Successful complex percutaneous intervention in patient with Fontan circulation and severe heart failure: A case report.

We report the case of a successful complex percutaneous intervention in a patient with Fontan circulation and severe heart failure. The patient presented with cyanosis; Fontan conduit stenosis was detected, and the fenestration was patent. The complex interventional procedure allowed for a long-term stabilization of the patient's condition.

Left ventricular systolic function impairment in children after balloon valvuloplasty for congenital aortic stenosis assessed by 2D speckle tracking echocardiography.

AIMS: The aim of the study was to evaluate left ventricular (LV) remodeling and systolic function using two-dimensional speckle tracking echocardiographic (2D STE) imaging in children at a long-term (more than 36 months, 107.5±57.8 months) after balloon valvuloplasty for aortic stenosis (BAV). METHODS AND RESULTS: 40 patients (mean age 9,68 years, 75% male) after BAV and 62 control subjects matched to the age and heart rate were prospectively evaluated. The 2D STE assessment of LV longitudinal and circumferential strain and strain rate was performed. Left ventricular eccentric hypertrophy (LVEH) was diagnosed in 75% of patients in the study group. Left ventricular ejection fraction (LVEF) was normal in all patients. In study group, global longitudinal strain (GLS), global longitudinal strain rate (GLSr) were significantly lower compared with the controls: GLS (-19.7±2.22% vs. -22.3±1.5%, P< 0.001), GLSr (-0.89±0.15/s vs. -1.04 ±0.12/s, P < 0.001). Regional (basal, middle and apical segments) strain and strain rate were also lower compared with control group. Global circumferential strain (GCS), global circumferential strain rate (GCSr) as well as regional (basal, middle and apical segments) strain and strain rate were normal. Multivariable logistic regression analysis included: instantaneous peak systolic Doppler gradient across aortic valve (PGmax), grade of aortic regurgitation (AR), left ventricular mass index (LVMI), left ventricular relative wall thickness (LVRWT), left ventricular end-diastolic diameter (LVEDd), peak systolic mitral annular velocity of the septal and lateral corner (S'spt, S'lat), LVEF before BAV and time after BAV and showed that the only predictor of reduced GLS was LV eccentric hypertrophy [odds ratio 6.9; (95% CI: 1.37-12.5), P = 0.045]. CONCLUSION: Patients at long-term observation after BAV present the subclinical LV systolic impairment, which is associated with the presence of its remodeling. Longitudinal deformation is the most sensitive marker of LV systolic impairment in this group of patients.

Can we predict potentially dangerous coronary patterns in patients with transposition of the great arteries after an arterial switch operation?

INTRODUCTION: Coronary artery complications are the main reason for early mortality after an arterial switch operation. Late complications are relatively rare, and there is no consensus regarding the need or indications for routine follow-up coronary artery evaluations or the best first-line assessment modality. The aim of this study was to present the long-term post-operative frequency of coronary abnormalities in asymptomatic patients with transposition of the great arteries discovered by coronary CT angiography and potential "red flags" revealed by other examinations. PATIENTS AND METHODS: A group of 50 consecutive asymptomatic patients who underwent routine long-term coronary artery evaluation after an arterial switch operation according to our institutional protocol were qualified for this study. This routine in-hospital visit included a detailed medical interview, electrocardiography, echocardiography, Holter electrocardiography examinations, and laboratory and cardiopulmonary exercise tests. Patients who showed significant abnormalities were qualified for perfusion scintigraphy. RESULTS: Unfavourable coronary abnormalities were detected in 30 patients (60%) and included ostial stenosis, muscular bridge, coronary fistula, interarterial course, proximal kinking, high ellipticity index, proximal acute angulation (<30 degree) of the left coronary artery, and proximal acute angulation of the right coronary artery. These features could not be predicted based on the medical interviews, surgical reports, or non-invasive screening test results. CONCLUSION: Complex coronary configurations with potentially dangerous coronary features are common in patients with transposition after an arterial switch operation. Such high-risk patients cannot be identified indirectly, and coronary CT angiography provides accurate information that is useful for post-operative management.

Glomerular filtration decrease after diagnostic cardiac catheterisation in children with congenital cardiac malformation - the role of serum creatinine, cystatin C, neutrophil gelatinase and urine output monitoring.

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.

