Transient Constrictive Pericarditis: Current Diagnostic and Therapeutic Strategies.

Transient constrictive pericarditis is increasingly recognized as a distinct sub-type of constrictive pericarditis. The underlying pathophysiology typically relates to impaired pericardial distensibility, associated with acute or sub-acute inflammation, rather than the fibrosis or calcification often seen in chronic pericardial constriction. Accordingly, patients may present clinically with concomitant features of pericarditis and constrictive physiology. Non-invasive multimodality imaging is advocated for diagnosis of transient constrictive pericarditis. Echocardiography remains the mainstay for initial evaluation of the dynamic features of constriction. However, cardiac magnetic resonance imaging can provide complimentary functional information, with the addition of dedicated sequences to assess for active pericardial edema and inflammation. Although transient pericardial constriction can spontaneously resolve, institution of anti-inflammatory therapy may hasten resolution or even prevent progression to chronic pericardial constriction. Non-steroidal anti-inflammatory agents remain the initial treatment of choice, with subsequent consideration of colchicine, steroids, and other immune-modulating agents in more refractory cases.

Use of Cardiac Magnetic Resonance Imaging Based Measurements of Inferior Vena Cava Cross-Sectional Area in the Diagnosis of Pericardial Constriction.

PURPOSE: To evaluate the value of cardiac magnetic resonance imaging (MRI)-based measurements of inferior vena cava (IVC) cross-sectional area in the diagnosis of pericardial constriction. METHODS: Patients who had undergone cardiac MRI for evaluation of clinically suspected pericardial constriction were identified retrospectively. The diagnosis of pericardial constriction was established by clinical history, echocardiography, cardiac catheterization, intraoperative findings, and/or histopathology. Cross-sectional areas of the suprahepatic IVC and descending aorta were measured on a single axial steady-state free-precession (SSFP) image at the level of the esophageal hiatus in end-systole. Logistic regression and receiver-operating curve (ROC) analyses were performed. RESULTS: Thirty-six patients were included; 50% (n = 18) had pericardial constriction. Mean age was 53.9 ± 15.3 years, and 72% (n = 26) were male. IVC area, ratio of IVC to aortic area, pericardial thickness, and presence of respirophasic septal shift were all significantly different between patients with constriction and those without (P < .001 for all). IVC to aortic area ratio had the highest odds ratio for the prediction of constriction (1070, 95% confidence interval [8.0-143051], P = .005). ROC analysis illustrated that IVC to aortic area ratio discriminated between those with and without constriction with an area under the curve of 0.96 (95% confidence interval [0.91-1.00]). CONCLUSIONS: In patients referred for cardiac MRI assessment of suspected pericardial constriction, measurement of suprahepatic IVC cross-sectional area may be useful in confirming the diagnosis of constriction when used in combination with other imaging findings, including pericardial thickness and respirophasic septal shift.

A failing right heart in an eggshell: A case report of idiopathic constrictive pericarditis.

key Clinical Message: Constrictive Pericarditis is a rare fibrotic conversion of the pericardium that results in non-specific clinical symptoms such as hepatomegaly, ascites, pleural effusions, and lower extremity edema. A multi-modal diagnostic approach with cardiac imaging tools, cardiac hemodynamic measurements, and tissue biopsy can be used to diagnose Constrictive Pericarditis. Abstract: Constrictive Pericarditis is a rare complication resulting in the fibrotic conversion of the pericardium secondary to idiopathic, infective, post-surgical, or post-radiation etiologies. The rigid and restrictive nature of the pericardium can result in non-specific symptoms of volume overload that can mimic liver cirrhosis or congestive heart failure. We present the case of a 73-year-old female with constrictive pericarditis who presented with vague symptoms of abdominal pain, abdominal bloating, and bilateral lower extremity edema. This case report highlights the clinical manifestation, invasive, and non-invasive diagnostic work-up, and management of constrictive pericarditis.

