RESUMO
CLINICAL DATA: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). CHEST RADIOGRAPHY: Cardiomegaly; attenuated peripheral vascular markings.Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. ECHOCARDIOGRAPHY: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. COMPUTED TOMOGRAPHY ANGIOGRAPHY: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. DIAGNOSIS: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. OPERATION: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.
Assuntos
Coração Entrecruzado , Transposição dos Grandes Vasos , Humanos , Feminino , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/diagnóstico por imagem , Lactente , Coração Entrecruzado/cirurgia , Coração Entrecruzado/diagnóstico por imagem , Ecocardiografia , Resultado do Tratamento , Transposição das Grandes Artérias/métodos , Angiografia por Tomografia Computadorizada , EletrocardiografiaRESUMO
ABSTRACT Clinical data: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. Echocardiography: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. Computed tomography angiography: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. Diagnosis: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. Operation: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.
RESUMO
OBJECTIVE: To examine the results of various myocardial revascularization techniques in pediatric patients to better understand the strategies for surgical treatment of coronary artery pathologies. METHODS: We analyzed 61 publications dedicated to the indications, methods, and results of coronary bypass surgery in children. Due to the small size of this cohort, case reports are also included in our review. RESULTS: The main indications for coronary bypass grafting in children are Kawasaki disease, myocardial revascularization as a necessary procedure during the congenital cardiac surgery, to manage intraoperative iatrogenic damage to coronary arteries, and homozygous familial hypercholesterolemia. The use of internal thoracic arteries as conduits for coronary bypass grafting in children with Kawasaki disease showed significantly better results in long-term functionality compared to autovenous conduits (87% and 44%, respectively, P<0.001). Acute and late coronary events after arterial switch operation for the transposition of the great arteries, anomalous origin of the left coronary artery from the pulmonary artery, and left main coronary artery atresia are the main congenital heart diseases where surgical correction involves interventions on the coronary arteries. CONCLUSION: The internal thoracic artery is a reliable and durable conduit that demonstrates proven growth potential in children.
Assuntos
Ponte de Artéria Coronária , Artéria Torácica Interna , Transposição dos Grandes Vasos , Criança , Pré-Escolar , Vasos Coronários , Humanos , Lactente , Artéria Torácica Interna/cirurgia , Síndrome de Linfonodos Mucocutâneos/cirurgia , Estudos RetrospectivosRESUMO
Abstract Objective: To examine the results of various myocardial revascularization techniques in pediatric patients to better understand the strategies for surgical treatment of coronary artery pathologies. Methods: We analyzed 61 publications dedicated to the indications, methods, and results of coronary bypass surgery in children. Due to the small size of this cohort, case reports are also included in our review. Results: The main indications for coronary bypass grafting in children are Kawasaki disease, myocardial revascularization as a necessary procedure during the congenital cardiac surgery, to manage intraoperative iatrogenic damage to coronary arteries, and homozygous familial hypercholesterolemia. The use of internal thoracic arteries as conduits for coronary bypass grafting in children with Kawasaki disease showed significantly better results in long-term functionality compared to autovenous conduits (87% and 44%, respectively, P<0.001). Acute and late coronary events after arterial switch operation for the transposition of the great arteries, anomalous origin of the left coronary artery from the pulmonary artery, and left main coronary artery atresia are the main congenital heart diseases where surgical correction involves interventions on the coronary arteries. Conclusion: The internal thoracic artery is a reliable and durable conduit that demonstrates proven growth potential in children.
Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Transposição dos Grandes Vasos , Ponte de Artéria Coronária , Artéria Torácica Interna/cirurgia , Estudos Retrospectivos , Vasos Coronários , Síndrome de Linfonodos Mucocutâneos/cirurgiaRESUMO
OBJECTIVE: To compare the early and long-term results of patients in whom was performed modified closed coronary transfer with the results of patients in whom was performed trap-door transfer techniques by utilizing propensity-matching analysis to provide optimal identical patient matching for the groups. METHODS: From August 2015 to December 2017, 127 consecutive patients underwent arterial switch operation due to simple and complex transposition of the great arteries, with or without additional arch and complex coronary pattern, by a single surgical team included into the study. Of these, in 70 patients it was performed modified closed coronary transfer technique and in 57 patients it was performed trap-door style coronary transfer technique. The patients were divided into two groups in terms of coronary transfer method. In the final model, after propensity matching, 47 patients from each group having similar propensity score were included into the study. RESULTS: There was no significant difference between the groups regarding patient characteristics. Cross-clamp time and operation time were significantly lower in the modified technique group compared with the other group (P=0.03 and P=0.05, respectively). When compared the early and late postoperative outcomes, there was no significant difference between the groups. Postoperative echocardiographic findings were mostly similar between the groups. CONCLUSION: The patients in whom was performed our modified technique demonstrate overall good outcomes and the current technique ensures shorter arterial cross-clamp and operation times. It may be an alternative method to the trap-door technique for the coronary transfer during the arterial switch operation.
