Background: The left pulmonary artery (LPA) may be kinked and stenotic, especially in tetralogy of Fallot, because of ductal tissue and anterior deviation of the conal septum. If LPA stenosis is not effectively treated during total correction, surgical angioplasty is occasionally performed. However, whether pulmonary artery (PA) angioplasty in adolescents or adults improves perfusion in the ipsilateral lung remains unclear. Methods: This retrospective review enrolled patients who underwent PA angioplasty for LPA stenosis between 2004 and 2019. Among patients who underwent a lung perfusion scan (LPS) or cardiac magnetic resonance imaging (cMRI) pre- and post-pulmonary angioplasty, those aged >13 years with <40% left lung perfusion (p-left) in the pre-angioplasty study were included. Preoperative and postoperative computed tomography, LPS, and cMRI data were collected. The perfusion ratio was analyzed according to the LPA’s anatomical characteristics. Results: Seventeen adolescents and 16 adults (≥18 years old) were finally included (median age, 17 years). The most common primary diagnosis was tetralogy of Fallot (87.9%). In all patients, LPA angioplasty was performed concomitantly with right ventricular outflow tract reconstruction. No patients died. Preoperative p-left was not significantly different between adolescents and adults; however, adolescents had significantly higher postoperative p-left than adults. P-left significantly increased in adolescents, but not in adults.Seven patients had significant stenosis (z-score <-2.0) confined only to the proximal LPA and demonstrated significantly increased p-left. Conclusion PA angioplasty significantly increased ipsilateral lung perfusion in adolescents. If focal stenosis is confined to the proximal LPA, PA angioplasty may improve ipsilateral lung perfusion, regardless of age.

Bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH) are potentially fatal complications in prematurely born infants. Extracorporeal membrane oxygenation (ECMO) may be a life-saving option for managing infants with BPD and PH. We present 2 patients who were successfully weaned off mechanical ventilators (MVs) through the application of ECMO. The patients were transferred to our institution after receiving MV care for 8 and 10 months, respectively, for BPD and PH. We were able to remove the patients from MVs after a period of ECMO-mediated lung rest. Although more research is required to determine specific criteria for ECMO use in patients with BPD and PH, our clinical experiences may contribute to the early application of ECMO in MV-dependent patients.

Laundry detergent pod (LDP) exposure has been reported to be fatal in children younger than 2 years, leading to respiratory or central nervous system depression. While gastrointestinal irritation is the most common symptom, there are reported cases of severe acidosis with respiratory depression or pneumonia, resulting in mortality. To our best knowledge, there is no report on a case of LDP exposure presenting with acute respiratory distress syndrome requiring extracorporeal membrane oxygenation support. Here, we present a case of a child with severe acute respiratory distress syndrome following LDP exposure, who was successfully treated with veno-pulmonary extracorporeal membrane oxygenation and steroids.

Cardiogenic shock is an acute circulatory failure due to compromised myocardial contractility associated with congenital heart diseases and cardiomyopathies, such as myocarditis. In this article, the authors present a 3-step overview of cardiogenic shock diagnosis and management to restore tissue oxygen delivery. The first step is early recognition of nonspecific signs of the shock. The second step is medical management, monitoring, and repeated assessment. In addition to conventional parameters, biomarkers may be useful to monitor the shock. The final step is mechanical circulatory support, such as ventricular assist devices, for children with the refractory shock. We also briefly describe the shock in multisystem inflammatory syndrome in children with coronavirus disease 2019.

Cardiogenic shock is an acute circulatory failure due to compromised myocardial contractility associated with congenital heart diseases and cardiomyopathies, such as myocarditis. In this article, the authors present a 3-step overview of cardiogenic shock diagnosis and management to restore tissue oxygen delivery. The first step is early recognition of nonspecific signs of the shock. The second step is medical management, monitoring, and repeated assessment. In addition to conventional parameters, biomarkers may be useful to monitor the shock. The final step is mechanical circulatory support, such as ventricular assist devices, for children with the refractory shock. We also briefly describe the shock in multisystem inflammatory syndrome in children with coronavirus disease 2019.

