Drenaje venoso anómalo. Vena cava superior izquierda persistente que drena en la aurícula derecha y vena cava superior derecha que drena en la aurícula izquierda: reporte de un caso / Anomalous Venous Drainage. Persistent Left Superior Vena Cava Draining into the Right Atrium and Right Superior Vena Cava Draining into the Left Atrium: Case Report

Introducción: Entre 0,3-0,5% de niños nacidos presentan una vena cava superior izquierda persistente, lo que lo hace una de las malformaciones congénitas más frecuentes del drenaje venoso. El drenaje de esta en la aurícula derecha, además del drenaje de la vena cava superior derecha en la aurícula izquierda, es extremadamente infrecuente. Caso Clínico: Se presenta el caso de un infante de 8 meses asintomático que es llevado a consulta por presentar desaturaciones. El examen físico es normal. El ecocardiograma de contraste con solución salina muestra una vena cava superior izquierda persistente que drena en la aurícula derecha y un retorno anómalo de la vena cava superior derecha. Se realiza una corrección quirúrgica y evoluciona de forma favorable. Conclusión: La presentación en simultáneo de una vena cava superior izquierda persistente que drena en la aurícula derecha y una vena cava derecha que drena en la aurícula izquierda es extremadamente rara. La mayoría de los casos registrados se diagnosticaron de manera incidental en personas adultas al no presentar síntomas.

Introduction: Between 0.3-0.5% of children born have a persistent left superior vena cava, which makes it one of the most frequent congenital malformations of venous drainage. Drainage of this vein into the right atrium, in addition to drainage of the right superior vena cava into the left atrium, is extremely rare. Clinical case: We present a case of an asymptomatic 8-month-old infant who was taken to the clinic for desaturations. Physical examination was normal. The contrast echocardiogram with saline solution showed a persistent left superior vena cava draining into the right atrium and an anomalous return of the right superior vena cava. Surgical correction was performed and the patient evolved favorably. Conclusion: The simultaneous presentation of a persistent left superior vena cava draining into the right atrium and a right superior vena cava draining into the left atrium is extremely rare. Most of the reported cases were diagnosed incidentally in adults in the absence of symptoms.

Factors associated with malnutrition in infants with congenital heart disease within one year after surgery / 中华儿科杂志

Objective: To explore the risk factors of malnutrition in infants with congenital heart disease within one year after surgery. Methods: This retrospective cohort study selected 502 infants with congenital heart disease who underwent surgical treatment in Guangzhou Women and Children's Medical Center from February 2018 to January 2019. Their basic information and clinical data were analyzed, and their nutrition status after the surgery was followed up by questionnaire survey. Weight-for-age Z score (WAZ)≤-2 one year after operation was defined as malnutrition group, and WAZ>-2 was non-malnutrition group. The perioperative indicators and complementary food advancement were compared between the two groups by chi-square test, t-test, and Kruskal-Wallis test. The risk factors of malnutrition were analyzed by Logistic regression. Results: A total of 502 infants were selected, including 301 males and 201 females, with the age of 4.1 (2.0, 6.8) months. There were 90 cases in malnutrition group and 412 cases in non-malnutrition group. The body length and weight at birth in the malnutrition group were lower than those in the non-malnutrition group ((47.8±3.8) vs. (49.3±2.5) cm, (2.7±0.6) vs.(3.0±0.5) kg, both P<0.001). The proportion of paternal high school education or above and the proportion of family per capita income of 5 000 yuan or above in the malnutrition group were lower than those in the non-malnutrition group ((18.9% (17/90) vs. 30.8% (127/412), 18.9% (17/90) vs. 33.7% (139/412), both P<0.05). Compared to the non-malnutrition group, the proportion of complex congenital heart disease in the malnutrition group was higher (62.2% (56/90) vs. 47.3% (195/412), P<0.05). The postoperative mechanical ventilation time, postoperative intensive care unit (ICU) stay time, postoperative hospital stay, total length of ICU stay and total hospital stay in the malnutrition group were significantly longer than those in non-malnutrition group (all P<0.05). The proportion of egg and fish supplementation over 2 times/week within one year after the surgery was also lower in the malnutrition group (both P<0.05). Logistic regression analysis showed that mother's weight at delivery (OR=0.95,95%CI 0.91-0.99), the pre-operative WAZ≤-2 (OR=6.04, 95%CI 3.13-11.65), the complexity of the cardiac disease (OR=2.23, 95%CI 1.22-4.06), the hospital stay after the surgery over 14 days (OR=2.61, 95%CI 1.30-5.26), the types of complementary food<4 (OR=2.57, 95%CI 1.39-4.76), and the frequency of meat and fish<2 times/week (OR=2.11, 95%CI 1.13-3.93) were the risk factors associated with malnutrition within one year after the surgery. Conclusion: Mother's weight at delivery pre-operative nutritional status, complexity of cardiac disease, postoperative hospital stay, types of daily supplements and frequency of fish are risk factors associated with malnutrition within one year after surgery in children with congenital heart disease.

