Clinical characteristics of cardiac defects fetuses and the impact of multi-disciplinary team cooperation approach on the pregnancy decision making / 中华妇产科杂志

Objective: To analysis the clinical characteristics of 400 fetuses with heart defects and the impactors of pregnancy decision making, and explore the influence of a multi-disciplinary team (MDT) cooperation approach on it. Methods: Clinical data of 400 fetuses with abnormal cardiac structure diagnosed at Peking University First Hospital from January 2012 to June 2021 were collected, which were divided into 4 groups according to the characteristics of fetal heart defects and the presence of extracardiac abnormalities or not: single cardiac defects without extracardiac abnormalities (122 cases), multiple cardiac defects without extracardiac abnormalities (100 cases), single cardiac defects with extracardiac abnormalities (115 cases), and multiple cardiac defects with extracardiac abnormalities (63 cases). The types of fetal cardiac structural abnormalities and genetic test results, and the detection rate of pathogenic genetic abnormalities, MDT consultation and management situation, and pregnancy decision of fetuses in each group were retrospectively analyzed. A logistics regression was used to analyze the influencing factors of fetal heart defects pregnancy decision. Results: (1) Among the 400 fetal heart defects, the four most common major types were ventricular septal defect 96 (24.0%, 96/400), tetralogy of Fallot 52 (13.0%, 52/400), coarctation of the aorta 34 (8.5%, 34/400), and atrioventricular septal defect 26 (6.5%, 26/400). (2) Among the 204 fetuses undergoing genetic examination, 44 (21.6%, 44/204) pathogenic genetic abnormalities were detected. (3) Detection rate of pathogenic genetic abnormalities (39.3%, 24/61) and pregnancy termination rate (86.1%, 99/115) in the single cardiac defects with extracardiac abnormalities group were significantly higher than those in the single cardiac defects without extracardiac abnormalities group [15.1% (8/53), 44.3% (54/122), respectively] and the multiple cardiac defects without extracardiac abnormalities group [6.1% (3/49), 70.0% (70/100), respectively, both P<0.05], and the pregnancy termination rate in the multiple cardiac defects without extracardiac abnormalities group and the multiple cardiac defects with extracardiac abnormalities group (82.5%,52/63) were significantly higher than that of the single cardiac abnormalities without extracardiac abnormalities group (both P<0.05). (4) After adjusting for age, gravity, parity and performed prenatal diagnosis, maternal age, the diagnosis of gestational age, prognosis grades, co-existence of extracardiac abnormalities, presence of pathogenic genetic abnormalities, and receiving MDT consultation and management were still independent influencing factors of termination of pregnancy of fetuses with cardiac defects (all P<0.05). A total of 29 (7.2%, 29/400) fetal cardiac defects received MDT consultation and management, and compared with those without MDT management, the pregnancy termination rate in the multiple cardiac defects without extracardiac abnormalities group [74.2%(66/89) vs 4/11] and the multiple cardiac defects with extracardiac abnormalities group [87.9%(51/58) vs 1/5] were lower, the differences were statistically significant respectively (all P<0.05). Conclusions: Maternal age, diagnosed gestational age, severity of cardiac defects, extracardiac abnormalities, pathogenic genetic abnormalities and MDT counseling and management are the influencing factors of fetal heart defects pregnancy decision. MDT cooperation approach influences pregnancy decision-making and should be recommended for the management of fetal cardiac defect to reduce unnecessary termination of pregnancy and improve pregnancy outcomes.

Diagnóstico antenatal de cardiopatías congénitas por telemedicina: experiencia en CERPO, 2017-2022 / Antenatal diagnosis of congenital heart disease by telemedicine: experience in CERPO, 2017-2022

Introducción: Las cardiopatías congénitas son las anomalías más frecuentes y la principal causa de muerte infantil y neonatal. El diagnóstico prenatal mejora el resultado perinatal determinando el lugar de nacimiento y el nivel de cuidado neonata. La telemedicina mediante videoconferencia en tiempo real permite mejorar la precisión diagnóstica y planificar el nacimiento. Objetivo: Determinar el diagnóstico y manejo perinatal de fetos con sospecha de cardiopatía congénitas, evaluadas a través de telemedicina en tiempo real atendidas en CERPO en el periodo 2017-2022. Material y métodos: Estudio retrospectivo de las evaluaciones mediante telemedicina en tiempo real realizadas en CERPO entre los años 2017 a 2022. Se revisó el resultado perinatal y se compararon los diagnósticos pre y postnatales, extraídos de la base de datos CERPO y Unidad de Neonatología del Hospital Luis Tisné Brousse. Resultados: La correlación del diagnóstico de cardiopatía congénita mediante telemedicina es de un 81,8% y de 89,8% con el diagnostico posnatal. Conclusiones: La evaluación por medio de telemedicina permite mejorar la precisión diagnostica de la cardiopatía congénita en áreas con escaso acceso a operadores experimentados en evaluación cardiaca fetal. Esto minimiza el impacto económico y social asociado al manejo perinatal de un feto con cardiopatía congénita en nuestro país.

