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Transcatheter pulmonary valve (TPV) replacement (TPVR) has become the standard therapy for postoperative pulmonary outflow tract dysfunction in patients with a prosthetic conduit/valve, but there is limited information about risk factors for death or reintervention after this procedure.This study sought to evaluate mid- and long-term outcomes after TPVR in a large multicenter cohort.International registry focused on time-related outcomes after TPVR.Investigators submitted data for 2,476 patients who underwent TPVR and were followed up for 8,475 patient-years. A total of 95 patients died after TPVR, most commonly from heart failure (n = 24). The cumulative incidence of death was 8.9% (95% CI: 6.9%-11.5%) 8 years after TPVR. On multivariable analysis, age at TPVR (HR: 1.04 per year; 95% CI: 1.03-1.06 per year; P 0.001), a prosthetic valve in other positions (HR: 2.1; 95% CI: 1.2-3.7; P = 0.014), and an existing transvenous pacemaker/implantable cardioverter-defibrillator (HR: 2.1; 95% CI: 1.3-3.4; P = 0.004) were associated with death. A total of 258 patients underwent TPV reintervention. At 8 years, the cumulative incidence of any TPV reintervention was 25.1% (95% CI: 21.8%-28.5%) and of surgical TPV reintervention was 14.4% (95% CI: 11.9%-17.2%). Risk factors for surgical reintervention included age (0.95 per year [95% CI: 0.93-0.97 per year]; P 0.001), prior endocarditis (2.5 [95% CI: 1.4-4.3]; P = 0.001), TPVR into a stented bioprosthetic valve (1.7 [95% CI: 1.2-2.5]; P = 0.007), and postimplant gradient (1.4 per 10 mm Hg [95% CI: 1.2-1.7 per 10 mm Hg]: P 0.001).These findings support the conclusion that survival and freedom from reintervention or surgery after TPVR are generally comparable to outcomes of surgical conduit/valve replacement across a wide age range.

Transcatheter closure of patent ductus arteriosus (PDA) in premature infants is a feasible, safe, and an effective alternative to surgical ligation and may be performed with an implant success rate of 97%. Major procedural complications related to transcatheter PDA closure in extremely low birth weight (ELBW) infants are relatively infrequent (

Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient

The novel coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is highly infectious, carries significant morbidity and mortality, and has rapidly resulted in strained health care system and hospital resources. In addition to patient-related care concerns in infected individuals, focus must also relate to diminishing community spread, protection of staff, case selection, and concentration of resources. The current document based on available data and consensus opinion addresses appropriate catheterization laboratory preparedness for treating these patients, including procedure-room readiness to minimize external contamination, safe donning and doffing of personal protective equipment (PPE) to eliminate risk to staff, and staffing algorithms to minimize exposure and maximize team availability. Case selection and management of both emergent and urgent procedures are discussed in detail, including procedures that may be safely deferred or performed bedside.

Patent ductus arteriosus (PDA) may be found in 0.1–0.2% of term infants, but the average incidence is at least five-fold higher in premature infants, correlating inversely with birth weight and gestational age. While not all patients with a PDA require treatment, the deleterious effects of persistent left-to-right shunting across the ductus can have important short- and long-term consequences. Medical and interventional approaches to PDA closure have evolved greatly in the past decade and add to the decision-making pathways. This article summarizes the pathophysiology of PDA and characterizes the medical, surgical and endovascular treatment approaches.

Determine outcomes of catheter intervention for aortic coarctation in infants. Aortic coarctation in infants following surgical repair and in high surgical risk native cases remains a challenging problem. Catheter intervention is an alternative to surgical intervention. Single-center, chart review of infants with biventricular anatomy who underwent coarctation stent placement or balloon angioplasty between 04/2004 and 04/2020. Outcomes of interest included change in aortic lumen diameter, peak gradient, number of re-interventions, time to re-intervention, and adverse events. Thirty-four patients were included in analysis, of those 16 underwent stent placement. Patients' mean age was 4.0 ± 3.0 months and weight of 5.3 ± 1.9 kg. Follow-up interval was 5.4 ± 5.1 years (0.2-16.1 years). Twelve (35%) patients underwent procedure due to ventricular dysfunction; the rest were for high resting gradient. Coarctation diameter increased from 2.4 ± 1.0 to 4.5 ± 1.3 (p 0.01) and gradient decreased from 32.0 ± 18.4 mmHg to 9.2 ± 8.8 mmHg (p 0.01). Thirteen (81%) of the stented patients required at least one re-intervention, at an average of 1.7 ± 3.2 years from the index procedure. Five (28%) of those undergoing balloon angioplasty required repeat intervention. There was no mortality due to the procedure and one late mortality. One patient had a serious procedural adverse event. On follow-up, 12 (35%) were on anti-hypertensive medications. Catheter intervention, including stent placement, for aortic coarctation in infants is feasible with an acceptable adverse event profile. Repeat interventions are common.

