Variáveis Ecocardiográficas Predizentes de Terapêuticas Apropriadas de Taquidisritmias Ventriculares em Doentes Submetidos a Terapêutica de Ressincronização Cardíaca Combinada

INTRODUCTION: The significant risk of sudden arrhythmic death in patients with congestive heart failure and electromechanical ventricular dyssynchrony has led to increased use of combined cardiac resynchronization therapy defibrillator (CRT-D) devices. OBJECTIVES: To evaluate the echocardiographic variables in patients undergoing CRT-D that predict the occurrence of appropriate therapies (AT) for ventricular tachyarrhythmia. METHODS: We analyzed 38 consecutive patients (mean age 60 +/- 12 years, 63% male) with echocardiographic evaluation before and 6 months after CRT-D implantation. Patients with AT were identified in a mean follow-up of 471 +/- 323 days. A standard echocardiographic study was performed including tissue Doppler imaging (TDI). Responders were defined as patients with improvement in NYHA class of < or = 1 in the first six months, and reverse remodeling as a decrease in left ventricular end-systolic volume of < or = 15% and/or an increase in left ventricular ejection fraction of > 25%. RESULTS: The responder rate was 74%, and the reverse remodeling rate was 55%. AT occurred in 21% of patients, who presented with greater left ventricular end-diastolic internal diameter (LVEDD) before implantation (86 +/- 8 vs. 76 +/- 11 mm, p = 0.03) and at 6 months (81 +/- 8 vs. 72 +/- 14 mm, p = 0.08), and increased left ventricular end-systolic internal diameter (66 +/- 14 vs. 56 +/- 14 mm, p = 0.03) and lower ejection fraction (24 +/- 6 vs. 34 +/- 14%, p = 0.08) at 6 months. In the group with AT, the responder rate was lower (38 vs. 83%, p = 0.03), without significant differences in reverse remodeling (38% for the AT group vs. 60%, p = 0.426) or in the other variables. By univariate analysis, predictors of AT were LVEDD before implantation and E' after implantation. Age, gender, ischemic etiology, use of antiarrhythmic drugs, reverse remodeling and the other echocardiographic parameters did not predict AT. In multivariate logistic regression analysis, both LVEDD before implantation (OR 1.24, 95% CI 1.04-1.48, p = 0.019) and postimplantation E' (OR 0.27, 95% CI 0.09-0.76, p = 0.014) remained as independent predictors of AT. CONCLUSIONS: In patients undergoing CRT-D, episodes of ventricular tachyarrhythmia occur with high incidence, independently of echocardiographic response, with LVEDD before implantation and E' after implantation as the only independent predictors of AT in the medium-term. These results highlight the importance of combined devices with defibrillation capability.

Trombose Muito Tardia de Stent Coronário Após Suspensão de Terapêutica Antiagregante Plaquetária

Percutaneous transluminal coronary angioplasty (PTCA) with stent placement is widely used to achieve myocardial revascularization in patients with symptomatic ischemic heart disease and significant coronary artery stenosis. Drug-eluting stents are used in most patients undergoing percutaneous angioplasty. Stent thrombosis is an uncommon but serious complication, manifested mostly by sudden death or acute ST-elevation myocardial infarction. The authors report the case of a 68-year-old patient with acute anterior ST-elevation myocardial infarction. Five years previously, she had had a similar presentation and underwent primary angioplasty of the left anterior descending artery with implantation of a drug-eluting stent. The patient was discharged under antithrombotic therapy. She discontinued antiplatelet therapy and two days later suffered an acute anterior myocardial infarction. Primary angioplasty revealed stent thrombosis.

Ocular Toxoplasmosis Reactivation During Pregnancy – A Case Report

Introduction: Toxoplasmosis is caused by Toxoplasma gondii and may be acquired from food or water contaminated with cat feces or by vertical transmission. Severe fetal complications can overcome during pregnancy. There are also rare case-reports of congenital toxoplasmosis from previously immunized pregnant women; usually these women being had prior retinal toxoplasmic lesions. Immunosuppresion is one of the risk factors which accounts for some of these cases. Case report: 30 year-old pregnant woman, OI 2002, brazilian, previously healthy, admitted in Ophtalmology Department because of sudden left eye amaurosis in June, 2010. The fundoscopy revealed retinal scars suggesting previous infections; she was treated with corticoids and spiramycin for ocular toxoplasmosis reactivation. Previous serum analysis (2008) showed immunity to T. Gondii, but in July the IgM was negative and high levels of specific IgG were found (1227UI/mL). The serologic findings were later confirmed by a more accurate laboratory technique which found the IgM to be also positive. An amniocentesis was performed and it was negative for fetal transmission. Clinical and ultrasound follow-up throughout the rest of the gestational period was normal; daily spiramycin intake was maintained. An uneventful term delivery was performed. Neither the newborn’s serum analysis nor the histopathological study of the placenta were positive for congenital infection. Conclusion: Toxoplasmosis reactivation in pregnant women without immunosuppression is rare but is more likely to occur if previous post-infectious retinal scars are present. T. gondii infection is endemic in Brazil, so the geographical origin is important. If risk factors are present, fundoscopy should be performed every three months during pregnancy and one should always be aware of any visual symptoms. If you suspect reactivation, start medical prophylaxis for fetal transmission, perform amniocentesis and regular ultrasound follow-up.

Acidose Tubular Renal Distal e Surdez Neurossensorial com Mutação no Gene ATP6V1B1

A acidose tubular renal distal é uma doença rara, caracterizada pela incapacidade na acidificação da urina, condicionando acidose metabólica hiperclorémica, hipocaliémia, hipercalciúria e nefrocalcinose, o que poderá causar atraso de crescimento, alteração do metabolismo ósseo e insuficiência renal crónica. A acidose tubular renal distal associada a surdez neurossensorial é uma doença de herança autossómica recessiva, causada por mutações do gene que codifica a subunidade B1 da H+ -ATPase (ATP6V1B1). Os autores relatam os casos de duas irmãs que apresentaram má progressão ponderal, alterações iónicas, do equilíbrio ácido base e surdez neurossensorial. Foi detectada em ambas as crianças a mutação homozigótica no gene ATP6V1B1. Com estes dois casos pretende -se destacar a importância de um diagnóstico precoce nesta patologia rara.

Short QT Syndrome Presenting As Syncope: How Short Is Too Short?

We report the case of a 52-year-old man who presented to our emergency department (ED) after three episodes of syncope in the seven hours before admission. During his stay in the ED he had recurrent ventricular tachycardia (VT) requiring external electrical cardioversion. A 12-lead electrocardiogram (ECG) showed a short QT (SQT) interval (270 ms, QTc 327 ms), with frequent R-on-T extrasystoles triggering sustained polymorphic VT. After exclusion of other precipitating causes, the patient was diagnosed as having SQT syndrome (SQTS) according to the Gollob criteria. To our knowledge, this is the first known documentation of an SQT-caused arrhythmic episode on a 12-lead ECG, as well as the first reported case of SQTS in Portugal. The patient received an implantable cardioverter-defibrillator and was discharged. At a follow-up assessment 14 months later he was symptom-free, interrogation of the device showed no arrhythmic events, and the ECG showed a QT interval of 320 ms (QTc 347 ms).

Non-Progressive Leukoencephalopathy with Bilateral Temporal Cysts

We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. Cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.