Outcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) in children is a challenging condition with poor outcomes. Post-operative stenosis can occur after repair of anomalous pulmonary venous return (APVR) or stenosis within native veins. There is limited data on the outcomes of post-operative PVS. Our objective was to review our experience and assess surgical and transcatheter outcomes. Single-center retrospective study was performed including patients < 18 years who developed restenosis after baseline pulmonary vein surgery that required additional intervention(s) from 1/2005 to 1/2020. Non-invasive imaging, catheterization and surgical data were reviewed. We identified 46 patients with post-operative PVS with 11 (23.9%) patient deaths. Median age at index procedure was 7.2 months (range 1 month-10 years), and median follow-up was 10.8 months (range 1 day-13 years). Index procedure was surgical in 36 (78.3%) and transcatheter in 10 (21.7%). Twenty-three (50%) patients developed vein atresia. Mortality was not associated with number of affected veins, vein atresia, or procedure type. Single ventricle physiology, complex congenital heart disease (CCHD), and genetic disorders were associated with mortality. Survival rate was higher in APVR patients (p = 0.03). Patients with three or more interventions had a higher survival rate compared to patients with 1-2 interventions (p = 0.02). Male gender, necrotizing enterocolitis, and diffuse hypoplasia were associated with vein atresia. In post-operative PVS, mortality is associated with CCHD, single ventricle physiology, and genetic disorders. Vein atresia is associated with male gender, necrotizing enterocolitis, and diffuse hypoplasia. Multiple repeated interventions may offer a patient survival benefit; however, larger prospective studies are necessary to elucidate this relationship further.

Left Ventricular Strain Normalizes After Balloon Aortic Valvuloplasty in Infants with Congenital Aortic Stenosis.

Severe aortic stenosis (AS) causes left ventricular (LV) afterload and subendocardial ischemia. Despite this, most infants with AS have normal LV ejection fraction (EF). Strain analysis using two-dimensional speckle tracking echocardiography (2DSTE) may identify more sensitive markers of systolic dysfunction. We sought to show changes in LV strain after balloon aortic valvuloplasty (BAV) in infants with AS. Twenty-seven infants ≤ 1 year of age with AS who underwent BAV from 2007 to 2017 were included. Echocardiograms before/after BAV were retrospectively analyzed with 2DSTE. Median age was 29 days (interquartile range 3-52) and LV EF was 64 ± 10%. Global longitudinal strain (GLS) significantly improved post-BAV (- 17 ± 5 vs. - 20 ± 4%, p = 0.001) with no difference in global circumferential strain. Peak longitudinal strain was abnormal at the inferoseptal base and mid-ventricle (- 15 ± 6 and - 17 ± 5 = 7%, respectively) and significantly improved in the basal and mid-anterolateral segments (- 17 ± 5 vs. - 21 ± 5%, p < 0.01; - 17 ± 6% vs. - 20 ± 5%, p = 0.01, respectively). Five (20%) patients underwent reintervention, and had significantly higher peak-to-peak pre and post-BAV AS gradients (86 ± 14 vs. 61 ± 20 mmHg, p = 0.02; 33 ± 17 vs. 21 ± 10 mmHg, p = 0.04, respectively). In conclusion, longitudinal strain is abnormal in infants with AS and improves to previously published normal values after BAV.

Transcatheter stenting of superior vena cava obstruction after pediatric heart transplantation: A single-center experience assessing risk factors and outcomes.

