiomyopathy with LVEF 30% diagnosed at age 39, and ESRD secondary to proliferative glomerulonephritis with monoclonal deposits. He underwent kidney transplant at age 27 but resumed peritoneal dialysis by age 29. Investigations: Coronary angiography revealed new triple-vessel CAD. Echocardiography showed LVEF 35%, moderately reduced RV function, low-flow low-gradient aortic stenosis, moderate-to-severe mitral annular calcification (MAC), mild mitral stenosis, and pulmonary hypertension. Non-contrast ECG-gated cardiac CT showed a 25 × 62 mm calcified apical mass, diffuse coronary and vascular calcifications, pleural calcifications, calcified pulmonary nodules, and dramatic “toothpaste-like” MAC suggestive of caseous transformation. Management: Despite extensive structural disease, the patient remained hemodynamically stable. A multidisciplinary heart team—including general cardiology, heart failure, and cardiothoracic surgery—guided evaluation. CT surgery was consulted for CABG consideration; further workup included a PET-FDG scan for myocardial viability. Pericardiectomy was discussed due to concern for constrictive pericarditis in the setting of pericardial calcifications, though right heart catheterization did not show hemodynamic findings of constriction. Ultimately, CABG was deferred due to patient stability and preference to delay intervention. Medical therapy included heparin, dual antiplatelet therapy, statin initiation, and renal-adjusted guideline-directed heart failure treatment. Conclusion: This case highlights extreme cardiovascular and extraosseous calcification in a young patient with ESRD and chronic heart failure. The “toothpaste” appearance of the mitral annulus suggests caseous transformation, a rare MAC variant. While vascular calcification is common in ESRD, this represents a severe form of CKD–mineral bone disorder. Multidisciplinary care is essential in managing overlapping cardiac, renal, and metabolic conditions, and in guiding personalized risk stratification.

Background: Tuberculous pericarditis (TBP) remains a significant cause of morbidity and mortality, particularly in regions where tuberculosis is endemic. While clinical presentation varies, the prognostic impact of demographic characteristics and echocardiographic parameters in TBP has not been comprehensively evaluated at scale. Objective: To determine the prognostic implications of demographic and echocardiographic features in a cohort of 668 patients diagnosed with tuberculous pericarditis. Methods: We conducted a retrospective analysis of 668 patients with confirmed TBP across multiple centers. Demographic data including age, sex, and HIV status were collected alongside echocardiographic parameters such as pericardial effusion size, presence of cardiac tamponade, ventricular function, and features suggestive of constrictive pericarditis. Clinical outcomes were evaluated over a defined follow-up period of 2years, focusing on mortality, progression to constriction, and need for pericardiectomy. Results: Among the 668 patients, median age was 37 years, and 57% were male (mean LVEF 56%). HIV co-infection was present in 36% of cases. Echocardiography revealed moderate to large pericardial effusions in 32% of patients, tamponade in 12%, and echocardiographic features of constriction in 23%. Multivariate analysis identified older age, HIV co-infection, large effusion size, and early echocardiographic evidence of constriction as independent predictors of adverse outcomes (p < 0.05). Preserved ventricular function was associated with improved survival, while reduced systolic performance, although uncommon, indicated a higher risk of mortality. Conclusion: In this large, predominantly young cohort of patients with tuberculous pericarditis, demographic factors, particularly age and HIV status alongside echocardiographic parameters such as effusion size, ventricular function, and signs of constriction were strong independent predictors of adverse outcomes. These findings support the integration of focused echocardiographic assessment with clinical profiling to guide early risk stratification, therapeutic decisions, and surgical referral in TBP, especially within high-burden, resource-constrained environments. Risk stratification using these indicators may inform early management decisions and improve patient outcomes.

A woman in her 40s presented with fever, dry cough, shortness of breath and weight loss for the last 2 weeks. Examination revealed pallor and decreased breath sounds on the right side of the chest. On transthoracic echocardiography, there was moderate pericardial effusion with features of constrictive pericarditis. Cardiac magnetic resonance imaging confirmed thickened pericardium with septations and multiple pockets in the pericardial cavity filled with pericardial effusion along with late gadolinium enhancement. Despite all the supportive treatment, the patient's condition worsened and pericardiectomy was planned. During surgery, the pericardium was found to be densely adherent to the myocardium and highly vascular; therefore, the procedure was abandoned. However, multiple pericardial biopsies were taken, which later revealed high-grade B-cell lymphoma. Postoperatively, the patient developed worsening shock. Despite aggressive resuscitation, her condition continued to decline, and her family chose no further escalation of care. The patient eventually passed away.

