Revisiting Atrioventricular Septal Defects: Exploring Chromosomal Abnormalities, Cardiac and Extracardiac Anomalies in a Contemporary Prenatal Cohort.

To estimate if there is an association between partial AVSD with chromosomal abnormalities, cardiac and extracardiac malformations, and to report the outcomes of prenatally diagnosed AVSD in a large, contemporary cohort. This is a retrospective cohort study of 190 prenatally diagnosed fetal AVSD between 2014 and 2023. Type of AVSD (complete vs partial), additional cardiac findings, extracardiac findings, presence of a heterotaxy, results of prenatal karyotype, and pregnancy outcomes were documented and analyzed. A total of 190 cases of fetal AVSD were analyzed. Complete AVSDs comprised 141 (74.2%) of the cohort, while partial AVSDs comprised 49 (25.7%). Karyotype was completed in 131 cases, and in 98 (74.8%) cases chromosomal abnormalities were identified, with trisomy 21 being the most common (53/131, 40.5%). Complete AVSDs were associated with trisomy 21 (45.5%, p = 0.04), Isolated cases of complete AVSDs (p = 0.03). Partial AVSDs were associated with trisomy 18 (53.1%, p < 0.001). In cases of partial AVSDs with aneuploidies, 7 (70%) had an ostium primum defect and 20 (90.9%) of AV canal type VSD. Isolated partial AVSD had no clear association with aneuploidies. There were additional cardiac anomalies in 96 (50.5%) and extracardiac anomalies in 134 (70.5%) of the cohort. There were no differences between partial and complete AVSD in rate of additional cardiac and extracardiac anomalies. AVSD was part of a heterotaxy in 47 (24.7%) of cases, and heterotaxy was associated with complete AVSD in the majority of cases (43/47, 91.4%, p = 0.003). Fetal partial AVSDs are associated with trisomy 18. Fetal complete AVSDs, even isolated, are associated with trisomy 21. There were no differences in association of other aneuploidies, additional cardiac findings, or extracardiac anomalies between prenatally diagnosed complete AVSDs and partial AVSDs.

The Lifesaving Impact of Transcatheter Interventions in the Early Post-Fontan Palliation Period.

Despite advancements in postoperative outcomes after Fontan surgery, there remains a risk of suboptimal outcomes and significant morbidity in the early postoperative period. Anatomical obstructions in the Fontan pathway can lead to prolonged pleural effusion or ascites, cyanosis, and low cardiac output syndrome (LCOS). Transcatheter interventions offer an alternative to early re-surgery for treating these complications. Over a 13-year period, early catheter angiography, performed within 30 days post-index procedure, was administered to 41 patients, identifying anatomical issues that necessitated re-intervention in 39 cases. This led to transcatheter interventions in 37 (10.4%) of the 344 Fontan surgery patients. The median age was 4.8 years (IQR: 4-9.4), and the median weight was 16.5 kg (IQR: 15-25.2), with females comprising 51.4% (19/37) of this group. The primary indications for the procedures were persistent pleural effusion or ascites in 27 patients (66%), LCOS in 8 patients (20%), and cyanosis in 6 patients (14%). Among the 37 undergoing transcatheter intervention, 30 were treated solely with this method and discharged, three died in ICU follow-up, and four required early re-surgery. No procedural mortality was observed. Our findings demonstrate that transcatheter interventions, including stent implantation, balloon angioplasty, and fenestration dilation, are safe and effective in the early post-Fontan period. Therefore, they should be considered an integral part of the management strategy for this patient group.

Navigating the spectrum of double-outlet right ventricle presentations: Outcomes from a contemporary cohort based on subtypes.

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.

Utility of Balloon Occlusion Testing in Determining Fontan Suitability Among Patients with Elevated Pulmonary Artery Pressure and Additional Antegrade Pulmonary Blood Flow.

