Congenital Heart Interventions

Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Replacement in the US Investigational Device Exemption Trial. Jones TK, McElhinney DB, Vincent JA, Hellenbrand WE, Cheatham JP, Berman DP, Zahn EM, Khan DM, Rhodes JF Jr, Weng S, Bergersen LJ. Circ Cardiovasc Interv. 2022 Jan;15(1):e010852. doi: 10.1161/CIRCINTERVENTIONS.121.010852. Epub 2021 Dec 21. PMID: 34930015    Take Home Points: Fifty-eight of 149 patients enrolled in the Melody valve US IDE study made it to 10 years follow up Freedom from mortality at 10 years was 90% Freedom from valve reintervention at 10 years was 53% Higher risk of reintervention in younger patients, in patients with primary indication of stenosis, in patients with higher residual post valve gradient, and in patient who had more prior open-heart surgeries Freedom from TPV endocarditis was 81% and from all site endocarditis was 76% at 10 years Most common cause of death was endocarditis (5/11) Commentary from Dr. Thomas Zellers (Dallas, USA), section editor of Congenital Heart Disease Interventions and ACHD Journal Watch: Summary: This is an important study because it provides 10-year follow up on a subset of patients implanted with the Melody valve during the US IDE study. A total of 171 patients were enrolled in the trial and 149 of the patients were followed for a mean of 8.4 years (5.4-10.1 years). Fifty-eight (58) of the 149 patients had 10-year assessment information.   Estimated freedom from mortality at 10 years was 90%. Eleven patients died, 5 of those from endocarditis. At 10 years, freedom from any reintervention was 55%, freedom from any TPV intervention was 60% and freedom from reoperation was 79%. Freedom from reintervention declined in a shorter period of time in patients implanted younger than age 21. There was a higher risk of reintervention in unprotected (non-stented) conduits, when the primary indication for TPV was stenosis, in those patients with a higher peak to peak gradient post transcatheter valve implant and in those patients who had more open-heart surgeries prior to TPV implantation.   Peak to peak gradients after TPV implant were low throughout the study period. In addition, at 10 years follow up, most patients had none or trivial pulmonary regurgitation; only 26% had mild and 3% had moderate pulmonary regurgitation. The majority of patients (78%) were characterized as NYHA class I.   Endocarditis is a significant complication and occurred in 28 of 149 patients (19%) with an annualized incidence on the TPV of 2% per year.   Limitations: There was a smaller number of patients who made it to 10-year follow up, but this is not unexpected as the extended follow up past 5 years was voluntary. In addition, there was no CORE lab involved in evaluating the imaging results. Finally, the implant procedure changed during the study (for example, pre-stenting of conduits).       Echocardiographic evaluation of valve gradients (above) and valve insufficiency (below). Gradients change little over time and most patients have mild or less regurgitation out to 10 years.     Freedom from intervention by age group     Freedom from endocarditis Kaplan Meier curves  

Balloon dilatation versus surgical valvotomy for congenital aortic stenosis: a propensity score matched study. Auld BC, Donald JS, Lwin N, Betts K, Alphonso NO, Venugopal PS, Justo RN, Ward CJ, Konstantinov IE, Karl TR, Anderson BW. Cardiol Young. 2021 Dec;31(12):1984-1990. doi: 10.1017/S1047951121001281. Epub 2021 Apr 16. PMID: 33858544   Take Home Points: Balloon aortic valvuloplasty and surgical aortic valvotomy both achieve acute relief of aortic stenosis with aortic regurgitation being more common in the balloon valvuloplasty group. Freedom from reintervention is similar between groups. Balloon aortic valvuloplasty has a much better freedom from reintervention in the neonatal population in a propensity matched comparison. Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch: Congenital aortic stenosis (AS) continues to have significant morbidity and mortality despite improvements in transcatheter balloon aortic valvuloplasty (BAV-including lower profile balloons, two balloon technique, methods to achieve balloon stability) and surgical aortic valvotomy (SAV-including use of commissurotomy, leaflet edge thinning, debulking of nodular dysplasia, leaflet extension) over the last several decades. There continues to be significant debate surrounding which strategy should be first line therapy with the type of intervention typically dictated by center preference. Given this, a randomized controlled trial is challenging as the large majority undergo surgical repair or balloon valvuloplasty at any given center. This study performed a retrospective propensity score matched analysis from a primary surgical center and primary transcatheter center (both in Australia) to evaluate outcomes of BAV versus SAV in the treatment of AS.   From 2005-2016 65 patients (median age 92 days, IQR 21-924) underwent BAV and 77 patients (median age 167 days, IQR 38-2873) underwent SAV and met inclusion criteria (no other significant congenital heart disease that would impact the decision of what procedure would be performed). There were no significant differences in aortic valve morphology, age (p value 0.08, trend towards younger in BAV group), weight, or pre-procedure mean AS gradient by echocardiogram between groups. In the BAV group a balloon size similar to the annulus was chosen with two balloon technique utilized in patients with a larger annulus (12-13 mm) and balloon stabilization techniques (rapid RV pacing or administration of adenosine) when possible. The surgical technique utilized was determined by the surgeon intraoperatively.   SAV achieve a mean residual gradient by echocardiogram of 14.9 mmHg (IQR 10.7-21.4) while the gradient following BAV was 25.5 mmHg (IQR 16-31.5). There was no difference in mortality between groups. Moderate or greater aortic regurgitation was more common in the BAV group (15 patients) than the SAV group (1 patient). Three patients had severe aortic regurgitation post BAV and ultimately underwent an early Ross procedure as did the one SAV patient with moderate aortic regurgitation. Freedom from reintervention was similar between groups at 2 (75% for BAV, 74% for SAV), 4 (71% for BAV, 73% for SAV), and 8 years (62% for BAV, 63% for SAV). There was no association between initial type of intervention and need for reintervention. A total of 96 patients (48 from each group) were matched for propensity scoring with good balance achieved. In this matched sample, freedom from valve replacement was 78% for BAV and 81% in the surgical group. There was a trend (p-value 0.068) towards lower need for reintervention in the neonatal groups at 2 (72% for BAV, 40% for SAV), 4 (72% for BAV, 40% for SAV), and 8 years (72% for BAV, 32% for SAV).   This study reports on the results of BAV versus SAV in a modern cohort with further analysis using a propensity matched comparison. In general, both methods achieve relief of aortic stenosis (while SAV achieved a statistically lower residual gradient, a 10 mmHg lower gradient is unlikely to be clinically significant) with a much higher rate of aortic regurgitation in the BAV group. Despite this, BAV and SAV valvuloplasty had similar short- and medium-term reintervention rates. The propensity matched comparison showed that BAV resulted in much more durable results in the neonatal population. This argues that BAV should be considered first line in this higher risk patient population at all centers to delay need for surgical intervention. A recent meta-analysis (Moroi, M., Bacha, E., & Kalfa, D. (2021). The Ross procedure in children: a systematic review. Annals of Cardiothoracic Surgery, 10(4), 420-432. Doi:10.21037/acs-2020-rp-23) showed that mortality rates were higher in the neonatal and infant population. Given these findings, achieving optimal outcomes in this patient population is essential. When thinking about these outcomes it is important to remember that each center used its own reintervention criteria. This could lead to some patients undergoing reintervention at one center while they would not have at the other. Additionally, due to the determination for BAV versus SAV being a center-wide decision each center had experience and expertise in their chosen method which likely favorably impacted outcomes. Lastly, in my practice, I typically start with a balloon that is 80-90% of the annulus size for initial valvuloplasty rather than similar to the annulus size as the authors report. If there is still a significant gradient a larger balloon is chosen as long as there is not already significant aortic regurgitation. This slightly more conservative approach is taken to decrease the risk aortic regurgitation while hopefully still alleviating aortic stenosis.   

Combined Echo and Fluoroscopy-Guided Pulmonary Valvuloplasty in Neonates and Infants: Efficacy and Safety. Brown NK, Husain N, Arzu J, Ramlogan SR, Nugent AW, Tannous P.Pediatr Cardiol. 2022 Mar;43(3):665-673. doi: 10.1007/s00246-021-02771-2. Epub 2021 Nov 28. PMID: 34839381    Take Home Points: Combining transthoracic echocardiography and fluoroscopy to guide balloon pulmonary valvuloplasty (BPV) is equally effective and safe as standard BPV in treating neonates and infants with isolated pulmonary valve stenosis. Echo-guided BPV results in significantly reduced exposure to radiation and contrast by essentially eliminating the need for cine angiography to measure the pulmonary valve annulus. The subcostal right anterior oblique view by echocardiography was most helpful for guiding the intervention. Commentary from Dr. Milan Prsa (Switzerland, Europe), section editor of Congenital Heart Disease Interventions Journal Watch: Balloon pulmonary valvuloplasty (BPV) is the therapy of choice for isolated valvar pulmonary stenosis (PS). In an effort to reduce the secondary risks of radiation and contrast use, the authors of this study developed an institutional protocol using echocardiography guidance as an alternative to fluoroscopy and/or angiography for parts of the procedure. They report their initial experience and compare the results to standard BPV. Between September 2019 and December 2020, 10 infants with isolated valvar PS (including two with critical PS) underwent echo-guided BPV. They were compared to 19 infants (including six with critical PS) who underwent standard BPV between December 2017 and October 2019. There was no difference in demographic or pre-procedural data between the two groups. All procedures were performed successfully (i.e. residual peak-to-peak gradient <35 mmHg) with no difference in residual cath gradient and no adverse events. There were no differences in the number of balloons used, final balloon-to-annulus ratio, and fluoroscopy time or total sheath time. Naturally, there was an 80% reduction in total dose area product (33.8 versus 167.4 cGY∙cm2, p<0.001) and an 84% reduction in contrast load (0.8 versus 5.0 ml/kg, p=0.003) in the echo-guided BPV group compared to the standard BPV group, principally owing to measuring the pulmonary valve by echo instead of angiography (Table 1).     Table 1. Comparison of results between standard and echo-guided BPV   Although this was a single-center retrospective case-control study, it shows that echo-guided BPV in neonates and infants can yield the same results as standard BPV while using much less radiation and contrast. In addition, the authors demonstrate a shallow learning curve, having performed five of their last six cases wholly without contrast. Their efforts underline the value of adopting a multi-disciplinary team approach in applying the ALARA (As Low as Reasonably Achievable) principle in pediatric cardiac catheterization, and should be emulated as much as possible.   

