RESUMO
OBJECTIVE: To evaluate through a retrospective cohort the anatomy and results of patients that were operated of truncus arteriosus. MATERIAL AND METHODS: Historic cohort. From January 2000 to December 2005 twenty eight patients with troncus arteriosus were operated. RESULTS: There were fifteen male (53.6%) and 13 female patients (46.4%). The median age, at the time of the surgery, was 10.5 months. The median weight, at the time of the surgery was 6 kg. All the patients were dominant aortic. A woven-dacron tube was used in 25 cases, Hanckock to 2 and Barbero Marcial procedure in one. The media diameter for the tube for the 27 patients (96.4%) was of 14 +/- 2 mm. Two patients required aortic valvular replacement at the same time of the surgery and two more during follow up, after the first surgery. Fifty per cent of the patients had pulmonary hypertension crisis after de surgery. There were three deaths: one patient had cardiogenic shock; another had pulmonary hypertension and one more had obstruction of the traqueostomy cannula. During study monitoring, 3 patients (10.7%) needed interventionist procedures. One case needed dilatation of the tube and two cases needed dilatation of the pulmonary branches to implant a stent device. There has been one tube change 4.2 years after the correction. Actuarial survival after 30 days was of 96.42%, and 88.9% after one and five years. CONCLUSIONS: The surgical correction of the truncus arteriosus has allowed changing the natural history of this disease. Mortality is low however our follow up is not very long yet.
Assuntos
Persistência do Tronco Arterial/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
Patients with hypoplastic left heart syndrome without surgery dye during the first year of life. In the literature there is only one report from our institution of a patient who survived to the second decade of life without surgery. The purpose of this work is to describe a case of a 24 years old female with hypoplastic left heart syndrome, who has survived until now in functional class I of New York Heart Association without surgery. In our patient, the survival is probably related with the presence of a ductus arteriosus, an interatrial septal defect and the secondary pulmonary arterial hypertension.
Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Adulto , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/terapia , SobreviventesRESUMO
OBJECTIVE: Kawasaki disease (KD) is a systemic vasculitis that affects young children. Coronary artery aneurisms, ectasia and stenosis are its main complications and may lead to ischemic heart disease or sudden death. Echocardiography evaluation it's mandatory in all patients with history of KD. Left ventricular longitudinal systolic strain (LVLSS) measured by speckle tracking it's an accurate tool to evaluate global and segmental left ventricle mechanics. Clinical utility of LVLSS in children with KD hasn't been established. The goal of this study was to analyse if the presence of coronary lesions alters segmental LVLSS and if there is a relationship with the affected coronary territory. METHOD: Case series. A complete transthoracic echocardiography with LVLSS was performed in children with history of KD with at least 6 months after the acute phase. RESULTS: Nine patients where studied, with a median age of 6 years (minimum 2 and maximum 17). A percentage of 56 were male, and 77% had coronary aneurisms. An abnormal LVLSS was found in 56% of the population studied. All of the patients that had an abnormal LVLSS had coronary aneurisms with stenosis or complete occlusion confirmed by invasive coronary angiography and abnormal Nuclear Medicine perfusion scans. CONCLUSIONS: On the population studied, all patients with an abnormal LVLSS had obstructive coronary lesions and ischemic heart disease.
