Perinatal multidisciplinary diagnosis and treatment of fetal D-transposition of great arteries: analysis of 37 cases / 中华围产医学杂志

Objective:To review the clinical outcomes following perinatal multidisciplinary diagnosis and treatment of fetal D-transposition of great arteries (D-TGA).Methods:This retrospective analysis involved 37 fetuses (two fetuses were one of the twins) with D-TGA that were diagnosed by prenatal ultrasound at the Women and Children's Hospital, Qingdao University from January 2016 to December 2020. All the subjects received perinatal multidisciplinary diagnosis and treatment, from the Departments of Fetal Medicine, Genetics, Obstetrics, Ultrasonography, Pediatric Cardiology, Neonatology, etc., and the outcomes were described and summarized.Results:The detection rate of D-TGA was 0.059% (37/62 413), among which intact ventricular septum with D-TGA accounted for 56.8% (21/37) and ventricular septal defect with D-TGA for 43.2% (16/37). All the 37 cases were observed with normal nuchal translucency and four of them were at high risk in fetal Down syndrome screening. All the 31 cases who received non-invasive cell-free fetal DNA screening had normal results and two of 26 cases who received amniocentesis for karyotype analysis and chromosome microarray analysis were abnormal. In terms of pregnancy outcome, 19 pregnancies (51.4%) were terminated, of which 10 cases were terminated for medical reasons and others for non-medical reasons, and 18 cases gave birth to alive body (48.6%, 18/37). Postnatal ultrasound re-examination of one neonate revealed D-TGA with ventricular septal defect, patent ductus arteriosus, and bicuspid pulmonary valve malformation and severe hypoxia and acidosis occured. The patient was discharged after withdrawing treatment and was lost to follow-up. The other 17 neonates all underwent successful surgical treatment with a mean age of (10.2±6.0) d and length of hospital stay of (26.3±9.3) d. Postoperative follow-up (3.3±1.2) years showed all with good cardiac function.Conclusion:Perinatal multidisciplinary diagnosis and treatment of D-TGA can improve the success rate of postnatal treatment and prognosis.

State-of-the-art pediatric coronary artery bypass surgery: a literature review

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.

Transposición congénitamente corregida de grandes arterias en adultos asintomáticos diagnosticada circunstancialmente por ecocardiografía: casos clínicos / Asymptomatic congenitally corrected transposition of the great arteries: report of two cases

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.

Modified closed coronary transfer is a good alternative to the trap-door method during arterial switch operation: a retrospective propensity-matched comparison

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.

Malformações congênitas: um relato de reabilitação fisioterapêutica em paciente com transposição das grandes artérias / Congenital abnormalities: a physical therapy rehabilitation report in patient with transposition of great vessels

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

Mid- and Longterm Neo-Aortic Valve Regurgitation after Jatene Surgery: Prevalence and Risk Factors / Regurgitação da Valva Neo-Aórtica a Médio e Longo Prazo após Cirurgia de Jatene: Prevalência e Fatores de Risco

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).

Éxitus materno tras cesárea en gestante con anomalía de Taussig-Bing y síndrome de Eisenmenger / Maternal Exitus after Cesarean Section in Pregnant Women with Taussig-Bing Anomaly and Eisenmenger Syndrome

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)

Sedación para recambio valvular transcatéter de una válvula pulmonar melody®: informe de caso / Sedation for transcatheter replacement of Melody® pulmonary valve: Case report

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.

Echocardiography in diagnosis of complete transposition of great arteries in fetus / 中国医学影像技术

Objective To investigate the echocardiographic features of complete transposition of the great arteries (TGA)in fetuses.Methods Prenatal echocardiographic data of 9 fetuses diagnosed as TGA by autopsy or postnatal echocardiography during January 2010 to January 2017 were retrospectively analyzed.Results All of 9 fetuses showed normal cardiac axis and atrioventricular connection on four-chamber view.Eight of them showed the baby bird's beak sign on left ventricular outflow tract view.On left and right ventricular outflow tracts view,the two great arteries were parallel in 8 fetuses.Totally 6 fetuses showed just 2 vessels on three vessels and tracheal (3VT) view.On aortic arch view,the radian of aortic arch had increased in different degrees in 7 fetuses.There were 4 fetuses with ventricular septal defect observed by both of four-chamber and left ventricular outflow tract views.Conclusion The echocardiographic features of fetuses with TGA are characteristic in left ventricular outflow tract,left and right ventricular outflow tracts,3VT and aortic arch views,including baby bird's beak sign,2 great arteries' parallel relations,only 2 vessels on 3VT view,and increased radian of aortic arch.Of these features,baby bird's beak sign is the most common.

Efficacy and prognostic factors of arterial switch operation in patients with complete transposition of great arteries / 中国医师杂志

Objective To investigate the clinical efficacy and influencing factors of arterial switch operation (ASO) in the treatment of complete transposition of great arteries (TGA).Methods Totally 156 children with TGA who underwent ASO surgery from January 2005 to December 2011 were selected as the subjects.The clinical curative effect and prognosis of all patients were observed,and the relationship be tween clinical features and prognosis was analyzed.Results Totally 156 cases of TGA children were successfully completed the operation,and 29 patients died during the 5 year follow-up period,the mortality rate was 18.59％.Univariate analysis showed that the death in children with TGA after ASO was closely related to coronary artery abnormality,cardiopulmonary bypass time,aortic occlusion time,postoperative low cardiac output syndrome and reoperation (P ＜ 0.05).Multivariate logistic regression analysis showed that coronary artery abnormalities,and low cardiac output syndrome were independent outcome factors leading to postoperative death in patients.Conclusions The postoperative death of ASO in TGA children is closely related to the incidence of coronary artery abnormalities,the time of cardiopulmonary bypass,the time of oc clusion of aorta and the occurrence of postoperative low cardiac output syndrome,which should be pay attention to and take relevant measures.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique

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.