Introduction: Congenital heart disease is the most common anomaly and the leading cause of infant and neonatal death. Prenatal diagnosis improves perinatal outcomes by choosing the right place of birth and level of neonatal care. Telemedicine by videoconferencing in real-time allows for improved diagnostic accuracy and birth planning. Objective: To determine the diagnosis and perinatal management of fetuses with suspected congenital heart disease, evaluated by telemedicine at CERPO in the period 2017-2022. Material and Methods: Retrospective study of evaluations via real-time videoconferencing performed at CERPO between 2017-2022. The perinatal outcome was reviewed, and pre and postnatal diagnoses were compared. The data was extracted from the CERPO database and the Neonatology Unit of the Luis Tisné Brousse Hospital. Results: The correlation of congenital heart disease diagnosis by telemedicine was 81.8% and 89.8% with postnatal diagnosis. Conclusions: Telemedicine assessment improves the diagnostic accuracy of congenital heart disease in areas with poor access to an experienced fetal cardiac specialist. This minimizes the economic and social impact associated with our countrys perinatal management of a fetus with congenital heart disease.

Effect and influence factors of cardiopulmonary resuscitation in children with congenital heart disease in pediatric intensive care unit / 中华儿科杂志

Objective: To investigate the prognostic factors of children with congenital heart disease (CHD) who had undergone cardiopulmonary resuscitation (CPR) in pediatric intensive care unit (PICU) in China. Methods: From November 2017 to October 2018, this retrospective multi-center study was conducted in 11 hospitals in China. It contained data from 281 cases who had undergone CPR and all of the subjects were divided into CHD group and non-CHD group. The general condition, duration of CPR, epinephrine doses during resuscitation, recovery of spontaneous circulation (ROSC), discharge survival rate and pediatric cerebral performance category in viable children at discharge were compared. According to whether malignant arrhythmia is the direct cause of cardiopulmonary arrest or not, children in CHD and non-CHD groups were divided into 2 subgroups: arrhythmia and non-arrhythmia, and the ROSC and survival rate to discharge were compared. Data in both groups were analyzed by t-test, chi-square analysis or ANOVA, and logistic regression were used to analyze the prognostic factors for ROSC and survival to discharge after cardiac arrest (CA). Results: The incidence of CA in PICU was 3.2% (372/11 588), and the implementation rate of CPR was 75.5% (281/372). There were 144 males and 137 females with median age of 32.8 (5.6, 42.7) months in all 281 CPA cases who received CPR. CHD group had 56 cases while non-CHD had 225 cases, with the percentage of 19.9% (56/281) and 80.1% (225/281) respectively. The proportion of female in CHD group was 60.7% (34/56) which was higher than that in non-CHD group (45.8%, 103/225) (χ2=4.00, P=0.045). There were no differences in ROSC and rate of survival to discharge between the two groups (P>0.05). The ROSC rate of children with arthythmid in CHD group was 70.0% (28/40), higher than 6/16 for non-arrhythmic children (χ2=5.06, P=0.024). At discharge, the pediatric cerebral performance category scores (1-3 scores) of CHD and non-CHD child were 50.9% (26/51) and 44.9% (92/205) respectively. Logistic regression analysis indicated that the independent prognostic factors of ROSC and survival to discharge in children with CHD were CPR duration (odds ratio (OR)=0.95, 0.97; 95%CI: 0.92~0.97, 0.95~0.99; both P<0.05) and epinephrine dosage (OR=0.87 and 0.79, 95%CI: 0.76-1.00 and 0.69-0.89, respectively; both P<0.05). Conclusions: There is no difference between CHD and non-CHD children in ROSC and survival rate of survival to discharge was low. The epinephrine dosage and the duration of CPR are related to the ROSC and survival to discharge of children with CHD.

Treatment and prognosis of 826 infants with critical congenital heart disease: a single center retrospective study / 中华心血管病杂志

Objective: To analyze the current status of clinical treatment and factors influencing postoperative mortality in infants with critical congenital heart disease (CCHD) in China, optimize the perioperative management of CCHD, and provide a new scientific basis for clinical decision-making for the optimal management of these patients. Methods: This is a retrospective single-center study. Infants diagnosed with CCHD in Guangdong Provincial People's Hospital from January 2017 to December 2019 (aged 0-1 years at admission) were enrolled. General clinical information, inpatient treatment information, prognosis and complications were collected and analyzed. Multivariate logistic regression analysis was used to explore the independent risk factors of postoperative death in infants with CCHD. Results: A total of 826 infants with CCHD were included, including 556 males (67.3%) and the age at first admission was 51.0 (5.0,178.3) days. 264 (32.0%) cases were tetralogy of Fallot and 137 (16.6%) cases were total anomalous pulmonary venous return. 195 cases (23.6%) were diagnosed prenatally. 196 cases (23.7%) were treated with prostaglandin. The preoperative invasive ventilation time was 0 (0, 0) hour, and the postoperative invasive ventilation time was 95.0 (26.0, 151.8) hours. A total of 668 cases (80.9%) underwent surgical treatment. The age was 100.5 (20.0, 218.0) days during operation and the operation time was 190.0 (155.0, 240.0) hours. Sixty-two cases (7.5%) received medical treatment, and 96 cases (11.6%) gave up treatment. A total of 675 cases (81.7%) were discharged with improvement, 96 cases (11.6%) were discharged after giving up treatment, 55 cases (6.7%) died and 109 cases (13.2%) were readmitted within one year. Complications occurred in 565 (68.6%) cases, including pneumonia in 334 cases (40.4%) and cardiac arrhythmias in 182 cases (22.0%). Multifactorial analysis showed that delayed chest closure (OR=49.775, 95%CI 3.291-752.922, P=0.005), prolonged post-operative invasive ventilator ventilation (OR=1.003, 95%CI 1.000-1.005, P=0.038) and cardiac hypoplasia syndrome (OR=272.658, 95%CI 37.861-1 963.589, P<0.001) were the independent risk factors for mortality in CCHD infants post-operation. Conclusions: Tetralogy of Fallot and total anomalous pulmonary venous return account for the majority of infants with CCHD. The proportion of infants diagnosed prenatally was less than 1/4. The majority CCHD infants received surgical treatment. The main complications are pneumonia and arrhythmia. Delayed chest closure, prolonged postoperative invasive ventilator ventilation and low cardiac output syndrome are the independent risk factors for postoperative death in infants with CCHD.