Assuntos
Transposição das Grandes Artérias , Transposição dos Grandes Vasos , Vasos Coronários , Ecocardiografia , Feminino , Coração , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgiaRESUMO
Abstract Objective: To compare the early and long-term results of patients in whom was performed modified closed coronary transfer with the results of patients in whom was performed trap-door transfer techniques by utilizing propensity-matching analysis to provide optimal identical patient matching for the groups. Methods: From August 2015 to December 2017, 127 consecutive patients underwent arterial switch operation due to simple and complex transposition of the great arteries, with or without additional arch and complex coronary pattern, by a single surgical team included into the study. Of these, in 70 patients it was performed modified closed coronary transfer technique and in 57 patients it was performed trap-door style coronary transfer technique. The patients were divided into two groups in terms of coronary transfer method. In the final model, after propensity matching, 47 patients from each group having similar propensity score were included into the study. Results: There was no significant difference between the groups regarding patient characteristics. Cross-clamp time and operation time were significantly lower in the modified technique group compared with the other group (P=0.03 and P=0.05, respectively). When compared the early and late postoperative outcomes, there was no significant difference between the groups. Postoperative echocardiographic findings were mostly similar between the groups. Conclusion: The patients in whom was performed our modified technique demonstrate overall good outcomes and the current technique ensures shorter arterial cross-clamp and operation times. It may be an alternative method to the trap-door technique for the coronary transfer during the arterial switch operation.
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição das Grandes Artérias , Ecocardiografia , Estudos Retrospectivos , Vasos Coronários , CoraçãoRESUMO
Congenitally corrected transposition of the great arteries is a rare congenital anomaly that can remain asymptomatic until adulthood, especially when there are no other associated congenital anomalies. We report two patients in their sixth decade of life with corrected transposition of the great arteries incidentally diagnosed by transthoracic echocardiography in a preventive medical check-up. The complementary use of cardiac computed tomography confirmed the diagnosis.
Assuntos
Humanos , Transposição das Grandes Artérias Corrigida Congenitamente , Artérias , Transposição dos Grandes Vasos/diagnóstico por imagem , EcocardiografiaRESUMO
OBJECTIVE: To examine associations between measurements of neurodevelopment and psychosocial health status at age 8 and 16 years in patients with repaired dextro-transposition of the great arteries. STUDY DESIGN: In the 16-year follow-up of the Boston Circulatory Arrest Study, 137 parents completed the Child Health Questionnaire-Parent Form-50, of whom 135 had completed the Child Health Questionnaire-Parent Form-50 when their child was age 8 years. Psychosocial and physical summary scores were used to assess change in health status from age 8 to 16 years. A comprehensive battery of neurodevelopmental testing was performed at ages 8 and 16 years to examine associations with adolescent health status. RESULTS: Lower psychosocial summary scores of 16 year old subjects with dextro-transposition of the great arteries were highly associated with numerous concurrent domains of neurodevelopmental function, most notably with higher (worse) scores on the Conners' Attention Deficit Hyperactivity Disorder/Diagnostic and Statistical Manual-4th Edition Scales (parent: r = -0.62, P < .001; adolescent: r = -0.43, P < .001) and the Behavior Rating Inventory of Executive Function Global Executive Composite (parent: r = -0.66, P < .001; adolescent: r = -0.39, P < .001). Psychosocial and physical summary scores tracked from ages 8 to 16 years (r = 0.44 and 0.47, respectively, P < .001 for each). Higher (worse) scores of multiple attention measures at age 8 years predicted worse psychosocial summary scores at age 16 years. CONCLUSIONS: Attention deficits at age 8 years were highly predictive of worse psychosocial health status in adolescence. Further studies are needed to assess whether treatment of childhood attention deficit hyperactivity disorder could improve adolescent well-being.
