Physician-Modified Endograft-Facilitated Transcatheter Pulmonary Valve Replacement in Large Right Ventricular Outflow Tract.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) in patients with a congenital or acquired abnormality resulting in enlarged right ventricular outflow tract (RVOT) is challenging and may preclude treatment with dedicated devices. We describe a technique using a physician-modified endograft to facilitate TPVR. METHODS: Six patients underwent physician-modified endograft-facilitated TPVR for severe symptomatic pulmonary insufficiency with enlarged RVOT. The fenestration was created in a commercially available endograft before implantation, which was then deployed from the dominant branch pulmonary artery into the RVOT, with the fenestration aligned with the ostium of the nondominant pulmonary artery. A covered stent was placed through the fenestration into the nondominant branch pulmonary artery, and a transcatheter heart valve was deployed within the endograft at the level of the original pulmonary valve. RESULTS: Four patients had tetralogy of Fallot, 1 had pulmonary atresia, and 1 had rheumatic valve disease. The RVOT/main pulmonary artery was severely enlarged (diameter, 44.2 [43.5-50.6] mm). All patients had reduced right ventricular (RV) function and dilated RVs (RV end-diastolic volume, 314 [235-316] mL). Successful endograft, covered stent, and transcatheter heart valve deployment were achieved in all cases without stent/valve embolization, vascular complications, or bleeding complications. At 30 days, 1 patient had mild pulmonary insufficiency, while others had none. The RV size measured by echocardiography was significantly reduced after TPVR (RV area, 34.4 [baseline] versus 29.0 [pre-discharge] versus 25.3 [30 days] cm2; P=0.03). During median follow-up of 221.5 (range, 29-652) days, there were no deaths or need for pulmonary valve reintervention. One patient developed severe tricuspid regurgitation due to entrapment of the anterior tricuspid leaflet by the endograft. The patient underwent successful tricuspid replacement and resection of the offending endograft with preservation of the pulmonary valve prosthesis. CONCLUSIONS: Simple fenestration of an off-the-shelf endograft and associated covered stent placement through the fenestration allows TPVR for patients with dysfunctional native or patch-repaired pulmonary valves and RVOT enlargement.

Comparison of treatment strategies for neonates with tetralogy of Fallot and pulmonary atresia.

OBJECTIVE: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established. METHODS: Neonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth. RESULTS: Of 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤ .001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P = .003) but was similar after CR (P = .837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR. CONCLUSIONS: In neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation.

Right ventricular outflow tract landing zone perimeter / circularised diameter - new imaging standards in pulmonary valve replacement reporting.

BACKGROUND: Right ventricular outflow tract intervention spans transcatheter, surgical, or hybrid pulmonary valve replacement methodologies. Standardised pre-procedure workup includes cardiac MRI to identify an intended valve site (landing zone). Our institutional practice includes measurement of the right ventricular outflow tract perimeter (circumference) of this site in end-systole. Our primary aim was to compare patients by their perimeter values to the palliative interventions performed (transcatheter versus surgical/hybrid methodologies). METHODS: Retrospective review of patients undergoing pulmonary valve replacement from January 2017 to 2021. We performed perimeter measurements at the intended valve site on advanced imaging; the outcomes of interventions were outlined via descriptive and statistical analyses. RESULTS: A total of 37 patients underwent pulmonary valve replacement that met study criteria - 21 transcatheter, 7 surgical, and 9 hybrid. Median age at intervention was 26 years (range 8-70). The mean end-systolic perimeter of the transcatheter cohort was 88.9 ± 8.7 mm and in the surgical/hybrid cohort measured 106.6 ± 7.5 mm. For the transcatheter cohort, the median "circularised" diameter derived from the perimeter measurement (divided by π) was 27.7 mm (range 24.3-32.4). Notably, this correlated (r = 0.93, p < 0.01) with the median diameter of the narrowest region during actual transcatheter right ventricular outflow tract balloon sizing (lateral imaging) of 27.1 mm (range 23.2-30.1). CONCLUSIONS: Right ventricular outflow tract perimeter measurement to determine circularised diameter is useful in planning pulmonary valve replacement in terms of candidacy of transcatheter versus the need for a surgical/hybrid approach. The circularised diameter correlates with transcatheter right ventricular outflow tract balloon sizing.