In individuals with a single ventricle undergoing evaluation before Fontan surgery, the presence of excessive pulmonary blood flow can contribute to increased pulmonary artery pressure, notably in those who had a Glenn procedure with antegrade pulmonary flow. 28 patients who had previously undergone Glenn anastomosis with antegrade pulmonary blood flow (APBF) and with elevated mean pulmonary artery (mPAP) pressure > 15 mmHg in diagnostic catheter angiography were included in the study. After addressing other anatomical factors that could affect pulmonary artery pressure, APBF was occluded with semi-compliant, Wedge or sizing balloons to measure pulmonary artery pressure accurately. 23 patients (82% of the cohort) advanced to Fontan completion. In this group, median mPAP dropped from 20.5 (IQR 19-22) mmHg to 13 (IQR 12-14) mmHg post-test (p < 0.001). Median PVR post-test was 1.8 (IQR 1.5-2.1) WU m2. SpO2 levels decreased from a median of 88% (IQR 86%-93%) pre-test to 80% (IQR 75%-84%) post-test (p < 0.001). In five patients, elevated mPAP post-test occlusion on diagnostic catheter angiography led to non-completion of Fontan circulation. In this group, median pre- and post-test mPAP were 23 mmHg (IQR 21.5-23.5) and 19 mmHg (IQR 18.5-20), respectively (p = 0.038). Median post-test PVR was 3.8 (IQR 3.6-4.5) WU m2. SpO2 levels decreased from a median of 79% (IQR 76%-81%) pre-test to 77% (IQR 73.5%-80%) post-test (p = 0.039). Our study presents a specialized approach for patients initially deemed unsuitable for Fontan due to elevated pulmonary artery pressures. We were able to successfully complete the Fontan procedure in the majority of these high-risk cases after temporary balloon occlusion test.

Pulmonary atresia and ventricular septal defect: How accurate is the fetal echocardiography, and do the major aortopulmonary collateral arteries matter?

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.

A Challenging Interventional Procedure: Transcatheter Closure of Tubular Patent Ductus Arteriosus in Patients with Pulmonary Hypertension.

Transcatheter closure of the tubular ducts remains the most challenging procedure, with higher complication rates than other types. This study evaluates the characteristics of transcatheter closure of tubular ducts with pulmonary hypertension. 73 patients with tubular ducts who underwent cardiac catheterization for transcatheter PDA closure were analyzed. The mean age and weight were 1.93 ± 2.68 years and 8.83 ± 6.14 kg, respectively. Transcatheter closure was attempted in 72 patients. Four cases (5.5%) were referred to surgery, while the procedure was completed in the remaining (94.5%). Amplatzer duct occluder (ADO) I or Cardiofix duct occluder (CDO) was the most commonly used devices. However, the use of Amplatzer vascular plug (AVP) II raised in recent years. The most common concern was aortic protrusion/stenosis in ADO I/CDO devices, but most regressed during follow-up. Iatrogenic coarctation of the aorta was observed in two with ADO I/CDO. Embolization of the device to the pulmonary artery was observed in three with CDO, AVP II, and AVP I. Significant left pulmonary artery stenosis requiring stenting developed in one after closure with an MVSDO device. Tubular ducts are highly associated with pulmonary arterial hypertension, and transcatheter closure of them is still challenging despite the developing device armamentarium. Although ADO I or similar devices are widely used, off-label devices are usually needed at increasing rates. The AVP II device is unsuitable for short tubular ducts but seems the best option for long ones.

Surgical and transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: cardiac magnetic resonance imaging characteristics and morphology of right ventricular outflow tract.

BACKGROUND: Pulmonary valve replacement is recommended in patients with repaired tetralogy of Fallot based on cardiac magnetic resonance imaging (MRI) criteria. This procedure is performed by surgical or transcatheter approaches. OBJECTIVE: We aimed to investigate the differences in preprocedural MRI characteristics (volume, function, strain) and morphology of the right ventricular outflow tract and branch pulmonary arteries in patients for whom surgical or transcatheter pulmonary valve replacement was planned. MATERIALS AND METHODS: Cardiac MRI of 166 patients with tetralogy of Fallot were analyzed. Of these, 36 patients for whom pulmonary valve replacement was planned were included. Magnetic resonance imaging characteristics, right ventricular outflow tract morphology, branch pulmonary artery flow distribution and diameter were compared between surgical and transcatheter groups. Spearman correlation and Kruskal-Wallis tests were performed. RESULTS: Circumferential and radial MRI strain for the right ventricle were lower in the surgical group (P=0.045 and P=0.046, respectively). The diameter of the left pulmonary artery was significantly lower (P=0.021) and branch pulmonary artery flow and diameter ratio were higher (P=0.044 and P = 0.002, respectively) in the transcatheter group. There was a significant correlation between right ventricular outflow tract morphology and right ventricular end-diastolic volume index and global circumferential and radial MRI strain (P=0.046, P=0.046 and P= 0.049, respectively). CONCLUSION: Preprocedural MRI strain, right-to-left pulmonary artery flow, diameter ratio and morphological features of the right ventricular outflow tract were significantly different between the two groups. A transcatheter approach may be recommended for patients with branch pulmonary artery stenosis, since both pulmonary valve replacement and branch pulmonary artery stenting can be performed in the same session.

