BACKGROUND: Despite the advances in the surgical treatment of Tetralogy of Fallot(TOF), pulmonary regurgitation (PR) remains as a possible complication with detrimental impact on right ventricular function (RV) in the long term. If these effects are not reversed timeously by replacing the pulmonary valve, serious morbidity and even mortality may occur. AIM: The aim of the study was to assess the outcomes in children who had complete repair of TOF at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH) with or without a RV outflow conduit with emphasis on the development of PR, as well as signs and symptoms that could be linked to PR. METHOD: A retrospective clinical audit on patients with a diagnosis of TOF and who had surgery at the CMJAH between 01/01/1994 and 31/12/2003 was undertaken. Data collected and entered onto a data collection sheet included ethnicity, sex, type of surgery which was either palliation with a Blalock-Taussig shunt (BTS) or corrective surgery, age at surgery, echocardiographic documentation of PR and RV size during follow-up. Other information included electrocardiogram (ECG) changes, abnormalities detected on chest X-ray (CXR), whether or not the patient was subjected to re-operation, as well as the indications of re-operation. RESULTS: Of the 72 patients with TOF and available data, fifty four patients (75%) had surgery, 50 (92.6%) of whom had corrective surgery and 4(7.4%) had palliative surgery in the form of a BTS only. Of the 50 who had corrective surgery, 2 (4%) patients had an initial BTS shunt while the remaining forty eight (96%) underwent initial primary corrective surgery. There were 46 males (63.9%) and 26 (36.1%) females. The median age at first visit was 13 months (range, 0.03 to 177), median age at corrective repair was 39.5 months (range, 3 to 210) and median age at palliation was 18 months (range, 1.5 to 29). Of the 50 patients undergoing corrective surgery, 60% had complex anatomy and 40% simple anatomy. More females compared to males had complex anatomy with a hypoplastic pulmonary valve (p= 0.047). Two patients undergoing a transannular patch (TAP) developed severe PR in the immediate postoperative period (less than 1 year) whilst no patients in the simple repair group developed severe PR (p= 0.110). In the intermediate postoperative period (1 to 5 years), there was 1 patient each in the TAP and simple repair group with severe PR (p = 0.476). Finally in the long term period (more than 5 years), 8 patients with TAP developed severe PR whilst 2 patients with simple repair developed severe PR (p= 0.005). Two patients out of fifteen patients (13.3%) with TAP had a pulmonary valve replacement (PVR). No patients with simple repair had PVR. CONCLUSION: Severe PR is an expected and serious complication accompanying all forms of repair techniques employed for the wide anatomical spectrum of TOF. In this study, comparison of the two most common types of repair, namely simple versus TAP, revealed a statistically significant occurrence of PR in the TAP group. Appropriate timing for PVR is paramount for the symptomatic patients in order to preserve RV function.

Thesis (doctoral)--Münster, 1982.

Thesis (M. Med. Sc.)--University of Hong Kong, 2004. / Also available in print.

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Submitted by Isaac Francisco de Souza Dias (isaac.souzadias@ufpe.br) on 2016-02-25T18:01:33Z No. of bitstreams: 2 license_rdf: 1232 bytes, checksum: 66e71c371cc565284e70f40736c94386 (MD5) DISSERTAÇÃO Adriana Cesar da Silveira.pdf: 1445651 bytes, checksum: b6869773e885c24e5210c5dc192f4b12 (MD5) / Made available in DSpace on 2016-02-25T18:01:33Z (GMT). No. of bitstreams: 2 license_rdf: 1232 bytes, checksum: 66e71c371cc565284e70f40736c94386 (MD5) DISSERTAÇÃO Adriana Cesar da Silveira.pdf: 1445651 bytes, checksum: b6869773e885c24e5210c5dc192f4b12 (MD5) Previous issue date: 2015-02-24 / A presente dissertação contempla um capítulo de revisão da literatura, outro de métodos e um artigo original, que apresenta os resultados do estudo. Apesar de já descrito na literatura que as cardiopatias congênitas possam cursar