Comparison of Patent Ductus Arteriosus Stent and Blalock-Taussig Shunt as Palliation for Neonates with Sole Source Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock-Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p < 0.001).

Direct Percutaneous Transthoracic Cardiac Access for Recanalization of Longstanding Branch Pulmonary Artery Atresia.

We describe the use of direct percutaneous cardiac access to recanalize an atretic right pulmonary artery in an adolescent with complex congenital heart disease and right heart failure. This case highlights the problems associated with loss of central venous access and potential advantages of a direct cardiac approach to catheterization. (Level of Difficulty: Intermediate.).

Racial and ethnic disparities in coronary, vascular, structural, and congenital heart disease.

Cardiovascular disease (CVD) remains the leading cause of death in the United States. However, percutaneous interventional cardiovascular therapies are often underutilized in Blacks, Hispanics, and women and may contribute to excess morbidity and mortality in these vulnerable populations. The Society for Cardiovascular Angiography and Interventions (SCAI) is committed to reducing racial, ethnic, and sex-based treatment disparities in interventional cardiology patients. Accordingly, each of the SCAI Clinical Interest Councils (coronary, peripheral, structural, and congenital heart disease [CHD]) participated in the development of this whitepaper addressing disparities in diagnosis, treatment, and outcomes in underserved populations. The councils were charged with summarizing the available data on prevalence, treatment, and outcomes and elucidating potential reasons for any disparities. Given the huge changes in racial and ethnic composition by age in the United States (Figure 1), it was difficult to determine disparities in rates of diagnosis and we expected to find some racial differences in prevalence of disease. For example, since the average age of patients undergoing transcatheter aortic valve replacement (TAVR) is 80 years, one may expect 80% of TAVR patients to be non-Hispanic White. Conversely, only 50% of congenital heart interventions would be expected to be performed in non-Hispanic Whites. Finally, we identified opportunities for SCAI to advance clinical care and equity for our patients, regardless of sex, ethnicity, or race.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease.

BACKGROUND: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. METHODS: Retrospective chart review of adjustable PAB-outline anatomical variants palliated and patient outcomes. RESULTS: Sixteen patients underwent dilatable PAB-median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication-Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). CONCLUSIONS: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients-allowing for interval growth or even serving as the definitive palliation.

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

BACKGROUND: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention. OBJECTIVES: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies. METHODS: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment. RESULTS: The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002). CONCLUSIONS: In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.

Rerouting of Cerebral Circulation: Extensive Transcatheter Aortic Arch Revision.

We describe an adolescent with long-standing atresia of the head/neck arteries and severe aortic coarctation. Because of progressive symptoms, a series of interventions was undertaken to provide direct aorta-to-carotid artery flow and coarctation treatment. This case highlights the unusual physiological features associated with atresia of all head and neck arteries. (Level of Difficulty: Advanced.).

Impact of Palliation Strategy on Interstage Feeding and Somatic Growth for Infants With Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative.

Background In infants with ductal-dependent pulmonary blood flow, the impact of palliation strategy on interstage growth and feeding regimen is unknown. Methods and Results This was a retrospective multicenter study of infants with ductal-dependent pulmonary blood flow palliated with patent ductus arteriosus (PDA) stent or Blalock-Taussig shunt (BTS) from 2008 to 2015. Subjects with a defined interstage, the time between initial palliation and subsequent palliation or repair, were included. Primary outcome was change in weight-for-age Z-score. Secondary outcomes included % of patients on: all oral feeds, feeding-related medications, higher calorie feeds, and feeding-related readmission. Propensity score was used to account for baseline differences. Subgroup analysis was performed in 1- (1V) and 2-ventricle (2V) groups. The cohort included 66 PDA stent (43.9% 1V) and 195 BTS (54.4% 1V) subjects. Prematurity was more common in the PDA stent group (P=0.051). After adjustment, change in weight-for-age Z-score did not differ between groups over the entire interstage. However, change in weight-for-age Z-score favored PDA stent during the inpatient interstage (P=0.005) and BTS during the outpatient interstage (P=0.032). At initial hospital discharge, PDA stent treatment was associated with all oral feeds (P<0.001) and absence of feeding-related medications (P=0.002). Subgroup analysis revealed that 2V but not 1V patients demonstrated significant increase in weight-for-age Z-score. In the 2V cohort, feeding-related readmissions were more common in the BTS group (P=0.008). Conclusions In infants with ductal-dependent pulmonary blood flow who underwent palliation with PDA stent or BTS, there was no difference in interstage growth. PDA stent was associated with a simpler feeding regimen and fewer feeding-related readmissions.

