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Background: Guideline-directed heart failure therapy with angiotensin receptor blocker/neprilysin inhibitor (ARNi) and sodium-glucose transporter inhibitors (SGLT2i) has been incrementally beneficial in improving outcomes in heart failure patients., Objective: Evaluate the feasibility and efficacy of guideline-directed medical therapy (GDMT) in adults congenital heart disease (ACHD) patients., Methods: In a retrospective cohort study, ACHD patients with either New York Heart Association (NYHA) Class II symptoms or systemic ejection fraction (EF) <45%, optimized on a combination of beta-blocker (BB), ARNi, mineralocorticoid receptor antagonist (MRA) and SGLT2i were evaluated., Results: Forty-six patients with a mean age 42.6 ± 12.1 years prescribed GDMT were identified. Twenty-eight (61%) were male, 20 (43%) had a systemic right ventricle (RV) and 9 (20%) had single-ventricle physiology. Over the optimization period, 20 (43%) were sustained on ARNi and 42 (91%) on SGLT2i in addition to treatment with BB and MRA. Over a period of 45 weeks, echocardiography parameters for left ventricle (LV) ejection fraction (EF) (+7.5%, p = 0.006), systemic ventricle (SV) velocity time integral (VTI) (+1.9 cm, p = 0.012) and LV global longitudinal strain (GLS) (-2.5%, p = 0.005) improved when 3-4 medications were used versus 1-2 medications alone. The use of either ARNi or SGLT2i (+8.1%, p = 0.017) or in combination (+7.0%, p = 0.043) increased LVEF compared to the use of neither medication., Conclusion: Combination GDMT is beneficial in improving myocardial characteristics in ACHD patients with systemic RV and LV., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Sports participation in patients with congenital heart disease is an evolving subject. The American Heart Association/American College of Cardiology released a set of guidelines that advise the type and level of sports participation based primarily on anatomical defects with secondary consideration given to hemodynamic effects. Recently, the European Association of Preventive Cardiology/European Society of Cardiology/Association for European Paediatric and Congenital Cardiology offered a contrasting approach to sports participation that is based on hemodynamic and electrophysiological profiles of each patient, regardless of anatomical consideration. These guidelines are drastically different in their approaches but do have some similarities. In this review, we compare both documents, focusing on the aim, population, classification of sports, and the methodology of making recommendations. This review aims to assist practicing cardiologists in integrating the available published data and recommendations when counseling patients for sports participation., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

The current surgical approach for patients with a single ventricle is the culmination of decades of dedicated research and groundbreaking innovation. From 1971 to the present day, a series of incremental advancements have significantly extended the life expectancy of these patients. Since the very beginning, Dr Guillermo Kreutzer and his team have pioneered different techniques with the ultimate goal of improving outcomes for these individuals. This is, narrated by him, the story of how it all began., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation. Pulmonary complications are common and may contribute to both circulatory and pulmonary insufficiency, leading ultimately to Fontan failure. These complications include restrictive lung disease, sleep abnormalities, plastic bronchitis, and cyanosis. Cyanosis post-Fontan procedure can be attributed to multiple causes including systemic to pulmonary venous collateral channels and pulmonary arteriovenous malformations. This review presents the unique cardiopulmonary interactions in the Fontan circulation. Understanding the cardiopulmonary interactions along with improved recognition and treatment of pulmonary abnormalities may improve the long-term outcomes in this growing patient population. Interventions focused on improving pulmonary function including inspiratory muscle training and endurance training have shown a promising effect post-Fontan procedure., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: The C3PO-QI (Congenital Cardiac Catheterization Project on Outcomes - Quality Improvement), a multicenter registry launched in 2015, instituted quality improvement (QI) initiatives to reduce patient radiation exposure. Through regular collaboration, this initiative would allow for harmony among active participants, maximizing efforts and efficiency at achieving radiation best practices. This study sought to report these efforts with a detailed methodology for which institutions can target initiatives, reducing radiation exposure, and increasing patient safety., Methods: Data were collected prospectively by 8 C3PO-QI institutions between January 1, 2015 and December 31, 2017. Radiation exposure was measured in dose area product per body weight (dose area product/kg; µGy*m 2 /kg) and reported by expected radiation exposure categories (REC) and institution for 40 published unique procedure types. Targeted interventions addressing selected strategic domains for radiation reduction were implemented in the pediatric catheterization labs of the C3PO-QI institutions., Results: The study consisted of 15 257 unique cases. Median exposure (dose area product/kg) was decreased by 30% for all procedures. Dose area product/kg was reduced in all 3 REC, with the greatest improvement observed in REC I (REC I, -37%; REC II, -23%; REC III, -27%). Although the baseline radiation exposures and exact percent decrease varied across all C3PO-QI sites, each institution demonstrated improvements in radiation dose over time. These improvements occurred with the implementation of institution-specific QI interventions accelerated by participation in the C3PO-QI multicenter collaborative., Conclusions: Substantial radiation dose reductions can be achieved using targeted QI methodology and interventions. Participation in a multicenter QI collaborative may accelerate improvement across all centers due to enhanced engagement and shared learning between sites.

