Echocardiographic Predictors of Readiness for Double Switch Operation and Postoperative Ejection Fraction in Patients With Congenitally Corrected Transposition of the Great Arteries Undergoing Left Ventricular Retraining.

BACKGROUND: In patients with congenitally corrected transposition of the great arteries (ccTGA), assessment of readiness for the double switch operation (DSO) after pulmonary arterial band (PAB) placement involves cardiac magnetic resonance imaging (cMRI) to measure left ventricular ejection fraction (LVEF) and mass and cardiac catheterization (catheterization) to assess the ratio of left ventricular to right ventricular pressure (LV:RVp). The aims of this study were to describe the relationships between echocardiographic and catheterization and cMRI measures of readiness for DSO and to develop risk factors for left ventricular (LV) dysfunction after DSO on the basis of echocardiographic measures of ventricular-arterial coupling (VAC). METHODS: Patients with ccTGA undergoing LV retraining at a DSO referral center were reviewed. LVEF measured by echocardiography was compared with that measured by cMRI, and LV:RVp measured by echocardiography was compared with that measured by catheterization using Bland-Altman analysis. The relationship between preoperative VAC markers and postoperative echocardiography was analyzed using ventricular end-systolic elastance (EES) and a novel marker consisting of the product of LVEF and LV:RVp (EFPR). RESULTS: Thirty-one patients with 56 evaluations for DSO were included, 24 of whom underwent DSO. Echocardiographic LVEF correlated well with cMRI LVEF (r = 0.79), and Bland-Altman analysis slightly overestimated cMRI LVEF (mean difference, +3%). Echocardiographic LVEF had a moderate ability to identify normal cMRI LVEF (area under the curve, 0.80) and at an optimal cut point of echocardiographic LVEF threshold of 61%, there was 71% sensitivity and 76% specificity to detect cMRI LVEF ≥ 55%. Echocardiographic LV:RVp correlated well with LV/RVp by catheterization (r = 0.77) and slightly underestimated the catheterization value (mean difference, -0.11). Echocardiographic LV:RVp had a good ability to identify adequate LV:RVp by catheterization (area under the curve, 0.95) and at an optimal echocardiography cut point of 0.75 had 100% sensitivity and 85% specificity to detect a catheterization LV:RVp >0.9. Echocardiography-based criteria for DSO readiness (echocardiographic LVEF of 61% and LV:RVp of 0.75) demonstrated specificity of 97% and positive predictive value of 96% for published criteria of DSO readiness (cMRI LVEF of 55% and catheterization LV:RVp of 0.9). EES and EFPR correlated with post-DSO LVEF (ρ = 0.72 and ρ = 0.60, respectively). EFPR of 0.51 demonstrated 78% sensitivity and 100% specificity for post-DSO LV dysfunction (LVEF < 55%). Age at first PAB also strongly correlated with post-DSO LVEF (ρ = 0.75). No patient with first PAB at <1 year of age exhibited post-DSO LV dysfunction. CONCLUSIONS: Echocardiographic measures of LVEF and LV:RVp are reliable indicators of reference standard modalities and can guide management during retraining. The preoperative VAC markers EES and EFPR may be useful markers of post-DSO LV dysfunction. Values of echocardiographic LV:RVp >0.75 are likely to meet pressure-generation criteria for DSO and should be considered for referral to catheterization and cMRI evaluation for DSO. PAB placement before 1 year of life may optimize LV outcomes in patients considered for DSO.

Is there a role for endovascular stent implantation in the management of postoperative right ventricular outflow tract obstruction in the era of transcatheter valve implantation?

