Heart neoplasms in children: retrospective analysis.

BACKGROUND: The advancement of noninvasive imaging methods has resulted in the increase in diagnosis of heart neoplasms. However, the literature has few studies involving primary cardiac neoplasms in children. OBJECTIVE: To retrospectively review cases of primary heart neoplasms in children, considering the initial clinical manifestations, diagnostic tests used, surgical indication, histopathological types and immediate postoperative course. METHODS: The retrospective study was based on the assessment of medical records from 1983 to 2011. Only cases that were referred for surgical treatment during that period were included. Age at admission, prenatal diagnosis, family history, initial symptoms and the results of performed tests were assessed. Moreover, the date and indication of the surgery, intraoperative findings, the result of the histopathological analysis, as well as the immediate postoperative complications were recorded. RESULTS: Of the 18 patients studied, the most frequent clinical manifestations were dyspnea and heart murmur (7 and 6 patients, respectively); the most often used adjunct diagnostic method was echocardiography (18 patients); cavitary obstruction or ventricular inflow or outflow tract obstruction was the main indication for surgery (12 cases); the most common histological profile was rhabdomyoma (7 patients) and most patients showed good clinical outcome. CONCLUSIONS: In this study, imaging diagnosis was basically attained through echocardiography, with good correlation with intraoperative findings. Histopathological findings were consistent with the literature, with rhabdomyoma being the most common neoplasm in children. The evolution after surgical treatment was favorable in most cases.

Tumores cardíacos em criança: análise retrospectiva / Heart neoplasms in children: retrospective analysis

FUNDAMENTO: Avanço dos métodos não invasivos de imagem proporcionou o aumento no número de diagnóstico de tumores cardíacos. Apesar disso, a literatura apresenta poucos trabalhos envolvendo tumores cardíacos primários em crianças. OBJETIVO: Avaliar retrospectivamente casos de tumores cardíacos primários em crianças, considerando manifestações clínicas iniciais, exames utilizados para o diagnóstico, indicação cirúrgica, tipos histopatológicos encontrados e evolução pós-operatória imediata. MÉTODOS: O estudo foi retrospectivo, baseado na avaliação de prontuários no período de 1983 a 2011. Incluímos somente casos que foram orientados para tratamento cirúrgico no período. Avaliaram-se a idade na admissão, o diagnóstico pré-natal, a história familial, os sintomas iniciais e os resultados de exames realizados. Foram coletados, ainda, a data e indicação de cirurgia, os achados intraoperatórios, o resultado do exame histopatológico, assim como as complicações imediatas no pós-operatório. RESULTADOS: Dos 18 pacientes estudados, as manifestações clínicas mais encontradas foram dispneia e sopro cardíaco (7 e 6 pacientes, respectivamente); o método de complemento diagnóstico mais usado foi o ecocardiograma (18 pacientes); a obstrução cavitária ou do trato de entrada ou saída ventricular foi a principal indicação de cirurgia (12 casos); o perfil histológico mais encontrado foi rabdomioma (7 pacientes); a maioria dos pacientes apresentou boa evolução clínica. CONCLUSÃO: Neste estudo o diagnóstico por imagem foi basicamente ecocardiográfico, com boa correlação com os achados intraoperatórios. Os achados histopatológicos foram concordantes com a literatura, com o rabdomioma apresentando-se como o tumor mais comum em crianças. A evolução após tratamento cirúrgico mostrou-se favorável na maior parte dos casos.

BACKGROUND: The advancement of noninvasive imaging methods has resulted in the increase in diagnosis of heart neoplasms. However, the literature has few studies involving primary cardiac neoplasms in children. OBJECTIVE: To retrospectively review cases of primary heart neoplasms in children, considering the initial clinical manifestations, diagnostic tests used, surgical indication, histopathological types and immediate postoperative course. METHODS: The retrospective study was based on the assessment of medical records from 1983 to 2011. Only cases that were referred for surgical treatment during that period were included. Age at admission, prenatal diagnosis, family history, initial symptoms and the results of performed tests were assessed. Moreover, the date and indication of the surgery, intraoperative findings, the result of the histopathological analysis, as well as the immediate postoperative complications were recorded. RESULTS: Of the 18 patients studied, the most frequent clinical manifestations were dyspnea and heart murmur (7 and 6 patients, respectively); the most often used adjunct diagnostic method was echocardiography (18 patients); cavitary obstruction or ventricular inflow or outflow tract obstruction was the main indication for surgery (12 cases); the most common histological profile was rhabdomyoma (7 patients) and most patients showed good clinical outcome. CONCLUSIONS: In this study, imaging diagnosis was basically attained through echocardiography, with good correlation with intraoperative findings. Histopathological findings were consistent with the literature, with rhabdomyoma being the most common neoplasm in children. The evolution after surgical treatment was favorable in most cases.