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included. Using expert consensus, 40 unique procedure types were defined by diagnostic characteristics or the intervention(s) performed, and dose area product (DAP) per kilogram of body weight (µGy × m 2 /kg) was summarized. Using empiric and consensus methods, three radiation risk categories were created. A total of 11,735 cases were included for analysis. Thirteen (n = 7918) procedure types with median DAP/kg < 100 were categorized in the low radiation exposure category (median DAP/kg 39). The medium exposure category (n = 1807) consisted of 16 procedure types with median DAP/kg values ranging 100 to < 200 (overall median DAP/kg 131). Finally, the high radiation exposure category (n = 1073) consisted of 11 procedure types with median DAP/kg ≥ 200 (overall median DAP/kg of 231). The radiation exposure risk categories created in this multi-center dataset are a critical step towards the development of a robust risk adjustment methodology for radiation exposure in catheterization for congenital heart disease.

Objectives: This study sought to evaluate the incidence of and risk factors for conduit and stent-related outcomes following transcatheter pulmonary valve replacement (TPVR)., Background: Stent fracture (SF) and right ventricular outflow tract (RVOT) reintervention are among the most important adverse outcomes after TPVR using the Melody valve (Medtronic, Minneapolis, Minnesota). The conduit environment and conduit preparation practices vary among patients who undergo TPVR., Methods: Data from 3 prospective Melody valve multicenter studies were pooled and analyzed. All patients who had successful implant of a Melody valve that was present at hospital discharge comprised the study cohort; patients who had TPVR into a stentless conduit comprised the analysis cohort. SF was diagnosed using protocol-specified or clinical fluoroscopy or radiography, and classified as major or minor., Results: Of 358 patients who underwent catheterization with intent to perform TPVR, 309 were discharged with the Melody valve in place (study cohort) of which 251 patients had TPVR into a stentless conduit (analysis cohort). Median follow-up was 5 years. New pre-stents were placed in 68% of patients with a stentless conduit, and 22% received multiple pre-stents. At 3 years, freedom from any SF and major SF was 74 ± 3% and 85 ± 2%, respectively, and freedom from RVOT reintervention was 85 ± 2%. New pre-stents were associated with longer freedom from SF and RVOT reintervention than was no pre-stent., Conclusions: Risks of SF and reintervention after TPVR with a Melody valve were reduced by implantation of pre-stents, which has become standard practice. This study supports pre-stenting as an important component of TPVR therapy., (Copyright © 2017. Published by Elsevier Inc.)

