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.

Serial Versus Direct Dilation of Small Diameter Stents Results in a More Predictable and Complete Intentional Transcatheter Stent Fracture: A PICES Bench Testing Study.

Balloon-expandable stents, implanted in infants and children with congenital heart disease (CHD), often require redilation to match somatic growth. Small diameter stents may eventually require longitudinal surgical transection to prevent iatrogenic vascular stenosis. Intentional transcatheter stent fracture (TSF) is an emerging alternative approach to stent transection, but little is known about the optimal stent substrate and best protocol to improve the likelihood of successful TSF. Bench testing was performed with a stent dilation protocol. After recording baseline characteristics, stents were serially or directly dilated using ultra-high-pressure balloons (UHPB) until fracture occurred or further stent dilation was not possible. Stent characteristics recorded were as follows: cell design, metallurgy, mechanism, and uniformity of fracture. Stents tested included bare-metal coronary stents, premounted small diameter stents, and ePTFE-covered small diameter premounted stents. Ninety-four stents representing 9 distinct models were maximally dilated, with 80 (85%) demonstrating evidence of fracture. Comprehensive fracture details were recorded in 64 stents: linear and complete in 34/64 stents (53.1%), linear and incomplete in 9/64 stents (14.1%), transverse/complex and complete in 6/64 stents (9.4%), and transverse/complex and incomplete in 15/64 stents (23.4%). Stent fracture was not accomplished in some stent models secondary to significant shortening, i.e., "napkin-ring" formation. Serial dilation resulted in evidence of fracture in 62/67 (92.5%) stents compared with 18/27 (66.7%) stents in the direct dilation group (p = 0.003). Intentional TSF is feasible in an ex vivo model. Serial dilation more reliably expanded the stent and allowed for ultimate stent fracture, whereas direct large diameter dilation of stents was more likely to generate a "napkin-ring" configuration, which may be more resistant to fracture. In vivo animal and human testing is necessary to better understand the response to attempted TSF for newly developed stents as well as those currently in use.

Drug-Coated Balloon Angioplasty: A Novel Treatment for Pulmonary Artery In-Stent Stenosis in a Patient with Williams Syndrome.

A 20-month-old boy with Williams syndrome had undergone multiple surgical and catheter-based interventions for resistant peripheral pulmonary arterial stenoses with eventual bilateral stent placement and conventional balloon angioplasty. He persistently developed suprasystemic right ventricular (RV) pressure. Angioplasty with a drug-coated balloon (DCB) was performed for in-stent restenosis and to remodel his distal pulmonary vessels bilaterally. This resulted in immediate improvement in the in-stent stenosis and resultant decrease in RV pressure. Follow-up catheterization two months later continued to show long-lasting improvement in the in-stent stenosis. We hypothesize that the anti-proliferative effects of DCBs may be of benefit in the arteriopathy associated with Williams syndrome. We report this as a novel use of a DCB in the pulmonary arterial circulation in a patient with Williams syndrome.

Efficacy and safety of catheter-based rheolytic and aspiration thrombectomy in children.

BACKGROUND: Vascular thromboses are a significant cause of morbidity and mortality in children. Data in children regarding catheter-based rheolytic and aspiration thrombectomy systems are limited. We sought to review the safety and efficacy of catheter-based rheolytic and aspiration thrombectomy systems in children. METHODS: Data of all children having undergone thrombectomy using specialized rheolytic or aspiration systems were reviewed. RESULTS: Thrombectomy was performed in 50 vessels in 21 patients, median age 1.9 months (8 days-18 yrs), median weight 4.3 (1.1-67.9) kg. Thrombectomy was performed using AngioJet in 16, Helix Clot Buster in 5, Fetch catheter in 8, Pronto catheter in 1, and a combination of other systems in 20 vessels (with AngioJet in 16). Thrombectomy was successful in 47/50 (94%) vessels in 18/21 (86%) patients with additional balloon/stent therapy or tPA administration performed in 16/18 (89%) of these patients. There were 2 (9.5%) major complications (both with AngioJet) consisting of asystole when thrombectomy was performed using activation times of >5 sec. At a median follow-up of 10 months (2 weeks-7 years), all 47 successfully treated vessels are patent, with 8/18 (44%) patients requiring reintervention with angioplasty/stent placement or repeat thrombectomy. CONCLUSIONS: Catheter-based thrombectomy systems are an important adjunctive tool in the treatment of children with thrombotic vessel occlusions. Significant hemodynamic compromise seen when using AngioJet may be minimized by using activation times of ≤5 sec. © 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.

