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Objectives:This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery.Materials and Methods:Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe.Results:Three patients had atrial flutter, two had Wolf–Parkinson–White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein’s anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement.Conclusion:Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right–left or biatrial maze procedure is mandatory for operational success.

Objective:Focal atrial tachycardia accounts for up to 10–15% of supraventricular tachycardiasubstrates in patients < 30 years. In this study, we aimed to demonstrate the outcome of transcatheter ablation procedures performed through three-dimensional electroanatomic mapping systems using minimal fluoroscopy in a paediatric cohort with focal atrial tachycardia.Methods:Forty-nine consecutive patients with focal atrial tachycardia who underwent an electrophysiologic study and a transcatheter ablation procedure in our hospital from September 2014 to February 2020 were included into the study.Results:The mean weight of the patients was 48.63 ± 15.4 kg, and the mean age was 14.56 ± 3.5 (5.5–18.4) years. The tachycardia was defined as incessant in 26 patients. Thirteen patients had left ventricular systolic dysfunction with a mean left ventricular ejection fraction of 38.47 ± 12.4% on echocardiography. The mean procedure time was 148.7 ± 94.5 minutes. Transseptal puncture and thus fluoroscopy were required in nine patients. The mean fluoroscopy time was 4.51 ± 5.9 minutes. No fluoroscopy was needed in ablations performed in the right atrium. The acute success rate of the ablation procedures was 97.9%. The mean follow-up period was 50.71 ± 23.5 months. Recurrence was noted in two patients (4.2%).Conclusion:The outcomes of three-dimensional electroanatomic mapping-guided transcatheter ablation procedures are promising with high acute success, low recurrence and complication rates in children with focal atrial tachycardia. The use of fluoroscopy can be significantly decreased with three-dimensional mapping systems in this group of patients.

Objective:Fascicular tachycardia is a common form of sustained idiopathic left ventricular tachycardia. This study aimed to achieve successful results with catheter ablation procedures performed through three-dimensional electroanatomic mapping using near-zero fluoroscopy in fascicular tachycardia patients.Methods and results:In this study, we included 33 consecutive children with fascicular tachycardia, for whom we performed a transcatheter radiofrequency ablation procedure using the EnSite® system. Activation mapping was performed in all patients during tachycardia, and the earliest presystolic purkinje potentials were the target site for radiofrequency lesions.Results:Twenty-five patients were male, and eight were female. The mean weight of the patients was 39.6 ± 10.4 kg, and the mean age was 13.6 ± 2.5 years. The mean procedure time was 121.3 ± 44.3 minutes. The mean follow-up period was 18.4 ± 6.5 months. No fluoroscopy was needed in 30 patients. The mean fluoroscopy time in the remaining patients was 166.6 ± 80 seconds. All of the patients had left posterior fascicular tachycardia except for one who had left anterior fascicular tachycardia. The acute success rate was perfect (100%). No patients developed left bundle branch block or complete atrioventricular block. Recurrence developed in one patient.Conclusion:We suggest that radiofrequency ablations via an electroanatomic mapping system are quite safe and effective, with high success rates in paediatric patients with fascicular tachycardia. This method has the advantage of avoiding ionising radiation exposure for both the patient and operator, thus reducing the lifetime risk of malignancy in the paediatric population.

Objective To determine the short- and medium-term outcomes of ductal stenting (DS) in patients with functionally univentricular hearts (FUHs) and ductal-dependent pulmonary blood flow. Background Several studies have evaluated the outcomes of DS in a limited number of patients with FUHs. Nonetheless, there is still no consensus regarding the indications for this procedure, and no appropriate patient selection criteria have been devised. Methods From 2005 to 2015, cardiac catheterization for DS was performed in 68 patients with FUHs. Of these patients, 49 had single source pulmonary blood flow from ductus arteriosus. Procedural and follow-up data were evaluated. Results The median weight of the patients was 3.6 kg (2.3–6.8 kg), and the median age was 26 days (3 days–8 months). The technical success rate of the procedure was 95% (65 of 68 patients). Mean oxygen saturation increased from 70% ± 7.6% to 87% ± 4.6% (P