Objective: Retrospectively assess the frequency of right ventricular outflow tract (RVOT) conduit disruption during transcatheter pulmonary valve replacement (TPVR) and the effectiveness and safety of NuMED Covered Cheatham-Platinum Stents™ (CCPS) for its prevention or treatment., Background: There have been no published reports to date describing the safety and effectiveness of covered stents to prevent or treat RVOT conduit disruption during TPVR., Methods: Data regarding 251 TPVR procedures performed at multiple US investigational sites were retrospectively reviewed to explore the incidence and potential predictors of conduit disruption. In addition, data on the use of 69 CCPS implanted in 50 patients during TPVR was reviewed., Results: The overall incidence of conduit disruption requiring intervention was 6%. The only predictor identified was a very elevated RVOT conduit systolic pressure gradient. A pre-existing conduit tear was present in nine, while 31 developed tears after conduit dilation and three, after TPVR. The CCPS was used prophylactically in seven. Conduit tears were prevented or repaired in 49/50 patients. No CCPS-related acute complications were reported. At 6-month follow-up, no patient had more than mild pulmonary regurgitation and the mean Doppler RVOT gradient (12.7 ± 5.8 mm Hg) comparing favorably with that reported in the Melody TPV® IDE trial (20.0 ± 8.6 mm Hg)., Conclusion: CCPS implantation can successfully treat RVOT conduit disruption without negative impact on the TPV function. This retrospective analysis suggests high RVOT conduit systolic pressure gradient is a risk factor for conduit tears during TPVR., (© 2014 Wiley Periodicals, Inc.)

Objectives: This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial., Background: TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years. The Melody TPV received U.S. Food and Drug Administration approval in 2010 as a Humanitarian Use Device., Methods: Patients with dysfunctional RVOT conduits were entered in this prospective, nonrandomized study at 10 centers. The primary endpoint was acceptable hemodynamic function at 6 months post-implantation, defined as a composite of RVOT echocardiographic mean gradient ≤30 mm Hg, pulmonary regurgitation less than moderate as measured by echocardiography, and freedom from conduit reintervention and reoperation., Results: Cardiac catheterization was performed in 120 patients for potential implantation of the Melody TPV; of these, 100 patients were implanted, with a 98.0% procedural success rate. There were no procedure-related deaths. Acceptable hemodynamic function at 6 months was achieved in 96.7% of patients with evaluable data (87.9% of the entire implanted cohort), with results maintained through 1 year. No patient had moderate or severe pulmonary regurgitation after implantation. No patient required catheter reintervention in the first year after implantation, and 2 patients required reoperation for conduit replacement. The rate of freedom from TPV dysfunction was 96.9% at 1 year., Conclusions: This first prospective, real-world experience with the Melody TPV in the United States demonstrates continued high procedural success, excellent short-term TPV function, and low reintervention and reoperation rates at 1 year. (Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692)., (Copyright © 2014. Published by Elsevier Inc.)

Pediatric and Congenital Interventional Cardiology is the practice of catheter-based techniques that improve cardiac physiology and circulation through the treatment of heart disease in children and adults with congenital or acquired heart defects. Over the last decade, and since last published training guidelines for pediatric cardiac catheterization and interventional cardiology were published in 2005 [1] the field of Pediatric and Congenital Cardiac Catheterization has evolved into a predominantly interventional discipline. As there is no sub-specialty certification for interventional cardiac catheterization in pediatrics, the Congenital Heart Disease Committee of the Society of Cardiovascular Angiography and Interventions has put together this consensus statement for advanced training in pediatric and congenital interventional cardiac catheterization. The statement puts forth recommendations for program infrastructure in terms of teaching, personnel, equipment, facilities, conferences, patient volume and trainee assessment. This is meant to set a standard for training programs as well as giving applicants a basis on which to judge and compare programs., (© 2014 Wiley Periodicals, Inc.)

