Left Atrial Deformation Analysis in Patients with Corrected Tetralogy of Fallot by 3D Speckle-Tracking Echocardiography (from the MAGYAR-Path Study) / Análise por Speckle-Tracking com Ecocardiografia 3D da Deformação de Átrio Esquerdo em Pacientes com Tetralogia de Fallot Corrigida (do MAGYAR-Path Study)

Abstract Background: Three-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF). Objectives: To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Methods: The study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years). They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men). Results: Increased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls. Conclusions: Significant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair.

Resumo Fundamento: Ecocardiografia tridimensional (3D) acoplada à técnica de speckle-tracking (3DSTE) é uma nova metodologia útil para a avaliação de volumes e propriedades funcionais do átrio esquerdo (AE). Há crescente interesse científico na análise da deformação miocárdica em adultos com tetralogia de Fallot corrigida (cTOF). Objetivos: Comparar os volumes de AE, propriedades funcionais baseadas no volume e parâmetros de strain entre pacientes com cTOF e controles saudáveis pareados por idade e sexo. Métodos: A população do estudo consistiu em 19 adultos com cTOF consecutivos, em ritmo sinusal, acompanhados na Universidade Szeged, Hungria (idade média: 37,9 ± 11,3 anos; 8 homens; com correção cirúrgica aos 4,1 ± 2,5 anos de idade). Todos foram submetidos a ecocardiografia transtorácica bidimensional com Doppler padrão e 3DSTE. Os resultados foram comparados aos de 23 controles saudáveis pareados por idade e sexo (idade média: 39,2 ± 10,6 anos; 14 homens). Resultados: Aumento dos volumes de AE e redução das frações de esvaziamento de AE em relação ao ciclo cardíaco foram demonstrados em pacientes com cTOF em comparação aos dos controles. Os volumes de ejeção de AE caracterizando todas as funções do AE não diferiram entre os dois grupos. Strains de AE global e segmentar médio uni- e multidimensional, caracterizando função de reservatório de AE, estavam diminuídos em adultos com cTOF em comparação aos de controles. À semelhança dos strains de pico, reduzidos strains de AE global e segmentar médio na contração atrial, caracterizando função de bomba atrial, foram demonstrados em pacientes com cTOF em comparação aos de controles. Conclusões: Demonstrou-se significativa deterioração das funções de AE em adultos com cTOF em fase tardia após correção.

Tetralogía de Fallot con atresia pulmonar: Morfopatología y anatomía quirúrgica / Tetralogy of Fallot with pulmonary atresia: Morphopathology and surgical anatomy

Se detalla la morfopatología de la tetralogía de Fallot con atresia pulmonar como un espectro de variaciones que sirve de fundamento para destacar la anatomía quirúrgica de esta cardiopatía y se muestra la base embriológica que determina su estructura. Se estudiaron 35 corazones con el sistema secuencial segmentario. Se determinó: situs atrial, conexiones entre los segmentos cardíacos y entre el ventrículo derecho y la vasculatura arterial pulmonar. El situs atrial fue solitus, predominó la conexión atrioventricular concordante (33), las conexiones ventriculoarteriales fueron concordantes (17), doble salida de ventrículo derecho (9) y única vía de salida (9). Todos presentaron atresia valvular pulmonar; el tronco pulmonar fue hipoplásico (20), atrésico proximal (6) y ausente (9), ramas pulmonares confluentes e hipoplásicas (25), ausencia de confluencia (5), ausencia total de ramas y de conductos arteriosos (5) y colaterales aortopulmonares (10). Los corazones mostraron un espectro morfopatológico de severidad que expresa la tendencia a la desaparición de la conexión entre el ventrículo derecho y la circulación arterial intrapulmonar. Se resaltan las formas de la irrigación arterial pulmonar como fundamento para unifocalizar el flujo hacia los pulmones y se hace hincapié en la utilidad de la clasificación anatomoquirúrgica de Barbero Marcial. El conocimiento embriológico es útil para entender las conexiones vasculares del ventrículo derecho con los derivados de los sextos arcos aórticos, los vasos arteriales intrapulmonares y las colaterales aortopulmonares.

The morphopathology of tetralogy of Fallot with pulmonary atresia is detailed as a spectrum of variations which is the foundation to highlight the surgical anatomy of this cardiopathy and it is shown the embryological basis which determines its structure. Thirty five hearts were studied with the methodology of the segmental sequential system. The atrial situs, the connections between the cardiac chambers and between the right ventricle and the arterial pulmonary vasculature were determined. The atrial situs was solitus, the concordant atrioventricular connection was the most frequent (33), the ventriculoarterial connections were concordant (17), double outlet right ventricle (9) and single outlet (9). All hearts had atresia of the pulmonary valve; the pulmonary trunk was hypoplastic (20), atretic proximally (6) and completely absent (9), confluent and hypoplastic pulmonary branches (25), absence of confluence (5), complete absence of pulmonary branches and arterial ducts (5) and presence of aortopulmonary collaterals (10). The hearts show a morphopathologic spectrum of severity which documents the tendency in disappearing the connection between the right ventricle and the intrapulmonary arterial circulation. The determination of the arterial supply to the lungs is highlighted to unifocalize the blood flow toward the lungs. The usefulness of Barbero Marcial's surgical classification is emphasized. The embryologic knowledge is basic in understanding the vascular connections between right ventricle and the derivatives of embryonic sixths aortic arches, the intrapulmonary arterial vessels and the aortopulmonary collaterals.