Transcatheter stent implantation for SVC obstruction following OHT has been well described, particularly in pediatric patients. This study describes a large single-center pediatric heart transplant experience that investigates the risk factors for SVC stenosis requiring stent implantation and its associated outcomes. All pediatric OHTs between January 1, 2000, and December 12, 2016, were examined for risk factors. Twelve of 349 (3.4%) OHTs required SVC stent implantation. Patients who required stents were younger (2.5 years vs 10.0 years, P = 0.0097), weighed less (8.6 kg vs 26.0 kg, P = 0.0024), and were more likely to have had CHD (83% vs 32%, P = 0.001) or previous SCPA (58% vs 18%, P = 0.002). Bicaval anastomosis was not associated with subsequent SVC stent implantation. Symptoms included SVC syndrome and chylous effusions. All 12 patients had evidence of significant SVC obstruction by both echocardiographic Doppler interrogation and transcatheter angiography. There were no acute procedural complications; however, reinterventions were common (four of 12 patients) and occurred at a mean of 5.7 ± 3.6 months poststent implantation. In conclusion, transcatheter SVC stent implantation is safe and effective after OHT. There were significant associations between SVC stent implantation and younger age, smaller weight, CHD, and history of SCPA, but not with bicaval anastomosis or donor-recipient weight ratio.

When Is It Better to Wait? Surgical Timing and Recurrence Risk for Children Undergoing Repair of Subaortic Stenosis.

Recurrence of subaortic stenosis (SAS) after surgery is common in children. The effects of patient characteristics and surgical timing on disease recurrence are largely unknown. We performed a retrospective study, assessing the relative effects of patient age and left ventricular outflow tract (LVOT) gradient on the need for reoperation for recurrent SAS. We included all children <20 years of age who underwent initial surgical resection of SAS at our center, January 2003-December 2013. Stratified logistic regression was performed, considering the effects of patient demographics, clinical characteristics, echocardiographic parameters, and operative technique, and clustering standard errors by surgeon. The multivariable model was used to simulate predicted probabilities of recurrent SAS for children at varying ages and baseline LVOT gradients. Sixty-three patients (38 males) underwent initial operation for SAS. Patients were followed for a median of 3.7 years (IQR 1.2-7.1). Twenty-one percent of patients (n = 13) underwent reoperation for SAS. Twelve were male. For every 10 mmHg increase in preoperative peak gradient in boys, the odds of reoperation for SAS doubled (OR 2.01, CI 1.5-2.72, p < 0.001), and for every additional 6 months of age, the odds of reoperation decreased by 14% (OR 0.86, CI 0.84-0.88, p < 0.001). Both younger age and higher preoperative outflow tract gradient are independently associated with risk of reoperation. Clinicians should consider the age and rate of LVOT gradient change-and not just the absolute gradient-in determining initial surgical timing.

Cardiac Catheterization in Pediatric Patients Supported by Extracorporeal Membrane Oxygenation: A 15-Year Experience.

Cardiac catheterization is commonly performed in patients being supported by extracorporeal membrane oxygenation (ECMO). We aimed to evaluate the safety, benefit, and outcomes of catheterization in pediatric patients supported by ECMO. Retrospective review of cardiac catheterizations performed in patients ≤18 years of age while on ECMO at a large tertiary care center between January 2000 and May 2015. A total of 55 catheterizations were performed on 51 patients during 53 unique ECMO courses. Indications for ECMO include ventricular dysfunction (22), cardiac arrest (20), inability to wean from cardiopulmonary bypass (7), and persistent cyanosis (4). Catheterizations included purely diagnostic studies (11), atrial septostomies (34), stenting of vessels or surgical shunts (6), adjustment of a stent (1), coil embolization (1), and endomyocardial biopsy (1). Septostomy was elective in 58.8% of cases (20) and emergent in 41.2% (14). Forty-six catheterizations had either surgical or catheter intervention during the same or subsequent study (83.6%). High severity complications occurred in three patients (5.6%), including one death due to hemothorax after pulmonary artery stent placement. There were no complications during patient transport. In total, 38 out of 53 (71.7%) ECMO courses resulted in decannulation, 29 (54.7%) patients survived to discharge from the hospital, and 25 (47.2%) were alive at follow-up. Cardiac catheterization can be safely performed on patients supported by ECMO. Cardiac catheterization is a critical tool in the early recognition, diagnosis, and direct treatment of hemodynamic/anatomic abnormalities in patients supported by ECMO.