Interventional treatment and early-stage follow-up of pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective: To explore the application value of percutaneous peripheral interventional therapy in pulmonary atresia with intact ventricular septal (PA-IVS). Methods: Retrospective case summary. The data was collected from 25 children who were hospitalized at the Children's Hospital,Zhejiang University School of Medicine from August 2019 to August 2022, had been diagnosed with PA-IVS by echocardiography, and underwent interventional treatment. The sex, age, weight, operation time, radiation exposure time, and radiation dose of the patients were collected. The patients were divided into the arterial duct stenting group and the non-stenting group. Preoperative tricuspid annular diameters and Z scores, right ventricular length diameters, and right ventricular/left ventricular length-diameter ratios were compared by paired t-tests. Right ventricular systolic pressure difference, oxygen saturation, lactic acid before and after the surgery were compared for 24 children who received percutaneous balloon pulmonary valvuloplasty. Right ventricular improvement in 25 children after operation was analyzed. The correlation between postoperative oxygen saturation and postoperative right ventricular systolic blood pressure difference, the degree of pulmonary valve opening and the Z value of tricuspid valve ring in the non-stenting group were analyzed. Results: A total of 25 patients with PA-IVS were enrolled in the study, of whom 19 were males and 6 females, with an age at surgery of 12 (6, 28) days and a weight of (3.7±0.5) kg. One of them underwent only stenting of the arterial duct; 20 children underwent only percutaneous pulmonary valve perforation and balloon angioplasty; 4 children underwent both procedures. The Z-value of the tricuspid ring was -1.5±1.2 in the group with arterial duct stenting, and -0.1±0.4 in the group without stenting (t=2.77, P=0.010). The tricuspid regurgitant flow rate 1 month after surgery was significantly lower than the preoperative ((3.4±0.6) vs. (4.8±0.9) m/s, t=6.62,P<0.001). In the 24 children with percutaneous pulmonary valve perforation and balloon angioplasty, the preoperative right ventricular systolic blood pressure was (110±32) mmHg, and the postoperative systolic blood pressure was (52±19) mmHg (1 mmHg=0.133 kPa) (F=59.55, P<0.001). The factors that may affect postoperative oxygen saturation in 20 cases of non-stenting group were analyzed. The results suggested that the pre and post-operative right ventricular systolic blood pressure differences (r=-0.11, P=0.649), and the pulmonary valve orifice opening (r=-0.31, P=0.201) and tricuspid annulus Z value (r=-0.18, P=0.452) at 1 month after the operation were not significantly correlated with the postoperative oxygen saturation. Conclusions: Interventional therapy can be used as the first choice for one-stage operation of PA-IVS. Percutaneous pulmonary valve perforation and balloon angioplasty are more suitable for children with well-developed right ventricles, tricuspid annulus, and pulmonary arteries. While the smaller the tricuspid annulus, the more dependent it is on the ductus arteriosus and thus patients are more suitable for arterial duct stenting.

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.

Incidência, fatores associados e impacto de complicações pulmonares no pós-operatório de cirurgia cardíaca pediátrica / Incidence, associated factors and impact of pulmonary complications in the postoperative period of pediatric cardiac surgery

Objetivo: determinar a incidência, os fatores associados e o impacto das complicações pulmonares no pós-operatório de cirurgia cardíaca pediátrica. Metodologia: estudo de coorte, prospectivo, que incluiu lactentes e crianças submetidas à cirurgia cardíaca em um hospital pediátrico, no período de novembro de 2016 a julho de 2019. Foram coletados dados dos prontuários referentes ao sexo, idade, presença de outras malformações associadas, tipo de cardiopatia, ocorrência de complicações pulmonares, tempo de ventilação mecânica (VM) e de internamento na unidade de terapia intensiva (UTI) e óbito. Resultados: a amostra foi constituída por 111 lactentes e crianças, mediana da idade de 13 meses (7-32 meses), 54,1% do sexo feminino. Quanto ao tipo de cardiopatia, 80,2% foram cianogênicas. As complicações pulmonares ocorreram em 44,1% dos casos, sendo a mais frequente a atelectasia. A mediana do tempo de VM foi 8 horas (1-48h) e 45 (40,5%) permaneceram na VM por mais de 24h. A mediana do tempo de internamento na UTI foi de 7 dias (4-12dias). Evoluíram a óbito 7 (14,3%) pacientes. Conclusão: a amostra investigada apresentou incidência elevada de complicações pulmonares no pós-operatório de cirurgia cardíaca