Assuntos
Saúde do Adolescente/estatística & dados numéricos , Nível de Saúde , Transtornos do Neurodesenvolvimento/epidemiologia , Transposição dos Grandes Vasos/complicações , Adolescente , Criança , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Transtornos do Neurodesenvolvimento/etiologia , Testes Neuropsicológicos/estatística & dados numéricos , Qualidade de Vida/psicologia , Fatores de Risco , Inquéritos e Questionários , Transposição dos Grandes Vasos/psicologia , Transposição dos Grandes Vasos/cirurgiaRESUMO
Objetivo: descrever uma abordagem fisioterapêutica na reabilitação neuropsicomotora e respiratória pediátrica em paciente com transposição das grandes artérias. Relato de caso: paciente R. S. C. R, sexo masculino, nascido de parto cesáreo, com diagnóstico clínico de malformações congênitas, portador de Transposição das Grandes Artérias TGA, Comunicação Interatrial CIA e Comunicação Interventricular Ampla CIV. Avaliado aos 09 meses de idade com diagnóstico fisioterapêutico de atraso no desenvolvimento neuropsicomotor. Conclusão: esse relato de caso evidenciou efetiva abordagem fisioterapêutica na reabilitação neuropsicomotora e respiratória de uma criança com TGA + CIA + CIV em acompanhamento clínico, resultando em melhora neuropsicomotora identificada pelo melhor controle cervical e maior tempo na posição sentada, e melhora do desconforto respiratório passando de moderado para leve
Assuntos
Humanos , Masculino , Lactente , Anormalidades Congênitas , Reabilitação , Transposição das Grandes ArtériasRESUMO
Abstract Background: Jatene surgery became the surgical procedure of choice to repair transposition of the great arteries (TGA) in neonates and infants. Late complications, mainly related to the pulmonary outflow tract and coronary arteries, are well known. The behavior of the neo-aortic valve is a cause of concern because of its potential for requiring late reoperation. Objectives: To assess the prevalence and risk factors of neo-aortic valve regurgitation in 127 patients in the late postoperative period of the Jatene surgery. Methods: Of the 328 survivors of the Jatene surgery at the Biocor Institute from October 1997 to June 2015, all patients undergoing postoperative follow-up were contacted via telephone, 127 being eligible for the study. The patients were divided into two groups, simple TGA and complex TGA groups, with follow-up means of 6.4 ± 4.7 years and 9.26 ± 4.22 years, respectively. Echocardiography was performed with adjusted measurements (Z-score) of the neo-aortic annulus, sinus of Valsalva, sinotubular region and ascending aorta, as well as quantification of the neo-aortic valve regurgitation grade. Results: The incidence of mild neo-aortic valve regurgitation was 29% in a follow-up of 7.4 ± 4.7 years. Moderate regurgitation was identified in 24 patients with age mean (± standard-deviation) of 9.81 ± 4.21 years, 19 of whom (79%) in the complex TGA group. Those patients had a higher aortic annulus Z-score. The reoperation rate due to neo-aortic regurgitation associated with aortic dilation was 1.5%, all patients in the complex TGA group. Conclusion: This study shows that, despite the low incidence of reoperation after Jatene surgery due to neo-aorta dilation and neo-aortic valve regurgitation, that is a time-dependent phenomenon, which requires strict vigilance of the patients. In this study, one of the major risk factors for neo-aortic valve regurgitation was the preoperative pulmonary artery diameter (p < 0.001).
Resumo Fundamento: A operação de Jatene tornou-se o procedimento cirúrgico de escolha para o reparo da transposição das grandes artérias (TGA) em neonatos e lactentes. Complicações tardias, principalmente relacionadas à via de saída pulmonar e às artérias coronarianas, já são bem reconhecidas. O comportamento da valva neo-aórtica tem sido motivo de crescente preocupação pelo seu potencial de necessidade de reoperações tardias. Objetivos: Avaliar a prevalência e os fatores de risco associados à regurgitação da valva neo-aórtica em 127 pacientes em pós-operatório tardio de cirurgia de Jatene. Métodos: Dos 328 sobreviventes da cirurgia de Jatene no Biocor Instituto operados de outubro de 1997 a junho de 2015, todos os pacientes em seguimento de pós-operatório foram contatados via ligação telefônica, sendo 127 elegíveis para o estudo. Os pacientes foram divididos em dois grupos, Grupo TGA simples e Grupo TGA complexa, com médias de follow-up de 6,4 ± 4,7 anos e 9,26 ± 4,22 anos, respectivamente. Foi realizada avaliação ecocardiográfica com medidas indexadas (escore Z) do anel da valva neo-aórtica, do seio de Valsalva, da região sinotubular e da aorta ascendente, bem como quantificação do grau de regurgitação da valva neo-aórtica. Resultados: A incidência de leve regurgitação da valva neo-aórtica em nossa casuística foi de 29% em um follow-up de 7,4 ± 4,7 anos. Regurgitação moderada foi observada em 24 pacientes, com idade média (± desvio-padrão) de 9,81 ± 4,21 anos, sendo 19 (79%) no Grupo TGA complexa. Nesses pacientes, observou-se maior escore Z do anel aórtico. A taxa de reoperação por regurgitação da valva neo-aórtica associada a dilatação da aorta foi de 1,5%, sendo todos os casos no Grupo TGA complexa. Conclusão: O estudo demonstra que, embora reoperações após cirurgia de Jatene por dilatação da neo-aorta e regurgitação da valva neo-aórtica tenham incidência baixa, esse é um fenômeno dependente de tempo, requerendo rígida vigilância desses pacientes. Na nossa casuística, um dos principais fatores de risco para regurgitação da valva neo-aórtica foi o diâmetro da artéria pulmonar no pré-operatório (p < 0,001).