The impact of preexisting myocardial remodeling on ventricular function early after tetralogy of Fallot repair.

BACKGROUND: Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction. METHODS: Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period. RESULTS: Histopathologic data revealed hypertrophy of the RV cardiomyocytes and increased interstitial collagen in both ventricles. Mean values of RV isovolumic acceleration decreased significantly at the third evaluation compared with the preoperative values (P = .006). RV myocardial fibrosis greater than 8.3% was associated with a probability of altered E' of at least 0.7 (odds ratio = 2.31). CONCLUSION: Preoperative histologic myocardial remodeling influenced the postoperative RV function in this group of patients with late repair. Further studies are necessary to evaluate the myocardium in younger patients and to define its influence in the long-term follow-up.

Correção de fístula coronariana sem circulação extracorpórea: técnica da sutura única / Off-pump correction of coronary fistula: the single suture technique approach

A técnica da sutura única foi desenvolvida para obter estabilização e exposição de todos os ramos coronarianos durante a cirurgia de revascularização do miocárdio, mantendo a estabilidade hemodinâmica durante o procedimento. Nós descrevemos a utilização desta técnica durante a correção de uma fístula arterial coronariana associada à dilatação coronariana direita, sem a utilização da circulação extracorpórea. A técnica sugerida é mais rápida e menos complexa do que a utilização do desvio cardiopulmonar. Além disso, essa técnica pode ser uma ferramenta útil para a correção de fístulas arteriais coronarianas em casos selecionados, permitindo a prática de abordagens menos invasivas nesses pacientes.

The single suture technique was developed to obtain stabilization and exposure of all all coronary branches during off-pump coronary artery bypass, while maintaining hemodynamic stabilily during the procedure. We describe the use of this technique during an off-pump correction of a coronary artery fistula associated with right coronary dilatation. The suggested technique is quicker and less complex than on-pump surgery. Furthermore, it can be a useful tool for congenital fistula correction in select cases, stimulating the practice of less invasive heart surgery in these patients.

The myocardium in tetralogy of Fallot: a histological and morphometric study.

BACKGROUND: Patients with Tetralogy of Fallot frequently develop ventricular dysfunction in the postoperative period. The histological basis of this functional alteration has been scarcely studied. OBJECTIVE: To evaluate myocardial remodeling in anatomical specimens, comparing the subepicardial and subendocardial regions, especially because the subendocardial region is easily approached by means of endomyocardial biopsy. METHODS: Transmural sections of myocardium from the right ventricular (RV) inflow tract, anterior wall and infundibulum, and from the left ventricular (LV) free wall were evaluated regarding the degree of cardiomyocyte hypertrophy, vascularization and interstitial fibrosis were analyzed. RESULTS: The mean diameter of subendocardial cardiomyocytes is similar to that of subepicardial cardiomyocytes in all regions, except for the RV infundibulum, in which subendocardial cardiomyocytes are significantly larger in relation to those of the subepicardium (p=0.007). The amount of interstitial collagen is in the upper limits of normal and was similar in the subendocardial layers in comparison with the subpericardial layer of each region; however, it was greater in the inflow tract and RV anterior wall than in the LV lateral wall. The numerical density of subendocardial capillaries was similar to that of the subepicardium and was lower than the mean minus two standard deviations of normal in all regions and layers, except for the infundibulum, in which the subepicardium showed normal values and the subendocardium showed values lower than the mean minus two standard deviations. CONCLUSION: The postnatal myocardial changes in Tetralogy of Fallot are homogeneously distributed in the subepicardial and subendocardial halves of the ventricular walls, except for the infundibulum, which has peculiar remodeling characteristics and, therefore, is not representative of the other ventricular regions and layers for morphometric studies.