com comprometimento do estado nutricional, ainda não estão completamente elucidados os efeitos nutricionais da tetralogia de Fallot, sobretudo em lactentes não tratadas cirurgicamente. Objetivo: avaliar o estado nutricional de lactentes portadores de tetralogia de Fallot em fase pré-operatória. Métodos: estudo do tipo série de casos, com análise retrospectiva de prontuários de lactentes portadores de tetralogia de Fallot em fase pré-operatória, admitidos no Pronto Socorro Cardiológico Universitário de Pernambuco, entre outubro de 2007 e outubro de 2012. Foram excluídos os prematuros, portadores de síndromes genéticas e de alergia alimentar, ou aqueles cujos prontuários não possuíam todas as variáveis selecionadas para o estudo. Foram coletados dados demográficos: sexo, idade, estado de origem e procedência; clínicos: defeito cardíaco associado à tetralogia de Fallot; fração de ejeção; superfície corpórea, tempo de internamento e desfecho clínico; antropométricos: peso e comprimento; nutricionais: tipo e via de administração da dieta na admissão, tempo de aleitamento materno exclusivo; os antecedentes perinatais: peso ao nascer e tipo de parto e índices hematimétricos: hemoglobina, hematócrito, volume corpuscular médio (VCM) e o coeficiente de variação da distribuição de hemácias (RDW-CV). Para a avaliação antropométrica utilizou-se os índices peso por idade, comprimento por idade, peso por comprimento, e o índice de massa corporal para idade, expressos em escore Z, considerando-se os pontos de corte estabelecidos e atualmente recomendados pela Organização Mundial da Saúde, sendo utilizado o software AnthroPlus v3. 2.2 (WHO, 2007) para o cálculo do escore. As crianças foram dividas em dois grupos: Grupo I: tetralogia de Fallot com hipoplasia leve a moderada da artéria pulmonar ou associada a outros defeitos; e Grupo II: tetralogia de Fallot com atresia pulmonar, hipoplasia grave da artéria pulmonar ou agenesia de valva pulmonar, ambos associados ou não a outros defeitos cardíacos. As análises estatísticas foram realizadas no software SPSS, versão 13.0. Para todas as análises, considerou-se significância estatística quando p≤0,05. O projeto foi aprovado pelo Comitê de Ética do Complexo Hospitalar HUOC/Procape (parecer número 168.174/2012). Resultados: Foram estudadas 31 crianças, sendo 74,2% do Grupo I, 64,5% do sexo masculino, e idade média de 10,3 (±6,5) meses. Observou-se alta frequência do comprometimento estatural (38,7%). Destaca-se que pelo IMC/I a maioria da amostra (61,3%) encontrava-se na faixa de risco de sobrepeso e 19,3% foi classificada como eutrófica. Pelo índice comprimento para idade houve diferença entre os grupos (p=0,01), com escores mais baixos no Grupo I. Foram observadas menores médias de hematócrito e hemoglobina entre as crianças com déficit nutricional segundo o índice peso por idade. A frequência de anemia foi de 10,7% (n=28). Conclusões: Evidenciou-se alta frequência de comprometimento do estado nutricional das crianças estudadas, sobretudo do crescimento linear. É necessária a utilização de outros parâmetros hematimétricos além da hemoglobina para a detecção da anemia e deficiência de ferro nesta população, sendo o VCM e o RDW-CV índices elegíveis. / This work comprises a literature review chapter, other methods and an original article, which presents the results of the study. Although already described in the literature that congenital heart disease may present with poor nutritional status, it isn’t totally elucidated nutritional effects of tetralogy of Fallot, especially in children not treated surgically. Objective: To assess the nutritional status of infants with tetralogy of Fallot in the preoperative phase. Methods: The study case series with retrospective analysis of medical records of infants with tetralogy of Fallot in the preoperative phase, admitted to the Emergency Hospital University of Pernambuco, from October 2007 to October 2012. It were not included in this study preterms, carriers genetic syndromes and food allergic people, or those whose medical records did not have all the variables selected for the study. Demographic