Assuntos
Doença da Artéria Coronariana/etiologia , Ventrículos do Coração/patologia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/patologia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia , Feminino , Humanos , Masculino , SístoleRESUMO
The most important feature of right atrial isomerism in the heart is the presence of both atria with morphologically right atrial appendages. The main aim of this study was to do a description of clinical and echocardiographic findings in adult patients with right atrial isomerism. A total of eleven consecutive patients were identified with a diagnosis of right atrial isomerism and complex congenital heart disease. A complete clinical history and transthoracic and/or transesophageal echocardiography were performed in all patients. Also a cardiac catheterization was realized in seven patients. The functional class according the New York Heart Association was I in one patient, II in 5 and III in 5. The laboratory studies showed severe polyglobulia. In 3 cases Howell-Jolly bodies were present. The electrocardiogram showed migratory pacemaker in the 54.5% of cases. The cardiac position was dextrocardia in six patients (54.5%) and levocardia in five (45.5%). Six patients (54.5%) had complete atrioventricular septal defects, 4 (36.4%) double inlet and one absence of a right atrioventricular connection (9.1%). The most frequent ventriculoarterial connection was double-inlet in 8 (72.76%) patients. In 4 cases hypoplasia of the pulmonary artery was found. Six patients had severe pulmonary stenosis and one pulmonary atresia. The pulmonary venous drainage was into the left-sided atrium in 3 cases (27.2%), into the right-sided atrium in 4 (36.4%), into the midline of the atrium through a collector in 3 cases (27.4%) and by vertical vein in one (9.0%). Insufficiency of the atrioventricular valve was found in 7 cases (63.6%). Three patients (27.3%) went to surgery for implantation of a fistula. It is a series of adult patients with right atrial isomerism and complex congenital heart disease that survived to adult live. The clinical and echocardiographic assessment actually allows establishing the correct diagnosis, to take therapeutic decisions and to do the follow-up in all patients. Till now we could not yet determine the differences of these patients in relation to the general population when they develop chronic degenerative disease.
Assuntos
Átrios do Coração/anormalidades , Átrios do Coração/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Adolescente , Adulto , Feminino , Átrios do Coração/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , UltrassonografiaRESUMO
Primary heart tumors are not very common, frequent ones during childhood are the rhabdomyomas. This diagnostic can be made since fetal life. Evolution variates, in some cases there are no symptoms and the diagnostic represents an echocardiographic finding, other cases are detected associated with tuberous sclerosis and a small group express by arrhythmias or low output. Because of the strange of this presentation this case is presented.
Assuntos
Neoplasias Cardíacas/patologia , Rabdomioma/patologia , Procedimentos Cirúrgicos Cardíacos , Ecocardiografia Doppler , Evolução Fatal , Doenças Fetais/diagnóstico por imagem , Doenças Fetais/patologia , Doenças Fetais/terapia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Humanos , Recém-Nascido , Masculino , Radiografia Torácica , Rabdomioma/diagnóstico por imagem , Rabdomioma/cirurgia , Ultrassonografia Pré-NatalRESUMO
We present a case of a patient with a cardiac malformation that represents a form of embryo-anatomical transition of an atrioventricular septal defect between a 2 valves form to a common valve form. This entity was associated with pericardium absence. Throughout several studies we have precisely established a diagnostic sequence by determining the adequate contribution of each method and we have been able to clear out the proper nomenclature of the atrioventricular cushion defect.
Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Valvas Cardíacas/anormalidades , Valvas Cardíacas/diagnóstico por imagem , Pericárdio/anormalidades , Pericárdio/diagnóstico por imagem , Adolescente , Feminino , Humanos , Tomografia Computadorizada por Raios XRESUMO
Pediatric Cardiology is a medical subspecialty that emerged in a systematic manner during the beginning of the 20th century. Throughout time, with the use of several methods we have been able to establish a series of diagnosis, offer surgical treatments and currently we evaluate and analyze the results of such proceedings. In the cardiac rehabilitation programs, children and adolescents are taught to identify the safety limits of their hearts, being able to relate them to their daily effort activities, providing them with a better quality of life and where they learn to live with the limitations that their illness implies.