Inequidad de la atención de las cardiopatías congénitas en los hospitales públicos de México. El falso derecho a la salud / Inequity of congenital heart disease care in the public hospitals of Mexico. The false right to health

Resumen La cardiología pediátrica y la cirugía cardiovascular han tenido avances importantes en los últimos años; las cardiopatías congénitas (CC) son una de las principales causas de mortalidad en niños. Muchos de los factores que determinan la evolución final de estos pacientes incluyen el tipo de cardiopatía y el tiempo del diagnóstico y tratamiento; infortunadamente, dos de los que presentan mayores efectos son el estado socioeconómico y el área geográfica de atención en México. El objetivo de este estudio es conocer el tipo de atención para los pacientes con CC en hospitales públicos del país.

Abstract Pediatric cardiology and cardiovascular surgery have made significant advances in recent years, congenital heart diseases (CHD) are one of the leading causes of mortality in children. Many of the factors that determine the final evolution of these patients include the type of heart disease, the time of diagnosis and treatment; unfortunately, in our country, two of those greatest impact area the socioeconomic status and the geographic area of attention. The objective of this study is to know the type of care for patients with CHD in public hospitals in the country.

Adolescentes y jóvenes portadores de cardiopatías congénitas en etapa de transferencia a la atención médica de adultos / Adolescents and young adults with congenital heart defects in transfer to adult care

Resumen: Introducción: Las cardiopatías congénitas (CC), la malformación congénita más frecuente, han experimentado un aumento de sobrevida y crecimiento exponencial de la población de adolescentes y adultos portado res de CC. Para el éxito a largo plazo urgen intervenciones que optimicen la transición de cuidados de salud desde los servicios pediátricos a los de adulto. Objetivo: Describir el conocimiento y manejo de la enfermedad, autoeficacia y calidad de vida en adolescentes y jóvenes con CC en periodo de transfe rencia en dos hospitales en Santiago de Chile. Pacientes y Método: Estudio no experimental, descrip tivo, de corte transversal. Se aplicó: a) Encuesta de datos sociodemográficos, conocimiento y manejo de su enfermedad y uso de servicios de salud; b) Escala Con-Qol de Calidad de Vida Relacionada con Salud (CVRS) en pacientes con CC y c) Escala de Autoeficacia Generalizada (EAG). Resultados: Se obtuvo una muestra de 51 pacientes, 53% hombres, edad promedio de 17 ± 2,49 años. El 22% de las CC fue de complejidad simple, 29% moderada y 49% alta. Presentaron alta autoeficacia y buenos niveles de calidad de vida, sin embargo, mostraron escaso conocimiento y manejo de su enfermedad cardiaca. Conclusiones: Destaca la poca preparación para lograr una transición exitosa a servicios de adultos y jóvenes portadores de CC, siendo fundamental implementar programas de transición centrados en educación, autocuidado y automanejo de la enfermedad.

Abstract: Introduction: Congenital heart defects (CHD), the most frequent congenital malformations, have shown an in creased survival and exponential growth of the adolescent and adult population living with CHD. Interventions that optimize the transition of patients from pediatric to adult health care services are essential for ensuring positive long-term outcomes. Objective: To describe the knowledge and management of this disease, self-efficacy, and quality of life of young people with CHD during the transition period in two hospitals in Santiago, Chile. Patients and Method: Non-experimental, des criptive, cross-sectional study. Patients completed: a) a survey of socio-demographic data, knowledge and management of their condition, and use of health services; b) the Health-Related Quality of Life (Con-HRQoL) Scale in patients with CHD; and c) the Generalized Self-Efficacy (GSE) Scale. Re sults: We obtained a sample of 51 patients, 53% of them were men, and the mean age was 17 ± 2.49 years. The complexity of the CHD was mild in 22%, moderate in 29%, and high in 49%. Although patients reported high self-efficacy and good levels of quality of life, there was a lack of knowledge and self-management of their heart disease. Conclusions: The study showed that adolescents and young people with CHD are not prepared to achieve a successful transition to adult health care services, and there is a need for the implementation of transition programs focused on education, self-care, and self-management of the disease.

