Congenital Heart Interventions

Transcatheter closure of ventricular septal defects: preliminary results in children weighing 10 kg or less. Mirza M K, Abqari S, Haseen A, Yadav M.Cardiol Young. 2023 Apr;33(4):539-545. doi: 10.1017/S1047951122001147. Epub 2022 May 2.PMID: 35491695 Take Home Points:  Device closure of perimembranous ventricular septal defects is feasible in patients ≤ 10 kg in weight. Careful attention must be given to aortic and tricuspid valve interactions using intraprocedural TEE guidance. Although a retrograde approach was preferred amongst the authors, flexibility regarding approach is needed to limit complications. Commentary from Dr. Arash Salavitabar (Ann Arbor, MI, USA), section editor of Congenital Heart Disease Interventions Journal Watch: The authors of this retrospective single-center study aimed to evaluate the efficacy and safety of transfemoral device closure of perimembranous ventricular septal defects (VSDs) in patients ≤ 10 kg in weight. The authors approached this procedure with initial left ventricular angiography and chose the device based on the presence of a ventricular septal aneurysm, length of subaortic rim, and VSD diameter. The VSD device was chosen to be 1-2mm greater than the largest measurement of VSD color flow by TEE. Over a 7-year period, 16 patients underwent this procedure at a median age of 11 months (IQR 9-15.5) and median weight 8.3 kg (7.2-9.5). The median defect size by TEE was 6.8mm (6-8.5) and median VSD device diameters were 6.2 mm (5.7-8.3). The following devices were use: Amplatzer Duct Occluder II (n=6); Lifetech Symmetric Membranous VSD Occluder (n=4), KONAR-MF VSD Occluder (n=4), Lifetech Eccentric Membranous VSD Occluder (n=1), and Occlutech PmVSD Occluder (n=1). A retrograde deployment was preferred (14/18 total device implantation attempts). The only comment regarding choice of device was regarding the tendency to use symmetric double-disc occluders in all but one patient. The procedural success rate was 88% (n=14). Of the two unsuccessful device placements, one involved development of moderate aortic regurgitation via retrograde approach and moderate TR via antegrade approach. The other unsuccessful case was a patient who developed progressive, moderate AR over 2 days post-procedure. Each of these patients required surgery. There were no device embolizations. On median follow-up of 40.5 months (25-64), none developed complete heart block. This paper nicely showed that this procedure can be performed safely in small patients with perimembranous VSDs, with careful consideration to VSD anatomy, aortic valve rim, and interactions with aortic valve and tricuspid valve apparatus. Importantly, the majority of the devices used by the authors are not available in the United States, which emphasizes the importance of formal investigation and future FDA approval of additional device options for this indication. Similar studies are needed to understand the details of ventricular septal anatomy, optimal device choice, and patient selection.

Pre-Fontan Assessment Utilizing Combined Cardiac Catheterization and Cardiac MRI: Comparison to the Pre-Fontan Catheterization. Eilers LF, Britt JJ, Weigand J, Penny DJ, Gowda ST, Qureshi AM, Stapleton GE, Khan A, Webb MK, Bansal M.Pediatr Cardiol. 2023 May 21. doi: 10.1007/s00246-023-03178-x. Online ahead of print. PMID: 37210685 Take Home Points: Cardiac magnetic resonance imaging (CMR) allows for a comprehensive assessment of ventricular function, aortopulmonary collateral burden, and lymphatic abnormalities. Combined CMR and cardiac catheterization led to a decrease in catheterization duration, amount of contrast received, and fluoroscopy time. CMR performed prior to the catheterization allowed for more targeted aortopulmonary collateral occlusion without a change in short term post-operative outcomes. Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch: Patients with single ventricle congenital heart disease undergo staged palliation to separate the systemic and pulmonary circulations. The final stage is an inferior cavopulmonary anastosis, or Fontan procedure. Survival through stage palliation has improved in the modern era, though there is still significant morbidity (including systemic ventricular dysfunction, protein losing enteropathy, plastic bronchitis, liver and kidney dysfunction, lymphatic dysfunction). This morbidity highlights the need to ensure appropriate patient selection for Fontan completion versus consideration for hear transplant evaluation. Typical evaluation prior to Fontan has included echocardiography and cardiac catheterization.  However, cardiac magnetic resonance (CMR) offers the ability to define anatomy, provide a more thorough assessment of ventricular function, better quantify AV valve regurgitation, quantitatively assess aortopulmonary (AP) collateral burden, and define lymphatic abnormalities prior to Fontan. The authors hypothesized that combined CMR and cardiac catheterization could offer a thorough hemodynamic and anatomic assessment and allow for targeted intervention. A single center, retrospective cohort study was performed on all patients who underwent combined pre-Fontan CMR and cardiac catheterization from 10/2018-4/2022.  