Percutaneous coronary artery revascularization procedures in pediatric heart transplant recipients: A large single center experience.

OBJECTIVES: To describe our experience, at a large pediatric heart transplant center, with percutaneous coronary interventions (PCI) for cardiac allograft vasculopathy (CAV). BACKGROUND: CAV is a leading cause of late graft failure, mortality, and re-transplantation in pediatric heart transplant (HTx) recipients. Studies of PCI in adult patients have shown some short-term improvements, but no significant change in long-term outcomes. There are limited data on PCI for CAV in pediatric patients. We describe the largest single-center experience to date. METHODS: We performed a retrospective chart review of all pediatric HTx recipients who underwent PCI for a diagnosis of CAV from 2005 to 2014. RESULTS: Twenty-three procedures were performed in 13 patients, at a median age of 16.4 years (range 5.6-21.2) and median time from HTx to first PCI of 8.3 years (range 2.9-20.3). Three cases consisted of angioplasty alone, two cases had bare metal stents implanted, and the remaining 18 had drug-eluting stents implanted. There was acute procedural success in all but one case, and there was only one procedure-related complication (rebleeding from access site). During the follow-up interval (median 10.4 months, range 0.2-111.8), 7/13 patients had repeat PCI performed, two patients died (at 1.8 and 5.8 months post-PCI), and five were re-transplanted (range 0.2-18 months post-PCI). Freedom from death or retransplant by Kaplan-Meier analysis was 54% at 1 year. CONCLUSIONS: PCI can be performed safely and effectively in pediatric HTx recipients with CAV. Similar to the adult experience, there remains a high rate of disease progression and graft failure. © 2016 Wiley Periodicals, Inc.

Aortic Root Distortion and Aortic Insufficiency During Balloon Angioplasty of the Right Ventricular Outflow Tract Prior to Transcatheter Pulmonary Valve Replacement.

OBJECTIVE: To describe the significance of aortic root distortion (AD) and/or aortic valve insufficiency (AI) during balloon angioplasty of the right ventricular outflow tract (RVOT) performed to rule out coronary artery compression prior to transcatheter pulmonary valve (TPV) implantation. METHODS: AD/AI was assessed by retrospective review of all procedural aortographies performed to evaluate coronary anatomy prior to TPV implantation. AD/AI was also reviewed in all pre-post MPV implant echocardiograms to assess for progression. RESULTS: From 04/2007 to 3/2015, 118 pts underwent catheterization with intent for TPV implant. Mean age and weight were 24.5 ± 12 years and 64.3 ± 20 kg, respectively. Diagnoses were: TOF (53%), D-TGA/DORV (18%), s/p Ross (15%), and Truncus (9%). Types of RV-PA connections were: conduits (96), bioprosthetic valves (14), and other (7). Successful TPV implant occurred in 91 pts (77%). RVOT balloon angioplasty was performed in 43/118 pts (36%). Aortography was performed in 18/43 pts with AD/AI noted in 6/18 (33%); 2 with D-TGA (1 s/p Lecompte, 1 s/p Rastelli), 2 with TOF, 1 Truncus and 1 s/p Ross. Procedure was aborted in the 2 who developed severe AD/AI. TPV was implanted in 3/4 patients with mild AD/AI. Review of pre-post TPV implantation echocardiograms in 83/91 pts (91%) revealed no new/worsened AI in any patient. CONCLUSION: AD/AI is relatively common on aortography during simultaneous RVOT balloon angioplasty. Lack of AI progression by echocardiography post-TPV implant suggests these may be benign findings in most cases. However, AD/AI should be carefully evaluated in certain anatomic subtypes with close RVOT/aortic alignments.

Simultaneous stent implantation for pulmonary artery bifurcation stenosis in infants.