Risk Factors for Red Blood Cell Transfusions in Children Undergoing Cardiac Catheterization.

OBJECTIVE: To identify risk factors associated with risk of red blood cell transfusions (RBCTs) following pediatric cardiac catheterizations. STUDY DESIGN: We performed a review of all pediatric cardiac catheterizations from 2012 to 2017. The primary endpoint was RBCT within 72 hours of pediatric cardiac catheterization. Patient and procedural factors were reviewed. Generalized linear modelling was performed to describe interactions among relevant risk factors. RESULTS: In total, 831 RBCTs occurred within 72 hours of 6028 pediatric cardiac catheterizations (13.8%). Univariate analysis revealed that the prevalence of RBCT was highest among infants (37.6% incidence of RBCT) and among those with higher estimated blood loss as a percent of blood volume (P = .03). Among infants, multivariate analysis revealed that weight (OR 0.72; 95% CI 0.63-0.81), complex 2-ventricle (OR 3.14, 95% CI 2.18-4.57), and single ventricle status (OR 5.21, 95% CI 3.42-8.01) were associated with risk of RBCT. Inpatient infants from intensive care (OR 4.74; 95% CI 3.49-6.49) or stepdown units (OR 2.33; 95% CI 1.58-3.46) were at higher risk. Length of procedure (OR 2.57; 95% CI 2.03-3.26) and oxygen saturation (OR 0.98; 95% CI 0.97-0.99; P < .01) were also associated with RBCTs. CONCLUSIONS: Hospitalized infants with single ventricle or complex 2-ventricle anatomy are at highest risk of RBCT. Length of procedure, blood loss, and oxygen saturations are additional risk factors associated with RBCT. Operators should consider these factors when planning pediatric cardiac catheterizations, particularly when exposure to RBCT is undesirable.

Comparison of Outcomes at Time of Superior Cavopulmonary Connection Between Single Ventricle Patients With Ductal-Dependent Pulmonary Blood Flow Initially Palliated With Either Blalock-Taussig Shunt or Ductus Arteriosus Stent: Results From the Congenital Catheterization Research Collaborative.

BACKGROUND: Patients with single ventricle anatomy and ductal-dependent pulmonary blood flow may be initially palliated with either modified Blalock-Taussig shunt (BTS) or ductus arteriosus stent (DAS). Comparisons of outcomes during the interstage period and at the time of superior cavopulmonary connection (SCPC) are lacking and may differ between palliation strategies. METHODS: Infants with single ventricle anatomy and ductal-dependent pulmonary blood flow palliated with either DAS or BTS from 2008 to 2015 were reviewed across 4 centers. Interstage outcomes, and for those who had SCPC, anatomy, hemodynamics, and perioperative clinical outcomes were compared. Thirty-five patients with DAS and 136 patients with BTS were included. RESULTS: At initial palliation, demographic, clinical variables, and pulmonary artery size were similar. Interstage death, transplant, or unplanned reintervention to treat cyanosis occurred in 25.7% of DAS and 35.8% of BTS, P=0.27. Reintervention was more common with DAS (48.6% versus 2.2%; P<0.001). Twenty-three DAS patients and 111 BTS patients underwent SCPC. Preoperative hemodynamics and overall pulmonary atresia growth were similar, although right pulmonary artery growth was better with DAS (change in z-score: 1.57 versus 0.65, P=0.026). SCPC intraoperative and postoperative courses were similar. CONCLUSIONS: In patients with single-ventricle anatomy and ductal-dependent pulmonary blood flow, interstage outcomes, hemodynamics before SCPC, and acute postoperative outcomes were similar. Overall reintervention was more common in the DAS group, driven by more frequent planned reintervention. Unplanned reintervention, death, and transplant were similar. Both groups demonstrated good pulmonary atresia growth. DAS is a reasonable initial palliative alternative to BTS in select patients.

