Background: Red blood cell (RBC) transfusion improves cardiorespiratory status of preterm infants by increasing circulating hemoglobin, improving tissue oxygenation, and reducing cardiac output. However, RBC transfusion itself has also been suggested to negatively affect short-term outcomes such as intraventricular hemorrhage (IVH), bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP), and necrotizing enterocolitis (NEC) in premature infants.Purpose: This study aimed to analyze the relationship between RBC transfusion and short-term outcomes in very low birth weight (VLBW) infants (birth weight, <1,500 g).Methods: We retrospectively reviewed the medical records of VLBW infants admitted to the Soonchunhyang University Bucheon Hospital between October 2010 and December 2017. Infants who died during hospitalization were excluded. The infants were divided into 2 groups according to RBC transfusion status. We investigated the relationship between RBC transfusion and short-term outcomes including BPD, ROP, NEC, and IVH.Results: Of the 250 enrolled VLBW infants, 109 (43.6%) underwent transfusion. Univariate analysis revealed that all shortterm outcomes except early-onset sepsis and patent ductus arteriosus were associated with RBC transfusion. In multivariate analysis adjusted for gestational age, birth weight and Apgar score at 1 minute, RBC transfusion was significantly correlated with BPD (odds ratio [OR], 5.42; <i>P</i><0.001) and NEC (OR, 3.40; <i>P</i>= 0.009).Conclusion: RBC transfusion is significantly associated with adverse clinical outcomes such as NEC and BPD in VLBW infants. Careful consideration of the patient’s clinical condition and appropriate guidelines is required before administration of RBC transfusions.

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Background and ObjectivesWe studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis.Subjects and MethodsWe reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014.ResultsOf the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age.ConclusionPatients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.