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CHDs affecting the right ventricular outflow tract often require repeated surgical or transcatheter interventions due to pulmonary insufficiency or stenosis. This study presents percutaneous implantation of large self-expanding valves in patients with complex right ventricular outflow tract anatomy after prior pulmonary branch stenting.

Competing Interests: The authors declare no conflict of interest.

We present the benefits of advanced multimodality imaging and virtual reality modelling in the diagnosis and treatment planning of a child with aneurysms after numerous interventions for treatment of a hypoplastic aortic arch and coarctation.

We report the first-stage percutaneous palliation in a newborn with a rare heterotaxy syndrome variant including interrupted inferior vena cava, partial anomalous pulmonary venous drainage, and restrictive interatrial communication. Virtual reality imaging aided visualisation, decision-making, and planning. Successful atrial septoplasty performed via the internal jugular vein and anomalous pulmonary vein was followed by stenting of ductus arteriosus.

Introduction: Precise electrocardiographic localization of accessory pathways (AP) can be challenging. Seminal AP localization studies were limited by complexity of algorithms and sample size. We aimed to create a nonalgorithmic method for AP localization based on color-coded maps of AP distribution generated by a web-based application., Methods: APs were categorized into 19 regions/types based on invasive electrophysiologic mapping. Preexcited QRS complexes were categorized into 6 types based on polarity and notch/slur. For each QRS type in each lead the distribution of APs was visualized on a gradient map. The principle of common set was used to combine the single lead maps to create the distribution map for AP with any combination of QRS types in several leads. For the validation phase, a separate cohort of APs was obtained., Results: A total of 800 patients with overt APs were studied. The application used the exploratory data set of 553 consecutive APs and the corresponding QRS complexes to generate AP localization maps for any possible combination of QRS types in 12 leads. Optimized approach (on average 3 steps) for evaluation of preexcited electrcardiogram was developed. The area of maximum probability of AP localization was pinpointed by providing the QRS type for the subsequent leads. The exploratory data set was validated with the separate cohort of APs (n = 256); p = .23 for difference in AP distribution., Conclusions: In the largest data set of APs to-date, a novel probabilistic and semi-automatic approach to electrocardiographic localization of APs was highly predictive for anatomic localization., (© 2024 Wiley Periodicals LLC.)

Percutaneous pulmonary valve implantation is established as a safe and effective method of treating patients with disfunction of right ventricular outflow tract. Modifications of this method allow for an increasingly wider use of this less invasive treatment. We present a staged percutaneous pulmonary valve implantation into a single-branch pulmonary artery in a paediatric patient with tetralogy of Fallot after patch repair.

Competing Interests: The authors declare no conflict of interest.

Competing Interests: The authors declare no conflict of interest.

Competing Interests: One of the authors is a proctor for Medtronic and the second author is a consultant for Xeltis.

Background: An additional shunt in single ventricle patients with Glenn anastomosis may increase pulmonary flow at the expense of ventricle volume overloading. The performance of the modification depends on pulmonary resistance, indicating better results in favorable hemodynamic conditions., Aims: The study aims at analyzing the influence of precisely adjusted pulsatile shunt in borderline high-risk Glenn patients on early and late results., Methods: The study involved 99 patients (including 21 children) with the bidirectional Glenn and accessory pulsatile shunt (BDGS group), and 78 patients with the classic bidirectional Glenn anastomosis (BDG group)., Results: There was 1 death in the BDGS group and 4 deaths in the BDG group. No difference in mortality (P = 0.71) was found. The Fontan completion was achieved in 69 (88.5%) children in the BDG group and 18 (85.7%) patients in the BDGS group, without fatalities. No intergroup differences in postoperative pulmonary artery pressure (P = 0.10), ventilation time (P = 0.12), the McGoon ratio (P = 0.9), or chylothorax frequency (P = 0.14) were observed. Intensive care unit (P = 0.28) and hospitalization (P = 0.05) times were comparable. Echocardiography revealed no significant differences in the ventricle and atrioventricular valve function between groups. In the BDGS group, higher blood oxygen saturation (P = 0.03) and increase of the McGoon index (P = 0.002) were noted., Conclusions: Bidirectional Glenn anastomosis with precisely adjusted accessory pulmonary blood flow provides stable hemodynamics and adequate oxygen saturation in borderline, profoundly hypoxic patients. An advantageous pulmonary artery development before Fontan completion was observed.

