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Purpose: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. Methods: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal–fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. Results: Ninety‐nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra‐cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty‐nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). Conclusion: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies. [ABSTRACT FROM AUTHOR]

Objective: To assess the accuracy of prenatal echocardiography in defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD). The second aim is to compare the perinatal and postnatal outcomes of different pulmonary blood supply types. Study design: The cases prenatally diagnosed with PAVSD between 2017 and 2022 in a single tertiary fetal medicine center were identified on the electronic database. Fetal echocardiography reports and images were reviewed retrospectively. Postnatal outcomes were acquired from the hospital records of relevant pediatric cardiology and cardiovascular surgery clinics. Fetal echocardiography results were compared with postnatal results. Perinatal and postnatal outcomes were compared between the different pulmonary vascular supply types. Results: Among the 24 PAVSD cases, six were diagnosed with major aortopulmonary collateral arteries (MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA) dependent pulmonary supply, and seven were diagnosed with double pulmonary supply (MAPCA + DA) on prenatal echocardiography. Seventeen cases were live‐born and have undergone postnatal investigations. Fetal echocardiography was 88.2% accurate about the type of pulmonary supply. The accuracy of fetal echocardiography regarding pulmonary vascular anatomy was 82.3%. Postoperative survival was 69.2%. Mortality before surgery and postoperative survival did not differ between pulmonary supply groups. Survival was impaired by the extracardiac anomalies. The need for early interventions was significantly higher in the DA group. Conclusion: Pulmonary vascularization in PAVSD can be defined precisely on fetal echocardiography. The source of pulmonary blood supply does not impact postnatal short‐term outcomes significantly but it impacts the management. The associated anomalies highly contribute to postnatal mortality. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to define cardiac and extracardiac malformations in fetuses with heterotaxy syndrome and to determine perinatal and childhood prognosis. Methods: In this retrospective study, fetuses diagnosed with heterotaxy syndrome on antenatal ultrasonography in a tertiary center between January 2014 and January 2021 were analyzed. Fetuses with heterotaxy syndrome were grouped as right atrial isomerism (RAI) and left atrial isomerism (LAI). Results: A total of 62 fetuses, 32 (51.6%) with RAI and 30 (48.4%) with LAI, were included in the study. Extracardiac anomaly was detected in 25% of fetuses with RAI and 44% of fetuses with LAI (p = 0.13). Patients with univentricular repair had a higher childhood mortality than patients with biventricular repair (p = 0.031). The presence of conotruncal anomaly was an independent factor affecting mortality (HR = 5.09, CI 95% 1.09–23.71, p = 0.039). Conclusion: Hydrops fetalis, univentricular physiology and conotruncal anomalies are associated with poor outcomes in heterotaxy syndrome. The severity of the cardiac malformation is the main determinant of the outcomes. The presence of extracardiac malformations is associated with increased morbidity and mortality. Key points: What's already known about this topic?Heterotaxy syndrome is a rare disease with cardiac and extracardiac malformations. Fetuses with right atrial isomerism have worse prognosis than fetuses with left atrial isomerism. The severity of the cardiac malformation is the main determinant of the outcomes. What does this study add?The presence of conotruncal anomalies as well as univentricular physiology is associated with poor prognosis. The presence of extracardiac malformations is associated with increased morbidity and mortality. [ABSTRACT FROM AUTHOR]

The article describes the case of a transjugular percutaneous secundum atrial septal defect (ASD) in a patient with Kartagener's syndrome, dectrocardia with situs inversus and interrupted inferior vena cava with azygos continuation. Topics discussed include results of the patient's transesophageal echocardiography, and the challenge of ASD closure with the transjugular appraoch.

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. Methods: This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single tertiary-care institution. Results: Twenty-two patients underwent midline single-stage unifocalization. The median age was 11 months (IQR: 5-21 months). The number of collateral arteries unifocalized was between one and three (median two). In-hospital mortality was 5%. Follow-up was complete; and the median follow-up regarding survival was 20 months (IQR: 10-28 months). There were three late deaths, and the estimated survival rate was 80% at 10 months and on. Out of 22 patients, ventricular septal defect was closed in the first surgery in three patients (14%) and the second surgery in four patients (19%). Total seven patients underwent surgical total repair (32%). Additionally, one out of four patients whose ventricular septal defects were closed with a fenestrated patch is under follow-up with a small ventricular septal defect, while two are waiting for ventricular septal defect closure. Therefore, total eight patients (36%) have reached total correction. Conclusion: Single-stage unifocalization is a feasible treatment option in ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. This cohort had unfavorable results regarding the rate of complete repair. The pitfalls encountered were related to problems with meticulous surgical technique, complete unifocalization, and correct implementation of the flow study. [ABSTRACT FROM AUTHOR]