The Society for Cardiovascular Angiography and Interventions (SCAI) Think Tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community annually for high-level field-wide discussions. The 2021 Think Tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease. Each session was moderated by a senior content expert and co-moderated by a member of SCAI's Emerging Leader Mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialog from a broader base, and thereby aid SCAI, the industry community and external stakeholders in developing specific action items to move these areas forward.

Objectives To define patient and ventricular size parameters to guide Impella device (Abiomed, Inc., Danvers, MA) placement for mechanical circulatory support (MCS) in small pediatric patients (10-30 kg). Background There are few options for MCS in children, and there are no data on minimum patient size requirements for placement of the Impella 2.5 device. Methods This was a multicenter study of echocardiographic and magnetic resonance imaging (MRI) derived dimensions of the left ventricle (LV) length and ascending aorta used to define minimum size parameters that are necessary for the placement of the Impella catheter. Results Data were collected from 44 cardiac MRIs conducted in healthy pediatric patients and 39 echocardiograms performed in pediatric patients with cardiomyopathy prior to surgical ventricular assist device (VAD) placement. The Impella 2.5 catheter is 7.5 cm from the pigtail to the aortic annulus marker, thus requiring an LV apical length of 7.5 cm to allow the device to function in an unconstrained fashion. In the cohort of patients undergoing consideration for VAD placement, a minimum LV length of 7.5 cm corresponded to a height of 122 cm, weight of 23 kg, and body surface area (BSA) of 0.89 m2 . In the MRI cohort, this corresponded to a height of 121 cm, weight of 23.9 kg, and BSA of 0.89 m2 . Conclusion MCS with Impella devices is feasible in pediatric patients. This study defines anthropomorphic and anatomic measurements to guide providers in patient selection for MCS using the Impella devices.

Endocarditis has emerged as one of the most impactful adverse events after transcatheter pulmonary valve replacement (TPVR), but there is limited information about risk factors for and outcomes of this complication.The purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort.The authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes.Investigators submitted data for 2,476 patients who underwent TPVR between July 2005 and March 2020 and were followed for 8,475 patient-years. In total, 182 patients were diagnosed with endocarditis a median of 2.7 years after TPVR, for a cumulative incidence of 9.5% (95% CI: 7.9%-11.1%) at 5 years and 16.9% (95% CI: 14.2%-19.8%) at 8 years (accounting for competing risks: death, heart transplant, and explant) and an annualized incidence of 2.2 per 100 patient-years. Staphylococcus aureus and Viridans group Streptococcus species together accounted for 56% of cases. Multivariable analysis confirmed that younger age, a previous history of endocarditis, and a higher residual gradient were risk factors for endocarditis, but transcatheter pulmonary valve type was not. Overall, right ventricular outflow tract (RVOT) reintervention was less often to treat endocarditis than for other reasons, but valve explant was more often caused by endocarditis. Endocarditis was severe in 44% of patients, and 12 patients (6.6%) died, nearly all of whom were infected with Staphylococcus aureus.The incidence of endocarditis in this multicenter registry was constant over time and consistent with prior smaller studies. The findings of this study, along with ongoing efforts to understand and mitigate risk, will be critical to improve the lifetime management of patients with heart disease involving the RVOT. Although endocarditis can be a serious adverse outcome, TPVR remains an important tool in the management of RVOT dysfunction.

Background There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve. Objectives This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve. Methods Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy. Results Twenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40 mm Hg. During limited follow-up (n = 349; median: 12 months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR. Conclusions Acute outcomes after TPVR with balloon-expandable valves were generally excellent in all types of RVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions.