BACKGROUND: The optimal management pathway for the dysfunctional right ventricular outflow tract (RVOT) is uncertain. We evaluated the long-term outcomes and clinical impact of stent implantation for obstructed RVOTs in an era of rapidly progressing transcatheter pulmonary valve technology. METHODS: Retrospective review of 151 children with a biventricular repair who underwent stenting of obstructed RVOT between 1991 and 2017. RESULTS: RVOT stenting resulted in significant changes in peak right ventricle (RV)-to-pulmonary artery (PA) gradient (39.4 ± 17.1-14.9 ± 8.3; p < 0.001) and RV-to-aortic pressure ratio (0.78 ± 0.22-0.49 ± 0.13; p < 0.001). Subsequent percutaneous reinterventions in 51 children to palliate recurrent stenosis were similarly effective. Ninety-nine (66%) children reached the primary outcome of subsequent pulmonary valve replacement (PVR). Freedom from PVR from the time of stent implantation was 91%, 51%, and 23% at 1, 5, and 10 years, respectively. Small balloon diameters for stent deployment were associated with shorter freedom from PVR. When additional children without stent palliation (with RV-to-PA conduits) were added to the stent cohort (total 506 children), the multistate analysis showed the longest freedom from PVR in those with stent palliation and subsequent catheter reintervention. Pulmonary regurgitation was well-tolerated clinically. Indexed RV dimensions and function estimated by echocardiography remained stable at last follow up or before primary outcome. CONCLUSION: Prolongation of conduit longevity with stent implant remains an important strategy to allow for somatic growth to optimize the risk-benefit profile for subsequent surgical or transcatheter pulmonary valve replacement performed at an older age.

Biventricular Repair in Borderline Left Hearts: Insights From Cardiac Magnetic Resonance Imaging.

Background: Cardiac magnetic resonance imaging (CMR) may augment 2-dimensional (2D) echocardiography in decision-making for biventricular repair in borderline hypoplastic left hearts. Objectives: This study evaluates: 1) the relationship between 2D echocardiography and CMR; 2) imaging variables affecting assignment to biventricular vs non-biventricular management; and 3) variables affecting transplant-free biventricular survival. Methods: We reviewed clinical, echocardiographic, and CMR data in 67 infants, including CMR-determined ascending aortic (AAo) flow and comparable left ventricular end-diastolic volume indexed (LVEDVi) by 2D-echocardiography and CMR. Results: Treatment assignment to biventricular repair was either direct (BV, n = 45) or with a bridging hybrid procedure (H1-BV, n = 12). Echocardiographic LVEDVi was <20 mL/m2 in 83% of biventricular repair infants and underestimated CMR-LVEDVi by 16.8 mL/m2. AAo flows had no/weak correlation with aortic and mitral valve z-scores or LVEDVi. AAo flows differed between BV, H1-BV, and single-ventricle groups (median): 2.1, 1.7, and 0.7 L/min/m2, respectively. Important variables for treatment assignment were presence of endocardial fibroelastosis, AAo flow, and mitral valve z-score. Biventricular repair was achieved in 54. The median follow-up was 8.0 (0.1-16.4) years. Transplant-free biventricular survival was 96%, 82%, and 77% at 1, 5, and 10 years, respectively. Patients without aortic coarctation repair were at higher risk of death, transplantation, or single-ventricle conversion (HR: 54.3; 95% CI: 6.3-47.1; P < 0.001) during follow-up. AAo flow had a smaller nonlinear effect with hazard ratio increasing at lower flows. Conclusions: Historical 2D echocardiographic criteria would have precluded many patients from successful biventricular repair. AAo flow, an integrative index of left heart performance, was important in assigning patients to a biventricular circulation and affected survival. Biventricular survival was strongly associated with the need for aortic coarctation repair.

Current-Era Outcomes of Balloon Aortic Valvotomy in Neonates and Infants.

Background: The optimal initial treatment pathway for aortic valve stenosis remains debated. Objectives: The objective of this study was to review current outcomes of balloon aortic valvotomy (BAV) in neonates and infants. Methods: Neonates and infants with a biventricular circulation treated with BAV between 2004 and 2019 were reviewed. Results: One hundred thirty-nine infants (48% neonates) with median (Q1, Q3) age of 33(7, 84) days and weight 4.0 (3.4, 5.1) kg were followed up for 7.1 (3.3, 11.0) years. BAV reduced peak-to-peak gradient from mean (SD) 52 (16) mmHg to 18 (12) mmHg; P < 0.001. Aortic regurgitation (AI) increased with time after BAV. Three children died during follow-up. Fifty-one reinterventions (26 BAV, 19 aortic valve replacements [AVRs], and 6 surgical valvotomies) were performed on 40 children. Freedom from AVR (95% CI) was 96% (93%-99%) at 1, 91% (86%-96%) at 5, and 86% (79%-93%) at 10 years. The predictors of AVR were a unicommissural valve (hazard ratio [HR] [95% CI]: 3.7 [1.4-9.6]; P = 0.007) and moderate to severe AI after index BAV (HR [95% CI]: 3.3 [1.1-9.7]; P = 0.029). Freedom from reintervention was 84% (78%-90%) at 1, 76% (69%-83%) at 5, and 69% (60-78%) at 10 years. Main predictors of reintervention were age below 1 month (HR [95% CI]: 2.1 [1.1-4.1]; P = 0.032) and postdilation peak-to-peak gradient (per 10-mmHg increase; HR [95% CI]: 1.36 [1.02-1.79]; P = 0.032). Conclusions: BAV is a safe and effective treatment for aortic valve stenosis in neonates and infants. Outcomes are competitive with contemporary published data on aortic valve repair in relation to mortality, gradient relief, long-term AVR, and reintervention rates. In the absence of significant AI, surgery can be reserved for those with gradients resistant to valve dilation.