The wedding cake solution: A percutaneous correction of a form fruste superior sinus venosus atrial septal defect.

We present the case of a patient with a superior sinus venosus defect (SSVD), with the atypical finding of an intact atrial septum and posterior atrial wall, which underwent percutaneous closure in the cardiac catheterization laboratory for repair. SSVDs are rare congenital cardiac anomalies, but when the anatomy is amenable to percutaneous strategies, a collaborative and creative approach can result in a safe and effective result.

Unusual variant of tetralogy of Fallot with pulmonary atresia: a right pulmonary artery from the ascending aorta and a coronary-to-left pulmonary artery collateral.

We present the case of a newborn with tetralogy of Fallot and pulmonary atresia, with a right pulmonary artery from the ascending aorta, and a left pulmonary artery arising from the right coronary artery via an indirect aortopulmonary collateral. The embryogenesis of this unusual combination of pulmonary blood supply has significant implications when considering normal migration of the aortopulmonary septum.

Triple outlet right ventricle: a previously unknown cardiac malformation.

We present the case of an infant with three distinct outflow tracts from the right ventricle. Three outlets from the heart have been previously named the "Tritruncal Heart". We review the two previously reported cases of tritruncal hearts and describe the anatomy, diagnosis, surgical management, and outcome of our case. Embryologic implications are also discussed.

Ischemic changes associated with a large patent arterial duct in small infants.

Infants with a large patent arterial duct (PDA) may develop signs of congestive heart failure secondary to pulmonary overcirculation. As the PVR decreases, the relative pulmonary blood flow (Qp :Qs ) increases and this may cause tachypnea, respiratory distress, failure to thrive, ventricular dysfunction and even ventilator dependency. The diastolic runoff from the systemic circulation may result in intestinal hypoperfusion resulting in necrotizing enterocolitis, and in severe cases, myocardial ischemia. Cardiac ischemia due to coronary steal is a recognized clinical entity. We present two cases of infants who developed coronary artery steal with the use of supplemental oxygen therapy during anesthesia induction for PDA occlusion. These cases highlight the importance of prudent use of supplemental oxygen therapy in infants with large PDAs.

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.

Unmasking of an isolated right subclavian artery from the pulmonary artery after device occlusion of a patent arterial duct.

Isolation of the right subclavian artery (RSCA) from the pulmonary artery is a rare anomaly of the aortic arch. We report a case of an isolated RSCA from the right pulmonary artery that was discovered at the time of device occlusion of a patent arterial duct. We review the literature and discuss management options.

Aortic valve morphology is associated with outcomes following balloon valvuloplasty for congenital aortic stenosis.

OBJECTIVES: Evaluate the incidence of various morphologic types of congenital AS, and the association between valve morphology and long-term outcomes, including repeat BAV, AVR, and death/transplant. BACKGROUND: Reports on long-term outcomes have low agreement on the influence of morphologic type. METHODS: We queried our institutional database and hospital billing records to identify all patients who underwent balloon aortic valvuloplasty (BAV) from 1992 through 2009. We excluded cases where morphology was not clear based on the description in the pre-BAV echocardiogram report and patients who underwent single ventricle palliation. The primary outcome of the study was the occurrence of any of the following events: repeat valvuloplasty, AVR, heart transplant, or death. RESULTS: There were 147 patients in our study cohort. The most common morphology was functionally bicuspid (n = 92, 63%), followed by functionally unicuspid (n = 20, 14%), dysplastic (n = 16, 11%), true bicuspid (n = 13, 9%), and true unicuspid (n = 6, 4%). The primary endpoint was less likely to occur in patients with functionally bicuspid valves (P < 0.01) and patients with true bicuspid valves (P = 0.03), whereas it was more likely to occur in patients with functionally unicuspid valves (P = 0.02) and patients with true unicuspid valves (P = 0.05). Multivariate Cox regression analysis demonstrated that valve type other than functionally bicuspid was associated with diminished freedom from repeat intervention, death or transplant (HR 3.3, CI 1.2 - 8.6, P = 0.02). CONCLUSIONS: In our cohort, patients with functionally bicuspid aortic valves, the most common type, had improved outcomes as compared with all other morphologic types.