Objective: To report clinical and procedural characteristics of twelve patients who received a covered stent for the treatment of aortic coarctation and concurrent patent ductus arteriosus (PDA). Methods: A single center database was retrospectively evaluated to obtain data of patients with combined aortic coarctation and PDA. We selected patients in whom a covered stent was used for the treatment of both pathologies. The stent length was chosen so as to cover the entire length of the lesion from healthy to healthy tissue and also cover the ampulla of PDA. Results: The median age of the patients was 15 (range, 6.5-35) years. The diameter of the coarctated segment increased from a median of 8.4 (range, 2.6-10.8) mm to 16 (range, 9-24) mm (p<0.005), whereas the pressure gradient decreased from a median of 43 (range, 10-71) mm Hg to 0 (range, 0-8) mm Hg (p<0.005). Fourteen covered stents were used for 12 patients. Following deployment, seven stents were flared with larger and low-pressure balloons because of the gap between the distal end of the stent and the poststenotic dilated segment of the aorta, which caused residual PDA shunts and/or instability of the stent. After the procedure, no residual PDA shunt was present in any patient. Conclusion: To the best of our knowledge, this study includes the largest series of patients reported in literature in whom covered CP stents were used for simultaneous percutaneous treatment of coarctation and PDA. The procedure was successful and stable results were obtained during follow-up in all cases. [ABSTRACT FROM AUTHOR]

Congenitally corrected transposition of the great arteries (cc-TGA), is also defined as double discordance and ventricular inversion. We conducted a retrospective evaluation of medical records of 79 patients diagnosed and followed-up with congenital transposition of great arteries between December 1993 and January 2014 at our pediatric cardiology department. Of 79 patients included in the study, 46 (58.2%) were male, mean age at diagnosis was 40±39.25 months and the mean follow-up time was 56±39.4 months. Thirteen (16.5%) patients had atrial situs inversus. The most common concomitant abnormality was found to be ventricular septal defet (29.1%). Fourty- five patients had pulmonary stenosis either in the form of valvular, subvalvular or both together. Twelve (15.2%) patients had pulmonary atresia. Systemic atrioventricular valve (AV) regurgitation was present in 51 (64.6%) patients, whereas 2 (2.5%) patients had severe systemic AV valve regurgitation. Ebstein-like AV valve was detected in 5 (6.3%) patients. Transcatheter interventions included ductal stent implantation (n=5; 6.3%), left ventriculopulmonary artery conduit angioplasty (n=2; 2.5%), RV-PA conduit angioplasty (n=1; 1.3%) and pulmonary balloon valvuloplasty (n=2; 2.5%). Eleven (13.9%) patients underwent interventional therapeutic cardiac catheterization. Glenn anastomosis was performed in 16 (20.3%) patients. Nine (11.4%) patients required a permanent pacemaker implantation. Individualization of treatments and managements for the patients is of prognostic importance for these patients needing lifelong follow-up due to the large number of accompanying anomalies and different degrees of abnormalities in cc-TGA patients. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Dr. Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Dr. Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Ductus venosus connects intraabdominal umbilical vein to inferior vena cava. Absence of ductus venosus may exists with cardiac, extracardiac, or chromosomal abnormalities. Opening site of umbilical vein is important in the absence of ductus venosus. Diagnosis of absence of ductus venosus can be made by fetal colour Doppler ultrasound. We presented a patient whose umbilical vein opened into portal sinus in the absence of ductus venosus [ABSTRACT FROM AUTHOR]

Copyright of Türk Pediatri Arşivi is the property of Aves Yayincilik Ltd. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: This study aimed to present our experience with transcatheter pulmonary debanding, focusing on patient outcomes., Methods: The retrospective study was conducted with 32 patients (17 males, 15 females; mean age: 3.6±2 years; range, 0.5 to 8.8 years) who underwent transcatheter pulmonary debanding between January 2010 and January 2024. The patients were evaluated in two groups. In Group 1 (n=24), total debanding was targeted for patients with spontaneously closed or restrictive ventricular septal defects or those suitable for transcatheter ventricular septal defect closure. In Group 2 (n=8), palliative debanding was utilized in children with ongoing band requirement., Results: The median body weight was 15 kg. In Group 1, the mean right ventricle-to-aortic pressure ratio (RVp/Aop) was 0.91±0.21 before the procedure, which decreased to a mean of 0.33±0.20 after the procedure. In Group 2, the mean RVp/Aop was 1.31±0.47, which decreased to 0.77±0.13 after transcatheter palliative debanding. The mean peripheral oxygen saturation was 80±6% before the procedure and 94±2.5% after the procedure. Transcatheter debanding was successful in all patients when surgical pulmonary banding was performed with 6-0 Prolene and polytetrafluoroethylene band material., Conclusion: Transcatheter banding is a safe and effective procedure that minimizes the need for reoperation., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish Society of Cardiovascular Surgery.)

