Coarctação Isolada da Aorta: Experiência em 100 Doentes Consecutivos

INTRODUCTION: Coarctation of the aorta (CoA) is a stenosis usually located in the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and presents excellent immediate results but significant residual problems often persist. OBJECTIVES: To describe the presentation, treatment and long-term evolution of a population of 100 unselected consecutive patients with isolated CoA in a single pediatric cardiology center. METHODS: This was a retrospective study of all patients with isolated CoA treated during4 the last 21 years (1987-2008). RESULTS: The patients (n=100, 68.3% male) were diagnosed at a median age of 94 days (1 day to 16 years). The clinical presentation differed between patients aged less or more than one year, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (88 and 63%, respectively; p < 0.01). Treatment, a median of 8 days after diagnosis, was surgical in 79 cases (20 end-to-end anastomosis, 31 subclavian flap, 28 patch) and percutaneous in the remaining 21 (15 balloon angioplasty, 6 with stenting). The mean age of surgical patients was younger than in those treated percutaneously (3.4 vs. 7.5 years; p < 0.01). Immediate mortality was 2% and occurred in the surgical group. There was no late mortality, in a mean follow-up of 7.2 +/- 5.4 years. Recoarctation occurred in 8 patients (6 surgical, 2 percutaneous). There are 46 patients who currently have hypertension (19 at rest, 27 with effort), their median age at diagnosis being older than the others (23 vs. 995 days; p < 0.01). CONCLUSIONS: Isolated CoA has an excellent short-term prognosis but a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment. Its potentially insidious presentation requires a high level of clinical suspicion, femoral pulse palpation during physical examination of newborns and older children being particularly important. Delay in treatment has an impact on late morbidity and mortality. Taking into account the data currently available on late and immediate results, the final choice of therapeutic technique depends on the patient's age, associated lesions and the experience of the medical-surgical team. Hypertension should be closely monitored in the follow-up of these patients, as well as its risk factors and complications.

Multifocalidade e Estereopsia

Introdução: A visão binocular proporciona uma interpretação única e tridimensional do mundo. Objectivo: Comparar a estereopsia dos doentes com de lentes intra-oculares (LIO) multifocais implantadas. Métodos: Os autores realizaram um estudo transversal com 40 doentes, que tinham sido submetidos a facoemulsificação com implante de LIO multifocal bilateralmente (26 Restor, 6 tecnis Multifocal e 8 "Mix & Match"). Estudaram a estereopsia de longe com o Vectograph e utilizaram o "Titmus Stereo Test" para perto, nos 3 grupos. Resultados: No grupo 1 (Restor bilateral: idade média 63,38 anos; 4 doentes do sexo masculino e 22 do feminino) obtivemos 80 a 90% de boa estereopsia; no grupo 2 (Tecnis Multifocal bilateral: idade média 73,67 anos; 6 doentes do sexo feminino) registou-se 100% de boa estereopsia e no grupo 3 ("Mix & Match": idade média 74,25 anos; 2 doentes do sexo masculino e 6 do feminino) obteve-se 50 a 75% de boa estereopsia. Conclusões: Os doentes com lentes intra-oculares multifocais similares implantadas bilateralmente obtiveram melhor estereopsia para perto e para longe.

Development Process and Cognitive Testing of CARATkids - Control of Allergic Rhinitis and Asthma Test for Children

Background: Allergic rhinitis and asthma (ARA) are chronic inflammatory diseases of the airways that often coexist in children. The only tool to assess the ARA control, the Control of Allergic Rhinitis and Asthma Test (CARAT) is to be used by adults. We aimed to develop the Pediatric version of Control of Allergic Rhinitis and Asthma Test (CARATkids) and to test its comprehensibility in children with 4 to 12 years of age. Methods: The questionnaire development included a literature review of pediatric questionnaires on asthma and/or rhinitis control and two consensus meetings of a multidisciplinary group. Cognitive testing was carried out in a cross-sectional qualitative study using cognitive interviews. Results: Four questionnaires to assess asthma and none to assess rhinitis control in children were identified. The multidisciplinary group produced a questionnaire version for children with 17 questions with illustrations and dichotomous (yes/no) response format. The version for caregivers had 4-points and dichotomous scales. Twenty-nine children, 4 to 12 years old, and their caregivers were interviewed. Only children over 6 years old could adequately answer the questionnaire. A few words/expressions were not fully understood by children of 6 to 8 years old. The drawings illustrating the questions were considered helpful by children and caregivers. Caregivers considered the questionnaire complete and clear and preferred dichotomous over the 4-points scales. The proportion of agreement between children and their caregivers was 61%. The words/expressions that were difficult to understand were amended. Conclusion: CARATkids, the first questionnaire to assess a child’s asthma and rhinitis control was developed and its content validity was assured. Cognitive testing showed that CARATKids is well-understood by children 6 to 12 years old. The questionnaire’s measurement properties can now be assessed in a validation study.