Objective: to determine the incidence, associated factors and impact of pulmonary complications in the postoperative period of pediatric cardiac surgery. Methodology: prospective cohort study, which included infants and children undergoing cardiac surgery in a pediatric hospital, from November 2016 to July 2019. Data were collected from medical records regarding sex, age, presence of other associated malformations, type of heart disease, occurrence of pulmonary complications, duration of mechanical ventilation (MV) and admission to the intensive care unit (ICU) stay and death. Results: the sample consisted of 111 infants and children, median age 13 months (7-32 months), 54.1% female. As for the type of heart disease, 80.2% were acyanotic. Pulmonary complications occurred in 44.1% of cases, with atelectasis being the most frequent. The median time on mechanical ventilation (MV) was 8 hours (1-48h) and 45 (40.5%) remained on MV for more than 24h. The median length of stay in the ICU was 7 days (4-12 days). 7 (14.3%) patients died. Conclusion: the investigated sample had a high incidence of pulmonary complications in the postoperative period of cardiac surgery

Results of percutaneous closure with device in congenital heart disease of the atrial septal defect type / Resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular

Congenital Interatrial Septal Defects (TEA) are among the most common Congenital Heart Diseases in the population, where Atrial Septal Defect stands out, which is produced by a left-right short circuit that provides greater survival and less morbidity and mortality. The objective was to determine the results of Percutaneous Closure with a Device in Congenital Heart Disease of the Atrial Communication type at the Carlos Andrade Marín Specialty Hospital (Quito) in the Hemodynamics service from January 2009 to July 2019. It is a retrospective descriptive cross-sectional study . With a population of 122 cases from the AS400 registry. Thus, it was determined: there is a female predominance in a ratio of 2:1, the ranges for defect intervention were 5.5 mm to 35 mm, post-procedure pulmonary artery pressures (systolic 18-25 mmHg and diastolic 6-10 mmHg) in 95% of patients, ventricular overload in 76% in pre-procedure patients. Concluding that 96% of our population has a significant post-procedure improvement.

Los defectos del tabique interauricular congénitos (TEA) se encuentran entre las cardiopatías congénitas más comunes en la población donde resalta la comunicación interauricular que es producido por un cortocircuito izquierda derecha que brinda mayor sobrevida y menor morbimortalidad. El objetivo fue determinar los resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular en el hospital de especialidades Carlos Andrade Marín (Quito) en el servicio de hemodinamia en el período enero 2009 a julio 2019. Es un estudio transversal descriptivo retrospectivo. Con una población 122 casos del registro de AS400. Encontramos un predominio en sexo femenino en una proporción de 2:1, los rangos para intervención del defecto fueron de 5,5 mm a 35 mm, las presiones de la arteria pulmonar pos-procedimiento (sistólica de 18-25 mmHg y diastólica de 6-10 mmHg) en un 95% de los pacientes, sobrecarga ventricular en un 76% en los pacientes pre-procedimiento. Concluyendo que el 96% de nuestra población tiene una mejoría significativa post-procedimiento.

Risk factors for postoperative delirium in children with congenital heart disease: a prospective nested case-control study / 中国当代儿科杂志

OBJECTIVES@#To study the risk factors for postoperative delirium (POD) in children with congenital heart disease.@*METHODS@#A prospective nested case-control study was performed on children with congenital heart disease who underwent surgery in Fuwai Hospital, Chinese Academy of Medical Sciences, from December 2020 to June 2021. The clinical data were compared between the POD group (n=114) and non-POD group (n=102). A multivariate unconditional logistic regression analysis was used to investigate the risk factors for POD in children with congenital heart disease.@*RESULTS@#The multivariate logistic regression analysis showed that age (OR=0.951, P<0.001), gender (OR=2.127, P=0.049), number of invasive catheters per day (OR=1.490, P=0.017), degree of postoperative pain (OR=5.856, P<0.001), and preoperative parental anxiety level (OR=1.025, P=0.010) were independent risk factors for POD in children with congenital heart disease.@*CONCLUSIONS@#The risk of POD increases in children with congenital heart disease who are younger, male, have higher number of invasive catheters per day, higher degree of postoperative pain, or higher preoperative parental anxiety level.

Medium and long-term follow-up of the Pul-Stent in treating postoperative branch pulmonary artery stenosis in children with congenital heart disease / 中华儿科杂志