Background: The aim of this study was to evaluate the outcomes of right ventricular outflow tract stenting for palliation during the newborn and infancy periods., Methods: Between January 2013 and January 2018, a total of 38 patients (20 males, 18 females; median age 51 days; range, 3 days to 9 months) who underwent transcatheter right ventricular outflow tract stenting in three centers were retrospectively analyzed. Demographic characteristics, cardiac pathologies, angiographic procedural, and clinical follow-up data of the patients were recorded., Results: The diagnoses of the cases were tetralogy of Fallot (n=27), double outlet right ventricle (n=8), complex congenital heart disease (n=2), and Ebstein"s anomaly (n=1). The median weight at the time of stent implantation was 3.5 (range, 2 to 10) kg. Five cases had genetic abnormalities. The median pre-procedural oxygen saturation was 63% (range, 44 to 80%), and the median procedural time was 60 (range, 25 to 120) min. Acute procedural success ratio was 87%. Reintervention was needed in seven of patients due to stent narrowing during follow-up. During follow-up period, seven cases died. Total correction surgery was performed in 26 patients without any mortality. While a transannular patch was used in 22 patients, valve protective surgery was implemented in two patients, and the bidirectional Glenn procedure was performed in two patients., Conclusion: Based on our study results, right ventricular outflow tract stenting is a form of palliation which should be considered particularly in cases in whom total correction surgery is unable to be performed due to morbidity., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2020, Turkish Society of Cardiovascular Surgery.)

Background: The aim of this study was to evaluate the predictability of postoperative pulmonary artery pressure (PAP) using intraoperative flow study in patients undergoing bidirectional Glenn operation., Methods: Patients who underwent Glenn operation under cardiopulmonary bypass (CPB) were included in the study. During the operation, after the completion of additional procedures under CPB, an intraoperative flow study was performed prior to Glenn anastomosis. After the completion of bidirectional Glenn, the patient was separated from the CPB and PAP was measured. The relationship between this pressure and flow study measurement was analyzed., Results: Nine patients who underwent bidirectional Glenn operation with additional procedures under CPB between July 2018 and January 2019 were included in the study. The median PAP was 9 mm Hg (interquartile range [IQR]: 7-10 mm Hg) in the flow study and 10 mm Hg (IQR: 8-11 mm Hg) after CPB, and the median difference between these pressures was 1 mm Hg (IQR: 1-3 mm Hg). There was a strong correlation between these two measurements ( r = 0.732; P = .025)., Conclusion: The results of this study show that PAP after the Glenn procedure can be estimated using an intraoperative flow study. We believe that this method may be useful in intraoperative decision-making for Glenn operation in single ventricular patients who require extensive pulmonary artery (PA) reconstruction due to limited PA development, branch PA stenosis, or nonconfluent PAs. Also, this method can be used as a sort of intraoperative pulmonary resistance reversibility study in patients with high preoperative pulmonary vascular resistance due to surgically correctable pulmonary venous hypertension.

Objective: The incidence of patent ductus arteriosus (PDA) is greater among patients living at high altitude. In this po-pulation, the ductal diameter is often larger and pulmonary hypertension is more frequent. The aim of this study was to evaluate the hemodynamic and morphological features of PDA and transcatheter closure procedures performed with various devices in a group of patients living at high altitude in Turkey., Methods: The data of 327 patients who lived at an altitude of at least 1600 m above sea level and who had undergone cardiac catheterization for isolated PDA between May 2010 and July 2018 were retrospectively analyzed., Results: The mean age was 7.33±7.67 years, and 62.4% of the patients were female. The mean ductal diameter was 3.74±2.14 mm. Pulmonary hypertension was present in 57.8%. Transcatheter closure was performed in 322 patients, with a 97.3% success rate. The Amplatzer duct occluder I (ADO I) was used most often, as well as off-label use of the Amplatzer vascular plug II (AVP) and the Amplatzer muscular ventricular septal defect occluder (AMVSDO). Pulmonary artery pressure decreased immediately in the vast majority after percutaneous closure. Transient left ventricular systolic dysfunction after ductal closure was seen only rarely. Follow-up was uneventful., Conclusion: Transcatheter PDA closure can be performed with high success rate in highlanders. Off-label devices may be required for these procedures. Pulmonary hypertension is frequent but regresses after ductal closure. Transient left ventricular dysfunction after transcatheter closure is rarely seen in these patients and resolves without any medication.

