Comparing Neurodevelopmental Outcomes in Infants With Patent Ductus Arteriosus Stenting Versus Blalock-Taussig-Thomas Shunt: A Pilot Study.

Background: Patent ductus arteriosus stenting (PDAS) is a nonsurgical alternative to Blalock-Taussig-Thomas shunt (BTTS) for infants with ductal-dependent congenital heart disease. In this single-center study, we aimed to compare neurodevelopmental outcomes in children who underwent BTTS as initial palliation versus PDAS. Methods: Bayley Scales of Infant and Toddler Development Screening Test (Bayley-III) reports and mode of feeding data were collected for any patient who underwent PDAS or BTTS at Rady Children's Hospital from 2013 to 2021. We also prospectively administered the Parents' Evaluation of Development Status questionnaire (PEDS) to parents of children aged 2-8 years in this patient population. Results: Of the 99 patients, 64 received a Bayley-III assessment and/or PEDS screen. Of the 35 who had a Bayley-III, there was a higher proportion of patients with PDAS who scored as developmentally appropriate compared with BTTS. PEDS screen showed that a higher proportion of patients with PDAS had no parental concern for delay than that of patients with BTTS (63% vs 30%). Patients with BTTS were more likely to undergo gastrostomy tube placement than patients with PDAS. Conclusions: Our study suggests that neurodevelopmental measures are feasible, clinically relevant, and should be included in comparative effectiveness studies of infant congenital interventions. Whether PDAS offers neurodevelopmental benefit over BTTS should be confirmed in a prospective powered randomized controlled clinical trial.

Transcatheter pulmonary valve replacement after arterial switch operation.

BACKGROUND: Patients with d-transposition of the great arteries (d-TGA) who have undergone an arterial switch operation (ASO) can develop right ventricular outflow tract (RVOT) dysfunction with pulmonary regurgitation (PR) or stenosis. In these patients, treatment may include transcatheter pulmonary valve replacement (TPVR). Coronary compression is a contraindication occurring in 5% of typical TPVR cases. After ASO, there are various anatomical considerations that can confound TPVR, including potential coronary artery compression. Our goal is to understand feasibility of TPVR in patients following ASO. METHODS: This was a retrospective multicenter cohort study of patients with RVOT dysfunction after ASO who underwent cardiac catheterization with intention to perform TPVR from 2008 to 2020. RESULTS: Across nine centers, 33 patients met inclusion criteria. TPVR was successful in 22 patients (66%), 19 receiving a Melody valve and 3 a SAPIEN valve. RVOT stenosis in isolation or with PR dictated need for TPVR in nearly all patients. One serious adverse event occurred with valve embolization. After TPVR, the RVOT peak gradient decreased from 43 to 9 mm Hg (p < 0.001); PR was trivial/none in all but one patient, in whom it was mild. Coronary compression prohibiting TPVR occurred in eight patients (24%) and two patients (6%) had severe aortic regurgitation from aortic root deformation precluding TPVR. Seven patients underwent RVOT reintervention with a median of 5.3 years post-TPVR. CONCLUSIONS: TPVR in patients with d-TGA after ASO is feasible, but in this cohort, coronary compression or aortic root distortion precluded TPVR in one-third of patients. The rate of RVOT reintervention after TPVR was higher in this cohort of ASO patients that in prior studies.

Stenting of the Patent Ductus Arteriosus.

Since PDA stenting was first attempted in the early 1990s, significant technical advancements have improved outcomes and some centers have even transitioned to exclusive PDA stenting for all infants with duct-dependent pulmonary circulation. In addition to its use in infants with duct-dependent pulmonary circulation, PDA stenting has also been adapted as a percutaneous palliative option for suprasystemic pulmonary arterial hypertension and as a component of the hybrid procedure. In this article, the authors aim to review indications and outcomes for PDA stenting, describe the procedure, and discuss future directions.

Early identification of SOX17 deficiency in infants to guide management of heritable pulmonary arterial hypertension using PDA stent to create reverse Potts shunt physiology.