The novel coronavirus (COVID-19) pandemic has placed severe stress on healthcare systems around the world. There is limited information on current practices in pediatric cardiac catheterization laboratories in the United States (US).To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic.A web-based survey was distributed regarding case candidacy and catheterization laboratory preparedness. Centers were categorized based on the current degree of disease burden in that community (as of April 1, 2020). Data and consensus opinion were utilized to develop recommendations.Respondents belonged to 56 unique US centers, with 27 (48.2%) located in counties with a high number of COVID-19 cases. All centers have canceled elective procedures. There was relative uniformity (88% agreement) among centers as to which procedures were considered elective. To date, only three centers have performed a catheterization on a confirmed COVID-19 positive patient. Centers located in areas with a higher number of COVID-9 cases have been more involved in a simulation of donning and doffing personal protective equipment (PPE) than low-prevalence centers (46.7% vs 10.3%, respectively; P.001). Currently, only a small fraction of operators has been reassigned to provide clinical services outside their scope of practice.At this stage in the COVID-19 pandemic, pediatric/congenital catheterization laboratories have dramatically reduced case volumes. This document serves to define current patterns and provides guidance and recommendations on the preservation and repurposing of resources to help pediatric cardiac programs develop strategies for patient care during this unprecedented crisis.

Objectives Characterize the safety and effectiveness of the Amplatzer Piccolo Occluder for patent ductus arteriosus (PDA) closure. Background The presence of a hemodynamically significant PDA has been associated with an increased risk of morbidity and mortality in children born premature. Methods This was a single arm, prospective, multicenter, non-randomized study to evaluate the Amplatzer Piccolo Occluder to treat PDA in patients ≥700 g. From June 2017 to February 2019, 200 patients were enrolled at nine centers, with 100 patients weighing ≤2 kg. Primary effectiveness endpoint was the rate of PDA closure at 6-month follow-up. Primary safety endpoint was the rate of major complications through 6 months. Secondary endpoint was rate of significant pulmonary or aortic obstruction through 6 months' follow-up. Results The implant success rate was 95.5% (191/200) overall and 99% in patients ≤2 kg (99/100). The primary effectiveness endpoint was achieved in 99.4% of implanted patients. Four patients experienced a primary safety endpoint event (2 transfusions, 1 hemolysis, and 1 aortic obstruction). There were no branch pulmonary artery obstructions. Five patients, all ≤2 kg, were noted to have worsening of tricuspid regurgitation (TR) after the procedure. None of the TR incidences manifested clinically. The Amplatzer Piccolo Occluder received FDA approval in January 2019 and became the first device approved for PDA closure in patients ≥700 g. Conclusions This study supports the safety and effectiveness of the Amplatzer Piccolo Occluder, particularly in patients between 700 g and 2 kg where there is currently a significant unmet need in the United States. ClinicalTrials.gov identifier: NCT03055858.

OBJECTIVES This study describes procedural and 1-year outcomes of the 16 mm Melody PB1016 valve in patients with dysfunctional RVOT conduits. BACKGROUND The Melody PB1016 is a standard Melody valve produced from a 16 mm bovine jugular vein and is intended for deployment up to 20 mm. METHODS This is a prospective, non-randomized, multicenter study of the procedural and short-term outcomes of Melody PB1016 TPV replacement within dysfunctional RVOT conduits. Data from eight centers were included in the analysis. RESULTS During the study period, 39 patients underwent attempted Melody TPVR. Of the 39 patients, 30 underwent successful Melody TPVR. The majority of patients underwent placement of one or more stents prior to TPVR. There was a significant reduction in peak conduit pressure gradient following TPVR (38 mmHg vs. 11 mmHg, P

Middle aortic syndrome (MAS) is characterized by severe narrowing of the abdominal aorta, frequently involving the great abdominal vessels. Although increasingly implicated in primary hypertension, up to one-third of cases affect the mesenteric vasculature, and the resultant manifestations may be underrecognized. Early intervention with interdisciplinary medical management and invasive arterial reconstruction affords preservation of end-organ function and improvement in patient survival. This article reviews the embryology of the thoracoabdominal aorta and major abdominal arteries, as well as the pathogenesis, clinical manifestations, and management of the nonatherosclerotic abdominal coarctation otherwise known as MAS.