Mulibrey nanism and immunological complications: a comprehensive case report and literature review.

Introduction: Mulibrey nanism (MUL) is a rare disorder caused by TRIM37 gene variants characterized by growth failure, dysmorphic features, congestive heart failure (CHF), and an increased risk of Wilms' tumor. Although immune system impairment has been documented in MUL, the underlying mechanisms remain poorly understood. Methods: We present a case of MUL with progressive lymphopenia and review similar cases from the literature. Results: Our patient presented with prenatal onset growth restriction, characteristic dysmorphic features, and Wilms' tumor. She developed progressive lymphopenia starting at 10 years of age, leading to the initiation of intravenous immunoglobulin (IVIG) replacement therapy and infection prophylaxis. Genetic analysis detected a likely pathogenic variant on the maternal allele and copy number loss on the paternal allele in TRIM37. Subsequently a cardiac magnetic resonance imaging was conducted revealing signs of pericardial constriction raising concerns for intestinal lymphatic losses. The cessation of IVIG therapy did not coincide with any increase in the rate of infections. The patient exhibited a distinct immunological profile, characterized by hypogammaglobulinemia, impaired antibody responses, and skewed T-cell subsets with an altered CD4+/CD8+ ratio, consistent with previous reports. Normal thymocyte development assessed by artificial thymic organoid platform ruled out an early hematopoietic intrinsic defect of T-cell development. Discussion: The immunological profile of MUL patients reported so far shares similarities with that described in protein-losing enteropathy secondary to CHF in Fontan circulation and primary intestinal lymphangiectasia. These similarities include hypogammaglobulinemia, significant T-cell deficiency with decreased CD4+ and CD8+ counts, altered CD4+/CD8+ ratios, and significantly modified CD4+ and CD8+ T-cell phenotypes toward effector and terminal differentiated T cells, accompanied by a loss of naïve CD45RA+ T lymphocytes. In MUL, CHF is a cardinal feature, occurring in a significant proportion of patients and influencing prognosis. Signs of CHF or constrictive pericarditis have been evident in the case reported here and in all cases of MUL with documented immune dysfunction reported so far. These observations raise intriguing connections between these conditions. However, further investigation is warranted to in-depth define the immunological defect, providing valuable insights into the pathophysiology and treatment strategies for this condition.

Whole Exome Sequencing Identified the Causative Mutation in a 4-Year-Old Female with Mulibrey Nanism: A Case Report.

Mulibrey Nanism is a rare multisystem disorder inherited in an autosomal recessive manner caused by mutations in the TRIM37 gene. Most of the reported cases are from Finland, but this condition has rarely occurred in other countries. Although the clinical diagnosis of Mulibrey nanism is a challenge during the first months of life, the disease can be suspected clinically due to the distinctive features of the patients. A 4-year-old female with pneumonia, cardiomyopathy, growth retardation, peripheral edema, and characteristic craniofacial features was referred to Tehran Hope Generation Foundation Genetic diagnosis Center, in October 2021. Genomic DNA was isolated from peripheral blood samples of the patient and her parents and Whole exome sequencing was performed for the patient. Whole exome sequencing revealed a homozygous G>A splice site variant (TRIM37; c.370-1G>A). Sanger sequencing confirmed the segregation of the variant with phenotype in this family. Whole exome sequencing can be helpful in the diagnosis of the patients suspecting to Mulibrey nanism and lacking sufficient clinical presentation according to the diagnostic algorithm.

Hemorrhagic pericardial effusion resulting in constriction in hereditary hemorrhagic telangiectasia.