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Insuficiência da Valva Aórtica/etiologia , Complicações Pós-Operatórias/etiologia , Transposição dos Grandes Vasos/cirurgia , Transposição das Grandes Artérias/efeitos adversos , Índice de Gravidade de Doença , Ecocardiografia , Prevalência , Estudos Retrospectivos , Fatores de RiscoRESUMO
La anomalía de Taussig-Bing es una cardiopatía congénita cianosante caracterizada por la dextrotransposición de grandes vasos. Esta produce una doble salida arterial desde el ventrículo derecho, asociado a una comunicación interventricular. Este cuadro puede generar una hipertensión pulmonar secundaria al aumento de las resistencias vasculares y un flujo reverso cardiaco, conocido como síndrome de Eisenmenger. Normalmente, se presenta antes de la pubertad, aunque en ocasiones, puede debutar en la vida adulta, progresando durante dicha etapa. Clínicamente, se caracteriza por rasgos crónicos, como las acropaquias, la disnea, la sensación de cansancio o la cianosis. El diagnóstico de este tipo de cardiopatías se basa en la clínica y en las pruebas de imagen, preferentemente en el estudio ecocardiográfico fetal o durante la edad pediátrica. El tratamiento de elección es la corrección quirúrgica de las malformaciones cardiacas, siendo preferente la rectificación de la salida de la aorta y el cierre de la comunicación interventricular. El pronóstico depende del grado de hipertensión pulmonar, del momento del diagnóstico y de la corrección quirúrgica precoz. A edades tempranas se obtiene un mejor resultado, aunque las tasas de mortalidad alcanzan 50 % en algunos casos, incluso tras una corrección quirúrgica óptima. La gestación no está recomendada en pacientes que padecen dicha patología, la cual se ha contraindicado, según algunos estudios, en ausencia de tratamiento adecuado. Así pues, describimos un caso en el que una gestante con una anomalía Taussig-Bing sufre una atonía uterina y un posterior paro cardiorrespiratoria tras el parto, realizado mediante cesárea electiva, tras la que la paciente falleció(AU)
The Taussig-Bing anomaly is a congenital cyanosis characterized by the dextrotransposition of large vessels. It produces a double arterial exit from the right ventricle, associated with an interventricular communication. This may lead to pulmonary hypertension secondary to increased vascular resistance and a cardiac reverse flow, known as Eisenmenger syndrome. Generally, it occurs before puberty, although occasionally, it can debut in adults, progressing during that stage. It is clinically characterized by chronic features, such as acropachies, dyspnea, tiredness or cyanosis. The diagnosis of this type of heart disease is based on clinical exam and imaging tests, if at all possible in the fetal echocardiographic study or during the pediatric age. The surgical correction of cardiac malformations is the treatment of choice, modifying the aortic exit and closing of ventricular septal defect. The prognosis depends on the degree of pulmonary hypertension, the time of diagnosis, and the early surgical correction. Better outcome is obtained at early ages, although mortality rates reach 50% in some cases, even after optimal surgical correction. Pregnancy is not recommended in patients suffering from this disease. Some studies contraindicate pregnancy in absence of proper treatment. Thus, we describe a case of a pregnant woman with a Taussig-Bing anomaly, who suffered uterine atony and a subsequent cardiorespiratory arrest after delivery. Elective cesarean section was performed. This patient died(AU)
Assuntos
Humanos , Feminino , Gravidez , Adulto , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/mortalidade , Morte Materna , Cesárea/mortalidade , Complexo de Eisenmenger/mortalidadeRESUMO
Introduction: Valved conduits for surgically reconstructing the outflow tract of the right ventricle have improved the prognosis for certain congenital heart diseases. When they become dysfunctional, transcatheter pulmonary valve replacement is safe and effective. Clinical findings, diagnostic evaluation and interventions: We report the case of a 21 years old female patient diagnosed with transposition of great vessels corrected in childhood. After several surgeries, she presents a dysfunction of the right ventricle. In response, a "Melody " pulmonary valve was implanted percutaneously under deep sedation. Conclusion: The ideal anesthetic technique for transcatheter pulmonary valve replacement is controversial. There is a debate over the merits of general anesthesia and deep sedation. Each case must be decided on after a careful preoperative evaluation, considering the risk/benefit and patient cooperation. The experience at our center using deep sedation is promising, but we have an alternative plan to convert to general anesthesia if necessary.
