Dilated Cardiomyopathy. New Therapeutic Approach

Inflammatory reaction has been associated with dilated cardiomyopathy. In this context, cardiac autoantibodies and inflammatory cell infiltration have been studied during the last two decades towards the understanding of their origin and the underlying pathogenic mechanisms. Recent research has increasingly focused on the development of an etiological therapeutic approach. Immunoadsorption has shown to improve clinical, echocardiographic, haemodynamic and laboratory parameters in patients with inflammatory dilated cardiomyopathy. In this article we review recent literature concerning this subject, including classification, pathophysiological mechanisms and therapy.

Understanding COPD: A Vision on Phenotypes, Comorbidities and Treatment Approach

Chronic Obstructive Pulmonary Disease (COPD) phenotypes have become increasingly recognized as important for grouping patients with similar presentation and/or behavior, within the heterogeneity of the disease. The primary aim of identifying phenotypes is to provide patients with the best health care possible, tailoring the therapeutic approach to each patient. However, the identification of specific phenotypes has been hindered by several factors such as which specific attributes are relevant, which discriminant features should be used for assigning patients to specific phenotypes, and how relevant are they to the therapeutic approach, prognostic and clinical outcome. Moreover, the definition of phenotype is still not consensual. Comorbidities, risk factors, modifiable risk factors and disease severity, although not phenotypes, have impact across all COPD phenotypes. Although there are some identified phenotypes that are fairly consensual, many others have been proposed, but currently lack validation. The on-going debate about which instruments and tests should be used in the identification and definition of phenotypes has contributed to this uncertainty. In this paper, the authors review present knowledge regarding COPD phenotyping, discuss the role of phenotypes and comorbidities on the severity of COPD, propose new phenotypes and suggest a phenotype-based pharmacological therapeutic approach. The authors conclude that a patient-tailored treatment approach, which takes into account each patient's specific attributes and specificities, should be pursued.

Aneurismas das Artérias Viscerais: Alternativa Terapêutica

The authors report two cases in which stent grafts were used to treat visceral artery aneurysms. Case number 1 was a 42-year old woman with a history of renal colic who was found to have a right renal artery aneurysm. Two 6-mm x 20-mm Wallgraft endoprosthesis (Boston Scientific, Watertown, Mass) were placed across the aneurysm neck. Case number 2 was a 72 year-old woman with a past medical history significant for hepatic angioma and hypothyroidism. She was found to have a superior mesenteric artery aneurysm that was treated with a 6-mm x 17-mm Jostent stent graft (Jomed, GmbH, Ra). In both cases the aneurysm was completely excluded and distal end-organ flow preserved. Stent graft placement is a safe and effective treatment for visceral artery aneurysms. If this approach proves durable and reproducible, it can become the method of choice for the management of visceral artery aneurysms in selected patients.

Consulta de Úlcera de Perna do Hospital de Santa Marta

Leg ulcers constitute a highly prevalent pathology in society, and are particularly common in the Angiology and Vascular Surgery outpatient clinic. The prevalence of these patients in this Department result from the fact that 70% of them display superficial and/or deep venous insufficiency of the lower limbs. To address this problem and optimize the therapeutic approaches available to the Chronic Leg Ulcer (CLU) patients, the Department of Angiology and Vascular Surgery has created, in March 2005, an appointment specific to CLU patients. An evaluation protocol was developed, including ulcer characterization, standardization of the conservative treatment, followed by surgical intervention, whenever required. The results obtained were evaluated 18 months after the onset of this protocol. Analysis of the results revealed that the majority of the patients responded positively to the new therapeutic approach, with closure of the ulcer in 43% of the patients and a significant improvement detected for an additional 30%. Furthermore, it was observed that a detailed evaluation of these patients should be regarded as a whole, followed by a standardized and targeted approach, resulting in a particularly successful approach on the treatment of this pathology.

Vasodilatadores Pulmonares Selectivos na Hipertensão Pulmonar Grave: Utilização de 3 Diferentes Tipos de Endpoint

The therapeutic approach to severe pulmonary arterial hypertension (PAH), whether primary or secondary to connective tissue disorders, thromboembolic phenomena or congenital heart disease with Eisenmenger syndrome, has evolved in recent years following the introduction of selective pulmonary vasodilators, including prostacyclin analogs and endothelin receptor antagonists. AIM: To correlate three different endpoints (6-minute walk test, Tei index and peak tricuspid regurgitation velocity by Doppler echocardiographic study) during follow-up of PAH patients under selective vasodilator therapy. METHODS: Eleven patients (9 female, age 42 +/- 18 years) with severe PAH (> or = 65 mmHg), 64% with Eisenmenger syndrome, in NYHA class > or = II, were assessed during a follow-up of 11 +/- 8 months. Eight patients were already under therapy with iloprost or bosentan. RESULTS: There was no correlation between the three endpoints before and after therapy as assessed by Pearson's correlation coefficient. There was, however, an improvement in all of them after selective vasodilatory therapy. CONCLUSION: Therapeutic response can be accurately measured by the traditional endpoint (6-minute walk test) or by echocardiographic endpoints. However, the lack of correlation between them excludes their use as alternatives in patient follow-up.