Background: The double-lumen cannula (DLC) has begun to be used worldwide for venovenous (VV) extracorporeal membrane oxygenation (ECMO). We aimed to examine whether the DLC could be an effective tool in the treatment of pediatric respiratory failure in Korea. Methods: We reviewed the records of patients weighing under 15 kg who underwent ECMO due to respiratory failure between January 2017 and December 2018. Outcomes of ECMO using a DLC and conventional ECMO using central method or 2 peripheral cannulas were compared. Results: Twelve patients were treated with ECMO for respiratory failure. Among them, a DLC was used in 5 patients, the median age of whom was 3.8 months (interquartile range, 0.1–49.7 months). In these patients, the median values of pH, partial pressure of carbon dioxide, and partial pressure of oxygen were 7.09, 74 mm Hg, and 37 mm Hg before ECMO and corrected to 7.31, 44 mm Hg, and 85 mm Hg, respectively, after ECMO cannulation. Median blood flow rate in the patients treated with ECMO using a DLC was slightly higher than that in the conventional ECMO group, but this difference was not statistically significant (86.1 mL/kg/min and 74.3 mL/kg/min, respectively; p=1.00). One patient from the DLC group and 3 patients from the conventional group were weaned off ECMO. Conclusion VV ECMO using a DLC provided adequate oxygenation, ventilation, and blood flow rate in Korean pediatric patients with respiratory failure. Further prospective and randomized studies are warranted.

No abstract available.
Arteries*

BACKGROUND AND OBJECTIVES: A blood transfusion is almost inevitable in neonatal cardiac surgery. This study aimed to assess the feasibility of using autologous cord blood for a cardiopulmonary bypass (CPB) priming as an alternative to an allo-transfusion in neonatal cardiac surgery. SUBJECTS AND METHODS: From January 2012 to December 2014, cord blood had been collected during delivery after informed consent and was stored immediately into a blood bank. Eight neonatal patients had their own cord blood used for CPB priming during cardiac surgery. RESULTS: All patients underwent surgery for their complex congenital heart disease. The median age and body weight at surgery was 11 days (from 0 to 21 days) and 3.2 kg (from 2.2 to 3.7 kg). The median amount and hematocrit of collected cord blood was 72.5 mL (from 43 to 105 mL) and 48.7% (from 32.0 to 51.2%). The median preoperative hematocrit of neonates was 36.5% (from 31.0 to 45.0%); the median volume of CPB priming was 130 mL (From 120 to 140 mL). Seven out of eight patients did not need an allo-transfusion in CPB priming and only one neonate used 20 mL of packed red blood cells in CPB priming to obtain the target hematocrit. CONCLUSION: Autologous cord blood can be used for CPB priming as alternative to packed red blood cells in neonatal congenital cardiac surgery in order to reduce allo-transfusion.
Blood Banks ; Blood Transfusion ; Body Weight ; Cardiopulmonary Bypass* ; Erythrocytes ; Fetal Blood* ; Heart Defects, Congenital ; Hematocrit ; Humans ; Infant, Newborn ; Informed Consent ; Thoracic Surgery*

Persistent fifth aortic arch (PFAA) is a rare congenital anomaly of the aortic arch frequently associated with other cardiovascular anomalies, such as tetralogy of Fallot and aortic arch coarctation or interruption. We report the case of a neonate with PFAA with coarctation who successfully underwent surgical repair.
Aorta, Thoracic* ; Humans ; Infant, Newborn ; Tetralogy of Fallot

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.
Arrhythmias, Cardiac ; Cesarean Section ; Delivery, Obstetric ; Ebstein Anomaly ; Female ; Fontan Procedure* ; Gestational Age ; Heart Failure ; Heart Ventricles* ; Humans ; Korea ; Neonatology ; Pregnancy* ; Risk Assessment