Experience of 500 cardiovascular magnetic resonance imaging and systematic analysis of cases.

OBJECTIVE: Cardiovascular magnetic resonance imaging (MRI) is a widely accepted reference imaging technique in routine cardiology clinics in many centers due to its advantages in providing preferable functional, morphologic information. However, there is little information about national experience in clinical application and findings of cardiovascular MRI. The objective of this study was to demonstrate the clinical and demographic characteristics of patients admitted to our cardiac imaging department. METHODS: A total of 500 cardiovascular MRI examinations performed between 2016 and 2019 were enrolled in this retrospective study. Clinical indications, demographic, and cardiovascular MRI findings of the patients were retrospectively evaluated. RESULTS: Five hundred patients (M/F=301/199) were included in this retrospective, single center study. The majority of the examinations were performed for the assessment of congenital heart disease (n=254, 50.8%). The other indications were for myocardial disease (n=160, 32%), cardiac mass (n=44, 8.8%), valvular heart disease (n=20, 4%), magnetic resonance angiography (n=12, 2.4% for aorta and pulmonary artery [n=9, 1.8%] and for coronary arteries [n=3, 0.6%]), and vasculitis (n=7, 1.7%), pericardial disease (n=3, 0.6%). Minor complication was seen during the contrast agent injection in three patients (0.06%). CONCLUSION: Cardiovascular MRI is a reliable and accurate imaging tool in identifying the various cardiac pathology with widely accepted use in the clinical area. Our single-center experience of 500 cases demonstrates the varieties of clinical indications in daily practice that may contribute to the national data pool.

Successful treatment of re-coarctation of the aorta with percutaneous stent placement via axillary artery access in a young patient.

In patients with critical coarctation of the aorta, percutaneous balloon angioplasty and/or stent placement is usually performed via the femoral route. When femoral access is not suitable for intervention, the trans-axillary approach can be chosen as an alternative access. We hereby present the first case of a patient who had a successful percutaneous stent placement via trans-axillary access in our institution.

Heterotaxy syndrome: Prenatal diagnosis, concomitant malformations and outcomes.

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.

Cardiac MRI in surgically repaired tetralogy of Fallot: Our initial experience.

OBJECTIVE: Pulmonary regurgitation (PR) required pulmonary valve replacement (PVR) is usually seen after surgically repaired tetralogy of Fallot (TOF). Assessment by cardiac magnetic resonance imaging (CMR) plays a crucial role in the decision of PVR. Herein, we presented our 3-year interdisciplinary CMR experience in the assessment of repaired TOF. METHODS: CMR examinations of 196 patients with repaired TOF performed between 2016 and 2018 were enrolled in this retrospective study. Only 165 were included in the study. CMR findings were assessed according to the American College of Cardiology/American Heart Association guideline and recommendations of Geva. RESULTS: Among those 165 patients (median age 14 years [mean age 15.62±7.42 years], M/F=114/61; 1.86/1), 73 patients were found eligible for PVR (59 patients for transcatheter while 14 patients for surgical). The mean QRS duration was 170.2±16.89 ms. On CMR assessment, mean indexed right ventricular end-diastolic volume, end-systolic volume, right, and left ventricular ejection fraction were 187.64±45.07 ml/m2, 39.90±6.60%, and 47.83±6.12%, respectively. The PR fraction was as 50.10±2.54% and 2.25±1.92. Balloon dilatation and/or stenting of branch pulmonary arteries in 12 patients and ventricular septal defect closure in four patients were performed at the same session of percutaneous PVR. At the time of the surgical PVR, repair of partial anomalous pulmonary venous return in one patient, ventricular septal defect in two patients, and subaortic membrane in one patient were performed. An implantable cardioverter-defibrillator was also performed in one patient. CONCLUSION: Our CMR experience has the largest patient population in our country and may contribute to the national data pool. We believe that our collaborative experience between radiologists, cardiologists, and cardiovascular surgeons may also enhance the use of CMR in determining the appropriate technique or timing for PVR.

Coronary artery-pulmonary artery fistula in pulmonary atresia with ventricular septal defect: report of two surgical cases.

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery-pulmonary artery fistula. We present two cases with coronary artery-pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Right ventricular outflow tract stenting during neonatal and infancy periods: A multi-center, retrospective study.

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.

Intraoperative Flow Study Predicted the Postoperative Pulmonary Artery Pressure in the Bidirectional Glenn Operation.

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.

