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.

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Background: The approach to coarctation of the aorta with hypoplastic aortic arch is controversial. We evaluated the outcomes in patients with coarctation of the aorta with or without hypoplastic aortic arch operated through a posterior left lateral thoracotomy. Methods: A retrospective cohort of patients with aortic coarctation, who underwent repair between January 2009 and October 2017, was analyzed. Preoperative, postoperative, and echocardiographic characteristics were reviewed. Statistical analysis examined survival, freedom from reintervention, and freedom from recoarctation. Results: In nine years, 389 patients who underwent surgical treatment for coarctation of the aorta were identified; after exclusion criteria and complete echocardiographic reports, 143 patients were analyzed, of which 29 patients had hypoplastic aortic arch. The modification in the extended end-to-end anastomosis technique was a wide dissection and mobilization of the descending aorta that was achieved due to the ligation and division of 3 to 5 intercostal vessels. In both groups, patients were close to one month of age and had a median weight of 3.6 and 3.4 kg for hypoplastic and nonhypoplastic arch, respectively. In postoperative events, there was no statistically significant difference between the groups (P ¼ .57 for renal failure, P ¼ .057 for transient, nonpermanent neurologic events, P ¼ .496 for sepsis), as for intensive care unit (P ¼ .502) and total in-hospital stay (P ¼ .929). There was one case of postoperative mortality in each group and both were associated with noncardiac comorbidities. Regarding survival (log-rank ¼ 0.060), freedom from reintervention (log-rank ¼ 0.073), and freedom from recoarctation (log-rank ¼ 0.568), there was no statistically significant difference between the groups. Conclusion: We believe that it is the modified technique that allowed greater mobilization of the aorta and successful repair of hypoplastic arch through thoracotomy, without an increase in paraplegia or other adverse outcomes.

Hace poco, durante mi práctica clínica, me encontré con una paciente de una comunidad indígena y su madre, que no hablaba español. La paciente, para fines de este texto, la que llamé la niña sin voz por su vulnerabilidad y por la falta de opinión que le daba su edad temprana, su falta de lenguaje y un retraso en su neurodesarrollo. Su madre, vulnerable también y con pobre capacidad de comunicación por las barreras de lenguaje, la llamé la madre invisible. El trabajo de comunicación y de comprensión con su madre y las barreras que nos implicaba el idioma, su cultura y la mía, me hizo reflexionar y en el siguiente texto comparto esa reflexión. Así mismo, comparto el proceso emocional y racional que surgió a partir de este caso. Este es un llamado a conservar la humanidad y el humanismo, tanto de las personas con las que nos encontramos, como el nuestro. Así como respetar el proceso humano y sensible que atravesamos nosotros a la hora de tomar decisiones humanas, aunque no siempre sea racional. 

Background: Acute respiratory failure (ARF) is the most frequent cause of cardiorespiratory arrest and subsequent death in children worldwide, therefore several efforts have been made to better understand its etiology and risk factors for further progression (1–4). The aim of this study was to calculate mortality and describe associated factors for severity and mortality in children with acute respiratory failure. Methods: The study was conducted within a prospective multicentric cohort that evaluated the natural history of pediatric acute respiratory failure (ARF). For this analysis three primary outcomes were studied: mortality, invasive mechanical ventilation, and pediatric intensive care unit length of stay. Setting: Pediatric emergency, in-hospital, and critical care services in three hospitals in Bogotá, Colombia, from April 2020 to June 2021. Patients: Eligible patients were children older than 1 month and younger than 18 years of age with respiratory difficulty at time of admission. Patients who developed ARF were followed at time of ARF, 48 hours later, at time of admission and at 30 and 60 days after discharge. Measurements and main results: Out of a total of 685 eligible patients, 296 developed ARF for a calculated incidence of ARF of 43.2%. Of the ARF group, ninety patients (30.4%) needed orotracheal intubation, for a mean of 9.57 days of ventilation (interquartile range = 3.00–11.5). Incidence of mortality was 6.1% (n= 18). The associated factors for mortality in ARF were a history of a neurologic comorbidity and a higher fraction of inspired oxygen at ARF diagnosis. For PICU length of stay associated factors were age between 2 and 5 years of age, exposure to smokers, and respiratory comorbidity. Finally, for mechanical ventilation, the risk factors were obesity and being unstable at admission. Conclusions: ARF is a common cause of morbidity and mortality in children. Understanding the factors associated with greater mortality and severity of ARF might allow earlier recognition and initiation of prompt treatment strategies.