Factors Influencing Reintervention Following Ductal Artery Stent Implantation for Ductal-Dependent Pulmonary Blood Flow: Results From the Congenital Cardiac Research Collaborative. Shahanavaz S, Qureshi AM, Petit CJ, Goldstein BH, Glatz AC, Bauser-Heaton HD, McCracken CE, Kelleman MS, Law MA, Nicholson GT, Zampi JD, Pettus J, Meadows J.Circ Cardiovasc Interv. 2021 Dec;14(12):e010086. doi: 10.1161/CIRCINTERVENTIONS.120.010086. Epub 2021 Nov 18.PMID: 34789017   Take Home Points: This study included 53 re-interventions in 41/105 (39%) patients <1 year of age who had PDA stent implantation for ductal-dependent pulmonary blood flow. Balloon pulmonary valvuloplasty was associated with decreased likelihood of re-intervention, while greater PDA tortuosity, use of drug-eluting stents, and anticipated single-ventricle physiology were associated with higher hazard of re-intervention. Commentary from Dr. Arash Salavitabar (Columbus, OH, USA), section editor of Congenital Heart Disease Interventions Journal Watch: The authors of this study sought to further understand and describe the rates and types of re-intervention following PDA stent implantation, as well as to identify risk factors associated with re-intervention. This was a retrospective, multi-center cohort study through the Congenital Cardiac Research Collaborative (CCRC) which included all infants <1 year of age with ductal-dependent pulmonary blood flow (DDPBF) and confluent pulmonary arteries palliated at <1 year of age with a PDA stent from 1/1/2008 through 11/1/2015. The primary outcome was re-intervention, whether catheter-based on the PDA or surgically to augment pulmonary blood flow.   This study included 105 patients of median age 9 days (5-15) at 1st intervention and weight of 3.2 kg (2.7-3.7). Prematurity was present in 26 (25%) patients. PDA tortuosity classification was 58 (55.2%) Type I and 47 (44.8%) Type II/III. Antegrade PBF was noted in 62% of the cohort and balloon pulmonary valvuloplasty (BPV) was performed either prior to or as a concomitant procedure with PDA stent implantation. Fifty-three re-interventions (7 surgical, 46 transcatheter) were performed in 41 (39%) patients, all but one occurring within 6 months of the initial procedure. Transcatheter interventions included 35 stent balloon angioplasties and 11 additional stent implantations.     The re-intervention and non-re-intervention groups were compared, showing no difference in baseline characteristics, however the following variables had associations with re-intervention: BPV was associated with decreased likelihood of re-intervention (HR 0.24 [0.10–0.60]; P=0.002); anticipated single-ventricle physiology had an increased hazard of re-intervention (HR, 3.27 [1.74–6.16]; P<0.001); PDA tortuosity classification of Type II/III had a higher risk of re-intervention (HR, 2.12 [1.15–3.91]; P=0.016); use of drug-eluting stents (DES) was associated with higher hazard of re-intervention (HR, 2.29 [1.04–5.02]; P=0.039). It was noted that DES were more likely be implanted in patients who had expected single-ventricle status and with single-source PBF. Interestingly, the number of patients whose ductal length was entirely covered did not differ between the re-intervention and non-re-intervention groups, nor did the PDA length or number of stents implanted. Patients undergoing unplanned re-intervention were noted to be significantly smaller at the time of the initial PDA stent implantation (P=0.045) and were more likely to have had partial or complete jailing of the pulmonary arteries at the initial implant (P<0.001). There was no significant difference in the duration of the interstage interval between the patients who underwent re-intervention related to the PDA stent to those without (P=0.522). No differences were seen in hazard of re-intervention based on antiplatelet/anticoagulant strategy.   The authors address an important question in this paper, and one that is constantly evolving with new techniques and stent types. Re-intervention is relatively safe and found to be nonurgent in this study. Further understanding of confounding variables in the understanding of the “natural history” of ductal stents will be important and ongoing.   

Transcarotid Approach to Ventricular Septal Defect Closure in Small Infants. Breatnach CR, Kenny D, Linnane N, Al Nasef M, Ng LY, McGuinness J, McCrossan B, Nölke L, Oslizlok P, Redmond M, Walsh K.Pediatr Cardiol. 2021 Oct;42(7):1539-1545. doi: 10.1007/s00246-021-02638-6. Epub 2021 Jun 3.PMID: 34081172   Take Home Points: Using a team approach, perimembranous ventricular septal defects can be closed via a minimally invasive, “pseudo-percutaneous” approach with good safety and efficacy. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: Percutaneous closure of perimembranous ventricular septal defects (pmVSD) is experiencing a resurgence as cumulative experience with newer devices demonstrates a favorable safety profile. The approach is generally limited to larger children due to the need for relatively large delivery systems. The authors undertook to describe their novel approach to closing pmVSDs in small children via a surgical right carotid cut-down.   Patients ≥5kg were included if they had evidence of a hemodynamically significant pmVSD without coronary cusp prolapse or aortic insufficiency. Procedures were done in a hybrid cardiac catheterization laboratory with both surgical and interventional teams. A 7 French sheath was placed in the right carotid artery via surgical cut down and the defects were primarily assessed via trans-esophageal echocardiography. The remainder of the procedural steps are summarized in the text but are generally similar to other descriptions of retrograde pmVSD closure. The Figure below demonstrates fluoroscopy of the steps in closure.   Between December 2016 and April 2020, 18 infants (median [IQR] age 7 [5-9] months and weight 7.1 [6.5-7.8] kg) underwent attempted pmVSD closure via this approach. Outcomes are summarized in the table below. Most patients (15/18) had their defects closed successfully with the remaining 3 requiring conversion to open repair (1 – device embolization, 1 – tricuspid valve injury, 1 – device instability). Most patients were discharge after overnight observation on the ward. All 12 patients who had follow up assessment of carotid artery demonstrated no abnormalities.   While not revolutionary, this very interesting report brings together 3 current trends in pediatric cardiac catheterization – the increasing interest in percutaneous closure of pmVSD, use of the carotid artery for interventions in young children, and a collaborative approach to dealing with issues in congenital heart disease. The authors are to be commended for their innovative thinking and good outcomes.       

Title: Echocardiographic Left Ventricular Z-score Utility in Predicting Pulmonary-Systemic Flow Ratio in Children with Ventricular Septal Defect or Patent Ductus Arteriosus Authors: Sumitomo NF, Kodo K, Maeda J, Miura M, Yamagishi H Circulation Journal 2022;86: 128-135, doi 10.1253/circj.CH-21-0559   Take Home Points: Left ventricular end diastolic dimension Z scores (cut point +1.76) have a good correlation (88% specificity overall and 96% specificity in children > 2 yo) with catheterization measured Qp:Qs (cQp:Qs) of 1.5 or more from a VSD or PDA. Left ventricular end systolic dimension Z scores also had a good correlation (72% specificity overall and 84% specificity in children > 2 yo) with cQp:Qs > 1.5 from a VSD or PDA. There was a reasonable correlation between echo derived and catheterization measured Qp:Qs with a r = 0.724 This cannot be applied to patients with genetic abnormalities, to those with moderate or greater TR or MR or concomitant ASDs > 5 mm or defects other than VSD or PDA as this was not studied in this population Commentary from Dr. Thomas Zellers (Dallas, USA), section editor of ACHD Journal Watch: This is a good study that evaluates the correlation between cardiac catheterization measured pulmonary to systemic flow ratios, cQp:Qs, with echo predicted eQp:Qs and then with Z-scores for echo measured LV end-diastolic and LV end systolic dimensions.   The study applies only to patients with VSDs or PDAs. There were a lot of exclusions including patients with more than mild tricuspid, mitral, aortic or pulmonary valve regurgitation, atrial septal defects > 5 mm, any patient with genetic abnormalities, low birth weight (< 2500 gms) or premature infants, patients with poor growth (SD <-2), age < 1 month, PAH with mean PA pressure of 40 mmHg or more, RV systolic pressure > 70% of systemic pressure, and LVEF < 55%.   The authors started with 175 patients evaluated between 2015 and 2019 at two centers in Tokyo and ended with 70 patients in the study because of all of the exclusions. All patients had a cardiac catheterization with Qp:Qs measurements using the Fick method and all had a transthoracic echo with imaging and Doppler within one week of the cardiac catheterization. The LVEDd and LVESd dimension were measured using a standardized M-mode format for each patient. One echocardiographer, blinded to cardiac catheterization data, reassessed all echo measurements and, to determine intra- and inter-observer variations, a sample of patient measurements were evaluated by 4 blinded physicians. The Z-scores were derived from Patterson’s methods. Patients were also divided into younger than and older than 2 years of age and comparisons made between the two age groups.   The authors found a strong and significant correlation between the cQp:Qs and LVEDd. Using regression analysis, the LVEDd Z-score of 1.76 served as a cut point for a cQp:Qs of 1.5:1 or greater with a specificity of 88% for the cohort and 96% in the children more than 2 years of age. In addition, the authors found a statistically significant correlation with the LVESd and cQp:Qs with a Z-score of 1.1 as a cut point for a cQp:Qs of 1.5 or greater. The sensitivity was 89% with a specificity of 72%.   While the correlation between cQp:Qs and eQp:Qs was good with an r = 0.724, the eQp:Qs is overestimated compared to cQp:Qs between 1 and 1.5:1 and is underestimated as the cQp:Qs values exceeded 2:1.   Limitations: This was a retrospective study from two centers with a modest sized group of patients (n=70) due to exclusions in a limited data set (VSD or PDA). The measured dimensions were taken from M-mode only but the inter and intra observer variability was low.   