data: sex, age, state of origin and provenance; clinical : heart defect with tetralogy of Fallot; ejection fraction; body surface, length of hospital stay and clinical outcome; the perinatal history: birth weight and parturition type; anthropometrics: weight and length; Nutritionals: Diet type and route of administration on admission, exclusive breastfeeding duration; hemoglobin, hematocrit, mean corpuscular volume (MCV) and the coefficient of variation of the distribution of red blood cells(RDW), were collected. For anthropometric assessment included the indexes weight for age, length for age, weight for length, and body mass index for age expressed as Z score, considering the established cut-off points and currently recommended by the World Health Organization health, using the AnthroPlus v3.2.2 software (WHO, 2007) to calculate the score. The children were divided into two groups: Group I: tetralogy of Fallot with mild to moderate hypoplasia of the pulmonary artery or associated with other defects; and Group II: tetralogy of Fallot with pulmonary atresia, severe hypoplasia of the pulmonary artery or agenesis of the pulmonary valve. Statistical analyzes were performed with SPSS software, version 13.0 for all analyzes, it was considered statistically significant when p ≤ 0.05. The project was approved by the Hospital Complex of the Ethics Committee of University Hospital Oswaldo Cruz / Procape (Opinion 168 174/2012). Results: 31 children were studied, 74.2% in group I, 64.5% male, mean age of 10.3 (± 6.5) months. A higher frequency of stature involvement (38.7%). It is noted height problems more frequently the majority of the sample (61.3%) was in the overweight range of risk and 19.3% were classified as eutrophic. According to the index height for age the was difference between groups (p = 0.01), with lower scores in Group I. There were lower mean hematocrit and hemoglobin among children with malnutrition according to the index weight for age. The frequency of anemia was 10.7% (n = 28). Conclusions: It was evidenced frequency high of the compromised nutritional status of the children, especially linear growth. It is necessary to use other parameters in addition to the RBC hemoglobin to the detection of iron deficiency anemia in this population, considering the MCV and the RDW eligible indexes.

No description available.

In der vorliegenden Arbeit wurden Myokardproben des rechtsventrikulären Ausflusstraktes von Patienten mit Fallot’scher Tetralogie sowie Double Outlet Right Ventricle vom Fallot - Typ untersucht. Hintergrund der Studie waren Untersuchungen anderer Autoren an Cx43 - knock - out Mäusen, die dort Veränderungen des kardialen Phänotyps beschrieben, die sie als Fallot - artig interpretierten. Daraus wurde die Hypothese entwickelt, dass Änderungen auf der Ebene des Connexin 43 ursächlich mit der Fallot’schen Tetralogie verbunden sein könnten. Es erfolgte eine histologische Analyse von 25 Patientenproben im Hinblick auf die Lokalisation von Connexin 43 sowie N - Cadherin. Es zeigte sich eine altersabhängige Verteilung von Connexin 43 und N - Cadherin. Insbesondere Patienten der Gruppe 1 (jünger als zwei Jahre) zeigten eine Verteilung sowohl an der lateralen Zellseite, als auch am Pol der Kardiomyozyten. Mit zunehmendem Alter beschränkten sich sowohl Connexin 43 als auch N - Cadherin auf die Disci intercalares zwischen den Kardiomyozyten und befanden sich dort in enger Nachbarschaft zueinander. Des Weiteren erfolgte eine Analyse der codierenden Region des Connexin 43 Gens mittels High Resolution Melting - PCR und sich daran anschließender Sequenzierung. Es zeigten sich sowohl bei den Kontrollen als auch den Patienten bereits bekannte Single Nucleotide Polymorphismen sowie bis dato unbekannte Sequenzvariationen. Allerdings wurden keine homozygoten Veränderungen der DNA festgestellt. Auch fand sich keine der heterozygoten Veränderungen in allen untersuchten Patienten. Somit ist es unwahrscheinlich, dass ein einzelner Basenaustausch zum komplexen Krankheitsbild der Fallot’schen Tetralogie beziehungsweise zum Double Outlet Right Ventricle vom Fallot - Typ führt.