Balloon Angioplasty for Native Aortic Coarctation in 3- to 12-Month-Old Infants.

BACKGROUND: Balloon angioplasty for native coarctation of the aorta (CoA) is successful in children and adults but in neonates results in frequent restenosis. The efficacy of balloon angioplasty for native CoA during infancy beyond the neonatal period was examined in infants aged 3 to 12 months of age. METHODS: A retrospective review of 68 infants who underwent balloon angioplasty for native CoA. 95% CI are in parentheses. RESULTS: Procedural age was (mean±SD) 6±3.4 months and weight was 7±1.8 kg. Balloon angioplasty produced a large decrease in both the noninvasive arm-to-leg blood pressure gradient (41.2±18.7 to 5.6±9.6 mm Hg) and the invasive peak systolic pressure gradient (34±12 to 11±9 mm Hg). Balloon angioplasty increased the CoA diameter from 2.7±1 mm to 4.6±1.2 mm. One patient was lost to follow-up. A catheter reintervention was required in 11.8% and surgery in 10.3%. The hazard of reintervention was highest early. Median freedom from reintervention was 89% (95% CI, 80%-96%) at 1 year, 83% (95% CI, 73%-92%) at 5 years, and 81% (95% CI, 69%-90%) at 10 years. Femoral artery thrombosis was documented in 6 (9%) infants without any long-term consequence. One patient developed a small aortic aneurysm late and has not required treatment. A robust estimate of the frequency of aortic aneurysms remains to be determined as the majority of subjects have not had cross-sectional imaging. CONCLUSIONS: Balloon angioplasty of native CoA is effective and safe in infants aged 3 to 12 months with outcomes comparable to those in older children and adults. Catheter reinterventions can avoid the need for surgery in most patients.

Automated 3-Dimensional Single-Beat Real-Time Volume Colour Flow Doppler Echocardiography in Children: A Validation Study of Right and Left Heart Flows.

BACKGROUND: Novel quantification of stroke volume according to mitral inflow and aortic outflow using automated real-time 3-dimensional volume colour flow Doppler echocardiography (3D-RT-VCFDE) is more accurate than 2-dimensional echocardiography and has excellent correlation with cardiac magnetic resonance imaging-based flows in adults. This technology is applied for the first time to the right heart and in children. METHODS: 3D-RT-VCFDE was performed in 61 image sets of flow through the aortic (AV), mitral (MV), pulmonary (PV), and tricuspid (TV) valves of 34 children. These were compared with stroke volumes of the right (RV) and left (LV) ventricles and ratio of pulmonary to systemic blood flow determined using the Fick method in 31 children with atrial shunts. RESULTS: The mean age was 8.0 ± 3.3 years, and the mean weight was 27.8 ± 10.0 kg. The mean temporal resolution for flow analyses was ≥ 22 volumes per second. In conditions with no shunt, the correlations were: AV with MV flows (r = 0.98), PV with TV flows (r = 0.96), RV stroke volume with PV flow (r = 0.95), and with TV flow (r = 0.93), LV stroke volume with AV flow (r = 0.87), and with MV flow (r = 0.89). Fick ratio of pulmonary to systemic blood flow correlations were: PV/AV ratio (r = 0.84), TV/MV ratio (r = 0.87), and RV/LV ratio (r = 0.70). CONCLUSIONS: Stroke volume determined using automated 3D-RT-VCFDE is feasible in children and in the right side of the heart. This technique potentially provides a noninvasive alternative to historically invasively acquired hemodynamic data and to cardiac magnetic resonance imaging.

Outcomes of Blalock-Taussig shunts in current era: A single center experience.