Current situation of congenital heart diseases in two public hospitals in the state of Jalisco / Situación real de las cardiopatías congénitas en dos hospitales públicos del estado de Jalisco

Abstract Introduction: Congenital heart disease represents a public health issue worldwide. Objective: To know the number of patients with heart disease treated in two public hospitals of the State of Jalisco, as well as the mortality and resources available to participating hospitals for the care of these patients in the period from 2015 to 2018; the information was requested to the National Transparency Platform, and the database of pediatric cardiology services and pediatric cardiovascular surgery of the participating hospitals were also reviewed. Results: The second level hospital has human resources, but not the material to attend to these patients; so it is not possible to offer any type of palliative or corrective treatment. A total of 624 patients were evaluated, of which 92.2% corresponded to non-critical heart disease; overall mortality was 12% but in critical heart disease it was 79.5%. The third level hospital has human and material resources to care for these patients. During the study period, 289 operations were performed and the overall mortality was 20.4%. Conclusion: Congenital heart disease in the State of Jalisco is an important cause of mortality, with a high incidence and a very limited resolution capacity since the health services in the State of Jalisco for the care of these patients are insufficient and inadequate. It is essential to strengthen the health system for the care for these patients.

Resumen Introducción: Las cardiopatías congénitas representan un problema de salud pública a nivel mundial. Objetivo: Conocer la cantidad de pacientes cardiópatas atendidos en dos hospitales públicos del estado de Jalisco, así como la mortalidad y los recursos con que cuentan los hospitales participantes para la atención de estos pacientes en el período del 2015 al 2018. Se solicitó la información a la Plataforma Nacional de Transparencia y además se revisaron las bases de datos de los servicios de cardiología pediátrica y cirugía cardiovascular pediátrica de los hospitales participantes. Resultados: El hospital de segundo nivel cuenta con los recursos humanos, pero no con el material para atender a estos pacientes, por lo que no es posible ofrecer ningún tipo de tratamiento paliativo o correctivo (sólo se cierran algunos conductos arteriosos en la etapa neonatal). Se valoró a un total de 624 pacientes, de los cuales el 92.2% correspondió a cardiopatías no críticas; la mortalidad global fue del 12% pero en las cardiopatías críticas fue del 79.5%. El hospital de tercer nivel cuenta con recursos humanos y material para atender a estos pacientes; en el período de estudio se realizaron 289 operaciones y la mortalidad global fue del 20.4%. Conclusión: Las cardiopatías congénitas en el estado de Jalisco son una causa importante de mortalidad, con una incidencia elevada y una capacidad de resolución sumamente limitada, ya que los servicios de salud de Jalisco para la atención de estos pacientes son insuficientes e inadecuados. Es esencial fortalecer el sistema de salud para atender a estos pacientes.

Síndrome de Edwards con cardiopatía congénita de larga supervivencia: reporte de caso y revisión de literatura / Prolonged survival in Edwards syndrome with congenital heart disease: a case report and literature review

El síndrome de Edwards o trisomía 18 es una entidad compleja, con afectaciones en los sistemas musculoesquelético, craneofacial, cardiovascular y neurológico. Su genética es variada, presentándose tanto de manera completa como en mosaicismo. Es infrecuente que la supervivencia supere el primer año de vida. Su caracterización fenotípica no es patognomónica, por lo cual el cariotipo es fundamental para el diagnóstico prenatal por medio de amniocentesis y cordocentesis mediante técnica de hibridación fluorescente in situ. Se presenta el caso de una paciente de ocho años que ha sobrevivido con esta condición, a pesar de presentar tetralogía de Fallot acompañada de malformaciones cardíacas graves. El diagnóstico comenzó por ecografía de tamizaje prenatal a las 16 semanas y ecografía de detalle, con amniocentesis y cariotipo de líquido amniótico, con resultado 47 XX+18. Ha sido tratada por múltiples especialidades médicas, debido a complicaciones osteomusculares, articulares, neurológicas, metabólicas y cardiovasculares que han limitado su calidad de vida. El manejo de estos pacientes requiere un equipo médico multidisciplinario. La consejería a los padres debe incluir aspectos relativos a la sobrevida, complicaciones frecuentes y riesgo-beneficio a evaluar antes de someter al menor a intervenciones quirúrgicas complejas o correctivas.

Edwards syndrome or trisomy 18 is a complex entity that involves the musculoskeletal, craniofacial, cardiovascular, and neurological systems. Its genetics are varied, presenting both in a complete and mosaic type. Survival rarely exceeds the first year of life. Its phenotype characterization is not pathognomonic, so karyotype is essential for diagnosis, prenatally by amniocentesis and cordocentesis by FISH technique. We present the case of an eight-year-old girl who has survived with this condition despite presenting tetralogy of Fallot and serious cardiac malformations. Diagnosis began with prenatal screening ultrasound at 16 weeks and detailed ultrasound, with amniocentesis and amniotic fluid karyotype, with a result of 47 XX+18. She has been treated by multiple medical specialties, due to musculoskeletal, joint, neurological, metabolic, and cardiovascular complications that have limited her quality of life. The management of these patients requires a multidisciplinary medical team, and counseling for parents should include aspects related to survival, frequent complications, and risk-benefit to be evaluated before subjecting the minor to complex or corrective surgical interventions.