Background: Data examining the effect of operator years in practice and volume on adverse events (AE) after cardiac catheterization in patients with congenital heart disease is limited., Methods and Results: Data were prospectively collected using a multi-center registry (C3PO). 10,885 catheterizations performed between 02/07 and 06/10 at eight institutions were included. AE rates were risk-adjusted for hemodynamic vulnerability, procedure type risk group, and age and compared between operators with different years in practice (YIP) and volume. AE occurred in 13% of procedures. Operators with less than five YIP had higher adjusted odds of any AE (OR 1.42, 95% CI 1.14-1.77) or a high severity AE (OR 1.35, 95% CI 1.04-1.75), when compared with operators with 5 to less than 25 YIP (5 < 25), while operators with ≥25 YIP had higher odds of a high severity (but not any) AE (OR 1.39, 95% CI 1.08-1.80). Operators with <5 YIP had a higher percentage of preventable AE (out of all AE, 16% vs. 8%, P < 0.001) as well as higher odds of vascular or cardiac trauma (OR 1.81, 95% CI 1.11-2.97), or technical AE (OR 1.98, 95% CI 1.31-2.99) when compared with operators with 5 < 25 YIP. There was no consistent relationship between operator volume, and incidence of AE., Conclusions: Operators with less than 5 years in practice have higher risk-adjusted AE rates. While an important consideration in guiding and mentoring operators with fewer years in practice, it is important to emphasize that reporting adverse events does not take into account procedural efficacy., (Copyright © 2013 Wiley Periodicals, Inc.)

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates., Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007., Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios., Results: Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41., Conclusions: Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary artery (PA) balloon angioplasty and/or stenting (PA rehabilitation) is one of the most common procedures performed in the cardiac catheterization laboratory, but comprehensive and consistently reported data on procedure-related adverse events (AE) are scarce., Methods and Results: Data were prospectively collected using a multicenter registry (Congenital Cardiac Catheterization Project on Outcomes). All cases that included balloon angioplasty and/or stent implantation in a proximal or lobar PA position were included. Multivariate analysis was used to evaluate for independent predictors of AE and need for early reintervention. Between February 2007 and December 2009, 8 institutions submitted details on 1315 procedures with a PA intervention. An AE was documented in 22% with a high severity (level 3 to 5) AE in 10% of cases. Types of AE included vascular/cardiac trauma (19%), technical AE (15%), arrhythmias (15%), hemodynamic AE (14%), bleeding via endotracheal tube/reperfusion injury (12%), and other AE (24%). AE were classified as not preventable in 50%, possibly preventable in 41%, and preventable in 9%. By multivariate analysis, independent risk factors for level 3 to 5 AE were presence of ≥2 indicators of hemodynamic vulnerability, age below 1 month, use of cutting balloons, and operator experience of <10 years. Reintervention during the study period occurred in 22% of patients undergoing PA rehabilitation., Conclusions: PA rehabilitation is associated with a 10% incidence of high-level severity AE. Hemodynamic vulnerability, young age, use of cutting balloons, and lower operator experience were significant independent risk factors for procedure-related AE.

Background: The Congenital Cardiac Catheterization Project on Outcomes (C3PO) was established to develop outcome assessment methods for pediatric catheterization., Methods and Results: Six sites have been recording demographic, procedural and immediate outcome data on all cases, using a web-based system since February 2007. A sample of data was independently audited for validity and data completeness. In 2006, participants categorized 84 procedure types into 6 categories by anticipated risk of an adverse event (AE). Consensus and empirical methods were used to determine final procedure risk categories, based on the outcomes: any AE (level 1 to 5); AE level 3, 4, or 5; and death or life-threatening event (level 4 or 5). The final models were then evaluated for validity in a prospectively collected data set between May 2008 and December 31, 2009. Between February 2007 and April 2008, 3756 cases were recorded, 558 (14.9%) with any AE; 226 (6.0%) level 3, 4, or 5; and 73 (1.9%) level 4 or 5. General estimating equations models using 6 consensus-based risk categories were moderately predictive of AE occurrence (c-statistics: 0.644, 0.664, and 0.707). The participant panel made adjustments based on the collected empirical data supported by clinical judgment. These decisions yielded 4 procedure risk categories; the final models had improved discrimination, with c-statistics of 0.699, 0.725, and 0.765. Similar discrimination was observed in the performance data set (n=7043), with c-statistics of 0.672, 0.708, and 0.721., Conclusions: Procedure-type risk categories are associated with different complication rates in our data set and could be an important variable in risk adjustment models for pediatric catheterization., (© 2011 American Heart Association, Inc.)