OBJECTIVES: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity. DESIGN: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression. RESULTS: Median age was 8 (0-126) days, weight 3.1(1.7-5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent had a sternotomy approach for shunt placement with 70% receiving a 3.5 mm graft. Mean graft index (shunt cross sectional area [mm2 ]/BSA [m2 ]) was 44.39 ± 8.04 and shunt size (mm) to body weight (kg) ratio 1.1 ± 0.2. Hospital mortality was 12%, with an interval mortality of 6%. Shunt thrombosis/stenosis occurred in 23% and pulmonary over circulation in 30%, while shunt reoperation was required in 12% and catheter intervention in 8% of the cohort. At 1-year, survival was 82.0% (95% CI [72.7%, 88.4%]), and survival free of major morbidity 61.4% (95% CI [50.7%, 70.5%]). Duct dependency predisposed to mortality (P = .01, HR 6.74 [1.54, 29.53]) and composite outcome (mortality and major morbidity) (P = .04, HR 2.15, CI [1.036, 4.466]) and higher graft index to mortality (P = .005, HR 1.07 [1.02, 1.12]). CONCLUSIONS: The commonest indication for a MBTS in the current era was single ventricle palliation. Morbidity and mortality was considerable, partly explained by the higher at risk population. Alternative methods to maintain pulmonary blood flow in place of a MBTS requires further investigation.

Clinical Impact of Stent Implantation for Coarctation of the Aorta with Associated Hypoplasia of the Transverse Aortic Arch.

The aim of this study was to explore the clinical impact of transverse aortic arch hypoplasia (TAH) after stent implementation for isthmal coarctation of the aorta (CoA). From a retrospective chart review, 51 children (median age 11.1 years) were identified who had TAH and a CoA stent implanted between 10/1995 and 4/2015. Arm-leg cuff blood pressure measurements, echocardiographic arch imaging, and 24-h ambulatory blood pressure monitoring, prior to and after stent implantation, were reviewed. At catheterization, peak systolic gradients across the CoA's were 25 mmHg before and 4 mmHg after stent implantation. At a median 37-month follow-up, echocardiographic imaging showed no significant catch-up growth in the transverse arch (median z-score; proximal and distal arch -1.54 and -1.99 vs. -1.78 and -1.63, p = 0.13 and 0.90). A trend to increasing systolic blood pressure (SBP) differentials between the right and left arms was noted (11 mmHg [prior to]; 16 mmHg [follow-up], p = 0.09). Age-adjusted percentiles for right arm SBP decreased from 99.7% prior to, and 87.6% in follow-up (p < 0.001). The median time to re-intervention was 5.6 years (95% CI [2.8, 7.8]) and the proportion of children using anti-hypertensive in follow-up was not significantly different before the implantation (38% [prior to]; 45% [follow-up]). Elevated right arm blood pressure persists after successful stent implantation in the setting of associated TAH and there appears to be no catch-up growth of the transverse arch with time. Medical management can be difficult and approaches to surgical arch augmentation or stent implantation should be considered to avoid unilateral arm hypertension.

Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

BACKGROUND: Cardiac catheterization is often required for patients on extracorporeal membranous oxygenation (ECMO) support, though its efficacy remains unclear. This study aimed to assess the impact of catheterization on successful ECMO weaning. METHODS: This is a single-center retrospective study from 2000 to 2014. Patients with congenital heart disease who underwent cardiac catheterization while on cardiac ECMO support were included. Logistic regression analysis and Kaplan-Meier survival analysis with log-rank test were performed to determine predictors for successful weaning and patient outcome. RESULTS: Forty-nine catheterizations in 47 patients with the median age of 65 days (range: 1 day-12 years) and the median body weight of 4.2 kg (range: 1.9-32.7 kg) were included. Median duration between ECMO cannulation and catheterization was 1 day (range: 0-11 days). Thirty-three patients (70%) succeeded in decannulation and 24 patients (51%) survived to hospital discharge. Absence of renal (P = 0.045) and respiratory complications (P = 0.031) were significant prognostic factors for successful weaning in multivariate analysis. Patients who received catheterization within 48 hr after ECMO cannulation demonstrated less respiratory complications (P = 0.006) and better survival at 30 days after ECMO initiation (P = 0.039) than those who underwent later catheterization. There was no mortality; however, nine major catheterization-related complications (18%) were detected. CONCLUSION: Catheterization for pediatric patients on ECMO support can be performed, although this group contained high risk of serious adverse events. The absence of ECMO complications is a predictor for successful weaning. An earlier catheterization appears to be associated with better short-term survival in this cohort. © 2016 Wiley Periodicals, Inc.