Introducción: Los conductos valvulados para reconstruir quirúrgicamente el tracto de salida del ventrículo derecho han mejorado el pronóstico de ciertas cardiopatía congénitas. Cuando éstos se vuelven disfuncionantes, el remplazo valvular pulmonar transcatéter es eficaz y seguro. Hallazgos clínicos, evaluación diagnóstica e intervenciones: Presentamos una paciente de 21 años, diagnosticada de transposición de grandes vasos corregida en la infancia, que tras varias intervenciones quirúrgicas presenta una disfunción del conducto del ventrículo derecho, por lo que se implanta percutáneamente una válvula pulmonar "Melody" bajo sedación profunda. Conclusión: La técnica anestésica ideal para el recambio valvular transcatéter pulmonar es controvertida, discutiéndose entre la anestesia general y la sedación profunda. Cada caso debe decidirse tras una evaluación preoperatoria cuidadosa, considerando el riesgo/beneficio y la colaboración del paciente. La experiencia en nuestro centro empleando la sedación profunda es prometedora, aunque debemos contar con un plan alternativo para reconvertir a anestesia general de ser necesario.
Assuntos
HumanosRESUMO
Abstract Objective: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. Methods: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. Results: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). Conclusion: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.
Assuntos
Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Transposição das Grandes Artérias/métodos , Anomalias dos Vasos Coronários/cirurgia , Transposição dos Grandes Vasos/cirurgia , Transposição das Grandes Artérias/mortalidade , Derivação Arteriovenosa Cirúrgica/métodos , Anomalias dos Vasos Coronários/mortalidade , Seguimentos , Comunicação Interventricular/cirurgia , Estimativa de Kaplan-Meier , Ilustração Médica , Duração da Cirurgia , Estudos Prospectivos , Reprodutibilidade dos Testes , Resultado do Tratamento , Transposição dos Grandes Vasos/mortalidadeRESUMO
OBJETIVO: O aumento da atividade miocárdica da Glicose 6-Fosfato Desidrogenase tem sido demonstrado na insuficiência cardíaca. Este estudo avalia a atividade miocárdica da Glicose 6-Fosfato Desidrogenase no treinamento do ventrículo subpulmonar de cabras adultas. MÉTODOS: Foram utilizadas 18 cabras adultas, divididas em três grupos: convencional (bandagem fixa), sham e intermitente (bandagem ajustável; 12 horas diárias de sobrecarga). A sobrecarga sistólica (70% da pressão sistêmica) foi mantida durante quatro semanas. As avaliações hemodinâmica e ecocardiográfica foram realizadas durante todo o estudo. Depois de cumprido o protocolo, os animais foram mortos para avaliação morfológica e da atividade da Glicose 6-Fosfato Desidrogenase dos ventrículos. RESULTADOS: Apesar de haver sobrecarga sistólica proporcionalmente menor no ventrículo subpulmonar do grupo intermitente (P=0,001), ambos os grupos de estudo apresentaram aumento da massa muscular de magnitude similar. Os grupos intermitente e convencional apresentaram aumento da massa de 55,7% e 36,7% (P<0,05), respectivamente, em comparação ao grupo sham. O conteúdo de água do miocárdio não variou entre os grupos estudados (P=0,27). O ecocardiograma demonstrou maior aumento (37,2%) na espessura do ventrículo subpulmonar do grupo intermitente, em relação aos grupos sham e convencional (P<0,05). Foi observada maior atividade da Glicose 6-Fosfato Desidrogenase na hipertrofia miocárdica do ventrículo subpulmonar do grupo convencional, comparada aos grupos sham e intermitente (P=0,05). CONCLUSÃO: Ambos os grupos de treinamento ventricular desenvolveram hipertrofia ventricular, a despeito do menor tempo de sobrecarga sistólica no grupo intermitente. A maior atividade de Glicose 6-Fosfato Desidrogenase observada no grupo convencional pode refletir um desequilíbrio redox, com maior produção de fosfato de dinucleotídeo de nicotinamida e adenina e glutationa reduzida, um mecanismo importante da fisiopatologia da insuficiência cardíaca.
