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.

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.

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.

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.

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.].

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.

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.

Infradiaphragmatic Total Anomalous Pulmonary Venous Return With Congenital Diaphragmatic Hernia: The Gut or the Heart-A Surgical Conundrum.

•Neonatal infradiaphragmatic total anomalous pulmonary venous return with diaphragmatic hernia is rare.•Stenting can be used as a consideration for step-wise repair.•Three-dimensional imaging and understanding vascular anatomy is useful prior to consideration of vertical vein stenting.

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

Background: Patent ductus arteriosus (PDA) stent placement and systemic-pulmonary surgical shunt procedure can both be performed as palliation for infants with duct-dependent pulmonary circulation. The aim of this meta-analysis and literature review was to compare outcomes and study populations between the 2 methods as well as review the technical considerations and complications of PDA stenting. Methods: A systematic search was conducted using the PubMed database and meta-analysis was performed. Risk ratio and mean difference were used to compare the reported outcomes of studies across patients receiving PDA stent and surgical shunt. Results: In total, 1094 patients from 8 comparative observational studies were included. The PDA stent group had a lower mortality rate and a shorter hospital length of stay than the systemic-pulmonary surgical shunt group, although at the expense of increased reintervention rates. There were higher proportions of patients with single-ventricle physiology and single-source pulmonary blood flow in the surgical shunt group. Conclusions: PDA stenting appears to be a noninferior or possibly superior method of palliation for duct-dependent pulmonary circulation compared with systemic-pulmonary surgical shunt, recognizing, however, that patients receiving surgical shunt more often had single-ventricle physiology or single-source pulmonary blood flow in this meta-analysis.

Priorities and Understanding of Pregnancy Among Women With Congenital Heart Disease: A Mixed-Methods Study.

Background: Many women with congenital heart disease (CHD) desire safe and successful pregnancies, but a significant proportion does not seek prepregnancy counseling. Objectives: This study aims to distinguish the personal priorities and perceptions about pregnancy in this growing population. Methods: Women aged 18 to 50 years with CHD were enrolled from 2 sites. Using a mixed-methods approach (Q-methodology), 179 participants sorted 23 statements representing a collection of views on pregnancy using priority forced ranking along a scale from "strongly agree" to "strongly disagree." Results: Majority of women were between 25 and 29 years of age, had moderate or severely complex CHD, and were married. Five unique group identities were elucidated from patient responses. Group 1 was centered around a strong desire to start a family. Women in group 2 had significant anxiety, and their psychological wellbeing interfered with their decision to start a family. Women in group 3 were concerned about premature death; if they do have kids, they want to be alive to see them grow old. Women in group 4 had strong objections to termination. Group 5 valued health care engagement. Group identities were unrelated to CHD complexity and demographic factors such as age and marital status. Six differentiating statements were identified that help distinguish which group a woman aligns with. Conclusions: Women with CHD have diverse priorities and values relating to pregnancy and heart disease. This study used a mixed-methods approach to provide a framework identifying several domains for targeted prepregnancy counseling in women with CHD.

Health Care Disparities in Congenital Cardiology: Considerations Through the Lens of an Interventional Cardiologist.

When resources in a society are dispersed unevenly, generally through allocation standards, distinct patterns emerge along lines of socially defined categories of people. Power, religion, kinship, prestige, race, ethnicity, gender, age, sexual orientation, and class all play a role in determining who has access to social goods in society. In most cases, social inequality refers to a lack of equality of outcome, but it can also refer to a lack of equality of access to opportunity. Unfortunately, health care is not immune to these social disparities and/or inequalities. These health care disparities in interventional cardiology were recently brought to the forefront by the Society for Cardiovascular Angiography and Interventions (SCAI) as a major focus of 2020-2021. In a recent publication, unique factors leading to disparities were reported to exist among the subsections of interventional cardiology. The congenital heart disease council of SCAI created a task force to further investigate the unique challenges and disparities impacting the practice of congenital heart disease and pediatric cardiology.