Cutting balloon angioplasty on branch pulmonary artery stenosis in pediatric patients. Cobb H, Spray B, Daily J, Dossey A, Angtuaco MJ. Catheter Cardiovasc Interv. 2021 Sep;98(3):526-532. doi: 10.1002/ccd.29803. Epub 2021 Jun 10. PMID: 34110668   Take Home Points: Cutting balloon angioplasty (CBA) for pulmonary artery stenosis (PAS) shows moderate medium-term success. Patients with TOF/PA/MAPCAs and especially those with genetic syndromes associated with PAS (Williams, Alagille, or arterial tortuosity syndrome) are significantly less likely to have a lasting success following CBA. Choosing a maximal balloon diameter as the smallest of either 150% of the distal vessel or 300% of the minimal luminal diameter is associated with sustained success following CBA. Commentary from Dr. Prsa Milan (Switzerland, Europe), section editor of Congenital Heart Disease Interventions Journal Watch: Cutting balloon angioplasty (CBA) has been used successfully to treat PAS resistant to standard pressure balloons. However, there is little data on long-term outcomes. The authors of this study describe the longest follow-up to date of CBA in PAS at a single institution and identify predictors of a successful outcome.   From 2005 to 2020, 44 patients (median age 20 months and weight 12.4 kg) underwent 148 CBAs on 116 unique pulmonary artery segments. The mean ratio of cutting balloon diameter to minimal luminal diameter (MLD) was 2.51 ± 1.06 (range 1.0-8.0). There was good initial success (≥50% increase in MLD) with an average immediate increase in MLD of 57.3% (CI, 39.0%-75.6%). In addition, there was sustained improvement at a mean follow-up time of 34.3 months (CI, 0-142 months) with an average increase in MLD of 78.7% (CI, 44%-114%). Complications included reperfusion injury in three patients and pulmonary hemorrhage in two, one of whom died from acute pulmonary artery rupture. Unsurprisingly, interventions were less successful in patients with TOF/PA/MAPCAs and those with genetic syndromes associated with branch PAS (Williams, Alagille, or ATS), as shown by the Kaplan-Meier curves in Figure 3.     Figure 3. Freedom from reintervention following CBA for all pulmonary artery segments in patients with TOF/PA/MAPCAs, genetic syndromes associated with PAS, and those without either diagnosis.   Multivariable logistic regression analysis confirmed that not having TOF/PA/MAPCAs and genetic syndromes increased the odds of a successful CBA by 70% (OR 0.30, CI 0.11-0.79, p = 0.01) and 91% (OR 0.09, CI 0.02-0.56, p = 0.01), respectively. Moreover, each increase in the maximum cutting balloon-to-MLD ratio by 0.5 improved the odds of success by a factor of 2.37 (CI 1.7-3.3, p = <0.0001).   This study further establishes CBA as a successful therapy for PAS resistant to conventional angioplasty. It also highlights two specific patient populations that remain difficult to manage. In TOF/PA/MAPCAs, PA stenoses may be very distal, long-segment lesions, at bifurcation points, or due to scarring at sites of surgical interventions. These patients may thus warrant earlier, more frequent and more aggressive catheterization. In Williams, Alagille and ATS, the abnormal arterial wall structure may prevent therapeutic tears required for a successful dilatation, and these patients may benefit more from surgical intervention instead.    

Impact of Transcatheter Pulmonary Artery Intervention Following Superior Cavo-pulmonary Connection on Pulmonary Artery Growth. Chaszczewski KJ, Huang J, Fuller S, Smith CL, Dori Y, Glatz AC, Gillespie MJ, Rome JJ, O'Byrne ML. World J Pediatr Congenit Heart Surg. 2021 Sep;12(5):635-642. doi: 10.1177/21501351211033238.PMID: 34597205   Take Home Points: Approximately 11% of patients developed branch pulmonary artery stenosis deemed significant enough to require intervention in this single center study. Balloon and stent angioplasty of pulmonary artery stenosis following superior cavo-pulmonary connection normalizes distal pulmonary artery growth relative to the unaffected branch pulmonary artery. Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch: Branch pulmonary artery (PA) stenosis is a common residual lesion following superior cavo-pulmonary connection (SCPC) that is performed as part of single ventricle palliation. Widely patent pulmonary vasculature is considered essential to long-term success with single ventricle palliation. Transcatheter therapy (balloon angioplasty or stent angioplasty) is the first line treatment for branch PA stenosis following SCPC. Previous studies have demonstrated favorable short- and long-term results with this. The authors in this study sought to determine how well branch PAs grow following intervention by comparing its growth rate to the unaffected branch PA. In order to do this, they retrospectively measured the distal branch pulmonary artery (DBPA) and lower lobe branch (LLB) in the affected PA and unaffected PA immediately following intervention and looked at the PA growth rate by performing the same measurements at subsequent cardiac catheterizations.   From January 2010-December 2018; 391 patients underwent SCPC and met inclusion criteria (bilateral bidirectional Glenn, previous hybrid palliation, 1.5 ventricle repair, and SCPC to one PA and systemic to pulmonary shunt to contralateral PA were excluded). 35 patients underwent a total of 54 unilateral transcatheter PA interventions (15 balloon angioplasty, 20 stent angioplasty) at a median of 70 days following SCPC (IQR 19-297) with those undergoing stent angioplasty occurring earlier in the post operative period. The narrowest segment was smaller in those who underwent stent angioplasty (2.2 ± 1 mm) versus balloon angioplasty (3.3 ± 1 mm) as was the DBPA raw measurement (4.2 ± 1.3 mm versus 5.6 ± 1.7 mm) and when indexed for body surface area (15.0 ± 10.1 mm/m2 versus 26.9 ± 15.2 mm/m2). 24 patients underwent subsequent cardiac catheterization prior to total cavopulmonary connection (i.e. Fontan) and had angiography available for serial PA measurements. There was growth of the DBPA and LLB in the treated and untreated branch PA in both the raw dimension and relative to body surface are. There was not significant difference in the growth rate between the unaffected PA and the treated PA.   The authors in this single center retrospective study conclude that distal pulmonary vasculature grows at a similar rate in PA branches requiring intervention versus those that do not in the short term (see below figure). While this model is not perfect, randomizing patients to receive or not receive PA interventions to compare growth is not realistic. This supports the idea that transcatheter intervention helps promote normal growth velocity in treated branch PAs. This is important not only for the central vessel but is likely important for the distal pulmonary vasculature to continue to grow and develop.     Figure 2. Graphical depiction of longitudinal pulmonary artery (PA) growth over time, measured at distal branch PA (DBPA) of the treated PA (A) and DBPA of the contralateral, untreated PA (B).   

Progression in Fontan conduit stenosis and hemodynamic impact during childhood and adolescence. Patel ND, Friedman C, Herrington C, Wood JC, Cheng AL. J Thorac Cardiovasc Surg. 2021 Aug;162(2):372-380.e2. doi: 10.1016/j.jtcvs.2020.09.140. Epub 2020 Oct 29.PMID: 33220959   Take Home Points: Patients with Fontan circulation and an extra-cardiac conduit experience decrease in the cross-sectional area (CSA) as early as 6 months post Fontan. Longer term, almost all patients develop some decrease in Fontan CSA. Proactive surveillance can help identify patients with narrowing of the Fontan conduit and they can be referred for intervention to restore the Fontan conduit size. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: Palliation for patients with single ventricle physiology culminates in a connection of the inferior vena cava (IVC) to the pulmonary arteries. The method of this connection has evolved over time but at many centers currently involves the use of Goretex conduit, typically 18 or 20mm diameter. The ideal diameter of this connection is unknown but it would be logical to think that it should approximate the size of the IVC. The authors sought to characterize changes to Fontan conduit size over time and determine if a decrease in cross sectional area (CSA) affects cardiac output, pulmonary artery growth or exercise capacity.   From January 2013 to October 2019; 165 patients with Fontan circulation underwent either cardiac catheterization (87) or cardiac MRI (93). After excluding those with a lateral tunnel Fontan and duplicate studies, 75 cardiac catheterizations and 83 cardiac MRI were included for analysis – with a mean time from Fontan to MRI/cath of 9.5 ± 3.9 years. The minimum Fontan CSA decreased by a median of 33% (24%, 40%) during a mean follow up of 9.6 years (Figure 1). Strikingly, even at less than 1-year post-Fontan the median decrease was already 33% (25%, 41%). The size of the conduit at implant did not impact the percentage decrease in CSA. Fontan CSA was not associated with median Nakata index (ρ = 0.09, P = 0.29) or cardiac index (ρ = -0.003, P = 0.013); but was moderately related with % predicted oxygen consumption (ρ = 0.31, p = 0.013).   Many additional analyses were performed and reported but the headline finding from this study is that Fontan CSA area decreases as early as 6 months post-Fontan. Luckily, children who are 3-5 years old have smaller IVC’s and can probably tolerate narrowing of the Fontan conduit. Looking at Figure 1, it also becomes clear that almost all patients who undergo an extra cardiac conduit Fontan will have some degree of conduit narrowing when the conduit is imaged. These findings should reinforce the importance of proactive Fontan imaging with some combination of cardiac MRI or cardiac catheterization. This would allow for the identification of patients who have a significant mismatch between their IVC and Fontan conduit and allow for intervention on the Fontan conduit to maximize the Fontan CSA and minimize resistance to venous return. It will be important to assess whether this approach will help to prolong Fontan longevity and minimize the incidence of Fontan failure.     Figure 1: A total of 158 patients underwent imaging of the Fontan conduit via MRI (n = 83) or cardiac catheterization (n = 75). There is a significant decrease in the minimum Fontan CSA that starts after the Fontan operation. MRI, Magnetic resonance imaging; CSA, cross-sectional area   