Global epicardial peeling: A novel approach for patients with “incurable” constrictive pericarditis resistant to conventional pericardiectomy

Pericardial effusion is an abnormal accumulation of fluid in the pericardial cavity. Because of the limited amount of space in the pericardial cavity, fluid accumulation leads to an increased intrapericardial pressure which can negatively affect heart function. A pericardial effusion with enough pressure to adversely affect heart function is called cardiac tamponade. Pericardial effusion usually results from a disturbed equilibrium between the production and re-absorption of pericardial fluid, or from a structural abnormality that allows fluid to enter the pericardial cavity. Tuberculosis involvement of the pericardium is well-known and can result in pericardial tamponade apart from other sequelae like constrictive pericarditis. Tuberculous pericarditis (TBP) is due to hypersensitivity to tuberculin protein produced by Mycobacterium tuberculosis and develops in 1-2% of pulmonary TB cases, representing about 1-2% of extrapulmonary tuberculosis. Complications occur in the form of acute pericarditis (4%) and cardiac tamponade (7%), which may require life-saving invasive procedures. Risk factors include diabetes, substance use disorder, HIV-positivity, renal insufficiency, biological or immunosuppressive therapy, and exposure to regions with a high prevalence of tuberculosis. Latent tuberculosis infection (LTBI) reactivation is a well-known risk associated with immunosuppressive therapies employed in the treatment of ankylosing spondylitis (AS). Tofacitinib, an approved medication for AS that inhibits Janus kinases, has been associated with an elevated risk of TB reactivation. Here we report a case of 40 years old male, who is a known case of ankylosing spondylitis on tofacitinib therapy presented with short duration of fever and acute onset breathlessness. Urgent echocardiography shows cardiac tamponade. Pericardiocentesis was performed immediately and more than one-liter hemorrhagic fluid drained. Patient was put on anti-tubercular treatment with oral steroid after adenosine deaminase positivity and gene X pert TB positivity in exudative pericardial fluid. Bangladesh Journal of Infectious Diseases, June 2025;12(1):167-173

Background and aims Sodium-glucose transporter 2 inhibitors have recently shown promise as a therapy to reduce mortality and hospitalisation for heart failure (HF) in patients with and without type 2 diabetes mellitus. The aim of this prospective study was to determine the results of a multiparametric evaluation after the addition of dapagliflozin to standard therapy in patients with heart failure with reduced ejection fraction (HFrEF). Methods From February to November 2022, 45 patients with chronic HF who regularly visited our HF outpatient clinic were selected for this study. Exclusion criteria were severe chronic renal insufficiency (GFR < 25 ml/min), type 1 diabetes, hypertrophic or restrictive cardiomyopathy, active myocarditis, constrictive pericarditis. The included patients took dapagliflozin once daily in addition to sacubitril/valsartan and other HF drugs. The following parameters were recorded before the start of therapy and at the 3-month follow-up: NYHA functional class, characteristics of the cardiopulmonary exercise test (CPET), parameters of the six-minute walk test (6MWT), quality of life (QoL) using the Kansas City Cardiomyopathy Questionnaire (KCCQ), echocardiographic evaluation. Results At 3-month follow-up, a significant increase in peak Vo2 (from 17.5 to 18.2, p < 0.001) and a significant decrease in VE/VCO2 (35.2 to 33.1, p = 0.011) were observed. In addition, Vo2/work gradient and pulse O2 increased significantly. Furthermore, a significant improvement in 6MWT, quality of life and left ventricular dimensions and systolic function was observed. Conclusion This prospective, multiparametric study showed that the additional administration of dapaglifozin to sacubitril/valsartan and other HF drugs is effective after three months.

ABSTRACT Heart failure with preserved ejection fraction (HFpEF) accounts for up to 50% of total heart failure cases globally, and yet diagnosis remains a challenge. Popular scoring systems like the HFA‐PEFF and the H 2 FPEF have since been recommended by guidelines, but limitations exist in their utility. We present a series of four cases referred to our heart failure clinic for undifferentiated dyspnoea suspicious of HFpEF, highlighting several instances where such discrepancies exist and discuss the implications from each of these cases. Two of these cases include a cardiometabolic phenotype of HFpEF (HFA‐PEFF score 6 and H 2 FPEF score 1) and exercise‐inducible case of HFpEF (HFA‐PEFF score 1 and H 2 FPEF score 4), respectively, which scored very differently depending on the scoring systems used. In addition, two additional cases of HFpEF mimics—a case of hypertrophic cardiomyopathy (HFA‐PEFF score 6 and H 2 FPEF score 1) and of constrictive pericarditis (HFA‐PEFF score 0 and H 2 FPEF score 5)—also demonstrated discrepancies in total scores when employing these internationally recognised scoring systems. HFpEF remains a largely heterogenous condition, which limits the robustness of existing scoring systems and guideline‐recommended diagnostic algorithms. Our case series, we believe, highlights the importance of combining both inductive‐deductive reasoning and pattern recognition in diagnosing HFpEF or its many mimics. Our case series also highlights how the diagnosis of HFpEF should ideally go beyond these scoring systems and additional effort should be placed in exploring underlying aetiologies and in phenotyping patients with HFpEF and their mimics.

