The effectiveness of the flip technique in vertical ductal stenting.

Background: In this study, the flip technique was compared with the classical method in terms of procedural success and procedure time during ductal stent implantation (DSI) via the carotid artery in patients whose pulmonary blood flow is dependent on the vertical type of ductus arteriosus (DA). Methods: Between January 2019 and June 2023, 40 patients (24 males, 16 females; mean age: 15.9±15.4 days; range, 1 to 68 days) with vertical ductus-dependent pulmonary circulation who underwent patent DA stent implantation via the carotid artery were included in the study. Patients were divided into two groups: those who underwent the flip technique (Group 1) and those who did not undergo the flip technique (Group 2). Data were retospectively compiled by reviewing patient files and catheter images. Results: Demographic findings were similar in the groups. The distribution of the DA in terms of tortuosity index was also similar in the groups. The procedure was successful in 18 (90%) patients in Group 1 and 20 (100%) patients in Group 2. There was no procedure-related mortality in both groups. The frequency of procedure-related complications was similar. Procedure duration was 53.6±18.4 min in Group 1 and 41.5±9.1 min in Group 2; the difference was significantly lower in Group 2 (p=0.028). The shorter follow-up in Group 2 was attributed to the flip method starting to be used later in the clinic. During follow-up, stent dilatation was required in two patients in Group 1 and one patient in Group 2, and a second stent implantation was needed in one patient in Group 2. No significant difference was observed between the two groups in terms of reintervention. Conclusion: The success rate of DSI using the carotid artery access is high with both the classical and the flip method in patients with vertical DA. However, the use of the flip technique could be preferred in terms of operator habituation, ergonomic use of the catheter, guidewires, and shorter procedure time.

Multicenter Experience for Early and Mid-Term Outcome of MyVal Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12-19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31-63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10-53 mmHg) pre-procedure to 10 mmHg (IQR 5-16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5-44 min). The large sizes-mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120-300 mL). Patients were followed for a median period of 360 days (IQR 164-525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Comparison of ultrasound guidance and palpation technique for femoral artery catheterization in children undergoing cardiac surgery.

OBJECTIVE: The aim of this study was compare the palpation technique and ultrasound-guidance for femoral artery catheterization in pediatric patients undergoing surgery for congenital heart disease. MATERIALS AND METHODS: This prospective and randomized controlled study included American Society of Anesthesiologists III-IV 40 children who underwent congenital heart surgery. The patients were divided into two groups; ultrasound-guided catheterization group and palpation-guided catheterization group. Demographic and clinical characteristics of the patients, access time, success rate, number of attempts, first-attempt success, number of trials, and failed cannulations were recorded. RESULTS: The diameter of the femoral artery was significantly shorter, access time and numbers of trials were significantly lower, and first-attempt success rate was significantly higher in the US group. The complication rate was significantly higher in the P group. The number of failed catheterization was higher in the P group. Total cost required for the procedure was significantly lower in the US group. CONCLUSION: We found that ultrasound-guided arterial catheterization increases the success rate and the number of successful catheterizations, while reducing the overall procedure time, incidence of complications, and cost. Therefore, we believe that the use of ultrasound guidance in arterial catheterization in pediatric cardiac surgery would be a better choice.

OBJETIVO: El objetivo de este estudio fue comparar la técnica de palpación y ecoguiado para el cateterismo de la arteria femoral en pacientes pediátricos operados de cardiopatías congénitas. MATERIALES Y MÉTODOS: Este estudio prospectivo, aleatorizado y controlado incluyó a 40 niños ASA III-IV que se sometieron a cirugía cardíaca congénita. Los pacientes se dividieron en 2 grupos; Grupo de cateterismo guiado por ecografía y grupo de cateterismo guiado por palpación. RESULTADOS: El diámetro de la arteria femoral fue significativamente más corto, el tiempo de acceso y el número de intentos fueron significativamente menores y la tasa de éxito del primer intento fue significativamente mayor en grupo estadounidense. La tasa de complicaciones fue significativamente mayor en el grupo P. El número de cateterismos fallidos fue mayor en el grupo P. El costo total requerido para el procedimiento fue significativamente menor en el grupo de EE. CONCLUSIONES: Encontramos que el cateterismo arterial guiado por ultrasonido aumenta la tasa de éxito y el número de cateterismos exitosos, al tiempo que reduce el tiempo total del procedimiento, la incidencia de complicaciones y el costo. Por tanto, creemos que el uso de guía ecográfica en cateterismo arterial en cirugía cardiaca pediátrica sería una mejor opción.

Comparison of Three Different Multiple Organ Dysfunction Scores for Predicting Mortality after Neonatal Cardiac Surgery.