OBJECTIVES: Pulmonary artery (PA) bifurcation stenosis often requires simultaneous stent placement, which may be technically challenging. Limited data exist regarding this practice in infants. We aim to report the procedural outcomes and safety of bifurcation stent placement in infants. METHODS: We performed a single-center retrospective review of infants younger than 12 months who underwent simultaneous stent placement for PA bifurcation stenosis from January 1, 2001 through December 31, 2019. RESULTS: Seventeen infants underwent simultaneous PA bifurcation stent placement. The median age was 6.4 months (1.1-10.1 months), and weight was 5.8 kg (3-10.6 kg). Nine (52.9%) patients had had prior PA intervention. Most stents were placed in central PAs (28, 82.4%), followed by lobar branches (6, 17.6%). All patients received pre-mounted stents. The peak gradient across each branch decreased from 47.4 ± 16 to 18.7 ± 13 mm Hg (P less than .0001). The right ventricle to systemic systolic pressure ratio decreased from systemic (1.0 ± 0.3) to just over half systemic (0.58 ± 0.2) (P = .0001). The minimum vessel diameter increased from 3.6 ± 1.5 to 6.0 ± 1.9 mm (P less than .0001). There were 4 (23.5%) patients with high severity adverse events. There were no procedure-related deaths. The median follow-up period was 83.8 months (5.3 months-19.4 years). All patients had subsequent PA re-intervention at a median time of 8.1 months (2.9 months-8.8 years), and median time to re-operation was 19.1 months (2.9 months-7.5 years). CONCLUSIONS: Simultaneous PA stent placement is an effective strategy for relief of bifurcation stenosis in infants. Future transcatheter interventions are necessary to account for patient growth, but may delay the need for re-operation.

Adverse events rates and risk factors in adults undergoing cardiac catheterization at pediatric hospitals--results from the C3PO.

OBJECTIVE: Determine the frequency and risk factors for adverse events (AE) for adults undergoing cardiac catheterization at pediatric hospitals. BACKGROUND: Adult catheterization AE rates at pediatric hospitals are not well understood. The Congenital Cardiac Catheterization Project on Outcomes (C3PO) collects data on all catheterizations at eight pediatric institutions. METHODS: Adult (≥ 18 years) case characteristics and AE were reviewed and compared with those of pediatric (<18 years) cases. Cases were classified into procedure risk categories from 1 to 4 based on highest risk procedure/intervention performed. AE were categorized by level of severity. Using a multivariate model for high severity AE (HSAE), standardized AE rates (SAER) were calculated by dividing the observed rates of HSAE by the expected rates. RESULTS: 2,061 cases (15% of total) were performed on adults and 11,422 cases (85%) were performed on children. Adults less frequently underwent high-risk procedure category cases than children (19% vs. 30%). AE occurred in 10% of adult cases and 13% of pediatric cases (P < 0.001). HSAE occurred in 4% of adult and 5% of pediatric cases (P = 0.006). Procedure-type risk category (Category 2, 3, 4 OR = 4.8, 6.0, 12.9) and systemic ventricle end diastolic pressure ≥ 18 mm Hg (OR 3.1) were associated with HSAE, c statistic 0.751. There were no statistically significant differences in SAER among institutions. CONCLUSIONS: Adults undergoing catheterization at pediatric hospitals encountered AE less frequently than children did. The congenital heart disease adjustment for risk method for adults with congenital heart disease is a new tool for assessing procedural risk in adult patients.

Congenital Pulmonic Valve Dysfunction Treated With SAPIEN 3 Transcatheter Heart Valve (from the COMPASSION S3 Trial).