The authors sought to assess the intermediate-term effects of percutaneous placed valves in the branch pulmonary artery (PA) position.Most patients with large right ventricular outflow tracts (RVOTs) are excluded from available percutaneous pulmonary valve options. In some of these patients, percutaneous branch PA valve implantation may be feasible. The longer-term effects of valves in the branch PA position is unknown.Retrospective data were collected on patients with significant pulmonary regurgitation who had a percutaneous branch PA valve attempted.Percutaneous branch PA valve implantation was attempted in 34 patients (18 bilateral and 16 unilateral). One-half of the patients were in New York Heart Association (NHYA) functional class III or IV pre-implantation. There were 2 failed attempts and 6 procedural complications. At follow-up, only 1 patient had more than mild valvar regurgitation. The right ventricular end-diastolic volume index decreased from 147 (range: 103 to 478) ml/mPercutaneous branch PA valve implantation results in a reduction in right ventricular volume with clinical benefit in the intermediate term. Until percutaneous valve technology for large RVOTs is refined and more widely available, branch PA valve implantation remains an option for select patients.

We present a case of successful bicaval Melody valve implantation to treat severe mitral regurgitation in a 44-year-old man with d-transposition of the great arteries, repaired via a Mustard operation. The patient sustained injury to the anterior mitral valve leaflet during laser extraction of a ventricular pacing lead resulting in acute, severe, symptomatic mitral regurgitation. The presence of systemic venous baffles of similar caliber to right ventricular outflow tract conduits allowed for implantation of Melody valves within each baffle. The patient remains asymptomatic with excellent Melody valve function 14 months post procedure.

Objectives The objective was to review the use of Impella devices (Abiomed Inc, Danvers, MA) for temporary circulatory support in pediatric and adolescent patients (age ≤ 21 yrs). Background Options for minimally invasive circulatory support in children are limited, and published data are confined to case reports and small case series. Methods This was a retrospective, multicenter review of Impella implants in pediatric and adolescent patients from 2009-15, using standardized data collection and INTERMACS definitions. Results A total of 39 implants were performed in 38 patients from 16 centers. Median age and weight were 16 yrs (4–21 yrs) and 62 kg (15–134 kg). The primary indication for implant was cardiogenic shock in 28 patients (72%). Cardiac allograft rejection, myocarditis, or cardiomyopathy were the underlying diagnosis in 23 patients (59%); 11 patients had congenital heart disease. The median duration of support was 45 hr (1–1224 hr). Indications for explant included ventricular recovery in 16 patients, transition to another device in 12, death in 5, and transplant in 1. Survival was 85% at 7 days and 68% at 30 days. Major adverse events occurred in 8 patients: hemolysis in 3, bleeding in 2, stroke in 1 (unclear if related to Impella), sepsis in 1, and critical leg ischemia in 1. An increase in aortic regurgitation was noted in three patients, with no evidence of valve injury. Conclusion Temporary circulatory support with Impella devices is feasible in pediatric and adolescent patients, with acceptable risk profiles. More experience and follow up is needed to improve technical performance and patient selection. © 2017 Wiley Periodicals, Inc.

Background There are limited mechanical circulatory support options for patients with single ventricle (SV) anatomy. This is a multicenter, retrospective study of the Impella pump to support the systemic ventricle in a cohort of SV patients with Fontan circulation. Methods Patients with SV anatomy supported with an Impella device from 2012 to 2015 were included. Demographic information, indication for support, adverse events and short-term outcome data were collected. Results Ten patients were included. The median age and weight at implant was 26 years (4-38 years) and 64 kg (15–102 kg). Indications for support were systemic ventricular failure with cardiogenic shock (n = 8) or high-risk electrophysiology (EP) procedures (n = 2). The median duration of support was 49 hr (2.7–264 hr). Support was discontinued for ventricular recovery in five patients, transition to another device in two patients, completion of EP procedure in two patients and death in one patient. Survival to hospital discharge was 80%. Adverse events occurred in 4 patients. There were two cases of hemolysis, one case of increasing aortic valve insufficiency with implant and one asymptomatic access site thrombus. There were no bleeding or thromboembolic events. Conclusions Impella devices can provide temporary support for the systemic ventricle in SV patients as a bridge to recovery or additional device. Procedural survival and adverse event profiles are favorable. © 2017 Wiley Periodicals, Inc.