Right Ventricular Outflow Tract Stenting in Tetralogy of Fallot Infants With Risk Factors for Early Primary Repair.

BACKGROUND: Tetralogy of Fallot with cyanosis requiring surgical repair in early infancy reflects poor anatomy and is associated with more clinical instability and longer hospitalization than those who can be electively repaired later. We bridged symptomatic infants with risk factors for early primary repair by right ventricular outflow tract stenting (stent). METHODS AND RESULTS: Four groups of tetralogy of Fallot with confluent central pulmonary arteries were studied: stent group (n=42), primary repair (aged <3 months) with pulmonary stenosis (early-PS group; n=44), primary repair (aged <3 months) with pulmonary atresia (early-PA group; n=49), and primary repair between 3 and 11 months of age (surg>3mo group; n=45). Stent patients had the smallest pulmonary arteries with a median (95% credible intervals) Nakata index (mm2/m2) of 79 (66-85) compared with the early-PA 139 (129-154), early-PS 136 (121-153), and surg>3mo 167 (153-200) groups. Only stent infants required unifocalization of aortopulmonary collaterals (17%). Stent and early-PA infants had younger age and lower weight than early-PS infants. Stent infants had the most multiple comorbidities. Stenting allowed deferral of complete surgical repair to an age (6 months), weight (6.3 [5.8-7.0] kg), and Nakata index (147 [132-165]) similar to the low-risk surg>3mo group. The 3 early treatment groups had similar intensive care unit/hospital stays and high reintervention rates in the first 12 months after repair, compared with the surg>3mo group. CONCLUSIONS: Right ventricular outflow tract stenting of symptomatic tetralogy of Fallot with poor anatomy (small pulmonary arteries) and adverse factors (multiple comorbidities, low weight) relieves cyanosis and defers surgical repair. This allowed pulmonary arterial and somatic growth with clinical results comparable to early surgical repair in more favorable patients.

Maladaptive aortic properties after the Norwood procedure: An angiographic analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial.

OBJECTIVES: Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk factors for abnormalities, and impact on right ventricular function in patients with single right ventricle lesions after arch reconstruction. METHODS: Prestage II aortic angiograms from the Pediatric Heart Network Single Ventricle Reconstruction trial were analyzed to define arch geometry (Romanesque [normal], crenel [elongated], or gothic [angular]), indexed neoaortic dimensions, and distensibility. Comparisons were made with 50 single-ventricle controls without prior arch reconstruction. Factors associated with ascending neoaortic dilation, reduced distensibility, and decreased ventricular function on the 14-month echocardiogram were evaluated using univariate and multivariable logistic regression. RESULTS: Interpretable angiograms were available for 326 of 389 subjects (84%). Compared with controls, study subjects more often demonstrated abnormal arch geometry (67% vs 22%, P < .01) and had increased ascending neoaortic dilation (Z score 3.8 ± 2.2 vs 2.6 ± 2.0, P < .01) and reduced distensibility index (2.2 ± 1.9 vs 8.0 ± 3.8, P < .01). Adjusted odds of neoaortic dilation were increased in subjects with gothic arch geometry (odds ratio [OR], 3.2 vs crenel geometry, P < .01) and a right ventricle-pulmonary artery shunt (OR, 3.4 vs Blalock-Taussig shunt, P < .01) but were decreased in subjects with aortic atresia (OR, 0.7 vs stenosis, P < .01) and those with recoarctation (OR, 0.3 vs no recoarctation, P = .04). No demographic, anatomic, or surgical factors predicted reduced distensibility. Neither dilation nor distensibility predicted reduced right ventricular function. CONCLUSIONS: After Norwood surgery, the reconstructed neoaorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these features.

Achievable radiation reduction during pediatric cardiac catheterization: How low can we go?