Background: Familial hypercholesterolemia (FH) increases the risk of atherosclerosis in children and adults. Atherosclerotic cardiovascular disease in young patients FH is usually subclinical but recognition of children with more pronounced changes is crucial for adjusting effective management. Aim of this research was to use ultrasonography with two-dimensional speckle tracking (2DST) and tonometry to evaluate atherosclerotic changes in patients with FH (parents and their offspring)., Methods: Applanation tonometry and carotid arteries sonography with evaluation of the intima-media complex thickness (IMCT) and application of the 2DST were performed in 20 families with FH (20 parents and 29 children). The same size control group (age and sex matched) was included. Results were compared between peers and between generations together with the correlation analysis., Results: Adults with FH, in comparison with healthy peers, presented significantly more atherosclerotic plaques (9 vs. 2, p = 0.0230), had significantly thicker IMC (0.84 ± 0.19 vs. 0.56 ± 0.06 mm, p < 0.0001) and had stiffer arterial wall (for stain: 6.25 ± 2.3 vs. 8.15 ± 2.46, p = 0.0103). In children from both groups there were no atherosclerotic plaques and IMCT did not differ significantly (0.42 ± 0.07 vs. 0.39 ± 0.04, p = 0.1722). However, children with FH had significantly stiffer arterial wall according to 2DST (for strain: 9.22 ± 3.4 vs. 11.93 ± 3.11, p = 0.0057) and tonometry (for the pulse wave velocity: 4.5 ± 0.64 vs.3.96 ± 0.62, p = 0.0047). These parameters correlated with atherosclerosis surrogates in their parents (p < 0.001) but were not significantly affected by presence of presumed pathogenic gene variant., Conclusions: Children with FH presented subclinical atherosclerosis manifested as decreased arterial wall elasticity. Degree of stiffening was associated with advancement of atherosclerosis in their parents but did not present significant association with gene variants. Sonography with application of 2DST seems to be a good candidate for comprehensive evaluation of atherosclerosis in families with FH.

Cardiac catheterisation in patients on extracorporeal membrane oxygenation (ECMO) may reveal new information leading to modification of a therapeutic plan and correction of newly recognised or residual lesions. Complications associated with cardiac catheterisation during ECMO are not uncommon and often related to the access site. We report a straightforward technique for accessing the ECMO circuit to perform an emergent cardiac catheterisation in two patients with hypoplastic left heart syndrome decompensated after Norwood I, due to presumed systemic-to-pulmonary artery shunt obstruction.

A 6-year-old patient was diagnosed with an arteriovenous fistula in the neck region. Non-invasive three-dimensional imaging, including computed tomography and ultrasonography, was used for guidance of percutaneous closure of the fistula and evaluation of the final outcome.

Step changes in angiographic imaging are not commonplace. Since the move from analogue to digital and flat detector plates, two-dimensional imaging technology has certainly evolved but not jumped forward. Of all the routine imaging techniques used in cardiology, angiography has been the last modality to embrace the third dimension. Although the development of rotational angiography was initially for the benefit of neuroimaging and fusion of cross sectional datasets was aimed at the treatment of descending aortic pathology, interventional physicians in congenital and structural cardiology have immersed themselves in this technology over the last 10 years. Like many disruptive technologies, its introduction has divided opinion. We aimed to explore the mindset of those in the field of interventional cardiology who are driving imaging forward. These structured interviews recorded during the 21 st Pediatric and Adult Interventional Cardiac Symposium illustrate the challenges and sticking points as well as giving an insight into the direction of travel for three-dimensional imaging and fusion techniques. Covering a wide range of career development, seniority and experience, the interviewees in this article are probably responsible for the majority of the published literature on invasive three-dimensional imaging in congenital heart disease., Competing Interests: G. Krings is a member of the Siemens Advisory Board. The other authors declare no conflict of interest., (Copyright: © 2019 Termedia Sp. z o. o.)