OBJECTIVE To assess the outcomes of the use of the percutaneous Impella RP device (Abiomed, Danvers, MA) in adolescents and young adults. BACKGROUND Results of the Impella RP device have been reported in adults, but a multicenter experience in adolescents and young adults has yet to be reported. METHODS Patients ≤21 years of age who underwent implantation of an Impella RP device for refractory right heart failure from June, 2016 to April, 2018 at nine U.S. Centers were included. RESULTS A total of 12 adolescents, median age of 18 (14-21) years and median weight 74.4 (49-112.4) kg underwent Impella RP implantation (INTERMACS Profile 1 in nine and Profile 2 in three patients. The central venous pressure decreased from 20 (16-35) to 12 (7-17) mmHg, (p = .001). One patient was concomitantly supported with an intra-aortic balloon pump (IABP) and the rest with a percutaneous/surgically placed left ventricular assist device. There was one adverse event related to the Impella RP device (thrombosis requiring explant). The support duration was 6.5 days (4.8 hr-18.4 days) and survival to hospital discharge was 83%. At a median follow-up of 11 months (5 days-2.5 years), 8 of 12 (67%) patients are alive. CONCLUSIONS In this multicenter experience, the Impella RP device was found to be efficacious and safe when used in adolescents and young adults. Further studies are warranted to identify suitable young/pediatric candidates for Impella RP therapy for right heart failure.

Objectives. To quantify radiation exposure during pediatric cardiac catheterizations performed by multiple operators on a new imaging platform, the Artis Q.zen (Siemens Healthcare, Forchheim, Germany), and to compare these data to contemporary benchmark values. Background. The Artis Q.zen has been shown to achieve significant radiation reduction during select types of pediatric cardiac catheterizations in small single-center studies. No large multicenter study exists quantifying patient dose exposure for a broad spectrum of procedures. Methods. Retrospective collection of Air Kerma (AK) and dose area product (DAP) for all pediatric cardiac catheterizations performed on this new imaging platform at four institutions over a two-year time period. Results. A total of 1,127 pediatric cardiac catheterizations were analyzed. Compared to dose data from earlier generation Artis Zee imaging systems, this study demonstrates 70–80% dose reduction (AK and DAP) for similar patient and procedure types. Compared to contemporary benchmark data for common interventional procedures, this study demonstrates an average percent reduction in AK and DAP from the lowest dose saving per intervention of 39% for AK and 27% for DAP for transcatheter pulmonary valve implantation up to 77% reduction in AK and 70% reduction in DAP for atrial septal defect closure. Conclusion. Use of next-generation imaging platforms for pediatric cardiac catheterizations can substantially decrease patient radiation exposure. This multicenter study defines new low-dose radiation measures achievable on a novel imaging system.

The society for cardiovascular angiography and interventions (SCAI) think tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community for high-level field-wide discussions. The 2020 think tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease (CHD). Each session was moderated by a senior content expert and co-moderated by a member of SCAI's emerging leader mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialogue from a broader base, and thereby aid SCAI and the industry community in developing specific action items to move these areas forward.

In the 20 years following the first described transcatheter pulmonary valve replacement (TPVR), there has been significant progress made in understanding the application and performance of this technology. For the Melody TPV (Medtronic, Minneapolis, MN) there is now over 10 years of follow-up data from prospective studies and multicenter registries that provide insight on acute procedural results, long-term valve performance, and risks to valve function such as stent fracture and infection. For the Sapien XT and S3 valves (Edwards Lifesciences, Irvine, CA) the duration of follow-up is shorter, but the breadth of experience is growing. Our understanding of procedural risks such a conduit injury and coronary compression has improved, as have our techniques for managing these potential complications. Studies have demonstrated that small conduits can be over-expanded and small bioprosthetic valves can be fractured to extend TPVR to a broader population of patients than originally envisioned. TPVR has been successfully applied to the patient with the native or patched right ventricular outflow tract (RVOT) and our understanding of pre-procedural imaging and screening for the native RVOT continues to improve. Future investigations should focus on the impact of TPVR on survival and long term health of the right ventricle as well as identifying and reducing risk factors that lead to infective endocarditis which continues to be a challenge in this patient population.