O miocárdio na tetralogia de Fallot: estudo histológico e morfométrico / The myocardium in tetralogy of Fallot: a histological and morphometric study / El miocardio en la tetralogía de Fallot: estudio histológico y morfométrico

FUNDAMENTO: Pacientes com tetralogia de Fallot freqüentemente cursam com disfunção ventricular no período pós-operatório. A base histológica dessa alteração funcional tem sido pouco estudada. OBJETIVO: Avaliar, em espécimes anatômicos, o remodelamento miocárdico comparando as regiões subepicárdica e subendocárdica, especialmente por esta última ser facilmente abordável por meio de biópsias endomiocárdicas. MÉTODOS:Análises em cortes transmurais de miocárdio da via de entrada, parede anterior e infundíbulo do ventrículo direito (VD) e da parede livre do esquerdo (VE), foram avaliados quanto ao grau de hipertrofia de cardiomiócitos, de vascularização e fibrose intersticial. RESULTADOS:O diâmetro médio dos cardiomiócitos do subendocárdio é semelhante ao do subepicárdio em todas as regiões, com exceção do infundíbulo do VD, em que os subendocárdicos se mostraram significativamente maiores em relação aos do subepicárdio (p=0,007). A quantidade de colágeno intersticial encontra-se nos limites superiores do normal e foi similar nas camadas subendocárdicas, comparada à subpericárdica de cada região, sendo, todavia, maior na via de entrada e na parede anterior do VD, do que na parede lateral do VE. A densidade numérica de capilares do subendocárdio foi semelhante à do subepicárdio e esteve menor que a média menos dois desvios-padrão do normal em todas as regiões e camadas, com exceção do infundíbulo, em que o subepicárdio mostrava valores normais e o subendocárdio valores menores que a média menos dois desvios-padrão. CONCLUSÃO: As alterações do miocárdio pós-natal na tetralogia de Fallot estão distribuídas homogeneamente nas metades subepicárdica e subendocárdica das paredes ventriculares, com exceção do infundíbulo, que apresenta características peculiares de remodelamento e que, portanto, não é representativo das demais regiões e camadas ventriculares para estudos morfométricos.

BACKGROUND: Patients with Tetralogy of Fallot frequently develop ventricular dysfunction in the postoperative period. The histological basis of this functional alteration has been scarcely studied. OBJECTIVE: To evaluate myocardial remodeling in anatomical specimens, comparing the subepicardial and subendocardial regions, especially because the subendocardial region is easily approached by means of endomyocardial biopsy. METHODS: Transmural sections of myocardium from the right ventricular (RV) inflow tract, anterior wall and infundibulum, and from the left ventricular (LV) free wall were evaluated regarding the degree of cardiomyocyte hypertrophy, vascularization and interstitial fibrosis were analyzed. RESULTS: The mean diameter of subendocardial cardiomyocytes is similar to that of subepicardial cardiomyocytes in all regions, except for the RV infundibulum, in which subendocardial cardiomyocytes are significantly larger in relation to those of the subepicardium (p=0.007). The amount of interstitial collagen is in the upper limits of normal and was similar in the subendocardial layers in comparison with the subpericardial layer of each region; however, it was greater in the inflow tract and RV anterior wall than in the LV lateral wall. The numerical density of subendocardial capillaries was similar to that of the subepicardium and was lower than the mean minus two standard deviations of normal in all regions and layers, except for the infundibulum, in which the subepicardium showed normal values and the subendocardium showed values lower than the mean minus two standard deviations. CONCLUSION: The postnatal myocardial changes in Tetralogy of Fallot are homogeneously distributed in the subepicardial and subendocardial halves of the ventricular walls, except for the infundibulum, which has peculiar remodeling characteristics and, therefore, is not representative of the other ventricular regions and layers for morphometric studies.