Assuntos
Cardiopatias Congênitas/reabilitação , Adolescente , Adulto , Criança , Anticoncepção , Feminino , Humanos , Masculino , Atividade Motora , EsportesRESUMO
Resumen Objetivo La enfermedad de Kawasaki (EK) es una vasculitis sistémica cuya complicación más grave es la formación de lesiones coronarias, las cuales pueden llevar a infarto del miocardio y muerte súbita. El estudio ecocardiográfico es parte del seguimiento obligado de los pacientes con EK. La deformación sistólica longitudinal (DSL) medida mediante speckle tracking es una herramienta precisa para evaluar la función de la fibra miocárdica (longitudinal) del ventrículo izquierdo. No se ha establecido la utilidad en la práctica clínica de la DSL en niños con antecedente de EK. El objetivo de este estudio fue analizar si la presencia de lesiones coronarias condiciona alteraciones en la DSL segmentaria y su correspondencia con el territorio coronario en donde se encuentra la lesión. Método Serie de casos. Se realizó un estudio ecocardiográfico completo y la evaluación de la DSL a niños con antecedente de EK al menos 6 meses después de la fase aguda. Resultados Se estudiaron 9 pacientes. La mediana de edad fue de 6 años (mínimo 2 y máximo 17). El 56% era de sexo masculino. El 77% presentó aneurismas coronarios. La DSL resultó alterada en el 56% de la muestra estudiada. Dentro de los pacientes que presentaron una DSL anormal, todos mostraron aneurismas coronarios y lesiones estenóticas u oclusivas demostradas mediante cateterismo de arterias coronarias, además de alteraciones de la perfusión miocárdica en estudio de Medicina Nuclear. Conclusiones En la muestra estudiada, los pacientes en quienes se encontró una DSL anormal, resultaron tener lesiones coronarias que condicionaban isquemia o infarto.
Abstract Objective Kawasaki disease (KD) is a systemic vasculitis that affects young children. Coronary artery aneurisms, ectasia and stenosis are its main complications and may lead to ischemic heart disease or sudden death. Echocardiography evaluation it's mandatory in all patients with history of KD. Left ventricular longitudinal systolic strain (LVLSS) measured by speckle tracking it's an accurate tool to evaluate global and segmental left ventricle mechanics. Clinical utility of LVLSS in children with KD hasn't been established. The goal of this study was to analyse if the presence of coronary lesions alters segmental LVLSS and if there is a relationship with the affected coronary territory. Method Case series. A complete transthoracic echocardiography with LVLSS was performed in children with history of KD with at least 6 months after the acute phase. Results Nine patients where studied, with a median age of 6 years (minimum 2 and maximum 17). A percentage of 56 were male, and 77% had coronary aneurisms. An abnormal LVLSS was found in 56% of the population studied. All of the patients that had an abnormal LVLSS had coronary aneurisms with stenosis or complete occlusion confirmed by invasive coronary angiography and abnormal Nuclear Medicine perfusion scans. Conclusions On the population studied, all patients with an abnormal LVLSS had obstructive coronary lesions and ischemic heart disease.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Doença da Artéria Coronariana/etiologia , Ventrículos do Coração/patologia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/patologia , Sístole , Ecocardiografia , Estudos TransversaisRESUMO
Presentamos el caso de una paciente con una malformación cardiaca que representa una forma de transición anatomoembriológica del defecto de la tabicación atrioventricular entre la forma de 2 válvulas y la que tiene una válvula común. Esta entidad además se asoció con ausencia de pericardio. A través de los diferentes estudios se ha establecido con precisión la secuencia diagnóstica, determinando cuál fue la aportación de cada método y aclarando además la nomenclatura del defecto de la tabicación atrioventricular.
We present a case of a patient with a cardiac malformation that represents a form of embryo-anatomical transition of an atrioventricular septal defect between a 2 valves form to a common valve form. This entity was associated with pericardium absence. Throughout several studies we have precisely established a diagnostic sequence by determining the adequate contribution of each method and we have been able to clear out the proper nomenclature of the atrioventricular cushion defect.