BACKGROUND: We report the first ante-mortem diagnosis of hemorrhagic pericardial effusion in hereditary hemorrhagic telangiectasia resulting in constriction; the case also demonstrates the unusual but well-described complication of right-sided heart failure requiring extracorporeal membrane oxygenation (ECMO) support after pericardiectomy. CASE PRESENTATION: A previously healthy 48 year old man with a strong family history of Osler-Weber-Rendu disease presented to our institution with signs and symptoms of advance heart failure. His workup demonstrated a thickened pericardium and constrictive physiology. He was brought to the operating room where old clot and inflamed tissue were appreciated in the pericardial space and he underwent complete pericardiectomy under cardiopulmonary bypass. Separation from bypass, hampered by the development of right ventricular dysfunction and profound vasoplegia, required significant pressor and inotropic support. The right heart dysfunction and vasoplegia worsened in the early postoperative period requiring a week of ECMO after which his right ventricle recovered and he was successfully de-cannulated. CONCLUSION: Given the poor outcome of severe postoperative right ventricular failure after pericardiectomy, with high central venous pressure, a low gradient between central venous and pulmonary artery pressures and high vasopressor requirements, ECMO should be instituted promptly.

The role of multi-modality cardiovascular imaging in a patient presenting with acute liver failure secondary to constrictive pericarditis: a case report.

Background: Constrictive pericarditis is a rare cause of heart failure which often presents a diagnostic challenge to clinicians. Case summary: We describe the case of a 62-year-old male who presented to our institution with symptoms and signs suggestive of acute liver failure. Abdominal imaging demonstrated congestive hepatopathy. Clinical suspicion despite a 'normal' echocardiogram and sub-threshold NT-proBNP led to multi-modality cardiovascular imaging investigations to determine the cause of the heart failure syndrome. His cardiovascular magnetic resonance scan confirmed ventricular interdependence and extensive late enhancement in the pericardium with associated pericardial effusion. An 18F-fluorodeoxyglucose-positron emission tomography scan confirmed active pericardial inflammation. Cardiac computed tomography showed minimal pericardial calcification. Following confirming the diagnosis of effusive-constrictive pericarditis with evidence of active pericardial inflammation on imaging, a discussion within the Heart multidisciplinary team concluded that a trial of medical therapy with steroids is justifiable to avoid high-risk pericardiectomy. The patient was successfully treated with a combination of ibuprofen, colchicine, and prednisolone resulting in clinical improvement and remission of his symptoms. The imaging investigations were repeated 6 months later and confirmed radiological remission and medical therapy was discontinued. Discussion: We stress the importance of multi-modality cardiovascular imaging in the diagnosis of constrictive pericarditis and also emphasize its role in identifying the subset of patients who may respond to medical therapy, therefore reducing the risk of high need surgical pericardiectomy.

Improvement in left ventricular mechanics following medical treatment of constrictive pericarditis.

OBJECTIVE: Patients with constrictive pericarditis (CP) with active inflammation may show resolution with anti-inflammatory therapy. We aimed to investigate the impact of anti-inflammatory medications on constrictive pathophysiology using echocardiography in patients with CP. METHODS: We identified 35 patients with CP who were treated with anti-inflammatory medications (colchicine, prednisone, non-steroidal anti-inflammatory drugs) after diagnosis of CP (mean age 58±13; 80% male). Clinical resolution of CP (transient CP) was defined as improvement in New York Heart Association class during follow-up. We assessed constrictive pathophysiology using regional myocardial mechanics by the ratio of peak early diastolic tissue velocity (e') at the lateral and septal mitral annulus by tissue Doppler imaging (lateral/septal e') or the ratio of the left ventricular lateral and septal wall longitudinal strain (LSlateral/LSseptal) by two-dimensional speckle-tracking echocardiography. Longitudinal data were analysed using a mixed effects model. RESULTS: During a median follow-up of 323 days, 20 patients had transient CP, whereas 15 patients had persistent CP. Transient CP had higher baseline erythrocyte sedimentation rates (ESR) (p=0.003) compared with persistent CP. There were no significant differences in LSlateral/LSseptal and lateral/septal e'. During follow-up, only transient CP showed improvement in lateral/septal e' (p<0.001) and LSlateral/LSseptal (p=0.003), and recovery of inflammatory markers was similar between the two groups. In the logistic model, higher baseline ESR and greater improvement in lateral/septal e' and LSlateral/LSseptal were associated with clinical resolution of CP using anti-inflammatory therapy. CONCLUSIONS: Improvement of constrictive physiology detected by lateral/septal e' and LSlateral/LSseptal was associated with resolution of clinical symptoms after anti-inflammatory treatment. Serial monitoring of these markers could be used to identify transient CP.

