Mitral commissural prolapse.

Mitral commissural prolapse or flail, either isolated or combined with more extensive degenerative valve disease, imposes several challenges both on its diagnosis and management while being a risk factor for valve reoperation after mitral valve repair. Accurate identification of the prolapsing segment is often not feasible with transthoracic 2D echocardiography, with transesophageal 3D imaging then required for correct diagnosis and surgical planning. Various surgical techniques employed alone or in combination have yielded good results in the repair of commissural prolapse. Herein, we analyze the specific characteristics of commissural disease focusing our attention on 2D and 3D echocardiographic findings and we briefly comment on techniques employed for surgical correction of the disease.

Unusual Complications of Infective Endocarditis.

BACKGROUND Despite advances in management, infective endocarditis remains a condition with high in-hospital and post-discharge mortality, especially when it is complicated by perivalvular extension and heart failure (HF). CASE REPORT Herein we describe two illustrative cases of endocarditis. The first case was complicated by left ventricle to right atrial fistula. The second cased was complicated by valvular perforation with a "windsock" appearance. Both patients developed acute HF. CONCLUSIONS Fistulas and severe valvular regurgitation are among the major causes of acute HF in the setting of infective endocarditis. In such cases, surgery should be considered to decrease mortality.

Triple prosthetic valve dysfunction 30 years after surgery.

Triple prosthetic valve operation carries a high peri-procedural risk and is associated with decreased long-term survival. Herein is reported the case of a 59-year-old female with a 30-year history of successful triple-valve replacement for rheumatic heart disease, who presented with symptomatic valvular dysfunction and rhythm disturbances. The patient was one of the few who had survived more than 30 years after triple-valve surgery. Illustrative echocardiograms and cinefluoroscopic images are provided, and issues regarding prosthetic valve dysfunction and rhythm disturbance management in operated patients are briefly discussed.

Right heart thrombus causing syncope in an elderly patient.

Pulmonary embolism and thromboembolic disease carry a high mortality if not recognised and managed appropriately. Herein we illustrate the case of a dehydrated elderly female patient with recurrent syncope who proved to have high risk pulmonary embolism and a free floating right heart thrombus. The echocardiographic findings of right heart thrombus and possible thrombi 'in transit' within a low flow inferior vena cava, guided a life-saving treatment in this frail elderly patient.

Mitral-Aortic Intervalvular Fibrosa Pseudoaneurysm.

Pseudoaneurysm of the mitral aortic intervalvular fibrosa (MAIVF-P) usually ensues as a complication of endocarditis or aortic valve surgery. When large, symptomatic or related to complications (rupture, compression of adjacent structures, embolic events, mitral regurgitation or heart failure) it warrants surgical excision. The natural course of uncomplicated/asymptomatic MAIVF-Ps is largely unknown since most patients are offered surgery. Increased surgical risk imposed by repeat operations in the majority of these patients is an important consideration and conservative treatment should not be excluded in selected cases. Herein we present two illustrative cases of MAIVF-P manifesting with significant arrhythmogenesis and complex endocarditis respectively. Both patients were managed conservatively. By briefly reviewing the existing literature, we discuss important diagnostic and therapeutic issues for MAIVF-Ps. To our knowledge complex ventricular arrhythmia has not been previously described as a prominent manifestation of MAIVF-P.

A Churg-Strauss syndrome patient with myopericardial involvement.

Churg-Strauss syndrome is a necrotizing vasculitis of small vessels characterized by upper and lower airway disease followed by peripheral eosinophilia and multiple organ involvement. Herein we present the case of a 45-year-old female patient with Churg-Strauss syndrome and myopericardial disease who improved upon cyclophosphamide treatment. Apart from discussing the characteristics of myopericardial disease in eosinophilic syndromes, we highlight the crucial role of cardiac imaging in the prompt recognition and management of such patients. .

Interleukin-21 is increased in active systemic lupus erythematosus patients and contributes to the generation of plasma B cells.

OBJECTIVES: Excessive interleukin- (IL-) 21 production by T cells has been implicated in the pathogenesis of systemic lupus erythematosus (SLE). We explored the expression and function of IL-21 in human SLE. METHODS: IL-21 and IL-21 receptor (IL-21R) expression was assessed by real-time PCR and flow cytometry in peripheral blood mononuclear cells (PBMCs) of SLE patients and healthy controls. PBMCs, purified CD19+CD27- naïve and CD19+CD27+ memory B cells were stimulated with IL-21 and CpG-ODN2006 (TLR-9 agonist) to examine generation of memory and plasma (CD19+CD38highIgD-) B cells. Apoptosis was assessed by 7AAD staining. RESULTS: Active SLE patients had 4-fold higher IL-21 mRNA and increased levels of intracellular IL-21 in peripheral blood CD4+ T cells (mean±SD fluorescence intensity, 1.7±0.1 in active versus 0.9±0.3 in inactive SLE and controls, p=0.035). IL-21R mRNA was comparable between SLE and healthy controls. Stimulation of PBMCs with IL-21 increased the proportion of memory and plasma cells; addition of CpG-ODN2006 enhanced these effects. Both naïve and memory B cells responded to IL-21/TLR-9 by increased generation of memory and plasma B cells, respectively; an anti-apoptotic effect was observed. In active SLE, PBMCs stimulation with IL-21 and/or CpG-ODN increased memory and plasma B cells, comparable to healthy controls. Addition of IL-21 to lupus autologous mixed lymphocyte cultures induced significant IgG production, and treatment with IL-21R.Fc to block IL-21/IL-21R interaction reduced the proportion of plasma cells. CONCLUSIONS: Increased IL-21 may synergise with TLR-9 signalling and contributes to generation of plasma cells in active SLE patients.