OBJECTIVE: Increased glucose 6-phosphate dehydrogenase activity has been demonstrated in heart failure. This study sought to assess myocardial glucose 6-phosphate dehydrogenase activity in retraining of the subpulmonary ventricle of adult goats. METHODS: Eighteen adult goats were divided into three groups: traditional (fixed banding), sham, and intermittent (adjustable banding, daily 12-hour systolic overload). Systolic overload (70% of systemic pressure) was maintained during a 4-week period. Right ventricle, pulmonary artery and aortic pressures were measured throughout the study. All animals were submitted to echocardiographic and hemodynamic evaluations throughout the protocol. After the study period, the animals were killed for morphological and glucose 6-phosphate dehydrogenase activity assessment. RESULTS: A 55.7% and 36.7% increase occurred in the intermittent and traditional right ventricle masses, respectively, when compared with the sham group (P<0.05), despite less exposure of intermittent group to systolic overload. No significant changes were observed in myocardial water content in the 3 groups (P=0.27). A 37.2% increase was found in right ventricle wall thickness of intermittent group, compared to sham and traditional groups (P<0.05). Right ventricle glucose 6-phosphate dehydrogenase activity was elevated in the traditional group, when compared to sham and intermittent groups (P=0.05). CONCLUSION: Both study groups have developed similar right ventricle hypertrophy, regardless less systolic overload exposure of intermittent group. Traditional systolic overload for adult subpulmonary ventricle retraining causes upregulation of myocardial glucose 6-phosphate dehydrogenase activity. It may suggest that the undesirable "pathologic systolic overload" is influenced by activation of penthose pathway and cytosolic Nicotinamide adenine dinucleotide phosphate availability. This altered energy substrate metabolism can elevate levels of free radicals by Nicotinamide adenine dinucleotide phosphate oxidase, an important mechanism in the pathophysiology of heart failure.
Assuntos
Animais , Glucosefosfato Desidrogenase/metabolismo , Hipertrofia Ventricular Direita/enzimologia , Miocárdio/enzimologia , Artéria Pulmonar/cirurgia , Transposição dos Grandes Vasos/cirurgia , Pressão Sanguínea , Biomarcadores/metabolismo , Modelos Animais de Doenças , Metabolismo Energético , Cabras , Hemodinâmica , Ventrículos do Coração/enzimologia , Ventrículos do Coração/fisiopatologia , Hipertrofia Ventricular Direita/fisiopatologia , Fatores de Tempo , Disfunção Ventricular Direita/enzimologia , Disfunção Ventricular Direita/fisiopatologiaRESUMO
A 15-day-old neonate with complete transposition of the great arteries/intact ventricular septum was admitted with life-threatening hypoxemia and heart arrest. After successful resuscitation, heart beat recovered but blood lactate began to arise and maintained above 15 mmol/L 6 hours later. Emergency arterial switch operation was done at 20 hours after resuscitation. Planned extracorporeal membrane oxygenation support was employed postoperatively. The baby experienced severe pulmonary hemorrhage and severe hypoxemia after weaning from cardiopulmonary bypass, which were treated with extracorporeal membrane oxygenation support.
Um recém-nascido de 15 dias de idade, com transposição completa das grandes artérias/septo ventricular intacto, foi internado com hipoxemia com risco de vida e parada cardíaca. Após a reanimação bem sucedida, o coração voltou a bater, mas o lactato elevou-se e manteve-se acima de 15 mmol/L, 6 horas após. Operação de emergência para correção da transposição das grandes artérias foi realizada 20 horas após a ressuscitação. Suporte de oxigenação por membrana extracorpórea foi empregado no pós-operatório. O bebê apresentou hemorragia pulmonar e hipoxemia graves após desconexão da circulação extracorpórea, que foram tratadas com suporte da xigenação por membrana extracorpórea.
Assuntos
Humanos , Recém-Nascido , Masculino , Baixo Débito Cardíaco/terapia , Oxigenação por Membrana Extracorpórea/métodos , Pneumopatias/terapia , Hemorragia Pós-Operatória/terapia , Transposição dos Grandes Vasos/terapia , Septo Interventricular/anatomia & histologia , Baixo Débito Cardíaco/etiologia , Reanimação Cardiopulmonar/métodos , Pneumopatias/etiologia , Hemorragia Pós-Operatória/etiologia , Radiografia Torácica , Fatores de Tempo , Resultado do TratamentoRESUMO
Merced a los continuos progresos en el tratamiento médico y quirúrgico, el número de pacientes adultos portadores de cardiopatías congénitas se encuentra en incremento, quienes con el paso del tiempo desarrollan diversas complicaciones, entre ellas insuficiencia cardíaca avanzada, la cual puede requerir terapéuticas como el trasplante cardíaco y en ocasiones plantea la necesidad del implante de un dispositivo de asistencia circulatoria, ya sea como puente al trasplante o como tratamiento definitivo. En esta presentación se describe un caso que ilustra la problemática de la población portadora de cardiopatías congénitas en el adulto. Se trata de un paciente de sexo masculino, de 41 años, portador de transposición corregida de los grandes vasos que desarrolló insuficiencia cardíaca avanzada del ventrículo morfológicamente derecho, el cual sostiene la circulación sistémica. Ante reiteradas descompensaciones bajo tratamiento médico, incluido el uso domiciliario de inotrópicos, se procedió al implante de un dispositivo de asistencia circulatoria inicialmente planteado como puente al trasplante. Tras diversas complicaciones posoperatorias, el paciente fue derivado a una institución de rehabilitación a la espera del trasplante.