Infants who undergo cardiac surgery frequently have complications that may advance to multiple organ failure and result in mortality. This study aims to compare three different multiple organ dysfunction scoring systems: the Neonatal Multiple Organ Dysfunction (NEOMOD) score, the modified NEOMOD score, and the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score in predicting postoperative 30-day mortality in neonates undergoing cardiac surgery. This retrospective cohort study was conducted between January 2019 and February 2021 in a single unit on neonates operated on due to congenital heart disease in the first 28 days of life. Patients who underwent off-pump surgeries were excluded from the study. The NEOMOD, modified NEOMOD, and PELOD-2 scores were calculated for each of the first 3 days following surgery. A total of 138 patients were included. All scores had satisfactory goodness-of-fit and at least good discriminative ability on each day. The modified NEOMOD score consistently demonstrated the best prediction among these three scores after the first day, reaching its peak performance on day 2 (area under curve: 0.824, CI: 0.75-0.89). Our findings suggest that NEOMOD and modified NEOMOD scores in the first 72 h could potentially serve as a predictor of mortality in this population.

Non-Conduit Repair of Truncus Arteriosus.

INTRODUCTION: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. METHODS: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. RESULTS: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). CONCLUSION: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

Non-Conduit Repair of Truncus Arteriosus

ABSTRACT Introduction: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. Methods: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. Results: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). Conclusion: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

A Better Way for Ductal Stenting in Patients with Duct-Dependent Pulmonary Flow and Vertical and Tortuous Patent Ductus Arteriosus.

BACKGROUND: In this study, we aimed to compare the femoral route and the carotid artery route in terms of procedural success of ductal stent implantation in patients with ductdependent pulmonary blood flow. METHODS: The study included 51 patients with duct-dependent pulmonary circulation who underwent ductal stent implantation upon their admission to our clinic between July 2017 and March 2021. In total, 23 patients (group I) underwent ductal stent implantation via the femoral route, while the remaining 28 (group II) underwent the procedure via the carotid artery. The groups were compared in terms of procedural success, time, post-procedural blood pH, lactate levels, and complications. RESULTS: Duct morphology was observed in group 1 as follows: type 1 in 12 patients, type 3 in 8, type 2 in 2, and type 6 in 1 patient. In group 2, 26 patients had type 3, 1 had type 2, and 1 had type 6. The tortuosity index of the patients in group 1 was 1 in 8 patients, 2 in 8 patients, and 3 in 7 patients, while in group 2, it was 1 in 5 patients, 2 in 15 patients, and 3 in 8 patients. The success rate was 69.6% (16/26) in group I and 93.5% (29/31) in group II (P=.030). The cumulative success rate was 88.2% (45/51). The procedural durations were 78.2 ± 34.1 and 52.1 ± 22.0 minutes in group I and group II, respectively (P=.002). The mean blood pH values upon the completion of the procedure were 7.26 ± 0.1 and 7.33 ± 0.0 in group I and group II, respectively (P=.038). The mean post-procedural lactate levels were 2.8 mmol/L and 2.3 mmol/L in group I and group II, respectively (P=.038). The 2 groups did not show any differences in terms of procedural complications. CONCLUSION: The carotid artery route can be preferred, especially in vertical and tortuous ductus arteriosus, as it is associated with a high success rate and a short procedural time, as well as a better metabolic condition after the procedure.

Ligation of patent ductus arteriosus through left anterior mini-thoracotomy in preterm infants.

OBJECTIVE: Patent ductus arteriosus is an important cause of morbidity and mortality, especially in very low birth weight infants. The aim of the study is to report our single-centre short-term results of preterm patients who underwent ligation through left anterior mini-thoracotomy . METHODS: Data of 27 preterm infants operated by the same surgeon who underwent Patent ductus arteriosus (PDA) closure with left anterior mini-thoracotomy technique between November 2020 and January 2022 at a single institution were reviewed. The patients were divided into two groups according to their weight at the time of surgery. Data on early postoperative outcomes and survival rates after discharge were collected. RESULTS: Twenty-seven patients with a mean (±SD) gestational age of 25.8 (±2.0) weeks and a mean birth weight of 1027 (±423) g were operated using left anterior mini-thoracotomy technique. The lowest body weight was 480 g. Complications such as bleeding, abnormal healing of incision, or pneumothorax were not seen. There were 8 mortalities after the operation (29,6 %). The causes of the deaths were sepsis, necrotising enterocolitis, hydrops fetalis, hepatoblastoma, and intracranial bleeding. There was no statistically significant difference in the rates of complication between the groups. CONCLUSIONS: Left anterior mini-thoracotomy technique can be performed as the first choice when transcatheter intervention cannot be applied in preterm infants. It provides easy access to the PDA, a good exposure, minimal contact with the lungs, good cosmetic results in early and mid-term and shortens the operation time, especially in very low birth weight preterm babies. However, early ligation may be helpful to minimise the complications related to PDA.