Factores asociados a la supervivencia al año de vida en neonatos con cardiopatía congénita severa en un Hospital Nacional de Perú / Factors associated with survival at one year of life in neonates with severe congenital cardiopathy in A National Hospital in Peru

RESUMEN Objetivos. Determinar los factores asociados a la supervivencia en el primer año de vida en neonatos con cardiopatía congénita severa atendidos en un hospital nacional de Perú. Materiales y métodos. Se estudiaron 160 niños nacidos entre el 2012 y 2015 con diagnóstico de alguna cardiopatía congénita severa que ingresaron al Servicio de Neonatología del Hospital Nacional Edgardo Rebagliati Martins del Seguro Social del Perú. En el análisis de supervivencia se utilizó el método de Kaplan-Meier y la prueba Log-Rank. Se realizaron análisis crudos y ajustados mediante modelos de regresión de Cox. Resultados. El 52,5% de los pacientes fueron de sexo masculino y la cardiopatía congénita severa más frecuente fue la atresia pulmonar (26,3%). El 33,7% de los pacientes fallecieron, siendo la supervivencia al año del 66,3% (IC95% 58,4-73,0). El diagnóstico prenatal mejoró la supervivencia (HRa 0,54, IC95%: 0,30-0,98) mientras que las cardiopatías de tipo cianóticas (HRa 2,93, IC95%: 1,36-6,34) y la presencia de otra anomalía congénita (HRa 3,28, IC95%: 1,79-6,01) la disminuyeron, estos factores fueron también significativos en un segundo modelo estratificado por tratamiento quirúrgico con excepción del modelo estratificado por complicaciones donde un diagnóstico prenatal dejó de ser significativo. Conclusiones. El diagnóstico prenatal incrementa la supervivencia ante una cardiopatía congénita severa y permitiría un tratamiento quirúrgico oportuno; sin embargo, se debe considerar que las cardiopatías de tipo cianóticas y la presencia de otras anomalías congénitas extracardíacas disminuyen la supervivencia si se realiza una intervención quirúrgica o se presentan complicaciones.

ABSTRACT Objectives. To determine factors associated with survival in the first year of life in neonates with severe congenital heart disease treated in a national hospital in Peru. Materials and Methods. 160 children born between 2012 and 2015 with a diagnosis of severe congenital cardiopathy were studied and admitted to the Neonatology Service of the Edgardo Rebagliati Martins National Hospital of the Peruvian Social Security. The Kaplan-Meier method and the Log-Rank test were used in the survival analysis. Crude and adjusted analyses were performed using Cox regression models. Results. Fifty-two, point 5 percent (52.5%) of patients were male and the most frequent severe congenital cardiopathy was pulmonary atresia (26.3%). Thirty-three, point seven percent (33.7%) of patients died, with a 66.3% (IC95% 58.4-73.0) one-year survival. Prenatal diagnosis improved survival (HRa 0.54, 95% CI 0.30-0.98) while cyanotic cardiopathies (HRa 2.93, 95% CI 1.36-6.34) and the presence of another congenital anomaly (HRa 3.28, 95% CI 1.79-6.01) decreased it; these factors were also significant in a second model stratified by surgical treatment with the exception of the stratified model by complications where a prenatal diagnosis ceased to be significant. Conclusions. Prenatal diagnosis increases survival from severe congenital heart disease. However, cyanotic heart diseases and other congenital anomalies, which decrease this chance, should be considered, if surgery is performed or complications occur.

El adolescente con cardiopatía congénita: transición y transferencia / The adolescent with congenital heart defects: transition and transfer