Objective: To report clinical and procedural characteristics of twelve patients who received a covered stent for the treatment of aortic coarctation and concurrent patent ductus arteriosus (PDA)., Methods: A single center database was retrospectively evaluated to obtain data of patients with combined aortic coarctation and PDA. We selected patients in whom a covered stent was used for the treatment of both pathologies. The stent length was chosen so as to cover the entire length of the lesion from healthy to healthy tissue and also cover the ampulla of PDA., Results: The median age of the patients was 15 (range, 6.5-35) years. The diameter of the coarctated segment increased from a median of 8.4 (range, 2.6-10.8) mm to 16 (range, 9-24) mm (p<0.005), whereas the pressure gradient decreased from a median of 43 (range, 10-71) mm Hg to 0 (range, 0-8) mm Hg (p<0.005). Fourteen covered stents were used for 12 patients. Following deployment, seven stents were flared with larger and low-pressure balloons because of the gap between the distal end of the stent and the poststenotic dilated segment of the aorta, which caused residual PDA shunts and/or instability of the stent. After the procedure, no residual PDA shunt was present in any patient., Conclusion: To the best of our knowledge, this study includes the largest series of patients reported in literature in whom covered CP stents were used for simultaneous percutaneous treatment of coarctation and PDA. The procedure was successful and stable results were obtained during follow-up in all cases.

A 5-year-old male with a double outlet right ventricle with noncommitted ventricular septal defect and pulmonary stenosis underwent a bidirectional Glenn operation at 2 years and a Fontan operation with ligation of the pulmonary trunk at 5 years. He presented with pleural effusion 3 months after the Fontan operation. Physical examination revealed a grade 3/6 systolic murmur in the pulmonary area. Echocardiographic evaluation revealed an antegrade pulmonary flow (APF) of gradient 80 mmHg across the ventriculopulmonary communication. Cardiac catheterization and angiography demonstrated the presence of residual antegrade pulmonary flow and stenosis at the pulmonary artery bifurcation. Both pathologies were treated using a single covered stent. Relief of the pulmonary artery stenosis and total occlusion of the residual APF was demonstrated on a control angiogram.

A descending aorta to vertebral venous plexus fistula is an extremely rare form of arteriovenous fistula. A 10-month-old infant was referred to the hospital for evaluation of a murmur. On examination, a continuous murmur was heard in the entire back. Transthoracic echocardiography revealed left heart chamber dilatation in the presence of preserved left ventricle systolic function. Computerized tomography angiography with 3-dimensional reconstruction, and lateral projection aortography revealed a descending aorta-vertebral venous plexus fistula measuring 4.8 mm in the aortic orifice. The fistula was embolized using an Amplatzer Vascular Plug 2.

Background: Aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves and is the rarest of septal defects., Aims: To present our experience with the diagnosis and outcome of APW cases., Study Design: Retrospective cohort study., Methods: Between June 2003 and October 2011, thirteen patients were diagnosed with APW. Clinical features of patients, findings of echocardiographic and angiographic examination, results of surgical intervention and follow-up were reviewed retrospectively., Results: Eleven children (10 days to 16 years), underwent surgical correction of APW. In a 12-month-old boy, the defect was repaired by the transcatheter approach. In addition to APW repair, closure of VSD was performed in 2 patients. APW were associated with interruption in two patients; one also had a complex pathology. None of the patients died due to complications of surgical or transcatheter procedures. After a median follow-up period of 40 months, the patients were asymptomatic and none of them required additional medication, except for the patient with complex pathology including an interrupted aortic arch, who underwent balloon angioplasty for recoarctation., Conclusion: In any infant with the findings of congestive heart failure and failure to thrive, APW must be kept in mind as a differential diagnosis. In isolated APW cases before 6 months of age, echocardiography is often sufficient for diagnosis. In complex cases, cardiac catheterisation is performed for the comprehensive evaluation of associated defects. After 6 months, cardiac catheterisation could be utilised to perform vasoreactivity testing and, if possible, to close the defect.