Experiences of Two Centers in Percutaneous Ventricular Septal Defect Closure Using Konar Multifunctional Occluder.

BACKGROUND: Transcatheter closure of perimembraneous ventricular septal defect still poses a challenge due to the adjacent structures of the tricuspid and aortic valves and the risk of atrioventricular block. We report our experience at 2 centers using the KONAR-MF (multifunctional occluder) ventricular septal defect device, which gained its CE mark in May 2018. METHODS: A retrospective study was carried out on all patients who underwent transcatheter ventricular septal defect closure with the KONAR-MF (multifunctional occluder) ventricular septal defect device at 2 centers. RESULTS: A total of 47 patients were identified. The median age and weight of the patients were 25.8 months and 11 kg. The ventricular septal defects that were closed in 5 cases were post-operative hemodynamically significant residual ventricular septal defects. Forty-eight devices were used in the 47 cases. As for the location of the ventricular septal defect, 40/48 (83.33%) ventricular septal defects were perimembranous and 8/48 (16.66%) were muscular. The percutaneous device closure was successful in 46 procedures (95.8%). Device embolization was observed in 2 patients, and a significant residual shunt was observed in 2 cases. In the follow-up, there was no enhancement in the residual shunt in the remaining cases. Temporary atrioventricular block occurred in 2 cases during the procedure and improved after long sheath withdrawal. CONCLUSION: Soft, flexible, and low-profile KONAR-MF (multifunctional occluder) occluders ensure easy and safe implantation, and small sheath sizes allow for their use in small infants. Although near and mid-term follow-ups did not indicate any permanent atrioventricular block, a larger sample of patients and a longer follow-up period is necessary to understand long-term outcomes.

Is arterial switch operation possible with neonatology-focused intensive care unit modality.

BACKGROUND: In this study, we aimed to examine the feasibility of arterial switch operation and its perioperative management with neonatology-focused intensive care modality in a region of Turkey where the birth rate and the number of asylum seekers who had to leave their country due to regional conflicts are high. METHODS: Between December 2017 and June 2020, a total of 57 patients (48 males, 9 females; median age: 12.2 days; range, 2 to 50 days) who were diagnosed with transposition of the great arteries in our clinic and underwent arterial switch operation were retrospectively analyzed. All patients were followed by the neonatologist in the neonatal intensive care unit during the preoperative and postoperative period. RESULTS: Thirty-eight (66.7%) patients had intact ventricular septum, 16 (28.1%) had ventricular septal defect, two (3.5%) had coarctation of the aorta, and one (1.7%) had Taussig-Bing anomaly. Coronary artery anomaly was present in 14 (24.5%) patients. The most common complications in the intensive care unit were renal failure requiring peritoneal dialysis in seven (12.3%) patients, supraventricular tachyarrhythmia in six (10.5%) patients, and eight (14%) patients left their chests open. The median length of stay in intensive care unit was 13.8 (range, 9 to 25) days and the median length of hospital stay was 24.5 (range, 16 to 47) days. The overall mortality rate for all patients was 12.3% (n=7). The median follow-up was 8.2 months. A pulmonary valve peak Doppler gradient of ≥36 mmHg was detected in five patients (8.7%) who were followed, and these patients were monitored by providing medical treatment. None of the patients needed reoperation or reintervention. CONCLUSION: We believe that arterial switch operation, one of the complex neonatal cardiac surgery, can be performed with an acceptable mortality and morbidity rate with the use of neonatology-focused intensive care modality, which is supported by pediatric cardiology and pediatric cardiac surgery.

Transcatheter Treatment of a Symptomatic Giant Iliac Artery Aneurysm with a BeGraft Peripheral Stent in a 2-Year-Old Child.

A 2-year-old girl presented with severe abdominal pain due to a giant iliac artery aneurysm. We embolized the internal iliac artery with microcoils and then eliminated the aneurysmal sac using a BeGraft peripheral stent without any complications. To the best of our knowledge, there are no previous reports of a transcatheter giant iliac artery intervention.

Problems of the neonates with congenital heart disease requiring early interventions: a regional report.