Percutaneous Common Carotid Artery Access for Cardiac Interventions in Infants Does Not Acutely Change Cerebral Perfusion. Lahiri S, Qureshi AM, Justino H, Mossad EB. Pediatr Cardiol. 2022 Jan;43(1):104-109. doi: 10.1007/s00246-021-02697-9. Epub 2021 Aug 7. PMID: 34363498   Take Home Points: NIRS can be used to assess regional cerebral oxygen saturation in patients receiving common carotid artery (CCA) vascular access in the congenital cardiac catheterization laboratory. There was no change in NIRS with CCA access during cardiac catheterization in neonates and infants. Further prospective studies are needed to understand trends of lower regional cerebral oxygen saturation with certain procedure lengths and cardiac physiologies. Commentary from Dr. Arash Salavitabar (Columbus, OH, USA), section editor of Congenital Heart Disease Interventions Journal Watch: The authors address the important and unknown impact of percutaneous common carotid artery (CCA) vascular access and cerebral perfusion in the cardiac catheterization laboratory using Near-Infrared Spectroscopy (NIRS) to assess regional cerebral oxygen saturation (rSO2). This was a single-center retrospective chart review at a large tertiary care center over a 10 year period. All patients who had ipsilateral cerebral NIRS monitored on the side of CCA access were included.   The typical institutional practice described by the authors was to place one NIRS probe on each side of the patient’s forehead if cardiac catheterization was being performed via CCA. NIRS data were collected continuously from 15 minutes prior until 15 minutes after the end of the procedure, and the mean NIRS were compared between pre-vascular access, during access, and following sheath removal. The primary outcome measure was the change in NIRS with CCA access. The secondary outcome measure was the area under the curve for rSO2 ≤ 45%, which was based off of thresholds of prior studies associated with neurodevelopmental outcomes. The value of “Integrated rSO2” (=minutes × (desaturation values ≤ 45%)) was chosen as a marker of neurodevelopmental outcomes, which was chosen over total duration of rSO2 ≤45% in order to better indicate the extent of desaturation below the threshold value.   This study included 21 patients. The median patient age was 23 days (IQR 7,79) and weight was 3.3 kg (IQR 2.8,2.9). The median sheath size was 4F (3.3–5F) and duration of the procedure was 1 h 48 min (range 22 min to 4 h). There was no significant difference found in mean NIRS before, during, or after the CCA catheterizations. Integrated rSO2 ≤ 45% was grouped into 3 groups (0 min, 0.1–39 min, ≥40 min). There were no significant differences in total integrated rSO2 ≤ 45%, total procedure duration, minimum integrated rSO2 ≤ 45%, average rSO2, or minimum rSO2 between the 3 groups. Of clinical importance, Group III had the longest procedure duration and lower average rSO2, of which one patient had an extremely low NIRS noted by the authors. When analyzing patients who had bilateral NIRS documented, neither integrated rSO2≤ 45% nor NIRS was significantly different between two sides during the procedure. Three total patients had significantly elevated integrated rSO2 ≤ 45%, two of which were thought to be secondary to ductal constriction, rather than CCA access.     This paper addresses an impactful topic that has not yet been fully explored. The authors provide important baseline findings that show that cerebral perfusion is not significantly or adversely affected in patients who receive CCA in the congenital cardiac catheterization laboratory. It is important to note that this study included a small sample size and was limited by retrospective data collection and analysis. To fully understand the trends seen in a small number of patients, it will be important to perform a prospective study evaluating CCA and markers of cerebral perfusion in a more rigorous way. Nonetheless, this data is an important first step to understanding the impact of this form of vascular access in what is often a fragile population.   

Impact of Specialized Electrophysiological Care on the Outcome of Catheter Ablation for Supraventricular Tachycardias in Adults with Congenital Heart Disease: Independent Risk Factors and Gender Aspects. Fischer AJ et al. Heart Rhythm. 2021; 18:1852-1859.Percutaneous Hydrogel Coil Embolization of Aneurysms and Coronary Artery Fistulae in Congenital Heart Disease. Healan SJ, Nicholson G, Doyle T, Janssen D.Tex Heart Inst J. 2021 Jul 1;48(3):e207312. doi: 10.14503/THIJ-20-7312.PMID: 34347100.   Take Home Points: Coils are commonly used for percutaneous embolization of vascular lesions (collaterals, fistulas) in patients with congenital heart disease. Hydrogel based coils (Azur CX coils and Azur hydrogel coils, Terumo medical) have the advantage of a less reliant on clot formation due to the expanding hydrogel. Azur hydrogel coils have a controlled electronic release mechanism designed to improve the precision of coil positioning, thus increasing procedural accuracy. Although promising, long term prospective data is needed in congenital heart disease patients. Commentary from Dr. Varun Aggarwal (Minneapolis, MN, USA), editor-in-chief and section editor of Congenital Heart Disease Interventions Journal Watch: Patients with congenital heart disease often have collateral vessels, fistulas, aneurysms, pseudoaneurysms etc. that necessitate use of vascular occlusion devices like vascular occlusion coils and plugs. Use of bare metal (Ruby coil, Penumbra) or fibered coils (eg. MReye® Embolization Coil, Cook Medical; Concerto Versa™ detachable coil, Medtronic) is common in congenital catheterization laboratory. Historically these coils have served well. One of the drawbacks of these coils is the dependence on clot formation besides mechanical occlusion. Compaction of these coil over time has also been reported. Hydrogel based coils have been more commonly used in neurological and peripheral interventional laboratories and have been demonstrated to have an advantage over bare metal or fibered coils with higher packing density (1), lower rate of recurrence of intracranial aneurysms (2), lower rate of recanalization (3) without any increased risk of procedural complications (1).   Healan SJ et al (4) in this case series describe the use of hydrogel-based coils (Azur CX and Azur hydro coils, Terumo medical) in 5 patients with congenital heart disease. Azur CX coil is filled with hydrogel forming a solid core and the Azur hydrogel coil a hydrogel coating, which increases the effective diameter of the coil after expansion, providing a higher packing density and thereby making it suitable for packing aneurysms. They have the advantage of reduced incidence of coil compaction and recanalization over time. The authors describe the use of these coils in a multitude of lesions such as aortic pseudoaneurysm, pulmonary arterial pseudoaneurysm, coronary artery fistula (in 3 patients). All patients underwent successful closure of the intended lesion without any complications. Azur hydrogel coils also contain an electronic release mechanism that improves coil positioning precision, resulting in higher procedural accuracy and lower complication rates.   In our experience, these coils especially the Azur hydrocoils may be a little stiffer as compared to the bare platinum or fibered coils. This can make these coils more technically challenging especially when using for the first few times. Also, the Azur hydrocoils swell up after coming in contact with blood and therefore provide a 3-5 minute window to either deploy the coil or retract back in the catheter. Despite these technical limitations, these coils have benefit at lower recanalization, higher packing density and lower rate of recurrence. Future longitudinal studies are warranted to compare the outcomes in congenital heart disease patients.     Example of a bare platinum coil in Figure A showing residual flow of contrast (marked by red circle) despite densely packed coil mass in the proximal vessel in a child with aortopulmonary collateral vessels. Figure B demonstrates no residual flow through an aortopulmonary collateral vessel in a patient with Fontan circulation post embolization using Azur hydrogel coil.   1. Taschner CA, Chapot R, Costalat V, Machi P, Courtheoux P, Barreau X, et al. GREAT-a randomized controlled trial comparing HydroSoft/HydroFrame and bare platinum coils for endovascular aneurysm treatment: procedural safety and core-lab-assessedangiographic results. Neuroradiology. 2016;58(8):777-86. 2. Xue T, Chen Z, Lin W, Xu J, Shen X, Wang Z. Hydrogel coils versus bare platinum coils for the endovascular treatment of intracranial aneurysms: a meta-analysis of randomized controlled trials. BMC Neurol. 2018;18(1):167. 3. Fohlen A, Namur J, Ghegediban H, Laurent A, Wassef M, Pelage JP. Midterm Recanalization after Arterial Embolization Using Hydrogel-Coated Coils versus Fibered Coils in an Animal Model. J Vasc Interv Radiol. 2019;30(6):940-8. 4. Healan SJ, Nicholson G, Doyle T, Janssen D. Percutaneous Hydrogel Coil Embolization of Aneurysms and Coronary Artery Fistulae in Congenital Heart Disease. Tex Heart Inst J. 2021;48(3).   

Impact of Patient Prosthesis Mismatch on the Outcome of Transcatheter Pulmonic Valve Implantation. Takajo D, Forbes TJ, Kobayashi D. Am J Cardiol. 2021 Jul 15;151:93-99. doi: 10.1016/j.amjcard.2021.04.022. Epub 2021 May 27.PMID: 34053630   Take Home Points: A pulmonary valve indexed geometric orifice area of 1.25 cm2/m2 was the optimal value for predicting a residual RVOT gradient of ≥ 15 mmHg and need for reintervention. PPM is a strong predictor of the need for re-intervention and should be taken into account when planning TPVI to ensure optimal patient outcomes. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: Patient-prosthesis mismatch (PPM) – a situation in which a prosthetic valve is smaller than a normal/native valve – is known to adversely affect outcomes in trans-catheter aortic valve implantation. This has not been systematically studied in patients undergoing trans-catheter pulmonary valve implantation (TPVI). The authors sought to define PPM with an optimal cut-off value using an indexed geometric orifice area (iGOA = [π x (a/2) x (b/2)]/BSA); and assess its effect on re-interventions.   From 2010 to 2020 101 patients were included (Sapien valves, bilateral Melody valve and Melody in LV-PA conduit were excluded) – median age 21.3 ± 10.2 years with 38 patients less than 16 years of age. The mean GOA was 2.22 ± 0.67 cm2 and iGOA was 1.42 ± 0.48 cm2/m2 with a significant negative correlation between post-TPVI residual RVOT gradient and iGOA. An ROC analysis identified an iGOA of 1.25 cm2/m2 as the best cut off for predicting a residual RVOT gradient of ≥ 15 mmHg. The cohort was then divided into 2 groups, those having PPM (n = 42, iGOA < 1.25 cm2/m2) and non-PPM (n = 59, iGOA ≥ 1.25 cm2/m2); unsurprisingly patients with PPM had a higher residual RVOT gradient post-TPVI. Over a mean follow up period of 6.9 ± 2.7 years, 22 patients (22%) required re-interventions. Residual RVOT gradient ≥ 15 mmHg and PPM were significantly associated with a need for re-intervention (Figure).   The authors conclude that PPM is a strong predictor of the need for re-intervention and should be considered when planning TPVI to ensure optimal patient outcomes. They provide a table (see below) to easily determine minimal stent diameters to ensure an iGOA ≥ 1.25 cm2/m2. The concept of PPM can help providers determine whether anticipated final conduit diameters will be adequate or will predispose patients to an unacceptably high risk of re-intervention. This becomes even more relevant as providers push to dilate surgical conduits beyond their nominal diameters and consider patients for valve in valve type procedures with implant of second and third trans-catheter valves.   Figure.     