Heritable pulmonary arterial hypertension (HPAH) is a rare progressive condition that includes patients with an identified genetic cause of pulmonary arterial hypertension (PAH). HPAH and idiopathic PAH (IPAH) have an estimated combined incidence of 0.5-0.9 cases per million children-years. Several pathogenic variants have been associated with HPAH in children and adults, including genes BMPR2, TBX4, and ACVRL1, and more rarely with variants in genes such as SOX17. HPAH is often difficult to manage and has poor prognosis despite advances in medical therapy with many patients progressing to lung transplantation, right heart failure and death. Surgical and transcatheter Potts shunt creation can reduce systolic burden and has shown reduction in morbidity and mortality in children. Early genetic testing can provide both diagnostic and prognostic value in managing and counseling children with severe PAH and it can guide transcatheter or surgical management in refractory cases despite maximal medical therapies. We describe a patient with HPAH (SOX17 mutation) who underwent percutaneous patent ductus arteriosus stent for right ventricle decompression at 2 months of age with clinical management guidance by genetic testing results.

Stage 1 and 2 Palliation: Comparing Ductal Stenting and Aorto-Pulmonary Shunts in Single Ventricles with Duct-Dependent Pulmonary Blood Flow.

Patent ductus arteriosus stenting (PDAS) for ductal-dependent pulmonary blood flow (DDPBF) provides a new paradigm for managing neonates with single ventricles (SV). Currently, sparse data exist regarding outcomes for subsequent palliation. We describe our experience with inter-stage care and stage 2 (S2P) conversion with PDAS in comparison to a prior era of patients who received surgical aorto-pulmonary shunts (APS). Retrospective review of 18 consecutive DDPBF SV patients treated with PDAS between 2016 and 2021 was done and compared with 9 who underwent APS from 2010 to 2016. Patient outcomes and pulmonary artery (PA) growth were analyzed. S2P was completed in all 18 with PDAS with no cardiac arrests and one post-S2P mortality. In the 9 APS patients, there was one cardiac arrest requiring ECMO and one mortality inter-stage. Off cardiopulmonary bypass strategy was utilized in 10/18 in the PDAS and 1/9 in the APS group (p = 0.005) at S2P. Shorter ventilation time, earlier PO feeding, and shorter hospital stay were noted in the PDAS group (p = 0.01, p = 0.006, p = 0.03) (S2P). Median Nakata index increase inter-stage was not significant between the PDAS and APS at 94.1 mm2/m2 versus 71.7 mm2/m2 (p = 0.94). Median change in pulmonary artery symmetry (PAS) was - 0.02 and - 0.24, respectively, which was statistically significant (p = 0.008). Neurodevelopmental outcomes were better in the PDAS group compared to the APS group (p = 0.02). PDAS provides excellent PA growth, inter-stage survival, progression along multistage single-ventricle palliation, and potentially improved neurodevelopmental outcomes. Most patients can be transitioned through 2 stages of palliation without CPB.

Chronic active Epstein-Barr virus and hydroa vacciniforme-like lymphoproliferative disorder in a pediatric patient complicated by fatal ruptured cerebral artery aneurysm.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a rare cutaneous form of chronic active Epstein-Barr virus (CAEBV) that presents with vesicular lesions induced by sun-exposure. Arterial aneurysm is a rare but potentially fatal complication of CAEBV and HV-LPD.

Surgical Mitral Valve Replacement Using a Catheter-Based MelodyTM Valve in a Landing Zone Constructed With a PTFE-Covered Expandable Cheatham-PlatinumTM Stent.

Mitral valve replacement using a Melody valve is a promising solution to the challenge of surgical mitral valve replacement in infants with a hypoplastic annulus. We report the creation of a landing zone in the mitral valve annulus using a Cheatham-Platinum (CP)-covered stent that facilitates Melody valve placement, helps prevent paravalvular leak, minimizes left ventricular outflow tract obstruction, and allows for potential future dilation of the valve.

Safety and Short-Term Outcomes for Infants < 2.5 kg Undergoing PDA Device Closure: A C3PO Registry Study.