Stenting of the inter-atrial septum in infants and small children: Indications, techniques and outcomes.

OBJECTIVES: To evaluate the procedural success and outcome of inter-atrial stenting. BACKGROUND: Inter-atrial stenting has been shown to be an effective way to maintain inter-atrial blood flow, however it is considered a high risk procedure, usually performed urgently in patients with significant hemodynamic compromise. METHODS: Between September 2004 and August 2016, inter-atrial stenting was attempted in 29 children. Procedural, clinical, and follow-up data were collected retrospectively. RESULTS: The procedures were completed successfully in 27 patients. Twenty-five procedures were undertaken percutaneously, with the remaining four being performed as hybrid procedures. The patients were considered as high risk for adverse events (82% scored as CRISP 4 and 5) with four deaths during the first 24 hr (14%). Procedural complications occurred in eight patients (28%) with related death in three patients (10%). One further patient died after an uncomplicated technically successful stent implantation performed as a salvage procedure. Procedural complications (71% vs. 14%) and mortality (43% vs. 5%) were higher in those, who weighed 3 kg or less (P < 0.05). Patency of the stents was maintained until planned staged surgery in 22 patients at a mean of 302 days. Three patients underwent further balloon dilation for flow restriction at 58-201 days. In two un-operated patients the stents remained patent at follow-up. One patient with severe pulmonary hypertension died with a patent stent. CONCLUSIONS: Inter-atrial stenting produces reliable patency with a very good success rate. Morbidity and mortality were related to low weight at the time of the procedure.

Magnetic resonance and computed tomography imaging fusion for live guidance of percutaneous pulmonary valve implantation.

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.

Coronary Artery Anomalies in Patients With Transposition of the Great Arteries and Their Impact on Postoperative Outcomes.

BACKGROUND: Coronary anomalies are frequently present in children with transposition of the great arteries (TGA). Such anomalies significantly increase the complexity of arterial switch operations and may have an effect on postoperative outcomes. In this study, we aimed to assess the frequency of coronary anomalies in children with TGA and describe their effect on postoperative outcomes. METHODS: All patients with TGA who underwent an arterial switch operation between 1991 and 2015 were consecutively enrolled in this study. Patient coronary patterns were obtained from corresponding operative reports and analyzed to determine their associations with adverse postoperative outcomes. RESULTS: The study included 715 patients with isolated and complex TGA. Coronary anomalies were present in 33.7% of patients and were significantly correlated with the side-by-side configuration of the great arteries. Coronary sinuses with more than 1 ostium were associated with a significantly increased risk of postoperative death (hazard ratio [HR], 2.58), and coronary arteries with a single ostium were associated with an increased risk of postoperative reinterventions (HR, 2.49). In contrast, the trap-door technique of coronary artery transplantation was associated with a reduced risk of reinterventions during postoperative follow-up (HR, 0.40). Complex coronary anomalies were significantly associated with postoperative coronary events (HR, 2.12). CONCLUSIONS: With the exception of patients whose circumflex artery branches off of the right coronary artery, an anomaly that clearly has no effect on postoperative follow-up, patients with unusual coronary patterns are at higher risk for adverse postoperative outcomes than patients with normal coronary anatomy and must be monitored carefully.

Novel Three-Dimensional Image Fusion Software to Facilitate Guidance of Complex Cardiac Catheterization : 3D image fusion for interventions in CHD.

We report initial experience with novel three-dimensional (3D) image fusion software for guidance of transcatheter interventions in congenital heart disease. Developments in fusion imaging have facilitated the integration of 3D roadmaps from computed tomography or magnetic resonance imaging datasets. The latest software allows live fusion of two-dimensional (2D) fluoroscopy with pre-registered 3D roadmaps. We reviewed all cardiac catheterizations guided with this software (Philips VesselNavigator). Pre-catheterization imaging and catheterization data were collected focusing on fusion of 3D roadmap, intervention guidance, contrast and radiation exposure. From 09/2015 until 06/2016, VesselNavigator was applied in 34 patients for guidance (n = 28) or planning (n = 6) of cardiac catheterization. In all 28 patients successful 2D-3D registration was performed. Bony structures combined with the cardiovascular silhouette were used for fusion in 26 patients (93%), calcifications in 9 (32%), previously implanted devices in 8 (29%) and low-volume contrast injection in 7 patients (25%). Accurate initial 3D roadmap alignment was achieved in 25 patients (89%). Six patients (22%) required realignment during the procedure due to distortion of the anatomy after introduction of stiff equipment. Overall, VesselNavigator was applied successfully in 27 patients (96%) without any complications related to 3D image overlay. VesselNavigator was useful in guidance of nearly all of cardiac catheterizations. The combination of anatomical markers and low-volume contrast injections allowed reliable 2D-3D registration in the vast majority of patients.