Acute myocardial infarction complicating active ulcerative colitis: a case report.

Ulcerative colitis (UC) is a chronic inflammatory disease that predominantly affects the gastrointestinal (GI) tract but can involve extraintestinal organs including musculoskeletal system and skin. The most frequent cardiac manifestations of UC are pericarditis and myocarditis. Patients display an increased risk for venous thromboembolic complications and mesenteric ischemia, but the association with ischemic heart disease and myocardial infarction is uncertain. We present the case of a 27-year-old man with anti-PRIII ANCA-positive ulcerative colitis and increased factor VIII activity who presented with an acute myocardial infarction. We discuss possible causative links between these clinical entities and demonstrate the role of cardiac magnetic resonance (CMR) in patients with underlying inflammatory conditions who present with chest pain and evidence of myocardial damage.

Childhood- and adult-onset lupus: an update of similarities and differences.

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune rheumatic disease. Although its highest prevalence is among women of childbearing age, the disease is not confined within this population. A total of 15-20% of cases of SLE are diagnosed in children younger than 16 years (childhood-onset lupus). Although there have been few studies directly comparing childhood- to adult-onset lupus, there is substantial evidence to suggest that pediatric lupus patients display some differences in their disease profile compared with adult-onset populations. Overall, an increased male-to-female ratio, a higher prevalence of nephritis and CNS involvement necessitating a more sustained need for steroids and immnosuppressive drugs, and a higher prevalence of progression to end-stage renal disease are distinguishing features of childhood-onset lupus. In contrast, a higher prevalence of pulmonary involvement, arthritis and discoid lupus are reported in adult-onset SLE patients. Furthermore, childhood-onset lupus patients may experience a serious negative impact on their psychosocial and physical development, issues that pose extra challenges to healthcare providers. Growth delay, osteoporosis, the psychological effect of steroid-induced alterations of the physical image, and often poor treatment compliance are the issues that need to be addressed in pediatric lupus populations. In this review, we compare the epidemiological, clinical and laboratory features, and treatment options of childhood- and adult-onset lupus, and comment on the applicability of the instruments that measure activity, severity and cumulative disease damage in childhood-onset disease. In addition, we highlight special issues of concern for pediatric lupus patients, discussing the significance in the transition from pediatric to adult rheumatology care.

Toll like receptors and autoimmunity: a critical appraisal.

There is a constant interplay between the innate and adaptive immune systems, which leads to a protective immune response against pathogens and contributes effectively to self-non-self discrimination. Toll-like receptors (TLRs) are key components of the innate immune system, which activate multiple inflammatory pathways and coordinate systemic defense against pathogens. In addition to recognizing unique molecular patterns associated with different classes of pathogens, TLRs may also recognize a number of self proteins and endogenous nucleic acids. Data originating predominantly from animal models of autoimmune disease and circumstantial data from human patients suggest that inappropriate activation of TLR pathways by endogenous or exogenous ligands may lead to the initiation and/or perpetuation of autoimmune responses and tissue injury.

Expansion of toll-like receptor 9-expressing B cells in active systemic lupus erythematosus: implications for the induction and maintenance of the autoimmune process.

OBJECTIVE: Toll-like receptors (TLRs) are pattern-associated receptors in innate immunity that may be involved in the recognition of self antigens and the production of pathogenic autoantibodies. This study was undertaken to examine the expression and function of various TLRs in subpopulations of peripheral blood mononuclear cells (PBMCs) of patients with systemic lupus erythematosus (SLE). METHODS: The expression of TLRs in PBMCs from 50 SLE patients with active disease (SLE Disease Activity Index [SLEDAI] score >or=8; n = 26) or inactive disease (SLEDAI score <8; n = 24) and 20 healthy controls was studied by flow cytometry. TLR expression was assessed on various subpopulations of PBMCs (TLR-2 and TLR-4 by membrane staining; TLR-3 and TLR-9 by intracellular staining). TLR function was accessed by stimulating PBMCs with specific ligands. RESULTS: The proportion of B cells and monocytes expressing TLR-9 was higher among patients with active SLE (mean +/- SD 49.5 +/- 24.4% and 30.7 +/- 24.1%, respectively) than among patients with inactive disease (22.8 +/- 19.6% and 14.3 +/- 8.4%, respectively; P = 0.02 and P = 0.03). Among B cells, the proportion of plasma cells and memory B cells expressing TLR-9 was increased in patients with active SLE. Increased percentages of TLR-9-expressing B cells correlated with the presence of anti-double-stranded DNA antibodies (P = 0.007). Treatment with serum from patients with active disease increased the percentage of TLR-9-expressing plasma cells in serum from healthy controls. Enhanced induction of HLA-DR after TLR-9 stimulation was documented in B cells from patients with active disease. CONCLUSION: In patients with active SLE, the proportion of peripheral blood memory B cells and plasma cells expressing TLR-9 is increased. Endogenous nucleic acids released during apoptotic cell death may stimulate B cells via TLR-9 and contribute to SLE pathogenesis.