These were then matched randomly by month of procedure to patients who underwent pre-Fontan catheterization only. A total of 37 combined patients and 40 cardiac catheterization only patients were included in the analysis. In the combined group, all patients were placed under general anesthesia and underwent the CMR prior to cardiac catheterization. The CMR protocol included cine SSFP function, cine SSFP anatomy, 3D MRA with gadolinium, and phase-contrast encoded velocity mapping of flows. In the combined group, an angiogram was routinely performed in the SVC with an aortagram performed at the discretion of the interventional cardiologist. Those who underwent a catheterization only also had angiograms performed in the IVC, descending aorta, and pulmonary vein(s). Patients in the combined group had a longer duration of intubation (293 minutes, SD 51 minutes versus 193 minutes, SD 73 minutes) and anesthesia time (320 minutes, IQR 290-352 minutes versus 200 minutes, IQR 165-255 minutes). Time in the catheterization lab, procedure time (start of access to sheath removal), contrast dose per kilogram, and fluoroscopy time were all lower in the combined group. Radiation dose was also lower in the combined group, though this was not statistically significant. Patients who underwent a cardiac catheterization only were more likely to have an intervention performed (48% versus 19%) with the majority of these being aortopulmonary collateral occlusion. Fontan completion, cardiopulmonary bypass time, ICU length of stay, and post operative chest tube duration was not significantly different between groups. CMR offers significant benefits as part of the pre-Fontan cardiac evaluation. In addition to evaluating systemic ventricular function more thoroughly than echocardiography, CMR also provides an objective assessment of AV valve regurgitation (via regurgitant fraction), can evaluate the lymphatic system, and allows quantification of aortopulmonary collateral burden. This study showed that CMR performed as part of the pre-Fontan cardiac catheterization can reduce case duration, iodinated contrast volume, and radiation exposure. Additionally, pre catheterization CMR allowed for more targeted interventions (especially aortopulmonary collateral occlusion) with objective data (elevated end diastolic pressure or 30% or greater collateral flow) determining intervention instead of the discretion of the interventional cardiologist. Using these guidelines, the authors showed that post-operative outcomes were similar between the groups.

Authors: Luxford JC, Adams PE, Roberts PA, Mervis J. Journal: Heart Lung and Circulation 2023 32, 638-644. Doi.org 10.1016 Take home points: Retrospective review of 20 patient (all < 3 months of age) from Sydney, Australia with primary RVOT stenting Average improvement with mean saturation increased from 80% to 91%. 18 survived (2 non cardiac deaths) with 12 requiring re intervention on the RVOT stent prior to open heart repair. All 18 achieved definitive repair at ~ 6 months of age. The need for more than one RVOT stent was associated with longer bypass and cross clamp. Commentary from Dr. Thomas Zellers (Dallas, USA), section editor of Congenital Heart Disease Interventions and ACHD Journal Watch: This is a retrospective study evaluating all patients undergoing RVOT stenting between January 2010 and December 2020. Only patients with native RVOT stenosis were included; no rescue, secondary implants or conduit implants were included in the analysis. The study was approved by the hospital Ethics Committee and statistics were summarized using standard statistical analysis on SPSS and GraphPad Prism software with a significant P value of < 0.05. Catheterization technique involve the use of a 4Fr or 5Fr right coronary catheter, a coronary wire and delivery without a long sheath in the RVOT. The group switched from bare metal to covered stents during the study evaluation because it reduced intimal proliferation. Aspirin 5 mg/kg/day dosing was recommended if there was no contraindication. Follow up was continued to their surgery and until their last follow up unless they died prior to surgery. The population included 12 with hypercyanotic spells and 8 who were PDA dependent and on prostaglandin. The majority were neonates and 8 were less than 2.5 kg, 18 were urgent procedures and 12 had a major co-morbidity or genetic syndrome. There was one major complication with need for isoprenaline for heart block, which resolved with time.The stents were enlarged to an average of 5-6 mm and 18 of 20 survived (No cardiac deaths). Thirteen required reintervention prior to definitive repair. Only one required reintervention within a week of the original procedure. Pulmonary artery growth increased with time to nearly z scores < -1 or greater in the majority. Tables: Conclusions: RVOT stenting became the standard of care at this hospital with excellent results (better than standard BT shunts in their population) and could be performed in small neonates safely. The majority transitioned to covered stents which reduced intimal hyperplasia but did not change the time to definitive repair or reintervention. The pulmonary artery growth was normal but more than one RVOT stent increased the cross clamp and bypass time.