Objective: To evaluate the effectiveness and safety of Pul-Stent as the treatment of postoperative branch pulmonary artery stenosis in children with congenital heart disease. Methods: This was a retrospective study. Thirty-three patients who underwent Pul-Stent implantation in Shanghai Children's Medical Center due to postoperative residual pulmonary artery stenosis from August 2014 to June 2015 were included. The immediate curative effect, follow-up and complications of Pul-Stent implantation were assessed. Comparisons between groups were performed with unpaired Student t test. Results: Pul-Stent implantation of 33 patients (19 males and 14 females) were performed successfully. Thirty-one patients underwent percutaneous stenting, and 2 patients underwent hybrid stenting. A total of 35 Pul-Stents were implanted (19 of model small, 15 of model medium and one of model large), 23 stents were planted in the proximal left pulmonary artery and 12 stents were in the proximal right pulmonary artery. The initial diameter of dilation balloon ranged from 6 to 16 mm, and the long sheath of percutaneous implantation ranged from 8 to 10 F in 29 patients (29/31, 94%). After stenting, the diameter of the narrowest segment of pulmonary artery increased from (4.0±1.7) mm to (9.1±2.1) mm in all patients (t=-21.60, P<0.001). The pressure gradient at the stenosis in 26 patients after biventricular correction decreased from (30.5±12.3) mmHg (1 mmHg=0.133 kPa) to (9.9±9.6) mmHg (t=12.92, P<0.001), and the right ventricular to aortic pressure ratio decreased from 0.57±0.14 to 0.44±0.12 (t=7.44, P<0.001). The pressure of the superior vena cava after stenting in 5 patients after cavopulmonary anastomosis decreased from (17.0±1.9) mmHg to (14.0±0.7) mmHg (t=2.86, P=0.046). Two patients died during reoperation for repairing other cardiac malformations. The remaining 31 patients were clinically stable during the follow-up period of (5.3±1.6) years, and one stent fracture was found on chest X-ray. Cardiac catheterization reexaminations in 16 patients showed that restenosis was found in one stent, while stent position and patency were satisfactory in the remaining stents. Nine children underwent post-dilation without stent fracture, displacement or aneurysm formation. Cardiac tomography showed no stent stenosis, fracture observed, or significant change in diameter of the stent in 8 patients. The inner diameter and pulmonary blood perfusion could not be accurately evaluated due to artifacts by cardiac magnetic resonance imaging in 4 patients. Conclusions: Pul-Stent has good compliance and adequate radial strength, and can dilate further over time to accommodate for somatic growth. It performs safely and effectively in treating post-operative branch pulmonary artery stenosis in children.

Preliminary practice in the postoperative cardiopulmonary exercise testing for children with congenital heart disease / 中华心血管病杂志

Objective: To investigate the postoperative cardiopulmonary fitness of children with congenital heart diseases (CHD). Methods: This is a retrospective study. A total of 136 children after cardiac correction undergoing cardiopulmonary exercise test (CPET) in Shanghai Children's Medical Center from March 1 to June 30, 2021 were selected. According to the surgical procedure, the children were divided into two groups: the biventricular correction group (BV group) (n=75) and single ventricular correction group (SV group) (n=61). The BV group was divided into two subgroups: simple congenital heart disease (S-CHD) group (n=35) and complex congenital heart disease (C-CHD) group (n=40). CPET parameters, including VO2 max, VO2/kg max, VO2/kg@AT, O2/HR max, HRR, PetCO2 max, CI, HRR at 1 min, VE/VCO2 slope, OUES/kg and EOV, were analyzed. CPET parameters of patients underwent different procedure were compared. In the subgroup analysis, CPET parameters of patients in S-CHD group and C-CHD group were compared. The parameters of the BV group were compared with the normal value. Linear correlation analysis was used to identify the correlation between the CPET parameters. Results: A total of 136 children with CHD after surgery were enrolled. The age was (9.4±3.1) (ranged 6.2-16.0) years, and there was 84 (61.8%) male. All the children completed CPET examination safely without experiencing serious circulation abnormalities. Compared with the SV group, the VO2/kg max ((32.6±6.9) ml·kg-1·min-1 vs. (23.5±5.9) ml·kg-1·min-1, P<0.001), O2/HR max ((7.24±2.93) ml/beat vs. (6.35±2.17)ml/beat, P=0.030), HRR at 1 min ((32.5±13.9) beat/min vs. (26.3±12.5) beat/min, P=0.036), OUES/kg (36.9±8.8 vs. 29.7±11.8, P=0.001) were significantly higher, VE/VCO2 slope (29.1±5.20 vs. 35.1±8.0, P<0.001) and incidence of EOV (32.0%(24/75) vs. 57.4%(31/61), P=0.027) were significantly lower in BV group. Compared with the simple CHD subgroup, VE/VCO2 slope and the incidence of EOV were higher, VO2/kg max, O2/HR max, HRR at 1 min and OUES/kg were lower in the complex CHD subgroup (all P<0.05). Cardiopulmonary function parameters of the BV group were lower than the normal value. Linear correlation analysis showed that VO2/kg@AT was strongly correlated with VO2/kg max (r=0.86, P<0.001), VO2/kg max was strongly correlated with OUES/kg (r=0.63, P<0.001), HRR was strongly correlated with CI (r=0.91, P<0.001), and VO2/kg max was strongly correlated with OUES/kg (r=0.63, P<0.001). VE/VCO2 slope was strongly correlated with PetCO2 max (r=1.00, P<0.001). Conclusions: The exercise cardiopulmonary function of children after single ventricular correction is weaker than that of biventricular correction, and the exercise tolerance of children after biventricular correction is lower than that of normal children. Among the children after biventricular correction, the exercise tolerance of children with complex CHD is lower than that of children with simple CHD. Postoperative CPET is of important realistic significance for CHD children.