Thromboprophylaxis strategies for children with single-ventricle circulations (superior or total cavo-pulmonary connections) after stent implantation.

OBJECTIVE: To define optimal thromboprophylaxis strategy after stent implantation in superior or total cavopulmonary connections. BACKGROUND: Stent thrombosis is a rare complication of intravascular stenting, with a perceived higher risk in single-ventricle patients. METHODS: All patients who underwent stent implantation within superior or total cavopulmonary connections (caval vein, innominate vein, Fontan, or branch pulmonary arteries) were included. Cohort was divided into aspirin therapy alone versus advanced anticoagulation, including warfarin, enoxaparin, heparin, or clopidogrel. Primary endpoint was in-stent or downstream thrombus, and secondary endpoints included bleeding complications. RESULTS: A total of 58 patients with single-ventricle circulation underwent 72 stent implantations. Of them 14 stents (19%) were implanted post-superior cavopulmonary connection and 58 (81%) post-total cavopulmonary connection. Indications for stenting included vessel/conduit stenosis (67%), external compression (18%), and thrombotic occlusion (15%). Advanced anticoagulation was prescribed for 32 (44%) patients and aspirin for 40 (56%) patients. Median follow up was 1.1 (25th-75th percentile, 0.5-2.6) years. Echocardiograms were available in 71 patients (99%), and advanced imaging in 44 patients (61%). Thrombosis was present in two patients on advanced anticoagulation (6.3%) and none noted in patients on aspirin (p = 0.187). Both patients with in-stent thrombus underwent initial stenting due to occlusive left pulmonary artery thrombus acutely post-superior cavopulmonary connection. There were seven (22%) significant bleeding complications for advanced anticoagulation and none for aspirin (p < 0.001). CONCLUSIONS: Antithrombotic strategy does not appear to affect rates of in-stent thrombus in single-ventricle circulations. Aspirin alone may be sufficient for most patients undergoing stent implantation, while pre-existing thrombus may warrant advanced anticoagulation.

Right ventricular myocardial performance index in pediatric patients with bronchopulmonary dysplasia-related pulmonary hypertension.

INTRODUCTION: This study aims to assess the utility of right ventricular myocardial performance index (RVMPI) as a potential echocardiographic tool to evaluate and serially follow patients with bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH). METHODS: We performed a retrospective chart review of all hemodynamic cardiac catheterizations performed January 2011 to December 2016 in patients born premature and with diagnosed BPD up to 4 years of age-excluding patients with significant congenital heart defects. Echocardiograms performed within 24 hours of the cardiac catheterization were reviewed, and a blinded RVMPI was calculated. The primary endpoint was correlation of invasive catheterization hemodynamics to noninvasive echocardiographic RVMPI measurement. RESULTS: A total of 49 individual patients met complete study criteria, and 10 of those patients underwent repeat cardiac catheterization. Median age at the time of assessment was 8 months (25%-75%, 4-18 months), and the cohort had a calculated RVMPI mean of 0.39 (±0.19), with 73% (43/59) having a RVMPI >0.28. A statistically significant correlation was found between the RVMPI and the baseline hemodynamics during catheterization with regard to the initial mean pulmonary arterial pressure (r = 0.58; P < 0.01) as well as the calculated pulmonary vascular resistance (r = 0.34; P = 0.01). CONCLUSIONS: This study demonstrates the utility of RVMPI for evaluation of PH in patients with prematurity and BPD. An increased RVMPI by noninvasive echocardiography was found to correlate with increased mean pulmonary arterial pressure and pulmonary vascular resistance measured during invasive cardiac catheterization. Large-scale validation of this study is being planned.