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...

OEIS complex with major cardiac malformation: a case report.

OEIS Complex is a rare congenital multisystem defect that consists of omphalocele, exstrophy, imperforate anus and spinal defects. We report a case of such complex with additional major cardiac and other multisystem anomalies which are rarely described in literature. The authors give a review of literature on this infrequent complex along with a discussion on its pathogenesis, differential diagnosis and prenatal diagnosis.

Tetralogy of Fallot in adults--107 cases.

Clinical and haemodynamic profile of 107 adult patients above the age of 15 years with TOF was analysed. Cardiac catherization and selective cine-angiography were performed in all cases. Infundibular pulmonary stenosis, mal-alignment type of ventricular septal defect, mitral-aortic fibrous continuity and equal systolic pressures in both the ventricles and aorta were considered mandatory for the diagnosis of Tetralogy of Fallot. Aortic regurgitation was seen in 26 cases (24%), tricuspid regurgitation in 22 cases (21%), absent pulmonary valve in 3 cases (3%), branch pulmonary artery stenosis in 9 case (8.4%), major aortopulmonary collaterals in 15 cases (14%), right atrial pressure was more than 10 mmHg in 10 cases (11%) and right ventricular end diastolic pressure more than 9 mmHg in 73 cases (68%). The left ventricular end diastolic pressure was above 13 mmHg in 58 cases (54%).

Tetralogia de Fallot: consideraçöes sobre aspectos morfológicos de uma entidade descrita com perfeiçäo / Tetralogy of Fallot. Considerations About Morphological Features of an Entity Well Described

PURPOSE--To analyze some morphological aspects of the tetralogy of Fallot which have been raising controversies in the literature, due to the different approach to the nomenclature of congenital heart defects by different authors regarding the definition of double outlet right ventricle and its concomitance with tetralogy. METHODS--We reviewed the original description of the anomaly, and also analyzed the morphology of 22 anatomical specimens, describing the degree of aortic overriding, the type of ventricular septal defect, the degree of sub-pulmonary stenosis, etc. RESULTS--Regarding the degree of aortic overriding, there was only one case where that vessel connected predominantly to the left ventricle. In the remaining hearts, the degree of overriding was between 50 per cent and 75 per cent in 10 cases and greater than 75 per cent in 11. The intensity of infundibular stenosis was evaluated as mild in half the available hearts, and the pulmonary valve was bicuspid in 11 cases. Right aortic arch was present in 40 per cent of the available hearts and the ventricular septal defect had muscular borders in just one case (5 per cent). CONCLUSION--We could conclude that if double outlet is only a type of ventricle-arterial connexion, and so, depending on the degree of overriding of the aorta over the trabecular septum, we may have the malformation coexisting with the tetralogy

Tetralogía de Fallot con drenaje venoso pulmonar anómalo total: Entidad clínica rara, pero asociaciación importante / Fallot's tetralogy with total anomalous pulmonary venous drainage: rare clinical entity, but important association

Se analiza el caso de una lactante menor con diagnóstico de tetralogía de Fallot, cardiopatía congénita cianógena más frecuente en nuestro medio, pero esta vez asociada a un drenaje venoso anómalo total, persistencia de la vena cava superior izquierda drenando en el seno coronario y comunicación interauricular. La entidad clínica descrita es rara, pero muy importante a considerar en el momento de tomar una decisión quirúrgica, ya que los procedimientos paliativos en este tipo de asociación, si llegan a realizarse son catastróficos y fatales. Se hace especial énfasis en los procedimientos no invasivos, como métodos que complementan la orientación diagnóstica del caso

Contribución a la anatomía patológica de la enfermedad azul (cianosis cardíaca)

En el año que acaba de terminarse se cumplió un siglo de la descripción de la Tetralogía de Fallot, publicada en Marseille Medical en 1888 por el Dr. Etienne-Louis Arthur Fallot. Como un justo homenaje a este personaje de la historia médica, reproducimos a continuación la traducción del Resumen de ese trabajo

Anatomia quirúrgica la tetralogía de Fallot / Surgical anatomy of tetralogy of Fallot

En 131 piezas antómicas se analizaron los rasgos más relevantes de la anatomía quirúrgica de la tetralogía de Fallot, cuyo centro de interés se enfocó en los diferentes elementos obstructivos a la vía de salida del ventrículo derecho, en las bandas de posición supraventricular, los tipos de comunicación ventricular y su relación con el sistema de conducción y la irrigación coronaria. Se corroboró la región infundibular como sitio anatómico medular de esta entidad cuyo septum desviado hacia adelante y a la ezquierda originó la estenosis pulmonar y los demás elementos que conforman el cuadro embriopatológico. Los resultados reforzaron la convicción de que cada caso, además de presentar los rasgos que definen esta malformación, posee particularidades que ameritan ser analizadas con el fin de conocerlas y tenerlas presentes. Estó es útil no solo al clínico, a quien le proporciona un sustrato morfológico para una interpretación más razonada de los estudios de gabinete, sino también al cirujano a quien le ofrece una visión general y específica de la anatomía quirúrgica de esta cardiopatía. Su cabal conocimiento le permitirá una mejor compresión de sus particularidades, las que conviene tener presentes en el momento de elegir el tipo de procedimiento quirúrgico más conveniente