Background: Prenatal cardiac interventions (PCI) for human fetal aortic valve (AoV) stenosis can reduce left ventricular hypoplasia and restore ventricular growth and function. However, 'freehand' needle delivery from the maternal skin through the uterine wall, fetal chest and ventricular apex to cross the fetal AoV remains technically challenging and time intensive, and is the rate-limiting step in the procedure., Methods: We developed a computer-assisted navigation (CANav) system that tracks the position and orientation of a two-dimensional (2D) ultrasound image relative to the trajectory of an electromagnetic (EM) embedded needle and stylet. We tested the CANav system in vitro using a water bath phantom, then in vivo using adult rats and pregnant (fetal) sheep., Results: The CANav system accurately tracked the delivered needle position in both in vitro phantom and adult rat model experiments. We performed 22 PCI attempts with or without CANav in a fetal sheep model. Maternal laparotomy was required to adjust the fetal position in 50% of the procedures. The time required to deliver the needle from the skin into the left ventricle (LV) using CANav was 2.9 +/- 1.7 (range 2-7) min (n = 14) vs. 5.5 +/- 4.3 (range 1-12) min (n = 8) without CANav (p < 0.05). The time needed to cross the aortic valve once the needle was within the LV was similar with and without CANav (p = 0.19)., Conclusions: CANav reduces the PCI time required to accurately deliver a needle to the fetal heart. Adaptations of this technical approach may be relevant to other congenital cardiac conditions and ultrasound-guided medical procedures., (2007 John Wiley & Sons, Ltd.)

This article considers the role transcatheter therapy plays in the treatment of each major type of congenital heart lesion. The focus is on the clinical ramifications of the interventions rather than their technical aspects.

Slotted stainless Palmaz stents (PS) remain the most commonly used in congenital heart disease (CHD). Limitations of PS include rigidity, foreshortening, poor conformability on expansion, balloon rupture, and jailing of side branches. Recently, stents with open-cell design (IntraStent; IS) in appropriate sizes for CHD have been introduced. We reviewed our experience with the IS, comparing performance with the PS in a retrospective nonrandomized, uncontrolled observational study. Between May 1999 and March 2001, 34 IS (10-36 mm) and 34 PS (10-40 mm) were implanted in 57 patients (3 months to 25 years old; median, 3.24 years) in congenital or postoperative lesions. There was no statistically significant difference between the two groups in efficacy, % diameter increase (162% +/- 203% for IS vs. 153% +/- 177% for PS), and % gradient reduction (69% +/- 23% for IS vs. 80% +/- 27% for PS). Other aspects of stent performance differed significantly (P < 0.05): PS forshortened more (mean 18% for PS vs. 0% for IS) and were associated with balloon rupture (9/34 for PS vs. 0/34 for IS; P = 0.002), but had no evidence of intimal protrusion in between struts (0/34 for PS vs. 14/34 for IS; P < 0.001) and no recoil > 15% (0/34 for PS vs. 7/34 for IS; P = 0.006); IS conformed almost twice as well to vessel curvature (P = 0.003). Although these factors did not affect immediate procedural success, balloon rupture in two PS resulted in stent malpositions. Among IS, the origins of three covered side branches were accessed and balloon dilated up to 6 mm through the side of the stent. In conclusion, stents with open-cell design have some characteristics that seem beneficial for their use in CHD: they foreshorten less, are conformable, are less likely to cause balloon rupture, and can allow access to jailed branches. However, they have a higher incidence of significant percent recoil and protrusion of intimal tissue in between struts, which may impact long-term stent performance., (Copyright 2002 Wiley-Liss, Inc.)

The article discusses on the thrombosis' prevention and treatment in pediatric and congenital heart disease. It explores the hemostasis in children with information on initiation of coagulation, amplification, and regulation of thrombin and fibrin. It highlights the agents for its treatment and prevention, such as heparin-induced thrombocytepenia and measures of anticoagulant effect. It also focuses on the special considerations with warfarin therapy in children.