Total anomalous pulmonary venous connection refers to a spectrum of cardiac anomalies where the pulmonary veins fail to return to the left atrium and the pulmonary venous blood returns through a systemic vein or directly to the right atrium. There is a wide anatomical variety of venous connections and degrees of pulmonary venous obstruction that affect the presentation, surgical repair, and outcomes. In this review, we explore the preoperative physiology, echocardiographic diagnosis, and approach to postoperative complications.

Transcatheter closure of ventricular septal defects (VSDs) was first documented in 1988. The early studies of VSD closure were successful but there were high complication rates, particularly early and late-onset complete heart block. However, the development and use of new vascular occlusion devices in an off-label fashion has improved the range of patients who can be treated and reduced the complication rates. In particular, the rate of complete heart block documented in contemporary studies of VSD closure has fallen to levels at or below those documented in the surgical VSD closure literature.

Background: Percutaneous transcatheter pulmonary valve replacement (TPVR) has good clinical and hemodynamic outcomes in treating dysfunctional bioprosthetic valves (BPV) in the pulmonary position. Valve-in-valve therapy can further decrease the inner diameter (ID), potentially resulting in patient-prosthesis mismatch in patients with smaller BPVs. Methods and Results: To evaluate feasibility and outcomes of intentional BPV fracture to enlarge the pulmonary valve orifice with TPVR, 37 patients from 13 centers who underwent TPVR with intended BPV fracture were evaluated. A control cohort (n=70) who underwent valve-in-valve TPVR without attempted fracture was evaluated. BPV was successfully fractured in 28 patients and stretched in 5 while fracture was unsuccessful in 4. A Melody valve was implanted in 25 patients with fractured/stretched frame and a Sapien (XT 3) valve in 8. Among patients whose BPV was fractured/stretched, the final ID was a median of 2 mm larger (0–6.5 mm) than the valve’s true ID. The narrowest diameter after TPVR in controls was a median of 2 mm smaller ( P P =0.070). There were no fracture-related adverse events. Conclusions: Preliminary experience shows intentional fracture of BPV frame can be useful for achieving larger ID and better hemodynamics after valve-in-valve TPVR.

BACKGROUND The hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) was first described in 1993 as a bridge to heart transplant for HLHS. There are limited data on this strategy as primary heart transplantation for HLHS has become less common. METHODS This is an observational, single-center study comparing pre- and post-transplant outcomes of patients listed for transplant following hybrid palliation with those following surgical stage 1 palliation. RESULTS From 2004 to 2017, 21 patients underwent hybrid palliation as a bridge to heart transplant and 28 patients were listed for transplant following surgical stage 1 palliation or aortic arch repair and pulmonary artery band placement. Premature birth and the presence of genetic or anatomic abnormalities were more common in the hybrid group. Need for extracorporeal membrane oxygenation (ECMO) support and ventricular dysfunction was more common in the surgical group. There was a trend toward shorter waitlist times in the surgical cohort (36 days vs 70 days, P = 0.06). There was no difference in waitlist mortality (19% vs 21%, P = 0.61). Survival at 1 and 5 years post-transplant was similar for the hybrid and surgical cohorts (5-year survival, 80% vs 85%, P = 0.94, respectively). There was no difference in the number of post-transplant interventions. CONCLUSIONS Although the hybrid patients represented a higher risk cohort and demonstrated longer wait times, the waitlist and post-transplant mortality was equivalent between the two groups. For high-risk patients, the hybrid palliation as a bridge to transplant appears to be a reasonable strategy.

Background Pulmonary insufficiency is a consequence of transannular patch repair in Tetralogy of Fallot (ToF) leading to late morbidity and mortality. Transcatheter native outflow tract pulmonary valve replacement has become a reality. However, predicting a secure, atraumatic implantation of a catheter-based device remains a significant challenge due to the complex and dynamic nature of the right ventricular outflow tract (RVOT). We sought to quantify the differences in compression and volume for actual implants, and those predicted by pre-implant modeling. Methods We used custom software to interactively place virtual transcatheter pulmonary valves (TPVs) into RVOT models created from pre-implant and post Harmony valve implant CT scans of 5 ovine surgical models of TOF to quantify and visualize device volume and compression. Results Virtual device placement visually mimicked actual device placement and allowed for quantification of device volume and radius. On average, simulated proximal and distal device volumes and compression did not vary statistically throughout the cardiac cycle (P = 0.11) but assessment was limited by small sample size. In comparison to actual implants, there was no significant pairwise difference in the proximal third of the device (P > 0.80), but the simulated distal device volume was significantly underestimated relative to actual device implant volume (P = 0.06). Conclusions This study demonstrates that pre-implant modeling which assumes a rigid vessel wall may not accurately predict the degree of distal RVOT expansion following actual device placement. We suggest the potential for virtual modeling of TPVR to be a useful adjunct to procedural planning, but further development is needed.