Fístulas Carótido-Cavernosas. Perspectivas de Diagnóstico e Terapêutica

Even though in clinical practice carotid cavernous fistulas (CCF) are not a frequent pathology, it should be a diagnostic hypothesis in face of a suggestive clinical presentation. We intended to review the diagnosis and the therapeutics, comparing them with the actual perspectives. Files of 25 patients with the diagnostic hypothesis of CCF, confirmed by conventional angiography in the HSAC Neuroradiology Department, were reviewed. In this group of patients, (5 males and 20 females), the fistulous aetiology was spontaneous in 10 and traumatic in 15. Clinically the symptom most often presented was diplopia (23 cases) and the most frequent encountered sign was ophthalmoplegia (20 cases). Of the Neuroradiologic investigation, CT scan (done to all patients) have shown an prominent superior ophthalmic vein as the most frequent abnormality. Angiographic study was based on Lasjaunias et al protocol (Surgical Neuroangiography, Vol. 2, Springer-Verlag). Surgery was the therapeutic approach for the traumatic fistulas; only 2 of the spontaneous were treated by endovascular route. Three patients are still under observation. In all the others cases there was a fistula exclusion.

ABO-Incompatible Liver Transplantation in Acute Liver Failure: A Single Portuguese Center Study

INTRODUCTION: ABO-incompatible liver transplantation (ABOi LT) is considered to be a rescue option in emergency transplantation. Herein, we have reported our experience with ABOi LT including long-term survival and major complications in these situations. PATIENT AND METHODS: ABOi LT was performed in cases of severe hepatic failure with imminent death. The standard immunosuppression consisted of basiliximab, corticosteroids, tacrolimus, and mycophenolate mofetil. Pretransplantation patients with anti-ABO titers above 16 underwent plasmapheresis. If the titer was above 128, intravenous immunoglobulin (IVIG) was added at the end of plasmapheresis. The therapeutic approach was based on the clinical situation, hepatic function, and titer evolution. A rapid increase in titer required five consecutive plasmapheresis sessions followed by administration of IVIG, and at the end of the fifth session, rituximab. RESULTS: From January 2009 to July 2012, 10 patients, including 4 men and 6 women of mean age 47.8 years (range, 29 to 64 years), underwent ABOi LT. At a mean follow-up of 19.6 months (range, 2 days to 39 months), 5 patients are alive including 4 with their original grafts. One patient was retransplanted at 9 months. Major complications were infections, which were responsible for 3 deaths due to multiorgan septic failure (2 during the first month); rejection episodes (4 biopsy-proven of humoral rejections in 3 patients and 1 cellular rejection) and biliary. CONCLUSION: The use of ABOi LT as a life-saving procedure is justifiable in emergencies when no other donor is available. With careful recipient selection close monitoring of hemagglutinins and specific immunosuppression we have obtained acceptable outcomes.

Síndrome de Bartter. Uma Nova Abordagem Terapêutica

A Síndrome de Bartter é uma tubulopatia hereditária perdedora de sal, rara (cerca de 1,2 novos casos por 100 000 nados vivos por ano1), caracterizada por alcalose metabólica, hipocaliémia, hiperreninémia e hiperaldosterolémia de gravidade variável. A indometacina e elevadas doses de potássio oral têm sido até hoje as estratégias terapêuticas usadas, com elevado risco de lesão gastrointestinal. Desde Abril de 2009, o aliscireno – inibidor da renina – tem sido utilizado em casos pontuais de Síndrome de Bartter no adulto, desconhecendo-se a sua utilização em Pediatria. Os autores apresentam o caso clínico de uma criança de oito anos com Síndrome de Bartter, medicada com cloreto de potássio e indometacina oral, a quem foi diagnosticada uma úlcera gástrica gigante. De modo a permitir a redução da quantidade de potássio administrado, optou-se por iniciar o aliscireno.

Abordagem Terapêutica dos Derrames Parapneumónicos e Empiemas

Face à inexistência de uma metodologia única e consensual na abordagem terapêutica dos derrames pleurais parapneumónicos e empiemas, os autores propõem um protocolo de orientação, que resultou da reflexão baseada na sua experiência e na bibliografia mais recente.