Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome

Abstract

Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive–constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.     

Pneumococcal pericarditis successfully treated with catheter drainage and intravenous antibiotics

Bacterial pericarditis is commonly a disease of children for which the primary treatment is surgical drainage. We report the case of a 19-yr-old patient presenting with pneumococcal pericarditis and tamponade treated successfully with intrapericardial catheter drainage and intravenous antibiotics. The technical aspects are discussed.     

Purulent meningococcal pericarditis: chronic percutaneous drainage with a modified catheter aided by echocardiography

A 7-month-old infant presented with suspected sepsis. On the third day of illness signs of cardiac tamponade developed. Tamponade was relieved by pericardiocentesis, and countercurrent immunoelectrophoresis (CIE) analysis of the fluid was positive for meningococcus group B. Antibiotic treatment was changed to penicillin G. After echocardiography demonstrated reaccumulation of fluid, a modified #16 gauge angiocatheter was placed percutaneously in the pericardial space. When drainage slowed it was repositioned using two-dimensional echocardiography. After 24 h the catheter was removed and no further accumulation occurred. The antibiotics were continued an additional 10 days and the infant recovered uneventfully. Modification of the catheter and echographic repositioning may decrease the need for surgical drainage in such patients.     

Doppler echocardiography in cardiac tamponade and constrictive pericarditis

Doppler echocardiography has greatly facilitated the assessment of patients with compressive cardiac disease. Patients in whom cardiac tamponade or pericardial constriction are suspected should undergo a complete echocardiographic examination including careful Doppler analysis of transmitral flow and inflow through the hepatic vein or superior vena cava (SVC). Monitoring of both the electrocardiogram and the phase of respiration are an integral part of this examination. Patients with cardiac tamponade exhibit a > 25% reduction in E wave velocity during the first inspiratory cardiac cycle; they exhibit predominant systolic inflow through the hepatic vein or SVC (with a predominant X descent with little or no Y descent). In constrictive pericarditis the pattern of transmitral flow variation is comparable to that observed in cardiac tamponade, however, a prominent Y descent is often observed on hepatic vein or SVC Doppler study. Similar changes with respiration may be observed in mitral inflow in obese patients or in those with chronic obstructive pulmonary disease, however, in these conditions the nadir of E wave velocity is observed 2-3 cardiac cycles after the first inspiratory beat. Restrictive cardiomyopathy may produce a similar systemic venous flow pattern, but increased inspiratory flow reversals and lack of respiratory variation in transmitral flow velocity distinguish it from constrictive pericarditis.     

Giant Purulent Pericarditis with Cardiac Tamponade Due to Streptococcus intermedius Rapidly Progressing to Constriction

Purulent pericardial effusion, although rare, is a life‐threatening condition usually produced by the extension of a nearby bacterial infection locus or by blood dissemination in the immune‐suppressed subjects or in the course of cardiothoracic surgery. Because clinical features of purulent pericardial effusion are often nonspecific, it can cause delay in diagnosis. Therefore, a high index of suspicion is required for timely diagnosis and management. Herein, we describe a case of giant purulent pericardial effusion due to Streptococcus intermedius with the history of bronchiectasis and pneumonia, which was successfully treated with pericardiocentesis via parasternal approach, appropriate antibiotics, and pericardiectomy.     

A case of rheumatoid pericarditis with high concentrations of interleukin-6 in pericardial fluid

A case of rheumatoid pericarditis that developed into cardiac tamponade without deterioration of rheumatoid arthritis is described. The concentration of interleukin-6 (IL-6) in pericardial fluid was notably increased compared with serum. IL-6 may be associated with progression or maintenance of rheumatoid pericarditis.


Keywords: rheumatoid pericarditis; cardiac tamponade; interleukin-6     

Unusual cause for loss of left ventricular capture in patient with cardiac resynchronization due to tuberculous pericarditis

We report a case of 37-year-old man implanted with cardiac resynchronization therapy-defibrillator presented with persistent low-grade fever and sudden loss of left ventricular (LV) capture from coronary sinus lead after generator replacement. ¹⁸F-fluorodeoxyglucose positron emission tomography with computed tomography scan showed increased uptake at posterolateral region of the pericardium adjacent to the LV lead, suggestive of possible lead-related infection. Combined percutaneous and surgical lead extraction revealed purulent pericarditis and polymerase chain reaction testing confirmed tuberculous (TB) pericarditis. TB pericarditis is an unusual cause of loss of LV capture, but should be considered in countries where TB is still endemic.     