Mullins-Sheath Facilitated Delivery of Gore Cardioform ASD Occluder Devices for Closure of Large or Challenging Secundum Atrial Septal Defects. Eilers LF, Gowda ST, Gowda S, Lahiri S, Aggarwal V, Stapleton GE, Gillespie MJ, Qureshi AM.J Invasive Cardiol. 2021 Jun;33(6):E425-E430. Epub 2021 Apr 23.PMID: 33893794   Take Home Points: Gore Cardioform Atrial Septal Defect occluder is a new device which is approved by US FDA for closure of secundum atrial septal defect. The relatively stiff device delivery system can pose special challenges in complex anatomy (large atrial septal defect in small children especially with deficient rims). Use of Mullins sheath has been shown to be safe and help to favourably exaggerate the curve on the device delivery system and achieve successful device placement Commentary from Dr. Varun Aggarwal (Minneapolis, MN, USA), editor-in-chief and section editor of Congenital Heart Disease Interventions Journal Watch: One of the challenges in placement of any device to close the atrial septal defect is to align the device discs parallel to the plane of the atrial septum. This remains true for the most recent addition (the Gore Cardioform Atrial Septal Defect (ASD) occluder) to the tool kit on congenital and structural interventional cardiologists who perform transcatheter closure of ostium secundum ASD’s. Gore Cardioform ASD occluder was approved by US FDA in 2019 for closure of ASD varying in size from 8-35 mm in diameter. The device comes preloaded loaded on delivery cable and ready to be used after deairing and pulling inside the delivery cable (Figure 1). There is a small curve on the delivery catheter to assist with device positioning, however this may not be enough in challenging ASD’s (large defects with deficient rims). The MullinsTM sheath has a pre-shaped curve near the tip. With the advancement of catheters, wires, balloons and stents, this curve straightens out, especially if restricted within a vessel or on a reasonably straight route. According to the experience by Eilers LF et al (1), even with the introduction of the Gore Cardioform ASD Occluder delivery catheter to accentuate the curve of the Gore Cardioform ASD Occluder delivery catheter to align the device parallel to the atrial septum, the MullinsTM sheath maintains enough of a curve at its distal tip when positioned in a large chamber, such as the left atrium (Figure 1). This can be very useful to align the device discs parallel to the atrial septum to allow for capture of the rims of the ASD.   Authors describe the experience on 98 consecutive patients who underwent closure of ASD using the Gore Cardioform ASD device from June 2017 to December 2019 at Texas Children’s Hospital/Baylor College of Medicine (Houston, TX). The use of Mullins TM sheath was at the discretion of the implanting physician and not randomized in the study. Of the 98 patients, 52 patients underwent closure of the ASD using the Mullins TM sheath while the remaining 46 patients underwent attempted secundum ASD closure with the Gore Cardioform delivery catheter through a short sheath in standard fashion as described in the IFU.   Table 1 shows the baseline characteristics of the two groups and notably the group with MullinsTM sheath had larger ASD (inherent selection bias) and more patients with deficient rims. The success rate was 88% in the group with MullinsTM sheath. Three of the six patients who did not have a successful closure with the MullinsTM sheath assisted technique were referred for surgery, two had their ASD successfully closed with the Gore Cardioform ASD Occluder using a HausdorfTM sheath, and one had their ASD successfully closed with an Amplatzer Septal Occluder device (ASO) (Abbott, Abbott Park, IL). The two patients who had failed closure because to the lack of a Mullins sheath received effective surgical closure. There were 2 major adverse events (atrial fibrillation requiring cardioversion). At a median follow up of 43 (IQR 1, 374) days, no patient had more than a mild residual shunt.   We recently described the deployment procedure and techniques for challenging atrial septal defects using the new Gore Cardioform ASD occluder (2). We encourage the implanters of this device to consider use of these techniques while attempting closure of large secundum ASDs or secundum ASDs with challenging anatomy. This technique may be useful until a specific delivery sheath is designed and available for delivery of the Gore Cardioform ASD Occluder in such circumstances.   Figure 1: Device positioning and delivery using standard Gore delivery catheter and with use of Mullins   Sheath demonstrating the exaggeration of the delivery catheter angle(1).   Table I. Baseline demographics and procedural characteristics   References: 1. Eilers LF, Gowda ST, Gowda S, Lahiri S, Aggarwal V, Stapleton GE, et al. Mullins TM sheath facilitated delivery of Gore Cardioform ASD Occluder Devices for closure of large or challenging secundum atrial septal defects. Journal of Invasive Cardiology 2021 (In Print).2021. 2. Aggarwal V, Mohan AK, Bass J, Steinberger J, Said SM, Qureshi AM. Gore Cardioform Atrial Septal Occluder: Deployment Procedure and Techniques for Closing Challenging Secundum Atrial Septal Defects. Cardiol Young. 2021:1-25.   

Impact of Patient Prosthesis Mismatch on the Outcome of Transcatheter Pulmonic Valve Implantation. Takajo D, Forbes TJ, Kobayashi D.Am J Cardiol. 2021 May 27:S0002-9149(21)00376-3. doi: 10.1016/j.amjcard.2021.04.022. Online ahead of print.PMID: 34053630   Take Home Points: Patient prosthesis mismatch (PPM) is an important factor of the outcome in transcatheter aortic valve implantation. However, the impact of PPM in transcatheter pulmonic valve implantation (TPVI) has not been studied. The Geometric Orifice Area indexed by the BSA had the best overall model quality and its optimal cut-off value of iGOA was 1.25 cm2/m2. Both PPM and significant residual RVOT gradient ≥ 15 mmHg after TPVI were significantly associated with the need of re-intervention (p < 0.05). Based on the proposed cut-off value of iGOA 1.25 cm2/m2, the authors made a Table (Table 1) which can be used as a reference value of the Melody stent valve diameters based on BSA and eccentricity index. Commentary from Dr. Varun Aggarwal (Minneapolis, MN, USA), section editor of Congenital Heart Disease Interventions Journal Watch: Patient prosthesis mismatch (PPM) means that the effective Geometric Orifice Area (GOA) of the prosthetic valve is smaller than of a normal human valve (1). The outcomes of transcatheter aortic valve placement have been shown to be affected by the GOA of the valve (2, 3). This however, has not been well studied for transcatheter pulmonic valve implantation (TPVI). In this paper, Takajo D et al (4) performed a single center retrospective review of 101 patients who underwent transcatheter Melody valve placement in the right ventricular outflow tract from 2010 to 2020.   The GOA was calculated with the ellipse formula based on the assumption that the measured narrowest diameters in two projections represent the major and minor axis of the orifice area: GOA = p*(a/2)*(b/2), Figure 1. The GOA was indexed to the BSA, weight and height to derive iGOA (cm2/m2), iGOA (cm2/kg), and iGOA (cm2/m), respectively. Significant RVOT residual gradient (≥ 15 mmHg) was observed in 31 patients (40%). The narrowest diameter in either AP or lateral views was 16.1±2.4 mm. There were 30 patients (30%) having the eccentricity index >1.1. The measured GOA was 2.22±0.67 cm2 and iGOA was 1.42±0.48 cm2/m2. There was significant negative correlation between the post-TPVI residual RVOT gradient and iGOA (cm2/m2) (Pearson correlation -0.620, p < 0.001). The iGOA indexed by the BSA had the best overall model quality (area under the curve 0.873, p < 0.001, Figure 2) and its optimal cut-off value of iGOA was 1.25 cm2/m2. Based on the cut-off value of iGOA, the cohort was divided into two groups: PPM group (n = 42, iGOA < 1.25 cm2/m2) and non-PPM group (n = 59, iGOA ≥1.25 cm2/m2).   Over the mean follow up period of 6.9±2.7 years, 22 patients (22%) required re-interventions (16 transcatheter, 11 surgical, and both in 5 patients). On the Kaplan-Meier survival analysis, both PPM and significant residual RVOT gradient ≥ 15 mmHg were significantly associated with the need of re-intervention (p < 0.05, Figure 3). The final multivariable model showed that the significant predictors were PPM (hazard ratio 2.67, p = 0.021) and homograft (hazard ratio 2.85, p = 0.022). Abnormal eccentricity index was not associated with the presence of PPM. Neither the Ensemble system size nor eccentricity index had no effect on the need of reintervention at follow-up. Based on the proposed cut-off value of iGOA 1.25 cm2/m2, the authors made a Table (Table 1) which can be used as a reference value of the Melody stent valve diameters based on BSA and eccentricity index. This is the first data depicting the importance or GOA and PPM in transcatheter pulmonary valve implantation. These factors should be factored into consideration by operators while performing TPVI using Melody valve.   Figure 1. Measurement of geometric orifice area in the transcatheter pulmonary valve implantation. The narrowest valve stent diameter is measured in anteroposterior (AP) and lateral views (4).     Figure 2: Receiver operator characteristic curve analysis to identify the optimal cut-off value of indexed geometric orifice area (iGOA) by body surface area, weight and height, to detect the significant RVOT residual gradient (≥ 15 mmHg) in transcatheter pulmonic valve implantation(4).     Figure 3: Kaplan-Meier survival curve showing the freedom from the re-intervention in 101 patients undergoing transcatheter pulmonic valve implantation using Melody valve, based on (A) residual right ventricular outflow tract (RVOT) gradient and (B) patient prosthesis mismatch (4).     Table 1: Clinical guide for the Melody valve stent diameter to avoid the patient prosthesis mismatch based on the body surface area (BSA) and eccentricity index, using the cut-off value of the indexed geometric orifice area (GOA) of 1.25 cm2/m2 (4).     References: 1. Muneretto C, Bisleri G, Negri A, Manfredi J. The concept of patient-prosthesis mismatch. J Heart Valve Dis. 2004;13 Suppl 1:S59-62.   2. Pibarot P, Clavel MA. Prosthesis-Patient Mismatch After Transcatheter Aortic Valve Replacement: It Is Neither Rare Nor Benign. J Am Coll Cardiol. 2018;72(22):2712-6.   3. Dayan V, Vignolo G, Soca G, Paganini JJ, Brusich D, Pibarot P. Predictors and Outcomes of Prosthesis-Patient Mismatch After Aortic Valve Replacement. JACC Cardiovasc Imaging. 2016;9(8):924-33.   4. Takajo D, Forbes TJ, Kobayashi D. Impact of Patient Prosthesis Mismatch on the Outcome of Transcatheter Pulmonic Valve Implantation. Am J Cardiol. 2021;151:93-9.    