Introducción: Los grandes avances en el diagnóstico y tratamiento de los pacientes con cardiopatías congénitas en las últimas décadas han permitido que mas del 90% lleguen a la adolescencia y edad adulta. Sin embargo, muchos de ellos requirieran seguimiento e intervenciones de por vida, por lo que necesitaran ser transferidos desde el hospital pediátrico al de adultos. Material y Métodos: Se incluyeron los pacientes mayores de 15 años que consultaron en el área ambulatoria del servicio de cardiología del Hospital Garrahan durante el periodo agosto 2017 - agosto 2018. Las variables analizadas fueron: procedencia, cardiopatía de base y variedad pronostica ,procedimientos intervencionistas factores asociados como síndrome genéticos y otras comorbilidades, cobertura social, nivel educativo, terapéutica medicamentosa, clase funcional, embarazos, prevalencia de cardiopatías en la descendencia y transición-transferencia al hospital de adultos. Resultados: Registramos 704 consultas de 309 pacientes con una edad media de 19,17 años (DS +- 4,62; (rango 15- 49,4 años). Fueron 112 mujeres y 197 varones. El 51,1 % provenían de Buenos Aires,40 % de las provincias del interior y 8,1% CABA. El 92% de los pacientes tenía cardiopatías de moderada y severa complejidad, y el 93,5% eran operadas. El 13,2 % eran síndromes genéticos. El 48.5% tenían comorbilidades, siendo los trastornos electrofisiológicos los más frecuentes en el 72,66% de los casos. El 63% tenía cobertura social pero solo el 2,6% prepagos con cobertura en centros alta complejidad. El 23.6% recibía terapia combinada con 2 o más drogas. El 48,78% ya presentaban antecedente de algún tipo de reintervención, 98,5% de estas se vincularon a las cardiopatías moderadas a complejas. Registramos 15 embarazos con 14 recién nacidos vivos, 1 con cardiopatía congénita. El proceso de transición ­ transferencia en el 55% (170 p) se había iniciado, siendo efectiva (8p), frustra (9p), compartida (49 p), y en proceso (103 p). Hubo un solo fallecimiento durante el periodo de estudio, vinculado a cardiopatía compleja, múltiples reintervenciones y endocarditis. Conclusiones: El 92% de los pacientes en nuestro estudio, tienen cardiopatías operadas de moderada y severa complejidad. Los trastornos electrofisiológicos y la necesidad de reintervenciones durante el seguimiento alejado han sido las complicaciones más frecuentes de esta población. El proceso de transición y transferencia desde el hospital pediátrico al de adultos es deficitario, principalmente por falta de cobertura y experiencia sobre todo para la atención continua de las cardiopatías moderadas y complejas (AU)

Introduction: In recent decades, important advances in the diagnosis and treatment of patients with congenital heart defects have allowed more than 90% of them to reach adolescence and adulthood. However, many patients required lifelong follow-up and interventions, and therefore the need to be transitioned from pediatric to adult care. Material and Methods: Patients older than 15 years who consulted at the outpatient clinic of the department of cardiology at Garrahan Hospital from August 2017 to August 2018 were included. The variables analyzed were place of origin, underlying heart disease, and diagnosis, interventions, associated factors, such as genetic syndromes and other comorbidities, insurance coverage, educational level, pharmacological treatment, functional class, pregnancies, prevalence of heart disease in offspring, and transition-transfer to adult hospital. Results: We recorded 704 consultations from 309 patients with an average age of 19.17 years (SD +- 4.62; range 15-49.4 years); 112 patients were female and 197 male. Overall, 51.1% came from the province of Buenos Aires, 40% from the other provinces, and 8.1% from the city of Buenos Aires. Of the patients, 92% had moderate and severe heart disease, and 93.5% had undergone surgery. Genetic syndromes were identified in 13.2%. Overall, 48.5% had comorbidities, of which electrophysiological disorders were the most common in 72.66% of cases. 63% had social insurance coverage but only 2.6% had a prepaid insurance with coverage in tertiary-level centers. Overall, 23.6% received combination therapy with 2 or more drugs. 48.78% had undergone some type of previous reintervention, 98.5% of whom had moderate-to-severe heart disease. We recorded 15 pregnancies with 14 live neonates, one of whom had congenital heart defects. The transition - transfer had been initiated in 55% (170 p) and was effective (8p), frustrated (9p), shared (49 p), or in progress (103 p). There was only one death during the study period, related to severe heart disease, multiple reinterventions, and endocarditis. Conclusions: 92% of patients in our study have moderate or severe operated heart disease. Electrophysiological disorders and the need for reintervention during the long-term follow-up were the most common complications of this population. The process of transition and transfer from pediatric to adult care is deficient, mainly due to lack of insurance coverage and experience especially for the ongoing care of moderate-to-severe heart disease

Evaluación de la transferencia de adolescentes desde una unidad de cardiología pediátrica a una de adultos / Transfering adolescents from pediatric to adult cardiology units: evaluation

Resumen: Objetivos: evaluar la transferencia de adolescentes desde Cardiología Pediátrica (CP) a Cardiología de Adultos (CA) en el Hospital Dr. Hernán Henríquez de Temuco (HHHT), determinando si recibieron su primera atención en CA en la fecha requerida y si los controles se ajustaron al plazo indicado. Evaluar si la complejidad de la cardiopatía y ruralidad se asocian al cumplimiento en ambos objetivos, en los pacientes sin seguimiento adecuado. Material y Método: revisión de fichas de pacientes atendidos en CP del HHHT que, cumplida la edad requerida, fueron transferidos a CA, entre Octubre 2014 - Julio de 2017. Resultados: De 53 pacientes transferidos de CPCA, 12 (22.6%) tuvo traspaso no efectivo; de ellos, 6 presentaban complejidad moderada-alta (66,6%) y 3 eran rurales (25%). De 28 pacientes ingresados a CA, 14 (50%) tenían seguimiento inefectivo; de ellos 13 (92.6%) tenían cardiopatías de moderada-alta complejidad y 3 (21.5%) provenían de áreas rurales. Conclusiones: la transferencia de CP - CA fue inefectiva en 22.6% de los pacientes; la complejidad de la cardiopatía no se asoció al resultado de la transferencia en este grupo. En CA el seguimiento fue inefectivo en la mitad de los pacientes; de ellos, 92.8% tenían cardiopatías de moderada-alta complejidad, lo que fue estadísticamente significativo respecto de la complejidad en todos los pacientes traspasados. El domicilio no estuvo asociado a la pérdida de pacientes en la transferencia ni en el seguimiento. Estos hallazgos nos permitieron tomar medidas de mejoría en la transferencia y los controles en CA que esperamos mejoren los resultados futuros.