Due to continuous advances in medical and surgical treatment, the large number of adult patients with congenital heart diseases is increasing; with the passing of time, these conditions develop several complications including advanced heart failure, which may require therapeutic approaches such as cardiac transplant and, in certain cases, the implantation of a circulatory support device, both as a bridge to transplant or as a definitive treatment. This report describes a case that shows the problem of the adult population with congenital heart disease. We present a 41 year-old male patient with congenitally corrected transposition of the great vessels who developed advanced heart failure of the morphologically right ventricle, which supports the systemic circulation. Due to several decompensations under medical treatment, including the home use of inotropes, a circulatory support device was implanted as an early bridge to transplant. After several postoperative complications, the patient was transferred to a rehabilitation center to wait for transplantation.
RESUMO
Merced a los continuos progresos en el tratamiento médico y quirúrgico, el número de pacientes adultos portadores de cardiopatías congénitas se encuentra en incremento, quienes con el paso del tiempo desarrollan diversas complicaciones, entre ellas insuficiencia cardíaca avanzada, la cual puede requerir terapéuticas como el trasplante cardíaco y en ocasiones plantea la necesidad del implante de un dispositivo de asistencia circulatoria, ya sea como puente al trasplante o como tratamiento definitivo. En esta presentación se describe un caso que ilustra la problemática de la población portadora de cardiopatías congénitas en el adulto. Se trata de un paciente de sexo masculino, de 41 años, portador de transposición corregida de los grandes vasos que desarrolló insuficiencia cardíaca avanzada del ventrículo morfológicamente derecho, el cual sostiene la circulación sistémica. Ante reiteradas descompensaciones bajo tratamiento médico, incluido el uso domiciliario de inotrópicos, se procedió al implante de un dispositivo de asistencia circulatoria inicialmente planteado como puente al trasplante. Tras diversas complicaciones posoperatorias, el paciente fue derivado a una institución de rehabilitación a la espera del trasplante.(AU)
Due to continuous advances in medical and surgical treatment, the large number of adult patients with congenital heart diseases is increasing; with the passing of time, these conditions develop several complications including advanced heart failure, which may require therapeutic approaches such as cardiac transplant and, in certain cases, the implantation of a circulatory support device, both as a bridge to transplant or as a definitive treatment. This report describes a case that shows the problem of the adult population with congenital heart disease. We present a 41 year-old male patient with congenitally corrected transposition of the great vessels who developed advanced heart failure of the morphologically right ventricle, which supports the systemic circulation. Due to several decompensations under medical treatment, including the home use of inotropes, a circulatory support device was implanted as an early bridge to transplant. After several postoperative complications, the patient was transferred to a rehabilitation center to wait for transplantation.(AU)
RESUMO
D-Transposition of great arteries with an aortopulmonary window is a rare congenital anomaly. We describe a case of D-Transposition of great arteries with an aortopulmonary window and multiple ventricular septal defects in a 5-month boy who underwent successful surgical repair.
A transposição das grandes artérias com uma janela aortopulmonar é uma anomalia congênita rara. Descrevemos um caso de transposição das grandes artérias com janela aortopulmonar e múltiplos defeitos do septo ventricular em um menino de 5 meses submetido a tratamento cirúrgico com sucesso.