Differences in Cost of Care by Palliation Strategy for Infants With Ductal-Dependent Pulmonary Blood Flow.

BACKGROUND: In infants with ductal-dependent pulmonary blood flow, initial palliation with patent ductus arteriosus (PDA) stent or modified Blalock-Taussig (BT) shunt have comparable mortality but discrepant length of stay, procedural complication rates and reintervention burdens, which may influence cost. The relative economic impact of these palliation strategies is unknown. METHODS AND RESULTS: Retrospective study of infants with ductal-dependent pulmonary blood flow palliated with PDA stent (n=104) or BT shunt (n=251) from 2008 to 2015 at 4 centers of the Congenital Catheterization Research Collaborative. Inflation-adjusted inpatient hospital costs were calculated for first year of life using Pediatric Health Information System data. Costs derived from outpatient catheterizations not in Pediatric Health Information System were imputed. Costs were compared using propensity score-adjusted multivariable models, to account for baseline differences between groups. After propensity score adjustment, first year of life costs were significantly lower in PDA stent ($215 825 [190 644-244 333]) than BT shunt ($249 855 [230 693-270 609]) patients ( P=0.05). After addition of imputed costs, first year of life costs were not significantly different between PDA stent ($226 403 [200 274-255 941]) and BT shunt ($252 072 [232 955-272 759]) groups ( P=0.15). Patient characteristics associated with higher costs included: younger gestational age, genetic syndrome, noncardiac diagnoses, procedural complications, extracorporeal membrane oxygenation, duration of ventilation, intensive care unit and hospital length of stay and reintervention ( P≤0.02 for all). CONCLUSIONS: In this first multicenter comparative cost study of PDA stent or BT shunt as palliation for infants with ductal-dependent pulmonary blood flow, adjusted for baseline differences, PDA stent was associated with lower to equivalent costs over the first year of life. Combined with previous evidence suggesting clinical noninferiority, these findings suggest that PDA stent provides competitive health care value.

Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting.

OBJECTIVES: To devise a classification scheme for ductal morphology in patients with ductal dependent pulmonary blood flow (PBF) that can be used to assess outcomes. BACKGROUND: The impact of ductal morphology on outcomes following patent ductus arteriosus (PDA) stenting is not well defined. METHODS: Patients <1 year of age who underwent PDA stenting for ductal dependent PBF at the four centers comprising the Congenital Catheterization Research Collaborative (CCRC) were included. A classification scheme for PDA morphology was devised based on a tortuosity index (TI)-Type I (straight), Type II (one turn), and Type III (multiple turns). A subtype classification was used based upon the ductal origin. RESULTS: One hundred and five patients underwent PDA stenting. TI was Type I in 58, Type II in 24, and Type III in 23 PDAs, respectively. There was a significant association between ductal origin and vascular access site (p < 0.001). Procedure times and need for >1 stent did not differ based on TI. Greater TI was associated with pulmonary artery (PA) jailing (p = 0.003). Twelve (11.4%) patients underwent unplanned reintervention, more commonly with greater TI (p = 0.022) and PA jailing (p < 0.001). At the time of subsequent surgical repair/palliative staging, PA arterioplasty was performed in 32 patients, more commonly when a PA was jailed (p = 0.048). PA jailing did not affect PA size at follow up. CONCLUSIONS: The proposed qualitative and quantitative PDA morphology classification scheme may be helpful in anticipating outcomes in patients with ductal dependent PBF undergoing PDA stenting.

Technical factors are associated with complications and repeat intervention in neonates undergoing transcatheter right ventricular decompression for pulmonary atresia and intact ventricular septum: results from the congenital catheterisation research collaborative.