Pre-Glenn aorto-pulmonary collaterals in single-ventricle patients. Sharma VJ, Carlson L, Esch J, Gopal M, Gauvreau K, Wamala I, Muter A, Porras D, Nathan M.Cardiol Young. 2023 Dec;33(12):2589-2596. doi: 10.1017/S1047951123000665. Epub 2023 Apr 17.PMID: 37066762 Take Home Points Male sex, age at pre-bidirectional Glenn catheterization and increased Qp:Qs are associated with a moderate to severe AP collateral burden. AP collateral burden is not associated with death, transplantation, or the incidence of pulmonary artery interventions. Of the patients who need an intervention for AP collaterals, more than half need only a single intervention. Commentary from Dr. Jonathon Hagel (C.S. Mott Children’s Hospital, University of Michigan), section editor of Congenital Heart Disease Interventions Journal Watch: Aorto-pulmonary (AP) collaterals are commonly found in single ventricle patients due to presumed hypoxia-induced vascular endothelial growth factor activity. The authors sought to describe the risk factors for developing AP collaterals following the Norwood procedure. They secondarily sought to determine in AP collateral burden has an impact on death, transplantation, or pulmonary artery interventions. From January 2011 until March 2016, 104 patients underwent the Norwood procedure for various forms of single ventricle heart disease, the most common of which was hypoplastic left heart syndrome (77.9%) with Sano shunt modifications in most cases (78.8%). 84 patients underwent both a pre-bidirectional Glenn catheterization and subsequent bidirectional Glenn procedure and thus were included in the analysis. AP collateral interventions were performed during the pre-bidirectional Glenn catheterization in 54.8% of cases. AP collateral interventions were performed after the bidirectional Glenn procedure in 82% of cases and of the cases that underwent intervention for AP collaterals, 45.2% underwent a single procedure to target the collaterals. On multivariable analysis, male sex (OR 3.36; 95% CI 1.17-11.4), age at pre-bidirectional Glenn assessment (OR 2.12; 95% CI 1.33-3.39 per month increase) and Qp:Qs ratio (OR 1.23; 95% CI 1.08-1.41 per 0.1 unit increase) were significantly associated with moderate to severe AP collateral burden. AP collateral burden diagnosed at the time of pre-bidirectional Glenn catheterization was not associated with death or transplantation (HR 1.19; 95% CI 0.37-2.61). The incidence of pulmonary artery intervention did not vary significantly based on severity of AP collateral burden. The authors conclude that AP collateral burden after the Norwood procedure is common and that Qp:Qs, male sex, and age of the pre-bidirectional Glenn catheterization are strong markers of AP collateral burden but the presence of AP collaterals does not confer an increased risk of death, transplantation, or pulmonary artery intervention. Due to the lack of well-established guidelines for the diagnosis or management of AP collaterals, the frequency and timing of intervention more represent an institutional approach rather than a clinical indication for intervention which makes inference on association with long term outcomes difficult though at least in this single center study, severity of AP collateral burden was not associated with adverse long-term outcomes.