Assuntos
Humanos , Lactente , Masculino , Defeito do Septo Aortopulmonar/cirurgia , Comunicação Interventricular/cirurgia , Transposição dos Grandes Vasos/cirurgiaRESUMO
FUNDAMENTO: A transposição corrigida das grandes artérias frequentemente evolui com disfunção ventricular direita. O preparo ventricular para a correção anatômica em pacientes adultos apresenta resultados desapontadores. OBJETIVO: Analisar a hipertrofia do ventrículo direito (VD) induzida por dois tipos de bandagem pulmonar (BP), convencional e intermitente em animais adultos. MÉTODOS: Dezenove cabras adultas foram divididas em três grupos: Convencional (seis animais), Intermitente (seis animais) e Controle (sete animais). O grupo Convencional foi submetido à BP fixa com fita cardíaca, enquanto no grupo Intermitente foi usado dispositivo de BP ajustável, que gerava sobrecarga sistólica por 12 horas, alternada com 12 horas de descanso do VD. As pressões de VD, tronco pulmonar e aorta foram medidas durante todo o estudo. Ecocardiograma foi realizado semanalmente. Após quatro semanas, os animais foram eutanasiados para avaliação morfológica dos ventrículos. O grupo Controle foi submetido a eutanásia para análise em condições basais. RESULTADOS: A sobrecarga pressórica foi menor no grupo Intermitente (p=0,001), comparada ao grupo Convencional. Houve aumento na espessura do VD do grupo Intermitente, medida pelo ecocardiograma, comparado ao seu momento basal (p<0,05). O índice de performance miocárdica do VD foi melhor no grupo Intermitente (p=0,024), comparado ao Convencional. Os grupos estimulados apresentaram aumento da massa muscular em comparação ao grupo Controle (p=0,001). Não houve diferença no conteúdo de água miocárdica. CONCLUSÃO: A BP intermitente desenvolveu hipertrofia de melhor desempenho funcional, sugerindo este protocolo como método preferencial de preparo ventricular.
BACKGROUND: Corrected transposition of great arteries often evolves with right ventricular dysfunction. The ventricular preparation for anatomic correction in adult patients has produced disappointing results. OBJECTIVE: To assess right ventricular hypertrophy (RV) induced by conventional and intermittent pulmonary banding (PB) in adult animals. METHODS: Nineteen adult goats were divided into three groups: conventional (six animals), intermittent (six animals) and control (seven animals). The Conventional group underwent fixed PB with cardiac tape, while the intermittent group received PB adjustable device, which generated systolic overload for 12 hours, alternated with 12 hours of rest of RV. The pressures of the RV, pulmonary artery and aorta were measured throughout the study. Echocardiography was performed weekly. After four weeks, the animals were euthanized for morphological evaluation of the ventricles. The Control group was put to euthanasia for analysis at baseline. RESULTS: Pressure overload was lower in the intermittent group (p = 0.001), compared to the conventional group. There was an increase in the thickness of the RV of the Intermittent group measured by echocardiography compared to their baseline values (p < 0.05). The myocardial performance index in the RV group was better in the Intermittent group (p = 0.024), compared to the Conventional group. The groups stimulated showed increased muscle mass compared to the Control group (p = 0.001). There was no difference in myocardial water content. CONCLUSION: The intermittent BP developed hypertrophy of better performance, suggesting this protocol as the preferred method of ventricular preparation.
FUNDAMENTO: La transposición corregida de las grandes arterias frecuentemente evoluciona con disfunción ventricular derecha. La preparación ventricular para la corrección anatómica en pacientes adultos presenta resultados desalentadores. OBJETIVO: Analizar la hipertrofia del ventrículo derecho (VD) inducida por dos tipos de vendaje pulmonar (VP), convencional e intermitente en animales adultos. MÉTODOS: Diecinueve cabras adultas fueron divididas en tres grupos: Convencional (seis animales), Intermitente (seis animales) y Control (siete animales). El grupo Convencional fue sometido al VP fijo con cinta cardíaca, mientras que en el grupo Intermitente fue usado dispositivo de VP ajustable, que generaba sobrecarga sistólica por 12 horas, alternada con 12 horas de descanso del VD. Las presiones de VD, tronco pulmonar y aorta fueron medidas durante todo el estudio. Ecocardiograma fue realizado semanalmente. Después de cuatro semanas, los animales fueron eutanasiados para evaluación morfológica de los ventrículos. El grupo Control fue sometido a eutanasia para análisis en condiciones basales. RESULTADOS: La sobrecarga presórica fue menor en el grupo Intermitente (p=0,001), comparada al grupo Convencional. Hubo aumento en el espesor del VD del grupo Intermitente, medida por el ecocardiograma, comparado a su momento basal (p<0,05). El índice de performance miocárdica del VD fue mejor en el grupo Intermitente (p=0,024), comparado al Convencional. Los grupos estimulados presentaron aumento de la masa muscular en comparación al grupo Control (p=0,001). No hubo diferencia en el contenido de agua miocárdica. CONCLUSIÓN: EL VP intermitente desarrolló hipertrofia de mejor desempeño funcional, sugiriendo este protocolo como método preferencial de preparación ventricular.