Cardiac magnetic resonance imaging in unclear cases of ventricular interdependence: a case series.

BACKGROUND: Diagnosis of constrictive pericarditis requires demonstration of interventricular interdependence which can prove difficult even with invasive haemodynamics. Its treatment requires invasive surgical procedures prior to which diagnostic certainty is necessary. Cardiac magnetic resonance imaging (MRI) is an underutilized tool for identification of this pathology. CASE SUMMARY: We present two cases of heart failure due to interventricular interdependence with inconclusive invasive haemodynamic. Prior to recommending invasive surgical treatment, confirmation of the diagnosis was required. This was achieved using cardiac MRI leading to pericardiectomy followed by clinical improvement. DISCUSSION: These cases demonstrate the clinical utility, sensitivity, and specificity of cardiac MRI for ventricular interdependence.

Effusive-constrictive pericarditis in the spectrum of pericardial compressive syndromes.

When pericardial fluid accumulates and exceed the reserve volume of the pericardium or when the pericardium becomes scarred and inelastic, one of three pericardial compressive syndromes may ensue, namely, cardiac tamponade (CT), characterised by the accumulation of pericardial fluid under pressure; constrictive pericarditis (CP), the result of scarring and loss of the normal elasticity of the pericardial sac; and effusive-constrictive pericarditis (ECP), characterised by the concurrence of a tense pericardial effusion and constriction of the heart by the visceral pericardium. Although relatively uncommon, prevalence estimates vary widely and depend on the nature of the cohorts studied, the methods used to diagnose ECP and the manner in which ECP is defined. Most cases of ECP are idiopathic, reflecting the frequency of idiopathic pericardial disease in general, and other causes include radiation, malignancy, chemotherapy, infection and postsurgical/iatrogenic pericardial disease. The diagnosis of ECP often becomes apparent when pericardiocentesis fails to decrease the right atrial pressure by 50% or to a level below 10 mm Hg. Important non-invasive diagnostic modalities include echocardiography, cardiac magnetic resonance and, to a lesser extent, cardiac CT. In cases with clear evidence of pericardial inflammation, a trial of an anti-inflammatory regimen is warranted. A complete pericardiectomy should be reserved for refractory symptoms or clinical evidence of chronic CP.

Anakinra for constrictive pericarditis associated with incessant or recurrent pericarditis.

OBJECTIVE: Frequent flares of pericardial inflammation in recurrent or incessant pericarditis with corticosteroid dependence and colchicine resistance may represent a risk factor for constrictive pericarditis (CP). This study was aimed at the identification of CP in these patients, evaluating the efficacy and safety of anakinra, a third-line treatment based on interleukin-1 inhibition, to treat CP and prevent the need for pericardiectomy. METHODS: Consecutive patients with recurrent or incessant pericarditis with corticosteroid dependence and colchicine resistance were included in a prospective cohort study from 2015 to 2018. Enrolled patients received anakinra 100 mg once daily subcutaneously. The primary end point was the occurrence of CP. A clinical and echocardiographic follow-up was performed at 1, 3, 6 months and then every 6 months. RESULTS: Thirty-nine patients (mean age 42 years, 67% females) were assessed, with a baseline recurrence rate of 2.76 flares/patient-year and a median disease duration of 12 months (IQR 9-20). During follow-up, CP was diagnosed in 8/39 (20%) patients. After anakinra dose of 100 mg/day, 5 patients (63%) had a complete resolution of pericardial constriction within a median of 1.2 months (IQR 1-4). In other three patients (37%), CP became chronic, requiring pericardiectomy within a median of 2.8 months (IQR 2-5). CP occurred in 11 patients (28%) with incessant course, which was associated with an increased risk of CP over time (HR for CP 30.6, 95% CI 3.69 to 253.09). CONCLUSIONS: In patients with recurrent or incessant pericarditis, anakinra may have a role in CP reversal. The risk of CP is associated with incessant rather than recurrent course.