FUNDAMENTO: Frecuentemente, pacientes con tetralogía de Fallot cursan con disfunción ventricular en el período postoperatorio. La base histológica de esa alteración funcional ha sido poco estudiada. OBJETIVO: Evaluar, en especímenes anatómicos, la remodelación miocárdica comparándose las regiones subepicárdica y subendocárdica, sobre todo por esta última ser fácilmente abordable por medio de biopsias endomiocárdicas. MÉTODOS: Se evaluaron análisis en cortes transmurales de miocardio de la vía de entrada, pared anterior e infundíbulo del ventrículo derecho (VD) y de la pared libre del izquierdo (VI), en cuanto al grado de hipertrofia de cardiomiocitos, de vascularización y fibrosis intersticial. RESULTADOS: El diámetro promedio de los cardiomiocitos del subendocardio se asemeja al del subepicardio en todas las regiones, con excepción del infundíbulo del VD, en que los subendocárdicos se mostraron significativamente mayores con relación a los del subepicardio (p=0,007). La cantidad de colágeno intersticial se encuentra en los límites superiores al normal y fue similar en las camadas subendocárdicas, comparada a la subpericárdica de cada región; pero se mostró mayor en la vía de entrada y en la pared anterior del VD, que en la pared lateral del VI. La densidad numérica de capilares del subendocardio se asemejó a la del subepicardio y se mostró menor que el promedio menos dos desviaciones estándar del normal, en todas las regiones y capas, con excepción del infundíbulo, en lo que el subepicardio revelaba valores normales y el subendocardio valores menores que el promedio menos dos desviaciones estándar. CONCLUSIÓN: Las alteraciones del miocardio postnatal en la tetralogía de Fallot están distribuidas de modo homogéneo en las mitades subepicárdica y subendocárdica de las paredes ventriculares, a excepción del infundíbulo, que presenta características peculiares de remodelación y que, por lo tanto, no es representativo de las otras regiones...

Off-pump correction of coronary fistula: the single suture technique approach.

The single suture technique was developed to obtain stabilization and exposure of all all coronary branches during off-pump coronary artery bypass, while maintaining hemodynamic stabilily during the procedure. We describe the use of this technique during an off-pump correction of a coronary artery fistula associated with right coronary dilatation. The suggested technique is quicker and less complex than on-pump surgery. Furthermore, it can be a useful tool for congenital fistula correction in select cases, stimulating the practice of less invasive heart surgery in these patients.

Classificaçäo anatômica e correçäo cirúrgica da atresia pulmonar com comunicaçäo interventricular / Anatomical classification and surgical repair of the pulmonary atresia with interventricular communication

OBJETIVO: Analisar as características anatômicas, o resultado das técnicas empregadas na correçäo cirúrgica de acordo com o número de procedimentos, assim como a mortalidade em cada grupo da classificaçäo de Barbero-Marcial para atresia pulmonar com comunicaçäo interventricular. CASUíSTICA E MÉTODOS: De janeiro de 1990 a novembro de 1999, 73 pacientes que foram submetidos a estudo cineangiocardiográfico previamente à primeira intervençäo cirúrgica, foram analisados. As características anatômicas das artérias pulmonares e artérias colaterais sistêmico-pulmonares, assim como as técnicas cirúrgicas que propiciaram tratamento paliativo, "paliativo definitivo" e definitivo foram estudadas. As causas de mortalidade também foram descritas. RESULTADOS: Dezenove pacientes apresentavam os segmentos pulmonares supridos por artérias pulmonares (grupo A), 45 por artérias pulmonares e artérias colaterais sistêmico-pulmonares (grupo B) e 9 somente por artérias colaterais sistêmico-pulmonares (grupo C). O grupo A apresentou maior proporçäo de tratamentos definitivos, o grupo B maior proporçäo de paliativos e o grupo C, maior proporçäo de "paliativos definitivos" (p< 0,0001). Näo houve diferença estatística significante de mortalidade entre os grupos (p=0,860), embora a proporçäo de óbitos nos grupos B e C foi mais que o dobro da proporçäo do grupo A. CONCLUSÖES: A classificaçäo de Barbero-Marcial permite estimar a probabilidade de um paciente ser submetido a tratamento paliativo, "paliativo definitivo" ou definitivo, assim como o risco de mortalidade em pacientes portadores de atresia pulmonar com comunicaçäo interventricular