OBJECTIVES: To assess the effectiveness of radiation-reduction measures implemented during pediatric catheterization, and provide data on the radiation doses for common interventional and diagnostic procedures, indexed to body weight. BACKGROUND: Ionizing radiation exposure must be minimized to "as low as reasonably achievable," by instituting radiation-limiting techniques and knowledge of expected radiation exposure. METHODS: Radiation-reduction measures included pulsed-fluoroscopy at 7.5 pulses/second (0.032-0.045 µGy/pulse), an air-gap magnification technique for children<20 kg, operator awareness, and additional exposure reduction techniques through projection optimization. Radiation doses for procedures performed between 2007 through 2014 were retrospectively reviewed, including dose area product (DAP) and DAP/kg of body weight for 25 procedural types. Median doses were compared with those previously published from other large centers and multi-institutional databases and assessed for changes over time. RESULTS: Reviewed were 5,196 cases, which included 2,819 interventional, 710 endomyocardial biopsies and 1,667 diagnostic studies, documenting a significant difference in exposure between various procedures and body weights. The absolute exposure was significantly greater in larger children (e.g., for ductal closure median DAP/kg: 17 µGy*m2/kg 10-20 kg children vs. 37 µGy*m2 /kg for those>30 kg, P<0.001). Dose exposure using radiation-reduction techniques were the lowest reported in the literature for all procedure types compared (e.g. median DAP for pulmonary valvuloplasty 163 µGy*m2 vs. 405 to 1,230 µGy*m2 reported by 3 large centers). Reduction of fluoroscopy acquisition to 7.5 pulses/second nearly halved radiation exposure (P<0.001). CONCLUSIONS: Implementing a radiation dose reduction and awareness program can lead to documented reduction in exposure, across a variety of procedures performed by multiple operators.

Pharmacokinetics of sirolimus-eluting stents implanted in the neonatal arterial duct.

BACKGROUND: Sirolimus-eluting stents may have clinical advantages over bare-metal stents in the extremely proliferative environment of the neonatal arterial duct. However, sirolimus has immunosuppressive actions and little is known regarding sirolimus pharmacokinetics in the newborn. METHODS AND RESULTS: This is a retrospective review of sirolimus pharmacokinetics in neonates who underwent sirolimus-eluting stent implantation in the arterial duct for pulmonary blood flow augmentation. Pharmacokinetic parameters were obtained by noncompartmental analysis and by a Bayesian one-compartment nonlinear mixed model. Nine neonates received a single sirolimus-eluting stent with a total sirolimus dose of 245 µg (n = 1), 194 µg (n = 5), or 143 µg (n = 3). Peak sirolimus concentrations were 13.6 ± 4.5 µg/L (24.8 µg/L highest) and clearance was 0.042 ± 0.03 L/hour (noncompartmental analysis) and 0.051 L/hour (95% credible intervals 0.037-0.069, nonlinear mixed model). Sirolimus remained > 5 µg/L, the trough level used in oral immunosuppressive therapy, for (95% credible interval) 15.9 (11.4, 22.8), 12.9 (7.6, 19.0), and 8.4 (2.3, 14.5) days for the 245, 194, and 143 µg sirolimus dose stents, respectively. Estimates of the duration of systemic immunosuppression are provided for combinations of 2 stents. CONCLUSIONS: In neonates after sirolimus-eluting stent implantation, peak sirolimus levels were 20 × higher and clearance 30 × lower than previously reported in older children and adults. Sirolimus levels were within the immunosuppressive range for a prolonged period, but with no observable clinically significant adverse outcomes.

Three-dimensional rotational angiography in the assessment of vascular and airway compression in children after a cavopulmonary anastomosis.

