Unusual place unusual number.

Cardiac papillary fibroelastoma is a rare, benign cardiac tumor. It often arises from the valvular endocardium and is usually solitary. Nonvalvular location is rare and even more rare is the presentation as multiple masses. A 71-year-old female patient was referred for echocardiographic evaluation due to progressive fatigue. The presence of multiple left atrial masses was observed on echocardiographic evaluation. The patient was treated surgically for the prevention of embolic complications, and the histologic diagnosis of multiple nonvalvular papillary fibroelastoma was made. This case highlights the need to consider this unusual location and presentation for this type of tumor.

[Pre- and post-test probability of obstructive coronary artery disease in two diagnostic strategies: relative contributions of exercise ECG and coronary CT angiography]. / Probabilidade teórica de doença coronária pré- e pós-teste em duas estratégias diagnósticas - Contributo relativo da prova de esforço e da angio-TC cardíaca.

INTRODUCTION: The purpose of this study was to assess the change in theoretical probability of coronary artery disease (CAD) in patients with suspected CAD undergoing coronary CT angiography (CCTA) as first line test vs. patients who underwent CCTA after an exercise ECG. METHODS: Pre- and post-test probabilities of CAD were assessed in 158 patients with suspected CAD undergoing dual-source CCTA as the first-line test (Group A) and in 134 in whom CCTA was performed after an exercise ECG (Group B). Pre-test probabilities were calculated based on age, gender and type of chest pain. Post-test probabilities were calculated according to Bayes' theorem. RESULTS: There were no significant differences between the groups regarding pre-test probability (median 23.5% [13.3-37.8] in group A vs. 20.5% [13.4-34.5] in group B; p=0,479). In group A, the percentage of patients with intermediate likelihood of disease (10-90%) was 90% before testing and 15% after CCTA (p<0,001), while in group B, it was 95% before testing, 87% after exercise ECG (p=NS), and 17% after CCTA (p<0,001). CONCLUSION: Unlike exercise testing, CCTA is able to reclassify the risk in the majority of patients with an intermediate probability of obstructive CAD. The use of CCTA as a first-line diagnostic test for CAD may be beneficial in this setting.

Chronotype and Time of Day Effects on a Famous Face Recognition Task with Dynamic Stimuli.

Chronotype and Time of Day (ToD) can modulate several aspects of cognitive performance. However, there is limited evidence about the effect of these variables on face recognition performance, so the aim of the present study is to investigate this influence. For this, 274 participants (82.5% females; age 18-49 years old, mean = 27.2, SD = 1.82) were shown 20 short videoclips, each gradually morphing from a general identity unfamiliar face to a famous face. Participants should press the spacebar to stop each video as soon as they could identify the famous face, and then provide the name or an unequivocal description of the person. Analysis of response times (RT) showed that evening-types recognised the faces faster than morning-types. Considering different ToD windows, the effect of chronotype was only significant in the 13h-17h and in the 21h-6h time-windows. Altogether, results suggest an advantage of evening-types on famous face recognition using dynamic stimuli with morning-types, being particularly slower during their non-optimal period.

El cronotipo y la hora del día pueden modular varios aspectos del rendimiento cognitivo. Sin embargo, existen pocas pruebas sobre el efecto de estas variables en el rendimiento en el reconocimiento de caras, por lo que el objetivo del presente estudio es investigar esta influencia. Para ello, se mostraron a 274 participantes (82.5% mujeres; edad 18-49 años, media = 27.2, DE = 1.82) 20 videoclips cortos, cada uno de los cuales pasaba gradualmente de una cara desconocida de identidad general a una cara famosa. Los participantes debían pulsar la barra espaciadora para detener cada vídeo en cuanto pudieran identificar la cara famosa y, a continuación, proporcionar el nombre o una descripción inequívoca de la persona. El análisis de los tiempos de respuesta (TR) mostró que los participantes vespertinos reconocían las caras más rápidamente que los matutinos. Considerando diferentes ventanas de hora del día, el efecto del cronotipo solo fue significativo en las ventanas temporales de 13h-17h y de 21h-6h. En conjunto, los resultados sugieren una ventaja de los tipos vespertinos en el reconocimiento de caras famosas al utilizar estímulos dinámicos, siendo los tipos matutinos particularmente más lentos durante su periodo no óptimo.

[Giant left atrial myxoma presenting as acute myocardial infarction]. / Enfarte agudo do miocárdio como forma de apresentação de mixoma gigante da aurícula esquerda.