Characteristics and transcatheter closure of patent ductus arteriosus in patients living at moderate to high altitude in Eastern Anatolia.

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.

A beneficial technique for preventing the device protrusion to the aorta during percutaneous patent ductus arteriosus closure: "Balloon-assisted device releasing technique".

BACKGROUND: Although percutaneous closure of patent ductus arteriosus is an established safe procedure, protrusion of the device to descending aorta may occur in various degrees during these procedures, especially in small infants. The aim of our study is to evaluate the benefits of balloon-assisted device releasing technique in the era of preventing device protrusion and conditions related to protrusion. METHODS: One hundred and fifty-five infants, who underwent patent ductus arteriosus closure with Amplatzer duct occluder I device between January, 2012 and December, 2018, were retrospectively analysed. Balloon-assisted device releasing technique was used in 20 cases (group 1, 12.9%), between January, 2015 and December, 2018. Procedures in which the technique had been used were compared with the remaining ones (group 2, 87.1%, n = 135) with regard to device stabilisation, aortic disc protrusion to the aorta, iatrogenic coarctation, and device embolisation. RESULTS: There was no significant difference by means of gender, age, weight, and the ductal diameter, whereas the average mean pulmonary artery pressure was significantly higher in group 1. Device protrusion and related complications were significantly higher in group 2; thus, additional catheterisations or surgical interventions were required, while no additional intervention was required in group 1. CONCLUSION: The balloon-assisted device releasing technique provides a good device stabilisation and prevents protrusion of the device and related complications during percutaneous patent ductus arteriosus closure in selected cases.

Midline one-stage complete unifocalization early outcomes from a single center.

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.

Risk Factors Associated with Ascending Aortic Aneurysms and Aortic Elasticity Parameters in Children with a Bicuspid Aortic Valve.

Aneurysms of the ascending aorta are frequently found in patients with a bicuspid aortic valve (BAV). This study assessed the risk factors of ascending aortic aneurysms and aortic elasticity in children with BAV. The study included 66 patients with no history of transcatheter intervention or surgical procedure who had been diagnosed with isolated BAV. Echocardiographic, blood pressure, and pulse measurements were obtained for all patients. The BAVs were classified as described by Sievers et al. (J Thorac Cardiovasc Surg 133:1226-1233, 2007), and aortic elasticity parameters were calculated using various formulas. The patients were divided into groups with and without cusp fusion, aortic stenosis (AS), aortic regurgitation (AR), or mixed lesions; the groups were then compared. The mean patient age was 10.43 ± 3.91 years; 15%he patients had no AS or AR, 33% had both AS and AR, 17% had AS alone, and 35% had AR alone. The most common type of BAV was type 5, and the ascending aorta z-scores were higher in children with mixed lesions and without a cusp fusion. Aortic distensibility (AD) was significantly higher, and the stiffness index was significantly lower, in patients with an ascending aorta z-score > 4. The ascending aortic z-scores were higher in the no-fusion and mixed lesion (AS + AR) groups, especially those originating from post-stenotic dilation due to AS. The AD was increased in patients with an ascending aorta z-score > 4. Patients should thus be monitored closely for dissection risk, and preventive medical treatment should be started early in those with AS without cusp fusion.

Preliminary results of the CeraflexTM PDA occluder and device behaviour during releasing.

INTRODUCTION: The CeraFlexTM PDA occluder is a new flexible device with a unique delivery system that may be beneficial with regard to not changing the device position after releasing. We prospectively evaluate the efficacy of the device and also the device behaviour patterns during release. METHODS: The study included 21 patients. Their median age was 1.2 years (from 6 months to 28 years) and weight was 9.6 kg (from 5.4 to 82 kg). All of the ducts were conical except one atypical ductus. Median ductal diameter at the pulmonary end was 3.8 mm (from 2.2 to 8.2 mm). The ductus was closed using an antegrade approach, but special attention was paid to the patterns of device behaviour during and just after releasing. RESULTS: Three different modes of device behaviour were observed during and just after releasing: (1) Neither difficulty nor change of position in 13 patients (62%), (2) a little difficulty in releasing but no change of position in 6 (29%), and (3) change of the device position in 2 (9%). There was no residual shunt on the next day except in one patient, in whom late device embolisation occurred. The device was retrieved and another, bigger device implanted. CONCLUSION: The CeraFlexTM PDA occlude device seems to be safe and efficacious for patent ductus arteriosus closure. Its unique delivery system generally fixes the device in a stable position that does not change after release (91%). Minor difficulty in releasing is not uncommon; however, the major disadvantage is the need for larger sheaths for delivery.