Hospitalizaciones por cardiopatías congénitas en la Unidad de Cardiología Pediátrica del Centro Hospitalario Pereira Rossell / Hospitalization due to congenital heart disease at the Pereira Rossell Pediatric Hospital's Cardiology Unit / Internações por cardiopatia cardíaca congênita na Unidade de Cardiologia Pediátrica do Centro Hospitalar Pereira Rossell

Introducción: las cardiopatías congénitas (CC) son patologías frecuentes en pediatría. Son causa de importante morbimortalidad en la infancia. Son pacientes complejos, que requieren abordaje integral, en equipo interdisciplinario. Objetivos: describir las características epidemiológicas, clínicas y evolutivas de niños portadores de CC asistidos en la Unidad de Cardiología del HP-CHPR durante el año 2015, así como su estado nutricional y los factores que pudieran influir en este. Material y método: estudio descriptivo, retrospectivo, a través de la revisión de historias clínicas de pacientes portadores de CC, hospitalizados en la Unidad de Cardiología del HP-CHPR en 2015. Se describieron características epidemiológicas y clínicas de los pacientes. Resultados: en el período evaluado egresaron 63 pacientes, que representaron una tasa de 6,58/1.000 egresos hospitalarios. Tenían un promedio de edad de 23,8 meses. Un total de 8 pacientes fueron diagnosticados en el período prenatal, 54 presentaban CC no cianóticas. Habían sido sometidos a tratamiento quirúrgico 24 niños, 13 con cirugía correctiva. Se diagnosticó desnutrición en 43%. Esta fue más prevalente en portadores de CC cianótica, de defectos complejos, y de cromosomopatías u otras malformaciones. Conclusión: los niños con CC representaron un pequeño porcentaje de quienes requirieron hospitalización en el HP-CHPR. El porcentaje de desnutrición fue elevado. Este estudio confirma la importante morbimortalidad de los niños pequeños portadores de CC. Es importante reconocer el mayor riesgo de estos pacientes y actuar en forma individualizada y oportuna.

Introduction: congenital heart disease (CHD) is a frequent pathology among children. It may cause significant morbidity and mortality during childhood. These are complex patients, who require a comprehensive approach and an interdisciplinary team. Objective: to describe the epidemiological, clinical and evolutionary characteristics of children with CHD assisted in the Cardiology Department of the Children Hospital-CHPR in 2015. Material and Methods: descriptive, retrospective study, review of clinical records of patients with CHD, hospitalized in the Cardiology Department of the Children's Hospital PRHC in 2015. Epidemiological and clinical characteristics of patients were described. Results: 63 patients were hospitalized during the evaluated period, a total rate of 6.58/1.000 discharges. They had an average age of 23.8 months. Eight patients were diagnosed in the prenatal period, 54 presented non-cyanotic CHD. Twenty-four children had undergone surgical treatment, 13 had had corrective surgery. Malnutrition was diagnosed in 43% and it was more prevalent in cyanotic CHD carriers, complex defects, and chromosomopathies or other malformations. Conclusion: CHD represented a small percentage of the children who required hospitalization at the PH-PRHC. The percentage of malnutrition was high. The study confirmed the significant morbidity and mortality of these patients. It is important to recognize the highest risk of these patients and act in a customized and timely fashion.

Introdução: as cardiopatias congênitas (CC) são patologias comuns em pediatria. Elas são uma causa significativa de morbidade e mortalidade na infância. Os pacientes são complexos e requerem uma abordagem integral e uma equipe interdisciplinar. Objetivos: descrever as características epidemiológicas, clínicas e evolutivas de crianças com CC atendidas na Unidade de Cardiologia do Hospital Pediátrico PHPR durante o ano de 2015, descrever seu estado nutricional e os fatores que poderiam influenciá-lo. Material e métodos: estudo descritivo, retrospectivo, feito por meio da revisão de prontuários de pacientes com CC, internados na Unidade de Cardiologia do HP-CHPR em 2015. Foram descritas as características epidemiológicas e clínicas dos pacientes. Resultados: no período avaliado, 63 pacientes receberam alta hospitalar, representando uma taxa de 6,5/1.000 altas hospitalares. Eles tinham uma idade média de 23,8 meses. Oito pacientes foram diagnosticados no período pré-natal, 54 apresentavam CC não cianótica. 24 crianças tinham sido submetidas a tratamento cirúrgico, 13 tinham recebido cirurgia corretiva. 43% foram diagnosticadas com desnutrição, a qual foi mais prevalente em portadores de CC cianótica, defeitos complexos e malformações cromossômicas ou outras. Conclusão: os CCs representaram um pequeno percentual de crianças que necessitaram de internação no HP-CHPR. O percentual de desnutrição foi alto. Este estudo confirma a morbidade e mortalidade significativas de crianças pequenas com CC. É importante reconhecer o risco aumentado desses pacientes e agir de forma individualizada e oportuna.