AIM: In this study, it was aimed to determine the problems of the neonates who were diagnosed with congenital heart disease requiring early intervention in our hospital. MATERIAL AND METHODS: The files of the newborn babies with congenital heart disease requiring early intervention who were admitted to the neonatal intensive care unit of our hospital between January 2011 and January 2013 were evaluated retrospectively. In all cases, echocardiography and ''Score for Neonatal Acute Physiology-II" (SNAP-II) scoring were performed within the first day of admission. The data were interpreted using Number Cruncher Statistical System 2007 software. The statistical significance was set at p<0.05. RESULTS: A total of 83 babies were included in the study. Forty six of the patients were male (55%), and 37 (45%) were female. Sixty eight percent of the patients were referred from the neighboring provinces and 32% were transferred from the centers within the city. The age range was between 0 and 28 (5.6±6.4 day) days. The SNAP-II scores upon admission ranged between 0 and 90 (mean: 20±20.3). None of the patients was diagnosed prenatally. The most common diagnoses included transposition of the great arteries (33.7%) and pulmonary atresia (19.3%). Nineteen (22%) patients were lost in the neonatal intensive care unit. There was a significant relationship between the mortality and the SNAP-II scores (p=0.0001) and use of vasopressors (p=0.004). The diagnosis, gender, use of alprostadil and age were not related to mortality. Three patients were discharged following planning of elective surgery and 60 patients were referred to a tertiary center by air ambulance. CONCLUSIONS: The results of our study indicated that prenatal diagnosis could not be made in neonates with congenital heart disease requiring intervention in our region. The mortality rates of these patients were related to the severity of the clinical status at presentation rather than to the age, gender and type of congenital heart disease. The mortality was much higher in the patients who developed circulatory failure. Most of the patients who survived were sent by air ambulance to the centers where the intervention was to be performed.

Mutations in BTD gene causing biotinidase deficiency: a regional report.

Biotinidase deficiency is an autosomal recessive inborn error of biotin metabolism. Children with biotinidase deficiency cannot cleave biocytin and, therefore, cannot recycle biotin. Untreated individuals become secondarily biotin deficient, which in turn results in decreased activities of the biotin-dependent carboxylases and the subsequent accumulation of toxic metabolites causing clinical symptoms. Biotinidase deficiency is characterized by neurological, cutaneous manifestations and metabolic abnormalities. The worldwide incidence of profound biotinidase deficiency has been estimated at 1:112,271. The human biotinidase gene is located on chromosome 3p25 and consists of four exons with a total length of 1629 base pairs. To date, more than 100 mutations in the biotinidase gene known to cause biotinidase deficiency have been reported. The vast majority of mutations are homozygous or compound heterozygous. Finding known mutations can be correlated with the biochemical enzymatic results. This report summarizes the demographic features of patients identified as biotinidase deficient from August of 2012 through August of 2013 and mutation analysis results for 20 cases in the southeast region of Turkey.

Transient nephrogenic diabetes insipidus caused by fetal exposure to haloperidol.

Haloperidol is commonly used in the treatment of psychiatric disorders. Data from animal experiments indicate haloperidol is not teratogenic, but is embryotoxic in high doses. For the first time, we report a neonate with transient nephrogenic diabetes insipidus (DI) caused by fetal exposure to haloperidol. The magnitude of risk associated with the use of haloperidol during pregnancy appears to be small, but nephrogenic DI secondary to haloperidol is a serious condition with the risk of hypernatremic dehydration. Haloperidol can have adverse effects on the fetus and newborn infant, that's why one should prevent the use of haloperidol during pregnancy and lactation.

Effect of mode of delivery on postnatal decline in pulmonary artery pressure.

OBJECTIVE: To evaluate the effect of the mode of delivery on the course of pulmonary artery pressure (PAP) in neonates. METHODS: Eighty healthy and term neonates delivered in the maternity wards of Dunya Maternity Hospital, Camlica Maternity Center, and Sultan Maternity Hospital in Diyarbakir, Turkey from June to August 2009 were included in the study. Tricuspid regurgitation (TR) was determined using color Doppler echocardiography, and the measurements were carried out with continuous wave technique on the first, third, and the fifth days of birth. The babies who had no TR flow on the first, third, or the fifth day were excluded, and the study was completed with 42 cases. Twenty-two of them were delivered by cesarean section (CS), and 20 by vaginal delivery. The peak flow velocity of TR was used in the calculation of the right ventricular pressure, which reflects PAP. RESULTS: In both groups, PAP continued to decline on the third and fifth days. However, in the vaginal delivery group, the decrease between the third and fifth days was statistically insignificant, whereas a statistically significant decline (p=0.01) in the CS group lasted until the fifth day. CONCLUSION: Although the birth method does not create a clinical morbidity on the circulatory system as seen in the respiratory system, it can be stated that the circulatory adaptation of vaginally delivered babies is completed earlier.

Retrograde transcatheter closure of large coronary fistulas with the Amplatzer vascular plug in children.

We report a retrograde approach for the successful closure of large left coronary artery fistulas in the cases of two children using the Amplatzer vascular plug. This method simplified the procedure by eliminating the need for making an arteriovenous loop.