Assuntos
Adolescente , Feminino , Humanos , Anormalidades Múltiplas , Defeitos dos Septos Cardíacos , Valvas Cardíacas/anormalidades , Valvas Cardíacas , Pericárdio/anormalidades , Pericárdio , Tomografia Computadorizada por Raios XRESUMO
La cardiología pediátrica es una subespecialidad que surgió de manera sistemática, al inicio del siglo XX. A lo largo del tiempo y a través de diversos métodos se han establecido diagnósticos, se ha ofrecido tratamiento farmacológico, intervencionista y quirúrgico y actualmente, se evalúan y analizan los resultados de dichos procedimientos. A través de los programas de rehabilitación cardiaca, se le enseña a conocer los límites seguros de su corazón en actividades de la vida diaria, brindando a los pequeños una mejor calidad de vida donde aprenderán a vivir con las limitaciones que la enfermedad trae consigo.
Pediatric Cardiology is a medical subspecialty that emerged in a systematic manner during the beginning of the 20th century. Throughout time, with the use of several methods we have been able to establish a series of diagnosis, offer surgical treatments and currently we evaluate and analyze the results of such proceedings. In the cardiac rehabilitation programs, children and adolescents are taught to identify the safety limits of their hearts, being able to relate them to their daily effort activities, providing them with a better quality of life and where they learn to live with the limitations that their illness implies.
Assuntos
Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Cardiopatias Congênitas/reabilitação , Anticoncepção , Atividade Motora , EsportesRESUMO
OBJECTIVE: To evaluate through a retrospective cohort the anatomy and results of patients that were operated of truncus arteriosus. MATERIAL AND METHODS: Historic cohort. From January 2000 to December 2005 twenty eight patients with troncus arteriosus were operated. RESULTS: There were fifteen male (53.6%) and 13 female patients (46.4%). The median age, at the time of the surgery, was 10.5 months. The median weight, at the time of the surgery was 6 kg. All the patients were dominant aortic. A woven-dacron tube was used in 25 cases, Hanckock to 2 and Barbero Marcial procedure in one. The media diameter for the tube for the 27 patients (96.4%) was of 14 +/- 2 mm. Two patients required aortic valvular replacement at the same time of the surgery and two more during follow up, after the first surgery. Fifty per cent of the patients had pulmonary hypertension crisis after de surgery. There were three deaths: one patient had cardiogenic shock; another had pulmonary hypertension and one more had obstruction of the traqueostomy cannula. During study monitoring, 3 patients (10.7%) needed interventionist procedures. One case needed dilatation of the tube and two cases needed dilatation of the pulmonary branches to implant a stent device. There has been one tube change 4.2 years after the correction. Actuarial survival after 30 days was of 96.42%, and 88.9% after one and five years. CONCLUSIONS: The surgical correction of the truncus arteriosus has allowed changing the natural history of this disease. Mortality is low however our follow up is not very long yet.
Assuntos
Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Persistência do Tronco Arterial , Procedimentos Cirúrgicos Cardíacos/métodos , Seguimentos , Estudos RetrospectivosRESUMO
Patients with hypoplastic left heart syndrome without surgery dye during the first year of life. In the literature there is only one report from our institution of a patient who survived to the second decade of life without surgery. The purpose of this work is to describe a case of a 24 years old female with hypoplastic left heart syndrome, who has survived until now in functional class I of New York Heart Association without surgery. In our patient, the survival is probably related with the presence of a ductus arteriosus, an interatrial septal defect and the secondary pulmonary arterial hypertension.