The role of echocardiography in diagnosis and management of isolated meningococcal pericarditis

Although pericarditis may complicate the course of meningococcemia, it is distinctly unusual as a presenting sign. Herein we report a case of a previously healthy 16-year-old male with isolated meningococcal pericarditis, in which transthoracic echocardiography was of great importance for the initial diagnosis and for guiding the therapeutic approach during the hospitalization period. The patient presented with symptoms of chest pain and fever that deteriorated into cardiac tamponade. Pericardiocentesis was successful and Neisseria meningitidis was identified as the causative agent in the pericardial fluid. Because of failure of clinical resolution, echocardiogram was repeated and showed evidence of maintenance of large echo dense content in pericardial space. The presence of purulent content was confirmed during open-chest surgery. The role of echocardiography for the correct management of this rare form of pericarditis is discussed.     

Cardiac tamponade due to post–cardiac injury syndrome in a patient with severe haemophilia A and HIV-1 infection

An 18-year-old man with severe haemophilia A (FVIII:C < 1%) and human immunodeficiency virus 1 (HIV-1) infection was admitted to the hospital with fever and chest pain for 7 days. Eight weeks prior to his admission he had an accident for which he underwent, at another hospital, clinical and laboratory examination that revealed bone fractures of the nose cavity, and he was given factor VIII concentrates for seven days due to nasal bleeding. On admission, chest roentgenogram showed a large cardiac silhouette and echocardiography confirmed the presence of a large quantity of pericardial fluid. A presumptive diagnosis of the post-cardiac injury syndrome was made and he was given anti-inflammatory drugs plus infusion of recombinant factor VIII concentrate (35 units kg-1 b.i.d.). On the seventh day he exhibited cardiac tamponade for which he underwent subxiphoid pericardiotomy with drainage of approximately 1500 mL of bloody exudate. He had an uncomplicated recovery and 10 days later he left hospital. He was given a continuous prophylactic treatment of 15 units kg-1 of recombinant FVIII every 2 days for 6 months, and 30 months after this episode the patient is free of any symptom.     

Cardiac tamponade preceding adrenal insufficiency - an unusual presentation of Addisons''s disease: a report of two cases.

Two cases of young healthy males presenting with cardiac tamponade and developing clinical adrenal insufficiency within a few weeks are described. On presentation they had a brisk inflammatory response with complement activation. Both had signs of subclinical hepatitis, and both have later shown evidence of thyroid involvement. The possibility of a connection between pericarditis and adrenal insufficiency is discussed.     

Treatment of Candida albicans pericarditis in a heart transplant patient

Pericarditis due to Candida species is a rare clinical entity, associated with thoracic surgery and immunosuppression. We report here the second case of pericarditis due to Candida albicans in a heart transplant patient, which presented as tamponade approximately 3 weeks post transplant, in the absence of evidence of sternal osteomyelitis. The patient was treated with pericardiocentesis and a combination of caspofungin and fluconazole, but the patient ultimately required the explantation of retained epicardial leads and the creation of a pericardial window. This case illustrates that Candida species must be considered in the differential diagnosis in post-transplant pericarditis, and that foreign body removal is, as always, key in helping to resolve such infections. This case also demonstrates the first use of caspofungin with fluconazole to treat Candida pericarditis. We discuss the conflicting data regarding the use of caspofungin, alone or in combination therapy, in treating infections involving biofilms, such as the infected pericardium.     

Purulent pericarditis due to group B streptococcus and mycotic aneurysm of the ascending aorta: case report

A 61-year-old female, with a history of uterine and cervical cancer treated with radical hysterectomy and 2 years of postoperative chemotherapy, presented to the emergency department with dyspnea on exertion. Computed tomography of the chest revealed a large pericardial effusion and a sacciform aneurysm of the ascending aorta. The patient subsequently underwent emergency pericardiocentesis with drainage of approximately 330 ml of a bloody and turbid effusion. Cultures from the effusion yielded group B streptococcus. Multiple organ failure and disseminated intravascular coagulation syndrome occurred in the acute phase, but gradually improved with continuous antibiotic therapy. On the 194th hospital day, in situ reconstruction of the ascending aorta was successfully performed using a synthetic graft. Although rarely reported, both purulent bacterial pericarditis and mycotic aneurysm can be life-threatening.     