Central venous oxygen saturation/lactate ratio and prediction of major adverse events after pediatric heart surgery

Abstract Introduction: Major adverse events (MAE) are unexpected but undesirably frequent after pediatric congenital heart surgery and contribute to poorer outcomes. The aim of this study was to test the predictive value of a ratio between central venous oxygen saturation and arterial lactate (ScvO2/lactate) for MAE after pediatric congenital heart surgery in a Brazilian university hospital. Methods: We conducted a retrospective observational study in a tertiary care university hospital, including 194 infants and children submitted to surgery for congenital heart disease. The predictive value of ScvO2, lactate, and ScvO2/lactate ratio were assessed by the area under the receiver operating characteristics curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). Results: The incidence of MAE was 16% — cardiac arrest/death, unplanned reoperation, and low cardiac output syndrome were the most common events. Overall, ScvO2/lactate ratio discriminated patients with and without MAE very well (AUC 0.842), performing better than either variable alone, with sensitivity of 48%, specificity of 94%, PPV of 60%, and NPV of 91%. Conclusion: A ScvO2/lactate ratio > 5 can accurately identify patients at low risk of MAE after pediatric congenital heart surgery, with very good specificity and NPV, but poor sensitivity and PPV.

WeChat-Assisted Preoperative Health Education Reduces Burden of Care on Parents of Children with Simple Congenital Heart Disease: a Prospective Randomized Controlled Study

Abstract Objective: The purpose of this study was to explore the clinical effect of preoperative health education based on the WeChat platform for parents of children with simple congenital heart disease. Methods: In this study, participants were randomly divided into an intervention group (WeChat group, n=40) and a control group (leaflet group, n=40) in our center. All parents were required to complete the Family Caregiver Task Inventory (FCTI) on the first visit and the Zarit Burden Interview (ZBI) and FCTI before the operation. Clinical, family and relevant data from children and parents were collected and subsequently analyzed. Results: Before the operation, the FCTI score and the ZBI score in the WeChat group were significantly lower than those in the control group (P=0.010, P=0.027, respectively). Compared to the FCTI score on the first visit, the preoperative status score was significantly lower in the WeChat group (P=0.008). The rate of loss to follow-up and complications in the WeChat group was also significantly lower than in the control group (P=0.003). Conclusion: Preoperative health education assisted by the WeChat platform for parents of children with simple congenital heart disease can effectively improve the parents' care ability and reduce the burden of care, preoperative complications and the rate of loss to follow-up.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Origen anómalo de una de las ramas de la arteria pulmonar (OAAP): tratamiento quirúrgico y resultados / Anomalous origin of one of the branches of the pulmonary artery (AOPA): Surgical treatment and outcome

Resumen Introducción: El origen anómalo de una de las ramas de la arteria pulmonar procedente de la aorta ascendente es poco frecuente. Objetivo: Identificar las características clínicas y quirúrgicas de los pacientes sometidos a reimplante de la rama afectada. Método: Se realizó un estudio observacional, transversal, descriptivo, retrospectivo y retrolectivo, en el que se incluyeron los pacientes diagnosticados de origen anómalo de alguna de las ramas de la arteria pulmonar y que fueron tratados mediante cirugía correctiva en el Instituto Nacional de Cardiología Ignacio Chávez, en el periodo del 1 de enero de 2003 al 31 de enero de 2019. De los expedientes se extrajeron las características demográficas, los antecedentes quirúrgicos, los diagnósticos, los reportes ecocardiográficos y tomográficos, la técnica quirúrgica y el estado posquirúrgico. Resultados: Se encontraron nueve pacientes sometidos a cirugía de corrección, con un promedio de edad de 2 ± 2 años, un peso de 11.4 ± 1.5 kg y una talla de 82 ± 15 cm. El 67% fueron de sexo masculino. La rama afectada con más frecuencia fue la derecha; el 68% se originaban directamente de la aorta y el 42% de un conducto arterioso persistente. La corrección con circulación extracorpórea tuvo un tiempo promedio de pinzamiento aórtico de 35 minutos y de soporte circulatorio de 45 minutos. Se utilizó el implante directo o con material sintético. Las complicaciones fueron falla ventricular, sangrado, arritmias y neumonía nosocomial. La mortalidad fue del 11%. Conclusiones: El tratamiento quirúrgico para el reimplante de la rama anómala de la arteria pulmonar es el procedimiento de elección, y con cuidados quirúrgicos y posteriores tiene un buen pronóstico a mediano y largo plazo.

Abstract Introduction: The anomalous origin of one of the branches of the pulmonary artery from the ascending aorta is rare. Objective: To identify the clinical and surgical characteristics of the patients undergoing reimplantation of the affected branch. Method: An observational, cross-sectional, descriptive, retrospective and retrolective study was performed; patients diagnosed with anomalous origin of one of the branches of the pulmonary artery and treated by corrective surgery at the Instituto Nacional de Cardiología Ignacio Chavez, in the period from January 1, 2003 to January 31, 2019, were included. It was extracted from the files: demographic characteristics, surgical antecedents, diagnoses, echocardiographic and tomographic reports, surgical technique and post-surgical status. Results: Nine patients underwent correction surgery, with an average age of 2 ± 2 years, 11.4 ± 1.5 kg and height 82 ± 15 cm; 67% were male. The most frequent affected branch was the right, 68% originated directly from the aorta and 42% from a patent ductus arteriosus. The correction with extracorporeal circulation had an average aortic cross-clamp of 35 minutes and circulatory support of 45 minutes, the direct or synthetic implant was used. The complications were ventricular failure, bleeding, arrhythmias and nosocomial pneumonia. Mortality was 11%. Conclusions: Surgical treatment for reimplantation of the anomalous branch of the pulmonary artery is the procedure of choice, which with surgical and subsequent care has a good prognosis in the medium and long term.