Abstracts: Aim: To evaluate the transfer of adolescents from Pediatric Cardiology (PC) to Adult Cardiology (AC) at Hospital Dr. Hernán Henríquez de Temuco (HHHT). We determined whether they got their first care in AC on the required date and whether controls were performed on the period prescribed. Also, to evaluate whether the complexity of cardiac disease and the patient's place of residence influenced the accomplishment of the objectives, in patients without adequate following. Material and Method: Retrospective review of clinical records of patients that were treated in PC at HHHT who, having the required age were transferred to AC, between October 2014 and July 2017. Results: Of 53 patients that were transferred from PC to AC, 12 (22.6%) had an non effective transfer; 6 of them had moderate to high complexity cardiac lesions (66.6%) and 3 lived in rural areas (25%). Of 28 patients admitted to AC, 14 (50%) had a non effective follow up and 13 (92.8%) had moderate to high complexity forms of heart disease; 3 (21.5%) came from rural areas. Conclusions: Transfer of patients from PC to AC was not effective in 22.6% of patients. The complexity of the disease was not related to the results of transfer in this group. At AC the follow-up was not effective in half the patients; from these, 92.6% had moderate to high complexity heart disease, which was significantly different from the complexity of all patients transferred. The place of residence was not relates to transfer neither to follow-up. These findings allowed us to make improvement in the transfer processes and the follow up at AC.

Bloqueio atrioventricular total congênito - características de 34 pacientes submetidos a implante ou troca de marcapasso definitivo / Congenital complete heart block - characteristics of 34 patients submitted to definitive pacemaker implant or replacement

Objetivos: Descrever as características de pacientes menores de 18 anos com bloqueio atrioventricular total congênito submetidos a implante ou troca de marcapasso definitivo. Métodos: Foram analisados retrospectivamente 34 pacientes que foram submetidos a implante ou troca de marcapasso entre janeiro de 2011 e maio de 2017 em um único centro. Os pacientes foram divididos em 2 grupos (grupo I: implante e grupo II: troca) e avaliados de acordo com o sexo, idade, cardiopatia congênita, indicação do implante, técnica utilizada, complicações e mortalidade. Resultados: 47,1% eram do sexo feminino. A mediana para idade no momento do implante foi de 2 anos e 6 meses e para peso foi de 17,5Kg. Cardiopatia congênita teve prevalência de 52,9% nos pacientes. As indicações para implante de marcapasso definitivo foram: frequência cardíaca menor que 70 bpm com cardiopatia ou disfunção (44,1%), frequência cardíaca menor que 55 bpm sem cardiopatia (26,5%), sinais e sintomas de baixo débito cardíaco (20,6%) e disfunção ventricular (8,8%). A técnica epicárdica foi realizada em 73,5% e a endocárdica em 26,5% dos pacientes. Foi implantado marcapasso bicameral em 53% e monocameral em 47% deles. Complicações foram observadas em 23,5% e a mortalidade foi de 8,8%. Conclusões: A criteriosa avaliação desses pacientes permite uma adequada escolha do sistema e da técnica de implante. Pouco mais da metade dos pacientes tinham cardiopatia congênita, a técnica epicárdica foi a mais realizada, a maioria recebeu marcapasso bicameral, e as complicações e a mortalidade foram semelhantes as encontradas na da literatura

Aims: Describe the characteristics of 34 patients under 18, with congenital complete heart block submitted for definitive pacemaker implant or replacement. Methods: We retrospectively analyzed patients who underwent pacemaker implant or replacement between January 2011 and May 2017 at a single center. The patients were divided into 2 groups (group I: implant and group II: replacement) and evaluated according to gender, age, congenital heart disease, implant indication, technique used, complications and mortality. Results: Thirty-four patients underwent definitive pacemaker implant or replacement, 47.1% of which were female. The median age at the time of implantation was 2 years and 6 months and median weight was 17.5 kg. Congenital heart disease was found in 52.9% of the patients. The indications for definitive pacemaker implant were heart rate lower than 70 bpm with heart disease or dysfunction (44.1%), heart rate less than 55 bpm without heart disease (26.5%), signs and symptoms of low cardiac output (20.6%) and ventricular dysfunction (8.8%). The epicardial technique was performed in 73.5% and the endocardial technique in 26.5% of the patients. A bicameral pacemaker was implanted in 53% and monocameral ventricular pacemaker in 47% of the patients. Complications were observed in 23.5%, with a mortality of 8.8% of the patients. Conclusions: The careful evaluation of the characteristics of these patients allows an adequate choice of the system and the implant technique. Just over half of the patients had congenital heart disease, the epicardial technique was the most performed, most received bicameral pacemaker, and complications and mortality were similar to the literature

Brigadas de salud en cardiología pediátrica: del triaje médico al triaje social / Pediatric cardiology health brigades: from medical triage to social triage