To evaluate short-term procedural outcomes and safety for infants < 2.5 kg who underwent catheterization with intended patent ductus arteriosus (PDA) device closure in a multi-center registry, as performance of this procedure becomes widespread. A multi-center retrospective review was performed using data from the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. Data were collected for all intended cases of PDA closure in infants < 2.5 kg from April 2019 to December 2020 at 13 participating sites. Successful device closure was defined as device placement at the conclusion of the catheterization. Procedural outcomes and adverse events (AE) were described, and associations between patient characteristics, procedural outcomes and AEs were analyzed. During the study period, 300 cases were performed with a median weight of 1.0 kg (range 0.7-2.4). Successful device closure was achieved in 98.7% of cases with a 1.7% incidence of level 4/5 AEs, including one periprocedural mortality. Neither failed device placement nor adverse events were significantly associated with patient age, weight or institutional volume. Higher incidence of adverse events associated with patients who had non-cardiac problems (p = 0.017) and cases with multiple devices attempted (p = 0.064). Transcatheter PDA closure in small infants can be performed with excellent short-term outcomes and safety across institutions with variable case volume.

Transcatheter Hepatic Conduit-Azygous Vein Connection Reduces Pulmonary Arteriovenous Malformations in a Cyanotic Fontan Patient.

The authors report a closed-chest, transcatheter large-vessel connection (hepatic conduit to azygous vein) to reverse pulmonary arteriovenous malformations in a 10-year-old patient after Fontan for heterotaxy/interrupted inferior vena cava, with an increase in oxygen saturation from 78% to 96%. Computational fluid dynamics estimated a 14-fold increase in hepatic blood flow to the left pulmonary artery (from 1.3% to 14%). (Level of Difficulty: Advanced.).

Exercise Stress Echocardiography in Kawasaki Disease Patients with Coronary Aneurysms.

The most significant sequelae of Kawasaki disease (KD) are coronary artery aneurysms, which can lead to risk of future myocardial ischemia. Exercise stress echocardiography allows for non-invasive assessment of myocardial dysfunction. We reviewed our single center experience with exercise stress echocardiography in patients with previous history of KD with coronary aneurysms. We reviewed the records of 53 KD patients who underwent exercise stress echocardiography from 2000 to 2020. Abnormal stress echocardiograms were defined as those showing no increase in biventricular systolic function post-exercise or regional wall motion abnormalities. Computed tomography angiography and cardiac magnetic resonance imaging were reviewed for patients with abnormal stress echocardiograms. Clinical data were reviewed and correlated with stress echocardiogram results. Of the 53 patients, three (5.7%) had an abnormal exercise stress echocardiogram. All three patients were classified as AHA Risk Level 4 or 5 by coronary Z-score (internal dimension normalized for body surface area) and were confirmed to have coronary aneurysms, stenosis, or myocardial tissue perfusion defects on advanced cardiac imaging that could account for the results seen on stress echocardiogram. Exercise stress echocardiography detected signs of myocardial ischemia in a subset of high-risk patients with Kawasaki disease and coronary aneurysms and may be considered as a useful screening tool for this complex patient cohort.

Bronchial Remodeling Following Airway Stenting in Pediatric Patients With Tracheobronchial and Congenital Heart Disease.

Background: Treatment of tracheobronchial disease in medically complex infants with congenital heart disease (CHD) is often challenging. When conservative management or surgery fails or is contraindicated, airway stenting can allow for advancement of care or weaning of respiratory support. Methods: We identified 8 cases of airway stenting with balloon-expandable coronary bare-metal stents performed at our institution between February 2019 and September 2022 to relieve conservative treatment-refractory tracheobronchial disease in pediatric patients with CHD. All patients underwent rigid microlaryngoscopy, bronchoscopy, and flexible bronchoscopy as well as computed tomography angiography. Results: Eight patients underwent technically uncomplicated placement of balloon-expandable coronary bare-metal stents in the trachea or bronchus. Immediate improvement in respiratory parameters was noted following stent placement. Six patients were able to wean mechanical ventilation following stent placement, with a median of 2.5 days of mechanical ventilation following the procedure (range, 0-219). All stents were subsequently endoscopically removed at a median of 6.8 months (range, 0.4-16.3 months). In 6 patients, bronchoscopy after stent removal demonstrated a rounder configuration of the airway consistent with bronchial remodeling. Conclusions: In pediatric patients with tracheobronchial and CHD, airway stenting with balloon-expandable bare-metal coronary stents relieved respiratory symptoms with minimal complications and resulted in bronchial remodeling after stent removal.