Stent implantation to ductus arteriosus in a patient with interrupted aortic arch guided by CT image overlay.

A 15-day-old premature patient with ventricular septal defect and interrupted aortic arch type B underwent "hybrid" initial treatment consisting of bilateral pulmonary artery banding followed by stenting of the ductus arteriosus. A pre-registered CT scan was re-purposed with a new three-dimensional image fusion software (VesselNavigator) to create a roadmap for stent delivery.

Fenestration closure with Amplatzer Duct Occluder II in patients after total cavo-pulmonary connection.

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.

Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation.

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.

Reoperations and catheter interventions in patients with transposition of the great arteries after the arterial switch operation.

OBJECTIVES: Reoperations and catheter interventions after the arterial switch operation (ASO) are relatively rare, but their frequency varies among different centres. They significantly impact the postoperative course of children with transposition of the great arteries (TGA). The aim of this study was to assess the frequency of reoperations and catheter interventions in patients with TGA after the ASO and to identify the potential risk factors. METHODS: For this retrospective case review study we included all consecutive 715 patients with TGA who underwent the ASO in the Department of Cardiac Surgery between the years 1991 and 2015. All of the surgical procedures were performed by one cardiac surgery team led by J.J.M., using the same surgical technique with his own specific modifications. RESULTS: The overall early mortality after the ASO was 7.4%; late mortality occurred in 15 cases (2.3%) and the mean clinical follow-up of our cohort was 10.5 years. Early reoperations (<30 days after surgery) were performed in 37 patients (5.1%). Reoperations were performed in 31 patients (4.7% of survivors), and their risk factors were previous early reoperation and left ventricle outflow tract obstruction, while isolated TGA reduced the risk of reoperations. Catheter interventions were performed in 25 patients (3.8% of survivors). In the majority of the cases, the indications for percutaneous procedures were pulmonary stenosis and recoarctation of the aorta. The statistically significant risk factors were aortic arch anomalies associated with TGA and neopulmonary artery anastomosis with a patch, while isolated TGA decreased the risk of reintervention. Freedom from cumulative reinterventions after the ASO was 90.4% at 5 years; 88.0% at 10 years; 86.5% at 15 years and 86.5% from 20 to 25 years. CONCLUSION: The frequency of reoperations and percutaneous interventions in patients with TGA after the ASO remains low. The majority of the procedures are performed because of pulmonary stenosis and recoarctation of aorta. Cardiac anomalies associated with TGA have a significant impact on the incidence of reoperation and reintervention.

Initial experience with live three-dimensional image overlay for ductal stenting in hypoplastic left heart syndrome.

AIMS: Precise visualisation of ductal morphology and adjacent vessels is crucial for accurate stent choice and placement during hybrid palliation of hypoplastic left heart syndrome (HLHS). We aimed to describe our initial experience with live three-dimensional reconstruction overlay derived from rotational angiography (RA) for ductal stenting in HLHS. METHODS AND RESULTS: We carried out a retrospective review of ductal stenting in 18 newborns with HLHS, including six patients with 3D reconstruction overlay used to guide the intervention. The median age at the intervention was 20 days (range 13-31 days) and the median weight was 3.25 kg (range 3-4 kg). Eleven RA runs were performed, pre and post stent implantation in five patients and before the intervention in one patient. 3D reconstructions from all RA runs had sufficient image quality to allow stent placement without additional contrast injections. Comparison with 2D angiography-guided ductal stenting showed similar contrast usage, with the 2D angiography patients receiving a higher radiation dose. CONCLUSIONS: Three-dimensional rotational angiography provides accurate visualisation of the ductal morphology and nearby structures. Three-dimensional reconstruction overlay with clear landing points enabled precise stent implantation with no additional contrast injections and lower radiation doses than conventional angiography in our patients.