Optimal Cut-Off Value for Homeostasis Model Assessment (HOMA) Index of Insulin-Resistance in a Population of Patients Admitted Electively in a Portuguese Cardiology Ward

INTRODUCTION: Insulin resistance is the pathophysiological key to explain metabolic syndrome. Although clearly useful, the Homeostasis Model Assessment index (an insulin resistance measurement) hasn't been systematically applied in clinical practice. One of the main reasons is the discrepancy in cut-off values reported in different populations. We sought to evaluate in a Portuguese population the ideal cut-off for Homeostasis Model Assessment index and assess its relationship with metabolic syndrome. MATERIAL AND METHODS: We selected a cohort of individuals admitted electively in a Cardiology ward with a BMI < 25 Kg/m2 and no abnormalities in glucose metabolism (fasting plasma glucose < 100 mg/dL and no diabetes). The 90th percentile of the Homeostasis Model Assessment index distribution was used to obtain the ideal cut-off for insulin resistance. We also selected a validation cohort of 300 individuals (no exclusion criteria applied). RESULTS: From 7 000 individuals, and after the exclusion criteria, there were left 1 784 individuals. The 90th percentile for Homeostasis Model Assessment index was 2.33. In the validation cohort, applying that cut-off, we have 49.3% of individuals with insulin resistance. However, only 69.9% of the metabolic syndrome patients had insulin resistance according to that cut-off. By ROC curve analysis, the ideal cut-off for metabolic syndrome is 2.41. Homeostasis Model Assessment index correlated with BMI (r = 0.371, p < 0.001) and is an independent predictor of the presence of metabolic syndrome (OR 19.4, 95% CI 6.6 - 57.2, p < 0.001). DISCUSSION: Our study showed that in a Portuguese population of patients admitted electively in a Cardiology ward, 2.33 is the Homeostasis Model Assessment index cut-off for insulin resistance and 2.41 for metabolic syndrome. CONCLUSION: Homeostasis Model Assessment index is directly correlated with BMI and is an independent predictor of metabolic syndrome.

Anti-Phospholipase A2 Receptor Antibodies in the Diagnosis of Idiopathic Membranous Nephropathy

Circulating anti-phospholipase A2 receptor antibodies (anti-PLA2R) have been described in 70% to 80% of the patients with idiopathic membranous nephropathy (iMN), but not in patients with secondary membranous nephropathy or other glomerular diseases. The goal of this study was to evaluate the sensitivity and specificity of the assay for anti-PLA2R in the diagnosis of iMN. Anti-PLA2R IgG, Elisa and immunofluorescence tests were used to detect circulating anti-PLA2R. These tests were applied in 53 patients who had a kidney biopsy. Of these, 38 had histological diagnosis of membranous nephropathy (MN) and the remaining had other glomerular diseases. The MN was classified as idiopathic in 33 patients after clinical exclusion of secondary causes. Anti-PLA2R were positive in 57.6% of the patients with iMN. All patients with secondary membranous nephropathy or other glomerular diseases did not show circulating anti-PLA2R. The sensitivity was 57.6% (CI 39.2-74.5) and specificity 100% (CI 47.8-100), AUC 0.788; p < 0.0001 for the detection of iMN. 71.4% of the iMN patients that tested negative for anti-PLA2R were in partial or complete remission. The detection of anti-PLA2R in the studied population had a specificity of 100% for the iMN diagnosis. Prior treatments seem to make the test negative and contribute to a lower sensitivity.