Predictors of Serious Adverse Events and High-Level Cardiorespiratory Support in Patients Undergoing Transcatheter Pulmonary Vein Interventions. Barreto JA, Gauvreau K, Porras D, Esch JJ, Maschietto N, Quinn B, Bergersen L, Stein M, Callahan R.Pediatr Cardiol. 2023 Apr;44(4):806-815. doi: 10.1007/s00246-023-03129-6. Epub 2023 Mar 3.PMID: 36869157 Take Home Points Adverse events related to transcatheter pulmonary vein (PV) interventions are common with an incidence of ~25% though major/catastrophic adverse events (i.e. death, ecmo, stroke) are rare (1.8%). There is no association between higher number of PVs or the number of balloon/stent angioplasties and serious adverse events or high-level cardio-respiratory support following catheterization. Younger patients and those with abnormal hemodynamics secondary to PVS are more likely to have a serious adverse event or require high-level cardiorespiratory support following catheterization. Commentary from Dr. Jonathon Hagel (C.S. Mott Children’s Hospital, University of Michigan), section editor of Congenital Heart Disease Interventions Journal Watch: Transcatheter pulmonary vein (PV) interventions are the mainstay in therapy for patients with recurrent pulmonary vein stenosis (PVS). The authors sought to describe the frequency and predictors of serious adverse events (AEs) in patients undergoing transcatheter PV interventions through a retrospective review of a large cohort of patients at a quaternary care facility with a well-established PVS program. They also aimed to determine risk factors associated with high-level cardio-respiratory support following transcatheter PV intervention. From March 1, 2014 to December 31, 2021, 240 patients with a total of 841 catheterizations involving PV interventions were included in this study. The median age at intervention was 12 months (IQR 9-26). A majority (84%) had PVS related to congenital heart disease, of which anomalous pulmonary venous return was the most common diagnosis (41%). More than half (57%) of cases involved five or more balloon dilations or stents. At least one AE was reported in 208 (25%) of cases with one serious AE in 100 cases (12%) major AE in 14 cases (1.7%) and catastrophic AE in 1 case. Age less than 6 months, weight less than 5 Kg, low systemic arterial saturation, severely elevated mean PA pressure and pre-catheterization hospital admission were associate with a higher odds of serious AEs. There was no association between number of PVs undergoing intervention or number of balloon/stent angioplasties and serious AEs or high-level cardiorespiratory support following catheterization. Hospitalization prior to catheterization, patients younger than one year at time of catheterization and moderate-severe RV dysfunction were more likely to require high-level cardiorespiratory support following catheterization. The authors conclude that given the lack of association between multiple interventions on a high number of PVs and adverse outcomes or high-level cardiorespiratory support, it is safe to plan such cases. Adverse events and high-level cardiorespiratory support are more likely in younger patients and those with abnormal hemodynamics secondary to PVS and therefore appropriate case planning for recovery and post-catheterization disposition should be made.  

Percutaneous approach to residual pulmonary bifurcation stenosis in conotruncal diseases.Castaldi B, Di Candia A, Cuppini E, Sirico D, Reffo E, Padalino M, Vida V, Di Salvo G.Cardiol Young. 2024 Jan;34(1):24-31. doi: 10.1017/S1047951123000999. Epub 2023 May 4.PMID: 37138545 Take home points: Percutaneous treatment of pulmonary bifurcation stenoses with kissing balloon inflation using high-pressure balloons is effective in most cases. Stenting of the pulmonary bifurcation is required when there is a concomitant indication for pulmonary valve implantation. Commentary from Dr. Milan Prsa (Switzerland, Europe), section editor of Congenital Heart Disease Interventions Journal Watch: Pulmonary bifurcation stenoses (PBS) are a relatively common problem after repair of conotruncal defects (CTDs). As surgical angioplasty is often inadequate due to residual stenosis, percutaneous treatment is usually preferred. However, successful ballooning and/or stenting of PBS can be very challenging and long-term results are scantly reported. In this retrospective study, the authors analyse the mid-term outcomes of different interventional strategies to treat obstructions involving the distal main pulmonary artery and/or the origin of pulmonary artery branches. Between January 2014 and December 2021, 39 patients (age 8.7 ± 7.8 years, weight 31.0 ± 26.3 kg) with repaired CTDs (22 with tetralogy of Fallot, 8 with truncus arteriosus, and 9 with d-transposition of the great arteries) underwent percutaneous treatment of hemodynamically significant PBS at the Padua University Hospital. Types of interventions included single high-pressure balloon inflation (SB), kissing balloon inflation (KB), standard stent implantation techniques without jailing of a branch pulmonary artery (SS) and more complex stent implantation techniques involving provisional jailing of a branch pulmonary artery (JS). Procedure success, defined as a residual invasive pressure gradient across the pulmonary bifurcation of <20 mmHg, was 15% (5/33) for SB, 60% (6/10) for SS, 82% (14/17) for KB and 100% (10/10) for JS. The treatment and outcome flowchart is presented in Figure 1. Eight patients undergoing JS also had transcatheter pulmonary valve replacement (TPVR). Complications included inadvertent jailing of a pulmonary artery in 2 patients undergoing SS and hemothorax from guidewire injury, requiring percutaneous drainage and blood transfusion, in 1 patient undergoing JS. At a mean follow-up of 20 ± 15 months, the maximum instantaneous gradient across the right ventricular outflow tract by echocardiography after successful SB, SS, KB and JS was 33.2 ± 14.7 mmHg, 30.0 ± 7.8 mmHg, 25.0 ± 11.2 mmHg and  25.4 ± 13.1 mmHg, respectively. When comparing KB to JS, patients treated with KB were younger (6.0 ± 5.2 years vs. 13.6 ± 5.4 years) and more heterogenous (6 with d-transposition of the great arteries, 8 with tetralogy of Fallot, and 3 with truncus arteriosus vs. 9/10 with TOF for JS). This retrospective single-center study involving a small and heterogeneous patient sample suggests that kissing balloon inflation with high-pressure balloons to treat PBS after repair of CTDs is successful and sufficient in most cases and might be preferred in small children and those not needing TPVR. When insufficient or when there is an indication for concomitant TAPVR, complex bifurcation stenting requiring considerable operator experience is necessary. Figure 1. Flowchart of treatment strategies and outcome of 39 patients with pulmonary bifurcation stenosis.

Management of Complex Pulmonary Vein Stenosis at Altitude Combining Comprehensive Percutaneous Interventional Treatment with Sirolimus, Pulmonary Hypertension Medications and Intraluminal Imaging with Optical Coherence Tomography. Shorofsky MJ, Morgan GJ, Mejia E, Rodriguez SA, Greene M, Sheaks P, Ivy D, Zablah JE.Pediatr Cardiol. 2023 Jun;44(5):1125-1134. doi: 10.1007/s00246-023-03102-3. Epub 2023 Feb 1.PMID: 36723625 Take Home Points: Treatment of pulmonary vein stenosis in pediatric patients with congenital heart disease is rapidly evolving with improving outcomes. Sirolimus therapy may be a useful adjuvant therapy in this complex group of patients as it seems to decrease disease progression and need for reintervention. Commentary from Dr. Konstantin Averin (Cohen Children’s Heart Center), catheterization section editor of Pediatric Cardiology Journal Watch: Pulmonary vein stenosis (PVS) in pediatric patients with congenital heart disease is a complex disease that carries a high degree of morbidity and mortality.  There have been significant improvements outcomes over the last few years utilizing a multi-modality treatment approach – including surgery, percutaneous intervention, and medical therapy.  There remains significant variability between centers and treatment algorithms.  The authors report a single center retrospective experience of using systemic sirolimus therapy (SST) in conjunction with pulmonary hypertension mediations and imaging with optical coherence tomography. From October 2020 to December 2021, 10 patients were started and followed on SST – median age at SST initiation 25 months, median weight 10.6 kg (IQR 5.4-21.5 kg).  The authors describe both their PVS medical management protocol and their SST protocol (see below) including how to adjust sirolimus dosing while on bosentan therapy for pulmonary hypertension.  There protocols should be of particular interest to any practitioner considering treatment of this complex group of patients.  Overall, when comparing pre- and post- SST, patients required less frequent catheterizations (3 months   which were also shorter in duration and the severity of disease recurrence was diminished as defined by the degree of vein re-stenosis.  There were no significant serious adverse effects from SST.This study clearly has limitations, including its retrospective nature, the small cohort size, and the inherent heterogeneity in this patient population. The authors conclude that addition of SST helps decrease disease progression with decreased frequency of interventions.  Despite the studies limitations, the conclusions are broadly consistent with the existing literature, suggesting that SST can be a critical adjunct to percutaneous intervention in improving outcomes in patients with moderate to severe PVS.  This study also highlights the need for a multidisciplinary, protocol driven approach to managing pediatric PVS and underscores the necessity of careful consideration of medication interactions, particularly in patients concurrently treated for pulmonary hypertension.  Figure 1. Figure 2.