BackgroundPerioperative complications following pericardiectomy in patients with constrictive pericarditis can significantly affect cardiac function recovery and postoperative outcomes. The prognostic nutritional index (PNI), a well-established nutritional marker, has been shown to predict outcomes in various diseases. However, its role as a predictive factor in patients with tuberculous constrictive pericarditis undergoing pericardiectomy remains unclear. This study aimed to evaluate the association between preoperative PNI and adverse perioperative outcomes in this patient population.MethodsThis retrospective cohort study included 158 patients with tuberculous constrictive pericarditis who underwent pericardiectomy between January 2016 and June 2024. Preoperative PNI was calculated using the formula: (10 × serum albumin [g/dL]) + (0.005 × total lymphocyte count [cells/mm³]). The optimal PNI cutoff value was determined via ROC curve analysis, and patients were categorized into two groups: PNI ≥ 36.11 and PNI < 36.11. Univariate and multivariate logistic regression analyses were performed to assess the association between PNI and adverse perioperative outcomes.ResultsOf the 158 patients, 67.7% had a PNI < 36.11. Significant differences were observed between the two groups in hemoglobin levels, platelet count, C-reactive protein, indirect bilirubin, lactate dehydrogenase, cholinesterase, B-type natriuretic peptide (BNP), D-dimer, positive ventricular septal bounce sign, pericardial calcification, pericardial effusion, and pleural effusion. Patients with PNI < 36.11 experienced longer total and postoperative hospital stays. The incidence of adverse perioperative events was 76.6% (82/107) in the PNI < 36.11 group. Both univariate logistic regression (OR = 4.324, 95% CI: 2.12–8.816) and multivariate logistic regression (OR = 3.586, 95% CI: 1.523–8.444) confirmed that lower PNI was significantly associated with increased adverse perioperative outcomes.ConclusionA lower preoperative PNI is strongly associated with a higher incidence of adverse perioperative outcomes in patients undergoing pericardiectomy for tuberculous constrictive pericarditis. As a reliable indicator of preoperative nutritional status, PNI can serve as an independent risk factor and a modifiable clinical parameter to reduce adverse outcomes.

A 55-year-old male patient, who was diagnosed as having constrictive pericarditis. He was transferred to our hospital for surgical treatment because of uncontrollable heart failure requiring catecholamine, which was uncontrollable by medical treatment. Postoperative drainage from the pericardial drain tube did not decrease, diuretics and anti-inflammatory drugs were not effective, so a pericardioperative peritoneal window was opened on 34 days after surgery. Postoperatively, it could be controlled with diuretics within two weeks after the window opening. He was discharged from the hospital 37 days after the window opening. Refractory pericardial effusions that do not improve with drug therapy require drainage, but if the drainage volume does not decrease, window opening into the abdominal or thoracic cavity may be an option.

Juvenile systemic sclerosis (JSSc) is a rare, progressive autoimmune disorder that can affect the skin, musculoskeletal system, gastrointestinal tract, lungs, heart, kidney and endocrine glands. Clinical presentation varies depending on organs involved and may include oesophageal dysmotility, interstitial lung disease, pulmonary hypertension, constrictive pericarditis, patchy myocardial fibrosis and glomerulonephritis with considerable morbidity and mortality.We are reporting a rare case of JSSc with multisystem involvement in an adolescent male. He presented with a 5-month history of polyarticular arthritis, skin thickening and hypopigmented skin lesions. After 2 months of treatment with methotrexate, he reported minimal improvement in joint pain, swelling and skin thickening, followed by worsening after self-cessation of treatment due to varicella zoster infection. His condition progressed with worsening skin thickening, polyarticular arthritis, joint contractures, restricted mobility, interstitial lung disease and dysphagia.

We report the case of a patient with a late diagnosis of camptodactyly-arthropathy-coxa vara-pericarditis syndrome, following his first episode of constrictive pericarditis in adulthood. We also aim to review the existing literature on this rare disease.