Integrated management during the perinatal period for total anomalous pulmonary venous connection / 中国当代儿科杂志

OBJECTIVES@#To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography.@*METHODS@#Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology.@*RESULTS@#Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis).@*CONCLUSIONS@#TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period.

A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant

Abstract Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.

Successful repair of infantile scimitar syndrome with contralateral pulmonary vein stenosis in an infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Síndrome de cimitarra, valoración con ecocardiografía transesofágica intraoperatoria. Informe de caso / Scimitar syndrome; intraoperative transesophageal echocardiography assessment. Case report

9-year-old patient diagnosed with scimitar syndrome through transthoracic echocardiogram and cardiac catheterization. Surgical correction is performed by anastomosing the collector of the pulmonary right vein to AI through the interatrial septum, tunneling it with a pericardic patch. The study findings and echocardiographic images of such surgical repair and the spectrum of pulsed Doppler differentiating between right and left pulmonary veins will be shown below. The connection through the septum was evidenced, simulating an interatrial communication. A difference was found among the measured points S, D, A and between the left and right pulmonary veins, without this resulting in a stenosis during the repair.

Paciente de 9 años de edad el cual es diagnosticado con síndrome de cimitarra a través de ecografía transtorácica y cateterismo cardiaco. Se realiza cirugía correctora la cual se anastomosa el colector de vena pulmonar derecha hacia AI a través del septum interauricular, tunelizándolo con parche pericárdico. A continuación se muestran los hallazgos e imágenes ecocardiográficas de tal reparación y el espectro doppler pulsado diferenciado entre las venas pulmonares derechas e izquierdas. Se pudo evidenciar la conexión a través del septum, simulando una comunicación interauricular. Se halló una diferencia entre los puntos medidos S, D, A entre las venas pulmonares izquierdas y derechas, sin que esto significara una estenosis en la reparación.

Remodelación de cavidades izquierdas en el posoperatorio de retorno venoso pulmonar anómalo total: utilidad del NTProBNP / Left chambers remodeling after repair of TAPVR connection: usefulness of NTProBNP

Resumen: El retorno venoso pulmonar anómalo total se caracteriza por la falla de conexión entre la aurícula primitiva y el retorno venoso pulmonar, este último se conecta al retorno venoso sistémico a través de la persistencia de conexiones embrionarias. En esta patología, el ventrículo izquierdo suele tener un tamaño en el límite inferior de la normalidad, con una aurícula izquierda pequeña y atrófica. En el período posoperatorio las cavidades izquierdas deben manejar todo el retorno venoso pulmonar, lo que podría determinar sobrecarga de estas cavidades. Presentamos dos casos de posoperatorio de retorno venoso pulmonar anómalo total, evaluando la relación entre el tamaño auricular izquierdo y los niveles de péptido natriurético. Se plantea como hipótesis una disfunción en el llenado de cavidades izquierdas como sustrato causal de este fenómeno, teniendo como consecuencia modificaciones adaptativas anatómicas y funcionales. La determinación de los niveles de péptido natriurético podría ser útil en la monitorización de este proceso adaptativo.

Summary: Total anomalous pulmonary venous return is a congenital heart disease characterized by failure of connection between the primitive left atrium and the pulmonary venous return, the latter drains to the systemic venous return trough persistent embryologic connections. In this pathology there is a normal size, but rather small, left ventricle with a small and undeveloped left atrium. In the postoperative period, the left chambers must handle all the pulmonary venous return, which could mean an increased wall stress. The study presents two cases of Total Anomalous Pulmonary Venous Return, and the behavior of left atrial size and natriuretic peptide level after surgery. We set a hypothesis by which a dysfunction in the filling of the left chambers could explain this phenomenon and how this triggers compensatory modifications. Analyzing the level of natriuretic peptide might help monitor this process.