Assuntos
Adulto , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico , Síndrome do Coração Esquerdo Hipoplásico , SobreviventesRESUMO
La esencia del dextroisomerismo en el corazón es la presencia bilateral de atrios con orejuelas de morfología derecha. Generalmente forma parte de un grupo de cardiopatías congénitas complejas. El objetivo de nuestro estudio es hacer una descripción de los hallazgos clínicos y ecocardiográficos en los pacientes con dextroisomerismo atrial que han sobrevivido a la vida adulta. Se estudiaron 11 pacientes adultos con cardiopatías congénitas complejas y dextroisomerismo. A todos los pacientes se les realizó historia clínica completa, ecocardiografía transtorácica y transesofágica. La clase funcional según New York Heart Association al inicio del estudio fue: I en un paciente, II en 5 y III en 5. Los estudios de laboratorio mostraron poliglobulia importante. En tres casos se encontraron cuerpos de Howell Jolly El electrocardiograma de superficie mostró marcapaso migratorio en el 54.5%. Seis (54.5%) pacientes estuvieron en dextrocardia y 5 (45.5%) en levocardia. Seis pacientes (54.5%) tuvieron defectos de la tabicación atrioventricular, 4 con doble entrada (36.4%) y uno con ausencia de conexión auriculoventricular derecha (9.1%). La conexión ventriculoarterial más frecuente fue la doble salida en 8 (72.76%). Seis casos tuvieron estenosis pulmonary uno atresia pulmonar. El retorno venoso pulmonar fue al lado izquierdo del atrio común en tres casos (27.2%), al lado derecho del atrio común en cuatro (36.4%), al techo del atrio común a través de un colector en 3 casos (27.4%) y vía vena vertical en uno (9.0%). Ninguno de ellos presentó obstrucción. Se encontró insuficiencia de la válvula atrioventricular en 7 casos (63.6%). Tres pacientes (27.3%) fueron llevados a cirugía para colocación de fístula. Su clase funcional mejoró. Se trata de una serie de casos en pacientes adultos con dextroisomerismo atrial y cardiopatías congénitas complejas que a pesar de ello llegaron a la vida adulta. La valoración clínica y ecocardiográfica nos permiten en la actualidad establecer el diagnóstico, tomar decisiones terapéuticas y hacer el seguimiento. Aún falta por determinar qué diferencias tendrán con el resto de la población cuando se presenten los padecimientos crónicos degenerativos.
The most important feature of right atrial isomerism in the heart is the presence of both atria with morphologically right atrial appendages. The main aim of this study was to do a description of clinical and echocardiographic findings in adult patients with right atrial isomerism. A total of eleven consecutive patients were identified with a diagnosis of right atrial isomerism and complex congenital heart disease. A complete clinical history and transthoracic and/or transesophageal echocardiography were performed in all patients. Also a cardiac catheterization was realized in seven patients. The functional class according the New York Heart Association was I in one patient, II in 5 and III in 5. The laboratory studies showed severe polyglobulia. In 3 cases Howell-Jolly bodies were present. The electrocardiogram showed migratory pacemaker in the 54.5% of cases. The cardiac position was dextrocardia in six patients (54.5%) and levocardia in five (45.5%). Six patients (54.5%) had complete atrioventricular septal defects, 4 (36.4%) double inlet and one absence of a right atrioventricular connection (9.1%). The most frequent ventriculoarterial connection was double-inlet in 8 (72.76%) patients. In 4 cases hypopla-sia of the pulmonary artery was found. Six patients had severe pulmonary stenosis and one pulmonary atresia. The pulmonary venous drainage was into the left-sided atrium in 3 cases (27.2%), into the right-sided atrium in 4 (36.4%), into the midline of the atrium through a collector in 3 cases (27.4%) and by vertical vein in one (9.0%). Insufficiency of the atrioventricular valve was found in 7 cases (63.6%). Three patients (27.3%) went to surgery for implantation of a fistula. It is a series of adult patients with right atrial isomerism and complex congenital heart disease that survived to adult live. The clinical and echocardiographic assessment actually allows establishing the correct diagnosis, to take therapeutic decisions and to do the follow-up in all patients. Till now we could not yet determine the differences of these patients in relation to the general population when they develop chronic degenerative disease.
Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Átrios do Coração/anormalidades , Átrios do Coração , Cardiopatias Congênitas , Átrios do Coração/fisiopatologia , Cardiopatias Congênitas/fisiopatologiaRESUMO
Los tumores primarios del corazón son poco comunes. Los más frecuentes durante la niñez son los rabdomiomas. El diagnóstico puede efectuarse desde la etapa fetal. La evolución es variada, en algunos casos no existe sintomatología y el diagnóstico representa un hallazgo ecocardiográfico, en otros se manifiesta por la presencia de soplo cardíaco, otros más se detectan asociados con esclerosis tuberosa y un pequeño grupo se manifiesta por arritmias o bajo gasto. Por lo raro de su presentación sobre todo en etapa fetal se presenta este caso.
