Application of a simple functional classification for aortic root pathology and aortic valve disease allows the logical application of 'valve-conserving' surgical procedures with excellent early and medium-term results.

There is a high incidence of VCP after arch repair via sternotomy. Laryngeal ultrasound seems to be an effective and noninvasive method for detecting VCP in neonates and young children.

Objectives: Pulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical intervention. We describe our experience with repair of this condition.Methods: From 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Children's Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients.Results: Operative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 AE 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic.Conclusions: Children with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis.

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SummaryOur understanding of human type 1 natural killer T (NKT) cells has been heavily dependent on studies of cells from peripheral blood. These have identified two functionally distinct subsets defined by expression of CD4, although it is widely believed that this underestimates the true number of subsets. Two recent studies supporting this view have provided more detail about diversity of the human NKT cells, but relied on analysis of NKT cells from human blood that had been expanded in vitro prior to analysis. In this study we extend those findings by assessing the heterogeneity of CD4+ and CD4-human NKT cell subsets from peripheral blood, cord blood, thymus and spleen without prior expansion ex vivo, and identifying for the first time cytokines expressed by human NKT cells from spleen and thymus. Our comparative analysis reveals highly heterogeneous expression of surface antigens by CD4+ and CD4 -NKT cell subsets and identifies several antigens whose differential expression correlates with the cytokine response. Collectively, our findings reveal that the common classification of NKT cells into CD4 + and CD4-subsets fails to reflect the diversity of this lineage, and that more studies are needed to establish the functional significance of the antigen expression patterns and tissue residency of human NKT cells.

Mortality was highest in patients who had preoperative pulmonary hypertension and those who were operated on before 1 year of age. Surgical technique did not influence the occurrence of pulmonary vein stenosis. Survivors were asymptomatic at the long-term follow-up.

Purpose: To evaluate the long-term outcomes following repair of truncus arteriosus and concomitant interrupted aortic arch.Methods: Between 1979 and 2016, 180 patients underwent repair of a truncus arteriosus. Twenty-two patients were identified with concomitant interrupted aortic arch, and were retrospectively reviewed.Results: Median age at repair was 45 days (mean 29 days, range 2 days to 164 days). Median weight at repair was 3.4 kg (mean 2.9 kg, range 1.2 kg to 3.6 kg). Interrupted aortic arch was type A in 4 patients, type B in 17 patients, and type C in 1 patient. Twenty-one patients underwent concomitant truncus arteriosus and interrupted aortic arch repair. Interrupted aortic arch repair was performed by end-to-side anastomosis in 19 patients, and by patch augmentation in 3 patients. Early mortality was 14% (3/22). There were no late deaths. Overall survival was 86.4 ± 7.3% (95% CI: 63.4, 95.4) at 30 years. Concomitant interrupted aortic arch was not associated with mortality. Six patients underwent 10 aortic reoperations. Freedom from aortic arch reoperation was 67.1 ± 11.2% (95% CI: 40.3, 83.9) at 30 years. One patient underwent balloon dilation of the aortic arch. Freedom from right ventricular outflow tract reoperation was 0% at 15 years. Follow-up was 95% complete (21/22), with median follow-up time of 19.4 years (mean 12.8 years, range 1 to 30 years). At last follow-up, mild residual aortic arch obstruction was identified in 1 patient, and all patients were in New York Heart Association Class I/II. Conclusion:Concomitant repair of truncus arteriosus and interrupted aortic arch is not associated with mortality and can be achieved with good long-term outcomes.

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