Background: The evidence regarding patient related outcomes in children with infrequent congenital heart defects (I-CHD) is very limited. We sought to measure quality of life (QoL) in children with I-CHD, and secondarily, to describe QoL changes after one-year of follow-up, self-reported by children and through their caregivers' perspective. Methods: We assembled a cohort of children diagnosed with an I-CHD in a cardiovascular referral center in Colombia, between August 2016 and September 2018. At baseline and at one-year follow-up, a clinical psychology assessment was performed to establish perception of QoL. The Pediatric Quality of Life Inventory (PedsQL) 4.0 scale was used in both general and cardiac modules for patients and for their caregivers. We used a Mann-Whitney U test to compare scores for general and cardiac modules between patients and caregivers, while a Wilcoxon test was used to compared patients' and caregivers' baseline and follow-up scores. Results are presented as median and interquartile range. Results: To date, QoL evaluation at one-year follow-up has been achieved in 112/157 patients (71%). Self-reported scores in general and cardiac modules were higher than the QoL perceived through their caregivers, both at baseline and after one-year of follow-up. When compared, there was no statistically significant difference in general module scores at baseline between patients (median = 74.4, IQR = 64.1-80.4) and caregivers scores (median = 68.4, IQR = 59.6-83.7), p = 0.296. On the contrary, there was a statistical difference in baseline scores in the cardiac module between patients (median = 79.6, IQR = 69.7-87.4) and caregivers (median = 73.6, IQR = 62.6-84.3), p = 0.019. At one-year of follow-up, scores for the general module between patients (median = 72.8, IQR = 59.2-85.9) and caregivers (median = 69.9, IQR = 58.1-83.7) were not statistically different (p = 0.332). Finally, a significant difference was found for cardiac module scores between patient (median = 75.0, IQR = 67.1-87.1) and caregivers (median = 73.1, IQR = 59.5-83.8), p = 0.034. Conclusions: QoL in children with I-CHD can be compromised. However, children have a better perception of their QoL when compared with their caregivers' assessments. To provide high-quality care, besides a thorough clinical evaluation, QoL directly elicited by the child should be an essential aspect in the integral management of I-CHD.

RESUMEN Objetivo: comparar los resultados de los abordajes abiertos y laparoscópicos en esplenectomía de pacientes con patología esplénica de origen hematológico. Métodos: se realizó un estudio observacional de corte transversal, teniendo como fuente de datos el registro institucional de las esplenectomías abiertas y laparoscópicas realizadas en la Fundación Cardioinfantil-Instituto de Cardiología entre 1996 y 2016. Se evaluaron variables preoperatorias, intraoperatorias y posoperatorias; se compararon la tasa de complicaciones, tiempo quirúrgico, estancia hospitalaria y la necesidad de re-intervención entre los dos abordajes.

BackgroundEbstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes.HypothesisPhenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors.MethodsA comprehensive cross‐sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis.ResultsThe most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff‐Parkinson‐White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities.ConclusionsThis study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting.

ObjectivesSerum lactate is a useful biomarker of tissue perfusion in critically ill patients. We evaluated the behavior of serum lactate in children in the pediatric intensive care unit (PICU) immediately after liver transplantation and its association with surgical complications, graft dysfunction and 90-day mortality.Materials and MethodsA prospective observational study carried out between November 2009 and December 2019. Multidisciplinary PICU at the University Children's Hospital, Fundación Cardioinfantil-IC, Bogotá, Colombia.Measurements and Main ResultsPatients between 1 month and 18 years of age who were in the immediate post-operative period following living-donor or cadaveric liver transplantation were included. A total of 145 patients with a median age of 14 months (IQR 8–60) met the inclusion criteria. Biliary atresia was the main diagnosis in 56.5% of the cases. A serum lactate level > 3.0 mmol/L on admission to the PICU was associated with biliary complications (AUC 0.73 95% CI 0.54–0.93; p = 0.05) and mortality (AUC 0.72 95% CI 0.63–0.8; p = 0.01). A lactate level > 2 mmol/L after 6 h in the PICU was associated with mortality (AUC 0.70 95% CI 0.54–0.83; p = 0.02). Higher lactate levels and lack of clearance were associated with the presence of tardus et parvus waveforms (p = 0.001) on liver Doppler, primary dysfunction (p < 0.001), arterial thrombosis (p < 0.001) and neurological complications (p = 0.04). There was an inverse correlation between admission lactate and the volume of fluids administered during surgery (rho = 0.36; p < 0.001). A total procedure time > 350 min, along with a vasopressor score > 7 and elevated lactate, were associated with worse outcomes (p < 0.001).ConclusionsIn post-operative pediatric liver transplant patients, the level of serum lactate is associated with post-operative surgical complications and mortality.

OBJECTIVES Globally congenital heart disease mortality is declining, yet the proportion of infant deaths attributable to heart disease rises in Colombia and other middle-sociodemographic countries. We aimed to assess the accessibility of paediatric cardiac surgery (PCS) to children <18 years of age in 2016 in the South American country of Colombia. METHODS In Bogotá, Colombia, a multi-national team used cross-sectional and retrospective cohort study designs to adapt and evaluate 4 health system indicators at the national level: first, the population with timely geographic access to an institution providing PCS; second, the number of paediatric cardiac surgeons; third, this specialized procedure volume and its national distribution; and fourth, the 30-day perioperative mortality rate after PCS in Colombia. RESULTS Geospatial mapping approximates 64% (n = 9 894 356) of the under-18 Colombian population lives within 2-h drivetime of an institution providing PCS. Twenty-eight cardiovascular surgeons report performing PCS, 82% (n = 23) with formal training. In 2016, 1281 PCS procedures were registered, 90% of whom were performed in 6 of the country’s 32 departments. National non-risk-adjusted all-cause 30-day perioperative mortality rate after PCS was 2.73% (n = 35). CONCLUSIONS Colombia’s paediatric population had variable access to cardiac surgery in 2016, largely dependent upon geography. While the country may have the capacity to provide timely, high-quality care to those who need it, our study enables future comparative analyses to measure the impact of health system interventions facilitating healthcare equity for the underserved populations across Colombia and the Latin American region.