When total correction is not possible in infants who have a cyanotic congenital heart disease, creation of a palliative aortopulmonary shunt is essential. A central aortopulmonary shunt is preferable, because of its technical and hemodynamic advantages. Overcirculation, thrombosis, and stenosis of the shunt are the main postoperative sequelae that necessitate urgent reintervention. Percutaneous transcatheter closure of aortopulmonary shunts can eliminate the need for reoperation and substantially decrease postoperative morbidity and mortality rates. We report our successful transcatheter closures of central aortopulmonary shunts in a 3-month-old infant and a 15-year-old girl, with use of an Amplatzer Duct Occluder II and an Amplatzer Vascular Plug I, respectively. To our knowledge, this is the first report of the transcatheter closure of central aortopulmonary shunts with these 2 devices.

Vasküler ring (halka), trakea ve özefagusta basıya bağlı olarak değişik klinik bulgulara yolaçan aortanın konjenital bir anomalisidir. Sekiz yaşındaki kız hasta bronşiyal astım tanısı ile izlenirken tekrarlayan akciğer enfeksiyonları ve yutma güçlüğü şikayeti olması üzerine çocuk kardiyolojisi polikliniğine yönlendirildi. Hastada sağ aortik arkın dominant formda olduğu vasküler ring anomalisi tespit edildi ve cerrahi düzeltme planlandı. Bu olgu sunumu, astım, tekrarlayan akciğer enfeksiyonları ve özellikle de yutkunma güçlüğü şikayeti olan hastalarda ayrıntılı ekokardiyografik incelemenin önemini vurgulamak amacıyla sunulmuştur.

The closure of a secundum atrial septal defect through the jugular vein in a child with interrupted inferior vena cava with azygos continuation by steerable delivery catheter is described in the present report. The steerable catheter can be used to correct the perpendicular position of the device over the margins of the defect, and is particularly useful in cases of large defects.

Oner T, Balli S, Hekim Yilmaz E, Bulut MO, Sasmazel A, Celebi A. Systolic dysfunction of systemic ventricle in patients who underwent a Fontan operation. Turk J Pediatr 2019; 61: 925-930. The aim of the study was to report the clinical and echocardiographic data of patients who underwent a Fontan operation, and define the group with systolic dysfunction in the systemic ventricle observed during postoperative follow-up. The medical records of 183 patients [mean age: 10.93 ± 5.89 years (range: 2.5-45 years)] who were referred to our center and underwent a Fontan operation were retrospectively reviewed. The clinical, echocardiographic, and postoperative follow-up data of the patients were recorded. Preoperatively, 68 (37.2%) patients experienced pulmonary stenosis, while 41 (22.4%) had pulmonary atresia and 74 (40.4%) had pulmonary hypertension. The most common pathology in patients who were ineligible for biventricular repair was tricuspid atresia, seen in 51 patients (27.9%), followed by double-inlet left ventricle pathologies in 40 patients (21.9%). In total, 38 (20.7%) patients had a biventricular structure; among those with a single ventricular structure, the systemic ventricle involved was the right ventricle in 51 (27.9%) patients and the left ventricle in 94 (51.4%) patients. During follow-up, a total of 31 (16.9%) patients underwent catheterization and ventricular systolic functions were preserved in 168 (91.8%) patients. In Fifteen (8.2%) patients developed systolic dysfunction (ejection fraction < 50%). The mean age of the group developing systolic dysfunction was 15.6 ± 2.63 years (median, 13 years; range: 5-45 years). Of this group, 10 patients had a left ventricular structure of the systemic ventricle, 14 had atrial situs solitus, and 4 had moderate atrioventricular valve insufficiency. Even if the systemic ventricle is in the left ventricular structure, systolic dysfunction in the systemic ventricle develops, especially after the first 10 years, and this makes us think rudimentary ventricle function should also be carefully monitored for intra-univentricular diastolic asynchrony.