Simultaneous Stenting With Edwards SAPIEN Transcatheter Pulmonary Valve Replacement.

Background: Prestenting of the landing zone for transcatheter pulmonary valve replacement (TPVR) with a balloon-expandable valve can dilate a stenotic right ventricular outflow tract (RVOT), prevent paravalvar leak (PVL), and protect against conduit tear. Simultaneous stenting (SS) with the Melody valve has been described, but to our knowledge, SS with a SAPIEN valve has not been reported. We report our experience with this novel technique. Methods: A retrospective chart review of patients who underwent TPVR at Rady Children's hospital and UCSD Medical Center was performed. Patients were included if they had underwent SAPIEN TPVR with SS. Rationale for stent choice was a bare metal stent to relieve long-segment stenosis and covered stents to prevent PVL or to protect against conduit tear. Results: A total of 17 cases were identified. The majority of RVOTs were transannular patches (n = 9, 56%), with a minimum diameter of 19.6 ± 5.2 mm, and the most common valve placed was an Edwards SAPIEN 26.0 mm (n = 10, 59%). All SAPIEN valves placed were of the S3 generation. The procedure was successful in all patients, with no conduit tears. Minor complications occurred in 3 patients (17.6%). Conclusions: Simultaneous stent deployment with a SAPIEN TPVR is an alternative 1-step technique for patients who require prestenting. SS simplifies the procedure, has low complication rates, and offers the benefits of a longer landing zone and decreased PVL.

Impact of Stenting on PDA Length, Curvature, and Pulsatile Deformations Based on CT Assessment.

Background: We sought to investigate the impact of stenting on native patent ductus arteriosus (PDA) length, curvature, and pulsatile deformations in patients with ductal-dependent pulmonary circulations. Methods: Patients with PDA stents who received contrast-enhanced 3-dimensional computed tomography with a view of the PDA, thoracic aorta, and pulmonary arteries were retrospectively included in this study. Geometric models of the prestented and poststented PDA were constructed from the computed tomography images, and PDA arclength, curvature, and pulsatile deformations were quantified. Results: A total of 12 patients with cyanotic congenital heart disease were included, 10 of whom received 1 stent in the PDA and 2 received multiple overlapping stents. From prestenting to poststenting, the PDA shortened by 26 ± 18% (P = .004) and decreased in mean and peak curvature by 60 ± 21% and 68 ± 15%, respectively (both P < .001). Pulsatile deformations varied highly for the native PDA, stented PDA, and stents themselves. Conclusions: The shortening and straightening of the PDA after stenting are significant and substantial, and their quantitative characterization will enable interventionalists to select stent lengths that span the entire PDA without encroaching on the aortic or pulmonary artery, which could cause hemodynamic interference, stent kink, and fatigue. Pulsatile PDA deformations can be used to design and evaluate devices tailored to congenital heart disease in neonates.

Erratum: Stenting of the Patent Ductus Arteriosus: A Meta-analysis and Literature Review.

[This corrects the article DOI: 10.1016/j.jscai.2022.100392.][This corrects the article DOI: 10.1016/j.jscai.2023.101051.].

Selective Use of Pulmonary Vasodilators in Patients with Fontan Physiology.