Effects of time of bed rest on vascular complications after cardiac catheterization in pediatric patients with congenital heart disease: A randomized controlled trial. Cho E, Jang MR, Moon JR, Kim MJ, Kim YM, An YJ, Kang IS, Song J.Heart Lung. 2023 Jul-Aug;60:52-58. doi: 10.1016/j.hrtlng.2023.02.023. Epub 2023 Mar 11. Take Home Points: Minimizing supine bedrest time for pediatric patients following cardiac catheterization can have a significant impact on the stress for the patient and their family. The standard 4 to 6 hours of supine bedrest that many centers practice may be more than is needed. Further studies with a larger cohort of patients that include analysis of sheath size in comparison with patient size could help answer this question more thoroughly Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch: Patients who undergo cardiac catheterization via the femoral vessels require supine bedrest following the procedure.  While there is significant central variation, this is frequently done for 2 to 6 hours.  This can be challenging in the pediatric population, especially in younger patients.  They may require sedative medications to assist in ensuring adequate immobility of the leg to prevent complications such as bleeding or hematoma development.  Recent adult studies have shown that 1.5 to 2 hours of supine bedrest is adequate.  However, there is a much wider variability in case duration and sheath size used in pediatric patients.  Given this, the authors thought to determine if there were differences in bleeding incidents, vascular complications, need for sedatives, and pain in patients who are required to complete 2 (experimental group) versus 4 (control group) hours of supine bedrest. A prospective, randomized open label trial was performed from 10/21 to 5/20/2022.  The study included patients who were between 30 days and 16 years old.  Patients with congenital bleeding disorders, in whom femoral vessel access was difficult, those requiring emergency cardiac catheterizations, those requiring ICU level care following the catheterization, and those with an ACT >250 seconds following the catheterization were excluded.  Bleeding and vessel complications were identified via inspection and palpation and scored as a 0 if there was no bleeding, 1 if there was a small amount of bleeding, 2 if there was a palpable hematoma and/or severe bleeding and 3 if there was arterial occlusion/aneurysm/AV fistula/abdominal hematoma).  Pain was assessed using verified rating scales (face–legs–activity–cry–consolability in <3 years, face pain rating scale 4-6 years, and numeric rating system 7 years and older).  At the occlusion conclusion of the case, an ACT was checked, and sheaths were removed. Clo-Sur plus hemostatic pads were applied to all patients and manual compression was performed until hemostasis was achieved.  A sandbag was placed on the site prior to leaving the cardiac catheterization lab.  Bleeding, vessel complications, pain at the access site, and administration of analgesics were evaluated every hour for 4 hours and again in 18 hours in both groups. A total of 84 patients were randomized (42 in each group).  Patients in the experimental group were slightly younger, smaller, and more likely to be taking aspirin or warfarin prior to their procedure than those in the control group.  There were no statistically significant differences in bleeding incidents, vessel complication, pain scores or sedative administration between the groups (no bleeding or vascular complications were seen in either group). This study sought to determine the amount of supine bedrest time needed in pediatric patients following a cardiac catheterization.  There is certainly value in determining this as it can be challenging, especially for younger patients, to remain supine following procedures.  The authors showed that 2 hours appears to be an adequate amount of supine bedrest time following a cardiac catheterization at their center and a small number of patients.  However, more data on this is likely needed before more widespread adoption of this practice.  The center where the catheterizations were performed used sandbags on all access sites.  This is not necessarily a standard practice at many pediatric centers as it can be quite uncomfortable. They did not seek to evaluate whether sheath size used for the procedure has an impact on bleeding/vascular complication risk, pain, and potential need for longer supine bedrest.  Before universally adopting 2-hour bedrest, it would be useful to determine if a sheath size to patient weight ratio placed patients at higher risk for bleeding and/or vascular complications.