Repeat balloon aortic valvuloplasty effectively delays surgical intervention in children with recurrent aortic stenosis.

OBJECTIVE: Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Recurrent AS following initial BAV or initial surgical valvotomy (SV) may require a second BAV (BAV2). We sought to determine the longterm outcomes of BAV2. DESIGN: We reviewed all cases of BAV2, defined as BAV following primary BAV or SV between 1988 and 2009. Cases were reviewed for pre- and post-BAV2 echocardiographic and procedural details. SETTING: Tertiary care dedicated children's hospital. PATIENTS: Between 1985 and 2009, 43 patients underwent BAV2 (23 primary SV, 20 primary BAV) at median age 1.9 years (1 month-21 years) and median weight 15 (3.3-55) kg. INTERVENTIONS: BAV2 performed following primary SV or primary BAV. MAIN OUTCOME MEASURES: We evaluated the following endpoints: ≥ moderate AI post-BAV2, aortic valve replacement (AVR), additional BAV or SV post-BAV2, death and heart transplantation. RESULTS: The gradient decreased from 61.4 ± 16.0 mm Hg to 26.0 ± 13.6 post-BAV2 (P < 0.01). Gradient prior to BAV2 was higher in primary SV patients (66 ± 13 mm Hg) than in primary BAV patients (56 ± 18 mm Hg, P = 0.04). 24 patients had no further events after BAV2, while 19 patients (44%) experienced 23 events including: AVR (n = 8), SV (n = 6), BAV3 (n = 2), death (n = 5), and transplant (n = 1). Regression demonstrated that adverse events were associated with higher post-BAV2 gradient (P < 0.01). Repeat intervention on the aortic valve and AVR were associated with higher post BAV2 gradient (P = 0.04, P = 0.01). Prior to BAV2, 7 patients (17%) had AI > mild, compared to 21 (51%) patients after BAV2. Cox regression revealed that primary BAV was associated with development of AI > mild after BAV2 (P < 0.01). CONCLUSION: BAV2 is associated with decreased valve gradient, though with an increase in AI. However, residual AS, not AI, is associated with poor outcomes following BAV2. BAV2 effectively treats recurrent AS and postpones need for surgical intervention.

A 25-year experience of endomyocardial biopsy safety in infants.

OBJECTIVES: To analyze the outcomes and risk factors associated with endomyocardial biopsy (EMB) in children less than one year of age. BACKGROUND: EMB has proven to be an integral diagnostic tool to evaluate suspected myocarditis, identify tumor histology, and provide tissue-graft surveillance after cardiac transplantation. The morbidity and mortality of EMB has been well established in the adult literature and reviewed in the general pediatric population, but there remains limited data for children in the first year of life. METHODS: We retrospectively reviewed the cardiology database at our institution to identify patients less than one year of age who underwent EMB between 1984 and 2008. Cardiac catheterization reports were reviewed for patient demographics, biopsy indication, procedural details, and complications. RESULTS: A total of 99 EMBs were performed, 49 for evaluation of suspected myocarditis, 43 for transplant rejection surveillance, 3 to identify tumor histology, and 4 for suspected endocardial fibroelastosis. Forty procedures were performed in children age < 6 months with 11 complications and 59 procedures performed in children age ≥ 6 months with four complications. In total, there were 12 EMB procedures (12.1%) with associated complications: 9 arrhythmias, 4 perforations requiring pericardiocentesis, 1 pneumothorax, and 1 death. Univariate analysis revealed a significant association between perforation and both weight <8 kg (P = 0.05) and age <6 months (P = 0.01). CONCLUSION: Endomyocardial biopsies can be performed safely in infants, although children under 6 months of age and less than 8 kg represent a high risk group and deserve special consideration due to the incidence of complications in this cohort.