Long-Term Follow-Up of the Half-Turned Truncal Switch Operation for Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis

The half-turned truncal switch (HTTS) operation has been reported as an alternative to the Rastelli or réparation à l'étage ventriculaire procedures. HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS. The advantages of the HTTS procedure are avoidance of late LVOT or right ventricular outflow tract (RVOT) obstruction, and of overstretching of the pulmonary artery. We report the case of a patient who underwent HTTS for TGA with VSD and PS, in whom there was no LVOT obstruction and only mild aortic regurgitation and mild RVOT obstruction, including observations at 12-year follow-up. Our experience with long-term follow-up of HTTS supports a solution for late complications after the Rastelli procedure.

Pulmonary Root Translocation with the Lecompte Maneuver: For Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis

A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.

Bandagem ajustável do tronco pulmonar: IX: atividade da G6PD do miocárdio de cabras adultas submetido ao treinamento ventricular / Reversible pulmonary trunk banding: IX. G6PD activity of adult goat myocardium submitted to ventricular retraining

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.

Extracorporeal membrane oxygenation as a support for TGA/IVS with low cardiac output syndrome and pulmonary hemorrhage / Oxigenação extracorpórea por membrana como suporte para TGA/IVS com síndrome de baixo débito e hemorragia pulmonar

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.

Asistencia ventricular sistémica con dispositivo HeartMate 2como puente al trasplante en la transposición corregida de los grandes vasos / Systemic Ventricular Assistance Using a HeartMate 2 Device as Bridge to Transplant in a Congenitally Corrected Transposition of the Great Vessels / Systemic ventricular assistance using a HeartMate 2 device as bridge to transplant in a congenitally corrected transposition of the great vessels

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.

The diagnostic-treatment-repair strategy in the TGA with ventricular septal defect and severe pulmonary hypertension more than 6 months / 中华胸心血管外科杂志

Objective To analyze and summarize the applicative experience and operative effective of the diaguostictreatment-repair strategy in the transpossion of great arteries(TGA) infants with ventricular septal defect and severe pulmonary hypertension more than 6 months.Methods From January 2010 to October 2011,17 TGA cases with ventricular septal defect and severe pulmonary hypertension.There were 13 male and 4 female.≥0.5-＜ 1.0 years old 6 cases,≥ 1.0-＜ 3.0 years old 3,≥3.0 years old 8 cases.Combine anomalies: patent ductus arteriosus in 6 cases,atrial septal defect in 5 cases,valve insufficency in 2 cases.All preoperative cases were performed echocardiography,right-sided heart catheterization 3 cases,coronary CT examinationll cases.After diagnostic-treatment 2-4 weeks,all cases performed arterial switch operation under compound intravenous and inhaled anesthesia.Results No operative death.After diagnotic-treatment,SPO2 improved 10％-21％,and mPAP decreased 10-20 mm Hg.Follow-up 11.2 (6,20) months,one dead.Postoperative residual pulmonary arterial hypertension in 35.29％,6/17cases,all of them were ≥3 years old.Continue to pulmonary arterial hypertension targeted drugs treatment for 6-20 months later,pulmonary artery pressure decreased obviously.Conclusion The TGA infants with ventricular septal defect pulmonary arterial hypertension more than 6 months,can be selectively performed arterial switch operation under went diagnostic-treatment-repair strategy,continue to pulmonary arterial hypertension targeted drug therapy postoperation,the effect is good.

Imaging of superior sinus venosus atrial septal defect by multi-slice CT / 中华放射学杂志

Objective To evaluate the effectiveness of MSCT in the diagnosis of superior sinus venosus atrial septal defect.Methods The MSCT features of superior sinus venosus atrial septal defect in twenty cases were evaluated retrospectively.The following data were recorded:the size and location of sinus venosus atrial septal defect,the anatomy of pulmonary vein,including number of anomalously draining pulmonary veins and their site of drainage,and associated anomalies.Results In all patients,the superior sinus venosus atrial septal defect locates in the extraseptal wall,which normally separates the right upper pulmonary vein from superior vena cava(SVC).And anomalous connection of right upper pulmonary venous and SVC was identified in all the patients.The mean value of the defect diameter was ( 17.1±5.8) mm.Left superior vena cava was identified in 3 patients.In an elderly patient,left anterior descending branch of coronary artery presented significant stenosis.And in another elderly patient with large atrial septal defect,severe pulmonary hypertension was identified by cardiac catheterization.MSCT findings of superior sinus venosus atrial septal defect in 6 cases were finally confirmed by surgical operation.Conclusions Contrastenhanced MSCT was a useful technique for the diagnosis of superior sinus venosus atrial septal defect,which accurately displayed the anatomical characteristics of the associated malformations for preoperative evaluation.

Congenitally Corrected Transposition of the Great Arteries / 대한내과학회지

No abstract available.