Glenn surgery: a safe procedure in the path of univentricular correction / Cirugía de Glenn: un procedimiento seguro en el camino de la corrección univentricular

Abstract Background: This study describes 35 years of experience in a tertiary care level hospital that treats cardiac patients with univentricular heart physiology who underwent Glenn surgery. Methods: The study consisted of a retrospective analysis of patients who underwent Glenn surgery, including variables related to pre-operative, intra-operative, and post-operative morbidity and mortality. Results: From 1980 to 2015, 204 Glenn surgeries were performed. The most common heart disease was tricuspid atresia IB (19.2%). In 48.1% of the cases, the procedure was performed with antegrade flow. A bilateral Glenn procedure was performed in 12.5% of the cases and 10.3% were carried out without using a cardiopulmonary bypass pump. Reported complications included infections, bleeding, arrhythmias, chylothorax, neurological alterations, and pleural effusion. The mortality rate was 2.9% Conclusions: Glenn surgery is a palliative surgery with good results. It significantly improves patient quality of life over a long period until a total cavopulmonary shunt is performed. The complications observed are few, and the mortality rate is low. Therefore, it is a safe surgery that should be used for univentricular congenital heart disease.

Resumen Introducción: El objetivo de este trabajo fue describir la experiencia de 35 años en un hospital de tercer nivel con pacientes cardiópatas con fisiología univentricular que fueron sometidos a cirugía de Glenn. Métodos: Se presenta un análisis retrospectivo de los pacientes sometidos a cirugía de Glenn. Se incluyeron variables relacionadas con la morbilidad y la mortalidad preoperatorias, transoperatorias y posoperatorias. Resultados: Desde 1980 hasta 2015 se realizaron 204 cirugías de Glenn para la corrección de cardiopatías. De ellas, la más frecuente fue la atresia tricuspídea IB (19.2%); en el 48.1% se realizó Glenn con flujo anterógrado, en el 12.5% Glenn bilateral y el 10.3% de las cirugías fueron sin apoyo de bomba de circulación extracorpórea. Las complicaciones reportadas fueron infecciones, sangrado, arritmias, quilotórax, alteraciones neurológicas y derrame pleural. La mortalidad fue del 2.9%. Conclusiones: La cirugía de Glenn es un procedimiento paliativo que se ha utilizado con buenos resultados. Mejora en forma importante la calidad de vida del paciente durante un largo periodo mientras que se realiza la derivación cavopulmonar total. Presenta pocas complicaciones y muy baja mortalidad, por lo que es una cirugía segura que debe ser utilizada para cardiopatías congénitas univentriculares.

Efectos de la impresión 3D en la planificación quirúrgica de las cardiopatías congénitas / Impact of 3D printing in surgical planning of congenital heart disease

Resumen Introducción: Los defectos cardíacos congénitos constituyen el 30% de todas las anomalías congénitas. La prevalencia es de 8/1,000 recién nacidos vivos, sin predominio de género. Para una planificación quirúrgica óptima es esencial una evaluación precisa de la anatomía en los defectos cardíacos congénitos. Las modalidades de imagen como el ecocardiograma, la angiografía por cateterismo cardíaco, la tomografía computarizada (TC) o la resonancia magnética (RM) se utilizan de forma regular para el diagnóstico de las cardiopatías congénitas. Estos métodos pueden proporcionar reconstrucciones virtuales en reconstrucción volumétrica o 3D, pero no réplicas táctiles reales de la anatomía cardíaca. Objetivo: Realizar modelos de corazón impresos en 3D con la finalidad de proporcionar réplicas táctiles 3D reales de la anatomía cardíaca para visualizar de forma detallada todas las perspectivas posibles de las estructuras extracardíacas o intracardíacas. Métodos: Los datos de la imagen se obtuvieron en formato DICOM, se editaron en el paquete de software "3D slicer 4.3" y se exportaron para la impresión en formato de archivo (.stl). Resultados y conclusiones: Con la impresión 3D se puede evaluar de forma detallada la anatomía intracardíaca y extracardíaca con modelos cardíacos en tiempo real. Esta técnica es de gran utilidad, sobre todo en los defectos cardíacos congénitos complejos, ya que permite hacer una planificación precisa del procedimiento quirúrgico.