Myxomas are the most common type of benign cardiac tumor. The most frequent clinical presentations are symptoms resulting from atrioventricular valve obstruction or systemic embolization. Coronary embolization is a rare, although real and potentially fatal, complication of cardiac myxomas. We present a case report and review of the literature on this disease association. A 57-year-old woman was admitted to our coronary care unit with a diagnosis of non-ST elevation acute myocardial infarction. Transthoracic echocardiography showed a large left atrial mass attached to the interatrial septum, coral-like and with a friable appearance, suggestive of myxoma. Coronary angiography revealed no significant lesions and the patient underwent surgical excision of the mass, which histological study showed to be compatible with myxoma. The postoperative period was uneventful and the patient is doing well, with no recurrence of myxoma.

The Interplay between Chronotype and Emotion Regulation in the Recognition of Facial Expressions of Emotion.

Emotion regulation strategies affect the experience and processing of emotions and emotional stimuli. Chronotype has also been shown to influence the processing of emotional stimuli, with late chronotypes showing a bias towards better processing of negative stimuli. Additionally, greater eveningness has been associated with increased difficulties in emotion regulation and preferential use of expressive suppression strategies. Therefore, the present study aimed to understand the interplay between chronotype and emotion regulation on the recognition of dynamic facial expressions of emotion. To that end, 287 participants answered self-report measures and performed an online facial emotion recognition task from short video clips where a neutral face gradually morphed into a full-emotion expression (one of the six basic emotions). Participants should press the spacebar to stop each video as soon as they could recognize the emotional expression, and then identify it from six provided labels/emotions. Greater eveningness was associated with shorter response times (RT) in the identification of sadness, disgust and happiness. Higher scores of expressive suppression were associated with longer RT in identifying sadness, disgust, anger and surprise. Expressive suppression significantly moderated the relationship between chronotype and the recognition of sadness and anger, with chronotype being a significant predictor of emotion recognition times only at higher levels of expressive suppression. No significant effects were observed for cognitive reappraisal. These results are consistent with a negative bias in emotion processing in late chronotypes and increased difficulty in anger and sadness recognition for expressive suppressor morning-types.

The Impact of Sleep on Face Recognition Memory: A Scoping Review.

Sleep has a major impact on a variety of human biological and cognitive functions. In particular, its impact on memory has attracted extensive research and has been amply demonstrated. However, it is still unclear whether sleep, or lack thereof, affects the ability to recognize faces. To clarify this, we conducted a scoping review on studies that included a face recognition memory task and any kind of sleep manipulation in adults without any sleep pathology. A systematic search and synthesis of peer-reviewed journal articles identified through the electronic databases Scopus, Web of Science, EBSCO, and PubMed was performed. A final sample of 18 articles, corresponding to 19 studies, met the eligibility criteria. The results of 13 articles suggested that sleep benefited face recognition ability, whereas two articles indicated a detrimental effect of sleep on performance, and four articles found no significant effects. This review highlights the high methodological variability between studies, in terms of sleep manipulation, retention interval, tasks used to probe face recognition, and other variables. In sum, although around one third of the studies show a beneficial effect of sleep on memory for faces, we suggest that future research should invest in replicating these findings with a stricter control of potentially confounding variables to allow stronger conclusions to be drawn.

Low frequency of CD4+CD25+ Treg in SLE patients: a heritable trait associated with CTLA4 and TGFbeta gene variants.