Background: Fontan-associated liver disease is a well-known sequela following the Fontan procedure for patients living with single-ventricle heart disease. Pulmonary vasodilators, such as phosphodiesterase type 5 inhibitors, have emerged as a potential therapeutic option for lowering central venous pressures by reducing pulmonary vascular resistance. Method: We performed a single-center retrospective review of Fontan patients who were placed on pulmonary vasodilator therapy with prehemodynamic and posthemodynamic, MR elastography, and histologic assessments. Results: A total of 125 patients with Fontan circulation underwent surveillance with cardiac catheterization during the review period. Fifty-three (42%) patients who did not have increased end-diastolic pressures at the time of cardiac catheterization were started on phosphodiesterase type 5 inhibitor therapy. Nine patients (17%) underwent posttherapy follow-up catheterization. The mean Fontan pressure decreased from 15.4 ± 3.3 mmHg to 13.3 ± 2.5 mmHg (p=0.026), after initiation of pulmonary vasodilatory therapy. There was no change in end-diastolic pressure, transpulmonary gradient, wedge pressure, pulmonary vascular resistance, cardiac index, or saturation. Eleven patients (21%) underwent pretherapy MR elastography testing with posttherapy follow-up MR elastography. We found no improvement in liver stiffness score following the application of pulmonary vasodilators. Three patients underwent pretherapy and posttherapy liver biopsies, with variable histological changes observed within the hepatic parenchyma. Conclusions: These data demonstrate indeterminate results for the selective use of pulmonary vasodilators but highlight the need for large prospective randomized control trials of pulmonary vasodilator therapies to fully assess the benefit of such therapies in Fontan-associated liver disease.

Pulmonary Atresia With Intact Ventricular Septum: From Radiofrequency Perforation to Transcatheter Pulmonary Valve.

OBJECTIVES: We intend to describe an entirely transcatheter management pathway for patients with pulmonary atresia with intact ventricular septum (PA/IVS). BACKGROUND: PA/IVS is a rare cyanotic congenital heart lesion traditionally palliated with multiple procedures and surgeries. Entirely non-surgical transcatheter management from infancy to adulthood may be possible; however, the pathway from neonatal radiofrequency (RF) pulmonary valve perforation to later transcatheter pulmonary valve replacement (TPV) is not well described. METHODS: This retrospective study was performed at a pediatric cardiac center between 2007-2018. All patients with PA/IVS who were managed exclusively with catheterization-based interventions as neonates (RF perforation, pulmonary valvuloplasty, or ductal stenting) were analyzed. Demographic, procedural and clinical data were collected. RESULTS: Fifteen patients had exclusively catheterization-based RV decompression as neonates, 7 of whom did not require subsequent surgery. Six patients required a right ventricular outflow tract (RVOT) augmentation later in life; all were born before 2013. All 6 later had a TPV placed. Two of the patients underwent a surgical Glenn shunt alone. Of the 7 patients that never had surgery, 3 have since undergone a TPV, and 4 are awaiting candidacy for TPV placement. No patients with PA/IVS have had an RVOT augmentation at our institution since 2012. CONCLUSIONS: Patients with PA/IVS who undergo catheterization-based neonatal interventions can make it to TPV without requiring surgery. At our institution, there has been a shift in management over the last 8 years favoring entirely non-surgical transcatheter management from infancy to adulthood.

Aortopulmonary collaterals: An etiology for pediatric tracheostomy hemorrhage.

OBJECTIVE: To report a single-institution's experience of symptomatic aortopulmonary collaterals presenting as tracheostomy tube hemorrhage. STUDY DESIGN: Retrospective case series and Contemporary Review. SETTING: Tertiary care children's hospital. METHODS: Retrospective review, from 2015 to 2020, of patients <18 years old who were treated for tracheostomy hemorrhage with endovascular embolization of aortopulmonary collateral (APC) vessels. RESULTS: 4 patients were identified, 2 males and 2 females, ages 15 months-to 14 years-old, with a range of cyanotic congenital heart diseases. Direct laryngoscopy and bronchoscopy were necessary for excluding proximal airway sources. Chest computed tomography angiography did not localize pulmonary hemorrhage, but helped identify aortopulmonary collaterals. Cardiac catheterization was both diagnostic and therapeutic with use of endovascular embolization techniques by pediatric interventional cardiology and interventional radiology. Previously reported APC-related pulmonary hemorrhages occurred in significantly different settings compared to our patients, and evaluation by an otolaryngologist was key to rule out upper airway etiologies. CONCLUSION: Symptomatic aortopulmonary collaterals is an important etiology in pediatric tracheostomy tube hemorrhage in patients with cyanotic congenital heart disease.