Abstract Introduction: Congenital heart disease makes up for 30% of all congenital anomalies. The prevalence is 8/1,000 live newborns, without predominance of gender. Imaging methods such as echocardiography, angiography, computed tomography or magnetic resonance imaging must be routinely used in congenital heart disease. The mentioned methods can provide virtual reconstructions in volumetric reconstruction or in three dimensional (3D), but only 3D-printed heart models can provide real 3D tactile replicas of cardiac anatomy. Objective: To make 3D printed heart models in order to provide real 3D tactile replicas of the cardiac anatomy that allow a detailed visualization from all possible perspectives, either of extracardiac or intracardiac structures. Methods: This information is useful for surgical decision making, especially in patients with complex cardiac defects. DICOM, edited in a software package "3D slicer 4.3" and exported for printing in file format (.stl). Results and conclusions: With 3D printing, the intracardiac and extracardiac anatomy can be evaluated in detail with real-scale cardiac models of the patient, avoiding unexpected findings. This technique is very useful especially in complex congenital heart defects, since it allows precise planning of the surgical procedure.

Quality of life of children and adults following cardiac surgery for congenital heart disease: A Mexican cohort / Calidad de vida en niños y adultos después de cirugía para cardiopatías congénitas: primera cohorte mexicana

Abstract Objective: The real burden of (congenital heart defects [CHD]) and the improvement after surgical correction or palliation is both reflected in the quality of life (QoL). There are few studies in Latin-America that evaluate QoL in the CHD population. The purpose of this study was to measure the QoL after corrective or palliative surgery for CHD. Materials and methods: An observational, cross-sectional, and comparative study was carried out at the Miguel Hidalgo Centennial Hospital. Patients from 8 to 18 years old who underwent surgery for CHD were included during a period of 8 months. A total of 40 patients were included, together with a group of 80 healthy controls. From all participants, a KIDSCREEN-52 questionnaire was taken. A comparative analysis of the results was performed. Results: Overall, patients with cardiac surgery had better QoL indexes than healthy controls (p < 0.0001). The difference was greatest in moods and emotions, autonomy, and parent relations. Conclusions: Self-perception of QoL in post-operative patients for congenital heart disease is similar and in certain dimensions higher than the healthy population, possibly due to socioeconomic differences, parental care, and styles of coping with their disease. Higher complexity studies that include psychosocial variables and parental perception are required, and a better understanding of the QoL determinants will improve the attention provided to the patient and their families.

Resumen Objetivo: Los efectos de las cardiopatías congénitas en los pacientes, así como la mejoría después de la paliación o la corrección quirúrgica, se reflejan en la calidad de vida (CV). Hay pocos estudios en Latinoamérica que evalúen la CV en esta población. El objetivo de este artículo es notificar la CV posterior a la operación paliativa o correctiva para defectos cardíacos congénitos. Material y métodos: Se llevó a cabo un estudio observacional, transversal y comparativo en el Centenario Hospital Miguel Hidalgo. Durante un período de ocho meses se incluyó a pacientes de 8 a 18 años sometidos a una intervención para cardiopatías congénitas. Se reunió a dos grupos de 40 pacientes y 80 controles sanos, pareados por edad y sexo. A cada participante se le aplicó el cuestionario KIDSCREEN-52 y se realizó un análisis comparativo de los resultados. Resultados: Los pacientes sometidos a operación cardíaca tuvieron mejores índices de CV que los controles sanos (p < 0.0001). La mayor diferencia se obtuvo en los aspectos de estado de ánimo y emociones, autonomía y relación con los padres. Conclusiones: La autopercepción de la CV después de una intervención para cardiopatías congénitas es similar y, en ciertas dimensiones, mejor que la de la población sana, tal vez por diferencias socioeconómicas, atención de los padres y modelos de adaptación a la enfermedad. Se requieren estudios más extensos que incluyan variables psicosociales y percepción parental. Una mayor comprensión de los determinantes de la CV podría mejorar la atención ofrecida al paciente y su familia.

Effect of calorie-enriched formula on postoperative catch-up growth in infants with cyanotic congenital heart disease: a propective randomized controlled study / 中国当代儿科杂志

OBJECTIVE@#To study the effect of calorie-enriched formula on postoperative catch-up growth in infants with cyanotic congenital heart disease (CHD).@*METHODS@#A total of 100 infants with cyanotic CHD who underwent surgical operation from January to December, 2017, were randomly divided into a high-calorie group (receiving calorie-enriched formula after surgery) and a conventional group (receiving standard formula after surgery), with 50 infants in each group. All infants were followed up for 6 months. The observation indices included body height, body weight, prealbumin, and N-terminal pro-brain natriuretic peptide before surgery, at the time of ventilator weaning and extubation after surgery, and at 1, 3, and 6 months after surgery. Height-for-age Z-score (HAZ), weight-for-age Z-score (WAZ), and weight-for-height Z-score (WHZ) were also assessed. Adverse reactions were recorded for both groups.@*RESULTS@#There were 25 cases (50%) and 21 cases (42%) of malnutrition in the high-calorie group and the conventional group respectively before surgery (@*CONCLUSIONS@#Compared with the standard formula, calorie-enriched formula can better help with postoperative catch-up growth in infants with cyanotic CHD.