Transesophageal echocardiography in pericardial disease and tamponade

While most pericardial disorders can be imaged by transthoracic echocardiography, transesophageal echocardiography may be required in those cases where pericardial pathology is clinically suspected, but cannot be imaged adequately with transthoracic echocardiography. Transesophageal echocardiography is especially helpful in patients after heart or chest surgery, with cardiac compression by a loculated pericardial hematoma, in patients with dissection, endocarditis, or interatrial shunting associated with pericardial effusion, in patients with pericardial tumors, and in the differential diagnosis between constrictive pericarditis and restrictive cardiomyopathy.     

Cardiac tamponade in a patient with Gaucher's disease.

Gaucher's disease, a familial inborn error of metabolism associated with hepatosplenomegaly and hypersplenism, was first described by Earnest Gaucher in 1882. By 1959, Hsia found published reports on more than 300 cases. Most reports mentioned the bleeding tendencies of patients with Gaucher's, but show that major hemorrhagic complications are rare. We report a case of hemorrhagic pericarditis with cardiac tamponade in a patient with Type I Gaucher's disease.     

Pericardial tamponade due to erosion of a Figulla Flex II device after closure of an atrial septal defect

We present a case of an 8‐year‐old boy suffering from sudden‐onset severe chest pain and cardiogenic shock due to cardiac tamponade caused by erosion of a Figulla Flex II device. His symptoms developed 4 days after transcatheter closure of an atrial septal defect. After emergent pericardiocentesis, surgery was performed to remove the device, close the atrial septal defect, and repair the laceration in the anterior‐superior wall of the right atrium and the perforation on the aortic wall adjacent to the right atrial free wall perforation site. All surgical procedures were successful, and the patient was discharged without sequelae. Although there have been few reports on the erosion of this flexible device, a lethal complication can occur when the right‐sided disc of the oversized device impinges perpendicularly on the aortic wall.     

Cardiac tamponade in a patient with moderate hemophilia A and factor VIII Inhibitors

Cardiac tamponade is a rare, life-threatening complication of hemophilia. The management of pericardial bleeding in hemophilia A patients with inhibitors is particularly challenging because antibodies to factor (F) VIII render the use of high-dose FVIII ineffective. Fortunately, the management of uncontrollable bleeding in patients with hemophilia and inhibitors has improved since the introduction of treatments that bypass the need for FVIII and FIX. A case of hemopericardium complicated by cardiac tamponade occurring one month following an upper respiratory tract infection in a patient with hemophilia and FVIII inhibitors is presented. Management of the present case was based on current guidelines on the use of recombinant FVIIa for acute bleeding in patients with hemophilia and inhibitors. The subsequent development of hemothorax in the present case indicates that a more protracted course of recombinant FVIIa is justified following pericardiocentesis for pericardial bleeding in hemophilia with inhibitors. Alternative approaches to the management of this complication are also reviewed.     

Exudative pericarditis in the evolution of a diffuse large B-cell lymphoma

Cardiac involvement in non-Hodgkin’s lymphoma is a rare occurrence with a dismal prognosis, which may evolve with different clinical presentations, the most frequent being heart failure. Diagnosis of cardiac involvement is generally made by cardiac ultrasound. We report a case of lymphomatous pericarditis in the evolution of a non-Hodgkin’s lymphoma, diagnosed by PET-CT scan, and occurring concomitantly with complete isotopic remission of enlarged mediastinal lymph nodes following chemotherapy.     

Echocardiographic detection of kaposi's sarcoma causing cardiac tamponade in a patient with acquired immunodeficiency syndrome

Pericardial effusions are common in patients with acquired immunodeficiency syndrome (AIDS). The differential diagnosis is diverse, and in most cases the etiology cannot be established. A cardiac tamponade was diagnosed in a 32-year-old male with AIDS and systemic Kaposi's sarcoma. Transthoracic echocardiography revealed a large pericardial effusion with right atrial collapse and a mobile multilobular mass at the apex protruding into pericardial space. Autopsy showed that this mass was Kaposi's sarcoma confined to the epicardial fat. This is the first case of cardiac Kaposi's sarcoma detected premortem by echocardiography.