Made available in DSpace on 2015-07-22T20:50:34Z (GMT). No. of bitstreams: 0 Previous issue date: 2009-09-30. Added 1 bitstream(s) on 2015-08-11T03:25:31Z : No. of bitstreams: 1 Publico-00388.pdf: 783398 bytes, checksum: 5ad5aa9fa9c1b1087f37cc5d3c15b281 (MD5) / Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) / Introdução e objetivos: as disfunções sistólica e diastólica do ventrículo direito, secundárias às lesões residuais após a correção cirúrgica da tetralogia de Fallot, contribuem para a morbidade e mortalidade no pós-operatório tardio. Embora estas lesões residuais possam ser avaliadas pela ecocardiografia Doppler, a relação entre os parâmetros ecocardiográficos e os níveis de proBNP (fragmento N-terminal do pró- BNP), um potencial biomarcador de sobrecarga ventricular direita, ainda não está bem estabelecida. Os objetivos do estudo foram analisar, em pacientes no pós-operatório de tetralogia de Fallot, a relação dos níveis plasmáticos de proBNP com parâmetros ecocardiográficos das dimensões das cavidades cardíacas direitas e da gravidade das lesões residuais, e os possíveis marcadores ecocardiográficos de níveis elevados de proBNP. Métodos: os níveis séricos de proBNP e os parâmetros ecocardiográficos foram obtidos no mesmo dia em 49 pacientes no pós-operatório tardio de tetralogia de Fallot (idade média de 14,7 anos, 51% do sexo feminino, com tempo médio de pós-operatório de 9,5 anos). Os parâmetros Doppler ecocardiográficos analisados foram: dimensões do átrio e ventrículo direitos, funções sistólica e diastólica ventricular direita e lesões pulmonares residuais. A relação entre estas variáveis e os níveis de proBNP foi analisada com testes de correlação de Pearson e análises uni e multivariada. Os valores de corte dos parâmetros ecocardiográficos preditores de níveis elevados de proBNP foram analisados pela curva ROC. Resultados: os níveis de proBNP estavam elevados em 53% dos pacientes e correlacionaram-se com o diâmetro diastólico do ventrículo direito (r= 0,41; p=0,003), com diâmetros longitudinal (r= 0,52; p=0,0001) e transversal (r= 0,47; p=0,001) do átrio direito, com o tempo de me0,41; p=0,005) e com o índice do refluxo pulmonar (r= -0,60; p<0,001). Pela análise multivariada o índice do refluxo pulmonar (R= -597; p=0,001; IC: -913,2 a -280,8) e o diâmetro longitudinal do átrio direito (R= 7,74, p<0,001; IC: 4,18 a 11,31) foram preditores independentes de proBNP elevado. Tempo de meia-pressão menor que 64 ms e índice do refluxo pulmonar menor que 0,65 apresentaram a melhor acurácia para indicar proBNP elevado. Conclusões: os níveis de proBNP podem estar elevados no pós-operatório tardio de tetralogia de Fallot e correlacionaram-se com as dimensões das câmaras direitas e com a gravidade do refluxo pulmonar. Valores de corte das dimensões das câmaras direitas e dos índices de gravidade do refluxo pulmonar podem se constituir em parâmetros úteis no seguimento dos pacientes no pós-operatório tardio de tetralogia de Fallot com lesão residual.ia-pressão da curva de velocidade do refluxo pulmonar (r= - / Background: Although the residual lesions after surgical correction of tetralogy of Fallot (TOF) can be evaluated by Doppler echocardiography (DE), the relation of DE parameters with the proBNP level, a potential biomarker of right ventricle overload, is not well known. The objective this study was to evaluate the DE parameters and their relation to proBNP levels. Methods: proBNP plasma level and Doppler echocardiography parameters were obtained in the same day in 49 patients later after repair of TOF (mean age of 14.7 years, 51% female, mean PO time of 9.5 years). The DE parameters studied were the dimensions of the right atrium (RA) and ventricle (RV), RV diastolic and systolic function and residual pulmonary lesions. The relation between them and proBNP levels were analyzed and the cutoff values of DE parameters for elevated proBNP determined. Results: proBNP was elevated in 53% and correlated with RV diastolic diameter (r=0.41; p=0.003), RA longitudinal (r=0.52; p=0.0001) and transversal (r=0.47; p=0.001) diameters, pressure half time of pulmonary regurgitation (PR) velocity (PHT) (r= -0.42; p=0.005) and the PR index (r=-0.60; p<0.001). By multivariate analysis the PR index (r=-597; p=0,001; CI: -913.2 to –280.8) and RA longitudinal (r=7.74; p<0,001; CI 4.18 to 11.31) were independent predictors of elevated proBNP. PHT lower than 64 ms (0.76) and PRi lower than 0.65 (0.81) had the best accuracy for elevated proBNP. Conclusion: proBNP may be increased in patients after surgical repair of TOF, correlated with the size of right cardiac chambers and the severity of PR. / TEDE / BV UNIFESP: Teses e dissertações