We aim to assess the safety, feasibility and efficacy of the new Nit-Occlud ASD-R (NOASD-R) device. From 2014 to 2015, transcatheter closure of atrial septal defect (ASD) using the NOASD-R was performed in 30 consecutive patients. The standard deployment technique as the left upper pulmonary vein approach was used in 25 patients. Right upper pulmonary vein approach was required in five. The median age was 6 years (range 3.5–60 years), and median weight was 21.5 kg (14–79 kg). Implantation was successful in all patients. The median size of devices was 16 mm (12.0–26.0 mm). The mean device size/2D defect diameter ratio was 1.26 ± 0.09 (1.12–1.40). The mean device size/color flow diameter ratio was 1.07 ± 0.06 (range 1.0–1.22). Releasing problem was encountered in three patients. A device-related erosion on the day after the closure was observed in one patient. No further device-related complication (erosion, embolization or dislodgement of the device) was encountered in a median follow-up period of 10 months (range 2–14 months). Complete occlusion has occurred in all at follow-up. NOASD-R is a feasible and effective device for use in the transcatheter occlusion of moderate to large secundum ASDs in selected patients. The occurrence of the erosion on the right atrial roof may be due to the high localization of the device and the larger size of the right disk.

We aimed to assess early and midterm outcomes of balloon valvuloplasty (BVP) procedure in patients with critical pulmonary stenosis (CPS) and to describe the predictors of the need for additional pulmonary flow and reintervention in this subgroup of patients. From 2005 to 2014, 56 neonates were diagnosed with CPS and were included in this study. All echocardiographic, catheterization and angiographic data obtained prior to the initial BVP and at follow-up were reviewed. BVP was successful in 55 neonates (98 %). Twenty-one neonates needed pulmonary blood flow augmentation after BVP (38 %). Ductal stenting (DS) was performed in 20. The patients' mean tricuspid valve (TV) annulus diameter was 10.4 ± 2 mm, and the Z score was -1.29 ± 1 (-3.7 to 0.78). The mean pulmonary valve (PV) annulus diameter was 6 ± 0.9 mm, and the Z score was -1.74 ± 1 (-4.34 to 0.05). A transcatheter or surgical reintervention was performed in 11 patients. A TV Z score

Extra genetic material in patients with Down syndrome (DS) may affect the function of any organ system. We evaluated cardiac functions using conventional tissue Doppler and two-dimensional speckle tracking echocardiography in patients with DS in the absence of congenital and acquired heart disease in patients. A total of 115 patients with DS between 6 and 13 years of age with clinically and anatomically normal heart and 55 healthy children were included in this cross-sectional study. DS was diagnosed by a karyotype test. Patients with mosaic type were not included in this study. Systolic and diastolic functions were evaluated by echocardiography. Pulsed waved Doppler transmitral early/late inflow velocity (E/A), tissue Doppler mitral annular early/late diastolic peak velocity (Ea/Aa), transtricuspid E/A and tricuspid valve annulus Ea/Aa, pulmonary venous Doppler systolic/diastolic (S/D) wave ratio were lower in patients with Down syndrome than in the control group (P=0.04, P=0.001, P

Goldenhar sendromu kulak, burun, yumuşak damak, dudak ve mandibulanın yetersiz gelişimiyle karakterize ender konjenital anomalidir. Birinci ve ikinci branşiyal arkın gelişim anomalisidir. Sık görülen belirtieri limbal dermoid, kulak önünde skin tag’ler, şaşılıkla karekterizedir. Burada biz 4 aylık bir kız çocuğunda izole parsiyel pumoner venöz dönüş anomalisini sunduk., Goldenhar syndrome is a rare congenital abnormality characterized by incomplete development of the ear, nose, soft palate, lip, and mandible. It is associated with anomalous development of the first and second branchial arch. Common clinical manifestations include limbal dermoids, preauricular skin tags, and strabismus. Here we present a case of a four months old female with Goldenhar’s syndrome and partial anomalous pulmonary venous return.