RESUMEN Este artículo explora aspectos socioculturales de un programa de brigadas de cardiología pediátrica para la atención de menores de poblaciones de escasos recursos que habitan en regiones periféricas de Colombia. Problematizamos las brigadas como estrategia humanitaria para cerrar las brechas de inequidad en el acceso a la atención en salud, y como contexto particular para el encuentro médico, la experiencia de la cardiopatía y la definición de las trayectorias de cuidado. A partir de la observación etnográfica de brigadas y de entrevistas a familias asistentes y personal de salud, realizadas durante el año 2016 en cinco ciudades diferentes, indagamos en las dinámicas que configuran el encuentro médico y cuestionamos los mecanismos (médicos y sociales) mediante los cuales se evalúa y decide qué familias pueden acceder a atención médica especializada en Bogotá. Se concluye que las brigadas, al ser iniciativas que continúan anclándose en el humanitarismo, en lugar de contribuir a la transformación de las condiciones que generan inequidades en salud acaban reproduciéndolas y exacerbándolas en la medida que seleccionan las vidas con prioridad para ser salvadas.

ABSTRACT This article explores the sociocultural aspects of a program of pediatric cardiology health brigades that provides care to children from low-income populations in peripheral regions of Colombia. We analyzed the brigades as a humanitarian strategy to close the gaps of inequity in access to health care, and as a particular context of the medical encounter, the experience of heart disease and the definition of care trajectories. Based on ethnographic observation of brigades and interviews with families receiving care and with health personnel, carried out in 2016 in five different cities, we looked at the dynamics that shape the medical encounter and questioned the mechanisms (medical and social) through which it is evaluated and decided which families can access care in Bogota. We conclude that the brigades, as initiatives that continue to be anchored in humanitarism instead of contributing to the transformation of the conditions that generate health inequities, reproduce and exacerbate such inequities by selecting which lives receive priority to be saved.

Fila de espera para tratamento de pacientes com cardiopatia congênita: relato de um Centro de Referência Amazônico / Waiting for cardiac procedure in congenital heart disease: portrait of an Amazonian Hospital

As cardiopatias congênitas são importantes causas de morbimortalidade infantil e, em cerca de 50% dos casos, é necessária a intervenção cirúrgica no primeiro ano de vida. Estima-se alto défice de procedimentos na Região Norte do Brasil.Analisar o tempo de espera para realização de tratamento eletivo cirúrgico e/ou intervencionista de crianças portadoras de cardiopatias congênitas em um centro de referência cardiológico, e fazer considerações sobre as cardiopatias e suas formas de tratamento na referida instituição. Estudo analítico, de caráter transversal do período de janeiro de 2012 a outubro de 2014, de pacientes com idade igual ou inferior a 14 anos diagnosticados com cardiopatias congênitas que estavam na fila de tratamento cardíaco eletivo cirúrgico ou percutâneo. Das 407 crianças que aguardavam por tratamento, a faixa etária mais prevalente foi a de > 2 a 6 anos (34,0%). O tempo médio de espera, em meses, foi 23,1 ± 18,3, com mediana de 19. As cardiopatias mais frequentes foram comunicação interventricular (28,98%), persistência do canal arterial (18,42%) e comunicação interatrial (11,05%). A maioria das crianças (63,4%) não pertencia à região metropolitana. As intervenções percutâneas representaram somente 27,84% do total de cateterismos e 14,85% de todos os tratamentos cardíacos. Cerca de 60% do volume de cirurgias pediátricas ocorreu em crianças sem cadastro prévio no sistema, devido ao caráter de urgência. Grande parte das crianças que aguardam por procedimento cardíaco é procedente de fora da região metropolitana e tem malformações potencialmente tratáveis por cateterismo. É necessário aumentar a capacidade operacional do único centro de referência público do Estado, além de descentralizar o atendimento em alta complexidade cardiológica da região metropolitana

Congenital heart disease is an important cause of morbidity and mortality in childhood, and in 50% of cases, surgery is required in the first year of life. A high deficit of surgical procedures is estimated in Northern Brazil. To analyze the waiting time for elective surgical treatment and/or intervention in children with congenital heart disease in a Cardiology referral center, and to make considerations about heart diseases and forms of treatment in that institution. A cross-sectional study of all patients aged less than 14 years, with a diagnosis of congenital heart disease that were waiting for elective surgical or percutaneous cardiac treatment. Among the 407 children with congenital heart defects, the most prevalent age group was > 2 to 6 years (34.0%). The average waiting time was 23.1 ± 18.3 months, with a median of 19. The most frequent heart disease was ventricular septal defect (28.98%), patent ductus arteriosus (18.42%) and atrial septal defect (11.05%). Most children (63.4%) were not from the metropolitan area. The percutaneous interventions represented only 27.84% of the catheterization procedures and 14,85% of all heart treatments. Approximately 60% of the pediatric surgeries occurred in children who were not previously registered due to urgency cases. Most of the children waiting for a cardiac procedure were not from the metropolitan area and had malformations potentially treatable by catheterization. It is necessary to increase the capacity of the single referral center in the state of Pará, as well as decentralize the high-complexity cardiological care in the metropolitan region