Falências de Orgão em Pediatria

Objectivos: 1) Caracterizar as falências mono (OF) e multiorgão (MOF) numa Unidade de Cuidados Intensivos Pediátricos em relação a; altura do internamento em que ocorrem; associação de orgãos em falência e evolução dos doentes com falência mono e multiorgão. 2) Avaliar a performance de um índice de gravidade, o Pediatric Risk of Mortality (PRISM), para a população total da Unidade e para o grupo das falências multiorgão. 3) Identificar marcadores de risco de mortalidade nos doentes com MOF. Métodos: Revisão de uma base de dados e análise retrospectiva de todos os doentes internados em relação aos critérios de OF e MOF, sugeridos por Wilkinson et al. População: Total de doentes internados na Unidade de Cuidados Intensivos Pediátricos (UCIP) de um Hospital Terciário, durante um período de dois anos (Abril de 1991 a Março de 1993). Resultados Principais / Conclusões: Foram avaliados 1120 doentes, com uma média de idades de 45.9 ± 51.1 meses, sendo 961 (85.8%) médicos e 159 (14.2%) cirúrgicos. Eram previamente saudáveis 695 (62.1%), sendo os restantes 424 (37.9%) portadores de doença crónica. A mortalidade global foi de 5% (56/1120 doentes). Cento e oitenta e sete doentes (16.7%) preencheram critérios de falência mono-orgão (OF), destes, 180 (96.3%) estavam em OF já à entrada e 7 (3.7%) tiveram falência não simultânea de mais de um orgão. A mortalidade dos doentes com falência mono-orgão foi de 3.7% (7 doentes). Cento e um doentes (9.02%) tiveram falência multiorgão (MOF), definida como falência simultânea de dois ou mais orgãos, em qualquer altura do internamento. Existia MOF já à entrada em 90 doentes (89.1%). Houve 47 doentes com falência máxima de 2 orgãos (46.6%), 42 (41.6%) com falência de 3 orgãos. 10 (9.9%) com falência de 4 orgãos e 2 (1.98%) com falência de 5 orgãos. A mortalidade por número de orgãos em falência foi respectivamente de 23.4%; 66.7%; 80% e 100%. A mortalidade global dos doentes com falência multiorgão foi de 48.5% (49/101 doentes). O PRISM revelou um bom valor predictivo quando aplicado na totalidade dos doentes: discriminação (W) (avaliada pela área sob curvas ROC) W = 0.959 SE = 0.00085 e calibração (H) (avaliada pelo Hosmer-Lemesshow goodness-of-fit test) H = 13.217 p = 0.104. Estes valores permitem considerar este índice de gravidade como estando bem aferido para a população da Unidade. Quando aplicado ao grupo das MOF a discriminação foi aceitável (W = 0.732 SE = 0.036) mas a calibração foi má (H = 29.780 p = 0.00026). A análise multivariada mostrou que um score de PRISM % 15 e um número de orgãos em falência % 3, tanto na admissão como em qualquer altura do internamento, têm uma importância significativa na probabilidade de morte.

Percutaneous Treatment of Mitral Valve Regurgitation: Initial Experience with the MitraClip Device

INTRODUCTION: Mitral regurgitation (MR) is the most common valvular disease and has recently become the target of a number of percutaneous approaches. The MitraClip is virtually the only device for which there is considerable experience, with more than 20,000 procedures performed worldwide. OBJECTIVE: To describe our initial experience of the percutaneous treatment of MR with the MitraClip device. METHODS: We describe the first six MitraClip cases performed in this institution (mean age 58.5 ± 13.1 years), with functional MR grade 4+ and New York Heart Association (NYHA) heart failure class III or IV (n=3), with a mean follow-up of 290 ± 145 days. RESULTS: Procedural success (MR ≤ 2+) was 100%. Total procedure time was 115.8 ± 23.7 min, with no in-hospital adverse events and discharge between the fourth and eighth day, and consistent improvement in the six-minute walk test (329.8 ± 98.42 vs. 385.33 ± 106.95 m) and in NYHA class (three patients improved by two NYHA classes). During follow-up there were two deaths, in two of the four patients who had been initially considered for heart transplantation. CONCLUSION: In patients with functional MR the MitraClip procedure is safe, with both a high implantation and immediate in-hospital success rate. A longer follow-up suggests that the clinical benefit decreases or disappears completely in patients with more advanced heart disease, namely those denied transplantation or on the heart transplant waiting list.