Percutaneous atrial septal defect closure in infants and toddlers: predictors of success.

Device closure of atrial septal defect (ASD) is commonly performed in older children and adults. Infants and toddlers (age <4 years) are seldom referred for ASD closure due to size constraints. However, in many cases device ASD closure can be performed in this population. Between 2002 and 2012, 61 infants and toddlers were taken to the catheterization laboratory at our institution for ASD closure. Precatheterization transthoracic echocardiograms, intracatheterization transesophageal echocardiograms, and catheterization reports were reviewed. Fifty-three infants and toddlers presented for percutaneous ASD occlusion. Forty-eight (79 %) underwent successful closure, and 13 were referred for surgery without device attempt (n = 8) or after unsuccessful device occlusion (n = 4). Median age and weight at time of ASD closure were 2.99 years (range 0.3-3.8) and 11.7 kg (range 3.7-16.5). The device-to-septal length ratio was 0.81 (range 0.44-1.03). The 12 unsuccessful cases occurred in patients with larger defects (ASD diameter 17.5 ± 6.1 vs. 12.1 ± 4.2, p < 0.01). Deficient rims (absent or ≤ 4 mm) were seen in 9 of 12 (75 %) unsuccessful cases and in 19 of 41 (46 %) successful cases (p = 0.12). Multivariate analysis showed that patient size and ASD size were not independently associated with procedural success but that ASD size-to-patient weight ratio <1.2 (hazard ratio 9.5 [range1.7-17]) was associated with successful ASD closure. ASD device occlusion can be safely achieved in small children. An ASD size-to-patient weight ratio >1.2, not absolute patient weight or age, is associated with failure of the percutaneous approach. The midterm outcomes in these young patients are excellent.

Use of the Inspiris valve in the native right ventricular outflow tract is associated with early prosthetic regurgitation.

OBJECTIVE: The Inspiris Resilia prosthesis (Edwards Lifesciences) has been increasingly used in the pulmonic position with limited performance data. We sought to investigate its durability as a surgical pulmonary valve replacement (PVR). METHODS: We retrospectively reviewed patients who underwent PVR or conduit replacement with an Inspiris or non-Inspiris valve/conduit from 2018 to 2022. The primary end point was freedom from a composite of at least moderate pulmonary regurgitation, pulmonary stenosis, or valve/conduit reintervention. Secondary end points were individual components of the composite outcome. To account for baseline differences, propensity matching identified 70 patient pairs. RESULTS: A total of 227 patients (median age: 19.3 years [interquartile range, 11.8-34.4]) underwent PVR or conduit replacement (Inspiris: n = 120 [52.9%], non-Inspiris: n = 107 [47.1%]). Median follow-up was 26.6 months [interquartile range, 12.4-41.1]. Among matched patients, 2-year freedom from valve failure was lower in the Inspiris group (53.5 ± 9.3% vs 78.5 ± 5.9%, P = .03), as was freedom from at least moderate pulmonary regurgitation (54.2 ± 9.6% vs 86.4 ± 4.9%, P < .01). There was no difference in 2-year freedom from at least moderate pulmonary stenosis (P = .61) or reintervention (P = .92). Inspiris durability was poorer when implanted in the native right ventricular outflow tract compared with as a conduit, with 18-month freedom from valve failure of 59.0 ± 9.5% versus 85.9 ± 9.5% (P = .03). CONCLUSIONS: Early durability of the Inspiris valve is poor when implanted in the native right ventricular outflow tract; its unique design may be incompatible with the compliant pulmonary root. Modified implantation techniques or alternative prostheses should be considered.