Introduction: Until recently, two-dimensional (2D) angiography was the mainstay of guidance for percutaneous pulmonary valve implantation (PPVI). Recent advances in fusion software have enabled direct fusion of pre-intervention imaging, magnetic resonance imaging (MRI) or computed tomography (CT) scans, to create a reliable three-dimensional (3D) roadmap for procedural guidance., Aim: To report initial two-center experience with direct 2D-3D image fusion for live guidance of PPVI with MRI- and CT-derived 3D roadmaps., Material and Methods: We performed a prospective study on PPVIs guided with the new fusion imaging platform introduced in the last quarter of 2015., Results: 3D guidance with an MRI- ( n = 14) or CT- ( n = 8) derived roadmap was utilized during 22 catheterizations for right ventricular outflow tract balloon sizing ( n = 7) or PPVI ( n = 15). Successful 2D-3D registration was performed in all but 1 patient. Six (27%) patients required intra-procedural readjustment of the 3D roadmap due to distortion of the anatomy after introduction of a stiff wire. Twenty-one (95%) interventions were successful in the application of 3D imaging. Patients in the CT group received less contrast volume and had a shorter procedural time, though the differences were not statistically significant. Those in the MRI group had significantly lower weight adjusted radiation exposure., Conclusions: With intuitive segmentation and direct 2D-3D fusion of MRI or CT datasets, VesselNavigator facilitates PPVI. Our initial data show that utilization of CT-derived roadmaps may lead to less contrast exposure and shorter procedural time, whereas application of MRI datasets may lead to lower radiation exposure., Competing Interests: The German Heart Center Berlin holds a research contract for the use and assessment of the VesselNavigator image fusion software and acts as a reference center for Philips, The Netherlands.

Introduction: Diagnosis of contrast induced-nephropathy (CIN) by a classic renal biomarker such as creatinine concentration can be delayed because of various factors that can influence this marker. Changes in new biomarkers such as neutrophil-gelatinase associated lipocalin (NGAL) and cystatin C are postulated to be more sensitive for recognizing patients prone to CIN-acute kidney injury (AKI)., Aim: To investigate the role of NGAL and cystatin C as early biomarkers in the diagnosis of kidney injury after cardiac catheterisation., Material and Methods: The study group consisted of 50 patients with congenital heart malformation admitted for scheduled cardiac catheterisation. The biomarkers serum creatinine, serum NGAL and serum cystatin C were tested at 5 time-points sequentially from start to 48 h after the procedure., Results: Significant changes were noted during the research in the serum creatinine concentration ( p < 0.001) and serum NGAL concentration ( p < 0.001). CIN-AKI, diagnosed by the modified Schwartz formula, occurred in 16 (32%) patients after 24 h and in 8 (16%) after 48 h. Subsequent analysis showed that serum creatinine significantly rose in the first 2 h of the study with simultaneous reduction in the eGFR. Maximum growth in serum NGAL occurred at 6 h after contrast administration and then returned to the baseline values at 24 h. Serum cystatin C level did not significantly change during the study., Conclusions: We observed a transient decrease in eGFR and a rise of serum NGAL after 2 h but NGAL was most pronounced at 6 h after the procedure. The potential role of cystatin C as a biomarker of CIN-AKI was not proved., Competing Interests: The authors declare no conflict of interest.