BACKGROUND: CD4+CD25+ regulatory T cells play an essential role in maintaining immune homeostasis and preventing autoimmunity. Therefore, defects in Treg development, maintenance or function have been associated with several human autoimmune diseases including Systemic Lupus Erythematosus (SLE), a systemic autoimmune disease characterized by loss of tolerance to nuclear components and significantly more frequent in females. RESULTS: To investigate the involvement of Treg in SLE pathogenesis, we determined the frequency of CD4+CD25+CD45RO+ T cells, which encompass the majority of Treg activity, in the PBMC of 148 SLE patients (76 patients were part of 54 families), 166 relatives and 117 controls. SLE patients and their relatives were recruited in several Portuguese hospitals and through the Portuguese Lupus Association. Control individuals were blood donors recruited from several regional blood donor centers. Treg frequency was significantly lower in SLE patients than healthy controls (z = -6.161, P < 0.00001) and intermediate in the relatives' group. Remarkably, this T cell subset was also lower in females, most strikingly in the control population (z = 4.121, P < 0.001). We further ascertained that the decreased frequency of Treg in SLE patients resulted from the specific reduction of bona fide FOXP3+CD4+CD25+ Treg. Treg frequency was negatively correlated with SLE activity index (SLEDAI) and titers of serum anti-dsDNA antibodies. Both Treg frequency and disease activity were modulated by IVIg treatment in a documented SLE case. The segregation of Treg frequency within the SLE families was indicative of a genetic trait. Candidate gene analysis revealed that specific variants of CTLA4 and TGFbeta were associated with the decreased frequency of Treg in PBMC, while FOXP3 gene variants were associated with affection status, but not with Treg frequency. CONCLUSION: SLE patients have impaired Treg production or maintenance, a trait strongly associated with SLE disease activity and autoantibody titers, and possibly resulting from the inability to convert FOXP3+CD25- into FOXP3+CD25+ T cells. Treg frequency is highly heritable within SLE families, with specific variants of the CTLA4 and TGFbeta genes contributing to this trait, while FOXP3 contributes to SLE through mechanisms not involving a modulation of Treg frequency. These findings establish that the genetic components in SLE pathogenesis include genes related to Treg generation or maintenance.

Cardiomyopathy in Churg-Strauss syndrome.

The authors present the case of a 56-year-old man, admitted to the hospital twice in ten days for acute coronary syndrome with normal coronary angiograms. In the second hospitalization, the patient had anginal crises that did not respond to anti-ischemic therapy, associated with cough and wheezing. The echocardiogram revealed worsening left ventricular systolic dysfunction. He had no cardiovascular risk factors but there was a history of bronchial asthma, allergic rhinitis and peripheral neuropathy of the left upper limb with paresthesias. Laboratory studies showed eosinophilia, detected in previous blood tests, although more marked than before. Chest X-rays showed non-fixed pulmonary infiltrates and bronchoalveolar lavage revealed increased lymphocytes and eosinophils, suggesting Churg-Strauss syndrome with the probable cardiac manifestation of coronary vasospasm. A cardiac MRI was also performed but was inconclusive due to the patient's intolerance of the exam.

Myocardial infarction as the first probable manifestation of caseous calcification of the mitral annulus.

Calcification of the mitral annulus is a common echocardiographic finding during routine evaluation of patients. Caseous calcification of the mitral annulus (CCMA) on the other hand is a rare variant, occurring in about 0.06-0.07% of echocardiographic studies. The authors present the case of a 73-year-old woman admitted to hospital with an anterior wall acute coronary syndrome, in whom transthoracic echocardiography showed a well-defined echogenic rounded mass measuring 27 x 22 mm in diameter attached to the posterior mitral annulus. After transesophageal echocardiography and magnetic nuclear imaging, which confirmed the characteristics of the mass, the patient underwent surgical resection of the mass and mitral replacement with a mechanical prosthetic valve. Histological examination confirmed the diagnosis. Systemic embolism associated with CCMA is a rare but possible complication and was probably the cause of the acute coronary syndrome in this patient.

Primary cardiac lymphoma in a patient with concomitant renal cancer.

Primary cardiac lymphoma is defined as non-Hodgkin lymphoma involving the heart and/or pericardium. It is a rare cancer that primarily affects the right heart and in particular the right atrium. By contrast, renal cell carcinoma is a relatively common cancer, which in rare circumstances can metastasize to the heart. It is now known that there is an association between non-Hodgkin lymphoma and renal cell carcinoma, although the underlying mechanisms are not fully understood. The authors present a case of primary cardiac non-Hodgkin lymphoma in a patient with concomitant renal cell carcinoma and explore the possible reasons for this association.

Effusive-constrictive pericarditis as the manifestation of an unexpected diagnosis.

Constrictive pericarditis is a clinical condition characterized by the appearance of signs and symptoms of right heart failure due to loss of pericardial compliance. Cardiac surgery is now one of the most frequent causes in developed countries, while tuberculosis remains the most prevalent cause in developing countries. Malignancy is a rare cause but usually has a poor prognosis. The diagnosis of constrictive pericarditis remains a clinical challenge and requires a combination of noninvasive diagnostic methods (echocardiography, cardiac magnetic resonance and computed tomography); in some cases, cardiac catheterization is needed to confirm the diagnosis. The authors present the case of a 51-year-old man, hospitalized due to cardiac tamponade. Diagnostic investigation was suggestive of tuberculous etiology. Despite directed medical therapy, the patient developed effusive-constrictive physiology. He underwent pericardiectomy and anatomopathologic study suggested a neoplastic etiology. The patient died in the postoperative period from biventricular failure.