An unusual interventricular septal bounce in a patient with dermatomyositis: a case report.

BACKGROUND: In literature it has been reported in 1998, for the first time, a case of a 54-year-old man who developed constrictive pericarditis (CP) 12 years after diagnosis of dermatomyositis (DM). To our knowledge, this may be the only case reported. CASE SUMMARY: A 16-year-old man presented to our institution because of symptoms posing a suspicion for an inflammatory disease; after physical examination, lab tests, and other investigations (electromyography, magnetic resonance, and muscular biopsy) was diagnosed as having DM. Patient also showed hepatomegaly and congested jugular veins: after clinical and imaging investigations (transthoracic echocardiography and transoesophageal echocardiography) he was diagnosed as having a CP. Patient underwent pericardial resection and the final outcome consisted of a completely regression of the symptoms. DISCUSSION: Cardiac involvement in patients with DM ranges between 6% and 75%, and it can be clinically manifest or, far more frequently, sub-clinic. Pericardial involvement as a complication of DM is widely reported in the literature, but in almost all cases as acute pericarditis, effusive pericarditis or cardiac tamponade and almost never as a CP.

Constrictive pericarditis: diagnosis, management and clinical outcomes.

Constrictive pericarditis (CP) is a form of diastolic heart failure that arises because an inelastic pericardium inhibits cardiac filling. This disorder must be considered in the differential diagnosis for unexplained heart failure, particularly when the left ventricular ejection fraction is preserved. Risk factors for the development of CP include prior cardiac surgery and radiation therapy, but most cases are still deemed to be idiopathic. Making the diagnosis may be challenging and requires meticulous echocardiographic assessment, often supplemented by cross-sectional cardiac imaging and haemodynamic catheterisation. The key pathophysiological concepts, which serve as the basis for many of the diagnostic criteria, remain: (1) dissociation of intrathoracic and intracardiac pressures and (2) enhanced ventricular interaction. Complete surgical pericardiectomy is the only effective treatment for chronic CP. A subset of patients with subacute inflammatory CP, often identified by cardiac MRI, may respond to anti-inflammatory treatments.

Surgical Management of Pericardial Diseases.

Disease of the pericardium represents a relatively rare indication for cardiac surgery, and there exist no widely accepted guidelines for surgical management. As such, the surgical approach to the pericardium has relied largely on institutional experience with a slow evolution based on published studies. In particular, management of pericardial constriction has varied widely from surgeon to surgeon and institution to institution, in large part due to a perception of inherent high risk to the procedure. This review covers the current practice of surgery for disease of the pericardium, with particular focus on the evolution of indications for pericardiectomy, new applications in inflammatory or relapsing pericarditis, and the progressive refinement in surgical technique and operative planning which have led to significantly improved outcomes in experienced centers.

Subacute effusive-constrictive pericarditis: Echocardiography-guided diagnosis and management.

A 49-year-old man presented with flu-like symptoms of two weeks. Electrocardiogram showed diffuse ST elevation. Blood samples revealed severe renal failure and moderate inflammatory results. Echocardiogram showed large pericardial effusion, dilated inferior vena cava, but no right ventricular collapse. The patient underwent hemodialysis, after which he developed clinical signs of cardiac tamponade with echocardiographic features of collapse of the right ventricle. Pericardial drainage was then performed revealing purulent fluid of 800 ml. Streptococcus agalactiae was found in the cultures of urine, blood, and pericardial fluid. Despite removal of the pericardial fluid, echocardiogram failed to show any improvement in dilated inferior vena cava and estimated right atrial pressure remained elevated. Thus, a diagnosis of subacute effusive-constrictive pericarditis was made. Following antibiotic treatment for purulent pericarditis, early pericardiectomy was performed under transesophageal echocardiographic monitoring which successfully guided surgeons to careful removal of thick and adhesive visceral pericardium as well as an additional Waffle procedure resulting in significant clinical and hemodynamic improvement. Echo-guided approach is most practical in establishing the diagnosis of effusive-constrictive pericarditis and also most helpful in obtaining successful surgical results. .