Significant pulmonary regurgitation (PR) and pulmonary stenosis are common after surgical repair of some congenital heart defects. This prospective, single-arm, multicenter trial enrolled patients who underwent transcatheter heart valve (THV) implantation with a SAPIEN 3 valve to treat dysfunctional right ventricular outflow tract (RVOT) conduits or pulmonic surgical valves (≥ moderate PR and/or mean RVOT gradient ≥35 mm Hg). The primary end point was a nonhierarchical composite of THV dysfunction at 1 year comprising RVOT reintervention, ≥ moderate total PR, and mean RVOT gradient >40 mm Hg. A performance goal of <25% of upper confidence interval (CI) was prespecified for the primary end point, using a 95% exact binomial CI. Patients (n = 58) were enrolled between July 5, 2016 and July 17, 2018, with mean age of 32 years. Prestenting was performed in 53.4%. At discharge, the device success was 98.1% (single valve without explant, < moderate PR, gradient <35 mm Hg). At 30 days, there were no major adjudicated adverse clinical events. At 1 year, the primary end point composite was 4.3% (95% CI 0.5 to 14.5). The composite components were 0% (0 of 56) RVOT reintervention, 2.1% (1 of 47) ≥ moderate PR, and 2.1% (1 of 48) mean RVOT gradient >40 mm Hg. No mortality, endocarditis, thrombosis, or stent fracture were reported at 1 year. In conclusion, the SAPIEN 3 THV was safe and effective in patients with dysfunctional RVOT conduits or previously implanted valves in the pulmonic position to 1 year. Clinical trial registration: NCT02744677; https://clinicaltrials.gov/ct2/show/NCT02744677.

Outcomes after neonatal cardiac surgery: The impact of a dedicated neonatal cardiac program.

OBJECTIVES: Prematurity is a risk factor for in-hospital mortality after cardiac surgery. The structure of intensive care unit models designed to deliver optimal care to neonates including those born preterm with critical congenital heart disease is unknown. The objective of this study was to evaluate in-hospital outcomes after cardiac surgery across gestational ages in an institution with a dedicated neonatal cardiac program. METHODS: This study is a single-center, retrospective review of infants who underwent cardiac surgical interventions from our dedicated neonatal cardiac intensive care program between 2006 and 2017. We evaluated in-hospital mortality and morbidity rates across all gestational ages. RESULTS: A total of 1238 subjects met inclusion criteria over a 11-year period. Overall in-hospital mortality after cardiac surgery was 6.1%. The mortality rate in very preterm infants (n = 68; <34 weeks' gestation at birth) was 17.6% (odds ratio, 3.52 [1.4-8.53]), versus 4.3% in full-term (n = 563; 39-40 weeks) referent/control infants. Very preterm infants with isolated congenital heart disease (without evidence of other affected organ systems) experienced a mortality rate of 10.5% after cardiac surgery. Neither the late preterm (34-36 6/7 weeks) nor the early term (37-38 6/7) groups had significantly increased odds of mortality compared with full-term infants. Seventy-eight percent of very preterm infants incurred a preoperative or postoperative complication (odds ratio, 4.78 [2.61-8.97]) compared with 35% of full-term infants. CONCLUSIONS: In this study of a single center with a dedicated neonatal cardiac program, we report some of the lowest mortality and morbidity rates after cardiac surgery in preterm infants in the recent era. The potential survival advantage of this model is most striking for very preterm infants born with isolated congenital heart disease.

Reintervention and Survival After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: 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. OBJECTIVES: This study sought to evaluate mid- and long-term outcomes after TPVR in a large multicenter cohort. METHODS: International registry focused on time-related outcomes after TPVR. RESULTS: 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). CONCLUSIONS: 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.

Multicenter Study of Endocarditis After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: 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. OBJECTIVES: The purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort. METHODS: The authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes. RESULTS: 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. CONCLUSIONS: 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.

Indications and results for hybrid interventions in patients with congenital heart disease.

A hybrid therapy or procedure is a new treatment modality that develops by combining therapies from different subspecialties. In congenital heart disease, a growing number of such procedures have been described in recent decades, as a result of increasing collaboration between surgeons and interventionalists. The ideas behind these therapies include enabling the performance of procedures of different complexity in a less invasive manner, shortening procedural times, avoiding cardiopulmonary bypass, facilitating vascular access and decreasing the number of complications associated with more invasive approaches. Over the years, hybrid therapy has gained a place as a widely accepted therapeutic option for the management of several conditions in high-risk patients with congenital heart disease.

