Background Re-coarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt (MBTS) or right ventricle-pulmonary artery shunt (RVPAS). We sought to determine incidence of re-coarctation, risk factors and outcomes in the SVR trial. Methods and Results Re-coarctation was defined by intervention, either catheter-based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed adjusting for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty; 39 surgical) for re-coarctation at median age 4.9 months (range: 1.1–10.5). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, re-coarctation was associated with the shunt type in place at the end of the Norwood procedure (HR 2.0 for RVPAS vs. MBTS, p=0.02), and Norwood discharge peak echo-Doppler arch gradient (HR 1.07 per 1 mmHg, p<0.01). Subjects with re-coarctation demonstrated comorbidities at pre-stage II evaluation including higher pulmonary arterial pressures (15.4±3.0 vs. 14.5±3.5 mm Hg; p=0.05), higher pulmonary vascular resistance (2.6±1.6 vs. 2.0±1.0 WU × m2; p=0.04) and increased echocardiographic volumes (end-diastolic volume: 126±39 vs. 112±33 ml/BSA1.3; p=0.02). There was no difference in 12-month post-randomization transplant-free survival between those with and without re-coarctation (p=0.14). Conclusions Re-coarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplant/mortality, further evaluation is warranted to evaluate effects of associated morbidities.

Contemporary community practice, procedural outcomes, and safety for 6 common congenital interventional procedures are reported. These benchmarks may be compared with individual center results and historical single-center and multicenter results.

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Percutaneous PDA closure during infancy is feasible and associated with few catastrophic AEs; however, the limitations constrain the interpretability and generalizability of the current findings.

Radiation dose in congenital cardiac catheterization varies by age and procedure type. A radiation monitoring and notification policy may have contributed to reduced radiation dose.

Although device closure of ASDs is associated with low morbidity and rare mortality, ongoing assessment of device safety profiles are warranted, and registries offer opportunities to facilitate the required surveillance.

Background As US healthcare increasingly focuses upon outcomes as a means for quantifying quality, there is a growing demand for risk models that can account for the variability of patients treated at different hospitals so that equitable comparisons between institutions can be made. We sought to apply aspects of prior risk-standardization methodology in order to begin development of a risk-standardization tool for the NCDR® IMPACT™ (Improving Pediatric and Adult Congenital Treatment) Registry. Methods and Results Using IMPACT, all patients undergoing diagnostic or interventional cardiac catheterization between January 2011 and March 2013 were identified. Multivariable hierarchical logistic regression was used to identify patient and procedural characteristics predictive of experiencing a major adverse event following cardiac catheterization. A total of 19,608 cardiac catheterizations were performed between January 2011 and March 2013. Amongst all cases, a major adverse event occurred in 378 (1.9%) of all cases. After multivariable adjustment, eight variables were identified as critical for risk-standardization: patient age, renal insufficiency, single-ventricle physiology, procedure-type risk group, low systemic saturation, low mixed venous saturation, elevated systemic ventricular end diastolic pressure, and elevated main pulmonary artery pressures. The model had good discrimination (C-statistic of 0.70), confirmed by bootstrap validation (validation C-statistic of 0.69). Conclusions Using prior risk-standardization efforts as a foundation, we developed and internally validated a model to predict the occurrence of a major adverse event following cardiac catheterization for congenital heart disease. Future efforts should be directed towards further refinement of the model variables within this large, multicenter dataset.

Background No prior reports documenting the safety and diagnostic yield of cardiac catheterization and endomyocardial biopsy (EMB) in heart transplant recipients include multicenter data. Methods Data on the safety and diagnostic yield of EMB procedures performed in heart transplant recipients were recorded in the Congenital Cardiac Catheterization Outcomes Project database at 8 pediatric centers over a 3 year period. Adverse events (AE) were classified according to a 5 level severity scale. Generalized estimating equation models identified risk factors for high severity adverse events (HSAE) (Levels 3-5) and non-diagnostic biopsy samples. Results A total of 2665 EMB cases were performed in 744 pediatric heart transplant recipients (median age 12 years [IQR: 4.8,16.7] and 54% male). AE occurred in 88 cases (3.3%), of which 28 (1.1%) were HSAE. AE attributable to EMB included tricuspid valve injury, transient complete heart block, and RBBB. Amongst 822 cases involving coronary angiography, 10 (1.2%) resulted in a coronary related AE. There were no myocardial perforations or deaths. Multivariable risk factors for HSAE included fewer prior catheterizations (p=0.006) and longer case length (p=<0.001). EMB yielded sufficient tissue for diagnosis in 99% of cases. Longer time since heart transplant was the most significant predictor of a non-diagnostic biopsy sample (p<0.001). Conclusions In the current era, cardiac catheterizations involving EMB can be performed in pediatric heart transplant recipients with a low AE rate and high diagnostic yield. Risk of HSAE is increased in early post-transplant biopsies and with longer case length. Longer time since heart transplant is associated with non-diagnostic EMB sample.