A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Cannot extubate a newborn patient after an arterial switch operation? check major aortopulmonary collaterals!

Abstract The standard treatment of transposition of the great arteries is the arterial switch operation (ASO). Despite successful surgical correction, patients cannot tolerate extubation after the operation. Major aortopulmonary collaterals (MAPCAs) are one of the rare causes of prolonged mechanical ventilation due to significant hemodynamic effects. We report a 28-day-old newborn with transposition of the great arteries and a ventricular septal defect (VSD) who underwent ASO and VSD closure. After postoperative extubation failed twice, four large MAPCAs were revealed during heart catheterization. After transcatheter closure of these four MAPCAs, the patient was extubated and discharged 27 days after the procedure.

Surgical strategies and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: experience of a single tertiary center

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

Late arterial switch surgery under ecmo support in a patient with transposition of the great arteries with intact ventricular septum: a case report

Abstract A 30-month-old male patient with transposition of the great arteries with intact ventricular septum (TGA/IVS) is presented. Arterial switch operation (ASO) was performed in the light of echocardiographic and angiographic findings. The patient remained under extracorporeal membrane oxygenation support for seven days postoperatively, and his cardiac functions returned to normal at the postoperative 10th day. He was discharged at the postoperative 20th day. The present case, which presents one of the most advanced ages at operation for TGA/IVS among previously reported cases, is used to discuss late ASO in this study.

The evaluation of nosocomial infections in pediatric patients with extracorporeal membrane oxygenation support

Abstract Introduction: Extracorporeal membrane oxygenation (ECMO) has become a standard technique over the past few decades in intensive care unit (ICU). Objective: A review of pediatric patients who received ECMO support in the pediatric cardiac ICU was conducted to determine the incidence, risk factors and causal organisms related to acquired infections and assess the survival rates of ECMO patients with nosocomial infections. Methods: Sixty-six patients who received ECMO support in the pediatric cardiac ICU between January 2011 and June 2014 were included in the study. Demographic, echocardiographic, hemodynamic features and surgical procedures were reviewed. Results: Sixty-six patients received a total of 292.5 days of venoarterial ECMO support. Sixty were postoperative patients. Forty-five patients were weaned from ECMO support with an ECMO survival rate of 68.2%. The rate of infection was 116.2/1000 ECMO days. Prolonged ICU stay, duration of ventilation and ECMO were found associated with development of nosocomial infection and only the duration of ECMO was an independent risk factor for nosocomial infections in ECMO patients. Conclusion: The correction of the underlying process leading to ECMO support and shortening the length of ECMO duration together with stricter application of ECMO indications would improve the infection incidence and hospital surveillance of the patient group.

The efficacy of thoracic ultrasonography in postoperative newborn patients after cardiac surgery

Abstract Objective: In this study, the efficacy of thoracic ultrasonography during echocardiography was evaluated in newborns. Methods: Sixty newborns who had undergone pediatric cardiac surgery were successively evaluated between March 1, 2015, and September 1, 2015. Patients were evaluated for effusion, pulmonary atelectasis, and pneumothorax by ultrasonography, and results were compared with X-ray findings. Results: Sixty percent (n=42) of the cases were male, the median age was 14 days (2-30 days), and the median body weight was 3.3 kg (2.8-4.5 kg). The median RACHS-1 score was 4 (2-6). Atelectasis was demonstrated in 66% (n=40) of the cases. Five of them were determined solely by X-ray, 10 of them only by ultrasonography, and 25 of them by both ultrasonography and X-ray. Pneumothorax was determined in 20% (n=12) of the cases. Excluding one case determined by both methods, all of the 11 cases were diagnosed by X-ray. Pleural effusion was diagnosed in 26% (n=16) of the cases. Four of the cases were demonstrated solely by ultrasonography, three of them solely by X-ray, and nine of the cases by both methods. Pericardial effusion was demonstrated in 10% (n=6) of the cases. Except for one of the cases determined by both methods, five of the cases were diagnosed by ultrasonography. There was a moderate correlation when all pathologies evaluated together (k=0.51). Conclusion: Thoracic ultrasonography might be a beneficial non-invasive method to evaluate postoperative respiratory problems in newborns who had congenital cardiac surgery.

Evaluation of anomalous coronary arteries from the pulmonary artery

Abstract Objective: This study evaluated clinical and diagnostic findings, treatment methods, and follow-up of cases of anomalous coronary arteries from the pulmonary artery. Methods: The study included all cases diagnosed with anomalous coronary arteries from the pulmonary artery between January 2012 and January 2016. Data from patients’ demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, intensive care unit stay, and follow-up were evaluated. Results: The study included 12 patients (8 male, 4 female), 10 with anomalous left coronary artery from the pulmonary artery (ALCAPA) and 2 with anomalous right coronary artery from the pulmonary artery (ARCAPA). Median age at diagnosis was 4 months (range, 1 month - 10 years old) and median weight was 5.5 kg (range, 3-30 kg). The most common complaints were murmur (n=7) and respiratory distress (n=5). In 4 cases, the initial diagnosis was dilated cardiomyopathy. Electrocardiographs were pathologic in all cases. Echocardiographic examination revealed medium to severe mitral valve regurgitation in 4 cases and reduced (< 40%) ejection fraction in 6 patients. Of the 12 patients, 8 underwent direct implantation of the left coronary artery into the aorta, 2 underwent implantation of the right coronary artery into the aorta, and the remaining 2 underwent a Takeuchi procedure. There were no early mortalities. Median hospital stay was 20 days (range, 5-35 days). Median follow-up duration was 18 months (range, 5-36 months), and no cases required further surgery during follow-up. Conclusions: Anomalous coronary arteries from the pulmonary artery can be successfully repaired providing there is early diagnosis and effective, appropriate intensive care unit follow-up. Therefore, coronary artery origins should be evaluated carefully, especially in cases with dilated cardiomyopathies.

Major intrahepatic veno-venous fistula after fontan operation treated by transcatheter implantation of amplatzer septal occluder through internal jugular vein

Abstract Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay. Three weeks after the first intervention, a second procedure was performed to retrieve the first device and to close the fistula distally. Multiple attempts with different types of gooseneck snares and a bioptome catheter failed to retrieve the first device, so a telescopic method was used to re-screw it. Using a Mullins long sheath and delivery sheath, the delivery cable was manipulated to fit into the slot of the end screw, and the cable was rotated gently in a clockwise direction to re-screw the device. Then, another Amplatzer septal occluder was placed at the distal end of the fistula. In conclusion, distal transcatheter occlusion of intrahepatic veno-venous fistulas might lead to better clinical outcomes in selected patients. Amplatzer septal occluder device can be retrieve without any complication within three weeks.