Objectives Peripheral pulmonary artery stenosis (PPS) is a heterogeneous disease associated with numerous clinical or syndromic disorders including Williams syndrome. Previous studies focusing on patients with Williams syndrome have documented spontaneous improvement in branch pulmonary artery (PA) stenosis and right ventricular pressure. The goal of this study is to identify angiographic evidence of spontaneous occlusion or loss of segmental PAs in patients with PPS. Methods Patients undergoing serial catheterization for PPS at Boston Children's Hospital between 1999 and 2014 were selected. Patients with structural heart disease such as tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, or single ventricle were excluded. Hemodynamic data were extracted from catheterization reports, all available angiograms were reviewed, and evidence of segmental PA loss was documented based on defined angiographic criteria. Results Forty-two patients were included in the study. Each patient underwent an average of 5 catheterizations over the 15-year study period. The average RV pressure at initial catheterization was near systemic levels. Evidence of segmental PA loss was present in 40% of patients. Almost 30% of patients with vessel loss demonstrated loss at their initial catheterization, and 65% of patients experienced loss of multiple vessels over time. Patients with vessel loss tended to be older at initial catheterization and have higher average RV pressures at their most recent catheterization than those without vessel loss. Conclusions Progressive segmental PA loss in patients with systemic arteriopathy and PPS has not been well described previously. In this cohort, segmental PA loss occurred in 40% of patients, resulting in persistently elevated PA pressures relative to patients who did not develop vessel loss. These findings highlight the importance of surveillance in this population of patients with systemic arteriopathy and severe PPS.

Background— The Melody transcatheter pulmonary valve (TPV) was approved for implantation in obstructed right ventricular outflow tract conduits in 2010 after a multicenter trial demonstrating improvements in conduit obstruction, regurgitation, and right ventricular pressure. A recognized risk and contraindication to TPV implantation is the demonstration of coronary artery (CA) compression during balloon angioplasty or stent placement in the overlying conduit. This study is the first to characterize the risk of CA compression in this population. Methods and Results— From 2007 to 2012, 404 patients underwent 407 catheterizations for potential TPV implantation (median age, 18 years) at 4 centers. Three hundred forty-three patients (85%) underwent valve implantation. Twenty-one patients (5%) had evidence of CA compression with simultaneous right ventricular outflow tract angioplasty and CA angiography. Sixty-eight patients (17%) had abnormal CA anatomy. Fifteen of 21 (71%) patients with CA compression had abnormal CA anatomy. Eight patients with tetralogy of Fallot and 7 patients with transposition of the great arteries demonstrated compression. Of the 34 patients with tetralogy of Fallot and abnormal CA, 7 (21%) demonstrated CA compression. Conclusions— CA compression following TPV implantation can be catastrophic. CA compression was observed in 5% of patients during test balloon angioplasty. No patients in this study developed clinically apparent CA compression after TPV implantation. CA compression was significantly associated with the presence of abnormal CA anatomy, especially in patients with tetralogy of Fallot or transposition of the great arteries. Preimplantation coronary angiography with simultaneous test angioplasty is an important step to evaluate for the presence of CA compression during TPV implantation.

Background— Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit. Methods and Results— This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mm Hg; P P Conclusions— Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis.