Bronkojenik kist (BK), trakeobronşial ağacın gelişimi sırasında anormal tomurcuklanmasıyla oluşur. Akciğerin konjenital bronkojenik kistik lezyonu infantta nadir görülür, genellikle benign lezyonlar olup daha az semptom üretme eğilimindedirler. Bronkojenik kist öksürük ve dispneye neden olabilir. Göğüs radyografisinde genellikle alt mediastende yuvarlak veya oval kitle şeklinde görülür. İnfant ve çocuklarda sıklıkla tesadüfen tanı alır. Bu makalede, öksürük ve dispneye neden olan sağ akciğer orta lobdaki bronkojenik kist olgusu (14 aylık) radyolojik bulguları ile birlikte sunulmuştur., Bronchogenic cysts are congenital anomalies that result from abnormal budding of the tracheobronchial tree. Congenital bronchogenic cystic disease of the lung in infant is rare, generally benign lesions, which tend to produce few to no symptoms.The cyst can produce cough and dyspnea. Chest, radiograph shows usually a round or oval soft tissue mass in the lower mediastinum. They can occur in infants and children, they are frequently detected coincidentally. In this article, we present a case with bronchogenic cyst of the right middle lobe (14 month) causing cough, dyspnea and radiologic findings.

Amaç: Prematürelerde patent duktus arteriyozus (PDA) boyutunu, pulsed wave Doppler (PWD) akım paternlerini, tedavi gerektiren akım paternlerini tespit etmek. Yöntemler: Bu retrospektif çalışmada 34. gebelik haftasından önce doğan 139 prematüre olgunun PDA’ları PWD ve renkli Doppler ile değerlendirilerek akım paternleri sınıflandırıldı. Olguların sol atriyum aort kök oranı değerlendirildi. PWD paternleri, duktal çap ve sol atriyum/ aort kök oranları ile karşılaştırıldı. PDA ibuprofen ile tedavi edildi. Paternlerin tedavi gereksi- nimleri ve trombosit sayısı değerlendirildi. Bulgular: 139 pretermin %85,5’i 1500 g altında idi. Ortanca gebelik haftası 28,5 (23-34), doğum ağırlığı 1050 (750-1850) g tespit edildi. 39 (%17,3) pulmoner hipertansiyon, 43 (%19,1) büyüyen, 68 (%30,2) pulsatil patern, 75 (%33,3) kapanma paterni, tespit edildi. Pulmoner hipertansiyon paterni ilk üç günde sık gözlendi (p0.05). Sonuç: Duktal çap ve akım paterni arasında önemli ilişki tespit ettik. Duktal patern ve trombosit sayısı arasında ilişki saptanmadı. Pulsatil paternde hemodinamik önem oranı yüksekti. Pulmoner hipertansiyon paterninde duktus çapı büyük olmasına rağmen duktal şant azdı. Hemodinamik açıdan önemli duktusların tespit edilmesinde hem akım paterni hem de duktal çapın önemli olduğuna inanıyoruz., Objective: Aim of study was to evaluate both ductal diameter (Dd) and pulsed wave Doppler (PWD) patterns of patent ductus arteriosus (PDA), the treatment requirement of different patterns. Methods: In this retropective study evaluation and classification of PDA of 139 preterms born before 34 weeks gestation was performed by color Doppler and PWD. Ratio of left atrium/aortic root were evaluated in parasternal long axis. Ductal patterns were compared with both Dd and left atrium/aortic root ratio. PDA was treated by ibuprofen. The treatment requirement and thrombocyte count of patterns was evaluated. Results: 85,5% of preterms were very low birth weight (0,05). Conclusion: We determined significant relationship between Dd and flow pattern. There was no relationship between ductal pattern and thrombocyte count. Pulsatile pattern were hemodyna- mically significant. Despite greater Dd, treatment requirement in PH pattern was less due to low left-right shunt. Both ductal diameter and pattern together is important to identify hemodyna- mically of hemodynamically significant PDA.