Preliminary testing and evaluation of the renata minima stent, an infant stent capable of achieving adult dimensions. Zahn EM, Abbott E, Tailor N, Sathanandam S, Armer D. Catheter Cardiovasc Interv. 2021 May 4. doi: 10.1002/ccd.29706. Online ahead of print. PMID: 33942962   Take Home Points: The Renata Minima Stent is a cobalt chromium, balloon-expandable stent that has diameter potential of 4-22mm. In a growing animal model, the vast majority of stents were implanted successfully and were subsequently successfully re-dilated with foreshortening noted with successively larger diameters This stent represents an important advancement in stent design to meet a critical need in congenital cardiac catheterization. Commentary by Dr. Arash Salavitabar (Ann Arbor Michigan) section editor of Pediatric Interventional Cardiology Journal Watch: The authors describe the Renata Minima Stent, which is a cobalt chromium, balloon-expandable stent designed for implantation at small diameters and with significant expansion potential, tested here in a growing animal model. The stent diameter ranges from 4-22mm, with all stents having an initial length of 17mm. The stent is designed with its own delivery system, which consists of an inner balloon and a braided “delivery sheath” designed to cover the stent for delivery and allows for hand angiograms during stent positioning, and is currently available with its custom delivery balloon in 6 or 8mm diameters for implantation. Importantly, this is a low profile system that has an outer diameter reportedly comparable to that of a 4-French sheath, and can be delivered over a 0.014” or 0.018” guide wire or via a 6-French sheath.   In this animal model, 21/22 (95%) stents were successfully implanted in 6 piglets (initial weight 4.6 +/- 0.5kg), with the one unsuccessful implant being delivered through an early delivery system prototype. The majority of stented sites were either in the aorta or branch pulmonary artery. Stents were implanted with an average balloon inflation pressure of 11.8 +/−2.1 atm, resulting in an implant diameter of 6.9 +/− 1.2 mm and stent length of 16.9 +/− 0.8 mm. Average stent recoil was 0.8 mm +/− 0.5 (9.8% +/− 6.2). Four piglets had subsequent catheterizations and redilations were performed in 17/22 total stents. Stents were successfully re-dilated at an average of 54 days post-implantation, at which time stent diameter increased by 54% +/− 25% (p < .001) with foreshortening to a final stent length of 16.1 +/−1.5 mm. Three piglets (mean weight 95kg +/- 13.1) with 11 total stents had re-catheterizations at 5 months post-implantation, with re-dilation performed on 8 of those stents. Stent dilation diameters represented a 61% +/− 28% increase from the prior procedure, or a 125% +/− 35% increase in diameter since stent implantation. Two stents were over-dilated with 18mm and 16mm balloons, respectively, with a foreshortening of 23% +/− 9% and average final stent length of   13.1 mm (+/−1.5). There were no complications, however a strut fracture was noted after re-dilation to 16mm in one aortic and one LPA stent.   Two piglets were sacrificed at 1 month for histopathology, which showed neointimal cell growth was present in varying degrees, fibromuscular tissue, and mild-to-moderate   strut-associated inflammation. In late histopathologic evaluation of a recently dilated LPA stent, irregular neointimal growth and vascular wall damage was identified.   These are promising early results for a new stent option that aims to solve a common and extremely important problem: stent implantation in the infant or child that will allow for lifelong expansion potential to match vessel and somatic growth. There are several aspects that will need to be further tested in animal models, such as treatment of truly stenotic vessels, followed by trials in humans in order to determine efficacy and safety. Nonetheless, this is a much needed advancement in congenital cardiac catheterization.     

Correction of sinus venosus atrial septal defects with the 10 zig covered Cheatham-platinum stent - An international registry. Rosenthal E, Qureshi SA, Jones M, Butera G, Sivakumar K, Boudjemline Y, Hijazi ZM, Almaskary S, Ponder RD, Salem MM, Walsh K, Kenny D, Hascoet S, Berman DP, Thomson J, Vettukattil JJ, Zahn EM.Catheter Cardiovasc Interv. 2021 Apr 28. doi: 10.1002/ccd.29750. Online ahead of print.PMID: 33909945 Take Home Points: Percutaneous correction of sinus venosus atrial septal defects in carefully selected patients is a feasible alternative to surgical closure. 10 zig covered Cheatham-platinum stents may facilitate this procedure by decreasing risk of embolization and minimizing the number of stents needed for defect closure. Careful assessment of pulmonary venous return remains a critical aspect of this procedure. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: Percutaneous correction of sinus venosus atrial septal defects (SVASD) is gaining acceptance as an alternative to surgical closure in anatomically appropriate defects.  This novel procedure may be aided by the availability of the 10-zig covered Cheatham-platinum stent (CCPS) due to its longer lengths, larger expansion diameters and less foreshortening.  The authors established an international registry and conducted a retrospective analysis of the outcomes of implantation of 10 zig CCPs in patients with SVASDs at 12 centers. From March, 2016 to February, 2021; 75 patients – median age 45.4 years (range 11.4-75.9) - underwent SVASD closure using a 10 zig CCPS.  The technical aspects of this procedure were not the focus of this paper and have previously been described.  Three primary techniques were utilized after wire externalization in the right internal jugular (RIJ) vein: (1) primary implant of a 10-zig CCPS (n=60); (2) use of 2 bare metal stents to “sandwich” the CCPS with one serving as a landing zone (n = 8); and (3) externalizing the mounting balloon via the RIJ, crimping the CCPS, and threading a long silk through a superior zig of the stent to facilitate stent positioning (n = 7).  Overall, more than one stent was used in 32 patients with a shorter CCPS clearly being a risk factor for requiring an additional stent (Table 1). There were 4 patients (5.3%) who experienced major complications – 2 stent embolizations after leaving the catheterization laboratory requiring surgical removal and repair of the defect; 1 patient with pericardial tamponade requiring sternotomy 3 days after the procedure (felt to be related to the trans-septal puncture); and 1 patient that ultimately developed right upper pulmonary vein atresia, hemoptysis and required a right upper lobectomy.  During a median follow up of 1.8 years all symptomatic patients improved and all patients who had echocardiograms available (66) had a small residual leak or less. Percutaneous correction of SVASD is again demonstrated to result in technical success with a low rate of complications in a relatively small number of patients.  The authors demonstrate that this procedure is facilitated by longer 10 zig stents.  The 10-zig CCPS seems to be a valuable tool in the armamentarium of providers seeking to undertake this challenging procedure and may decrease the need for multiple stents while maintain a low risk for stent embolization.

Patent Ductus Arteriosus Stenting for All Ductal-Dependent Cyanotic Infants: Waning Use of Blalock-Taussig Shunts. Ratnayaka K, Nageotte SJ, Moore JW, Guyon PW, Bhandari K, Weber RL, Lee JW, You H, Griffin DA, Rao RP, Nigro JJ, El-Said HG. Circ Cardiovasc Interv. 2021 Mar;14(3):e009520. doi: 10.1161/CIRCINTERVENTIONS.120.009520. Epub 2021 Mar 9. PMID: 33685211   Take Home Points: PDA stenting in all neonates with ductal dependent pulmonary blood flow is feasible and results in good outcomes. Providers with less experience should consider gaining experience with stenting lower risk PDAs and then progress to higher risk PDA phenotypes. High risk characteristics may include: PDA tortuosity, small pulmonary artery size, at risk for PA discontinuity, or concern for PDA stent bronchus compression.   Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: Stenting of the patent ductus arteriosus (PDA) in patients with ductal dependent pulmonary blood flow (DDPBF) has shown to be non-inferior (and likely superior) to traditional palliation with a surgical modified Blalock-Taussig shunt (BTS). Most reports to date have reported on selective experience with PDA stenting (i.e. excluding patients felt to be at higher risk due to certain factors – PDA tortuosity, small pulmonary artery size, at risk for PA discontinuity, or concern for PDA stent bronchus compression, see Figure below). The authors report on a single center experience transitioning from selective PDA stenting to attempting PDA stenting in all patients with DDPBF.   The study compared 2 distinct periods: selective PDA stenting (Era 1, 2013-2017) and stenting all patients with DDPBF (2018-2020). A total of 88 patients were included for analysis (Era 1 = 66, 41 BTS and 25 PDA stent; Era 2 = 22 PDA stent, no BTS). The patients in the 2 eras were comparable. There was no difference in mortality (or other secondary outcomes measures) between treatment eras or between BTS and PDA stenting. Complication rates were similar between treatment eras and palliation approaches. Post-procedure length of stay was shorted in Era 2 (v Era 1). PDA stent patients had short post-procedure length of stay and more symmetric branch PAs at subsequent surgery.   The authors conclude that PDA stenting for all neonates with DDPBF is safe and effective and may have lower morbidity than selective PDA stenting. As more centers undertake PDA stenting as an alternative to palliation with surgical BTS it is important to understand what the best approach to introduce this procedure is. Centers new to PDA stenting may initially refer patients felt to be at highest risk for PDA stenting directly for surgical BTS. Experienced providers have demonstrated that even these high-risk patients can successfully undergo PDA stent implantation with outcomes that are equivalent to lower risk subtypes. Ratnayaka et al, very nicely describe a programmatic shift from performing selective PDA stenting to stenting PDAs in all patients with DDPBF with excellent overall outcomes. However, the excellent outcomes demonstrated in this report may be the result of earlier experience with lower-risk PDA stenting patients and less experienced providers should still be cautious when considering high-risk PDA phenotypes.   Preintervention, procedural, and postintervention angiograms of transaxillary PDA stenting for ductal dependent pulmonary blood flow (DDPBF) are shown. A patient with severe PDA tortuosity (type 3, multiple complex turns in ductus) is shown in A to C. A patient with small pulmonary arteries (also tortuosity index 3) is shown in D to F. A patient at risk for pulmonary artery discontinuity (3D reconstruction shows at risk left pulmonary artery [LPA]) because of ductal tissue is shown in G, H, and I. G, Posterior view demonstrating the LPA in purple and PDA and right pulmonary artery in beige. The arrow shows the origin of the LPA. Tortuous PDAs have been intentionally straightened in all 3 cases by a stiff guidewire (Ironman, Abbott, Santa Clara, CA).  