Safety and Outcomes of Transcatheter Closure of Patent Ductus Arteriosus in Children With Pulmonary Artery Hypertension.

To investigate whether transcatheter device closure of patent ductus arteriosus (PDA) is safe in children with pulmonary artery hypertension, we retrospectively analyzed our experience with 33 patients who underwent the procedure from January 2000 through August 2015. Pulmonary artery hypertension was defined as a pulmonary vascular resistance index (PVRI) >3 WU · m2. All 33 children (median age, 14.5 mo; median weight, 8.1 kg) underwent successful closure device implantation and were followed up for a median of 17.2 months (interquartile range [IQR], 1.0-63.4 mo). During catheterization, the median PVRI was 4.1 WU · m2 (IQR, 3.6-5.3 WU · m2), and the median mean pulmonary artery pressure was 38.0 mmHg (IQR, 25.5-46.0 mmHg). Premature birth was associated with pulmonary vasodilator therapy at time of PDA closure ( P=0.001) but not with baseline PVRI (P=0.986). Three patients (9.1%) had device-related complications (one immediate embolization and 2 malpositions). Two of these complications involved embolization coils. Baseline pulmonary vasodilator therapy before closure was significantly associated with intensive care unit admission after closure (10/12 [83.3%] with baseline therapy vs 3/21 [14.3%] without; P <0.001). Of 11 patients receiving pulmonary vasodilators before closure and having a device in place long-term, 8 (72.7%) were weaned after closure (median, 24.0 mo [IQR, 11.0-25.0 mo]). We conclude that transcatheter PDA closure can be performed safely in many children with pulmonary artery hypertension and improve symptoms, particularly in patients born prematurely. Risk factors for adverse outcomes are multifactorial, including coil use and disease severity. Multicenter studies in larger patient populations are warranted.

Non-Elective Pediatric Cardiac Catheterization During COVID-19 Pandemic: A New York Center Experience.

BACKGROUND: COVID-19 has led to major changes in hospital systems across the world. In an effort to reduce viral transmission, conserve resources, and in accordance with institutional and state mandates, all elective procedures and surgeries were postponed during the initial outbreak. Guidelines for case selection are limited and management for pediatric catheterization laboratories during this crisis is unprecedented. OBJECTIVES: To report the protocols and case selection of a high-volume pediatric cardiac catheterization laboratory in the epicenter of the novel coronavirus (COVID-19) pandemic. METHODS: All pediatric cardiac catheterization procedures from March 16, 2020 through May 10, 2020 were reviewed. Changes to case selection and periprocedural workflow are described. Data were collected on COVID-19 testing status and primary procedure type, and all procedures were classified by urgency. RESULTS: There were 52 catheterizations performed on 50 patients. Endomyocardial biopsies were the most common procedure (n = 27; 52%). Interventional and diagnostic procedures represented 27% (n = 14) and 21% (n = 11) of cases, respectively. Two emergent procedures (3.8%) were performed on patients with positive COVID-19 testing. Most cases were performed on patients with negative COVID-19 testing (n = 33; 94%). CONCLUSIONS: Adjusting to the COVID-19 pandemic in a high-volume pediatric cardiac catheterization laboratory can be safely and effectively managed by prioritizing emergent and urgent cases and modifying workflow operations. The experience of this center may assist other pediatric cardiac catheterization laboratories in adapting to similar practice changes as the pandemic continues to evolve.

Resource Allocation and Decision Making for Pediatric and Congenital Cardiac Catheterization During the Novel Coronavirus SARS-CoV-2 (COVID-19) Pandemic: A U.S. Multi-Institutional Perspective.

BACKGROUND: 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). OBJECTIVES: To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis.

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.

Mid-Term Valve-Related Outcomes After Transcatheter Tricuspid Valve-in-Valve or Valve-in-Ring Replacement.