Primary heart tumors are not very common, frequent ones during childhood are the rhabdomyomas. This diagnostic can be made since fetal life. Evolution variates, in some cases there are no symptoms and the diagnostic represents an echocardiographic finding, other cases are detected associated with tuberous sclerosis and a small group express by arrhythmias or low output. Because of the strange of this presentation this case is presented. (Arch Cardiol Mex 2004; 74:49-52).
Assuntos
Humanos , Recém-Nascido , Masculino , Neoplasias Cardíacas/patologia , Rabdomioma/patologia , Procedimentos Cirúrgicos Cardíacos , Ecocardiografia Doppler , Evolução Fatal , Doenças Fetais/patologia , Doenças Fetais/terapia , Doenças Fetais , Neoplasias Cardíacas , Neoplasias Cardíacas/cirurgia , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Ventrículos do Coração , Radiografia Torácica , Rabdomioma , Rabdomioma/cirurgia , Ultrassonografia Pré-NatalRESUMO
El aneurisma del septum interatrial es una malformación poco frecuente, que se ha visto en forma aislada o asociada a diversas cardiopatías congénitas y a trastornos del tejido conectivo. Se ha reportado una frecuencia en adultos del 0.2 por ciento y en niños de hasta 4.9 por ciento, lo que sugiere una remisión espontánea durante el desarrollo. Dentro de las complicaciones secundaria a esta malformación destacan: fenómenos tromboembólicos, obstrucción valvular y arritmias. La frecuencia de las arritmias supraventriculares se refiere hasta de un 16 por ciento, siendo las más habituales: taquicardia paroxística supraventricular, extrasístoles auriculares y flútter auricular. Aún existe controveria sobre las relación causal de las arritmias con los aneurismas interatriales. Se presentan dos casos de aneurismas interatriales en pacientes neonatos, que tenían como manifestación predominante arritmia cardiaca: extrasístoles supraventriculares con conducción aberrante y flútter auricular con conducción variable, respectivamente. Uno de los pacientes requirió manejo con diversos antiarrítmicos para su control. Durante su seguimiento hubo remisión de las arritmias e involución de los aneurismas, en ambos pacientes. La presencia de arritmias supraventriculares en pacientes en edad neonatal obliga a descartar como causa a los aneurismas del septum interatrial
Assuntos
Recém-Nascido , Humanos , Aneurisma Cardíaco/diagnóstico , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , Ecocardiografia/estatística & dados numéricos , Septos Cardíacos/fisiopatologiaRESUMO
Objetivo: conocer la frecuencia y tipo de alteración nutricional del niño con cardiopatía congénita. Material y Métodos: Se estudiaron 66 niños con cardiopatía gongénita. A todos se les midió peso, talla, perímetro de barazo, pliegues tricipital y subescapular; se tomaron nuestras senguíneas para biometría hematica y albúmina sérica, t también se colectó orina para determinación de creatinina. Se calcularon las áreas muscular y grasa del brazo y el índice creatina talla. Para comparar los hallazgos de los niños con ambos tipos de cardiopatía se utilizó X² tomando como significativa una p< 0.05. Resultados: 41 fueron niñas y 25 niños; 42 con cardiopatía acianógena y 24 cianógena. Cincuenta fueron desnutridos (26 compensados, 23 descompensados y uno agudo); 16 fueron normales. El 85 por ciento de los niños presentaron área muscular disminuida y 97 por ciento el área grasa. El índice creatinina/talla se encontró disminuido en 94 por ciento de los casos. No se demostraron diferencia significativas entre ambos grupos, en los parámetros estudiados. Conclusión: Los resultados sugieren que la desnutrición en el niño con cardiopatía congénita es muy frecuente, de evolución prolongada, predominantemente compensada y la afectación del estado nutricio es a expensas tanto de la masa muscular como de la grasa