Introduction: Creation of a fenestration during completion of a total cavopulmonary connection (TCPC) has been associated with a reduction in early mortality and morbidity. However, the long-term benefits are negated by an associated limitation in exercise tolerance and the potential risks of thrombo-embolic complications. We sought to describe the safety and efficacy of an Amplatzer Duct Occluder II (ADO II) for transcatheter fenestration closure following TCPC., Material and Methods: Between January 2000 and July 2014, 102 patients underwent percutaneous closure of extra-cardiac TCPC fenestrations with a range of devices. Patients in whom fenestration closure was performed with an ADO II and who had at least 6 months of follow-up were included in this study., Results: Forty-seven patients had successful fenestration occlusion with an ADO II. The mean oxygen saturation and mean systemic venous pressures increased from 84.8 ±6.1% before to 97.6 ±2.9% ( p < 0.001) after and from 14.2 ±2.15 mm Hg before to 15.6 ±2.2 mm Hg after closure ( p < 0.001). Eight patients developed heart failure symptoms, managed by optimization of medical therapy, with 1 patient requiring device removal to reopen the fenestration. Color Doppler transthoracic echocardiography demonstrated residual flow across the device in 18 (38%), 10 (22%), 5 (11%) and 4 (9%) patients before discharge, at 1 and 6 months, and at the latest outpatient visit, respectively., Conclusions: The ADO II can be safely and effectively used to close fenestrations in extra-cardiac type Fontan completions. Many of the design features of this device confer potential benefit in this population., Competing Interests: The authors declare no conflict of interest.

Background: Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or pulmonary artery (PA) surgical interventions. The objective was to present immediate and long-term outcomes of trans¬cutaneous pulmonary valve replacement., Methods: Between 06/2009 and 06/2016, 46 patients underwent TPVR. Initial diagnoses included tetralogy of Fallot, common arterial trunk, transposition of great arteries post Rastelli correction, left ventricle outflow obstruction after Ross operation, pulmonary atresia, and isolated dysplastic pulmonary valve stenosis. Thirty eight (78%) patients had previously implanted conduits in the pulmonary position, the rest had either RVOT patch reconstruction (n = 6; 13%) or biological valve implantation (n = 2; 4%). They presented primarily with pulmonary stenosis (n = 18; 39%) or regurgitation (n = 28; 60%)., Results: All procedures were successful - 44 Melody and 2 Edwards-Sapien valves were implanted. Before each procedure exclusion of potential coronary compression and RVOT prestenting was performed. Significant RVOT systolic gradient reduction (from 35.3 ± 19.5 to 13.5 ± 7.1 mm Hg; p < 0.001) and decrease of right to left ventricle systolic pressure ratio from 0.58 ± 0.18 to mean 0.37 ± 0.1 (p < 0.001) was achieved. Also, in every patient PA-RVOT competence was restored, with minor in¬competence in only a few patients. Post procedure follow-up ranged from 2 to 86 (mean 35.2) months. Follow-up fluoroscopy or chest X-ray revealed 6 stent fractures (2 stent defragmentation - with only 1 significant valve stenosis)., Conclusions: Transcutaneous pulmonary valve replacement is a safe procedure with encouraging results, it also enables deferring surgical reintervention in the majority of patients.

This case describes a successful percutaneous stent implantation to critical stenosis of the right ventricle outflow tract in a female neonate with tetralogy of Fallot. At the time of the procedure she had poor development of the pulmonary arteries (McGoon and Nakata index 1.45 and 120, respectively). Stent implantation ensured an immediate increase in oxygen saturation level, and the physiological pulsating blood inflow caused good development of the pulmonary arteries during 12 months of follow up (McGoon 2.5; Nacata Index 436). After this time she was qualified for surgery and underwent surgical correction without using a patch or conduit implantation.

Due to the complex anatomical and haemodynamic consequences of hypoplastic left heart syndrome (HLHS), patients with the condition require multistage surgical and supportive interventional treatment. Percutaneous interventions may be required between each stage of surgical palliation, sometimes simultaneously with surgery as hybrid interventions, or after completion of multistage treatment. Recent advances in the field of interventional cardiology, including new devices and techniques, have significantly contributed to improving results of multistage HLHS palliation. Knowledge of the potential interventional options as well as the limitation of percutaneous interventions will enable the creation of safe and effective treatment protocols in this highly challenging group of patients. In this comprehensive review we discuss the types, goals, and potential complications of transcatheter interventions in patients with HLHS.