4. Mid-Term Outcomes Following Percutaneous Pulmonary Valve Implantation Using the "Folded Melody Valve" Technique. Jalal Z, Valdeolmillos E, Malekzadeh-Milani S, Eicken A, Georgiev S, Hofbeck M, Sieverding L, Gewillig M, Ovaert C, Bouvaist H, Pillois X, Thambo JB, Boudjemline Y. Circ Cardiovasc Interv. 2021 Mar 17:CIRCINTERVENTIONS120009707. doi: 10.1161/CIRCINTERVENTIONS.120.009707. Online ahead of print. PMID: 33726503   Take Home Points: Folding the ends of the Melody valve stent leads to a shortened overall stent valve length that can allow for placement in more challenging or complex RVOTs. This modified valve was implanted with excellent procedural outcomes of reduced RVOT gradient and minimal pulmonary insufficiency. Mid term outcomes are favorable and consistent with unaltered Melody valve results in terms of valve failure (due to stenosis or regurgitation) and infective endocarditis.   Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch:   Transcatheter pulmonary valve replacement (TPVR) is a now widely available option for the treatment of dysfunctional right ventricular outflow tracts. The Melody valve (Medtronic, Minneapolis, MN) and Sapien XT and S3 valves (Edwards Lifesciences, Irvine, CA) are both now FDA approved for this indication. In order to safely implant the valves, there needs to be an adequate landing zone to accommodate the length of the valved stent. If the valve is placed too proximal, there is the risk of ventricular arrhythmias or ventricular embolization. If the valve is placed too distal, there is the risk of branch PA obstruction. A standard Melody valve length ranges from 24.6-28.8 mm depending on the valve diameter. The Sapien XT length is 14.3-19.1 mm depending on the valve diameter (the Sapien S3 did not receive FDA approval for TPVR until 2020 and hence was not discussed in this article). This group described a technique where they folded the Melody valve stent extremities to shorten the total valved stent length to allow for placement in challenging RVOTs. They were able to achieve Melody valve lengths at 22 mm of 20.9 mm by folding one end and 16.7 mm by folding both ends. In this study, Dr. Jalal and his co-authors report on the midterm outcomes of these valves.   A total of 49 patients from 7 European centers underwent successful TPVR with a from April 2012 to June 2018 with a “folded Melody valve.” The majority of these patients’ (68%) underlying anatomy was a tetralogy of Fallot variant. The primary indication for TPVR was pulmonary stenosis in 39%, pulmonary regurgitation in 22%, and mixed disease in 39%. The patient population had native/patch RVOTs in 31%, RV to PA homografts or conduits in 47%, and bioprosthetic valves in 22%. The primary indication for using the folded valve technique was a short RVOT in 57%, though the authors also report using this in bioprosthetic valves (done to minimize stent protruding into the RV and ensuring the entire stent valve apparatus is protected by the bioprosthetic valve apparatus to minimize risk of stent fracture), in situations where a shorter stent would avoid coronary artery compression, prevention of retrosternal compression, and to increase the devices outer diameter. RVOT presenting was performed in 89% of patients with a stent length of 32 +/- 6.8 mm. The authors were able to achieve reductions in the RV to PA gradient (~35 mmHg to ~12 mmHg), RV systolic pressure (~60 mmHg to 41 mmHg), and RV:Aorta ratio (0.68 to 0.38). Trivial pulmonary regurgitation was seen post procedure in 10% of patients with the remaining patients having no pulmonary regurgitation.   Follow up data was available for 47/49 patients with a median follow up duration of 28 months (range 4-80). At the time of last follow up, transthoracic echocardiography showed a mean RVOT gradient of 15 +/- 12 mmHg and RV pressure of 35.8 +/- 15.6 mmHg.There was trivial pulmonary regurgitation in 2 patients with the remainder having none. Three patients underwent reintervention for valve related complications (2.1% per patient per year incidence). Two patients developed infectious endocarditis (1.4% per patient per year) with an increased pressure gradient across the Melody valve. One patient was able to be treated percutaneously after antibiotic treatment (balloon angioplasty followed by Melody valve in valve implantation 3 years later). The other required surgical explant after antibiotic treatment. A third patient developed Melody valve stenosis at 24 months without an obvious etiology and underwent Melody valve in valve implantation.   Mid term outcomes of TPVR using the folded Melody valve technique are favorable with preserved valve function and no increased risk of infective endocarditis compared with the unaltered Melody valve (2-3%). Since the Sapien valve is now available, there is a decreased need to Fold the Melody valve given the shorter length of the Sapien. However, the smallest Sapien valve is 23 mm. Given this, the folded Melody valve technique is still a good option for patients with smaller conduits (14-19 mm). These mid term results are encouraging that this technique does not significantly alter the valve durability.     Photo showing the process of folding the Melody stent valve on a 10 mL syringe. Both ends of the stent have been folded, shortening the length. Shortened valve on the Ensemble delivery system.  

Percutaneous Implantation of Adult Sized Stents for Coarctation of the Aorta in Children ≤20 kg: A 12-Year Experience Brian A Boe 1, Aimee K Armstrong 1, Sarah A Janse 2, Eméfah C Loccoh 3, Katie Stockmaster 1, Ralf J Holzer 4, Sharon L Cheatham 1, John P Cheatham 1, Darren P Berman 1 Circ Cardiovasc Interv. 2021 Feb;14(2):e009399.  doi: 10.1161/CIRCINTERVENTIONS.120.009399. Epub 2021 Feb 5. PMID: 33544625 DOI: 10.1161/CIRCINTERVENTIONS.120.009399   Take Home Points: Stent [which can be dilated to adult size; 18mm] implantation can be performed for coarctation of aorta in children ≤20 kg. Vascular injury is the main concern due to small femoral arterial size and relatively large size of the sheath required for stent implantation. Reintervention is common and needed for either balloon angioplasty of the stent or intervention due to stent fracture. Development of lower profile stents in the future can help improve outcomes.   Commentary from Dr. Varun Aggarwal (Minneapolis, MN, USA), section editor of Congenital Heart Disease Interventions Journal Watch: Most institutions perform primary surgical repair for native coarctation in infants. However, balloon angioplasty or stent implantation is offered to patients with recurrent coarctation of aorta. Stent implantation as the primary intervention for native coarctation is performed in older children and adults when the femoral artery can accommodate the large arterial sheath needed for stent placement. Compared to balloon angioplasty, stent implantation provides better relief of obstruction but results in a fixed diameter (of the stent) which does not grow as the child grows. This is specifically important in young children who will need re-dilation of the stent. The stent which can be placed in small children and subsequently dilated to adult sizes ( 18mm) are limited by the large sheath size.   This study is a retrospective review of the outcomes at Nationwide Children’s hospital of children <20 kg who underwent stent implantation (which can be dilated to adult size in the future) for coarctation of aorta from 2004-2015. The study flow sheet is shown in the Figure 1. 39 patients with a median age and weight of 1.1 (range, 0.3–7.9) years and 9.9 (5.5–20.4) kg, respectively met the inclusion criteria. This cohort comprised of 20 patients with weight 10kg. Acute procedural success was achieved in all patients except one (38/39, 97%) who developed an aneurysm of the aorta requiring placement of a covered CP Stent. The mean gradient across the coarctation was 0mmHg post stent deployment. Adverse events were seen in 7 patients (18%). These consisted of femoral arterial complications at the site of vascular access (n= 3), vascular injury at the site of CoA (n=2), intraprocedural hypotension with bradycardia (n=1), and stent embolization (n=1). Two of the three patients with femoral arterial complication had normal femoral artery on follow up ultrasound. There was no mortality or limb loss due to femoral arterial injury. 28 patients had follow up evaluation of the femoral artery and 4 had persistent vascular injury documented (3 femoral arterial occlusions and one with femoral artery stenosis).   72% (n=28) patients needed reintervention at the median follow up of 67.2 (IQR, 33.8- 116.1) months, Figure 2. The median time to reintervention was significantly shorter in patients who were 10kg at the time of stent implantation (26.5 vs 62.3 months, p=0.01). The most common reintervention was balloon angioplasty of the previously placed stent, however 8 patients had stent fractures (all in Palmaz Genesis XD stents).   This retrospective review by Boe B et al (1) exquisitely describes the technical feasibility of implantation of a stent (which can potentially be dilated to adult size) in children 20kg for coarctation of aorta. Acute success of stent implantation is higher as compared to balloon angioplasty alone. Procedural complications were seen in 18% patients. Vascular access complication (femoral arterial injury) remains a concern in small patients and careful preprocedural assessment of femoral arterial size using ultrasound, avoiding multiple sheath exchanges, use of smaller profile stents and institutional protocol for pulse loss post cardiac catheterization can be a key to a safe and successful outcome. Development of low profile stents can help improve the outcomes in the future.   FIGURE 1: Study Flow sheet (1) [Figure reproduced from Circ Cardiovasc Interv. 2021 Feb;14(2):e009399. (1)]     Figure 2: Kaplan-Meier curve showing time to first reintervention for the entire cohort (solid line) and separated by weight (≤10 kg, dashed line; >10 kg, dotted line). [Figure reproduced from Circ Cardiovasc Interv. 2021 Feb;14(2):e009399. (1)]     1. Boe BA, Armstrong AK, Janse SA, Loccoh EC, Stockmaster K, Holzer RJ, et al. Percutaneous Implantation of Adult Sized Stents for Coarctation of the Aorta in Children </=20 kg: A 12-Year Experience. Circ Cardiovasc Interv. 2021;14(2):e009399.   