Rhythmic pulsations in inferior vena cava in pericardial constriction.

A case of pericardial constriction with rhythmic, nonrespiratory pulsations in inferior vena cava is presented. Hepatic vein flow showed wave form classical of pericardial constriction. Backward transmission of changes in right atrial pressure resulted in rhythmic, nonrespiratory pulsations in inferior vena cava. This echocardiographic finding could help in diagnosis of pericardial constriction.

Untying the Gordian knot of pericardial diseases: A pragmatic approach.

Pericardial disorders constitute a relatively common cause of heart disease. Although acute pericarditis, especially the idiopathic forms that are the most prevalent, is considered a benign disease overall, its short- and long-term complications, namely, recurrent pericarditis, cardiac tamponade and constrictive pericarditis, constitute a matter of concern in the medical community. In recent years, several clinical trials contributed to redefining our traditional approach to pericardial diseases. In this review, we provide the most recent evidence concerning diagnosis, treatment modalities and short- and long-term prognosis of the most common pericardial disorders.

MR imaging evaluation of pericardial constriction.

Abnormal thickening or rigidity of the pericardium may compromise normal cardiac function. This condition is known as pericardial constriction, or constrictive pericarditis. Several imaging modalities are used to evaluate the pericardium, including MR, computed tomography, and echocardiography, which can all play a complementary role aiding diagnosis. This article focuses on MR imaging and its role in the detection and evaluation of pericardial constriction. MR imaging has many advantages compared with other modalities including precise delineation of the pericardial thickness, evaluation of ventricular function, detection of wall motion abnormalities, and provision of information about common (and potentially harmful) sequelae of pericardial constriction.

Constrictive Pericarditis: Etiologic Spectrum, Patterns of Clinical Presentation, Prognostic Factors, and Long-term Follow-up.

INTRODUCTION AND OBJECTIVES: Some reports have described a change in the etiologic spectrum of constrictive pericarditis. In addition, data on the relationship between its clinical presentation and etiology are lacking. We sought to describe the etiologies of the disease, their relationship with its clinical presentation and surgical findings, and to identify predictors of poor outcome. METHODS: We analyzed 140 consecutive patients who underwent surgery for constrictive pericarditis over a 34-year period in a single center. RESULTS: The etiology was idiopathic in 76 patients (54%), acute idiopathic pericarditis in 24 patients (17%), tuberculous pericarditis in 15 patients (11%), purulent pericarditis in 10 patients (7%), and cardiac surgery, radiation and uremia in 5, 3 and 2 patients respectively (4%, 2% and 1%). Mean duration of symptoms before pericardiectomy was 19 months (standard deviation, 44 months), the most acute presentation being for purulent pericarditis (26 days [range, 7-60 days]) and the most chronic for idiopathic cases (29 months [range, 4 days-360 months]). Perioperative mortality was 11%. There was no difference in mortality between etiologies. Median follow-up was 12 years (range, 0.1-33.0 years) in which 50 patients died. In a Cox-regression analysis, age at surgery, advanced New York Heart Association functional class (III to IV) and previous acute idiopathic pericarditis were associated with increased mortality during follow-up. CONCLUSIONS: Most cases of constrictive pericarditis are idiopathic. Cardiac surgery and radiation accounted for a minority of cases. Etiologic investigations are warranted only in acute or subacute presentations. Age, advanced functional class, and previous acute idiopathic pericarditis are associated with increased mortality.