Cierre percutáneo de conducto arterioso mediante un asa arteriovenosa en un paciente con síndrome de la cimitarra / Percutaneous closure of ductus arteriosus through an arteriovenous loop in a patient with scimitar syndrome

Resumen: Introducción: El síndrome de la cimitarra consiste en una rara malformación, caracterizada por una conexión anómala parcial de una o ambas venas pulmonares derechas a la vena cava inferior, hipoplasia de pulmón derecho y circulación sistémica desde la aorta descendente. El síndrome de la cimitarra en ocasiones se asocia con otras malformaciones congénitas, entre las que se incluye la persistencia del conducto arterioso (PCA). Caso clínico: Paciente de sexo femenino de cuatro años de edad con síndrome de la cimitarra, variedad ''adulto'', asociado con PCA sintomático. Se realizó exitosamente oclusión del conducto mediante un asa arteriovenosa femoral con un dispositivo Amplatzer® PDA, sin complicaciones. Conclusiones: El manejo del síndrome de la cimitarra es complejo y amerita de un estudio hemodinámico completo para determinar el tratamiento adecuado. La hipertensión arterial pulmonar es un factor de mal pronóstico.

Abstract: Background: Scimitar syndrome consists in a rare malformation characterized by a partial abnormal connection in one or both right pulmonary veins to the inferior vena cava, right lung hypoplasia and systemic circulation from the descending aorta. Scimitar syndrome is occasionally associated to other congenital malformations, such as patent ductus arteriosus (PDA). Case report: We report a 4-year-old patient with ''adult'' variety of scimitar syndrome associated to symptomatic PDA, which was successfully occluded using retrograde guidewire-established femoral arteriovenous loop with an Amplatzer® PDA occluder, without complications. Conclusions: Scimitar syndrome is complex and requires a complete hemodynamic study for the determination of the appropriate treatment. Pulmonary arterial hypertension is a factor associated with poor prognosis.

Major intrahepatic veno-venous fistula after fontan operation treated by transcatheter implantation of amplatzer septal occluder through internal jugular vein

Abstract Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay. Three weeks after the first intervention, a second procedure was performed to retrieve the first device and to close the fistula distally. Multiple attempts with different types of gooseneck snares and a bioptome catheter failed to retrieve the first device, so a telescopic method was used to re-screw it. Using a Mullins long sheath and delivery sheath, the delivery cable was manipulated to fit into the slot of the end screw, and the cable was rotated gently in a clockwise direction to re-screw the device. Then, another Amplatzer septal occluder was placed at the distal end of the fistula. In conclusion, distal transcatheter occlusion of intrahepatic veno-venous fistulas might lead to better clinical outcomes in selected patients. Amplatzer septal occluder device can be retrieve without any complication within three weeks.

Post-op systemic hypertensive crises in an infant with total anomalous pulmonary venous connection.

Systemic hypertension is not usually a complication following repair of total anomalous pulmonary venous connection (TAPVC). We report an infant with supracardiac TAPVC with hypertensive crises post-operatively resulting in pulmonary edema. We feel this might have been related to the pre-operative hemodynamics as described. Beta-blockers improved the hypertensive crises.

Successful pulmonary venous channeling in a case of Scimitar syndrome

A young girl child with recurrent pneumonias, failure to thrive and dextroposed heart was diagnosed with and surgically treated for scimitar syndrome. Early diagnosis and treatment of this syndrome prevents serious morbidity

El síndrome de la cimitarra en lactantes y niños / The scimitar syndrome in infants and children

Introducción: El síndrome de la cimitarra (SC) puede presentar un perfil clínico diferente de acuerdo con la edad de presentación. Objetivo: Comparar aspectos clínicos y terapéuticos entre los niños menores y mayores de 1 año. Material y métodos: En el período 1997-2003 se diagnosticaron 14 pacientes consecutivos con SC. Se dividieron en dos grupos: Grupo I: 9 pacientes con edad X: 2 meses (rango 2 días-7 meses) y grupo II: 5 pacientes con edad X: 4,8 años (rango 1 año-12,7 años). Se compararon la presencia de insuficiencia cardíaca (IC), hipertensión pulmonar (HP), la necesidad de embolización de colaterales sistémicas, cirugías y evolución clínica. Resultados: Ingresaron con IC 9 pacientes, todos del grupo I (p = 0,0012). Presentaron HP 7 pacientes. Grupo I: 7/9 y grupo II: 0/5 (p = 0,005).Se realizaron 20 embolizaciones de colaterales sistémicas. Grupo I: 17 procedimientos en 9/9 pacientes, 5 de ellos necesitaron 2 o más embolizaciones por repermeabilización de las colaterales. Grupo II: 3 embolizaciones en 2/5 pacientes (p = 0,008). Fueron operados 11 pacientes. Grupo I: 8/9 y grupo II: 3/5 (p = 0,52). Se reintervino quirúrgicamente a un paciente del grupo I por obstrucción de la tunelización. No hubo mortalidad quirúrgica. Falleció un paciente del grupo I con indicación quirúrgica, al que se le habían realizado 4 embolizaciones de colaterales. Conclusión: Los pacientes menores de 1 año presentaron mayor incidencia de IC, HP y colaterales y requirieron un número mayor de embolizaciones de colaterales. Los pacientes mayores de 1 año presentaron una evolución clínica más benigna.