Despite advances in surgical techniques, right ventricular outflow tract (RVOT) conduits are prone to fail over time. Percutaneous pulmonary valve implantation was introduced to expand the lifetime of these conduits and to decrease the number of open heart operations during a patient's lifetime. The procedure can be performed with excellent results; however, serious complications such as coronary arterial compression and conduit rupture have been reported. We present percutaneous treatment of a patient after Ross-Konno operation with RVOT conduit dysfunction and a potentially problematic course of the left anterior descending artery.

We report a case of a 19-year-old patient with double outlet right ventricle (RV) and recurrent giant RV outflow tract pseudoaneurysm, after multiple redo surgery. The patient underwent implantation of a 10 mm Amplatzer Septal Occluder to close the pseudoaneurysm. Postinterventional echocardiography revealed dislocation of the device into the cavity of the pseudoaneurysm. Consecutive computed tomography enabled three-dimensional measurements of the pseudoaneurysmand its orifice and resulted in implantation of a 20 mm occluder.

In presented case rotational angiography with three dimensional reconstruction (3DRA) was used for diagnostic and control angiograms during covered CP stent implantation in patient with critical aortic coarctation and patent ductus arteriosus. Administering less contrast then for standard two perpendicular projections, good quality images were obtained in at least seven projections.

We present the case of a 12 month-old infant with critical coarctaion of aorta, arch hypoplasia, atrial and ventricular septal defects who underwent interventional treatment with stent implantation due to recoarctation of aorta. In neonatal period the patient went through complete surgical correction. At the age of four months balloon angioplasty of recurrent coractation was conducted. Eight months later the patient was hospitalised with heart failure (HF) symptoms. Clinical and echographic examination confirmed critical stenosis of aortic isthmus. We performed heart catheterisation with stent implantation to transverse and descending arch with immediate reduction of gradient from 45 mm Hg to 0 mm Hg, widening of the isthmus from 2 to 8 mm and gradual regression of HF symptoms. At 3 years of observation the patient has not presented with any signs of recurrence of the stenosis. The child remains free of HF symptoms with arterial hypertension controlled with pharmacotherapy and with good left ventricular function on echocardiographic examination.

Background: Presence of a restrictive interatrial communication in patients with univentricular anatomy significantly affects surgical outcomes. In patients with univentricular hearts, wide open atrial communication leads to lower pulmonary artery pressure, which is one of the most important factors influencing the success of bidirectional Glenn and Fontan operations. In some patients, recurrence of restricted interatrial communication can be observed despite initially successful interventional or surgical creation of unrestrictive interatrial communication., Aim: To evaluate efficacy of stent implantation into the interatrial septum in patients with univentricular heart and a secondary restriction of interatrial communication., Methods: In 2006-2010, we created unrestrictive interatrial communication by stent implantation into the interatrial septum in 7 children with univentricular anatomy with systemic right ventricle (4 patients with hypoplastic left heart syndrome and 3 patients with mitral atresia). In all patients we diagnosed recurrent restriction of interatrial communication despite prior surgical or interventional creation of unrestrictive interatrial communication. Patient age at stent implantation was 3 to 30 months. Maximal systolic pressure gradient between the left and the right atrium was 6-29 mm Hg and left atrial pressure ranged from 20/17/19 mm Hg to 40/29/32 mm Hg. In all patients, we implanted a Palmaz-Genesis stent (length 18-29 mm) with subsequent balloon redilatation., Results: In all 7 patients, we created unrestrictive interatrial communication with mean pressure gradient reduction from 13.14 mm Hg to 0.86 mm Hg (p < 0.006). Mean interatrial communication diameter increased from 4.14 mm to 10.57 mm (p < 0.0001)., Conclusions: Percutaneous stent implantation into the interatrial septum in children with univentricular heart and secondary restriction of interatrial communication is a safe and effective method. Kardiol Pol 2011; 69, 11: 1137-1141.