The aim of the study was to examine the role of three-dimensional rotational angiography (3DRA) in assessing vascular and airway narrowing in children with a bidirectional cavopulmonary anastomosis (BCPA). The course of children with single ventricle physiology is often complicated by left pulmonary artery (LPA) and/or bronchial stenosis and may be related to aortic compression. 3DRA may be useful in evaluating this complex anatomy and possible mechanisms for the observed obstruction. Clinical data and imaging (2D angiography and 3DRA) of children with a BCPA were reviewed retrospectively. Measurements were taken at similar locations along the pulmonary arteries in both modalities and in the airways on 3DRA. Twenty-five children with a previous BCPA were assessed at mean age of 3.1 ± 2.0 years and weight of 13.6 ± 3.6 kg. Excellent correlation was found between 3DRA and 2D angiographic LPA measurements (r = 0.89, p < 0.0001). Twelve children had qualitative LPA stenosis on 3DRA, with a stenotic dimension of 6.6 ± 2.2 mm on 2D angiography and 6.8 ± 1.9 mm on 3DRA (r = 0.94, p < 0.0001). Ten cases with LPA stenosis also had bronchial stenosis (83 %). Qualitative airway assessment correlated with quantitative bronchial dimensions from 3DRA-derived tomographic images: Bronchial stenosis measured 4.4 ± 1.6 versus 5.9 ± 1.1 mm in those with a normal appearing bronchus (p = 0.009). Hybrid patients (initial palliation with bilateral pulmonary artery banding and arterial ductal stenting, n = 5) and all patients with a Damus-Kaye-Stansel (DKS) anastomosis (n = 9) were more likely to have LPA and left bronchial stenosis (OR 7.7, p = 0.04). 3DRA is a useful and accurate tool in assessment of LPA and airway narrowing after BCPA. Hybrid and DKS patients are more prone to LPA and bronchial stenosis, and 3DRA can provide insight into the mechanism.

Percutaneous pulmonary valve implantation: 5 years of follow-up: does age influence outcomes?

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a safe, less invasive alternative to surgical valve replacement for the congenital heart disease patient with right ventricular (RV) outflow tract dysfunction. The aim of this study was to determine whether reverse RV remodeling after PPVI was persistent in the longer term and whether timing of PPVI influenced outcomes. METHODS AND RESULTS: Consecutive patients from the pediatric and adult congenital heart disease programs were enrolled. Cardiac MRI, echocardiography, metabolic exercise testing, chest radiography, and hemodynamics before intervention were compared with repeated follow-up measurements to assess changes over time. Fifty-one patients (including 23 patients <16 years old) were followed for a mean 4.5±1.9 (0.9-6.9) years after implantation, 59% of patients having available comparative cardiac MRI data. Freedom from any reintervention was 87% and 68% at 3 and 5 years, and freedom from surgery was 90% at 5 years. For every decade younger at implantation, there was an increase of 3.9%±1.0% in cardiac MRI left ventricular ejection fraction (P<0.001) and 2.4±0.9 mL/kg/min in maxVO2 (P=0.005) and a decrease of 0.7±0.2 cm in RV end-diastolic dimension (P<0.001) after intervention. Younger patients displayed an additional decline in the RV/left ventricular end-diastolic volume ratio (P=0.05) and trended toward improved RV ejection fraction in late follow-up (50%±7% versus 41%±12%, P=0.07). CONCLUSIONS: This is the largest series to show that PPVI at a younger age yields incremental improvements in RV size and maximum oxygen consumption. Early valve implantation is associated with better RV function and should be considered in management planning for this population.

Pulmonary artery stenosis in hybrid single-ventricle palliation: High incidence of left pulmonary artery intervention.

OBJECTIVE: Pulmonary artery growth is an important determinant of outcome in single-ventricle strategies. Higher rates of pulmonary artery intervention have been reported with hybrid-based palliation when compared with Norwood palliation. METHODS: We performed a retrospective review of pulmonary artery growth and clinical outcomes in patients undergoing hybrid-based single-ventricle palliation. RESULTS: The stage I hybrid procedure was performed in 72 patients between 2004 and 2012, of whom 54 were on a Fontan palliative pathway. Thirty-four infants completed stage II, and 20 infants underwent the Fontan operation. The mean diameters of the right pulmonary artery (5.6 ± 1.9 mm) and left pulmonary artery (5.6 ± 2.1 mm) were similar before stage II. After stage II, the right and left pulmonary artery diameters were 8.5 ± 2.1 mm and 5.8 ± 1.3 mm, respectively (P < .001), and after the Fontan operation, these were 8.8 ± 2.0 mm and 6.4 ± 1.1 mm, respectively (P = .002). The mean right pulmonary artery z score was normal throughout, but the left pulmonary artery did not maintain a normal size. The cumulative pulmonary artery intervention rate was 50% at any time after stage II. Fifteen interventions (88%) were performed after stage II (35% during the same hospitalization, 71% <60 days). The most intervened site was the midsection of the left pulmonary artery (41%). Initial pulmonary artery intervention was balloon dilation in 59% of patients and stent implantation in 41% of patients. Half of patients with initial balloon dilation required reintervention. CONCLUSIONS: There is significant risk of left pulmonary artery compromise after the second stage of hybrid palliation associated with a high intervention rate.