Anatomic Approach and Outcomes in Children Undergoing Percutaneous Pericardiocentesis. Myers F, Aggarwal V, Bass JL, Berry JM, Knutson S, Narasimhan S, Steinberger J, Ambrose M, Shah KM, Hiremath G. Pediatr Cardiol. 2021 Feb 16. doi: 10.1007/s00246-021-02563-8. Online ahead of print. PMID: 33590324   Take Home Points: Percutaneous echocardiography-guided pericardiocentesis can be performed safely from a variety of anatomical approaches. Non-subxiphoid approaches are associated with shorter procedure times. The exact approach should be tailored to the clinical characteristics and operator experience.   Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: Pericardiocentesis is a routine procedure that is traditionally performed via a subxiphoid approach. A variety of other anatomic approaches have been described. Data regarding the safety and efficacy of non-subxiphoid approaches in children are lacking. The authors report on procedural and short-term single center outcomes from a variety of anatomic approaches to performing pericardiocentesis.   From August, 2008 to December, 2019 104 patients – median age 52 months (15.6-133.4) and median weight 16.4 kg (10.4-37) - underwent percutaneous pericardiocentesis to drain effusions from a variety of causes (post-hematopoietic stem cell transplant was most common, 53%). All patients had echocardiographic guidance utilized. A non-subxiphoid approach was slightly more common than subxiphoid (58.6% v 41.4%) – figure below details the different approaches. The non-subxyphoid approach resulted in shorter procedure times (21 v 37 min, p=0.005) and was performed in larger (23.6 v 11.2 kg, p=0.013) and older patients (95.9 v 21.7 months, p = 0.006). There were no significant complications in either group.   The authors conclude that percutaneous pericardiocentesis can be performed safely from a variety of anatomical approaches. Echocardiographic guidance can facilitate the performance of this procedure, especially from non-traditional approaches. Non-subxiphoid approaches are associated with shorter procedure time but ultimately the exact approach should be tailored to the clinical characteristics (i.e. location of fluid) and operator experience.     

Supravalvular and Valvular Pulmonary Stenosis: Predictive Features and Responsiveness to Percutaneous Dilation. Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, Dahdah N. Pediatr Cardiol. 2021 Jan 19. doi: 10.1007/s00246-021-02545-w. Online ahead of print. PMID: 33464372   Take Home Points: Echocardiogram has a 56% sensitivity and 82.5% specificity (using angiogram as gold standard) to accurately identify valvular pulmonary stenosis from supra-valvular pulmonary stenosis. Post balloon dilation, patients with supra-valvular pulmonary stenosis had a higher RV-PA gradient as compared to patients with valvular PS. Patients with supra-valvular pulmonary stenosis, balloon-annulus ratio <1.2 or an immediate post intervention gradient 30mmHg had a higher rate of reintervention in the follow up.   Commentary from Dr. Varun Aggarwal (Minneapolis, MN, USA), section editor of Congenital Heart Disease Interventions Journal Watch: Valvar pulmonary stenosis is a common congenital cardiac lesion. Classic valvular pulmonary stenosis with thin doming pulmonary valve leaflets responds very well to balloon pulmonary valvuloplasty. However, patients with coexistent supra-valvular pulmonary stenosis may not respond as well to balloon dilation.   Poupart S et al (1) aimed to evaluate the diagnostic accuracy of echocardiography to differentiate valvular from supravalvular pulmonary stenosis and evaluate the outcomes of percutaneous balloon dilation in supravalvular pulmonary stenosis compared to valvular pulmonary stenosis. They conducted a retrospective analysis of 106 patients from 2006-2017 at a single center in Montreal, Quebec. Both groups had comparable RV-PA gradients prior to intervention (SVPS: 68.04 ± 21.65 vs. VPS: 64.10 ± 14.37; p = 0.312). Using angiogram as a gold standard, the echocardiogram had a low sensitivity of 56% and specificity of 82.5%. A smaller pulmonary artery to pulmonary valve diameter ratio was noted in SVPS as compared to VPS. A pulmonary artery to pulmonary valve annulus ratio (as measured on echocardiogram) of 1.16 had a 64.7% sensitivity and 80.3% specificity to accurately diagnose SVPS.   Post balloon dilation (with similar balloon-annulus ratio in the two groups), the RV-PA gradients were higher in SVPS group as compared to VPS (26.8 ± 12.6 mmHg vs. 11.5 ± 8.5 mmHg; p ≤ 0.001). 71.4% patients with SVPS were deemed resistant to balloon dilation as compared to only 25.9% patients with VPS, p=0.001. Receiver operator curve analysis yielded a cut-off value of 1.20, below which the pulmonary stenosis would likely be resistant to percutaneous angioplasty (AUC = 0.730, p = 0.002).   Figure 1 is a figure from the article (1) demonstrating the follow up echocardiographic gradients in the two groups. The rate of future reinterventions was also higher in the SVPS group (32%) as compared to VPS (6.2%), p<0.05. Despite similar balloon annulus ratios in the two groups, VPS had a higher prevalence of high-grade pulmonary valve insufficiency as compared to SVPS. This study highlights some of the challenges and lower success rate of balloon dilation for SVPS as compared to pure valvular PS. The echocardiographic evaluation prior to the procedure and paying attention to main pulmonary artery diameter to pulmonary valve annulus ratio can help identify these patients more accurately. This information can be helpful in counseling families prior to intervention.   FIGURE 1 Echocardiographic follow-up of RV-PA gradients. Follow-up excludes all data measured after a second intervention, of any kind, on the PV or PA. There was significant decrease in RV-PA gradients in both groups; however, gradients in patients with SVPS were significantly higher than patients with VPS (1).     1. Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, et al. Supravalvular and Valvular Pulmonary Stenosis: Predictive Features and Responsiveness to Percutaneous Dilation. Pediatr Cardiol. 2021.   

Risk Factors for Adverse Events in Children with Pulmonary Hypertension Undergoing Cardiac Catheterization. Vaiyani D, Kelleman M, Downey LA, Kanaan U, Petit CJ, Bauser-Heaton H.Pediatr Cardiol. 2021 Jan 29. doi: 10.1007/s00246-020-02535-4. Online ahead of print.PMID: 33512547   Take Home Points: Longer procedure duration and female gender is correlated with increased risk of adverse events in the cardiac catheterization laboratory. Patients with higher pulmonary artery pressure and PVR with certain levels of pulmonary vasoreactivity testing are more likely to have adverse events.   Commentary from Dr. Arash Salavitabar (Ann Arbor, MI, USA), section editor of Congenital Heart Disease Interventions Journal Watch: The authors sought to describe risk factors for adverse events in children with pulmonary arterial hypertension during cardiac catheterization. This was a 5-year single-center, retrospective study that included patients with indexed pulmonary vascular resistance (PVRi) >3 WU*m2, pulmonary artery (PA) pressure 20 mmHg, and PA wedge pressure 15 mmHg. This institution’s protocol for pulmonary vasoreactivity testing was to perform baseline hemodynamics on room air, followed by 100% FiO2, followed by 100% FiO2 and 40ppm inhaled nitric oxide; however, this was not performed in all children. Adverse events were defined as unplanned events related to anesthesia induction or catheterization from which patient harm could have resulted and occurred within 24 hours of cardiac catheterization.   The authors report 198 cardiac catheterizations in 191 patients that met inclusion criteria. There were 33 patients who received transcatheter interventions. There were 28 (14.1%) adverse events (Table 1). There were 6 deaths, with 4 patients developing PH crisis several days following cardiac catheterization.   There were no significant associations between adverse events and patient age, prematurity, Trisomy 21, congenital heart disease, need for baseline respiratory support or history of bronchopulmonary dysplasia, chronic lung disease, use of pulmonary vasodilators, diuretics, vasoactive medications, or use of iNO. Females were more likely to experience an adverse event than males. Risk factors for adverse events are shown in Table 5, notably showing associations between adverse events and procedure duration, PA pressures and PVR on oxygen, as well as PA pressures on oxygen and iNO. These values were not significantly associated at baseline. There was also a more significant change in PA pressures (but not PVR) in response to oxygen and iNO in those patients who had adverse events. The odds of an adverse event increased by 22% for every 15 minutes increase in procedure times. Odds for an adverse event were also increased for every 10 mmHg increase in mPAP while on oxygen (OR 1.58, CI 1.11-2.26) and 61% while on oxygen and iNO (OR 1.61, CI 1.18-2.21). Controlling for procedure time, females had a 388% increase in the odds of experiencing an adverse event when compared to males (OR 3.88, 95% CI; [1.44–10.40], p = 0.007). The patients with adverse events were more likely to have undergone an intervention.     The authors speculated that the association between adverse events and longer procedure times was due to additional opportunities to develop hypoxia and acidosis, thus predisposing to increased PVR. The procedure duration was rightfully identified in this paper as a potentially modifiable risk factor. This paper also is the first to point to female gender as a risk factor, however there were no additional comments made regarding this statistic and its potential cause. This study nicely adds to the existing literature on risk factors for adverse events in this particularly challenging patient population and will allow for better pre-procedural stratification and counseling.