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*

Cardiac resynchronization therapy (CRT) is a new treatment for refractory heart failure. However, most patients with heart failure treated with CRT are adults, middle-aged or older with idiopathic or ischemic dilated cardiomyopathy. We treated a 12-year-old boy, who was transferred after cardiac arrest, with dilated cardiomyopathy, left bundle-branch block, and ventricular tachycardia. We performed cardiac resynchronization therapy with a defibrillator (CRT-D). After CRT-D, left ventricular ejection fraction improved from 22% to 44% assessed by echocardiogram 1 year postoperatively. On electrocardiogram, QRS duration was shortened from 206 to 144 ms. The patient's clinical symptoms also improved. For pediatric patients with refractory heart failure and ventricular arrhythmia, CRT-D could be indicated as an effective therapeutic option.
Adult ; Arrhythmias, Cardiac* ; Bundle-Branch Block ; Cardiac Resynchronization Therapy* ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Child* ; Defibrillators ; Electrocardiography ; Heart Arrest ; Heart Failure* ; Heart* ; Humans ; Male ; Stroke Volume ; Tachycardia, Ventricular

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

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