BACKGROUND: Transcatheter right ventricle decompression in neonates with pulmonary atresia and intact ventricular septum is technically challenging, with risk of cardiac perforation and death. Further, despite successful right ventricle decompression, re-intervention on the pulmonary valve is common. The association between technical factors during right ventricle decompression and the risks of complications and re-intervention are not well described. METHODS: This is a multicentre retrospective study among the participating centres of the Congenital Catheterization Research Collaborative. Between 2005 and 2015, all neonates with pulmonary atresia and intact ventricular septum and attempted transcatheter right ventricle decompression were included. Technical factors evaluated included the use and characteristics of radiofrequency energy, maximal balloon-to-pulmonary valve annulus ratio, infundibular diameter, and right ventricle systolic pressure pre- and post-valvuloplasty (BPV). The primary end point was cardiac perforation or death; the secondary end point was re-intervention. RESULTS: A total of 99 neonates underwent transcatheter right ventricle decompression at a median of 3 days (IQR 2-5) of age, including 63 patients by radiofrequency and 32 by wire perforation of the pulmonary valve. There were 32 complications including 10 (10.5%) cardiac perforations, of which two resulted in death. Cardiac perforation was associated with the use of radiofrequency (p=0.047), longer radiofrequency duration (3.5 versus 2.0 seconds, p=0.02), and higher maximal radiofrequency energy (7.5 versus 5.0 J, p<0.01) but not with patient weight (p=0.09), pulmonary valve diameter (p=0.23), or infundibular diameter (p=0.57). Re-intervention was performed in 36 patients and was associated with higher post-intervention right ventricle pressure (median 60 versus 50 mmHg, p=0.041) and residual valve gradient (median 15 versus 10 mmHg, p=0.046), but not with balloon-to-pulmonary valve annulus ratio, atmospheric pressure used during BPV, or the presence of a residual balloon waist during BPV. Re-intervention was not associated with any right ventricle anatomic characteristics, including pulmonary valve diameter. CONCLUSION: Technical factors surrounding transcatheter right ventricle decompression in pulmonary atresia and intact ventricular septum influence the risk of procedural complications but not the risk of future re-intervention. Cardiac perforation is associated with the use of radiofrequency energy, as well as radiofrequency application characteristics. Re-intervention after right ventricle decompression for pulmonary atresia and intact ventricular septum is common and relates to haemodynamic measures surrounding initial BPV.

Angiographic follow-up of infants and children undergoing percutaneous carotid artery interventions.

OBJECTIVE: The purpose of this study was to review the outcomes following the percutaneous carotid arterial (PCA) approach in infants and children with congenital heart disease. BACKGROUND: PCA access is becoming more commonly adopted following reports demonstrating it is a safe alternative to surgical carotid cutdown and even the femoral arterial route. However, follow-up outcomes after PCA remain unreported. METHODS: We reviewed all cases with PCA access and follow-up catheterizations which included carotid artery (CA) angiography between May 2012 until December 2016. We examined for evidence of CA stenosis at follow-up angiography and assessed any other CA complications associated with vascular access. RESULTS: There were 61 PCA catheterizations performed in 55 unique patients. Follow-up CA imaging with angiography was available in 43 patients (78%, 43/55). There was no vessel stenosis nor lumen irregularity in 28 (65%) patients. In 15 cases (35%), there was a mild degree of irregularity or narrowing by angiography (median 4.1%, range 2.3%-12.5%). Nine patients underwent repeat PCA catheterizations. Seven of these had no visible vascular stenosis on follow-up angiographic imaging, including a patient who was accessed three separate times from the CA. No statistically significant risk factors for developing mild CA stenosis were identified. CONCLUSIONS: PCA access for pediatric interventional catheterization appears to be safe with a very low rate of mild stenosis, and very few complications. Follow-up outcomes in our series are excellent, with a CA patency rate of 100%, even after multiple procedures. Mild CA stenosis was not associated with patient size or sheath introducer caliber. While the acute results from percutaneous CA catheterization have proven safe in recent literature, longer-term outcomes remain unreported. At our institution, the outcomes following percutaneous carotid access are associated with an excellent patency rate of 100%, even after multiple procedures on the same vessel. A low incidence of mild vessel stenosis can be appreciated on follow-up angiography.