Elevated factor VIII in a patient with acute coronary syndrome.

Factor VIII is a clotting factor that plays a crucial role in the coagulation cascade. Above-normal levels are found in 11% of the general adult population. Various studies have established a causal association between elevated factor VIII and venous thrombosis; some studies also suggest a relation with arterial thrombosis, particularly myocardial infarction and stroke. We report the case of a 36-year-old man with obesity, smoking and dyslipidemia as cardiovascular risk factors and a history of acute myocardial infarction at age 26. He was admitted to the coronary care unit with a diagnosis of ST-elevation myocardial infarction. Coronary angiography showed a thrombus in the distal segment of the first obtuse marginal artery, which was causing the obstruction. The thrombus was aspirated but there was no reflow. A coagulation study revealed elevated factor VIII; other parameters were normal. Even though this patient presented several cardiovascular risk factors, we highlight the need for more studies on the effect of elevated factor VIII on thrombus formation leading to acute coronary syndrome. Another important question is the use of oral anticoagulation in these patients as an integral part of the management of acute coronary syndrome.

Alexithymia in systemic lupus erythematosus patients.

This study aimed to determine the prevalence of alexithymic characteristics in patients with systemic lupus erythematosus (SLE), comparing them with a population of healthy subjects. Fifty-three SLE patients [American College of Rheumatology (ACR) criteria] and 31 healthy volunteer subjects were administered validated scales for alexithymia (Toronto alexythimia scale-20), psychopathology (brief symptom inventory; hospital anxiety and depression scale), personality dimensions (NEO five-factor inventory), and quality of life (short form-36 health survey). The SLE patient's clinical and laboratory evaluations were performed by computerized indicators of activity (SLE disease activity index), of accumulated damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index), length of disease, and therapy. A high prevalence of alexithymia was found in SLE patients. Alexithymia was associated with psychopathology, personality, and quality of life dimensions. Clinical variables and therapy were not correlated significantly with alexithymia, psychopathology, or quality of life dimensions. Multiple regression analysis showed that openness and depression were the two predictors for alexithymia in SLE patients. The present findings showed that alexithymia may play an important role in SLE patients. The difficulty in the management of emotions may lead to psychological distress and instability affecting the patient's quality of life, a relevant finding for the psychological, psychiatric, and clinical intervention and approach.

[Genetic polymorphisms of low molecular weight protein tyrosine phosphatase (LMW-PTP): relationship with erythrocyte enzymatic phenotype in patients with Systemic Lupus Erythematosus]. / Polimorfismos Genéticos da Proteína Tirosina Fosfatase de Baixo Peso Molecular (LMW-PTP): sua relaçao com o fenótipo enzimático eritrocitário em doentes com Lúpus Eritematoso Sistémico.

Systemic Lupus Erythematosus (SLE) is a multisystemic autoimmune disease characterized by the loss of self-tolerance revealing defective immune regulatory mechanisms. ACP1 is a highly polymorphic erythrocyte LMW-PTP isozyme encoded by a gene presenting three alleles (A B C). These alleles determine different fast slow isozyme proportions having distinct cellular localization and biological functions. Several studies demonstrated the involvement of LMW-PTP in the PDGFr, IL4 and insulin dependent cellular signalling regulation. The most relevant phenotypic effects of LMW-PTP over expression are the strong reduction of cell growth rate in response to PDGF stimulation together with up regulation of cell adhesion and chemotaxis. Our aims were the characterization of LMW-PTP genetic polymorphisms and the evaluation of erythrocyte LMW-PTP enzymatic activity in a lupus and a control population group and the determination of genotype phenotype relationship in SLE. The results revealed a predominance of AA (n=8) and AB (n=21) genotypes in SLE which are responsible for the low enzymatic activity forms while in the control group there was a predominance of high enzymatic activity genotypes AB (n=52) and BB (n=36). The difference between the two groups was statistically significant (p=0,04 - chi2 for 4 freedom degrees). The LMW-PTP median enzymatic activity in the SLE group (260.53+/-96.18mmol p-nitrophenolate/gHb/h) was lower than in the control group (339.84+/-113.78mmol p-nitrophenolate/gHb/h) (p<0.001). These results suggest a relevant role for LMW-PTP in the context of SLE namely at cellular proliferation and oxidative stress level.