BACKGROUND: Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis. OBJECTIVES: The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes. METHODS: Patients who underwent TTVR after prior surgical TV replacement or repair were collected through an international registry. Time-related outcomes were modeled and risk factors assessed. RESULTS: Data were collected for 306 patients who underwent TTVR from 2008 through 2017 at 80 centers; 52 patients (17%) had a prior history of endocarditis. Patients were followed for a median of 15.9 months after implantation (0.1 to 90 months), with 64% of patients estimated to be alive without TV reintervention or a valve-related event at 3 years. The cumulative 3-year incidence of death, reintervention, and valve-related adverse outcomes (endocarditis, thrombosis, or significant dysfunction) were 17%, 12%, and 8%, respectively. Endocarditis was diagnosed in 8 patients 2 to 29 months after TTVR, for an annualized incidence rate of 1.5% per patient-year (95% confidence interval: 0.45% to 2.5%). An additional 8 patients were diagnosed with clinically relevant valve thrombosis, 3 in the short term, 2 within 2 months, and 3 beyond 6 months. Only 2 of these 8 patients received anticoagulant therapy before thrombus detection (p = 0.13 vs. patients without thrombus). Prior endocarditis was not a risk factor for reintervention, endocarditis, or valve thrombosis, and there was no difference in valve-related outcomes according to TTVR valve type. CONCLUSIONS: TV dysfunction, endocarditis, and leaflet thrombosis were uncommon after TTVR. Patients with prior endocarditis were not at higher risk for endocarditis or other adverse outcomes after TTVR, and endocarditis occurred with similar frequency in different valve types. Though rare, leaflet thrombosis is an important adverse outcome, and further study is necessary to determine the appropriate level of prophylactic therapy after TTVR.

Percutaneous balloon atrial septostomy on top of venoarterial extracorporeal membrane oxygenation results in safe and effective left heart decompression.

BACKGROUND: Transcatheter techniques are emerging for left atrial (LA) decompression under venoarterial extracorporeal membrane oxygenation (VA-ECMO). We aimed to assess whether balloon atrioseptostomy (BAS) is a safe and efficient strategy. METHODS: All patients who underwent percutaneous static BAS under VA-ECMO at four tertiary institutions were retrospectively reviewed. RESULTS: From 2000 to 2014, BAS was performed in 64 patients (32 adults and 32 children). Indications for ECMO support included acute myocarditis (31.3%) and non-myocarditis cardiac disease, mostly end-stage dilated cardiomyopathy (32.8%). BAS was required because of pulmonary oedema/haemorrhage and left ventricular (LV) distension. The mean balloon diameter was 21.8 ± 8.4mm. Adequate LA decompression was achieved in all patients. Mean LA pressure fell from 24.2 ± 6.9 mmHg to 7.8 ± 2.6 mmHg ( p < 0.001). The left-to-right atrial pressure gradient fell from 17.2 ± 7.1 mmHg to 0.09 ± 0.5 mmHg ( p < 0.001). Echocardiography showed an unrestrictive left-to-right atrial shunting in all patients. Improvement of day 1 chest X-ray was observed in 76.6% of patients, clinical status in 98.4% of patients and pulmonary haemorrhage in 14 out of 14 patients. Complications occurred in 9.4% of patients, representing pericardial effusion, fast atrial fibrillation, ventricular fibrillation requiring defibrillation, transient complete heart block and femoral venous dissection requiring covered stent placement. In the 37 (57.8%) patients who were successfully decannulated, the median ECMO duration was 9 (range: 4-24) days. After a median follow-up of 12.3 (range: 0.1-142) months, 35.9% patients died, 17.2% received a LV assist device as a bridge to transplantation, 31.2% were transplanted and 56.2% were home discharged and alive. CONCLUSIONS: Percutaneous BAS may be a safe and efficient strategy for discharging the LA in both adults and children supported by VA-ECMO.