Uso de eritropoyetina en cirugía cardiovascular pediátrica. Presentación de un caso / Erythropoietin use in pediatric cardiovascular surgery. A case presentation

Se presenta el caso de un niño de 5,500 kg., con diagnóstico de drenaje venoso anómalo pulmonar total en aurícula derecha, al que se le realiza corrección total de la anomalía, mediante cirugía cardiovascular con circulación extracorpórea. Se realiza preparación prequirúrgica con administración de Fe (ev-vo) y EPO (ev-sc) y se utiliza en forma combinada técnicas de hemodilución intraquirúrgica, hipotermia y reinfusión de la sangre remanente en el circuito de CEC. Todo ello, más la flexibilización de los gatillos transfusionales permite realizar la cirugía sin la administración de componentes sanguíneos.

Síndrome de cimitarra asociado a atresia pulmonar con comunicación interventricular: Primer caso reportado / Scimitar syndrome associated to pulmonary atresia with interventricular communication: First reported case

Se presenta el caso de un masculino de 15 meses de edad con síndrome de cimitarra y atresia pulmonar con comunicación interventricular. El diagnóstico se hizo mediante cateterismo cardíaco y angiocardiografía y confirmado por el estudio de autopsia. Se hacen las consideraciones clínicas y quirúrgicas de esta excepcional asociación que a la fecha es, hasta donde sabemos la primera reportada en la literatura.

We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.

Wolff-Parkinson-White syndrome associated to scimitar syndrome. Percutaneous radiofrequency catheter ablation previous to surgical repair

La asociación de los síndromes de Wolff-Parkinson-White y de cimitarra no ha sido reportada en la era de la ablación transcatéter. Presentamos nuestra experiencia en torno a la ablación percutánea con radiofrecuencia, un reto particular ante las modificaciones cardiacas propias del síndrome de cimitarra, y el seguimiento posquirúrgico para esta última.

Síndrome da cimitarra: relato de caso com falsos diagnósticos e conduta adequada / Scimitar syndrome: case report with false diagnoses and adequate procedure

A síndrome da cimitarra, embora rara, é importante de ser conhecida por pediatras e pneumologistas, pois a imagem radiológica, apesar de sugerir o diagnóstico, pode ser mal-interpretada, levando a falsos diagnósticos. A partir da suspeita diagnóstica, a investigação com ecocardiograma e angiografia é fundamental, mesmo no paciente assintomático, pois pode-se evidenciar hipertensão pulmonar importante, de tal forma que o tratamento cirúrgico é indicado para evitar complicações futuras irreversíveis. Criança, apesar das dificuldades diagnósticas iniciais, foi operada precocemente, havendo excelente evolução durante o seguimento ambulatorial

A importância da radiogarafia de tórax no diagnóstico da síndrome da cimitarra / The importance of chest radiograph in the diagnosis of scimitar syndrome

Objetivo: Alertar sobre a importância da radiografia de tórax no diagnóstico da Síndrome da Cimitarra, apresentado como elementos principais a drenagem anômala venosa do pulmäo direito e da artéria pulmonar, anomalias da segmentaçäo brônquica e da vasculatura arterial pulmonar. Métodos: Os autores relatam um caso da Síndrome da Cimitarra em um escolar de 8 anos e discutem os métodos diagnósticos e o tratamento cirúrgico. Resultados: A criança apresentou evoluçäo favorável após abordagem cirúrgica e seguimento sem complicaçöes. Conclusäo: A Síndrome da Cimitarra é uma entidade rara, que pode cursar com manifestaçöes clínicas pouco evidentes. Nesses casos, a radiografia de tórax torna-se um exame de grande relevância no rastreamento dessa anomalia, já que demonstra uma persistente imagem paracardíaca direita, conseqüente à drenagem da veia pulmonar direita na veia cava inferior ("Sinal da Cimitarra").