Right Ventricular Strain in Patients With Ductal-Dependent Tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) dysfunction is an independent predictor of poor outcomes in patients with tetralogy of Fallot (TOF), and global longitudinal strain (GLS) is a well-validated echocardiographic technique to measure RV function. Although trends in RV GLS have been examined in patients with TOF, they have not been studied specifically in those with ductal-dependent TOF, a group in which there is not a clear consensus on the best surgical strategy. The aim of this study was to assess the midterm trajectory of RV GLS in patients with ductal-dependent TOF, drivers of this trajectory, and differences in RV GLS between repair strategies. METHODS: This was a retrospective two-center cohort study of patients with ductal-dependent TOF who underwent repair. Ductal dependence was defined as being initiated on prostaglandin therapy and/or undergoing surgical intervention on or before 30 days of life. RV GLS was measured on echocardiography preoperatively, early after complete repair, and at 1 and 2 years of age. RV GLS was trended over time and compared between surgical strategies and with control subjects. Mixed-effects linear regression models were used to evaluate the factors associated with changes in RV GLS over time. RESULTS: Forty-four patients with ductal-dependent TOF were included in the study, of whom 33 (75%) underwent primary complete repair and 11 (25%) underwent staged repair. Complete TOF repair was performed at a median of 7 days in the primary-repair group and 178 days in the staged-repair group. RV GLS improved over time from post-complete repair echocardiography through 2 years of age (-17.4% [interquartile range, -15.5% to -18.9%] vs -21.5% [interquartile range, -18.0% to -23.3%], P < .001). However, compared with age-matched control subjects, patients had worse RV GLS at all time points. There was no difference in RV GLS between the staged and primary complete repair groups at 2-year follow-up. Shorter intensive care unit length of stay after complete repair was independently associated with improvement in RV GLS over time. Strain improved by 0.07% (95% CI, 0.01 to 0.12) for each fewer day in the intensive care unit (P = .03). CONCLUSIONS: RV GLS improves over time among patients with ductal-dependent TOF, though it is consistently reduced compared with control subjects, suggesting an altered deformation pattern in patients with ductal-dependent TOF. There was no difference in RV GLS between the primary- and staged-repair groups at midterm follow-up, suggesting that repair strategy is not a risk factor for worse RV strain in the mid postoperative period. A shorter complete-repair intensive care unit length of stay is associated with an improved trajectory of RV GLS.

Ross Procedure in Neonate and Infant Populations: A Meta-Analysis Review.

OBJECTIVE: This study aims to perform a meta-analysis of early and late outcomes of the Ross/Ross-Konno procedures in neonates/infants. METHODS: A meta-analysis was performed in accordance with PRISMA guidelines. We used Ovid versions of MEDLINE/PubMed for relevant studies and included those that reported Ross/Ross-Konno operations in neonates/infants and at least one of the predetermined clinical outcomes. I2 and double arcsine methods assessed the heterogeneity between pooled estimates. We used a random-effect model to account for heterogeneity with MetaXL. We calculated point estimates of a pooled estimates along with its 95% CI. RESULTS: 587 neonate/infant patients were included with median age of 87.5 days old. The follow-up range was five days to 23 years. Early mortality reported in 25 studies with pooled estimates of 18.3% (95% CI: 13.6%-23.5%). Estimates ranged from 0% to 50% with relatively substantial heterogeneity (P = .01, I2 = 48.6%). Late mortality reported in 22 studies with pooled incidence of 9.7% (95% CI: 5.9%-14.3%). Estimates ranged from 0% to 53% with relatively substantial heterogeneity (P = .01, I2 = 46.1%). Autograft reintervention reported in 18 studies with pooled estimate of 19.2% (95% CI: 7.3%-34.5%). Estimates ranged from 0% to 81.8% with high heterogeneity (P < .001, I2 = 90.5%). Right ventricle-to-pulmonary artery conduit reintervention reported in 16 studies with pooled estimates of 32.0% (95% CI: 20.9%-44.12%). Estimates ranged from 0% to 92.3% with high heterogeneity (P < .001, I2 = 75.9%). CONCLUSIONS: The data suggest that the Ross/Ross-Konno procedure in neonates/infants still carries significant risk of early/late mortality and autograft/conduit reintervention. The high variability of results among centers confirms the need for surgical expertise and good patient selection. Prospective multicenter studies are warranted to investigate the rate of autograft reintervention and the impact on long-term survival in this specific population.