BACKGROUND The hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) was first described in 1993 as a bridge to heart transplant for HLHS. There are limited data on this strategy as primary heart transplantation for HLHS has become less common. METHODS This is an observational, single-center study comparing pre- and post-transplant outcomes of patients listed for transplant following hybrid palliation with those following surgical stage 1 palliation. RESULTS From 2004 to 2017, 21 patients underwent hybrid palliation as a bridge to heart transplant and 28 patients were listed for transplant following surgical stage 1 palliation or aortic arch repair and pulmonary artery band placement. Premature birth and the presence of genetic or anatomic abnormalities were more common in the hybrid group. Need for extracorporeal membrane oxygenation (ECMO) support and ventricular dysfunction was more common in the surgical group. There was a trend toward shorter waitlist times in the surgical cohort (36 days vs 70 days, P = 0.06). There was no difference in waitlist mortality (19% vs 21%, P = 0.61). Survival at 1 and 5 years post-transplant was similar for the hybrid and surgical cohorts (5-year survival, 80% vs 85%, P = 0.94, respectively). There was no difference in the number of post-transplant interventions. CONCLUSIONS Although the hybrid patients represented a higher risk cohort and demonstrated longer wait times, the waitlist and post-transplant mortality was equivalent between the two groups. For high-risk patients, the hybrid palliation as a bridge to transplant appears to be a reasonable strategy.

Ebstein’s anomaly of the tricuspid valve (TV) refers to an embryological derangement of TV formation causing tethering of the septal and posterior leaflets of the valve to the underlying myocardium and apical displacement of the effective valve annulus, resulting in significant TV insufficiency and dilation of the right heart structures. The pathological abnormalities of the valve can vary significantly, resulting in a wide range of clinical presentations. Fetal diagnosis and neonatal presentations of the disease are typically the most severe and are associated with the highest mortality rates. Patients with less-severe disease will present later in life with symptoms of right heart failure and tachyarrhythmias. Medical and surgical management strategies are driven by the age at presentation, severity of disease, and any associated cardiac abnormalities. There are an increasing number of surgical options focused on valve repair.

Background— Treatment with endovascular stents has become increasingly common for the management of vascular stenosis in congenital heart disease. The use of stents in smaller patients has been tempered by concerns about the potential for stent expansion to accommodate somatic growth. One solution to limited stent diameter is the intentional fracture of maximally dilated stents, which can be accomplished using ultra–high-pressure (UHP) balloons. Methods and Results— This retrospective cohort study compared procedural characteristics and adverse events between a cohort of patients with branch pulmonary artery (PA) stents who underwent stent fracture using UHP balloons and control patients who underwent UHP redilation of previously placed PA stents without stent fracture between 2004 and 2014. Two control patients were selected for every case. Thirty-three PA stents were fractured in 31 patients with a median of 10 years after initial stent placement. The median balloon:waist ratio was 1.17 (1–1.71), and the median inflation pressure was 20 (8–30) atm. There were significant reductions in pressure gradient after angioplasty, with no difference in postangioplasty gradients between cases and controls. There were no major PA complications in the stent fracture group and no difference in the number of adverse events between the 2 groups. Conclusions— In this small series, PA stent fracture using UHP balloon angioplasty was feasible and did not result in major complications although predictors of successful fracture were not identified. Intentional fracture with UHP balloon angioplasty may be considered when treating stents that have become restrictive despite maximal dilation.

Peripheral pulmonary artery stenosis (PPS) is a heterogeneous disease associated with numerous clinical or syndromic disorders including Williams syndrome. Previous studies focusing on patients with Williams syndrome have documented spontaneous improvement in branch pulmonary artery (PA) stenosis and right ventricular pressure. The goal of this study is to identify angiographic evidence of spontaneous occlusion or loss of segmental PAs in patients with PPS.Patients undergoing serial catheterization for PPS at Boston Children's Hospital between 1999 and 2014 were selected. Patients with structural heart disease such as tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, or single ventricle were excluded. Hemodynamic data were extracted from catheterization reports, all available angiograms were reviewed, and evidence of segmental PA loss was documented based on defined angiographic criteria.Forty-two patients were included in the study. Each patient underwent an average of 5 catheterizations over the 15-year study period. The average RV pressure at initial catheterization was near systemic levels. Evidence of segmental PA loss was present in 40% of patients. Almost 30% of patients with vessel loss demonstrated loss at their initial catheterization, and 65% of patients experienced loss of multiple vessels over time. Patients with vessel loss tended to be older at initial catheterization and have higher average RV pressures at their most recent catheterization than those without vessel loss.Progressive segmental PA loss in patients with systemic arteriopathy and PPS has not been well described previously. In this cohort, segmental PA loss occurred in 40% of patients, resulting in persistently elevated PA pressures relative to patients who did not develop vessel loss. These findings highlight the importance of surveillance in this population of patients with systemic arteriopathy and severe PPS.