Background In the Single Ventricle Reconstruction (SVR) trial, one-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt (MBTS) in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups. Methods Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial. Results Transplant-free survival for the RVPAS vs. MBTS groups did not differ at 6 years (64% vs. 59%, P=0.25) or with all available follow-up of 7.1±1.6 years (log-rank P=0.13). The RVPAS vs. MBTS treatment effect had non-proportional hazards (P=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before Stage II surgery (HR=0.66; 95% CI 0.48–0.92). The effect of shunt type on death or transplant was not statistically significant between Stage II to Fontan surgery (HR 1.36, 95% CI 0.86–2.17, p=0.17) or after the Fontan procedure (HR 0.76, 95% CI 0.33–1.74, p=0.52). By 6 years, RVPAS patients had a higher incidence of catheter interventions (0.38 vs. 0.23/patient-year, P<0.001), primarily due to more interventions between the Stage II and Fontan procedures (HR=1.72, 95% CI 1.00–3.03). Complications did not differ by shunt type; by 6 years, one in five patients had had a thrombotic event and one in six, seizures. Conclusions By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS vs. MBTS groups. Children assigned to the RVPAS group had 5% higher transplant-free survival but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications. Clinical Trial Registration Registered with ClinicalTrials.gov number, NCT00115934 URL: http://clinicaltrials.gov/show/NCT00115934

Objective To determine whether a collaborative learning strategy derived clinical practice guideline (CPG) can reduce the duration of endotracheal intubation following infant heart surgery. Design Prospective and retrospective data collected from the Pediatric Heart Network in the 12 months pre- and post-CPG implementation at the four sites participating in the collaborative (active sites) compared to data from five PHN centers not participating in collaborative learning (control sites). Patients Data were collected for infants following 2 index operations: 1) repair of isolated coarctation of the aorta (birth-365 days) and 2) repair of tetralogy of Fallot (29–365 days). There were 240 subjects eligible for the CPG at active sites and 259 subjects at control sites. Measurements and Main Results After CPG implementation, the rate of early extubation at active sites increased significantly from 11.7% to 66.9% (p<0.001) with no increase in reintubation rate. The median duration of post-operative intubation among active sites decreased from 21.2 hours to 4.5 hours (p<.001). No statistically significant change in early extubation rates was found in the control sites 11.7% to 13.7% (p=0.63). At active sites CPG implementation had no statistically significant impact on median intensive care unit (ICU) length of stay (LOS) (71.9 hours pre- vs. 69.2 post-implementation, p=0.29) for the entire cohort. There was a trend toward shorter ICU LOS in the tetralogy of Fallot subgroup (71.6 hours pre- vs. 54.2 post-implementation, p=0.068). Conclusions A collaborative learning strategy designed CPG significantly increased the rate of early extubation with no change in the rate of reintubation. The early extubation CPG did not significantly change post-operative ICU LOS.

Objective To assess health-related quality of life (HRQOL) in a large multicenter cohort of children and young adults with Marfan syndrome (MFS) participating in the Pediatric Heart Network Marfan Trial. Study design The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales were administered to 321 subjects with MFS (5–25 years). PedsQL scores were compared with healthy population norms. The impact of treatment arm (atenolol versus losartan), severity of clinical features, and number of patient-reported symptoms (PRS) on HRQOL was assessed by general linear models. Results Mean PedsQL scores in children (5–18 years) with MFS were lower than healthy population norms for physical (P ≤ .003) and psychosocial (P<0.001) domains; mean psychosocial scores for adults (19–25 years) were higher than healthy norms (P<0.001). HRQOL across multiple domains correlated inversely with frequency of PRS (r=0.30–0.38, P<0.0001). Those <18 years of age with neurodevelopmental disorders (ND, mainly learning disability, attention deficit disorder and/or hyperactivity) had lower mean PedsQL scores (5.5–7.4 lower, P<0.04). A multivariable model found age, sex, PRS, and ND to be independent predictors of HRQOL. There were no differences in HRQOL scores by treatment arm, aortic root z-score, number of skeletal features, or presence of ectopia lentis. Conclusions Children and adolescents with MFS were at high risk for impaired HRQOL. PRS and ND, but not treatment arm or severity of MFS-related physical findings, were associated with lower HRQOL.

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Exposure to ozone at near ambient levels induced lung function effects, airway injury, and airway inflammation in older healthy adults. Clinical trial registered with www.clinicaltrials.gov (NCT01487005).